the tof child
DESCRIPTION
Guide for parents and carersTRANSCRIPT
The TOF
This book is dedicated to TOF children and their families,in memory of Benjamin Pouncey.
About this book:Co-ordinated and edited by Vicki Martin BSc BVScassistance from Sue Goodley (Nottingham Neonatal Service) and TOF parent Janita Clamp MA.
Illustration & design/layout by Vicki Martin, Andrea Slane
and Jeremy Dix of Vicki Martin Design
(www.vetdesign.co.uk). Published by TOFS, St George's
Centre, 91 Victoria Road, Netherfield, Nottingham NG4 2NN.Tel: 0115 961 3092 Fax: 0115 961 3097Email: [email protected] Web site: www.tofs.org.uk
First published in 19
ISBN 0 9536265 0 4
The TOF Child
This book is dedicated to TOF children and their families, in memory of Benjamin Pouncey.
About this book: ordinated and edited by Vicki Martin BSc BVSc, with
assistance from Sue Goodley (Nottingham Neonatal Service) and TOF parent Janita Clamp MA.
Illustration & design/layout by Vicki Martin, Andrea Slane
Jeremy Dix of Vicki Martin Design
(www.vetdesign.co.uk). Published by TOFS, St George's
Centre, 91 Victoria Road, Netherfield, Nottingham NG4 2NN.Tel: 0115 961 3092 Fax: 0115 961 3097 Email: [email protected] Web site: www.tofs.org.uk
First published in 1999
ISBN 0 9536265 0 4
Centre, 91 Victoria Road, Netherfield, Nottingham NG4 2NN.
1
The TOF Child
Discharge from Hospital ................ 37
Preparing for Discharge ............ 38
Equipment ............................... 39
Feeding .................................... 39
Care of Nasogastric Tubes ........ 42
Care of Gastrostomy Tubes ...... 43
Gastro-Oesophageal Reflux ....... 44
Tracheomalacia........................ 45
Medicines ................................ 45
Chest Infections ...................... 47
Physiotherapy .......................... 47
Additional Problems ................. 47
Follow Up ................................ 48
Genetic Counselling
for Tracheo-Oesophageal Fistula ... 49
What is it? ............................... 49
Why did it Happen? .................. 50
Will it Happen Again? ............... 50
Can Anything be Done
to Prevent a Recurrence? ......... 51
Feeding the TOF Child .................. 52
Breast Feeding ......................... 52
Weaning and Solid Foods ......... 52
Practical Tips on Feeding ......... 53
Reluctance to Feed ................... 54
Feed-Related Problems ............. 54
Troublesome Foods .................. 55
Feeding Difficulties .................. 55
Summary ................................ 57
Strictures and Reflux .................... 61
What is a Stricture? ................. 61
Symptoms of Stricture ............ 61
Diagnosis of Stricture .............. 62
Treatment of Stricture .............. 63
Complications of
Stricture Dilatation ................. 64
What is Reflux? ....................... 64
Symptoms of Reflux ................. 65
Diagnosis of Reflux .................. 66
Treatment of Reflux ................. 67
Contents
Introduction ..................................... 4
Editorial ........................................... 6
What is TOF / OA? ............................ 7
What is Wrong in TOF/OA? ........... 8
The Need for Surgery .................. 10
Diagnosis ................................... 10
Antenatal Diagnosis of TOF/OA
using Ultrasound ........................ 11
Conditions Occurring
with TOF/OA ............................... 14
Transfer to Another Hospital ...... 14
On Learning Your Child is a TOF ....15
Father ......................................... 16 Mother ........................................ 16
Coming to Terms with Reality .... 17
Post-Operative Period.................. 17
Prenatal Diagnosis ..................... 18
The Management of TOF/OA ........... 21
Specialist Information:
Transfer of the TOF/OA baby ……21
Pre-Operative Management .......... 21
Long-gap Oesophageal Atresia .... 24
Oesophageal
Substitution Procedures .................. 26
Colon Interposition .................... 27
Gastric Tube Oesophagoplasty .. 28
Gastric Transposition ................. 28
Post-Operative Care ..................... 29
Outlook ...................................... 29
The Post-Operative Care
of Children with TOF/OA ................ 30 Monitoring ................................. 30
Infusions or Drips ...................... 31
Ventilators .................................. 32 Chest Drains ............................... 33
The Chest Wound ........................ 33
Feeding ....................................... 33
Recovery ...................................... 34
Other Problems ............................ 34
2
Respiratory Problems in TOFs ........ 71
The Respiratory System ............... 71
Tracheomalacia ................................... 73 Infections .............................................. 77
Outlook in the Longer Term ........ 80
Growth in TOF Children ................ 82
Introduction .......................................... 82
Normal Growth ............................. 82 Factors Affecting Growth
in Children .......................................... 83
Growth of TOF Children.................... 83
Who to talk to if you are worried
about your child's growth .......... 85
The TOF Family .............................. 86
Initial Anxieties .................................... 86 Getting Help and Support ............... 86
Spreading the Load ....................... 87
What to Tell the Child? ...................... 88 The Child's Viewpoint......................... 89
Siblings ..................................................... 89
Play Therapy ................................. 90
Friends ........................................................ 91
Growing up .................................... 92
Moving on ................................................. 93 Summary ....................................... 94
Long Term Outcome;
Results of a Follow-up Study ......... 95
Feeding ............................................. 95
Chest Problems ............................... 95
Exercise, Education
and Lifestyle ............................................ 95
Height and Weight ........................ 96
Lung Function........................................
96
Summary ....................................... 96
VACTERL ......................................... 98
Diagnosis ................................... 98
Cause ........................................... 98 Antenatal Diagnosis .................... 99
Symptoms and Treatment ............ 99
The VACTERL Child .................. 103
Radial Deformities in VACTERL .. 106
Nomenclature............................ 106
The Forearm Anomaly:
Radial Club Hand. .................... 106 Treatment of the Foreall n ........ 107
The Hand Anomaly ................... 111
Treatment of the Hand .............. 112 Late Reconstruction
and Secondary Surgery ............ 115
Summary .................................. 115
The Role of the Professional;
Who to Ask for Help ....................... 116
Parents' Worries ............................ 120
X-rays ....................................... 120
Antibiotics and Anaesthetics .... 120
Paediatric Resuscitation ................ 121
Glossary ........................................ 124
Index ............................................. 127
3
Introduction by the TOFS group.
When the parents of a baby diagnosed
with tracheo-oesophageal fistula (TOF)
or oesophageal atresia (OA) are first
told of the problems which exist and
the operation(s) necessary to correct
any defects, they are not usually in a
suitable frame of mind to absorb all the
information given to them, let alone
understand the technical language.
As the news sinks in, questions arise;
some can be difficult to ask and some
will inevitably crop up at a time when
there is no one to respond. It is hoped
that many of these questions will be
answered in this book.
Later on, when their child leaves
hospital, parents are often told that there
should be no subsequent problems. This
can make them reluctant to go back to
the hospital with queries; however because
the condition is comparatively rare, few
General Practitioners have experience of
tracheo-oesophageal fistula (TOF) or
oesophageal atresia (OA). The lack of a
local source of advice can be frustrating —
or even frightening — for those involved.
This book contains contributions from
medical experts as well as from parents
and TOFs themselves. The hopes are
twofold; firstly that parents will benefit
from reading the book, but also that
medical professionals and nursing staff —
many of whom may not have encountered
many TOF children, if any at all — will
gain insight into the problems which can
occur in both the medical management
and the day-to-day care of a child with
TOF / OA or VACTERL (a group of
conditions which can include TOF/OA).
4
The TOF Child
oesophageal fistula (TOF)
because
rare, few
—
twofold; firstly that parents will benefit
—
have encountered
One of the questions which parents
may want to, but cannot or do not ask
is "what are the chances of my child's
survival?" The answer will naturally
depend on the number, type and severity
of the defects, but it is a fact that
nowadays virtually no child dies from
either tracheo-oesophageal fistula or
oesophageal atresia. There is always a
small risk involved in having an
anaesthetic, and that would apply for
any child, TOF or not. There may also
be other problems of a more serious
nature, such as a severe heart defect,
but a child with just TOF and/or OA has
excellent chances of survival.
And how well they can do! Vicki
Martin, who produced this book, was
Above:
Richard Briers, patron of the TOFS group.
One of the questions which parents
may want to, but cannot or do not ask
is "what are the chances of my child's
has
The TOF Child
born with TOF and OA. Having qualified
as a veterinary surgeon, she decided to
combine her knowledge with her artistic
talents to follow a career as a medical
illustrator and designer. She is a keen
sportswoman, and has ridden a bicycle
from Land's End to John O'Groats. At the
1989 TOFS Conference in Sheffield, two
other adult TOFs spoke; Andrew Wood,
the father of three healthy children
himself, and David Bland – who as
well as playing squash, also rock climbs,
hill walks and is a fanatical white water
canoeist. Vicki and David n o w r u n
t h e i r o w n s u c c e s s f u l businesses.
In the TOFS office, letters are regularly
received describing the achievements of
younger TOFs.
Children with TOF and/or OA are
harder work in the early years than a
normal healthy child, but after a few years
they usually improve to such an extent
that for most of the time both the
parents and the children forget that
anything was ever wrong.
The funding for this book has been
provided by the family of a TOF child,
Benjamin Pouncey – who at the age of
6, having overcome various problems
related to the TOF condition, tragically
died in a swimming accident. His family
set up a memorial fund, to be used by
the Tracheo-Oesophageal Fistula Support
group (TOFS) for a special project, and
it was agreed that the production of a
book would be appropriate.
This book is therefore dedicated to
TOF children and their families, in
memory of Benjamin Pouncey, with
many thanks to his family and friends.
`Our Son' by the parents of a TOF child
Our son was born i n ' 95
Nine weeks ear ly – lucky to be a l ive
Unable to swallow, he was critically ill
A major op' followed, we watched over him still
Twelve months on, he still cannot eat
He's fed by a pump, but we won't admit defeat
We long for the day when he'll have his first meal
And just can't imagine what rapture we' II feel
An anxious year over, he's still tiny in size
We care for him hourly, but he is our prize
His smile is electric, his heart so strong
He's so young and brave – it all seems so wrong
May '96 bring him only joy
For we love him to bits, he's our special boy
5
The TOF Child
Editorial Vicki Martin BSc, BVSc.
Although I had seen the occasional TOFS
newsletter, the first contact I really had
with TOFS was when I was asked to give a
talk about my life at their conference.
Armed with various slides depicting
myself from before my TOF/OA operation
through to my recent graduation
ceremony, I set out to give a rather
light-hearted account of my progress to
date.
My mother had often spoken of the lack
of support she had received in my early
years, but it was only through listening to
the other speakers and talking to parents
attending the conference that I came to
appreciate the implications of the
problem I was born with and the
importance of the TOFS group for
families. The presence of a source of
advice, support and reassurance does
alot to remove much of the sense of
frustration, despair and isolation that
seems to be all too common.
Once the shock of diagnosis has been
overcome, many of the difficulties faced
by TOF families are of a physical nature
e.g. feeding and respiratory problems.
This book will help parents by providing
factual information about these areas.
However, I hope it will also improve the
quality of life for TOF families by
removing much of the anxiety over the
condition – for after all, life is about much
more than just survival. Although the
early years with a TOF child may bring
their challenges, they will also contain
plenty of pleasurable moments as in any
'normal' family. If this book manages to
facilitate the latter, then my work will have
been successful.
I have learnt a lot in the course of
putting together this book; facts about the
condition, about other people and about
myself. The first was to be expected. The
second was a valuable bonus – sharing
personal moments with parents and other
TOFs, and gaining insight into others' lives
and attitudes. The last was in some ways
quite unexpected ... as a result of some
personal detective work to learn more
about the genetic implications of TOF/OA
and VACIERL, I requested a referral
appointment with the local genetics
service. They decided to run a few tests in
order to ascertain whether I had any parts
of the VACIERL syndrome, and to our
surprise we found that I have only one
kidney and an extra vertebra and pair of
ribs. Neither of these should cause me any
future problems whatsoever although it can
in certain situations be useful to know that
one has only a single kidney and I
therefore now wear a "MedicAlert"
bracelet. At the time I was born the various
tests to check for associated anomalies
were not routinely carried out when a
diagnosis of TOF/OA is confirmed, as they
are now.
My thanks and those of the TOFS group
go to the medical professionals who have
contributed to this book – for without them
the publication would not have been
possible, especially to Sue Goodley at
Nottingham Neonatal Service who helped
with the planning of the book as well as
giving me the chance to visit her Unit and
learn about the initial care of TOF babies.
Special thanks must also go to all the
TOFS members who shared their thoughts
and feelings, and to TOF parent Janita
Clamp who read through the many
responses and organised them into
appropriate sections as well as looking the
book over before going to press.
6
The TOF Child
What is TOF OA?
J .A.S . Dickson FRCS, FRCSE, FRCPCH , Consultant Paediatric Surgeon,
Sheffield Children's Hospital.
The story of oesophageal atresia can
reasonably be taken to start with this
description from Thomas Gibson's
`Anatomy of Humane Bodies
Epitomized' published in 1697:-
About November 1696 1 was sent for to an infant that would not swallow.
The child seem' d very desirous of food, and took what was offered it in a
spoon with greediness but when it went to swallow it, was like to be choked,
and what should have gone down returned by the mouth and nose, and it fell
into a struggling convulsive sort of fit but the next day died. The parents being
willing to have it opened, 1 took two physicians and a surgeon with me...We
blew a pipe down the gullet, but found no passage for the wind into the
stomach. Then we made a slit in the stomach, and put a pipe into its upper
orifice, and blowing, we found the wind had a vent, but not by the top of the
gullet. Then we carefully slit open the back side of the gullet from the
stomach upwards, and when we were gone a little above half way
towards the pharynx we found it hollow no further we began to slit it open
from the pharynx downward, and it was hollow till within an inch of the
other sl i t , and in the imperforate part of was narrower than in the
hollowed. This isthmus (as it were) did not seem ever to have been hollow,
for in the bottom of the upper, and the top of the lower cavity, there was
not the least print of any such thing, but the parts were here as smooth as the
bottom of an acorn-cup. Then searching what way the wind had passed when
we blew from the stomach upwards, we found an oval hole (half an inch
long) on the fore-side of the gullet opening into the aspera arteria* a
little above its fast division, just under the lower part of the isthmus above
mentioned.
* trachea or windpipe
This dramatic account very clearly
describes the commonest forms of
oesophageal atresia (OA) and tracheo-
oesophageal fistula (TOF).
7
The TOF Child
What is Wrong in TOF/OA?
The normal oesophagus
The oesophagus (gullet) is the passage
through which food moves on its route
from the mouth to the stomach. It starts in
the neck, just behind the larynx
(Adam's apple), and ends below the
diaphragm where it joins the stomach at
an acute angle.
Oesophageal atresia
The word `atresia' is taken from ancient
Greek and means 'no passage / no way
through.' Thus in oesophageal atresia there
is a break in the continuity of the
oesophagus. The end nearest the mouth is
not attached to the end which enters the
stomach, the gap usually occurring high up
in the chest. The presence of a blind-
ending pouch in the upper oesophagus
means that food is unable to reach the
stomach; any swal lowed mi lk or
sa l iva ins tead returns to the mouth.
The normal trachea
The trachea (windpipe) starts at the
larynx (the voice box, seen from the
outside as the 'Adam's apple' in the neck)
and passes in front of the oesophagus
before it enters the upper chest, where it
divides into two tubes, the main
bronchi, which go to the right and left
lungs.
Tracheo-oesophageal fistula
A fistula, from the Latin meaning 'a
pipe,' is an abnormal connection running
either between two tubes or between a tube
and a surface. In tracheo-oesophageal
fistula it runs b e t w e e n t h e t r a c h e a
a n d t h e oesophagus. This connection
may or may not have a central
cavity; if it does, then food within the
oesophagus may pass into the trachea
(and on to the lungs) or alternatively, air
in the trachea may cross into the
oesophagus.
Variations
I n ' A n A t l a s o f O e s o p h a g e a l
Atresia' the German surgeon Dietrich
Kluth describes 93 different variants of
these abnormalities. Fortunately only
five are at all common...
Atresia with lower pouch fistula
In 85% of cases the upper oesophagus
ends blindly, usually in the upper part of
the chest. The lower part starts at the
fistula from the trachea, which is located
near the point where it divides into the
main bronchi.
Atresia without fistula
In 8% of cases there is no fistula.
These babies are often more difficult
to treat because the gap between the
two ends of the oesophagus is usually
very wide and the stomach very small.
Fistula without atresia
Here the continuity of the oesophagus
is not affected, there being no atresia,
however there is a fistula joining the
oesophagus and the trachea. This variation
occurs in about 5% of cases and is often
called an 11 fistula,' although the fistula is
nearly always oblique and therefore more
like an 'N.'
Atresia with upper pouch fistula
Here the fistula runs from the upper
sac of the oesophagus to the trachea. This
is one of many variants making up the
remaining 2% of cases.
Double fistula
Occasionally there may be more
than one fistula present, with or
without an oesophageal atresia.
Laryngeal cleft
In this very rare condition the noimal
separation between the air passage and
the oesophagus fails to form, so that food
spills over into the windpipe on
swallowing.
8
Types of Oesophageal
Tracheo-Oesophageal Fistula
Oesophageal atresiawith lower pouch fistula
(most common type - 85%
(atresia)
Fistul
a
The TOF Child
Types of Oesophageal Atresia and
Oesophageal Fistula
Oesophageal atresia with lower pouch fistula
85%) Oesophageal atresia with upper pouch
fistula (less than 2%)
-1._ ` No separation of
upper airway and oesophagus
Laryngealcleft
The TOF Child
Laryngeal
9
The Need for Surgery
TOF/OA requires surgery for
correction of the defect(s);
i) the two ends of the oesophagus must
be joined together to allow the baby to
swallow food such that the nutrients it
contains can be digested and absorbed.
ii) any connection(s) with the trachea
m u s t b e c l o s e d o f f t o p r e v e n t
swallowed food / fluids passing from
the oesophagus into the lungs – also to
stop air passing from the trachea to the
oesophagus and then into the stomach.
Why Tracheo-Oesophageal Fistula has to be Treated...
1. With a tracheo- oesophageal
fistula, air can pass from the trachea to
the stomach, causing distension.
2. Food in the oesophagus may
also pass into the trachea and lungs,
causing pneumonia.
Presence of an atresia will affect
the relative likelihood of
these events.
Why Oesophageal Atresia has to be Treated...
1. With an oesophageal atresia, food
cannot enter the stomach and
therefore the baby has no means of gaining nutrition.
2. Unswallowed fluid may also spill
down the trachea into the lungs,
causing pneumon a.
10
The TOF Child
the two ends of the oesophagus must
be joined together to allow the baby to
swallow food such that the nutrients it
m u s t b e c l o s e d o f f t o p r e v e n t
Diagnosis Pre-
natal diagnosis
Oesophageal atresia may be
suspected before birth:
i) The inability of the baby to swallow
may lead to excess fluid collecting in
the womb in 20-25% of babies. This is
called hydramnios or polyhydramnios.
ii) In those babies with no fistula from
the lower oesophagus to the windpipe,
the stomach is also very small, there being
nothing passing into it to encourage it to
stretch and grow.
The combination of excess fluid around
the baby and a small or undetectable
stomach on a prenatal ultrasound scan is
therefore strong presumptive evidence
that the baby has this rare form of
oesophageal atresia with tracheo-
oesophageal fistula.
iii) The level of alpha feto protein (used
to detect spina bifida and abdominal
wall defects) in the mother's blood may
also be slightly raised.
Under these circumstances, an
amniocentesis test may be carried out,
to exclude the possibility of a
chromosomal (genetic) defect which
may be incompatible with the baby's
survival; examples of such defects include
Trisomy 13, 18 and 21. If these are
present the mother should be given the
option of an elective termination of the
pregnancy. Otherwise, the pregnancy
should be allowed to c o n t i n u e t o
t e r m a n d t h e b a b y delivered by the
normal vaginal route.
The TOF Child
The inability of the baby to swallow
may lead to excess fluid collecting in
25% of babies. This is
In those babies with no fistula from
the lower oesophagus to the windpipe,
the stomach is also very small, there being
The combination of excess fluid around
f alpha feto protein (used
may
amniocentesis test may be carried out,
include
the
the
delivered by the
Antenatal Diagnosis of TOF 10A
using Ultrasound
by Dr Alan Sprigg
When babies cannot swallow, excess fluid (known as polyhydramnios) accumulates around them in the womb.
This can happen in oesophageal atresia
particularly when there is no fistula. A distal fistula allows fluid a route to the stomach by providing a way for it to travel from the trachea to the oesophagus and on to the stomach, bypassing the atresia.
Polyhydramnios also happens in severe cases of cleft lip (hare lip) and palate but these conditions can usually be picked up on the scan.
Inability to swallow is also seen with brain problems, which can not be seen on a scan.
The other feature which is seen in scans of oesophageal atresia babies, a very small or absent stomach, has to be noted on several examinations to be significant.
Overall it is not possible to put a mathematical figure on the predictive value of these signs. In the commonest form oesophageal atresia with tracheooesophageal fistula, even in the presence of polyhydramnios, it is not possible to make an definite antenatal diagnosis by conventional methods.
Right:
Two scans of babies who were horn with
oesophageal atresia.
Both scans show the head to the right as
in the normal scan above them.
i) Neither scan shows a stomach
(compare with the normal scan above).
ii) The black area (fluid) around the
is greater than normal, indicating excess
fluid (polyhydramnios).
The TOF Child
TOF 10A
When babies cannot swallow, excess fluid (known as polyhydramnios) accumulates
This can happen in oesophageal atresia
particularly when there is no fistula. A distal fistula allows fluid a route to the stomach by providing a way for it to travel from the trachea to the oesophagus and on to the
Polyhydramnios also happens in severe cleft lip (hare lip) and palate but
these conditions can usually be picked up
Inability to swallow is also seen with brain problems, which can not be seen
The other feature which is seen in scans of y small or
absent stomach, has to be noted on several
Overall it is not possible to put a math-ematical figure on the predictive value of these signs. In the commonest form of oesophageal atresia with tracheo-
fistula, even in the presence of polyhydramnios, it is not possible to make an definite antenatal diagnosis by
Two scans of babies who were horn with
Both scans show the head to the right as
(compare with the normal scan above).
The black area (fluid) around the baby
excess
Above:
Normal antenatal ultrasound scan, with
the baby's head and body, bladder (B) and stomach (S) labelled. The black area
immediately above the baby is fluid.
The TOF Child
11
and stomach (S) labelled. The black area
Diagnosis after birth
With most TOF/OA babies, there is
no warning before birth that anything is
wrong. The main characteristic of
oesophageal atresia in a newborn baby
is the inability to swallow saliva –frothing
at the mouth and 'blowing bubbles.' Fluid
gaining entry to the trachea and lungs,
either through a tracheo-oesophageal
fistula or directly from the mouth down
the trachea, may cause problems with
breathing with the baby making
exagerated efforts to breathe and/or
showing blueness of the lips and finger
tips. Any air passing into the stomach
from the trachea via a tracheo-oesophageal
fistula (often as a result of the respiratory
movements) may also distend the tummy.
If an oesophageal atresia is
suspected, the passage of a tube down
the oesophagus from the mouth will either
confirm the diagnosis (if it is held up in
the blind-ending sac) or exclude it (if the
tube reaches the stomach). It is
important that the tube used is a
reasonably stiff one; too fine a tube
may curl up in the upper pouch and give
a misleading result.
Radiographs (X-rays) of the chest are
essential to see where the tube is being
held up and to examine the lungs and
heart. Radiographs of the abdomen may
also be taken; the presence of air in the
stomach means that the lower oesophagus
is attached to the trachea (i.e. a tracheo-
oesophageal fistula).
Radiographs using barium to further
examine the oesophagus are not usually
necessary. Once the diagnosis has been
confirmed, many surgeons will start the
operation by examining the trachea and
oesophagus using endoscopy (i.e.
bronchoscopy and oesophagoscopy) to
identify the length of the upper
oesophagus and position of any fistulas.
12
Diagnosis of Oesophageal Atresia
is
frothing
lips and finger
oesophageal
either
held up in
and give
oesophagus
Radiographs using barium to further
examine the oesophagus are not usually
necessary. Once the diagnosis has been
confirmed, many surgeons will start the
operation by examining the trachea and
In the absence of
oesophageal atresia,
a tube will pass freely down the
oesophagus to the
stomach.
If the tube will not pass beyond a small distance from the mouth, an oesophageal
atresia is suspected...
...it is very important to use a stiff tube because a flexible one might coil up in an upper pouch, giving misleading results.
Above right:
Radiograph taken looking from the side with the baby facing to the left. The jaws and base of the skull is seen at the top, the
ribs show as horizontal stripes in the chest, and the vertebrae of the spine appear as blocks to the right. The main features are: i) There is air in the stomach (the dark oval shape near the base of the rib cage).ii) A tube has been inserted through the mouth and down the oesophagus. It has been held up at the site of the atresia. These findings indicate that the baby has an oesophageal atresia and a distal tracheo-oesophageal fistula.
Below left:
Radiograph of the chest of a TOF 10A
baby, looking from the front. The ribs
show as horizontal stripes; the spine and
sternum are superimposed in the midline.
i) Barium (a so-called 'contrast agent'
which appears white on a radiograph) has
been given to the baby to swallow; it has
collected in the upper pouch of an atresia
(a little way down from the top, centrally).
ii) The stomach is very distended with air
(the large black circle, bottom right).
The TOF Child
Radiograph taken looking from the side with the baby facing to the left. The jaws and base of the skull is seen at the top, the
There is air in the stomach (the dark oval shape near the base of the rib cage).
A tube has been inserted through the mouth and down the oesophagus. It has
These findings indicate that the baby has
show as horizontal stripes; the spine and
sternum are superimposed in the midline.
te on a radiograph) has
been given to the baby to swallow; it has
collected in the upper pouch of an atresia
(a little way down from the top, centrally).
The stomach is very distended with air
Above:
Radiograph of the chest from the front showing the inadvertant and undesired outcome of a contrast swallow. The contrast agent is in the respiratory tract, highlighting the two main bronchi which branch into smaller airways in the left and right lungs. The contrast agent has also gained access to the stomach however, through a fistula. This is not a standard procedure because contrast agent is not good for the lungs.
The TOF Child
13
The TOF Child
Conditions Occurring with TOF/OA
Other associated congenital
abnormalities may also be detected in
the abdominal radiograph.
The commonest are gut problems either
in the rectum (the last part of the large
bowel) and/or in the duodenum (the part
of the small bowel next to the stomach).
These have all been successfully corrected
even in very small babies. Rarely all
three are seen in the same baby.
It is usual to examine the heart and
kidneys using ultrasound scanning;
defects in these important organs are
common because of the associations
between TOF/OA and two groups of
abnormalities – VATER syndrome (more
recently termed VACTERL) and
CHARGE.
The letters VATER stand for Vertebral
(spine), Anal (backpassage), Tracheal,
Esophageal (from the American spelling;
the UK spelling is `oesophageal'), Renal
(related to the kidney) and Radial (the
radius bone in the forearm); the extra
letters in VACTERL stand for Cardiac
(affecting the heart) and Limb (since
there are other limb abnormalities other
than those involving only the radius).
There are separate chapters on
VACTERL in this book which give
further details.
The CHARGE association includes
Coloboma (defects affecting the pupil
of the eye), Heart disease, Atresia of
the choanae (the passages at the back of
the nose), Retarded growth, Genital
hypoplasia (underdevelopment of the
genital organs) and Ear anomalies/
deafness.
Transfer to Another Hospital
The TOF/OA condition requires
prompt surgical intervention, so where
the diagnosis is suspected before birth
it may be decided to arrange delivery
in a special maternity unit which is in
close contact with a paediatric surgical
centre.
If this is not possible, it should not
cause a problem – for example, many
years ago a baby born in Hong Kong
was transferred to London for the
operation, and arrived in excellent
condition. As soon as the diagnosis has
been confirmed, transfer to a specialist
paediatric surgical unit is arranged,
accompanied by suitably qualified staff
(this is discussed further in the next
chapter). Since the mother is rarely able to
accompany the baby, a sample of her
blood will be also required for cross-
matching before the operation.
Parents should be able to see and touch
their baby before transfer, and the
father allowed to go with the baby in
the ambulance. Polaroid photographs are
often taken, and copies given to both
parents. Arrangements for mother to
join her baby depend on her health and
how well the receiving hospital is able
to look after her; some hospitals are
reluctant to look after mothers before the
eighth day after delivery.
14
The TOF Child
On Learning Your Child is a TOF
Dr Lorraine Ludman BSc(Hons) PhD, a Chartered Psychologist, is a Research
Fellow in the Department of Paediatric Surgery, and Honorary Research Fellow in
the Behavioural Sciences Unit at the Institute of Child Health in London. She has worked
as a teacher, music therapist, and then as a researcher in psychology at Great
Ormond Street Hospital and the Institute of Child Health. Her main research interest has
been concerned with infants who require major neonatal surgery for congenital
anomalies; she has studied the psychological effects – social, emotional and
intellectual, on the developing child, as well as the effects on the family as a whole.
Recent work includes a major study of children and adolescents treated for anorectal
malformations, and evaluation of therapeutic intervention with children who have
anorectal malformations and Hirschsprung' s disease. She also lectures part-time in
Developmental Psychology and Health Psychology.
The birth of a baby with a congenital
abnormality such as tracheo-
oesophagea l f i s tula /oesophageal
atresia (TOF/OA), which has not been
identified prenatally, and is therefore
totally unexpected, comes as a great shock
to parents. The first question many
parents ask immediately after the
birth, is "is the baby all right?" To be
told that the eagerly anticipated joyful
event – the birth of a perfect baby – has
not been fulfilled, is very frightening.
Weary and emotionally sensitive after the
physical exertion of giving bir th, to
f ind that the baby needs major
surgery, must be like the worst kind of
nightmare. This crisis, this 'threat' to
their new precious baby, precipitates a
range of responses in different individuals
– grief, disbelief, dismay, numbness and
confusion.
At first, many parents find it difficult
to understand or take in what was
happening. The world probably seems
unreal and in a turmoil.
"How can this be happening to me?"
"What did I do?"
"Who is to blame?"
"Will my baby die?"
"Will (s)he be 'normal'?"
... are questions voiced or thought
about at the time.
Despite the turmoil however, decisions
have to be made. Because s u r g e r y
n e e d s t o b e c a r r i e d o u t urgently
and hospitalisation is obligatory, the baby
should be transferred to a hospital
specialising in neonatal surgery, unless
there is a neonatal surgical unit in the
hospital where the baby is born. Parents
are asked to give consent for the
surgery and some may even be asked
whether they want to have the baby
christened before the transfer.*
After the initial shock, the role of
each parent generally differs and this
carries with it different stresses.
*If specialist treatment is possible in the hospital where the baby is born, parents will not experience
all the difficulties mentioned in the next section — however, the infant will undergo surgery soon
after birth, and will initially be cared for in an intensive care unit (ICU). Separation of the baby
from the parents is therefore inevitable.
15
The TOF Child
Father
Depending on her physical
state the mother will generally
remain in the maternity unit after
the baby's transfer. On the other
hand the father may well
accompany the baby to the
specialist hospital. His roles of
keeping the mother informed
about the baby and giving her
reassurance are very important in
helping her cope with the
mixture of emotions she
experiences — loneliness, fear,
sadness, anxiety, impatience,
self-blame, anger — as well as
dispelling any irrational thoughts
about the reasons for the baby's
defect.
On arrival, he may have had the
opportunity to meet a member of the
surgical team who would have been
able to give a general explanation about
the baby's abnormality. A more complete
assessment of the baby's condition and
prognosis has to wait until the results
from various investigations become
available and the baby has been seen by
a senior member of the surgical team.
During this initial difficult period
the father may have had the added
responsibility of looking after the
couple's other children, or making
arrangements for them to be looked after,
as well as taking care of household and
family matters. It is quite common for the
effects of the emotional upheaval and the
daily stresses and strains that fall upon
fathers to be underestimated and
overlooked. Moreover, the father may
feel it is unmanly to express his feelings
and anxieties and may be reluctant to
discuss them either with his partner or
with family and friends.
Mother
After the baby's transfer many mothers
have to come to terms with being without
their baby. They may become very
anxious and harbour secret fears —
will they be able to love their baby
despite his or her faults? Will they
fail to 'bond' because they have been
separated from their baby? They should be
reassured however. Contrary to a
widespread emphasis in the popular
press and academic literature in the 70's
and 80's on the importance of bonding
and attachment — an 'instantaneous
gluing' which was thought to happen in
the first few minutes after the birth —
there is no strong supporting evidence that
this o c c u r s , o r i n d e e d i s a u s u a l
o r necessary response for the
development of a loving mother-child
relationship. We know that the
development of the bond with the baby
usually occurs over the course of time,
and that this may vary f rom a few
hours or days to several months.
Moreover, separation by itself is not
likely to be the cause of disturbed mother-
child relationships.
16
The TOF Child
Coming to Terms with Reality
After the initial shock, and once the
mother is over the delivery and is able
to visit the baby, life does not necessarily
return to normal. Having to leave the
maternity unit and return home without
her baby is a situation which can be very
traumatic for the mo t h e r . H e r
c i r c u ms t a n c e s a r e contrary to the
expectations of her friends and family
and she may find she is involved in
trying to explain a situation which she
herself does not fully understand. This can
become tiresome and distressful.
Relationships with those close to her, as
well as with acquaintances, can become
strained. Few can understand the situation
the parents are facing or the emotions the
parents are experiencing. Moreover, each
parent will react differently to the
` c r i s i s . ' H o w e v e r c l o s e t h e i r
relationship was before the birth, they
may find it difficult to understand their
partner's behaviour and reactions. Being
able to talk to each other about the i r
fears and anxiet ies is of ten helpful
in overcoming feelings of isolation and
stress. This can however take time, and
may require a lot of patience and mutual
understanding.
Post-Operative Period
Many mothers have told me that during
the early weeks after the initial surgery,
they functioned as if in a dream. Their
waking hours were often spent in the
intensive care unit or wherever the baby
was being cared for, and the world
around them appeared unreal. In addition,
hospitals and the hi-tech environment of
the intensive care unit were very
frightening for some parents. Getting to
sleep was often very difficult, and in
general they appeared to be existing in a
haze of
constant anxiety. Some mothers told
me that they felt guilty. They found it
very boring and depressing having to
spend most of the early days and weeks
after the initial operation(s) sitting by
their baby's cot. In spite of this, they
felt that they had to do what was
expected of them and were afraid to
discuss these feelings, in case they might
be considered potentially poor and
neglectful mothers. On the other hand,
some mothers became distressed if they
were not able to be with their baby all the
time, and were not given enough
opportunity to participate in their care.
A number of mothers told me that
sleeping at home in their own beds helped
them to feel more secure and more in
touch with reality. If they lived far from
the hospital, and had chosen t o s t a y
i n o r ne a r t h e s pe c i a l i s t hospital,
the ability to return home at least once
or twice a week was beneficial .
Conversely there were those who
would not be apart from the baby; if the
baby was in hospital for several weeks or
even months, this can put quite a strain
on the parental relationship. The father
might begin to feel left out and feel that
his needs (both emotional and physical)
were being ignored. Nevertheless, it is
wise to remember that this particular
problem (a mother's preoccupation
with her baby) is not unique to parents
with a sick baby, since similar problems
occur after the birth of a healthy child.
Many fathers will return to work
shortly after the birth. For some, this
will help as they will be distracted from
dwelling on the baby's problems. For
others, it will just be a further burden
which adds to an already stressful
situation. Those who live some distance
from the hospital may
17
find it impossible to visit the baby except
at weekends. Consequently the father may
begin to feel somewhat remote from his
partner's problems and anxieties; this can
increase feelings of guilt and be an
additional cause of strain between the
parents.
In conclusion, coping with a crisis of
this nature and magnitude will affect
individuals in different ways. Responses
will be determined by a multitude of
factors such as whether they are the
mother or the father, their age, family
and social circumstances, the way they
react to stress, and the severity of the
baby's condition. During the early
traumatic stages, it may help to know
that, given time, the nightmare fades. As
the baby recovers from the operation(s)
and is able to go
home to be
cared for by
his or her
parents, a
relatively
`normal'
pattern of
family life
will, in the
majority of
cases,
slowly
become
established.
Although
there may be
many crises
still to come,
many of the
parents interviewed told me that by the
time the baby was 10-12 months old they
could start to compare their baby with
others who had not had such a dramatic
introduction to life, and begin to feel
less afraid for their infant's future
development.
18
The TOF Child
it impossible to visit the baby except
at weekends. Consequently the father may
remote from his
and anxieties; this can
of
interviewed told me that by the
12 months old they
Prenatal Diagnosis
In the last few years, it has become
possible to identify a TOF baby before
birth at the time of ultrasound scanning.
Consequently, a small number of parents
may have prior warning that all is not well
with their baby and that he or she may
have OA. Mothers who develop
polyhydramnios in later pregnancy
may also be aware of an impending
abnormal infant.
How do parents cope with this? To
some extent, this may depend on whether
the TOF appears to be an isolated
problem, or part of a constellation of
anomalies. With support and advice from
very well informed professionals, and
possibly families who have been in similar
situations, these parents will have time
to come to terms with the
idea that the baby will not be
'perfect.' This period may
also provide time to
understand and acknowledge
some of the difficulties that
lie ahead. Since a high
proportion of TOF babies
have a very good chance of
surviving the birth and
subsequent corrective
surgery, parents may be less
anxious, and await the birth
relatively calmly. The
manner in which individuals
cope with stress of this nature
will however also depend on their
personality, social situation and the
quality of support from those close to
them.
Prenatal diagnosis may also mean
that the birth may be prearranged to
take place at a specialist centre,
overcoming any need for transfer of
the baby after birth.
The TOF Child
The TOF Child
Quotations from TOFs, their families and friends and medical professionals
"The most vivid memory of those blurred
early days was when Jacob w a s
w h e e l e d t o t h e a t r e i n h i s
incubator – so fragile and vulnerable,
and us as parents holding each other
and sobbing feeling helpless and useless. I
cry even now to think of it."
"To see doctors and nurses in the
background running around your
small child I think really brought home
what was really happening to us and
to Jamie as up until then I think we were
wandering about in a daze for the first
4 – 5 weeks, as we were travelling around
60 miles per day to the hospital. I think all
the running around you have to do
doesn't give you any time to think about
what is happening."
"I have never really felt 'why me?' I
believe about 1 baby in 3,000 has problems
like Colin' s, but statistics are meaningless
when your baby is the one. I did feel angry
when I had to go into the day room where
smoking was allowed, to find a phone. I
had been so careful when I was pregnant –
yet my baby had problems, and here were
these women puffing away, whose
babies were healthy. It didn't seem fair. It
also annoys me, even now, if parents
say they were disappointed to have a girl
instead of a boy, or vice versa. I say, as
politely as possible, that they should be
glad to have a healthy baby."
"Nicholas' older brother has a special
relationship with his granny who he had to
live with for some time. He also feels left
out at home because of the attention given
to his brother."
"One of the nurses said to me "Don't
worry – we get plenty of TOFs in
here." Strange as it seems now, at that time
I had never heard the term TOF,' I had
just been told there was a pouch in
Colin' s oesophagus. So I thought she
had said 'We get plenty of tofs in here' ,
meaning that they treated a lot of rich
kids and were therefore well-trained in
giving of their best. I thought this was a
very odd thing to say, and replied Tut he's
just an ordinary little b o y ' w h i c h s h e
m u s t h a v e f o u n d strange. It wasn't
until several days later that I realised what
she had said."
"The initial elation of an apparently normal
home delivery changed by the next day to
shock, anxiety and sorrow as severe
problems presented and prognosis for
Nicholas was poor. The major surgery over
many weeks seemed incredible for such a
tiny baby. The courage and support that
Mary and Paul gave to each other and the
continued care of Timothy (aged 3) was a
real example to all the family as they
waited to discover what the future held."
"I was frightened to begin with as the
child was so ill after birth. As I found
out more about the condition I became
less worried, but those first few months
were terrifying."
"My f irst reaction was horror and
panic. When Nicholas' physical internal
problems were explained to me I did not
expect him to survive. I was amazed at what
was surgically done for his repair and that
it was successful."
"The quote I remember from hospital
during his initial stay was "two steps
forward and one step back," at times it
seemed the numbers were reversed."
19
"Of course we were all devastated when
Thomas was born to find that he had
lots of problems. The children were
very upset, particularly Ben (8). He
suffered nightmares for about four months
after Thomas was born. Sonja (6) was
much more resilient. It took Ben a week
or two before he would hold Tom, but
Sonja was there just as soon as possible —
in hospital, holding Tom with all his
tubes. Ben and Tom are very close now.
Ben is away at Cardiff University and Tom
misses him dreadfully."
"We were very worried and afraid for
Jordan's life. We were horrified by him
not having a complete gullet and wanted to
know why our grandson? Why our
daughter? How?" Above:
Tom Dobbs awaiting surgery.
The TOF Child
The Management of TOF/OA
Professor L Spitz PhD FRCS, Nuffield Professor of Paediatric Surgery,
Institute of Child Health (University College London) and Great Ormond Street
Hospital for Sick Children, London.
21
Specialist Information: Transfer of the
TOFIOA baby
Following delivery of the baby it is essential that the airway is kept clear of secretions at all times and that aspiration pneumonia is prevented. This is best achieved by passing a large calibre naso-oesophageal tube of the Replogle type to which continuous suction is applied. If this type of catheter is not available then a similar sized nasogastric tube should be passed into the upper pouch and it should he intermittently aspirated using a hand syringe. The baby's mouth should also be kept clear of secretions by intermittent aspiration of the oral cavity.
The best position for the infant during transfer is supine or on the right side. The baby must he kept warm at all times and transferred in a portable incubator, accom-panied by a qualified nurse experienced in caring for small, at risk infants.
Vitamin K by injection may be given at the referral hospital but if administered this fact must be recorded in the transfer notes and referral letter Fluid administration in the, first few hours of life is not a requirement.
If the baby is premature and/or has breathing difficulties, such as respiratory distress syndrome, mechanical ventilation may be required during transfer. This must he carried out with extreme care as it may result in distension of the stomach which will further impair respiratory problems. It is clearly in the best interest of the baby that transfer should take place to a large neonatal surgical unit which is staffed by experienced paediatric surgeons and by fully trained paediatric intensive care
nurses. Units which regularly treat infants
Pre-Operative Management
Emergency surgery for the infant with
oesophageal atresia is no longer necessary.
Indeed, a period of preoperative
stabilisation before submitting the baby
to a major operation will facilitate the
postoperative recovery. The infant 's
temperature should be kept within the
normal range and any fluid or
electrolyte imbalance should be
corrected before surgery. The operation
can safely be postponed until the
daylight hours rather than having to
be carried out in the early hours of the
morning when the surgeon may not be
at his or her best, when theatre staffing is
at emergency levels and when the ward
may have a reduced complement of
experienced nurses. However, the lack of
availability of a regular operating theatre
may dictate that the surgery has to be
undertaken at night rather than waiting
until the following evening.
The only clear exception to this is the
infant who has respiratory difficulties. It
is particularly the premature infant with
respiratory distress syndrome who is at
risk. The baby may require mechanical
ventilation because of the "stiff lungs"
and in the presence of a distal tracheo-
oesophageal fistula, the high pressures
necessary to-keep lungs inflated may
cause a significant proportion of the
inspired gases to escape down the fistula
into the stomach. The stomach becomes
increasingly distended, worsening the
respiratory status, and
the stomach itself may eventually
rupture resulting in a catastrophic
situation which is immediately life-
threatening. We have found that the
best method of managing this
sequence of events is to undertake an
emergency operation to ligate the fistula. If
the infant's respiratory status dramatically
improves, the surgeon may be happy to
continue with the p rocedure , bu t
more usua l l y t he infant's condition
is so unstable that the operation is
best left at this stage and the repair
postponed for a few days when the
problem with the lungs should have
resolved.
Another circumstance which may delay
surgery or dictate a different approach is
where the infant has a major congenital
heart abnormality. Detailed investigations
of the precise anatomy of the heart will be
required (using echocardiography) and
medical treatment for cardiac failure
instituted. In rare circumstances, where
medical treatment is unsuccessful, cardiac
surgery may be advisable before correction
of the oesophageal atresia in order to give
the baby its best chances of survival.
Surgery:
Primary Anastomosis
The aim of surgery for oesophageal
atresia is to divide the tracheooesophageal
fistula and to perform a `primary
anastomosis,' joining the two ends of
the oesophagus dur ing the initial
operation.
Occasionally, primary anastomosis
w i l l n o t b e p o s s i b l e a t t h e f i r s t
operation because the gap between the
two ends of the oesophagus is too
wide. Surgery in these cases is described
later in this chapter, under long-gap
oesophageal atresia.'
22
The TOF Child
emergency operation to ligate the fistula. If
infant's respiratory status dramatically
infant's condition
Another circumstance which may delay
heart abnormality. Detailed investigations
of the precise anatomy of the heart will be
medical treatment is unsuccessful, cardiac
surgery may be advisable before correction
order to give
oesophageal
ends of
w i l l n o t b e p o s s i b l e a t t h e f i r s t
operation because the gap between the
two ends of the oesophagus is too
Passage of gases into stomach during mechanical respiration in
a TOF baby with stiff lungs due to respiratory distress syndrome
Preparation
Following pre-operative assessment
and stabilisation, the infant is prepared
for the operative correction of the
oesophageal atresia.
Consent for the procedure is obtained
and the risks of the operation and the
chances of the baby's survival are
carefully explained to the parents.
Upper pouch (atresia)
Fistula
Gases from mechanical respiration pass into stomach
Enlarged stomach
Risk of stomach rupture
Alleviation of pressure in the stomach by ligation of the fistula
Upper pouch (atresia)
Fistula 'tied off'
Gases confined to
airways
The TOF Child
in
Gases from mechanical
pass into
confined to
We have recently proposed a new
classification scheme whereby if the
infant 's birthweight is greater than
1500 grams and there is no evidence of
major congenital heart anomaly, the
chances of survival are close to 100%.
the baby weighs less than 1500 g r a m s a t
b i r t h o r h a s a m a j o r congenital
heart anomaly, the chances of survival
are reduced to 60%. If the baby's weight
is below 1500 grams and there is a
major heart anomaly, the chances of
survival are much further reduced to around
20%.
The baby is then taken to the operating
theatre where anaesthesia is induced by an
experienced paediatric anaesthetist. The
procedure is carried out under general
anaesthesia with continuous and close
monitoring of all vital functions (i.e.
heart rate, electrocardiogram, blood
oxygen saturation level, body
temperature).
Incision
The usual incision is along a curved
line on the right side of the chest close
to the back to just under the tip of the
shoulder blade. It should not extend close
to the nipple.
Positioning and Incision Site for Surgery
Arm placed alongside
head
Shoulder blade
Procedure
The chest is entered between the
fourth and fifth ribs and without opening the membrane which encloses
the lung, the area of the oesophageal atresia
is approached. The distal tracheo-
oesophageal fistula is
Line of incision
The TOF C
Surgery for TOF/OA
Division oftracheo-
oesophagealfistula
Oesophagus Trachea
Closure oftracheal end
of fistula:tube in
upper pouch
Sutures beingplaced for theanastomosisbetween theupper pouch
and distal(lower)
oesophagus
Continuityrestored:
transanastomotictube inserted
Diaphragm
Transanastomotictube
Stomach
We have recently proposed a new
infant 's birthweight is greater than
1500 grams and there is no evidence of a
chances of survival are close to 100%. If
g r a m s a t
congenital
of survival
baby's weight
there is a
ced to around
The baby is then taken to the operating
theatre where anaesthesia is induced by an
anaesthetist. The
out under general
continuous and close
.e.
The usual incision is along a curved
line on the right side of the chest close
to the back to just under the tip of the
shoulder blade. It should not extend close
opening the membrane which encloses
the lung, the area of the oesophageal atresia
The TOF Child
Division of -
oesophageal fistula
Closure of tracheal end
of fistula:
upper pouch
Sutures being placed for the anastomosis between the upper pouch
and distal
oesophagus
Continuity
transanastomotic tube inserted
Diaphragm
Transanastomotic
23
carefully identified and traced towards
its entry into the trachea, where it is
divided and the defect in the trachea
closed with fine interrupted sutures.
The proximal (upper) oesophagus is
now identified with the help of the
anaesthetist applying pressure on the
tube in the upper oesophageal pouch.
The upper pouch is mobilised sufficiently
to allow anastomosis (joining) to take
place with the least possible tension. If
there is significant tension at the
anastomosis, the infant may be electively
paralysed and mechanically ventilated (i.e.
put on a life-support machine') to reduce
the likelihood of a leak in the anastomosis.
The anastomosis is performed using
a single layer of full thickness fine
interrupted sutures. The anastomosis
should be watertight.
Transanastomotic tube
Most surgeons will pass a trans-
anastomotic tube (trans = across;
anastomosis = join) from the nose through
the oesophagus into the stomach to remove
any pressure in the stomach during the
first few days after the operation, and to
allow feeding to o c c u r o n t h e
t h i r d o r f o u r t h postoperative day.
Chest drain
Some surgeons leave a chest drain
in position for up to one week, others
have omitted this as it does not appear
to be of any great value in detecting an
anastomotic leak (a defect in the join
that allows oesophageal contents to leak
into the surrounding tissues).
Closure
The wound is closed in layers, with a
subcuticular suture to the skin (a stitch that
is buried in the skin, underneath the
surface). This type of stitch does not
require removal.
24
The TOF Child
carefully identified and traced towards
The proximal (upper) oesophagus is
The upper pouch is mobilised sufficiently
may be electively
paralysed and mechanically ventilated (i.e.
the likelihood of a leak in the anastomosis.
The anastomosis is performed using
anastomosis = join) from the nose through
the oesophagus into the stomach to remove
the operation, and to
Some surgeons leave a chest drain
have omitted this as it does not appear
leak
a
subcuticular suture to the skin (a stitch that
Long-gap Oesophageal Atresia
Sometimes the gap between the two
ends of the oesophagus is too large to
bridge immediately (long-gap
oesophageal a t res ia ') and in these
cases a gastrostomy is performed.
A gastrostomy is a tube placed
through the wall of the abdomen (on
the left side) and passes into the
stomach to allow the baby to be fed.
After dividing the TOF, the distal
( lower) oesophagus is c losed off ,
unless it is already blind, and a tube
left in the upper pouch which is either
left attached to a suction machine or
manually aspirated at regular intervals,
to prevent overflow of saliva into the
lungs. This latter tube needs careful
and meticulous nursing care.
The gap between the upper and l o w e r
e n d s o f t h e o e s o p h a gu s i s assessed
radiologically at around six weeks to
ascertain whether a delayed primary
anastomosis is feasible. A delayed
pr imary anastomosis is a direct
joining of the two ends of the oesophagus
which has been delayed to allow the
oesophagus a little further time in which
to lengthen.
The TOF Child
oesophageal a t res ia ') and in these
unless it is already blind, and a tube
manually aspirated at regular intervals,
lungs. This latter tube needs careful
assessed
time in which
Above:
Detail of gastrostomy tube showing how
the tube is attached
Gastrostomy Tube
Upper pouch(atresia)
with tubeinserted
Gastrostomytube
Front surface of the stomach is sutured to the abdominal wall
Stomach
The TOF Child
Detail of gastrostomy tube showing how
A further radiological study may be
carried out at 12 weeks but delaying
further than this is unproductive and
child should be submitted to an
oesophageal substitution procedure rather
than persisting with the
intention of perfot ining a delayed
primary anastomosis after 12 weeks
(see next chapter).
If the gap between the ends of the
oesophagus is excessive (for example,
greater than the length of six vertebrae
when viewed on a radiograph), the
surgeon may decide not to wait the six
to twelve weeks before reassessment
as described above, and will instead
proceed immediately to the procedures
related to oesophageal substitution.
Gastrostomytube
The TOF Child
A further radiological study may be
further than this is unproductive and the
oesophageal substitution procedure rather
ining a delayed
y anastomosis after 12 weeks
oesophagus is excessive (for example,
greater than the length of six vertebrae
surgeon may decide not to wait the six
re reassessment
proceed immediately to the procedures
25
Oesophageal
Substitution ProceduresProfessor L Spitz PhD FRCS, Nuffield Professor of Paediatric Surgery,
Institute of Child Health (University of London) and Great Ormond Street
Hospital for Sick Children, London.
There are occasions when a primary
anastomosis (join) of the oesophagus,
either immediate or delayed, may be
considered an impossibility due to the
gap being too wide.
Under these circumstances the baby
is encouraged to thrive until old enough
(around 6-9 months) to undergo an
oesophageal substitution procedure. This is
an operation in which an alternative part
of the gastro-intestinal tract is utilised to
bridge the gap between the two ends of the
oesophagus, so substituting for the
normal oesophagus as a 'pipe' between
mouth and stomach.
During the months before this can be
carried out, the baby needs to gain the
experience of food in his/her mouth, to
learn to chew and swallow, and to
associate that with the feeling of
satisfaction at having been fed. Learning
these actions and associations later on,
after replacement surgery, is very
much harder to achieve.
It is therefore necessary to bring the
end of the upper oesophagus out in the
neck as a cervical oesophagostomy
and to give the infant a feeding
gastrostomy. If there is an associated
tracheo-oesophageal fistula, it will require
division and closure.
Feeding is then provided by the
g a s t r o s t o m y a n d t h e b a b y i s
encouraged to suck and take small
amounts of feed by mouth (`sham feeding,'
described in the next chapter) even though
this will not provide any
26
The TOF Child
Substitution Procedures Professor L Spitz PhD FRCS, Nuffield Professor of Paediatric Surgery,
Institute of Child Health (University of London) and Great Ormond Street
Under these circumstances the baby
oesophageal substitution procedure. This is
alternative part
ilised to
bridge the gap between the two ends of the
During the months before this can be
the
after replacement surgery, is very
end of the upper oesophagus out in the
neck as a cervical oesophagostomy
oesophageal fistula, it will require
a n d t h e b a b y i s
amounts of feed by mouth (`sham feeding,'
described in the next chapter) even though
Cervical Oesophagostomy and Gastrostomy
nourishment and will soil the neck at
the site of the oesophagostomy. Failure
of the baby to sham feed can cause major
swallowing difficulties to occur when the
oesophageal substitution is eventually
carried out.
Surgery for additional abnormalities
may be necessary around the time of
the birth. These may be correction of a
duodenal atresia or an anorectal
malformation where either a
colostomy is performed or a perineal
operation is carried out to create an
anal orifice.
Sham feed
Cervical oesophagostomy
Gastrostomy tube
The TOF Child
the site of the oesophagostomy. Failure
oesophagostomy
There are various options for
oesophageal substitution surgery:
i) colonic interposition
ii) gastric tube oesophagoplasty
iii) gastric transposition
iv) jejunal interposition
v) gas t r i c e l onga t i on The first
three are the most widely used. Jejunal
interposition is rarely
used because of the precarious blood
supply to the interposed jejunal
segment. Gastric elongation by
dividing the lesser curvature of the
stomach has been proposed by Scharli
but is not widely employed.
Colon Interposition
A section of colon is taken from its
normal location in the gut, and
transposed with its blood supply intact
into the chest, where it is joined to the
oesophagus above and the stomach
below.
The Gastro-Intestir
Tract with Long-Gap OA
Oesophagus (gullet)
Diaphragm
Duodenum
Jejunum and ileum
Colon
Rectum
Anus
Caecum
(appendix) •
The TOF Child
three are the most widely used. Jejunal
stomach has been proposed by Scharli
transposed with its blood supply intact
This is still the most widely used
procedure.
Advantages
i) The length of the graft required is
not a problem, unlike other options.
ii) Provides a tube of a good diameter.
Disadvantages
i) Blood supply to the transplanted
section of colon is precarious.
ii) Poor peristalsis (the muscular action
which transports food down the tube).
iii) High incidence of leakage
(30% of patients).
iv) Stricture (narrowing) can occur
(20% of patients).
(v) Redundancy (lack of any muscular
activity) can develop long term.
Colon Interposition
Colon
Blood supply
Upper end of oesophagus
Colon
Rectum
Anus
SuturesTransposed
colon
Sutures
Lower end of
oesophagus
Stomach iv
Blood supply to colon
It is then placed in position, between the two ends of the oesophagus,
and sutured in place.
A section of colon is removed from its usual situation,
together with its blood supply...
The TOF Child
The length of the graft required is
Provides a tube of a good diameter.
Poor peristalsis (the muscular action
Stricture (narrowing) can occur
(v) Redundancy (lack of any muscular
Colon
Blood supply
Sutures
27
The TOF Child
Gastric Tube Oesophagoplasty
A longitudinal segment is taken from
the stomach, which is then swung up into
the chest and joined to the oesophagus.
Advantages
i) Size of the graft is appropriate.
ii) Good blood supply.
Disadvantages
i) Very long suture-line.
ii) High incidence of leakage (70% of
patients).
iii) High stricture rate (50% of
patients).
iv) Reflux commonly occurs.
Gastric Transposition
The whole stomach is freed, mobilised
and transposed into the chest. The upper
end of the oesophagus is then
anastomosed to the top of the stomach in
the neck.
This is a relatively new technique
and its long-term effectiveness is
therefore not yet proven.
Advantages
i) Excellent blood supply.
ii) Incidence of leakage and strictures
both reduced to 6% of patients.
iii) Relatively simple procedure.
Disadvantages
i) Poor gastric emptying.
ii) Bulk of the stomach is in the chest;
the sheer volume of this affects breathing
so that respiratory capacity is reduced.
iii) Reflux can be a problem.
iv) 'Dumping' occurs when food enters
the intestine quite quickly. It causes
sweating, dizziness and diarrhoea. It
usually lasts only a few months and then
disappears.
28
The TOF Child
Post-Operative Care
Oesophageal substitution, irrespective
of the technique used is a major operation
requiring technical skill, expert
anaesthesia and high level intensive care
post-operatively.
Elective paralysis and mechanical
ventilation (using drugs to inhibit
breathing so that respiration has to be
taken over by machines) for a few days
post-operatively are generally
recommended; this puts less stress on
the suture lines and therefore gives them a
better chance to heal.
The commencement of oral feeding may
be a difficult and prolonged process,
particularly if sham feeding was
neglected. For colon interposition and
gastric tube oesophagoplasty patients,
gastrostomy feeds can be used as a
'backup' until oral feeding is established.
For gastric transposition patients, the
stomach itself has been moved, so a
gastrostomy tube is not an option. A
jejunostomy tube (a tube into the
jejunum, which is a part of the gut a
little way below
the stomach)
is therefore
usually
inserted for
feeding
purposes.
Outlook
Unfortunately, long-term
complications must be anticipated in
all replacement procedures.
For colonic interposition, strictures at
the anastomotic site early on, and
redundancy and food stasis in the long
term are well known problems.
The gastric tube oesophagoplasty is
prone to stricture formation and reflux
of acid content into the upper oesoph-
agus. This may produce inflammatory
changes (Barrett's oesophagitis).
The gastric transposition may be
complicated by delayed gastric emptying
and dumping in the short term, and iron
deficiency in the long term (probably
caused by food moving swiftly
through the part of the gut where iron
absorption occurs, so that there is little
opportunity for uptake).
Should the chosen technique for
oesophageal replacement fail or be
unsuitable, one of the alternative
methods should be implemented.
Reduced respiratory function is a
feature of all oesophageal
replacements but generally the
children cope extremely well.
Left:
After a primary anastomosis
failed, Emma Louise Dodd was
given a cervical oesophagostomy
and gastrostomy, and was sham
fed for many months before a
colon interposition was
performed. She has also under-
gone surgery for scoliosis.
She is shown here aged 19 years,
a University student who lives a
full and happy life. Swallowing is
not entirely trouble-free, however
she has been discharged from all
the hospitals she was attending.
29
The TOF Child
The Post-Operative Care
of Children with TOF/OA RG Buick, Consultant Paediatric Surgeon and AD Lander, Senior Lecturer in
Paediatric Surgery, Birmingham Children's Hospital NHS Trust.
Most babies with OA and TOF will be
operated on within the first few days of
life. Before the operation, some parents
have the opportunity to see the ward
where the baby will be recovering and
to meet the key medical staff who will
be involved. In this case, much of what
will happen after the operation can be
explained in advance.
However, it is not uncommon for a baby
to be transferred to a more specialised
hospital, many miles from the one in
which they were born and where the
diagnosis came as a surprise shortly after
birth. In many hospitals the condition
may be seen only once a year — or less
— making it difficult for the staff there to
answer all the parents' questions. Parents
are often unable to accompany their baby
to the new hospital; the mother will still be
r eco ve r in g f ro m th e d e l i ve r y o r
perhaps from a caesarean section, and
the father may wish to stay with the
mother or be required to look after other
children in the family. So for many
parents, the first time they see their baby
after his/her transfer is after a major
operation; by this stage, the baby will be
on a specialised ward which is staffed
and equipped to look after little babies.
Seeing their baby in this high-tech
environment can be stressful for parents.
The ward may be called the Intensive
Care Unit (ICU), Intensive Therapy
Unit (ITU), Special Care
Baby Unit (SCBU) or it may just have
its own name, unrelated to its function.
These wards look after those babies
who are very ill or who have had major
operations. Many of the babies are
quite small and may have been born
prematurely. Initially, the ward can be
intimidating and frightening with all
the equipment, noises and flashing lights,
and may seem unfriendly because of the
busy staff, lack of space and the limits on
the number of visitors. However,
parents will soon find that they are
welcome. There will be one specific nurse
looking after the baby during any one
shift; he or she will be able to explain
much about the baby's care and progress,
and can also introduce
parents to the routine of the ward.
Monitoring
Immediately after the operation, the baby
needs close monitoring and frequent care.
Usually a baby is nursed on a special
high table, with sides and an overhead
heater. Most babies have their heart rate,
temperature and blood oxygen saturation
level monitored. There may be a heart
tracing known as an ECG
(electrocardiogram) which is displayed on
a screen; this is obtained through thin
wires attached to the arms and chest.
The blood oxygen saturation level is
monitored from a small device applied to a
hand or foot and
connected by a narrow cable to a
30
The TOF Child
The Post-Operative Care
of Children with TOF/OA RG Buick, Consultant Paediatric Surgeon and AD Lander, Senior Lecturer in
Paediatric Surgery, Birmingham Children's Hospital NHS Trust.
Most babies with OA and TOF will be
operated on within the first few days of
life. Before the operation, some parents
have the opportunity to see the ward
where the baby will be recovering and
to meet the key medical staff who will
be involved. In this case, much of what
will happen after the operation can be
explained in advance.
However, it is not uncommon for a
baby to be transferred to a more
specialised hospital, many miles from
the one in which they were born and
where the diagnosis came as a surprise
shortly after birth. In many hospitals
the condition may be seen only once a
year – or less – making it difficult for
the staff there to answer all the parents'
questions. Parents are often unable to
accompany their baby to the new hospital;
the mother will still be recovering from
the delivery or
perhaps from a caesarean section, and
the father may wish to stay with the
mother or be required to look after other
children in the family. So for many
parents, the first time they see their baby
after his/her transfer is after a major
operation; by this stage, the baby will be
on a specialised ward which is staffed and
equipped to look after little babies.
Seeing their baby in this high-tech
environment can be stressful for parents.
The ward may be called the Intensive
Care Unit (ICU), Intensive Therapy
Unit (ITU), Special Care
Baby Unit (SCBU) or it may just have
its own name, unrelated to its function.
These wards look after those babies
who are very i l l o r who have had
major operations. Many of the babies
are quite small and may have been
born prematurely. Initially, the ward
can be intimidating and frightening
with all the equipment, noises and
f l a s h i n g l i g h t s , a n d m a y s e e m
unfriendly because of the busy staff,
lack of space and the limits on the number
of visitors. However, parents will soon
find that they are welcome. T h e re wi l l
b e on e sp ec i f i c nu r se looking after
the baby during any one shift; he or she
will be able to explain much about the
baby's care and p rogres s , and can
a l so i n t roduce parents to the routine of
the ward.
Monitoring
Immediately after the operation, the baby
needs close monitoring and frequent care.
Usually a baby is nursed on a special
high table, with sides and an overhead
heater. Most babies have their heart rate,
temperature and blood oxygen saturation
level monitored. There may be a heart
tracing known as an ECG
(electrocardiogram) which is displayed on
a screen; this is obtained through thin
wires attached to the arms and chest.
The blood oxygen saturation level is
monitored from a small device applied to a
hand or foot and
connected by a narrow cable to a
30
nearby unit with a screen which also
displays the baby's pulse rate. There
may be a thin wire attached to a foot to
monitor body temperature.
Above:
Oxygen saturation and heart rate monitors.
To keep the upper oesophagus clear
of saliva immediately after the operation
suction may occasionally be applied at a
measured distance from the mouth, so
that the inserted tube does not damage
the sutures at the join or anastomosis.
The TOF Child
nearby unit with a screen which also
displays the baby's pulse rate. There
may be a thin wire attached to a foot to
Oxygen saturation and heart rate monitors.
To keep the upper oesophagus clear
of saliva immediately after the operation,
applied at a
the mouth, so
does not damage
or anastomosis.
Infusions or Drips
There may be a number of infusions
of fluid running through tubing to one
or more special needles (cannulas) in
baby's veins. The cannulas are usually
sited on the backs of hands or feet, in the
arms or on the scalp and are secured with
tape. Once inserted they are not painful.
When fluid is running through the cannula
into the vein it is
Below:
Jacob Mayo, aged 3 days.
This picture shows the monitoring leads and infusion lines which are commonly used post-operatively. 1. Heart rate and breathing monitors are seen on the chest.
2. The oxygen saturation measuring probe is on the baby's right hand. 3. Intravenous infusions are attached to the right arm. 4. There is a chest drain tube leading away to the bottom right. 5. The baby is attached to a respirator.6. There is a trans-anastomotic tube.
The TOF Child
There may be a number of infusions
of fluid running through tubing to one
or more special needles (cannulas) in the
usually
feet, in the
secured with
are not painful.
When fluid is running through the cannula
This picture shows the monitoring leads and infusion lines which are commonly
Heart rate and breathing monitors are
The oxygen saturation measuring probe
Intravenous infusions are attached to
The baby is attached to a respirator. anastomotic tube.
31
commonly known as a 'drip' – a term
more applicable to the equivalent system in
adult patients, where fluids are usually
delivered from bottles or 'packs' of fluid
which drips down into tubes. In infants,
fluid administration is now nearly always
controlled by more accurate electronic
pumps; there may be a collection of these
small pumps attached to a stand near the
cot. The infusions may carry drugs (e.g.
antibiotics and drugs for pain-relief) as
well as the essential fluid and salts
(electrolytes) that the baby's body requires.
Ventilators Some babies may need help with their
breathing; this is supplied by a ventilator.
Certain babies may have been on a
ventilator before the operation – even
before transfer – perhaps because of
immature lungs or aspiration pneumonia
(inflammation in the lungs which results
from milk spilling into the lungs; this may
have happened during the first attempts to
feed, from which the oesophageal atresia
was suspected). In the latter situation, it is
likely that the baby will still need to be
ventilated for a period after surgery,
however many
babies who were not ventilated
before the operation will be
ventilated afterwards for anything
from a few hours to a few days.
Some surgeons
choose to paralyse (i.e. give a
drug which prevents movement
and breathing effort) and ventilate
babies for a period of five days if
there is tension on the repaired
oesophagus.
The ventilator is usually
contained in a unit on a stand
near the cot. It is connected to
an oxygen supply on the wall
and by two plastic tubes to the
baby.
32
The TOF Child
more applicable to the equivalent system in
fluid administration is now nearly always
these
near the
body requires.
breathing; this is supplied by a ventilator.
from milk spilling into the lungs; this may
have happened during the first attempts to
was suspected). In the latter situation, it is
T he a t t achment t o t he baby i s
usual ly the thing that f i rs t s t r ikes
parents when they see their baby after an
operation, because the two tubes and their
attachments lie over the baby's face and
forehead. One of these tubes carries oxygen
to the baby; the other takes away the gas
which is breathed out. A smaller tube joins
these two larger tubes to the baby's airway
(trachea) though the nose or mouth.
The ventilator makes a cyclical blowing
noise as it pushes oxygen-enriched air
into the lungs; the tubes to the baby
often move in time with this as the
pressures within them change. If the
baby has been paralysed this action may
repeat between 20 and 60 times a mi n u t e ,
b u t l a t e r o n a s t he b ab y "wakes up"
and makes some efforts at breathing, the
ventilator may help with breaths as little as
5 times a minute.
Ventilated babies usually have an
"arterial line" which allows the oxygen
Below;
Eleanor Butcher, 3 days with parents
looking on. This photograph shows the
two tubes leading from the baby's mouth
to the ventilator.
forehead. One of these tubes carries oxygen
may
mi n u t e ,
s up"
The TOF Child
and carbon dioxide levels in arterial blood
to be measured from samples taken every
few hours. Blood samples taken in this way
are painless and the results allow the
ventilator to be adjusted appropriately.
The arterial line consists of a cannula
which has been inserted into an artery and
is attached to an infusion pump.
Sometimes a blood pressure monitor is
also attached to the line and the results
displayed on a screen.
Chest Drains
The most common operation to repair a
TOF/OA involves dividing the t racheo-
oesophagea l f i s tu l a and joining the
upper and lower parts of the
oesophagus. This procedure is performed
through the chest (as described in a
previous chapter).
Some surgeons leave a plastic tube
in the chest to drain any fluid or air
that might otherwise build up in the
space between the lung and the chest
wall. If the join in the oesophagus leaks,
this fluid will also come out through the
drain. The drain is
attached to a fat rubber tube that is secured
to the cot/incubator and passes to a large
bottle on the floor. The bottle contains
water, and the end of the tube lies below
the level of this water, providing an
underwater seal to the tube that allows
fluid and air to escape from the chest, but
not get back in.
The drain may be left in for just a
few days or until a contrast radiograph
(which uses a special dye in the
oesophagus) has shown that there is no
leakage from the join, usually at about
7 days after the operation.
When the time comes, the tube is easily
removed. The hole closes and heals well
without further intervention and leaves
only a small scar.
The Chest Wound
The incision in the chest is usually on
the baby's right side, below the shoulder
blade, and will have been closed with
dissolvable sutures that lie under the skin.
The wound may or may not have a
dressing; at first the wound may be a little
red but this settles down.
Feeding
The oesophagus will have been repaired
around a plastic tube 3 or 4 mm in
diameter. This tube allows milk to be
given, either continuously or as small,
frequent meals. The tube passes from the
nose to the stomach and is ca l l ed a
nasogas t r i c or NG tube ; because the
tube passes through the place where the
oesophagus has been joined, or
anastomosed, staff may also call it a
transanastomotic tube. Occasionally
surgeons may remove this tube
immediately after the operation.
Feeding is started anywhere between
2 and 7 days after the operation. Some
surgeons allow this to be by mouth
from the very beginning – others wait until
full feeds have been achieved via the
nasogastric tube. If a chest drain has been
inserted, feeding by mouth may be
delayed for 7 days until the join in the
oesophagus has been shown to be water
tight on a contrast radiograph.
Small or sick babies and those with
other problems or abnormalities may
not be able to feed so promptly or by
mouth. Sometimes the surgeon will have
placed a gastrostomy tube during the
operation, which passes into the stomach
through the front of the abdomen and can be used for feeding.
Intravenous feeding, known as
parenteral nutrition – or sometimes total
parenteral nutrition (TPN) – is also
used. This may be given through a short
33
cannula in a limb or through a long, very
fine feeding line which has been threaded
up a vein in the head, neck, groin or in a
limb into a big vein near the heart. The
advantage of these "long lines" into bigger
veins is that more concentrated nutrition
can be given than is possible through
normal "drip" lines.
Below:
Kris Spender, 19 weeks, being fed. The
tubes under the nose give the baby
supplemental oxygen.
Recovery
As progress is made the baby will
require fewer tubes and less frequent
monitoring. He or she may move to a
more normal-looking cot, and may even
move to a less intensive ward while
feeding is established.
Most babies are ready to go home after
7-10 days of satisfactory feeding on the
breast or by bottle, but others may take
longer because of prematurity, other
anomalies or feeding / breathing problems.
Every baby is different. Sometimes the
last few days of
34
The TOF Child
fine feeding line which has been threaded
advantage of these "long lines" into bigger
concentrated nutrition
after
on the
anomalies or feeding / breathing problems.
recovery are made at the referring
hospital near the parents' home.
When discharged from hospital,
arrangements are made for the surgeons to
see the baby in the outpatient department
to review progress.
Other Problems
Leaks
A major leak of fluid from the join
in the oesophagus is now an unusual event
thanks to modern surgical advances, but
remains a serious one. If a leakage
occurs, it usually does so in the first
three or four days after the operation. A
leak may be suspected because the baby
becomes ill with a fever, and may require
more oxygen or ventilation. A chest
radiograph may show some air or
f luid around the lung. If there is any
doubt over the diagnosis a small amount of
liquid w h i c h s h o w s u p c l e a r l y
o n a radiograph can be put into the
oesophagus and radiographs taken to
s ee whe the r t h i s appea r s t o l eak
around the anastomosis area at all. A small
leak may be treated with antibiotics and
stopping the feeds by mouth or tube. A
larger leak, which the surgeons believe will
not heal up on its own, will require surgery.
Tracheomalacia
When a baby is born with tracheo-
oesophageal fistula, the trachea is
frequently not as ridged as normal.
Normally the trachea is held open by
stiff rings of cartilage which stop it
collapsing when the baby breathes; these
rings play the same role as the stiff
rings that keep the hose pipe open on
vacuum cleaners. Any area lacking these
supporting rings is likely to be a bit
floppy and may narrow during breathing.
This narrowing may be a minor problem
that the baby grows out
larger leak, which the surgeons believe will
of – however, sometimes when the baby
is distressed and crying the floppy trachea
narrows so much that the baby cannot
breathe and goes blue. If these episodes are
considered severe and/or often enough to
be dangerous, later surgery is often
recommended (see chapter on respiratory
problems).
Jaundice
The baby may become yellow, or
jaundiced, during the first week of life.
This is not uncommon in otherwise noinial
newborn babies. This yellow colour
should gradually fade and disappear
without treatment; if the jaundice
increases to a certain level, a special
ultraviolet light will be shone on the
baby to reduce the jaundice.
Gastro-oesophageal reflux
This is another common problem seen
with oesophageal atresia. In this condition,
milk returns back up the o e s o p h a g u s
a f t e r i t h a s b e e n swallowed.
Sometimes stomach acid is also forced
up the oesophagus, where it can cause
inflammation and scarring (see chapter
on Strictures and Reflux).
The TOF Child
however, sometimes when the baby
is distressed and crying the floppy trachea
much that the baby cannot
breathe and goes blue. If these episodes are
considered severe and/or often enough to
recommended (see chapter on respiratory
g the first week of life.
This is not uncommon in otherwise noinial
special
on the
This is another common problem seen
with oesophageal atresia. In this condition,
o e s o p h a g u s
swallowed.
also forced
it can cause
(see chapter
Quotations from TOF s, their families and friends and medical professionals
"One of the amazing things was seeing
how quickly tiny babies can recover
heal after the kind of major surgery which
would render adults incapacitated for
months.
During Hannah's first week, there were
times when my feeling of separation from
Hannah was overwhelming, particularly at
night when I could hear other babies
crying and I was alone in my room. I often
found I naturally awoke when Hannah was
due her "all care" every 3 hours, and
would trek down to SCBU. The staff were
wonderful, giving us the encouragement
and confidence that quickly enabled us
do tube feeds and cuddle or bath Hannah
with leads and tubes in tow. We were
encouraged to make decisions, whenever
possible, about Hannah's care. After the
first difficult day of trying to breast-feed
when Hannah was 11 days old, I asked if it
was possible for Hannah and I both to be
transferred to the high dependency ward
to establish feeding. This was quickly put
into place and helped enormously. l' 11
never forget that feeling of elation
when Hannah and I were together both
day and night. I couldn't stop looking at
her!
I found it incredibly difficult to leave
Hannah at all, although as the weeks
became months, I knew that I had to
spend the occasional night away from
the hospital."
Left:
Jacob Mayo aged 17 months.
This is the baby shown photographed in
intensive care at the start of this chapter.
The TOF Child
families and friends and medical
"One of the amazing things was seeing
how quickly tiny babies can recover and
heal after the kind of major surgery which
During Hannah's first week, there were
times when my feeling of separation from
s overwhelming, particularly at
crying and I was alone in my room. I often
found I naturally awoke when Hannah was
would trek down to SCBU. The staff were
ncouragement
and confidence that quickly enabled us to
Hannah
We were
encouraged to make decisions, whenever
possible, about Hannah's care. After the
feed
when Hannah was 11 days old, I asked if it
Hannah and I both to be
the high dependency ward
to establish feeding. This was quickly put
into place and helped enormously. l' 11
that feeling of elation
and I were together both
I couldn't stop looking at
spend the occasional night away from
This is the baby shown photographed in
intensive care at the start of this chapter.
35
The TOF Child
"Steve and I seemed to be emotionally
strong at different times, and with the help
of family, friends and the medical staff, we
were able to encourage one another."
"On the day we left hospital I was very
apprehensive as to what may lie ahead
for him. I am pleased to be able to tell
you, however, since bringing Craig
from hospital that day, we have never
looked back.
I was always careful in the early days
to ensure food was kept in small portions
or reasonably mashed down to be on
the safe side. I kept up with the
communications from TOFS and took
their advice; they were a great comfort
through the early years.
I have taught him not to `eat in a hurry'
He is not under the hospital any more.
The consultant has not seen him since
he was nine and when we did see him
it was always a happy event telling
him how well Craig was."
"In the parents' waiting room at the Neo-
natal Unit there is a photo-album of all
the babies who have stayed on the unit.
Most of them were of TOF babies and
children who had obviously grown up and
were enjoying a happy childhood. It was
that album which gave me and my husband
such
comfort. To see those children who had
been so ill like our Mark make such a
remarkable recovery gave us tremendous
hope."
"I left hospital about four days after Tom
was born and I spent my days at the
hospital with Tom, coming home so that
I was here when Ben and Sonja came
home from school, then once they were in
bed and we'd had a bedtime story, I
returned to the hospital and stayed until
about midnight. It was tiring, but I felt
that it was unfair on Ben and Sonja to
disrupt their lives any more."
"Freddie recovered very well from his
surgery and was able to come home after
two weeks. He was a very tense, unhappy
baby for the first five months a n d I
t h i n k t h e n a t u r a l b on d i n g process
took longer for us than it would have
with a 'normal' child. The trauma and
worry of having a TOF baby robs you of
some of the joy of becoming parents at
first, but once you start to relax and live
with the condition it is amazing how
quickly avoiding! dealing with chokes
becomes part of your routine. Freddie is
now nearly three and is a wonderfully
happy and physical little boy; he has
only had one more stay in hospital to
r e m o v e s o m e p e a s h e w a s
inadvertently given at nursery. Freddie
now has a brother, William. His birth
enabled us to experience some of what
we missed when Freddie was in special
care – the pleasure of taking your
child straight home from hospital and
caring for him during those first few weeks
– but it is strange to have a baby who at
seven months could already munch his way
through foods Freddie cannot manage!"
36
Discharge from HospitalJM Beck, Consultant Paediatric Surgeon, and Penny Seymour,
Neonatal Surgical Outreach Sister, The
Taking your baby home from the
hospital is a big step, but one that you
should be looking forward to. After the
trauma of the hospitalisation period
you will need privacy and time to
establish a routine in the comfort of
your own home.
Planning and preparing for
discharge will help to give you the
confidence you need to handle any
difficulties which may arise. You will
then know where to look for support,
help and advice in case of problems
and what to do in an emergency.
Treat your baby as normally as possible; play with your child, talk to them, provide stimulation, love and security. Remember too that a little discipline and order are required in order to establish healthy routines.
The TOF Child
Discharge from Hospital JM Beck, Consultant Paediatric Surgeon, and Penny Seymour,
Neonatal Surgical Outreach Sister, The General Infirmary at Leeds.
hospital is a big step, but one that you
should be looking forward to. After the
trauma of the hospitalisation period
establish a routine in the comfort of
discharge will help to give you the
confidence you need to handle any
difficulties which may arise. You will
then know where to look for support,
help and advice in case of problems
possible; play with your child, talk to them, provide stimulation, love and security. Remember too that a little discipline and order are required in order to establish healthy routines.
Normality also entails attendance a
clinics for developmental checks and
routine health care. Although your
TOF baby may well need some
specialised care, in other respects they
need exactly the same attention as any
other baby.
Vaccinations
It is important that your baby is
protected from the common infectious
diseases of childhood, so the full
standard vaccination programme is
essential.
Despite the fears and worries
periodically raised about vaccination,
it is beneficial — and for TOF babies
the prevention of whooping cough is
particularly important.
The TOF Child
Normality also entails attendance at
clinics for developmental checks and
specialised care, in other respects they
need exactly the same attention as any
the common infectious
standard vaccination programme is
periodically raised about vaccination,
and for TOF babies
the prevention of whooping cough is
37
The TOF Child
Preparing for Discharge
Babies born with OA/TOF can develop
further problems after they have been
discharged from hospital. It is important
that you have the opportunity to learn as
much as possible about these potential
problems before you go home. Many of
the medical professionals you meet outside
the specialist unit will not be familiar with
oesophageal atresia since it is a relatively
rare condition.
The most common problems to be
aware of relate to feeding, gastro-
oesophageal reflux and respiratory
difficulties, all of which are discussed
in detail elsewhere in this book.
Not all babies born with oesophageal
fistula will have problems, however a
significant number are affected to a
lesser or greater extent. It is therefore
best to be prepared.
Increasing care responsibilities
As the time for discharge from hospital
approaches you will be encouraged to take
an increasing amount of responsibility for
your baby's care.
If your baby is not being breast fed
you will need to learn to make up and
give feeds, either by bottle or tube. You
w i l l a l s o h a v e t o m e a s u r e a n d
administer any medicines accurately
and provide all the normal day to day care
your baby needs.
There may also be 'add on' bits of
care that you will have to be familiar
with, like the management of tubes
and stomas. If physiotherapy, suction or
oxygen are still required in hospital by
the time you are thinking towards
discharge, then it is likely that your baby
will continue to require this at home and
such treatments will need to become part
of your normal routine.
Medical and nursing staff will be
spending time talking to you about
your baby and what extra care, if any,
you will need to learn about and continue
at home.
Discuss your home circumstances
and the level of support available to
you with hospital staff; if there are
problems they are there to help.
Make sure you understand
When you are given explanations in
hospital, do not allow professionals to
get away with jargon that you don't
fully comprehend. If terms are used that
you don't understand, do not hesitate to ask
for the advice to be repeated in simple
language. Many parents report that nursing
staff are far better at explaining problems
and techniques in simple language than the
medical or surgical staff.
A number of explanatory leaflets
are available, the best being produced
by TOFS. Ask what leaflets the unit
has, read them and discuss the contents
with the staff.
Be prepared
The thought that you may one day need
to resuscitate your baby is a very
frightening one. The need very seldom
arises, however it will increase your
confidence enormously if you can have
a demonstration of basic first aid
resuscitation techniques.
Many units have 'dolls' on which
you can practice and videos
demonstrating what to do. (A chapter
discussing resuscitation is found
elsewhere in this book).
The transition period
On the day of discharge you will be
leaving an environment where there is a
team familiar with the care of babies
with tracheo-oesophageal fistula /
oesophageal atresia (TOF/OA) and
associated problems.
38
The TOF Child
The neonatal surgical outreach nurse,
where there is one, provides an invaluable
link between the hospital, the primary
health care team and yourself.
Your GP, health visitor and other
advisors in your local primary health
care team have, in all probability, never
looked after a TOF baby before. The
emphasis of their interest in your baby is
on general well -being and child
health issues, and they can offer a vast
amount of experience of common
childhood illnesses. These aspects are of
equal importance to the care of the
specific problems with TOF/OA for a
TOF child.
The surgical unit will contact your local
healthcare team before you go home to tell
them about the problems which might be
anticipated and whether any medicines,
equipment or special supplies will be
required. Like you, they must know
what action to take and who to contact if
problems arise.
The neonatal surgical outreach nurse
will be able to provide them with
additional information and assistance
with regard to the TOF/OA condition,
on an ongoing basis. As well as meeting
members of your primary health care
team, the outreach nurse can also visit
you at home and ensure that your baby
gets good continuity of care.
Equipment
If your baby needs a feed pump, suction
or oxygen at home, this will be provided
and you will be taught how to use it.
Health authorities vary in the
arrangements they make for the supply
of such equipment and with the assistance
they can provide at home. Very often the
surgical outreach nurse or hospital liaison
visitor is the best source for this kind of
information.
Ensure that you know who is
responsible for the supply of equipment,
how to obtain fresh supplies, replace-
ments and repairs. When you leave
hospital you should have sufficient
supplies to last you at least 2-3 weeks
because it takes time for fresh supplies
to be organised.
All these arrangements need to be
planned in advance. You may find it
useful to write down lists of the things
you will need when you go home; you
can then discuss them with staff on the
ward. The more preparation you do
before you go home, the less you have
to worry about when you get there.
Feeding
Feeding is described in the
following categories:
a) Oral feeding: normal feeding from
the breast or by bottle.
b) Tube feeding: milk is given
directly into the stomach by nasogastric
tube or gastrostomy, or into the small
intestine by a jejunostomy tube.
c) Sham feeding: for babies who have
not yet undergone oesophageal
anastomosis, but have been given a
cervical oesophagostomy where the upper
oesophagus is brought out to the surface
at the neck. A gastrostomy feed is
given simultaneously.
Oral feeding
The normal oesophagus is a co-
ordinated muscular tube which
actively passes swallowed drink and
food from the mouth to the stomach.
It has mechanisms to prevent
regurgitation, or reflux of stomach
contents back up the oesophagus.
Such co-ordinated action is not present
in TOF babies because of the lack of
continuity between oesophageal
segments. They are therefore liable to
choke, splutter and inhale feeds.
39
T h e s e p r o b l e m s c a n l e a d t o
alarming "blue do's" (cyanotic spells)
due to obstruction of breathing, and panic.
As soon as the baby stops feeding and
takes a breath, their colour will usually
return to normal promptly. If it doesn't,
tip the baby's head down o v e r y o u r
k n e e a n d t a p s ma r t l y b e t w e e n
t h e s h o u l d e r b l a d e s t o dislodge
anything which may be causing an
obstruction. If your baby remains blue and
breathing does not improve, this is an
emergency and you should call or phone
for help at once and begin the
emergency rout ine which you have
been taught.
If your baby starts to have any
problems with feeding, you should
contact the surgical unit.
Poor oesophageal co-ordination means
that TOF babies tend to take longer to
feed. They may need more breaks during
their feed, and suck more slowly to allow
time for the feed to reach their stomach.
Smaller, more frequent feeds are often
better tolerated.
Some babies seem more able to
coordinate sucking and swallowing if
the feed is made a bit thicker.
F e e d i n g b a b i e s i n a n u p r i g h t
position encourages milk to reach the
stomach more easily. Gravity will also
help to prevent gastro-oesophageal reflux
(a problem where the acidic stomach
contents are pushed back up into the
lower oesophagus, causing pain) if the
baby is kept in an upright or sitting
position for an hour after feeds.
Difficulty with feeding will be
aggravated if there is a narrowing
(stricture) due to scarring at the join
between the upper and lower parts of
the oesophagus. Often this problem only
becomes obvious when weaning to
solids starts, so solids should be
introduced into the diet with caution.
40
The TOF Child
due to obstruction of breathing, and panic.
takes a breath, their colour will usually
If it doesn't,
ns blue and
should call or phone
ordination means
more slowly to allow
to reach their stomach.
stomach more easily. Gravity will also
oesophageal reflux
Should difficulties arise, stop giving solids
until you have consulted your surgeon.
Introduction of solids is not
recommended in any baby before three
or four months of age and can be delayed
much longer if the baby is growing well on
milk feeds alone. (Strictures are discussed
further elsewhere in this book).
Tube feeding
For babies who are tube fed, it is
important (with very few exceptions)
to persevere with some oral feeds. In
many cases, oral feeding can then be
gradually increased and the tube
eventually discarded, in a natural
progression with no sudden changes.
Tips for tube feeding include
encouraging the baby to suck a dummy
during the feed and placing emphasis on
the social aspects of feeding by cuddling
and/or looking at the baby during the
feed.
As the child gets older, make sure
that they are included in family meal
times, have the opportunity to taste
and smell food and to copy nonnal
eating behaviour.
Above: Anthony Pierce at 2'12 months, apparently
content with his nasogastric tube.
Should difficulties arise, stop giving solids
growing well on
Nasogastric Tube
If your baby is tube fed for any
length of time, ask for referral to the
hospital speech therapist who can
devise an 'oral stimulation programme.'
This will help to maintain the skills
your baby will need to eat and drink
normally as they grow up. A baby who
does not eat through his/her mouth
may not get to learn how to use their
mouth normally; the exercises from
the speech therapist are designed to
encourage and develop muscular
function and sensation in the mouth,
which will facilitate the transition to
normal feeding later on.
Nasogastric tubes
These may be necessary in the
following instances:
i) babies who are unable to take
adequate volumes orally.
ii) babies who have severe gastro-
oesophageal reflux.
iii) babies who have very small
stomachs and need continuous feeds.
iv) babies with severe oesophageal motility and/or coordination problems.
v) babies who need supplementary
feeds at night to improve weight gain.
The TOF Child
Gastrostomy Babe
Site of anastomosis
Gastrostomy
tube
If your baby is tube fed for any
length of time, ask for referral to the
hospital speech therapist who can
devise an 'oral stimulation programme.'
This will help to maintain the skills
your baby will need to eat and drink
normally as they grow up. A baby who
outh
may not get to learn how to use their
mouth normally; the exercises from
the speech therapist are designed to
function and sensation in the mouth,
which will facilitate the transition to
-
stomachs and need continuous feeds.
oesophageal motility and/or coordination problems.
babies who need supplementary
feeds at night to improve weight gain.
If your baby is to be discharged
with a nasogastric tube, you will be
taught how to care for the tube, how to
use it safely, how to check its position
etc. Since babies pull nasogastric tubes
out at the most inconvenient times,
most parents quickly learn to re-pass
them. For those parents who do not
feel confident when their babies first
go home, alternative arrangements can
be made with a local community nurse
or hospital.
Gastrostomy tubes
I f the need f or tube f eed ing
continues or is expected to continue
over several months, a gastrostomy
tube becomes an option. This is a tube
that is positioned during a small
operation, and is inserted directly into
the stomach, through the stomach wall.
It can be left in place for much longer
than a nasogastric tube, is difficult to
pull out accidentally, and is less
obvious since it is hidden beneath the
baby's clothing.
There are a number of different
tubes; if your baby needs one, your
surgeon will select the most
appropriate size and type.
The TOF Child
Gastrostomy
tube
If your baby is to be discharged
with a nasogastric tube, you will be
taught how to care for the tube, how to
heck its position
etc. Since babies pull nasogastric tubes
pass
them. For those parents who do not
feel confident when their babies first
go home, alternative arrangements can
h a local community nurse
I f the need f or tube f eed ing
continues or is expected to continue
over several months, a gastrostomy
tube becomes an option. This is a tube
operation, and is inserted directly into
the stomach, through the stomach wall.
It can be left in place for much longer
than a nasogastric tube, is difficult to
pull out accidentally, and is less
obvious since it is hidden beneath the
tubes; if your baby needs one, your
41
The TOF Child
Feeding techniques
Feeds given via a nasogastric
or gastrostomy tube can be given
a s s epara t e feeds eve ry 3 -4
hours, or as a slow continuous flow
administered by a computerised
pump.
Babies with gastrooesophageal
reflux or small stomachs are amongst
those who m a y b e n e f i t f r o m
s l o w continuous feeding. This
may be given over a 24 hour period,
in which case a portable pump is
essential. Many babies however
manage small, oral feeds during
t h e d a y a n d h a v e t h e i r
continuous feeds over a
10-12 hour period at night. This
minimises disruption to family life
and encourages more noiinal patterns
of eating during the day.
Jejunostomy tubes
Babies with severe gastro-
oesophageal reflux may benefit
f rom jejunal feeding. Babies
who have undergone gastric
t ransposit ion surgery cannot
have a gastrostomy tube inserted
and therefore a jejunostomy tube
is the only option.
The jejunum is at the top of
the small intestine, below the
stomach and duodenum.
A tube into the jejunum can be
positioned either through the nostril
and stomach, via a gastrostomy or
directly through the body wall to
the jejunum.
Jejunostomy is used much
less commonly than gastrostomy
or nasogastric feeding because the
tube is more difficult to position and
care for, and continuous pump
feeding is required over 20-24 hours.
Care of Nasogastric Tubes
Single-use PVC tubes are usually replaced on a weekly basis. Longer term feeding tubes made of polyurethane and silicone can he left in place for much longer periods if they do not become blocked and are not pulled out accidentally. Tubes should be used, changed and re-sterilised according to manufacturer's
recommendations.
The tube length and adhesive tape should he checked at least daily.
Feeding
Check the tube position before feeding. Its opposite end should he in the stomach, the contents of which are acidic. I f a few drops of fluid which has been aspirated from the tube are applied to blue (alkaline) litmus paper, the paper should therefore turn pink.
The child should be sat up or laid at a 30 ° angle during the feed and for at least half an hour after-wards to minimise the risk of reflux and vomiting.
Bolus feeds are administered either by gravity or via a suitable enteral feeding pump. The paediatric medical staff or dietician will recommend the best method of feeding your child.
After use, flush the tube with 5m1 boiled, cooled water to prevent blockage and reduce the risks of infection.
Vent the tube if the child is unable to burp after the feed or has abdominal discomfort or distension. This involves removing the cap or spigot from the tube, for up to half an hour, to allow air to escape. When disconnecting after feeds, attach a syringe without its plunger in place, or leave the feeding set attached; if food comes hack with the wind, it can get messy! Some children may also require venting before feeds.
Re-passing tubes
When re-passing nasogastric tubes always check the length carefully and mark the depth of insertion with a small piece of tape if there is no obvious mark on the tube. Try different types of adhesive tape until you .find the best for your baby and use alternate nostrils to give the other side of the nose and face a rest.
Drug administration
Medications should be given as suspensions, and diluted using boiled, cooled water. Any tablets must he thoroughly crushed and dissolved before administration.
Flush with boiled, cooled water before and after each medication to prevent the tube blocking.
42
The TOF Child
43
Care of Gastrostomy Tubes
Principles of gastrostomy care remain the same whichever type of tube is used.
Before cleaning the gastrostomy tube or site, and before feeding,
wash your hands with soap and water. The skin around the
gastrostomy tube must he kept clean and dry (use warmed saline or water) and should be examined after feeds in case of leakage.
Tissue overgrowth or granuloma can he a problem around
gastrostomies. Using a mild antibiotic steroid ointment
(e.g. Sofradex eye ointment) on the granuloma will keep it
under control.
Baths are allowed; ensure the feeding port is closed and
the skin around the stoma is completely dry afterwards.
The end of the tube must not inadvertantly he left inside the nappy!
When the stomach and stoma are fully healed, usually after 3 -4 weeks, water activities such as swimming may be resumed.
Ensure the tube is well secured.
Safety
Check tube placement daily; blue (alkaline) litmus paper applied to fluid
from the tube should turn pink due to acidic stomach contents.
Check the tube length and/or position of the retention disc daily. Check
the balloon weekly (if applicable; not all tubes have balloons).
Ensure connectors for feeding do not stretch the gastrostomy tube. If the
connectors are too large or are jammed in too tightly, the feeding port
will stretch and milk will leak out.
Feeding
The child should be sat up or laid at a 30° angle during the feed and
for 30 minutes afterwards to reduce the risk of refluxlvomiting.
Bolus feeds should be run in by gravity and should take the same
time as a meal i.e. 15-20 minutes. Never use force.
Continuous feeds should he administered by a suitable enteral feeding
pump. The paediatric medical staff or dietician will recommend the best
method of feeding your child.
After use, flush the tube with 5 -10m1 boiled, cooled water to ensure 'Keeney
and to keep bacteria to a minimum.
Vent the tube if the child is unable to burp after the feed or has abdominal
discomfort or distension. This involves removing the cap or spigot from
the tube, for up to half an hour, to allow air to escape. When disconnecting
after feeds, attach a syringe without its plunger in place, or leave the
feeding set attached; if food comes back with the wind, it can get messy! Some children may also require venting before feeds.
Drug administration
Suspensions are recommended; dilute these with boiled, cooled water.
Ensure that any tablets are thoroughly crushed and dissolved prior to
administration.
to prevent blocking. Flush with boiled, cooled water before and after each medication
The TOF Child
With a jejunostomy, the not ' tal
digestive function of the stomach is
bypassed, so food can be more difficult
to digest. Special formula milk may
therefore be needed which is easier to
digest and absorb.
Sham feeding
The importance of promoting normal
patterns of feeding has already been
mentioned. It is particularly important for
babies who have not yet had an
oesophageal repair and who have been
given a cervical oesophagostomy. In this
procedure, the blind end of the upper
pouch of the oesophagus is brought out
as an opening or "stoma" in the side
of the neck, to allow saliva to drain.
A baby with such an oesophagostomy
will also have a gastrostomy tube.
Babies should be breast or bottle fed
a t t h e s a m e t i m e a s g i v i n g a
gastrostomy feed; the milk taken orally
will of course drain out onto the neck.
Any attempts to catch the fluid is usually
in vain (although in larger babies and
older children a colostomy bag may be
useful); wrapping an absorbent towel
around the baby at feed time is often the
best way to soak it up. This procedure is
known as "sham feeding."
Giving a gastrostomy feed at the same
time as a sham feed teaches the baby to
associate sucking and swallowing with the
feeling of a full stomach. It may seem a
waste of milk, and later on of food, since
it is of no nutritional value to the baby,
but it is of great psychological benefit. It
is preparing the way for normal eating
in the future, and gives comfort and
p l easu re a t t he s ame t ime . Af t e r
surgery to reconstruct the oesophagus,
feeding problems are less in babies who
sham fed well.
Gastro-Oesophageal Reflux
There is a valve-like mechanism at
the lower end of the oesophagus which
prevents return of stomach contents to
the oesophagus (gastro-oesophageal
reflux). The mechanism is weakly
developed in most babies, hence their
tendency to vomit at the slightest
provocation. The mechanism increases
in strength and efficiency during the
first few months or years of life.
Because of the abnormality of the
oesophagus in TOF/OA, the tendency to
gastro-oesophageal reflux can be
particularly worrying and severe. It can
contribute to failure to gain weight,
feeding difficulties and chest infections.
Treatment of reflux depends at first
on posture, thickening of feeds, and drugs
to improve the motility of the oesophagus
and to promote emptying of the
stomach. Drugs to neutralise or prevent
acid secretion by the stomach can be a
useful addition which also helps to prevent
the discomfort associated with acid reflux.
44
The TOF Child
There is often heated debate about
the best and safest posture to counteract
reflux, but there is little doubt that being
kept propped up in a chair (but not
slumped over) is safe, effective and allows
the baby to see what is going on.
When babies are lying down, reflux
is reduced by lying them on their
stomachs, head up, in the prone position.
However, this position is not
recommended for most babies because
of the increased risk of cot death. If a baby
has severe gastro-oesophageal reflux, the
prone position may nonetheless be the
safest option.
If, for medical reasons, your doctor
has advised you to put your baby on its
stomach to sleep, then an apnoea alarm
(a monitor which registers a baby's
breathing and sounds an alarm if breathing
stops) can give additional s e c u r i t y . I t
s h o u l d b e c l e a r l y understood that
any movement a baby makes will be
interpreted by the monitor as breathing and
therefore a baby struggling to take a
breath will not necessarily trigger the
alai'''.
Research has shown that another good
position to minimise reflux is lying on the
left side, probably because it reduces the
pressure on the stomach.
Keeping the baby tipped head up will
help to keep milk in their stomach and
lessen any discomfort in the oesophagus.
Raise the top of the cot or p r am to
c rea t e a gent l e s l ope by placing a
pillow or folded blanket under the
mattrress, but ensure that the slope is not
so steep that the baby slips down under the
blankets.
Try to avoid lying your baby flat on
their back after feeds. Changing nappies
before feeding will make vomiting or
reflux less likely than doing so when their
stomach is full.
For more information, refer to the
chapter on strictures and reflux.
Tracheomalacia
This can cause problems, but only in
a minority of babies. It is a possible cause
of so-called 'blue do's' sometimes seen in
babies born with OA/TOF.
Positioning can play a part in the
management of this condition: babies
with tracheomalacia can find breathing
difficult when lying flat on their backs,
particularly if they are crying, and may
be more comfortable sat up in a baby
chair. Your doctor will advise you on the
safest sleeping position for your baby.
Babies with tracheomalacia can have
noisy breathing (stridor) when they are
otherwise healthy and well. It can
therefore be a good idea to visit your GP
for the first time when your baby is well,
so that the doctor can hear what this
sounds like. You may need to explain to
friends, relatives and health care
professionals in the community that the
noises your baby makes are quite normal
for them and that they do not have an
infectious condition. Babies with OA/TOF
often have a very distinctive cough
(referred to as 'the TOF cough') which can
sound alarming to others.
For more information, refer to the
chapter on respiratory problems.
Medicines
Most TOF babies do not require
medication on discharge from the
neonatal unit. Those who do may need
it to control gastro-oesophageal reflux
or because they have other associated
anomalies (e.g. babies with renal problems
may need antibiotics).
If your baby is going home on
medication, find out what it is, why
they need it and how long for. Practise
measuring it out and giving it before
you leave the hospital.
45
The TOF Child
Medications
Anti-reflux medication
Cisapride: increases gut movements and helps the stomach to empty more quickly so that reflux is both less likely to occur and there is less material available to be refluxed back into the oesophagus. Sometimes leads to loose bowel actions, because food passes through the gut more rapidly and there is less time for foodstuffs and liquids to be absorbed.
Ranitidine or Cimetidine: reduce the acidity of the stomach contents, so reducing the risk of damage to the lower oesophagus and preventing
discomfort from acid reflux.
Feed thickeners
The following may he prescribed to keep milk in the baby's stomach and reduce vomiting:
Infant Gaviscon: a powder which can he added to the feed, or given afterwards mixed with a little milk or water. Gaviscon is particularly useful for breast fed babies.
Carobel: an instant thickener, which is added to the feed just before it is given to the baby.
Thixo-D or Thick' n Easy: can be added just prior to feeding or mixed in advance when feeds are prepared and stored before use.
Nestargel: added to recipe when feeds are made up and needs cooling to keep it smooth.
Antibiotics
Babies with associated renal complications may he discharged home on a low dose antibiotic. This will discourage some of the common bacteria which lead to urinary tract infections. It will not prevent all urine infections, so if the baby becomes unwell then the urine should always be checked.
Feed supplements
Babies who have had neonatal surgery, particularly those with feeding problems, may need extra calories in their feeds to help them grow. These are prescribed under guidance from a dietician. You will need to know how to make the feeds up before your baby goes home.
Supplements are designed to give calories in the form of carbohydrates and/or fat. They come in powder or liquid form and are added to the feed when it is made up. Common examples are Maxijul, Duocal, Polycal, Liquigen and Calogen.
Giving medicines
Medicines are usually given to
babies as a syrup from an oral
syringe. Many of the doses are very
small and need careful measuring. Oral
syringes for small doses can be
difficult to obtain from the chemist at
home, so take some spare ones with
you from the hospital.
Syringes can be sterilised either in
solution or a steam steriliser. Some
syringe markings fade very quickly
and it may be wise to mark the
correct level with (e.g.) a freezer pen
as an additional guide.
Although there are a few
medicines which can be added to
feeds, most are better given before
the feed, either by syringe or in a
small amount of milk. If medicines
are added to complete feeds and the
baby doesn't finish the bottle, you
won't know how much of the
medicine the baby has had.
Read the instructions on the label;
if medicines which are given once a
day say (for example) 'give at night'
then there will be a reason for this. If
you are unsure what the reason is, ask
before you go home.
Take great care with the storage of
medicines, especially if there are other
children in the house. Some medicines
may need to be stored in a refrigerator,
others at room temperature.
Remember to ask your GP for
repeat prescriptions before you have
run out of medicine, and allow
enough time for a possible delay at the
chemist in case they haven't got it in
stock.
46
The TOF Child
Chest Infections Respiratory infections are dealt
with in a separate chapter, but it is
important to be aware that breathing
problems can develop in TOF babies;
if your baby's breathing is becoming
noisier or more difficult, contact your
GP or the hospital for advice.
TOF babies are prone to chest
infections: if they are frequent, further
investigation will be necessary.
Occasionally a recurrence of the
original tracheo-oesophageal fistula
will be detected, but more often than
not no single cause is found.
Contributing factors include gastro-
oesophageal reflux and
tracheomalacia.
Repeated courses of antibiotics and
aggressive treatment of gastro-
oesophageal reflux may be needed.
To help prevent chest infections, good
nutrition, a healthy atmosphere and
absence of overcrowding are all
important. It has been shown time and
again that tobacco smoke in the
household is a risk; if people you
know insist on smoking, keep them
away from your baby and ask them not
to smoke indoors.
Physiotherapy
Chest physiotherapy is used to
encourage drainage of secretions from
the lungs in babies producing
excessive secretions and/or tending to
inhale or aspirate feed or saliva. It may
be needed by some TOF babies at
home, especially if they have a cold.
The physiotherapy usually consists of
various actions over the chest, while
the baby is lying over your knees. It
must only be used when it has been
professionally demonstrated and
appropriate equipment provided,
because this sort of handling can
encourage regurgitation of stomach
contents and aggravate the situation.
Once the technique has been suitably
mastered, physiotherapy should be
done when the baby's breathing is
moist, bubbly and noisy.
Postural drainage
There are various body positions
which help to drain secretions from
different areas of the lung; several
positions (as taught by the physio-
therapist) are used at each session.
Percussion
This technique loosens secretions in the chest, and is either done in a rhythmic clapping action with a small `mask' or with a cupped hand.
Vibration
This is a shaking action using the
hand on the chest, which aims to move
secretions into the larger airways when
the child exhales.
During and/or after physiotherapy it is hoped that the child will cough and clear secretions. Young children may find it hard to cough out the secretions and often swallow them instead; this is not a problem.
Chest physiotherapy is done before
meals, usually three or four times a day.
Each session should last around a
quarter of an hour.
Additional Problems A number of TOFs have associated
anomalies which may add to their care.
P r o b l em s l i k e t h e V A TER
syndrome, congenital heart problems,
the CHARGE syndrome and others
will have been described to you if your
baby is affected, and any care
procedures fully explained.
47
The TOF Child
Follow Up
Whatever happens, prolonged hospital
follow up will be necessary for your
baby. This follow up may be arranged at
the Regional Neonatal Surgical Unit, but it
is often both more convenient and efficient
for routine follow up to be carried out at
your District Hospital, at a special clinic
attended by one of the surgeons from
the Neonatal Surgical Unit together with
the local paediatricians.
The purpose of these follow up
clinics is twofold:
i) to check on your baby's progress —both
the particular medical problem(s) and
general growth and development,
ii) to allow you to discuss
problems that have arisen at home and
to try to answer any questions that you
have.
The dosage of any medications may
need adjusting to allow for the baby's
growth, which is best assessed by
routinely weighing the baby at each
check-up under the same conditions and
on the same set of scales.
Arrangements for further investigations
and treatment may be made; if there are
feeding problems, an assessment has to be
made whether these might be caused by
a stricture at the anastomosis, gastro-
oesophageal reflux, or poor oesophageal
motility. Feeding problems may be
aggravated by tracheomalacia, which is
often associated with breathing
difficulties; signs of chronic chest
problems need to be looked for and
appropriately dealt with.
Advice on weaning and the introduction
of various foods may be given, including
advice on types of food to avoid if there
are problems related to oesophageal
motility, stricture, or gastro-oesophageal
reflux
(the chapter 'Feeding the TOF Child'
also gives further information).
Remember that it is not necessary to
wait for a routine follow up appointment
if there is something that you are
worried about. The neonatal unit or the
ward where your baby was nursed, will
always be happy to speak to you over
the telephone, or to make special
arrangements to see your baby if
necessary.
Keep a record of important telephone
numbers next to your phone.
Finally ...
Many babies have their TOF repaired,
go home and thrive without requiring
re-admission to hospital or any
unusual treatment.
It is not always possible to predict
which babies will develop problems after
discharge, so it is wise to be prepared.
The more complicated your baby's
surgery, the more likely they a r e t o
h a v e d i f f i c u l t i e s w i t h
swallowing or feeding at some stage.
If your baby does have problems,
be reassured that they will diminish
with time.
The vast majority of TOF babies grow
up to be healthy adults who lead normal,
active lives.
48
The TOF Child
Genetic Counselling
for Tracheo-Oesophageal Fistula Dorothy Trump MD MRCP and Helen Firth MRCP DCH,
Department of Medical Genetics, Addenbrooke' s Hospital, Cambridge.
People often seek genetic counselling
to find out more about a specific condition
which has occurred in a member of the
family. They are referred to a Clinical
Genetics Centre by their General
Practitioner and will be offered an
appointment with either a Clinical
Geneticist (a doctor) or Clinical Nurse
Specialist in Genetics. During the
appointment the counsellor (the doctor
or nurse specialist) will often draw a
family tree in order to identify any other
relative who may be affected by the same
condition.
The process of genetic counselling
seeks to answer four common questions:
What is it?
Tracheo-oesophageal fistula (TOF)
can occur in isolation or as part of a
variety of different conditions. One UK
study found that just under half the
time (45%) TOF occurs in isolation as
the only developmental anomaly.1 In
the remainder of instances it occurs
together with other congenital anomalies.
Isolated TOF (i.e. TOF without any other
anomaly) is a 'one
off'occurrence. Where it occurs with
other anomalies there is usually an
underlying diagnosis, which falls into
one of four categories:
i) the commonest is an 'association'
such as VACTERL and CHARGE
ii) the next main group is that where
there is an underlying chromosome
abnormality e.g. Downs syndrome or
Edward's syndrome
iii) occasionally TOF is associated
with exposure to an environmental
agent e.g. excessive vitamin A or
maternal alcohol abuse. TOF is also
more common amongst twins
iv) very rarely TOF occurs as part of a
syndrome specified by a single gene
e.g. Treacher-Collins syndrome.
Below; a family tree
A geneticist will often ask for details of three generations of a family and will then draw a 'pedigree diagram' such as this to summarise the information gained. Studying the pattern of occurrence using this kind of chart helps to predict the
likelihood of recurrence.
49
The TOF Child
Above..
Diagnostic categories associated with TOE.
Why did it Happen?
It is usually not possible to answer this
question precisely. Most of the time we do
not know why a TOF occurred. However,
given the immense complexity of the
embryological processes involved in
forming a baby it is perhaps not surprising
that occasionally one of these processes
goes slightly awry.
During the sixth and seventh week of
pregnancy, the upper part of the primitive
foregut divides lengthways into a
digestive tract (oesophagus) and a
respiratory tract (trachea).2 If this process is
disturbed, a TOF may occur.
Above:
Embryology ... the oesophagus and
trachea develop from the primitive foregut
during the 6th and 7th week of pregnancy.
The disturbance can be due to one of the
situations described above – a genetic
or chromosomal problem affecting this
stage of development, or exposure to toxic
environmental agents – but is it usually not
possible to determine exactly why it
happened.
Will it Happen Again?
Fortunately in the great majority of
instances the answer is very reassuring
and recurrence is unlikely. Isolated TOF
occurs as a sporadic 'one-off' event and
the chances of a recurrence is
approximately 1%.3 Likewise TOFs
occurring as part of an 'association'
are usually sporadic and also have a
low recurrence risk, in the region of
3%. In the few instances where an
environmental agent has been identified,
steps can be taken to avoid exposure in
a subsequent pregnancy. It is therefore
appropriate to be optimistic about a future
pregnancy since the chance of a recurrence
of TOF is low.
Rarely TOF may be a feature of a
rare genetic disorder. It is listed as a
feature of more than 20 very rare genetic
syndromes.4 Many of these follow
predictable Mendelian inheritance
patterns; the recurrence risk is that of
the underlying disorder, which is usually
high e.g. 25-50%. The geneticist will
explain the inheritance pattern and
recurrence risks to members of the family.
In some cases a genetic test may be
available (e.g. Treacher-Collins syndrome)
and this would be discussed during the
consultation.
Specialist genetic advice should be
sought, but is especially important where:
i) a TOF is not isolated, but occurs in
conjunction with other anomalies
ii) more than one individual in the
family is affected by TOF.
50
The TOF Child
Category of individual Isolated 'Associations' Complex
TOF (e.g. VACTERL) TOFs
Parents with one affected child: seek individual risk to future children 1% 3% advice
Individual with TOF: seek individual seek individual seek individual
risk to their own children advice advice advice
Siblings of individuals with TOF: seek individual risk to their children
negligible negligible advice
Can Anything be Done
to Prevent a Recurrence? With the exception of TOFs
occurring as part of a rare genetic
syndrome, the chance of a recurrence of
TOF is low.
Detailed anomaly ultrasound
scanning by an experienced person can
sometimes indicate a TOF antenatally. If
there is oesophageal atresia the baby is
unable to swallow the amniotic fluid
which therefore accumulates. This is
tellned `polyhydramnios.' The baby's
stomach may also appear small.
Absence of these signs may provide
reassurance in a subsequent pregnancy.
Above:
TOF Hannah Sancto with her younger
sister Rebecca, a healthy baby born
without the TOF condition.
Above:
Recurrence risks for TOF.
References
1. David T. J. and O'Callaghan S. E. (1975). 'Oesophageal atresia in the South West of England.'
J Med Genet 12: 1-11.
2. Moore P. 'Development of the Trachea' in 'The Developing Human Clinically Oriented Embryology' (5th edition), WB Saunders, Philadelphia: 227-29.
3. Warren J. E. K. and Carter C. 0. (1979). 'Offspring of patients with tracheo-oesophageal fistula.'
J Med Genet 16: 338-40.
4. Winter R. B. M. (1996).
London Dysmorphology Database, Oxford University Press.
51
The TOF Child
Feeding the TOF Child
JWL Puntis BM DM FRCP FRCPCH, Consultant Paediatric Gastroenterologist,
The General Infirmary at Leeds, also Senior Lecturer in Paediatrics and Child
Health, University of Leeds and Celia Shore, Clinical Nurse Specialist, Queen
Elizabeth Hospital for Children, London.
Feeding problems, although not always
present in children born with TOF/OA,
are understandably a major cause of
concern to parents – who may feel that
one of their basic roles has been denied
by a child who will not or cannot eat.
In 1988 a questionnaire survey was sent
to all 230 families belonging to the
Tracheo Oesophageal Fistula Support
group (TOFS) requesting information
about their experiences with feeding. 124
replies were received and for comparison,
parents of 50 children without any kind of
surgical or medical problems were also
interviewed (the `control' group) using the
same questionnaire. While it is possible
that families of TOF children with severe
feeding problems would have been more
likely to answer the questionnaire than
those with children who had little or no
difficulties (indeed this seems to have been
the case given the high proportion of
children with delayed repairs /
oesophagostomy), some detailed and
useful information was collected which
gives an idea of just how frequent feeding
problems are, and how they tend to
improve over the course of time.
When analysing the results, the children
were divided into two groups; firstly those
who had their oesophagus joined up at the
first operation (`early join,' 74%) and
secondly – the minority – those who had
an oesophagostomy before more definitive
surgery at a later date (late join,' 24%).
Breast Feeding
There is no reason why TOF infants
cannot be breast fed once oral feeding
is permitted. In the survey, 50% of `early
join' mothers and 28% of 'late join'
mothers had breast fed their baby for an
average period of two and a half months.
This rate was a little below the
percentage of all infants who are breast
fed in England and Wales (about 65%,
falling to 45 % by 6 weeks).
Although some hospital staff may have
counselled against breast feeding, there is
no good reason for this in any baby who
would be considered able to bottle feed,
whether the surgical join-up operation was
an early or late one.
No mothers in the survey who breast fed
reported giving up due to difficulties
related specifically to a TOF repair.
Weaning and Solid Foods
Feeding times should be relaxed and
unhurried, with the baby held relatively
upright to assist swallowing.
Weaning does not need to be delayed
and solids can be introduced from 3 to 4
months. This is important since it
seems likely that children are particularly
receptive to new tastes from around 4-6
months of age, and become more
conservative if their experience remains
limited. Difficulty coping with lumpy
food may mean that soft puree baby
foods must be given for longer than
normal. When lumps are introduced,
plenty of fluid should be given with
meals, and the
52
older child must be reminded to chew food
well and not to hurry. The social aspect of
meal times is important to all of us, and
eating with others has an effect of
increasing food intake. The coughing and
spluttering child should be allowed to
recover and continue with eating rather
than being made to feel excluded from the
rest of the family meal.
Practical Tips on Feeding
Parents need a great deal of support
and advice when it comes to weaning
and eating, and families should be
encouraged to ask to see the dietician
each hospital visit, or a specialist nutrition
nurse if one is available in their area.
Questions and discussions with the GP
and health visitor about feeding
management should also be encouraged
together with use of appropriate
supporting information sheets or leaflets
(see references).
Parents should be encouraged to use
home cooked food when changing from
puree foods to more lumpy textures,
since many commercial wet and dry
foods contain lumps, which my cause
coughing and choking
Vegetables, soft rice or pasta
can be well mashed with a fork,
adding pureed meat or fish. Sauce
or gravy to make food moist is
valuable for swallowing food
trouble free. Fresh fruit may be
mashed, such as banana, ripe
peaches or strawberries.
The TOF Child
older child must be reminded to chew food
aspect of
of us, and
coughing and
be allowed to
recover and continue with eating rather
than being made to feel excluded from the
eal of support
and advice when it comes to weaning
encouraged to ask to see the dietician at
each hospital visit, or a specialist nutrition
their area.
with the GP
management should also be encouraged
supporting information sheets or leaflets
Parents should be encouraged to use
home cooked food when changing from
textures,
and dry
my cause
Fibrous fruits like pineapple,
oranges and blackcurrants are not
suitable for weaning foods, and
should be avoided.
Fruits that are cooked such as apples and
pears can be mixed with yoghurt or
fromage frais to introduce babies and
young children to more interesting and
nutritious foods.
As the
baby's teeth
come through at about 7-8 months,
encouragement with chewing may be
stimulated with finger foods that are crisp
or firm e.g. sweet and savoury crackers,
grated cheese, well cooked carrot slices,
green beans or thin strips of ham. All of
these will dissolve easily once in the
mouth and so reduce any coughing and
choking that may
occur from pieces becoming stuck.
Drinks from a beaker or feeding bottle help with
swallowing.
The TOF Child
Fruits that are cooked such as apples and
young children to more interesting and
stimulated with finger foods that are crisp
or firm e.g. sweet and savoury crackers,
carrot slices,
of ham. All of
easily once in the
any coughing and
occur from pieces becoming stuck.
53
The TOF Child
Reluctance to Feed
Reluctance to feed can arise in any
child who has been very ill, unable to
eat, and therefore deprived of the normal
oral stimulation which is part of
feeding. Inflammation of the oesophagus
due to reflux or stricture can also be a
cause of poor feeding or feed refusal
since swallowing is then painful; this may
occur even when vomiting is not a
prominent symptom.
Most TOF children should not fall
into this category. Those who cannot have
their oesophagus joined up early on are
given a cervical oesophagostomy and
receive 'sham feeds' by mouth. Food then
come out of the upper pouch of the
oesophagus, which now opens onto the
neck. At the same time, feeds are given
directly into the stomach through a
gastrostomy tube. In this way, the child
learns how to suck, swallow, and to
enjoy different tastes and textures until
they have grown enough for further
surgery which will allow them to eat food
in the normal way.
Feed-Related Problems
The following graph shows how often
parents reported feed related symptoms
with two or more milk feeds each day.
The average age for starting solids
was 4 months in the normal infants,
and 6 months in the TOFs. Later on,
normal infants were on average
introduced to lumpy foods at 8 months
and TOFs at 12 months.
Whilst it is clear that even normal
children may have some feed related
problems, the questionnaire showed
that the frequency of these had halved
by weaning, and they had virtually
disappeared completely by the age of
one year. In contrast, TOF children were
five or six times as likely to be considered
slow with feeds or to experience coughing
and choking, and twice as likely to vomit
during feed or refuse to take milk
altogether. This situation had not
changed very much by the time solid
food was being introduced, but did seem
to improve considerably over a longer
period of time. Of 88 TOF children at
one year of age, only 18 (20%) were
free of all feed related symptoms, but
amongst 21 children who had been
followed up for s e v e n y e a r s , 1 2
( 5 7 % ) w e r e completely symptom
free.
Once children in 'late join' had
undergone further surgery and closure
of oesophagostomy they had a similar
frequency of feeding difficulties to `early
join' children.
54
Troublesome Foods
Although it is clear from this and other
studies that feeding problems generally do
get better with time, some TOFs may
continue even in adult life to associate
swallowing problems with
certain types of food which they
then avoid. This is probably
because there is still some incoordination in the
oesophagus. In the
survey the most
frequently identified
"problem" foods were
meat (37% of children), apple (23%),
bread (23%), oranges (14%) and raw
vegetables (12%). These are foods which take longer to grind
up when chewing and tend to be
swallowed as larger lumps.
In fact, a further 38 problem
foods were listed, but often only
one item was mentioned by any
one family. Making sure that
food is well
chewed seems to be the message here,
together with the acknowledgement
that it is often impossible to predict
whether or not particular foods will cause
difficulties in any one individual.
Feeding Difficulties
There are a number of reasons why TOF
children have feeding difficulties.
Normally, food is propelled through
the oesophagus by a wave of muscle
contraction which sweeps it along down
to the stomach.
In TOF children, this process is never
completely normal (even in the child
without symptoms). Coughing or choking
occurs when incoordination of the
oesophageal muscle means that food or
fluid stays in the oesophagus and spills
back into the trachea. Sometimes on a
radiograph, fluid can
The TOF Child
Although it is clear from this and other
studies that feeding problems generally do
get better with time, some TOFs may
continue even in adult life to associate
meat (37% of children), apple (23%),
bread (23%), oranges (14%) and raw
chewed seems to be the message here,
together with the acknowledgement
whether or not particular foods will cause
There are a number of reasons why TOF
Normally, food is propelled through
contraction which sweeps it along down
In TOF children, this process is never
completely normal (even in the child
choking
the
food or
and spills
back into the trachea. Sometimes on a
be seen to go up and down the
oesophagus like a yo-yo.
Less commonly, there may be a
narrowing (stricture) at the point in the
oesophagus where the surgeon has joined
the two ends together, which
simply doesn't open up to let food
through. This is usually caused by
a natural process of scarring as
the wound heals, and is
treated using dilatations
(stretching). Gastro-
oesophageal reflux is
another cause of
feeding difficulties; both
of these problems are discussed in a
separate chapter.
Sometimes food — or occasionally
an unusual object swallowed by a
young child, often
termed a 'foreign
body' — may get
stuck in the I —
oesophagus and
cause a blockage
(obstruction). This
can be either 'functional'
(due to incoordination of the
oesophageal muscle, so that food is not
propelled down to the stomach) or
`mechanical' (due to a stricture i.e. a
physical narrowing of the oesophagus
due to scar tissue such that solids cannot
pass through).
Whatever is involved in the blockage,
the obstruction it causes may mean that
swallowing even saliva from the mouth
is impossible.
If the child becomes very distressed
the blockage fails to clear within an hour
or two despite taking sips of water,
hospital assessment will be necessary. You
should telephone your local paediatric unit
and arrange to go to the ward. This kind of
problem is more common in the early
years after surgery and rare beyond ten
years of age.
The TOF Child
narrowing (stricture) at the point in the
oesophagus where the surgeon has joined
through. This is usually caused by
a natural process of scarring as
treated using dilatations
oesophageal reflux is
feeding difficulties; both
or occasionally
an unusual object swallowed by a
young child, often
termed a 'foreign
may get
oesophagus and
cause a blockage
muscle, so that food is not
physical narrowing of the oesophagus
due to scar tissue such that solids cannot
Whatever is involved in the blockage,
mean that
the mouth
If the child becomes very distressed or
the blockage fails to clear within an hour
hospital assessment will be necessary. You
r local paediatric unit
and arrange to go to the ward. This kind of
years after surgery and rare beyond ten
55
Oesophageal Function
Normal: wave of peristalsis moves food down the oesophagus
Oesophageal incoordination: muscular activity is abnormal
Stricture: narrowing prevents food passing through the oesophagus
56
The TOF Child
Oesophageal Function
Normal: wave of peristalsis moves food down the oesophagus
Oesophageal incoordination: muscular activity is abnormal
Stricture: narrowing prevents food passing through the oesophagus
The TOF Child
Summary
From the survey it appeared that most
parents felt they had not been warned
about feeding difficulties likely to be
encountered at home, or given advice
regarding diet and weaning. Some parents
stated that feeding difficulties dominated
their lives and many expressed feelings
of isolation and helplessness.
Further Reading
1. Tracheo Oesophageal Fistula – TOF's
Guide to Weaning Foods. Leaflet by
Celia Shore, sponsored by Milupa
and available from the Queen
Elizabeth Hospital, London E2 8PS.
ISBN 1 898081 15 8.
2. "What's for Dinner Today, Mum?"
A short guide to weaning.
Leaflet available from the Department
of Health, PO Box 410, Wetherby,
LS23 7LN.
3. Puntis JWL, Ritson DG,
Holden CE, Buick RG.
Growth and feeding problems after
repair of oesophageal a t res ia .
Arch Dis Child 1990; 65: 84-88 (this is
the TOFS survey).
4. Smith IJ, Beck J.
Mechanical feeding difficulties
a f t e r p r i m a r y r e p a i r o f
oesophageal atresia. Acta Paediatr
Scand 1985; 74: 237-9.
5. Orringer MB, Kirsh MM, Sloan H.
Long-term esophageal function
following repair of esophageal atresia.
Ann Surg 1977; 186: 436-43.
6. Dellert SF, Hyams JS, Treem WR,
Geertsma MA. Feeding resistance
and gastroesophageal reflux in
infancy. J Paediat Gastroent Nutr
1993; 17: 66-71.
The TOF Child
From the survey it appeared that most
warned
to be
encountered at home, or given advice
regarding diet and weaning. Some parents
stated that feeding difficulties dominated
y expressed feelings
TOF's
Leaflet by
by Milupa
E2 8PS.
"What's for Dinner Today, Mum?"
Leaflet available from the Department
of Health, PO Box 410, Wetherby,
Growth and feeding problems after
repair of oesophageal a t res ia .
88 (this is
a f t e r p r i m a r y r e p a i r o f
oesophageal atresia. Acta Paediatr
Orringer MB, Kirsh MM, Sloan H.
repair of esophageal atresia.
Dellert SF, Hyams JS, Treem WR,
Geertsma MA. Feeding resistance
infancy. J Paediat Gastroent Nutr
In children who have been joined up
early on, swallowing difficulties should
be anticipated – although some seem to
have no problems at all. Experienced
nursing staff, dieticians, speech therapists
and occupational therapists may be able to
give useful advice. Much practical and
emotional support for parents struggling
with feeding difficulties has come from
other parents within support groups such as
TOFS, many of whom have seen their
children get over their problems and grow
to be strong and healthy.
Quotations from TOF s, their families and friends and medical professionals
"Jacob is now 17 months old and won't
eat or drink anything. We try and be
positive that things will improve, but
feel we need more help on the psych
ological difficulties of re-learning
swallowing and not retching and
panicking every time food goes into the
mouth. We have had to fight hard for
the support we do receive, which is
hard with all your other worries."
Above;
James Wharfe at 1 year, who had a
gastrostomy tube until 27 months old. At 14
years old, he is said to eat like a horse!
The TOF Child
In children who have been joined up
early on, swallowing difficulties should
although some seem to
nursing staff, dieticians, speech therapists
and occupational therapists may be able to
Much practical and
for parents struggling
with feeding difficulties has come from
other parents within support groups such as
many of whom have seen their
get over their problems and grow
and friends and medical
"Jacob is now 17 months old and won't
eat or drink anything. We try and be
positive that things will improve, but
feel we need more help on the psych-
panicking every time food goes into the
mouth. We have had to fight hard for
gastrostomy tube until 27 months old. At 14
57
The TOF Child
"The pressure on TOF families is huge.
When you tube feed it's such a
performance wherever you go. My
husband used to go fishing and I was so
desperate one day to get out of the house I
took the stand and fed her out of the car
window in a field. The sickness and mess
was always a problem. You were always
tense hoping she wouldn't bring any
food back and at other children's parties
you had to explain to people without being
boring. Those curled up bibs were a
godsend."
"On buying him his first ice-cream cornet
he turned it upside down as he was
more interested in the wafer as he hadn't
been allowed it before."
"We used to lie to strangers about his age
– he only weighed 12 lbs aged one. a
photographer said how alert he was for a
10 week old (he was 10 months at the
time but I didn't explain.) He gulped some
fillet steak which he had taken from
someone else's plate age 6. The surgeon
said there was enough meat for a pie
stacked in his gullet. I was very
embarrassed, it seemed as though I'd been
feeding him giant fillet steaks."
"Our lasting memory of the postoperative
period has to be the sham feeding, and the
look of horror on the face of a workman,
who was artexing our ceilings. He came
to ask me a question as I was feeding
Alexander and the vision of me standing
with a syringe of milk in one hand and a
bottle in the other along with the
squelching noise coming from Alexander's
neck region, was all too much for him, he
made a very fast exit."
"But we have to admit all the hard work
of sham feeding is worth every second, just
to see Alexander tucking into his meals
with no problems is a pleasure."
"Breast feeding was a very difficult task.
After surgery Catherine refused the breast
and would fight whenever she came in
contact. I expressed and tried to put her to
the breast for 10 minutes at every feed. At
about 4 weeks I discovered nipple shields
and so she fed from me happily for the first
time. After a hernia repair at 3 months
while still half asleep she fed without the
nipple shield! It was a wonderful moment.
Catherine was breast fed for 17112 months.
I feel that breast feeding her was my
greatest ever achievement."
"I have always been told he will eat one
day, but as the months pass you do start to
wonder if people are telling you the
truth. Well last month Matthew actually
took some yoghurt in his mouth and
swallowed it. I just couldn't believe it, I
don't know how to explain it. He still
takes small amounts but it is a start and
I can actually see a small hole at the end
of the tunnel."
"I had no idea what was in store when I
started giving him solid food. It took hours
to get anything into him; even scrambled
egg had to go through a sieve, and often
enough he brought it all back up again.
At one stage, when he was a little older,
I used to count out, individually, ten Rice
Krispies, which (if I was lucky) he could
eat, without milk. He used to look at the
food the other children had, and say
hopefully 'tiny bit?' (having heard me
say he could try a tiny bit on a few
occasions). He longed to eat crisps, but
could never keep them down. It was
wonderfu l when we found he could
cope with Skips. His brother and sister
naughtily taught him to make a sicky
noise and say "been sick," which had
me rushing up with a cloth a few times, to
find it wasn't true!"
58
"I nearly burst with love and pride when we first took him to a Beefeater Restaurant (he was 3) and he managed a Mister Men meal of soup, fish-stars, potato-letters and baked beans, followed by clown sundae ice-cream, without getting stuck once."
Above:
Freddie Clamp-Gray enjoying his 2nd
birthday party with the help of Wotsits, thin
crust pizza and potato salad.
"One amusing thing that Philippa sometimes does when she's stuck is to bounce around on her hopper. I don't know if it helps but she at least feels as if she's doing something."
"She is used to drinking at mealtimes – between mouthfuls and before she starts eating – and even close relatives cannot help themselves commenting that the f luid wil l f ill her up. She ignores this and so do we, because this together with chewing food very extensively has enabled her to eat more or less "normally." If she feels unable to cope with something in her mouth, she will remove it – say grape skins or a tough piece of meat."
The TOF Child
"I nearly burst with love and pride when we first took him to a Beefeater Restaurant (he was 3) and he managed
stars,
cream,
birthday party with the help of Wotsits, thin
sometimes does when she's stuck is to bounce around on her hopper. I don't
t least feels as
"She is used to drinking at mealtimes between mouthfuls and before she
and even close relatives cannot help themselves commenting that the f luid wil l f ill her up. She
because this
extensively has enabled her to eat more or less "normally." If she feels unable to cope with something in her
say grape
"Rachelle' s school friends don't tend to invite her home for tea or to parties because their parents are terrified of her getting something stuck and not being able to cope. This is very unfair on Rachelle. We have had to stop going out to restaurants so often and can't just pop into McDonald' s because Rachelle can't cope with the food there."
"We know that Olivia feels different at mealtimes, at nursery, birthday parties and family gatherings. She looks nervous and uncomfortable. As she watches
what others are doing you can tell she wants to join in by eating normally but doesn't feel she can or know how."
"Our son has been extremely lucky in that he has never had any problems following the repair of his OA at birth. He was breast fed until 11 months; went on to solids at four months and eats whatever we eat. In the beginning I would never let anyone else feed him would not have those worries now."
"Laura's school provides supervised lunch times but they call me in if she has a bad choke. I asked them to normalise the situation as much as possible which they have done. I don't want Laura to feel too different from the other children."
"Eleanor has been attending nursery school for a few hours a week since she was 19 months old. She stayed for lunch from 21 months old and we ensured the nursery nurses were fore-warned about Eleanor's occasional problems of getting food stuck. Also that they were not to put any pressure on her to eat (I could top her up with a gastrostomy feed when I got her home). We feel sure that Eleanor was encouraged with her eating by sitting with and watching other
children at mealtimes."
The TOF Child
"Rachelle' s school friends don't tend to invite her home for tea or to parties because their parents are terrified of her getting something stuck and not being able to cope. This is very unfair on Rachelle. We have had to stop going
often and can't
Rachelle can't cope with the food there."
"We know that Olivia feels different at mealtimes, at nursery, birthday parties and family gatherings. She looks nervous and uncomfortable. As she watches
others are doing you can tell she wants to join in by eating normally but
"Our son has been extremely lucky in that he has never had any problems following the repair of his OA at birth.
; went on to solids at four months and eats whatever we eat. In the beginning I would never let anyone else feed him I would not have those worries now."
"Laura's school provides supervised lunch times but they call me in if she
normalise the situation as much as possible which they have done. I don't want Laura to feel too different from
"Eleanor has been attending nursery school for a few hours a week since she was 19 months old. She stayed for lunch rom 21 months old and we ensured the
warned about getting
not to put could top
her up with a gastrostomy feed when I eel sure that Eleanor
eating by
59
"He started on solid foods (a very, very
sloppy consistency) at five months old.
failed at all attempts to introduce any
second stage foods and eventually gave
up trying until I felt he was more able.
He ate 'mush' until he was quite old.
He disliked intensely any commercial baby
foods and I always gave him ordinary food
blended and sieved. His general health
improved during the summer of 1996 and
I requested to visit a speech therapist
after reading an article on a child with an
eating disorder. Through my own efforts
we visited a wonderful lady who
specialised in feeding difficulties. This
visit began the road to recovery. Kris
was introduced to 'bite and dissolve'
finger foods which he took to instantly. I
was advised never to introduce lumps into
food but just to make the consistency
firmer. Over the next few months I
followed her advice to the letter and Kris
made rapid progress. He is now 23
months old and eats an almost normal
diet. I do not however give him foods
that I feel may cause a problem. I spend
more time preparing Kris's food. He eats
all cooked veg and fruit, but I avoid
giving him all foods of a solid
consistency such as raw veg, apples and
foods in a natural skin such as sausages
and oranges. Meat he has shredded to a
crumb-like state."
"Sarah says she feels different at school
dinner time because she has two drinks
instead of one, and other children say it's
not fair. She tells them it's because she
gets `stucks' ."
"Whenever Sarah attends a party with
her friends I tell the parents her problem
but without trying to alarm them. School
have been good, but sometimes I feel if she
hasn't got something stuck for some time,
they forget to give her extra drinks. I
have to go and remind them."
60
The TOF Child
"He started on solid foods (a very, very
sloppy consistency) at five months old. I
failed at all attempts to introduce any
second stage foods and eventually gave
up trying until I felt he was more able.
He disliked intensely any commercial baby
foods and I always gave him ordinary food
summer of 1996 and
he took to instantly. I
to introduce lumps into
as sausages
two drinks
children say it's
have been good, but sometimes I feel if she
"Harriet's brother is four years old. He
is wonderful with Harriet and asks if
she can eat things before he gives her
anything. He has accepted her problems
more easily than anyone."
"Sometimes when I have things like lamb
chops or chewy meat I feel a lump in
my throat, so to get rid of it I push with
my thumb to help the food go down."
"Having a TOF child is a full time job.
My son Matthew is 2 and is still tube
fed overnight and we have just started
introducing food to him. A meal time
for Matthew takes over an hour to
complete. I can't go to work as I can't
leave him with anybody."
"...one of the hardest things is when you
see other children eating in, say, a Mac-
Donald's – they don't know how lucky
they are. Matthew is the happiest, most
contented child I know. He never cries,
whines or complains even though he has
probably had more operations than most
people would have in a lifetime."
Above:
Leslie Brown aged 11 months. Leslie took some 16 months to get to the stage of attempting to eat finger food; at 11
months she had a jejunostomy tube.
The TOF Child
Strictures and Reflux Mark D Stringer MS FRCS FRCP, Consultant Paediatric Surgeon, Leeds.
Both of these are quite common in infants
born with oesophageal atresia with or
without a tracheo-oesophageal fistula, but
only a minority will require open surgery to
correct the problems.
What is a Stricture?
A s t r i c t u r e i s t h e c o m mo n e s t
problem requiring surgical treatment after a
TOF repair, occurring in about one third of
babies, particularly in the first few months
after surgery. It is a narrowing in the
oesophagus, usually due to scarring at the
join (the anastomosis) between the upper
and lower parts of the oesophagus, in
which case it is called an anastomotic
stricture. Occasionally, a stricture develops
lower down in the oesophagus and in this
area it is usually due to gastro-oesophageal
reflux. Very rarely, children with TOF/ OA
are actually born with a stricture in the
lower oesophagus; this is termed congenital
stricture.
Anastomotic strictures are more likely
in certain infants, in particular, those
where the surgical join in the oesophagus
has been particularly difficult because of
the wide gap between the ends of the
oesophagus. If the anastomosis is under
tension, the blood supply to the join site is
impaired and it will tend to heal as a
tougher and more fibrous scar. Some of
the old-fashioned stitch materials like
silk were more likely to cause strictures.
Strictures are also believed to be
worsened by gastro-oesophageal reflux;
acid from the stomach damages the
delicate join in the oesophagus and
encourages more scarring.
Symptoms of Stricture
Typically, symptoms develop within a
few weeks or months after the baby's
oesophagus has been joined up. The baby
may be slower to feed or may choke and
splutter during the feed. This is not a
'one-off' episode, but happens with each
feed. A stricture may become apparent for
the first time when the baby is tried with
solid food. Advice should be sought from
the surgical unit.
Not all swallowing problems are due
to strictures. We know that abnormal
contractions of the oesophagus (called
dysmotility) and difficulties with learning
to feed are important causes of feeding
problems in some TOF children (see
Chapter 'Feeding the TOF Child').
In older children with a stricture,
there may be difficulties swallowing
lumpy food but it is important to
remember that TOF children often have
difficulties in this area anyway.
Types of Stricture
61
If a child has previously coped well
with a certain consistency of solid food
and then starts to have problems with
swallowing, this suggests a stricture.
Dealing with obstructions
Occasionally, a piece of solid food may
actually get stuck in your child's
oesophagus (called a bolus obstruction).
This may be vomited back or remain stuck
and cause distress.
Once the child has calmed down,
see if they are dribbling their saliva.
If they are, then there is a complete
blockage and you will need to seek
expert medical attention.
If the child is not dribbling, try and
get them to sip some water since food
materials will often dissolve and pass
through. If the child cannot tolerate
liquids within an hour or two then you
will need expert advice.
62
The TOF Child
with a certain consistency of solid food
Occasionally, a piece of solid food may
This may be vomited back or remain stuck
If the child is not dribbling, try and
get them to sip some water since food
materials will often dissolve and pass
thin an hour or two then you
A f o o d b o l u s o b s t r u c t i o n c a n
happen in an oesophagus with only a
minor narrowing and typically occurs
with unchewed lumps of meat, apple,
sausages, etc. However, a food bolus
obstruction can also indicate a stricture
that needs dilatation. Very occasionally, a
stricture comes to light if a child swallows
an unusual object (known as a foreign
body) such as a coin which then gets
stuck.
Diagnosis of Stricture
A stricture can be confirmed using
either of two techniques:
Radiography
The child is given a drink of barium
or some other safe liquid which shows
up white on a radiograph. This is done
in the radiography department where
the passage of the dye (called a contrast
material) is watched in motion as it goes
down the oesophagus. The use of X-rays in
this way is called fluoroscopy.
A stricture may be seen.
Alternatively, there may be minimal
narrowing in the oesophagus in which
case the feeding difficulty has another
cause such as oesophageal dysmotility.
Endoscopy
The other way to diagnose a stricture
is to look down the oesophagus with a
telescope (called oesophagoscopy), a
procedure which requires a short
Left:
Anastomotic stricture: contrast material
(e.g. barium) is seen outlining the
oesophagus down to the s tomach ( the
white area at the bottom). An anastomotic
stricture can be seen about a third of the
way down this view. The white stripes passing
from top left diagonally downwards are the
ribs; the thicker hand passing them at
right angles in the top left is the spine.
a
swallows
rays in
Alternatively, there may be minimal
narrowing in the oesophagus in which
case the feeding difficulty has another
general anaesthetic and is necessary in
cases of persistent food bolus obstruction
since the obstructing material can be
removed with the aid of the endoscope.
Grasping forceps can be passed
through the endoscope and the material
withdrawn with the scope, or the food
can be dissolved with squirts of water,
or occasionally, the food bolus can be
simply pushed on down the oesophagus
into the stomach.
Above:
Foreign body in the oesophagus.
The patient has swallowed dye (contrast
material) which has flowed around the
foreign body to outline the oesophagus.
The foreign body (the circular, white area)
is lodged above an oesophageal stricture.
The TOF Child
tic and is necessary in
cases of persistent food bolus obstruction
removed with the aid of the endoscope.
material
withdrawn with the scope, or the food
of water,
bolus can be
the oesophagus
The patient has swallowed dye (contrast
material) which has flowed around the
foreign body to outline the oesophagus.
The foreign body (the circular, white area)
is lodged above an oesophageal stricture.
Treatment of Stricture
A stricture can be successfully treated
by stretching it up (called a dilatation).
This is done under a general anaesthetic
but can be carried out in several ways.
Most commonly, the surgeon passes
telescope (called an endoscope or
oesophagoscope) down through the mouth
to the level of the stricture. Then a fine
plastic rod is passed through the stricture.
A series of gradually larger rods are then
passed through until the stricture has been
sufficiently dilated.
An alternative method is for an X
ray doctor (radiologist) to pass a thin
flexible wire (known as a guide-wire)
through the stricture whilst checking
its position with a radiograph. Once
the wire is safely through the stricture
and into the stomach, a thin hollow plastic
tube is passed over the wire and down into
the oesophagus. This tube has a balloon
on the end which can be gently inflated
when it lies across the stricture. As the
balloon is blown up, the stricture is
dilated (called balloon dilatation).
The TOF Child
A stricture can be successfully treated
by stretching it up (called a dilatation).
general anaesthetic
out in several ways.
Most commonly, the surgeon passes a
mouth
a fine
plastic rod is passed through the stricture.
A series of gradually larger rods are then
passed through until the stricture has been
An alternative method is for an X-
ray doctor (radiologist) to pass a thin
wire)
through the stricture whilst checking
the wire is safely through the stricture
and into the stomach, a thin hollow plastic
down into
has a balloon
ich can be gently inflated
stricture. As the
63
The TOF Child
When performed by experts both
methods are safe and effective and relieve
the symptoms. The child is able to feed
within a few hours and is often able to
go home the same day or soon after. A
chest radiograph may be performed to
check that there is no sign of a
complication (see below).
A very small number of strictures keep
coming back despite repeated dilatations.
In these circumstances any gastro-
oesophageal reflux must be treated since
the presence of stomach acid in the
oesophagus may be aggravating the
situation. Occasionally, further operative
surgery may be needed.
Some surgeons try injecting the
stricture scar tissue with steroids which
have an anti-inflammatory effect. This
injection can be carried out with a special
needle through the endoscope. The
idea is that when the stricture is then
dilated it might heal with less scarring
and therefore be less tight.
Other options include actually cutting
out the stricture and rejoining the
oesophagus or considering a major
operation to replace most of the
oesophagus. Such operations include
removing most of the oesophagus and
using a piece of large bowel to bridge
the gap between the healthy upper
oesophagus and the stomach (colonic
interposition) or bringing up the stomach
through the chest and joining it to the
upper oesophagus in the neck (gastric
transposition). (See chapter on
Oesophageal Substitution Procedures).
Complications of
Stricture Dilatation
Stretching a stricture can lead to
complications but these are rare when
the procedure is performed by
experienced personnel.
The most worrying complication is
splitting the oesophagus which causes it
to leak (called an oesophageal perforation).
This is most often recognised by seeing a
pocket of air outside the oesophagus on
the chest radiograph or if the child
becomes unwell after the dilatation. An
oesophageal perforation can be dangerous
and requires prompt treatment with
antibiotics, intravenous fluids and
sometimes, a tube put into the chest to
drain away any infected fluid. Oesophageal
perforations are rare and this kind of
treatment would allow most to heal up.
Only rarely would an operation be required
for this complication.
Any child born with a cardiac (heart)
abnormality ought to receive antibiotics
before the dilatation to minimise any
chance of an infection settling in the
heart.
Anastomotic strictures usually get better
after one or two dilatations but
occasionally, several stretches are
necessary. If the stricture keeps coming
back, it might be because there is a
p a r t i c u l a r l y d e n s e s c a r a t t h e
oesophageal join, it may be because gastro-
oesophageal reflux is making the stricture
worse, or it may be because there is a
congenital stricture. These problems all
require separate solutions which may
involve further surgery.
What is Reflux? Gastro-oesophageal reflux (GOR) is
the involuntary passage of gastric contents
into the oesophagus. With GOR, as the
stomach empties into the bowel (the
duodenum), part of its contents are also
squeezed back into the oesophagus. Since
the stomach contents are acidic ,
i r r i ta t ing acid passes into the
oesophagus.
The lower oesophagus has several
features that normally prevent GOR
64
The TOF Child
causing problems. There is a sort of valve
in the lower oesophagus made up of a
high pressure area, a sharp angle between
the oesophagus and stomach and a
pursing of the lining of the oesophagus at
its junction with the stomach. This valve-
like mechanism is not s t rongly
developed in normal babies and
explains their tendency to vomit. This
tendency reduces during the first few
months or years of life as the antireflux
mechanisms in the oesophagus get
stronger.
In TOF babies, GOR is even more
common. This is partly because the
o e s o p h a g u s h a s n o t d e v e l o p e d
normally and partly because repair of
the oesophageal atresia often pulls the
junction between the oesophagus and
stomach upwards, weakening the
antireflux barrier. TOF babies can
therefore vomit more easily than normal
babies. In time the tendency to GOR
lessens, but in some children with
repaired oesophageal atresia, GOR causes
major problems. GOR is more likely to
be a problem if there has been a wide
gap between the oesophageal ends and the
repair has been particularly tight.
Antireflux mechanisms
Symptoms of Reflux
Remember that all healthy babies
have a tendency to GOR which
improves with time, so the question
really is whether GOR is causing a
problem in your baby – rather than
whether GOR is occurring at all.
Reflux causes frequent vomiting after
feeds. This is not the small mouthfuls of
vomit (`possets') seen in all babies, but
the vomiting of large amounts of the feed.
This can happen immediately after a feed
or occur repeatedly right up until the
next feed. If GOR is severe, the baby has
great difficulty gaining weight because
he/ she is unable to absorb all of the feed.
Other problems may also occur.
The oesophagus may become sore
from the acid (the adult equivalent
which is heartburn) leading to
irr itabil ity and poor feeding in the
child. In some cases, bleeding from the
oesophagus causes anaemia or signs of
blood in the vomit (haematemesis).
Anastomotic strictures can be made worse,
as mentioned previously.
Rarely, reflux can happen so quickly
that it leads to the baby inhaling vomit,
leading to a chest infection or difficulty
with breathing. In severe cases, the
baby may temporarily stop breathing
(called 'apnoea').
Most TOF babies have mild reflux
( i .e . a tendency to vomit ing af ter
feeds) which gets better by itself or
with medicines but a few have severe
reflux which needs intensive treatment
with several medicines or even an
operation.
65
The TOF Child
Diagnosis of Reflux
Listening to the history often gives the
doctor vital clues as to whether GOR is
causing problems. Sometimes, it is
difficult to tell whether a TOF baby's
feeding problems are due to GOR or a
stricture or some other problem. There are
several ways to diagnose GOR.
Radiography
The commonest method is to get the child to swallow some dye (contrast
material) in the radiography department.
Normally, the stomach contracts and
empties the dye into the first part of
the bowel (the duodenum). The dye is
prevented from passing upwards into
the oesophagus by the valve-like
mechanism mentioned above. If the child
has gastro-oesophageal reflux, as the
stomach contracts to empty, the dye is
also seen travelling up the oesophagus.
The severity of the reflux can be
assessed by how much dye passes
upwards and whether it just enters the
lower oesophagus or passes right up to
the throat.
pH Study
This involves passing a fine tube
through your child's nose down into the
lower oesophagus. A radiograph is
sometimes necessary
to check the position
of the tube. The tip of
the tube has a small
sensor which
measures the
surrounding acidity
(or pH) which is
recorded by a small
computer. The pH
study usually runs
for 24 hours, during
which the child can
eat and drink and live
a fairly normal life
within the constraints
of the equipment (as
carried by the boy
shown here).
Any antireflux
medicines must be
stopped before the test
to avoid giving a false reading. Normal children have a
little acid in the lower oesophagus for
brief periods only (less than 5-10% of
the time) but children with significant
GOR have acid present for much longer
periods.
Left:
Gastro-oesophageal reflux: contrast
material (white) is seen passing up the
oesophagus from the stomach (the round,
solid-looking area on the right), as
indicated by the open arrows. The slightly
cloud-like accumulations of contrast
material below and to the left of the
stomach represent stomach contents
which have passed on beyond the stomach
into the duodenum and small intestine.
66
Endoscopy
Persistent gastro-oesophageal reflux
of acid causes the lining of the lower
oesophagus t o become in f lamed
(called oesophagitis) and this redness
and soreness can be seen by endoscopy
(oesophagoscopy).
Above:
Oesophagoscopy: normal lower
oesophagus, viewed through an endoscope.
Above:
Oesophagoscopy: endoscopic view of
lower oesophagus, with oesophagitis.
The TOF Child
oesophageal reflux
of acid causes the lining of the lower
oesophagus t o become in f lamed
(called oesophagitis) and this redness
seen by endoscopy
oesophagus, viewed through an endoscope.
lower oesophagus, with oesophagitis.
Milk scans
These are used in some centres. This
technique is also known as gastro-
oesophageal scintigraphy and involves
the baby taking a small amount of radio
active material by mouth, followed by a
milk feed; the baby is then scanned by a
special camera which detects the
distribution of the radioactive marker.
test does not require sedation and the
radiation dose is less than that involved in
performing a barium meal. The presence of
radioactivity in the oesophagus indicates
GOR but there is none of the anatomic
detail seen with a barium swallow which is
the more popular investigation.
Treatment of Reflux This depends on the severity of the
reflux and how much trouble it is causing
your child.
Mild GOR, which is probably present
in all TOF infants (and many otherwise
healthy babies), tends to improve spont
aneously with age and often gets a little
better when the baby is able to wean on to
more solid food. Simple measures that are
helpful include changing the position of
your child (posturing) and, in babies,
thickening the milk.
Posturing
There has been some argument about
the best and safest posture to counteract
reflux. GOR tends to be worse when lying
flat and therefore a gentle raise of the
head of baby's cot can be useful.
can be done by putting a pillow or folded
blanket under the mattress to create a
gentle head-up slope. Never attempt to let
your baby sleep directly on a pillow
which could be dangerous. During the day,
keeping your baby propped up in a chair
(but not slumped over) can help prevent
reflux. Changing the nappy before feeds
makes vomiting
The TOF Child
. This
oesophageal scintigraphy and involves
the baby taking a small amount of radio-
active material by mouth, followed by a
milk feed; the baby is then scanned by a
ioactive marker. The
the
radiation dose is less than that involved in
performing a barium meal. The presence of
radioactivity in the oesophagus indicates
GOR but there is none of the anatomic
llow which is
reflux and how much trouble it is causing
Mild GOR, which is probably present
in all TOF infants (and many otherwise
improve spont-
aneously with age and often gets a little
better when the baby is able to wean on to
more solid food. Simple measures that are
helpful include changing the position of
your child (posturing) and, in babies,
re has been some argument about
the best and safest posture to counteract
reflux. GOR tends to be worse when lying
the
head of baby's cot can be useful. This
can be done by putting a pillow or folded
attress to create a
attempt to let
which could be dangerous. During the day,
keeping your baby propped up in a chair
(but not slumped over) can help prevent
feeds
67
The TOF Child
less likely than doing so when his/her
tummy is full.
Milk feed thickeners
There are many varieties of milk
thickeners. These are some of the
commoner ones:
Carobel, Nestargel
Made from Carob seed flour. Added
just before a feed to thicken it instantly.
Thixo-D, Thick and Easy
Made from maize starch. Can be added
just before a feed or mixed in advance
when feeds are prepared and stored.
Gaviscon
A powder which can be added to infant
formulas or given after a breast feed.
Feeding your baby with slightly
smaller volumes of milk given at more
frequent intervals may also be helpful.
If your child has bad reflux with
frequent vomiting, oesophagitis, recurrent
stricturing or poor weight gain, then
treatment with antireflux medicines or
antireflux surgery will be necessary.
Antireflux medicines
Antireflux medicines work in different
ways. In general, they either reduce the
severity of the reflux by improving the
downward movement (i.e. motility) of
the oesophagus and stomach, or by
reducing the acidity of the stomach so
that the reflux is less damaging to the
oesophageal lining.
These are the names of some
commonly used drugs:
Motility drugs
Cisapride (Prepulsid ®) and domperidone
(Motilium ®) act by strengthening the
antireflux valve at the bottom of the
oesophagus and speeding up stomach
emptying. There has been a lot of concern
about cisapride in premature babies and
this has caused a 'knock-on' anxiety
concerning the use of this
drug in GOR generally. Most clinicians
are still using the drug since it can be very
helpful; there are, however, various
precautions to be taken, which you must
discuss with your doctor.
Acid lowering drugs
Cimetidine (Tagamet ®) and ranitidine
(Zantac ®) are the drugs most frequently
used to lower the stomach's acidity.
Omeprazole (Losec ®) is a more powerful
acid-lowering drug.
Other medicines
Gaviscon contains antacids and tends to
float on top of the feed, thickening it.
Any medicine can have side effects
but these are not known to be common
with these drugs. Occasionally, a child
may get diarrhoea with cisapride,
develop unusual movements or a rash –
in which case you must stop the medicine
and seek medical advice. Cisapride must
not be given together with certain other
medicines such as erythromycin (an
antibiotic) or fluconazole (sometimes used
to treat thrush) because harmful effects
have been reported in this situation.
Any unusual reaction to a medicine
should always be reported to your doctor
and the drug should be stopped at least
temporarily.
Medicines are usually given to babies as
a syrup from a syringe which allows you to
measure small doses. You should be given
a supply of syringes from the hospital as
small syringes are n o t a lw a ys e a s y
t o ge t f r o m t h e chemist. Most of
these medicines are best given before
feeds. However, Infant Gaviscon is
supplied as sachets which need to be
mixed with the feed or, in breast fed
infants, mixed with water and given after
the feed. Remember to s tore the
medic ines safely out of reach of other
children (some need to be kept in a fridge).
68
Antireflux medication often needs
to be continued for many months and
can then be gradually removed when
the reflux has improved. In some
children, reflux is persistent but mild,
causing occasional heartburn or
discomfort but never severe enough to
need surgery. In these patients, antireflux
medicines need to be used occasionally or
only when they have symptoms.
Sometimes, infants and children do not get
better with the m e d i c i n e s o r t h e y
h a v e m a j o r problems with reflux such
as repeated stricturing, chest infections
from overspill of gastro-oesophageal
reflux
Duodenum Stomach
The fundus of the stomach isw r a p p e d a r o u n d t h e l o w e r
oesophagus, creatin a valve to prevent refl ux
Fundoplication
Nissen Fundoplication Fundus of stomach
Sling around oesophagus
Oesophagus
Opening in diaphragm
for oesophagus
Opening in diaphragm tightened
Gastrostomytube
The TOF Child
Antireflux medication often needs
to be continued for many months and
ed when
children, reflux is persistent but mild,
discomfort but never severe enough to
need surgery. In these patients, antireflux
medicines need to be used occasionally or
Sometimes, infants and children do not get
m e d i c i n e s o r t h e y
problems with reflux such
as repeated stricturing, chest infections
Stomach
the stomach is w r a p p e d a r o u n d t h e l o w e r
into the lungs, persistent severe
oesophagitis or inadequate weight gain.
In these children antireflux surgery has
to be considered.
Antireflux Surgery
The usual antireflux operation is known
as the Nissen fundoplication. There are
others, for example the Thal fundoplication
– the choice of antireflux operation partly
depends on what your surgeon believes
gives the best results –but for TOF
children the Nissen operation is usually the
one selected.
The Nissen fundoplication is a fairly
big operation. Most paediatric surgeons
do the operation through a cut in the upper
part of the tummy but some surgeons are
evaluating whether doing the procedure
through multiple telescopes (`keyhole
surgery') has any advantages.
Procedure
In the Nissen fundoplication, the lower
oesophagus is freed up and the top part
of the stomach (known as the `fundus')
is wrapped around the lower oesophagus
to make a valve at the junction of the
oesophagus and stomach. The hole in
the diaphragm through which the
oesophagus passes is also tightened.
With an experienced surgeon, the
operation can be done safely with few
serious risks.
Use of gastrostomy tube
Sometimes a feeding gastrostomy is
inserted at the same time to provide
supplementary feeding afterwards.
Gastrostomy feeding may be given as a
continuous overnight feed with a pump
or as separate volumes (boluses) during
the day or a combination of both,
depending on the needs of your child (see
the section on gastrostomy feeding in
the chapter on Discharge from Hospital).
Sling around oesophagus
Gastrostomy tube
The TOF Child
oesophagitis or inadequate weight gain.
antireflux surgery has
The usual antireflux operation is known
as the Nissen fundoplication. There are
others, for example the Thal fundoplication
the choice of antireflux operation partly
believes
children the Nissen operation is usually the
The Nissen fundoplication is a fairly
big operation. Most paediatric surgeons
do the operation through a cut in the upper
surgeons are
the procedure
In the Nissen fundoplication, the lower
oesophagus is freed up and the top part
of the stomach (known as the `fundus')
ped around the lower oesophagus
oesophagus and stomach. The hole in
operation can be done safely with few
Sometimes a feeding gastrostomy is
Gastrostomy feeding may be given as a
continuous overnight feed with a pump
or as separate volumes (boluses) during
(see
in
the chapter on Discharge from Hospital).
69
The TOF Child
Post-operative considerations
Creating a valve at the lower end of
the oesophagus prevents reflux of gastric
acid, thereby allowing any oesophagitis to
heal. Vomiting is abolished and
stricturing improves. T h e
o p e r a t i o n i s u s u a l l y v e r y
successful but has some disadvantages.
Firstly, the child may not be able to
burp and can therefore be more
`windy' (called gas bloat); this often
improves after a few months and if
there is a gastrostomy tube in place,
you will be taught how to wind your
child through this.
Secondly, the child may have some
retching instead of vomiting; again this
often disappears after a few weeks or
months. Because part of the stomach is
used to create the antireflux valve, the
stomach volume is a little smaller to begin
with and smaller, frequent meals are
needed. In a crisis, your child may learn
to vomit past the valve but if your
child is poorly and keeps trying to
vomit, it is best to open the gastrostomy
tube to relieve him/her. If your child does
not have a gastrostomy
tube and is unwell with repeated retching,
it may be necessary to bring him/her to
hospital to have a nasogastric tube passed
to relieve the discomfort. It is
especially important to seek medical
attention if your child has bad tummy
pain and retching since this could mean
a blockage in the bowel due to an internal
scar (adhesions) from previous surgery.
Rarely, a fundoplication causes
intermittent diarrhoea or sweaty
episodes (`dumping') after a feed or,
if the valve is too tight, swallowing
difficulties occur.
Repeat surgery
Nissen fundoplication may stop working
properly in some cases after a few
months or years because the valve
weakens and then some of the symptoms
of GOR return. In this case, antireflux
medicines may be effective but if the
reflux is severe, the operation needs to be
repeated. A 're-do' Nissen fundoplication
means undoing what is left of the old
stomach wrap and doing the operation
again.
70
Respiratory Problems in TOFs
by Una M MacFadyen, Consultant Paediatrician, Stirling Royal Infirmary.
The Respiratory System
The respiratory system starts at the nose
and extends through the trachea, bronchi
and bronchioles to tiny air sacs (alveoli)
the lungs, where oxygen passes from the
air to the blood. Every tissue in the body
needs oxygen for it to work properly;
oxygen carried in the blood is pumped
around the body by the heart and
delivered to the tissues.
Because of the vital importance of
respiration, the body has natural defence
systems in the respiratory tract to
protect it from damage and to help it
recover from illness or injury.
The lining cells of the respiratory tract
are uniquely designed to do different
jobs. For example, the hair and cells
lining the nose filter out dust and other
particles in inspired air. Some of these
cells (goblet cells) also produce mucus (a
specialised, sticky fluid) that will mop up
anything that might irritate the airway;
mucus also helps to keep the air warm
and moist,
Ciliated Cells and Goblet Cells
The TOF Child
Respiratory Problems in TOFs
by Una M MacFadyen, Consultant Paediatrician, Stirling Royal Infirmary.
The respiratory system starts at the nose
and extends through the trachea, bronchi
and bronchioles to tiny air sacs (alveoli) in
the lungs, where oxygen passes from the
tissue in the body
to work properly;
blood is pumped
Because of the vital importance of
defence
protect it from damage and to help it to
The lining cells of the respiratory tract
different
jobs. For example, the hair and cells
and other
particles in inspired air. Some of these
cells (goblet cells) also produce mucus (a
specialised, sticky fluid) that will mop up
y;
mucus also helps to keep the air warm
Ciliated Cells and Goblet Cells
protecting the lower airway from getting
too dry. Other cells (`ciliated cells') have
tiny arm-like projections (`cilia') which
wave continuously in a to-and-fro
motion. Collectively, these cilia act
like a microscopic conveyor belt
which carries particles trapped in the
mucus to the pharynx where both the
mucus and its trapped impurities can
either be swallowed or coughed out.
Action of Cilia
Trachea The Lower Respiratory Tract
Bronchiole
Right lung
Bronchi
The TOF Child
protecting the lower airway from getting
too dry. Other cells (`ciliated cells') have
like projections (`cilia') which
motion. Collectively, these cilia act
the
the
can
Respiratory Tract
71
The TOF Child
Further down the airway, cel ls
lining the trachea do a similar job in
clearing even smaller impurities in the
air. Here the cilia have to carry the mucous
coating upwards (and therefore against
gravity) to the pharynx; this is known as
the `mucociliary escalator.'
During infections (e.g. coughs and
colds) mucus production is increased
to speed the removal of bacteria. The
airway also becomes more sensitive
meaning that accumulations of mucus
or irritant substances are quickly
coughed out. Reflex coughing also
occurs if something goes down the
wrong way on swallowing.
What is different in the TOF?
In the commonest type of TOF/OA
there is a blind ending upper oesophagus
(the atresia) and a connection between
the lower end of the oesophagus and the
trachea (the fistula). Other forms of TOF
have different connections but the
effect on the tissues of the oesophagus
and trachea are the same:
i) the muscle of the oesophagus is
interrupted at the atresia and even after
the repair there will be an area of the
oesophagus that does not transmit
swallowing action (peristalsis) as
smoothly as normal.
ii) the supporting tissue of the trachea
called cartilage (the gristle) is not fully
formed at the site of the fistula. The
cartilage forms arches around the front
of the trachea to give it support and to
hold it open all the time. Without this
cartilage the trachea is softer than it should
be. If there is a long section without
cartilage this can cause floppiness of the
trachea called tracheomalacia.
iii) the air filter cells (goblet cells and
ciliated cells) are replaced at the site of
the fistula by less specialised cells
(squamous cells) that are less efficient at
keeping the airway protected. After a
TOF repair a deficiency is therefore present
in the mucous coating and the conveyor
belt system.
In the baby born with TOF/OA
there is an abnormality of the structure
of both the respiratory tract and the
oesophagus. The trachea sustains a major
surgical injury in the first few days, yet in
almost all cases recovers well so that
there are no long term serious problems by
the time the TOF child has grown up.
Respiratory consequences of the differences in the oesophagus
The problems with swallowing in a
TOF child can have secondary effects on
the respiratory tract. In the immediate new-
born period before the oesophagus is
repaired, saliva or any milk that they try
to swallow will spill over into the
trachea causing coughing and choking.
After the TOF repair this "spill over" can
still happen if the repair site in the
oesophagus narrows (oesophageal stenosis)
so that swallowed liquids get held up in the
oesophagus. Some babies with TOF
develop problems with gastro-
72
The TOF Child
oesophageal reflux, when the lower end
of the oesophagus has a tendency to fill
up with stomach contents after a meal.
This sometimes allows
aspiration (the passage of material into
the respiratory tract) to happen quite a
long time after drinking or eating. A few
such babies tend to wheeze when there is
reflux into the oesophagus without
aspirating, so different kinds of tests
and treatments may be needed to find out
what is actually happening before treatment
can be given.
Respiratory consequences of the differences in the trachea
Most people hardly notice that they
have any mucus to clear and just
automatically clear their throat while
the cells lining the trachea do the rest of
the job. In a TOF child, the gap in the
normal protective lining cells (at the site
of the fistula) can be enough to make the
clearance of mucus less efficient and/or
the mucus drier than normal. The child
may therefore have to cough hard to
clear this mucus.
The `TOF cough' results from the need
to clear this dry mucus by coughing
through a trachea with a slightly floppy
section that makes the flow of air less
smooth during the cough. Anything that
increases mucus production will make
the TOF cough worse, e.g. colds, liquids or
food getting into the airways (aspiration) or
asthma.
When are problems at their worst?
The problems with the respiratory tract
following TOF repair tend to be at
their worst in the first two years.
This may be because the trachea of normal
infants contains immature cartilage which
is quite soft. The area of absent cartilage
from the TOF trachea has a much greater
effect when surrounded by a softer
cartilage framework; when there has been
more
growth of the normal section of the
trachea and the tracheal cartilage is
firmer, the deficit is easier to cope with.
In addition, many normal babies tend
to reflux during the first year, so any
problem related to reflux will therefore
show at its worst. The repair site
occupies a large part of the young baby's
oesophagus so any section that is
narrow or has poor activity has a major
effect on function.
Tracheomalacia
Most babies who have had a TOF
repair do not have major difficulties
coping with tracheomalacia and it
becomes less and less significant as they
get older.
There are however a small number who
have serious problems because the
trachea actually collapses so that no
air can pass through it. In its most severe
form this complication causes problems
which have been referred to as 'near
death episodes,'when the baby seems to
be choking and unable to breathe.
Because these are rare, many doctors
have never witnessed one of these
episodes. It helps enormously if parents
are able to describe what happens in as
much detail as possible, since the child
is often perfectly well by the time they
get to hospital or are seen by a doctor.
The factors causing the airway collapse
can sometimes help to explain the time
of the attacks and the way in which the
child is affected:
Airway collapse when breathing in
When we breathe in, we suck air into
our lungs through the airways. The
lungs are filled with air like a bellows as
the chest wall moves out and the
diaphragm muscles move down. The
walls of the airways outside the chest
tend to be pulled in during this
73
The TOF Child
Effects of Tracheomalacia
74
The TOF Child
action. Normal people feel this when
they try to take extra large breaths
through the nose and feel a 'drag' on
the windpipe.
If the trachea is floppy this 'drag' draws
the walls of the trachea together; without
the normal supporting cartilage the
trachea may close off completely. This is
like suffocation and is very frightening
for the baby (and anyone who witnesses
it). The baby goes blue and may lose
consciousness or have a brief convulsion.
This is obviously very dramatic,
nevertheless the baby relaxes as he/she
becomes unconscious which allows the
trachea to open up again; breathing is
then at once effective.
If the baby is still conscious with their
mouth open, gently pulling the tongue
forward may help as a first aid measure to
open the airway.
If however the baby is unconscious
and not breathing, blowing gently into
the airway by mouth to nose-and-mouth
respiration is the correct course of action.
Airway collapse when breathing out
If the trachea is floppy in the lower
part (within the chest) then its walls
will be pulled open during breathing in
(inspiration). Breathing out (expiration)
is usually simply a relaxation from the
effort of breathing in and causes no
difficulty. The only times when a
physical effort is made to breathe out
are when crying or coughing hard. Babies
with severe intra-thoracic tracheomalacia
may experience
tracheal collapse during these moments.
As above, they can go blue and lose
consciousness, at which point they relax
and the airway opens up again. If a
quick recovery does not occur, the airway
can be gently blown open with mouth to
nose-and-mouth breathing.
Diagnosis
The infant with severe tracheomalacia
usually becomes symptomatic around the
age of 4-6 months when there may be an
exaggeration of the TOF cough, excessive
wheezing or cyanosis (blue attacks)
during feeding. In extreme cases, the
infant may experience acute life-
threatening episodes.
For a doctor, the story of a baby
going blue with hard crying may
suggest common breath holding
attacks. However, these tend to occur
in older toddlers who are either angry
or very upset, and happen at the end of
a big breath in or out; the 'near death
episodes' usually happen in the midst
of a normal crying spell. Observers who
can observe and describe these kind of
details about the episodes can help greatly
in making a correct diagnosis.
In addition to problems due to the
pressure changes of breathing efforts, a
soft trachea can also be squashed from
the outside. This may occur when a
narrowed oesophagus causes a hold up,
so that the upper oesophagus fills up
and stretches so much that it pushes
against the neighbouring trachea. Without
a strong enough cartilage framework to
keep it open, the trachea can become
closed off until the oesophagus empties –
either by a vomit or by the contents
passing through the narrowed region. The
same situation can arise if the lower
oesophagus fills up with refluxed stomach
content. The latter episodes can be hard
to explain as they may not seem to
be l inked to any reason for a
breathing problem, but again the more
detail observed the better.
75
Tracheal Compression
by Oesophagus
Oesophagus
Left lung
Diaphragm
Stomach
Swallow fills out oesophagus
above stenosis
Wall of trachea
squashed
Distension above
stenosis
Distension following reflux
Wall of trachea
squashed
Reflux distending oesophagus
76
The TOF Child
Tracheal Compression Tests for tracheomalacia
There are a number of different tests
that can be done to help with diagnosis
of tracheomalacia, but since there is often
more than one problem present it can
be difficult to work out which is the
most important for the baby, and then to
decide on the best treatment.
Most babies have some floppiness in
the trachea after a TOF repair, but the
majority are not troubled by it and any
problems will improve with age. It is
therefore not enough simply to look for
floppiness, especially when a problem in
the oesophagus may actually be the origin
of the symptoms.
Tracheomalacia also needs to be
distinguished from gastro-oesophageal
reflux, which can present in a similar
fashion.
Radiography
The diagnosis of tracheomalacia can
be suspected on a lateral (side-on)
radiograph of the neck during inspiration
and expiration, which will show a
collapsing trachea.
A barium meal study is often useful to
check for any oesophageal malfunction.
Bronchoscopy
Bronchoscopy (looking into the airway
via an endoscope) is the most reliable
method of reaching a diagnosis. The area
of collapse is classically 2-3 cm above
the bifurcation (splitting) of the trachea,
where the tracheooesophageal fistula
entered the trachea.
Respiratory function tests
Tests of the baby's breathing can
measure how much work the baby has
to do in breathing in and out, but cannot
give information about the length of the
trachea which is abnormal.
When the extent of the problem has
been ascertained, treatment options can
be discussed and any actions planned.
fills out oesophagus
Wall of trachea
squashed
The TOF Child
The TOF Child
Treatment of tracheomalacia
Surgery for tracheomalacia aims to
give the trachea extra support.
Tracheopexy
This operation involves placing
s t i t ches to a t t ach the wal l of t he
trachea to the back of the breast bone
(the sternum). These sutures anchor
the trachea and make it more difficult
for the walls of the lower (intrathoracic)
trachea to collapse together. The procedure
can help if the tracheomalacia is mostly in
the lower trachea and the baby has
problems during expiration.
Aortopexy
The aorta is a major artery which
l i e s i n f r o n t o f t h e t r a c h e a . I n
aortopexy, the aorta is displaced forwards
by suturing it to the back of t h e
s t e r n u m ( b r e a s t b o n e ) ; t h i s
maintains the trachea in the open position.
The procedure acts at the precise site of the
collapse in the lower trachea and is
instantaneously effective in relieving the
obstructive symptoms.
Which of these operations is used
depends on the site of floppiness and what
the surgeon thinks will work best for
the individual baby.
Tracheostomy
Both of the above operations work
best for intrathoracic tracheomalacia
that is short enough to be supported by
a few stitches. If the floppy part of the
airway stretches down into the smaller
a i r wa ys (b r on ch i ) i t ma y n o t be
possible to give the wall of the trachea
support f rom the outside. In such
cases, a tracheostomy tube may be
inserted to ensure that the baby has a
safe airway.
This involves making an opening in
the front wall of the trachea in the
neck, and placing a little tube through
the opening to hold the trachea open
all the time. In severe cases of
bronchomalacia (where the floppiness
stretches beyond the trachea into the
bronchi) it may be necessary to apply
CPAP (Continuous Positive Airway
Pressure — a constant flow of air or
oxygen at a pressure that gives extra
support to the airway from the inside)
or the modification known as BIPAP.
Very few TOF children have this
type of problem and most who do
have a good chance of improving
greatly with treatment and age.
Infections
There is no evidence that TOF children
have more respiratory infections than
other children, but they may have more
trouble coughing enough to clear the
airways of the extra mucus during
normal infections.
Most childhood chest infections are
caused by viruses. The body fights these
by producing specific antibodies which
endow the child with resistance to
future attacks by the same virus.
This defence system is called the immune
system; TOF children have a normal
immune system and make antibodies in
the normal way. Viruses are not killed
by antibiotics; some viruses irritate the
airways and cause them to become
swollen and tight.
Problems arise with TOFs"normal'
chest infections for two reasons:
i) the impaired clearing of airway
secretions can allow mucus to settle in
the lungs. Bacteria (germs) can gather
in these local accumulations of mucus
and cause a more serious infection to
develop. This is often called a 'super-
infection' because it is on top of the first
infection — not because there is anything
very special about the germ involved.
Bacteria are killed by antibiotics, so if
there are signs that a virus infection has
not cleared naturally then antibiotics are
recommended. Often antibiotics are given
right from the start
77
of respiratory infections in young TOFs
because they are likely to have difficulty
coughing up phlegm. As the child gets
older he/she may not need so many
antibiotics. Taking antibiotics does not
stop the body's own defences from
fighting the virus and the child will build
up immunity in the normal way. Most
bacterial infections respond to the
common antibiotics, but occasionally extra
courses or wider acting (`broad-spectrum')
antibiotics are required. This especially
applies if an area of the lung has
abnormally small airways, as with
bronchomalacia or bronchiectasis (where
the walls of small airways are weakened
and stretched so that infection
Constriction of the Airway in Respiratory Disorders
Normal mucus
Normal small airway bronchiole)
Lining Muscle in wall
Tight (constricted) e.g. mild asthma
Muscle tightens
temporarily
Swollen with mucus (infection)
Swelling
Tight and swollen with mucus
(severe asthma or asthma plus virus infection)
78
The TOF Child
biotics, but occasionally extra
spectrum')
collects in tiny sacs deep in the lungs).
In such cases chest physiotherapy helps
to clear secretions from the lungs and
antibiotics may be prescribed for an
extended period (weeks or months)
until the lungs are completely clear of
signs of persisting infected phlegm.
ii) the airways of TOF children may be
more sensitive to viruses causing swelling
and tightening, making it hard to move
air in and out. The effect is the same as is
seen in asthma and responds to the same
type of treatment.
Prevention of sensitive or tight airways (`wheeze')
When the airways narrow from swelling
or tightening, the child feels that he/she
cannot breathe properly. This is very
frightening and can be dangerous if so
little air moves that the amount of
oxygen reaching the blood is reduced.
There are several ways of preventing
and/or treating this situation.
i) protect the lungs as far as
possible by having the child immunised
against whooping cough, Hib
(Haemophilus influenzae), measles and,
if advised by your doctor, against
influenza and pneumococcus.
ii) avoid situations that cause the problem.
This may be easier said than done, but for
example if you know the child is
sensitive to animals then it is wise to
avoid getting a furry pet.
iii) one of the commonest causes of
wheezing in young children (and also
of more troublesome respiratory
infections and ear infections) is being
in an atmosphere where people smoke
(passive smoking). Avoiding smoking
in the home where chesty children live
is therefore important.
extended period (weeks or months)
swelling
same as is
to the same
swelling
influenzae), measles and,
Treatment of sensitive or tight airways ('wheeze')
i) keep calm and know what to do to help
the child. The muscles which tighten
around the airways react to fear and
anxiety; children are very sensitive
fear in adults, so it helps if adults know
how to deal with breathlessness and can
give the child confidence. Learn the basic
treatment steps, what to give when and
how to get help when needed, and you will
be helping your child.
ii) medications: 'drug' is the technical
name for medication i.e. the word does
not mean the same as the addictive drugs
you read about in the papers. These fall
into two main categories:
Relievers (bronchodilators)
These work by making the tight
muscles around the airway relax and
most effective when inhaled directly into
the lungs. They act quickly which
`relieves' both the breathlessness and
the fear that accompanies it. The effect
wears off in four hours or less, so treat
ment may need repeating frequently.
There are various inhaler methods
which allow different ages of child to
breathe in the medication:
puffers: metered dose inhalers.
spacers: demand less co-ordination to
use but are bulky.
dry powder inhalers: where the child
sucks the dose into the lungs.
nebulisers: convert the drug into a fine
mist which is administered through a
mouthpiece or mask.
Several pharmaceutical companies
manufacture relievers, so there are a
number of different names for the
same kind of drug (Ventolin, Bricanyl,
Aerolin). Most in the UK come in a blue
container, so this is the one to use when
sudden tightness occurs.
The technique for use of relievers
The TOF Child
keep calm and know what to do to help
tighten
around the airways react to fear and
anxiety; children are very sensitive to
know
and can
child confidence. Learn the basic
treatment steps, what to give when and
how to get help when needed, and you will
medications: 'drug' is the technical
name for medication i.e. the word does
not mean the same as the addictive drugs
ou read about in the papers. These fall
muscles around the airway relax and are
most effective when inhaled directly into
the breathlessness and
the fear that accompanies it. The effect
wears off in four hours or less, so treat-
There are various inhaler methods
which allow different ages of child to
ordination to
where the child
convert the drug into a fine
mist which is administered through a
armaceutical companies
manufacture relievers, so there are a
number of different names for the
same kind of drug (Ventolin, Bricanyl,
Aerolin). Most in the UK come in a blue
container, so this is the one to use when
or use of relievers
should be explained by a doctor or nurse
who can make sure that the treatment
plan makes sense both to the child and
parents, and that when and how to get
emergency help is fully understood.
Other less common relievers which
act in slightly different ways may be
recommended for children with more
troublesome asthma symptoms. These
have a longer lasting action; examples
include salmeterol (Serevent) and
theophylline (Uniphyllin).
The wheeze of some very young
babies is more due to swelling than
tightening of the airway muscles and
so they may not respond to the usual
bronchodilators. Such babies may be
treated with ipratropium (Atrovent).
Preventers (prophylaxis)
Because the airways are sensitive even
when they are not tight, it is often
preferable to use a regular treatment which
helps them to withstand whatever irritates
them. This both reduces the number of
breathless attacks and makes reliever
treatment more effective.
Preventers are administered in the
same ways as the relievers. There are
two main types – sodium
cromoglycate (Intal, in a red and white
pack) and inhaled steroids (usually in a
brown or orange pack, e.g. Pulmicort,
Becotide, Aerobec). Although the steroid
preventers are related to the
Inhaler
...follow the instructionsprovided by your doctor
or as given by the literature with the inhaler
The TOF Child
should be explained by a doctor or nurse
who can make sure that the treatment
plan makes sense both to the child and
parents, and that when and how to get
Other less common relievers which
recommended for children with more
troublesome asthma symptoms. These
have a longer lasting action; examples
tightening of the airway muscles and
Because the airways are sensitive even
preferable to use a regular treatment which
helps them to withstand whatever irritates
them. This both reduces the number of
breathless attacks and makes reliever
Preventers are administered in the
There are
cromoglycate (Intal, in a red and white
pack) and inhaled steroids (usually in a
brown or orange pack, e.g. Pulmicort,
Becotide, Aerobec). Although the steroid
instructions provided by your doctor
literature with the inhaler
79
strong steroids that are taken by mouth
for other conditions, the dose is so small
when breathed into the lungs that they
are much safer than oral steroids. They are
not at all like the body building steroids
that some athletes take against medical
advice.
If the airways become very swollen,
the response to relievers may not be
adequate. The child may then need a short
course of stronger steroids (e.g.
prednisolone), taken by mouth, to reverse
this potentially dangerous problem. Your
doctor will advise when this is
required. When used in this short
term manner, the risks of steroids are far
less than the risks of being unable to
breathe.
Outlook in the Longer Term
TOFS children, on average, have more
respiratory symptoms than other children.
This is most marked in the first nine
years, however in most cases they can
be just as fit and active as their friends of
the same age, albeit perhaps needing
some medication to help. As they grow
older any symptoms often improve
greatly, although specialised breathing
tests may still show differences from
normal.
Perhaps with the exception of
activities depending on above average
lung function or those where exposure
to airway irritants is common, there should
be no restrictions on what a TOF can do
for work or for leisure.
80
The TOF Child
strong steroids that are taken by mouth
are much safer than oral steroids. They are
If the airways become very swollen,
adequate. The child may then need a short
problem. Your
are far
TOFS children, on average, have more
children.
to airway irritants is common, there should
Quotations from TOF s, their families and friends and medical professionals
"However much you convince yourself
that it doesn't bother you, it is actually
very difficult to cope with the stares
and comments you receive when your child
barks away in John Lewis."
"Henry has his amazing TOF cough which
always causes much attention. It causes
even more of a stir when he does it in
conjunction with picking up a discarded
cigarette butt in the park!"
Below:
Craig Palmer, 10 years.
and comments you receive when your child
The TOF Child
"It wasn't until I had sat in a GP's waiting
room with Eleanor who gave a "TOF
cough" did I realise the true intolerance
of other people! The bark is there,
particularly on those days when she has a
cold and chestiness. People will always
stare or comment, and there was a time
waiting to be seen in a Casualty
Department that some people actually
moved away from us! Even today, it is the
most common statement made to her –
"Oh, you have got a bad cough"– even
among people who are aware of her
condition!"
"Strangers told us we shouldn't take out
a baby with such a bad cough –this
became very irritating and gave insight
into the impact of well-meaning but
ignorant people who assume all kinds of
things."
"Sometimes my brother will wake up
during the night coughing and
spluttering. When I was young this used
to scare me because I thought he was
choking but now I understand the
problem it's OK."
"The rest of the family have dealt with
the TOF condition very well – maybe they
fuss a little too much over asthma, colds
and the TOF cough which we get used to
and learn to ignore the stares and
comments of complete strangers."
81
Growth in TOF ChildrenPAT Chetcuti DM FRCP FRCPCH, Consultant Paediatrician,
The General Infirmary at Leeds
Introduction
Parents of TOF children are sometimes
concerned about their child's growth.
For these children, milk — the source of
energy promoting growth — is unable to
pass through to the stomach and small
b o w e l a t b i r t h . E v e n f o l l o w i n g
corrective surgery, complications may
be present which threaten the supply of
nutrition, mostly in the first two to
three years of life.
This chapter considers noll _ ial
growth, factors which influence growth
and growth as it specifically relates to
oesophageal atresia.
Normal Growth
The most important influence on a
child's height and their future height as
an adult is parental height. Generally,
tall parents have tall children and small
parents have small children. However,
a tall and a short parent may have a child
who is tall, average or short, and
occasionally the height of grandparents
has a bigger influence on a child's stature.
Despite these obvious observations, many
genetically small children are referred to
the medical services for assessment.
Right:
Representation of a growth chart, showing
the different centile lines. Different charts
are used for boys and girls; usually a
series of measurements are taken of
height to determine the 'growth velocity.'
(Illustration based on the Buckler Tanner
growth chart, © Castlemead Publications)
82
The TOF Child
Growth in TOF Children DM FRCP FRCPCH, Consultant Paediatrician,
b o w e l a t b i r t h . E v e n f o l l o w i n g
corrective surgery, complications may
nutrition, mostly in the first two to
nce growth
child's height and their future height as
parents have small children. However,
occasionally the height of grandparents
has a bigger influence on a child's stature.
Despite these obvious observations, many
Representation of a growth chart, showing
the different centile lines. Different charts
are used for boys and girls; usually a
series of measurements are taken of
growth chart, © Castlemead Publications)
Growth Charts
Growth charts are available for
children, based on measurements in
large numbers of normal children of
different ages. Charts exist for males
and females and consist of different
centile lines. The 50th centile line is
the average line. The majority of
children have heights between the 97th
centile and the 3rd centile.
In assessing growth in children, a single
measurement is of limited value. Several
measurements over a period of time —
preferably at least a year — will
determine the rate of growth, known as
the height velocity. The height velocity
might show that (for example) a short
child is growing normally or a tall child
has stopped growing.
0 2 4 6 8 1 0 1 2 1 4 1 6 1 8
height (cm)
97th centile
50th centile
3rd centile
—age (years)
180
160
140
120
100
80
60
The TOF Child
Crucial Periods in Growth
Growth is at its fastest in the first
20 weeks of foetal life. Any major health
events in the early weeks of the
pregnancy can have a significant effect
on the process of growth. Growth in the
first 2 years of life is also rapid, and –
like foetal growth – is predominantly
dependant on adequate nutrition.
Whilst growth remains important
throughout childhood, the final important
phase of rapid growth occurs in
adolescence; prior to this growth spurt, the
growth rate tends to slow down. Pre-
pubertal growth occurs mainly in the
limbs whereas pubertal growth occurs
mainly in the trunk.
Growth hormone is important in
influencing growth in older children,
and the sex hormones have a role in
the pubertal growth spurt.
Factors Affecting Growth in Children
Nutrition Lack of nutrition, to the foetus or in the
first year of life, for whatever reason, may
result in stunting of growth –possibly with
lifelong consequences.
Chronic Illness
Rare chronic disorders affecting the
bowels, kidneys, heart and lungs can affect
growth. This is the result of a
combina t ion of decreased ca lor ie
intake and the increased energy
requirements that children have in coping
with these illnesses.
S y n d r o m e s a n d
Chromosomal Abnormalities There are a few rare conditions which
may be inherited or occur by chance in
which children are born with a number
of different abnormalities and despite
good nutrition do not grow normally.
Growth Hormone Deficiency
This is rare, occurring in 1 in 4,000
children, and can occur at any age.
Children with this problem either fail to
grow or grow poorly despite a d e q u a t e
n u t r i t i o n . C a r e f u l investigation
reveals that their growth hormone levels
are low. It is very important to make this
diagnosis because these children respond
well to growth hormone replacement.
Children who grow poorly for the
other reasons stated above, including
inadequate nutrition, have normal
growth hormone levels and therefore
do not respond to extra doses of growth
hormone.
Growth of TOF Children When assessing growth in a TOF
child, one must consider two factors;
size at birth and subsequent growth.
Some infants with oesophageal atresia
are small at birth. The reasons for this
are unclear because in foetal life the
nutritional supply comes from the
placenta. It may be that those with
poor growth at birth have other major
problems such as heart problems which
have affected their growth.
In a review of over 300 children and
adults born with oesophageal atresia
the majority had grown normally. The
graphs on the next page illustrate the
distribution of height measurements on
the centiles. The findings are very
encouraging. While there are slightly more
in the lower growth centiles, p r o v i d i n g
e v i d e n c e t h a t g r o w t h problems
and poor weight gain are more
prevalent in children under 5 years
of age, the vast majority have a final adult
height which is normal.
In practical terms, parents may be
relentlessly pursued about their child's
undernourished appearance by well-
83
The TOF Child
meaning relatives, friends and health
professionals inexperienced in managing
children born with oesophageal atresia.
This can cause unnecessary distress and
anxiety which may rebound on the
children themselves, affecting morale.
In the longer term however the figures
suggest that the majority experience `catch
up' weight gain to achieve a normal final
adult height.
Inadequate Nutrition
If there are significant problems
with feeding, such as solids impacting
the gullet (oesophageal obstructions) or
vomiting, then the nutritional intake may
be reduced. This is most likely to be a
problem in the first years of life. With very
careful management
however – stretching of narrowed areas,
and medical and sometimes surgical
treatment of gastro-oesophageal reflux
– nutritional depletion can be minimised.
Some TOF children may be given oral
concentrated nutritional diets or
supplements to boost calorie intake; very
occasionally a gastrostomy (a tube
inserted directly into the stomach) is
needed to maintain nutrition.
Respiratory Problems
For the first few years of life after
surgical repair, it is common for TOF
children to have a persistent cough and/or
recurrent minor chest infections. Rarely,
the chest infections may be more severe
and infants may be chronically breathless.
In this rare situation, the extra energy
used to breathe out can result in poor
weight gain because the dietary intake is
not adequate to allow for this.
Other Associated Problems
Half of all children born with
oesophageal atresia have other significant
abnormalities. The majority of these
abnormalities are not major. However
some children may have
Above:
Percentage distribution of all TOF patients
for height centiles: there are slightly more
patients in the lower growth centiles.
Above:
Percentage distribution of adult TOFs for
height centiles: the distribution is normal,
indicating that any growth problems in
early life have been overcome.
abnormalities that might affect growth,
particularly children with heart problems,
chromosomal abnormalities or
syndromes.
Growth holinone deficiency is not
associated with oesophageal atresia,
therefore growth hormone treatment is
not beneficial in short children or children
with poor growth velocities.
84
Who to talk to if you are
worried about your child's
growth
Parents with concerns about their
children's growth should make contact
with the surgeon and centre where the
initial surgery took place to discuss
their worries. For parents moving
around the UK, all Medical Schools
Teaching Hospital Centres in the
country will have a Paediatric Surgical
Department with experience in
oesophageal atresia and its
Quotations from TOF s, their families and friends and medical professionals
"I wonder whether the TOF condition
has affected Thomas' growth, particularly
in the early years when his diet was
limited, or should I say hampered, by
the condition. Did it actually put him off
eating quantity of food?"
"I feel different when all my friends
are doing things I can't do and when
people call me small!'
"We would like Chris to gain a little more
weight and be a little taller. It only
worries us when he gets upset because he
says he is the smallest in his class."
"Craig is eleven now. He has never had
problems with eating, he eats everything
– he has never needed a dilation. The
only reminder is a `TOF bark' when he
has a cough. He only has the occasional
chest infection, no more than any
'normal' child. He has lots of stamina,
he plays football, golf and swims very
well. His weight is average for his age,
he's a super boy!"
The TOF Child
Parents with concerns about their
children's growth should make contact
with the surgeon and centre where the
initial surgery took place to discuss
around the UK, all Medical Schools and
country will have a Paediatric Surgical
families and friends and medical
"I wonder whether the TOF condition
ularly
limited, or should I say hampered, by
him off
"I feel different when all my friends
are doing things I can't do and when
more
only
worries us when he gets upset because he
"Craig is eleven now. He has never had
problems with eating, he eats everything
he has never needed a dilation. The
bark' when he
has the occasional
s of stamina,
and swims very
well. His weight is average for his age,
complications. Advice and referral to
these can be obtained directly from a
General Practitioner.
Another important source of help is
the Liaison Surgical Nurse Specialist.
These are very experienced Senior
Nurses attached to most Paediatric
Surgical Units who often visit and get
involved with families at home.
Remember that even though
problems may seem very difficult at
the time, the longer tern' outcome in
teiins of growth is very good.
Above:
James Wharfe, 14 years, who had a
gastro s tomy tube un t i l t he age o f 27
months. After various set-hacks James now
enjoys an active life and is reported to eat
`like a horse' although remaining slim.
The TOF Child
complications. Advice and referral to
these can be obtained directly from a
Another important source of help is
aison Surgical Nurse Specialist.
Surgical Units who often visit and get
problems may seem very difficult at
tern' outcome in
gastro s tomy tube un t i l t he age o f 27
hacks James now
enjoys an active life and is reported to eat
85
The TOF Child
The TOF Family
Dr Glenys Parkinson, 0 T PhD (Developmental Psychology) is a Chartered
Clinical Psychologist, working at the Brookside Family Consultation Clinic in
Cambridge, where she often uses play therapy to help children with emotional
psychological problems. She also has 15 years' experience in the paediatrics
department of Addenbrookes Hospital, Cambridge, where she works closely with
the play specialists who prepare children for medical procedures.
This chapter looks at the way the TOF
condition affects the family.
Letters and questionnaires from many
families have documented the experiences
of TOF children, siblings, parents,
grandparents and friends. Extracts from
these communications have been grouped
to illustrate the key points, with an
accompanying narrative.
Initial Anxieties
It is devastating for parents to realise
that their soon to be born or born child
has a serious problem. When the news
comes that surgery is required, their joy is
turned into anxieties... how can such a
small baby be operated on?
"Waiting for Freddie to come out of
surgery was dreadful. We were both
exhausted but unable to rest; we had only
just got to grips with the basic condition
as described by the surgeon and until
the operation was over we would not know
the extent of the problems. We had
barely held our baby and now his life
was in someone else's hands."
"I wondered how a baby with such
enormous physical problems could
survive. Feeding is such a fundamental
requirement that it necessarily affects
relationships in ways which are
impossible to know."
The message that comes through from
those who contributed to this book is that
parents do cope — not without cost — but
they manage to give their child as
normal a life as possible. It isn't easy.
"I think my sister wishes the family would
sometimes realise the extra work load she
has and the worry she often feels.
Because Matty looks and acts like any
normal child, people don't realise the
extra work and stress a TOF child can
give."
Life with a TOF child is seldom easy
with demands being made upon all
three generations of the family. Daily life
and routines are never the same again.
But as time goes on, consideration turns
to the wider view; what about baby-
sitters, nursery school, children's parties?
Getting Help and Support
A child in a wheelchair gives other
people a notion of what to do; a child
with no obvious sign of difficulty may
be overlooked by others, not taken
seriously enough, or taken so seriously no-
one is willing to take responsibility.
"Over the years-we have come up against
problems getting people to understand
TOF. Parents of school friends don't
understand and think you are a bit
neurotic and overprotective. The school
have been quite good but
86
The TOF Child
there is still a lack of knowledge. That
has always been an obstacle – trying
to get the message through. They often
take it quite lightly and think you are
fussing! This makes one quite angry
and a little anxious should your son
get into difficulties when having tea
somewhere. Then, if you over explain they
get frightened of feeding him!"
The focus on the child is a two fold
problem: it is very difficult to avoid
being overprotective, and such a focus
tends to make siblings feel this child is
special and is 'worth' more than they are.
"As a baby my family were frightened
of handling Emma and wouldn't baby-
sit. However, they call her their
`miracle' and have a closer relationship
with her than they might have done were
she not a TOF. Grandparents still panic
when she goes into hospital and one set
cannot face visiting."
There is an understandable terror of
dealing with a child who may choke.
Coping with this means acquiring the
skil ls and feel ing protected while
doing so, in order that the anxiety may
diminish. Parents normally get the
opportunity to learn the necessary
skil ls during the period when their
child is in hospital. Since every child is
different these skills are fine-tuned
over a period of time and it is easy to forget
that others have not had the benefit of this
supportive environment when the child has
been discharged.
However, it is important for all sorts
of reasons that others are able to feed
and handle the TOF child. It helps to
take the focus off the affected child if
an aunt, uncle or grandparent can feed
all the children, not just the `nolinal'
ones. It also means that if parents fall
ill or are otherwise engaged, there is
someone who can be called in to help.
Once the parents have gained
confidence in dealing with the baby, it
might be helpful to have a grandparent
or baby-sitter watch for some time; then
deal with the baby themselves with
mother or father standing by. Then with
the parent in the kitchen, in the garden,
outside the gate ...
"Grandparents spoil him and treat him
as though he were made of china but as
time goes on they are getting better"
Eventually the carer may have the
confidence to deal with the child
themselves, having done so in protected
circumstances. It is very important that
both the carer and the parents feel
confident that the baby is in safe hands.
Communication and time can reap
enormous rewards in this respect.
"The family dealt very well with his
condition. We always asked lots of
questions so everyone has a good
understanding and was involved in
bringing about Louis being well."
Spreading the Load
The pendulum can swing the other way;
the parents feel such guil t a t
having produced a baby with a TOF
that they want to 'make up for i t . '
Parents' job is to protect their young; when
they feel they haven't done so of course
they want to compensate for it. However,
in reality parents also need time to be on
their own and should not hope to devote
every moment to the child, nor to take
responsibility for every component of their
care. To attempt to do these things does
not help anyone. It would be useful if
consultants could tell parents that it is NOT
their fault – nobody could have prevented
it.
The best way to bring a TOF child up
is to treat him or her the same as their sibs
whenever possible. This will
87
The TOF Child
not be feasible all the time because of
the risks of choking, the anxiety over
having someone else look after the child,
the problems of diet, weight, growth etc.
Nonetheless, there are ways of sharing
these burdens.
If possible the parent should find a
doctor, dietician and health visitor they
have confidence in. The dietician can
advise on appropriate food for growth, the
doctor or health visitor on development.
Parents do not have to carry the whole of
the burden themselves; they carry the most
onerous part – the anxiety – already. Let
someone else, with the necessary
knowledge, do the monitoring.
When there is the need to seek
immediate medical attention, it may be
arranged with the consultant beforehand to
go straight to the ward where the child has
been treated (if this is local) to be seen by
the consultant or senior registrar; the nurses
should also know what to do. If these kind
of arrangements are not made for you, it is
well worth asking; a note from the
consultant confirming that this
permission has been granted may also
be beneficial. If the specialists who treated
your child in the early weeks and months
are not local, alternative arrangements must
be made; this book may also be of
assistance.
What to Tell the Child?
When is the best time to tell the TOF
child about their difficulties? It has been
found to be useful to do so as soon as
possible. From a very early stage the child
should be told in words he or she can
understand what the difficulties are.
Most children are aware of their
condition from an early age and their
first attempts as language may lead them to
describe the feeling if something is stuck in
their oesophagus. Teinis such as "Got a
stuck," or "Got stuck" followed by "Got
stuck gone" are examples.
Explanations from the parent or carer
can be very simple at first, for example
"When you eat something, you have a
tube leading from your mouth to your
tummy. The food you eat makes you
grow. Your tube is not as wide as
other children's, so you need to drink to
make the food soft."
A demonstration can be made with
some plastic tubing and wet sand, which
has difficulty passing down the tube.
Water allows it to pass freely.
Young children need to see something
'concrete' rather than just a picture or
photograph – which will only mean
something to them when they are rather
older.
"There have been some lighter
moments over the years however.
It was very comical hearing my two
year old son coming out with words
straight from a medical dictionary. He
often asked our friends and visitors if
they had an oesophagus."
When young the child can feel very
alone. The presence of the parents'
visit can make all the difference and
provides the basis for a secure future.
"When I was younger I used to get ill
in the night and cough. My mother,
who is a light sleeper, used to hear me
and come and see me. It used to make
me feel very close to my mum.
Now I don't cough so much but I still remember the feeling of closeness."
As the child's understanding grows,
the information can be elaborated on.
Always explain things truthfully, however
in simple terms. That way information can
he expanded and parents don't have to
say: "It wasn't quite how we told you
then."
Some families find that compiling a
scrapbook with photographs starting from
birth, including some of the child
88
The TOF Child
in hospital and then at home,
accompanied by simple captions is
beneficial. Looking at these may be
painful for parents, but children are
often fascinated by a book about them
and can accept what has happened in a
very matter-of-fact way.
The Child's Viewpoint
Children soon learn how best to get
their parents' attention and a TOF
child may use his/her condition to test
boundaries at home. If a child can
manoeuvre parents into doing things he
wants, he feels he is stronger than they
are. It may bring satisfaction in the
short term, but in the longer term it
makes the child feel unsafe because
underneath it all, he knows how fright-
ened he can be. If he is stronger than
his parents, who can take care of him?
"Jake already understands that feeding
is an issue that causes frustration and
concern and he can play on the stress we
feel as parents."
Young children with illnesses or disabilities often think they are being punished for something. An explanation of their condition helps this, but one or two play therapy sessions with an exper-ienced play therapist can also assist.
Children express things with play they don't have words for; thoughts come to the surface and can be expressed non-verbally in play.
This gives the therapist – who is seen as authority – the opportunity to say:
"Some children think that because they have something different about them, they have done something wrong. I am telling you that this is not so. It is very unfair you have trouble with your throat and lots of other children don't, but it is not your fault."
"I recall enormous stress around feed
times as a child but didn't think much
of it. Now, as an adult, I can recognise
an underlying sense that I was at some
level 'at fault' for any problems in the
family.
Later in life, to understand how the anxiety
around my condition must have affected the
family, and to recognise that it was not my
fault, is an
enormous relief"
Siblings
The child is not the only one who
may feel guilty. Siblings often resent a
newcomer in the family, or they resent
the time taken for a child with a
disability. Some even wish they were
the TOF child. These feelings are
absolutely normal and children should be
encouraged to express such feelings,
hard though it is for parents. As with the
TOF child, the siblings should have
explanations as soon as possible.
"I'm told that when my mother was
pregnant with Simon my favourite game
was using her as a trampoline, and
I've always felt a sense of guilt that
somehow this was connected to his
being a TOF, it was only when I really
understood what TOF is that I no
longer felt this guilt."
"Looking back I appreciate how hard
my mother tried to spend time with us
older kids and to visit Simon in hospital.
However, I was only two when he was
born, and one of my earliest memories
is resenting the fact that my mother was
never at home at bedtime. I didn't really
get to know Simon until he was
permanently at home, before that he was
just something covered in wires. In fact I
only enjoyed going to visit him
because of the hospital play room."
89
Play Therapy
Dr Glenys Parkinson uses Projective Play
Therapy which was developed by Margaret
Lowenfeld. The child uses the available toys
as they wish, to express thoughts and feelings
which may not have been given words.
The therapist reflects back what the child is
doing and may make general comments such
as "lots of children may think ..."
This is a non-intrusive form of therapy;
the relationship between the child and
therapist is not worked with — only the
child's thoughts and feelings, as expressed
through play. Children love coming for this
work and look forward to it.
Which children benefit?
Children exhibiting the following
behaviours may benefit from play therapy:
• Behavioural difficulties
• Being sad over a long period
• Short temper
• Aggression • Worries over illness
• Withdrawal, inability to make friends
Making friends is possibly the best
prognostic sign and is therefore important
to take note of. Friends are a valuable source
of support and without them, a child may
feel very isolated.
Referrals
GPs will normally refer to a psychiatrist
at the local Child and Family Centre, who
usually works with other colleagues such as
Clinical Psychologists, Speech Therapists or
Occupational Therapists. These work
together as a team, and the most appropriate
person to see the child and family will be
decided on together.
After the first consultation, the family may
be seen together, or the child may be seen
individually.
Not all clinics offer play therapy.
90
The TOF Child
Dr Glenys Parkinson uses Projective Play
Therapy which was developed by Margaret
Lowenfeld. The child uses the available toys
as they wish, to express thoughts and feelings
The therapist reflects back what the child is
doing and may make general comments such
intrusive form of therapy;
the relationship between the child and
only the
child's thoughts and feelings, as expressed
through play. Children love coming for this
Children exhibiting the following
Making friends is possibly the best
prognostic sign and is therefore important
to take note of. Friends are a valuable source
of support and without them, a child may
GPs will normally refer to a psychiatrist
at the local Child and Family Centre, who
usually works with other colleagues such as
Clinical Psychologists, Speech Therapists or
Therapists. These work
together as a team, and the most appropriate
person to see the child and family will be
After the first consultation, the family may
be seen together, or the child may be seen
In sand tray work, the child is given a free choice from a selection of toys.
Wet or dry sand can be used: the therapist reflects on what the child is doing, always working with the child.
Mosaics are a little different; whereas sand tray toys have their own identity, mosaics can be given meaning(s) from the child's thought's and feelings. The
variously coloured shapes can be assembled into something which is either concrete or abstract. The child's thoughts and feelings about the mosaic are explored afterwards.
The TOF Child
On the other hand they may also feel protective, and worry about their sibling with TOF.
"I get worried when she goes into
hospital. I don't like her being away. I
like playing with the hospital toys and
visiting the nurses. I know sometimes
she has to go into hospital but I don't
like her upset."
"I love him so much because I waited so
long for my brother to come home from
hospital. He was so small when I saw him
born, but I couldn't cuddle him with his
tubes. I like him, he's nice, he's special to
me. He's my brother"
"Emma and I are very close (mother)
and we both try not to overprotect but
probably fail. We do try and encourage
her to be physically active to build her
strength and become adventurous. Adam,
her five-year-old brother, has a good
relationship with Emma, helped by the
fact she has always brightened and
smiled even on her worst days in hospital
when he comes to visit her."
It is quite possible for these
contrasting feelings to go together, one
being experienced one day, the other
another – or even both together.
"I couldn't help feeling a bit jealous of
all the t ime my parents spent with
Colin. However, I also remember feeling
a great affection for Colin and a sense
of protectiveness towards him. One
memory which sticks in my mind is
trying to feed Colin a packet of crisps,
which he found very difficult to eat. I sat
with him patiently for what seemed like
hours, giving him tiny pieces of crisp
alternated with a drink. He almost
managed it, and I was so disappointed
when he ' got stuck' as we used to call it.
People who didn't know about his TOF
would get alarmed when he started to
choke and cough,
but for me it was just a part of life. I
forgot that other children didn't have to
worry about what they ate. It is not until
now, looking back, that I realise how
incredibly brave and courageous Colin
was, to struggle through he hard times and
keep going."
Having a brother or sister with TOF
brings its problems, however many
seem to have gained in some way from
the experience.
"It was very traumatic and stressful at
times but we feel that we have learnt a
great deal from our younger brother's
stay in hospital."
"Tim was almost four-and-a-half years
old when Matt was born. We explained
as best we could why Matt was still in
hospital and how we would have to
gastrostomy tube feed him. Tim took
this all very well, and so when I was
allowed home from Aberdare Hospital
we took Tim with us to see Matt for the
first time (my husband had been to see
Matt several t imes but T im and I
hadn't seen him). Tim wasn't allowed
into the Special Baby Care Unit and so
he had to be lifted up to look through
the glass in the door. The nurse lifted Matt
from the incubator and held the open
ended gastrostomy tube aloft. Tim
looked lovingly at his little brother and
then, in a loud excited voice, said, "I can
see his stomach pump!" "
Friends
The support of friends can be a
huge help to the TOF child, their
siblings and parents.
For parents, in particular, friends can
be an important safety valve, giving
them the chance to discuss issues and
voice fears that other members of the
family are too close to. Parents may
find that they confide closely in certain
91
The TOF Child
friends and down-play things with
others in order to preserve a 'normal'
social life.
"Many of my friends are often over-
concerned about her health and
probably feel I should 'wrap her up
warmer' , others are very supportive
and lend an ear when I am down."
Friends can often seem overprotective:
"Friends have been concerned and
wanted to help, however, they can be
overpowering. You may need time alone
considering all the stress involved.
However, it helps to talk."
Entirely the wrong kind of response
can also be given, although well-meaning.
This is demonstrated by the following
words from a TOF parent:
"I gave my sister something to study
when she was training to be a nurse."
Even when the right response is
given, at times it just isn't enough.
"We have a few close friends who are
really good and understanding. Most
people don't have any idea what it's
like however and mothers commenting
on his size and speech etc., or trying to
compare drives me insane! Parents with
healthy children will never understand the
feeling of seeing your child suffer with a
long-term condition."
The support gained from friends is
crucial. However, in order to avoid
embarrassment, any particular difficulties
which the TOF child has should be
explained; siblings can also be
encouraged to explain about the TOF
condition to their friends.
"Though Simon is now fine, his regular
mealtime vomiting was unpleasant. As
a family we soon became used to this, yet I
remember feeling embarrassed by my
friends' reaction to him, as it was this that
made me realise he was
`different' . A few months ago Simon was
discharged from hospital and the entire
family felt a sense of relief. Though we've
always accepted him the way he is, that
occasion made us appreciate how lucky
we are that he now leads a totally normal
life."
The opportunity to meet with a group of
other TOF parents – especially those
whose children are older so that they can
pass on the benefit of their experience –
can be very helpful to parents who feel on
their own.
"The contact I have had with other TOF
parents with older children, breast
f eed ing counse l l o r s and 24 hour
contact with the nurses at Great Ormond
Street has been extremely useful. During
visits home in-between hospital stays I
have taken Amy to a couple of postnatal
classes at which I have managed to be
open about her condition, gaining interest
from outsiders. This has been very
important as it is too easy to feel different
and alone with suppressed emotions!"
Growing up
Moving on to playgroup constitutes
another milestone, as does nursery and
school. This book will be valuable to these
institutions.
"Playgroup were marvellous and managed
well without complaint. Nursery was a
disaster – they made a huge deal out of
Nicholas problems and I got quite
depressed about his condition and my
ability to cope. School have been
marvellous. The school doctor got a bit
overexcited, probably because she sees so
many ordinary children. Because we
moved just before Nicholas started school
the other parents do not know him and I
get the feeling he is regarded as very
92
The TOF Child
naughty by other mothers. (He isn't,
he's just astonishingly lively)."
"Eleanor started primary school when
she was four-and-a-half and I had briefed
her teacher about TOFs with the aid of
the helpful TOFS Group leaflet. They all
must have read it and t ak en i t o n
b o ard b ec au se so m e months later, I
heard that the dinner ladies paid
particular attention to the fact that
Eleanor must drink plenty of fluid while
eating!"
As the child grows up, details can be
added to the explanations. Up to at least
7 or 8 years of age, demonstrations will
aid a child's understanding, and help
them to cope with his/her difference.
Some children will find this difference
hard to cope with, especially as they
grow older.
"Philippa feels left out sometimes, as she
can't eat the same as her friends. At school
she has a lunch box, as do the majority of
six-year-olds, so no-one is any the wiser.
She misses eating chips and looks forward
to being older when she should be able to
manage them. She doesn't want to stand
out as being different from the other
children."
"Philippa's problems have not affected her
schooling, apart from occasional time-off
for check-ups. One friend's mum daren' t
have Philippa for tea in case she gets
stuck, although I think the fact that the
children can be argumentative perhaps
had more to do with it. She's OK at parties.
I used to look to see what was on the menu
and point out to Philippa what not to have.
She now knows for herself what to avoid."
Other children are capable of being
open about their condition.
"Charlie had a foley catheter for a long
time before getting a gastrostomy button.
The day after his button was fitted he went
to nursery determined to show all his
friends.
When I collected him later in the day it
was to be told by the staff that Charlie
and his button had kept the other children
quiet for some time.
Apparently, they had all disappeared into
the play house and were found lying on
the floor, T shirts pulled up, using cups
and saucers as 'buttons' so they could be
like Charlie. Shoe laces were removed
and used as extension sets and kettles in
place of 50ml syringes.
I never expected anything like this level
of acceptance from Charlie's peers and
he was so pleased that everyone liked
his new button – what more could we ask
for from the under fives?"
Moving on
The strain of a TOF child can be
enormous on families. Different
aspects of the experience stand out for
different families.
"All the problems made me much closer to
my TOF daughter than I might otherwise
have been and the memory of that time
remains to get me over 'teenager'
crises."
"Something out of the ordinary seems to
polarise people – they either embrace the
challenge with enthusiasm (or well-
disguised fear) or don't want to try and
understand or accommodate."
"I have a very close, caring relationship
with Lorenzo (TOF/VACTERL) as we
have been through so much together. I
think Joe, Lorenzo's brother, suffered
slightly because of all the extra
93
The TOF Child
attention Lorenzo got. We still find it
difficult to cope with, it has affected our
lives very deeply; we are all closer and are
all involved in Lorenzo's care. Friends try
and understand but it's impossible – they
don't understand our continual underlying
anxiety."
"I was frightened to be left alone with
Ryan. He would choke and take his
breath, so my daughter could not
consider going back to work at all. All
in all it has made us closer, but been very
stressful on us all."
"Even now, when he is so fit and well,
we often look at him and remember what
happened. It never leaves you."
Nonetheless, families come through
the challenges and learn to come to terms
with and even to cherish the special nature
of the experience.
"I think that we are a closer family
because of the trauma of having a
TOF child."
"Alistair was our first child and we tended
to feel he was very special to us because
of his condition and problems. The
arrival of our second child showed us
that the way we felt was because they were
ours, not because there were problems. I
did have to learn to relax and cope with
Alistair's problems and this probably
made me more relaxed with our other two
children, but I think this is probably true
with the oldest in every family – problems
or not.
We have moved house since Alistair had
much of his surgery and went through the
main recovery phase. If the subject arises,
we explain what Alistair was born with
and the main reactions are interest in the
condition and s y m p a t h y a t w h a t h e
h a d t o g o through. We have held
regular coffee mornings for TOES in the
village in an
effort to raise people's awareness, but
talk about it less as Alistair gets older
– he finds it embarrassing to be singled
out, as any teenager would."
Summary In summary, although a TOF child is
different, and family life is very much
affected by the arrival of a TOF child, it
is important that the child is not lead
to feel different – at least not so far as
possible.
The anxieties associated with the
c on d i t i on c an h a ve an e no r mo us
impact on a family but friends and
relatives together with the backing of
the medical team can help by offering
practical help and emotional support.
Patience and understanding may be
required for others to accept the
condit ion, but the rewards of such
initial efforts are well worth it – not
only for the parents' peace of mind and
well-being, but also for the child's
development in the long-term. If help
is needed, ask for it.
The term 'a TOF' – which is often
used – can too easily become a 'label'
for what is in many ways a normal child
with a particular difficulty, born to live
a fulfilling and enjoyable life:
"I was very scared, especially as he
choked quite badly the first time I looked
after him. I panicked but once he's
done it to you know what to look out
for and to expect the next time. It never
stopped me looking after him or treating
him any differently."
94
The TOF Child
Long Term Outcome;
Results of a Follow-up Study PAJ Chetcuti DM FRCP FRCPCH, Consultant Paediatrician, The General Infirmary at Leeds
Considerable information has been
provided on the possible problems
encountered in children born with TOE
This gives rise to two questions ... how
common are these problems and do they
improve as the child gets older?
These questions were answered by a
detailed follow up study from Melbourne,
Australia in 1987. A total of 366 patients
who had undergone TOF surgery (from the
first successful operation in 1948 to those
of the mid-1980's) were invited to attend
the hospital for review. Over 300
attended and half were adults. Patients
and/or their parents were asked about their
current health and previous problems;
height and weight measurements were
taken, a clinical examination made and
lung function tests performed.
Feeding
One in 3 under 5 years of age
experienced feeding difficulties with food
sticking and vomiting but this improved
dramatically in older children. Two thirds
had been re-admitted to hospital due to
swallowing problems, mostly in the
first 5 years of life; just under half had
needed a minor operation to stretch a
narrowing in the oesophagus at the
original operation site (a dilatation
procedure). Two thirds of adults stated
t h a t t h e y w e r e a w a r e o f m i l d
swallowing difficulties, however this
did not interfere with what they ate and
they were able to manage such
difficulties by drinking fluid with their
meals. Only one adult had a narrowing
in his oesophagus which needed a
dilatation procedure.
Chest Problems
Nearly half had been admitted to
hospital with 'noisy' breathing and
chest infections; again the majority of
these episodes were in the first 5 years
of life. The characteristic `TOF cough' was
present in three quarters under 5 years of
age, but became less frequent and less
severe in older children. Just under half the
adults said that whenever they coughed it
was still the harsh brassy cough, but that
this did not trouble them at all. Minor
chest infections occurred in only 1 in 5
adults, but responded very quickly to
antibiotics and did not require hospital
admission. One third of adults
occasionally experienced a `wheeze' (a
whistling nose coming from the chest)
but in over half this was because they had
asthma and was unrelated to the operation.
Exercise, Education and Lifestyle
Two thirds of older children and adults
felt that they were extremely fit and
regularly took part in sports where they
competed equal ly wi th thei r peers.
The rest reported only a slight reduction in
ability to sustain vigorous exercise
compared to their peers.
95
The TOF Child
40% of 5-10 year olds missed more
than one week of school per year because
of illness compared to 20% of 10-15
year olds. Only 10% of adults missed more
than one week of work because of any
illness. They were involved in a wide
variety of occupations and all said that
their operation(s) had not interfered with
their choice of career. The majority of
adults were married and their 15 children
were born with no significant health
problems.
Height and Weight
The height measurements in the group
were similar to children and adults who
had not experienced any major
illnesses. 10% were extremely thin, but
these were nearly all under 5 years of
age, suggesting that nutrition and
weight gain improved in older children
and adults.
Lung Function
Tes ts showed tha t 1 i n 10 had
slightly reduced lung volumes and lin
5 had slight obstruction to the flow of
air out of the lungs. The patients with
these findings were in no way
disadvantaged and led normal lives.
Summary
A number of children born with TOF
will develop feeding difficulties and
chest problems. These problems are
always at their worst in the first 2 years of
life and after 5 years of age are
uncommon. Some parents experience
great difficulties in these early years and
may need considerable support.
However, this very large study – the
largest of its kind on TOF follow up in
the world – is very reassuring. It shows
that the majority of children are able to
participate fully in sport and to live up
to their full academic potential.
Furtheimore, they grow up
comparable to their peers and their
achievements as adults are not
impaired by their earlier problems.
Quotations from TOF s, their families and friends and medical professionals
"Despite Jacob's continuous hospital
admissions during his first year (and his
inevitable small size), he makes up for
this in strength of character and cheeky
charm. He is already walking and is
incredibly curious and determined."
"It has undoubtedly been a very difficult
time – Jacob was our first child and we
spent much of his early life in hospital. He
still doesn't eat and is slow to thrive.
Despite all this he is our complete and
utter pride and joy and we love him with
a surging protectiveness that is hard to
describe."
"Hannah is a lively 2 year old now, and
although she has regular 'sticking' and
'choking' instances, and a cough that
everyone remarks upon, we enjoy a
wonderful 'normal' family life. We now
have another daughter, Rebecca, who
seems perfectly healthy and we look
forward to a positive future."
"James is a very lively, funny boy who
enjoys every minute of his life and
perhaps doesn't take things for granted,
like so many of us do, without realising
it often enough. We're so lucky to have
James. James is a perfect example to
show that this condition can be overcome
with the best possible outcome."
"I believe she is some sort of 'miracle'
child to come through all she had to
suffer in her baby and infant life. She is
very special."
96
"Matthew is now nearly eight. A strong
willed energetic lad who is madly keen on
football. TOF has not held him back and
he is a picture of health. He is a real
success story and an encouragement to
all TOF parents."
"Ben has been relatively healthy for a
TOF. He has been dilated 3 times –Oxford,
Bristol and Montreal, Canada. Fortunately
we were well insured!!! He liked to visit a
variety of hospitals! Winter coughs were a
major problem until we discovered a
wonderful GP who liaised with Oxford. We
always kept a low dose antibiotic ready
and used it when and if we needed it. He
seemed to grow out of it at about 7 years
as our Consultant had predicted although I
never really believed it could happen.
He is now 10 years old. Happy and
healthy. He still gets stuck occasionally,
but pushes it down with a drink. He
represents the county in gymnastics, plays
the trumpet and leads a normal life. The
traumas were many along the way but
he's been worth every minute. We also
have an eight year old daughter who has
no problems at all."
"For my second baby I was booked into
different hospital where they had the
facilities for high-image scanning in order
to look out for a possible TOF, and at
about 29 weeks we saw our baby actually
swallowing! In our heart of hearts we
were not entirely convinced he was fine
until he was born and breastfed very
efficiently within 45 minutes of being
born."
"Matthew (7) is obviously aware of his
condition. We have never made a big issue
out of it and have not therefore explained
the what TOF actually is. He still thinks
the scars came from when he rescued his
Dad from sharks in the sea! We will
explain it to him, quite openly, when he
has a more understanding mind."
The TOF Child
"Matthew is now nearly eight. A strong-
willed energetic lad who is madly keen on
football. TOF has not held him back and
health. He is a real
encouragement to
"Ben has been relatively healthy for a
Oxford,
Bristol and Montreal, Canada. Fortunately
we were well insured!!! He liked to visit a
variety of hospitals! Winter coughs were a
em until we discovered a
wonderful GP who liaised with Oxford. We
always kept a low dose antibiotic ready
and used it when and if we needed it. He
seemed to grow out of it at about 7 years
as our Consultant had predicted although I
could happen.
healthy. He still gets stuck occasionally,
plays
life. The
way but
We also
have an eight year old daughter who has
"For my second baby I was booked into a
image scanning in order
to look out for a possible TOF, and at
t 29 weeks we saw our baby actually
hearts we
he was fine
efficiently within 45 minutes of being
"Matthew (7) is obviously aware of his
ever made a big issue
explained
still thinks
the scars came from when he rescued his
explain it to him, quite openly, when he
Above:
Sandra Kay Lawton at age 3 years.
Above:
Christine Micolaud, who was born with
TOF/OA, photographed on her wedding
day. She is now mother to two chidren.
The TOF Child
Christine Micolaud, who was born with
TOF/OA, photographed on her wedding
day. She is now mother to two chidren.
97
The TOF Child
VACTERL
R Lee BMedSci, Senior Medical Student and L Kapila OBE FRCS, Consultant
Paediatric Surgeon, Queens Medical Centre, Nottingham.
WATER' is an acronym for:
Vertebral (spinal) defects
Anorectal atresia (failure of the anus
and lower end of the gut to form)
Tracheo-oesophageal fistula with or
without Esophageal atresia
(American spelling)
Renal anomalies (abormalities of the
(kidney) and radial limb dysplasia
(abnormal forearm development).
The relationship between these
congenital anomalies was first noted in
1968, when it was called Say-Gerald
Syndrome. Other synonyms have included
Kaufman syndrome, PIV and PIAVA, but
the term which is currently favoured (and
is replacing VATER) is VACTERL. The
extra letters stand for Cardiac (heart) and
Limb defects respectively. They were
added because it is now appreciated that
over 70% of affected children have heart
problems, and limb abnormalities are not
always restricted to the forearm, with
40% of cases having defects of the foot or
leg.
Diagnosis To qualify as a `VACTERL' child, three
of the seven components mentioned above
must be present. There may also be any
number of other characteristics which
occur more frequently in affected
children than the rest of the population;
these include ear abnormalities, genital
anomalies, cleft lip and/or palate, thumb
abnormalities, various changes in gut
development and in the fetus , the
presence of a single artery in the
umbilical cord (normally there are two).
No individual
is likely to display all these features
and no two individuals are likely to be
affected in exactly the same way.
Cause
It is estimated that for every 6,250
births, one child will have VACTERL.
This is rare compared with many other
conditions; one baby in 2,500 is born
with cystic fibrosis, and one in 700 has
Down syndrome. Large numbers of
patients are required to scientifically study
any medical condition, so the small
numbers of children with VACTERL, and
their wide variety of features, has made
research difficult. Consequently, little is
known.
Growth of the fetus in the womb is
enormously complex. The original two
cells (the sperm and the egg) which
combine and develop into a new
individual divide, and their progeny
divide and so on, until the millions of
cells which make up a human being
are formed. This is not just a process
of multiplication, and different cells
take on different jobs. Large groups of
cells will become muscle, others will
make up bone and eventually every type
of cell in the body is represented.
Ensuring that the right cells go to the right
place at the right time is a delicate task; in
spite of the massive scientific efforts
aimed at understanding the intricate
mechanisms involved, many pieces of the
jigsaw are still missing.
The cause of VACTERL is unclear.
One theory suggests that cells are
disrupted at an early stage of develop-
ment. There are only three types of cell
98
in the three-week-old fetus, one of which
is called a mesodermal cell. These cells go
on to form the gut wall (including the
oesophagus), the kidneys and bone
(including the spine and the skeleton of the
limbs). In principle, a change in a
mesodermal cell could result in a change in
any of these body parts, which would
explain the wide variety of abnormalities
present in VACTERL. Efforts have been
made to identify agents, such as drugs,
which could adversely affect these cells,
but so far nothing has been proved.
It is important to stress that VAC
_____ IERL is not an inherited
condition. This means that the future
offspring of a family with one
VACTERL child have no greater chance
of having VACTERL than the children of
unaffected families. There is, however,
an exception to the rule. Chromosomes
are the inherited structures of DNA which
determine everything about an individual.
Two chromosomal abnormalities (the
more common of which is Edwards
syndrome) may result in the features of
VACTERL –two rare cases in which the
cause of VACTERL is known. In order to
exclude this, the chromosomes of all
VACTERL babies are examined.
Antenatal Diagnosis
The regular use of ultrasound to
examine the fetus is greatly increasing
the number of abnormalities detected
before birth. Many VACTERL babies
will not be picked up during a routine
scan, but in pregnancies which merit
detailed ultrasound investigation it is
more likely that skeletal abnormalities,
kidney defects and a single umbilical
artery will be seen. Perhaps, as ultrasound
scanning continues to improve, ante-natal
diagnosis of VACTERL will become the
norm.
The TOF Child
old fetus, one of which
cell. These cells go
on to form the gut wall (including the
(including the spine and the skeleton of the
mesodermal cell could result in a change in
any of these body parts, which would help
explain the wide variety of abnormalities
present in VACTERL. Efforts have been
made to identify agents, such as drugs,
could adversely affect these cells,
This means that the future
VACTERL child have no greater chance
of having VACTERL than the children of
unaffected families. There is, however,
an exception to the rule. Chromosomes
res of DNA which
determine everything about an individual.
Two chromosomal abnormalities (the
result in the features of
two rare cases in which the
cause of VACTERL is known. In order to
the chromosomes of all
examine the fetus is greatly increasing
the number of abnormalities detected
before birth. Many VACTERL babies
will not be picked up during a routine
can, but in pregnancies which merit
detailed ultrasound investigation it is
more likely that skeletal abnormalities,
kidney defects and a single umbilical
sound
natal
ACTERL will become the
Symptoms and Treatment
VACTERL children are categorised
as TOFs because this is a cardinal feature
of the condition, but their problems and
hospital management differ because of
their multiple abnormalities. Only 60% of
all `VACTERLs' will have a TOF to
repair.
Vertebral Abnormalities
Vertebrae are flat bones which are
stacked on top of each other to form the
spine. If they are deformed, an abnormal
spinal shape may result (` scoliosis' or
`kyphosis'). Abnormal bone develop
ment may also be accompanied by
abnormalities in the associated muscles
and nerves, and these children can appear
slightly lopsided if the left and right
sides of the body are affected unequally.
Fortunately, these deformities are often
mild.
Treatment of an abnormally shaped
spine depends on the severity of the
deformity. If minor, no intervention may
be required. More severe cases often
necessitate use of a brace (a contraption
worn by the child which exerts a force in
order to straighten or prevent further
curvature of the spine). This is a long term
option and can be worn for
several years. Gross
distortions require
surgical correction as
they may cause severe
disability, perhaps
decreasing the size of the chest and
therefore the
space available
for the lungs to
expand. This is
a major
undertaking
with a long
post-operative
course.
Scoliosis
The TOF Child
VACTERL children are categorised
as TOFs because this is a cardinal feature
of the condition, but their problems and
hospital management differ because of
malities. Only 60% of
stacked on top of each other to form the
spine. If they are deformed, an abnormal
spinal shape may result (` scoliosis' or
`kyphosis'). Abnormal bone develop-
abnormalities in the associated muscles
appear
right
sides of the body are affected unequally.
Fortunately, these deformities are often
an abnormally shaped
deformity. If minor, no intervention may
necessitate use of a brace (a contraption
worn by the child which exerts a force in
curvature of the spine). This is a long term
Scoliosis
99
Anorectal Atresia
This is a broad term which describes
range of abnoinialities. At one extreme,
children have an intact bowel with a
blind end Clow imperforate anus'). At the
other extreme, the bowel stops quite some
distance short of what should be the anal
opening, and there are often abnormal
connections between the bowel and the
bladder or vagina.
A low imperforate anus is treated by
a single surgical procedure soon after
birth which has few long-term
complications. The more severe (high)
case requires two or three separate
operations, the first of which creates a
`co los tomy. ' This i s an a r t i f i c ia l
opening in the skin which is attached
to the bowel on the inside and a plastic
bag on the outside. It enables the child
to defaecate and is easily managed by
regular bag changes. In subsequent
operations the bowel and anus are
reconnected and, after healing, the
colostomy is closed. Continence will
develop if the surrounding muscles and
nerves are able to function adequately.
Cardiac Abnormalities
`Ventricular septal defect' (VSD) is
the commonest type of cardiac abnormality
(abnormality of the heart), a c c o u n t i n g
f o r mo r e t h a n t h r e e - quarters of all
cases. It is a hole in the wall that
separates the two large chambers of the
heart. Consequently, the heart does not
function efficiently. The child is
symptom-free if the hole is small, but a
doctor would be able to detect it. Moderate
cases may cause breathlessness on
feeding and recurrent chest infections,
but these problems tend to subside with
age. Babies will be short of breath at an
earlier age with severe defects and often
sweat. They may be underweight and
become severely ill.
100
The TOF Child
This is a broad term which describes a
children have an intact bowel with a
blind end Clow imperforate anus'). At the
other extreme, the bowel stops quite some
A low imperforate anus is treated by
complications. The more severe (high)
l
opening in the skin which is attached
to the bowel on the inside and a plastic
bag on the outside. It enables the child
the commonest type of cardiac abnormality
c o u n t i n g
quarters of all
is small, but a
le to detect it. Moderate
Above:
Anorectal Atresia and Hypospadias.
T h i s c h i l d d o e s n o t h a v e a n a n u s
(anorectal atresia) and his urinary orifice
is situated on the under-surface of the penile
shaft (hypospadias).
He also has an abnormal connection
between the bowel and bladder; faeces
are therefore seen at the urinary orifice.
With all VSD's there is a greater risk
of heart infection, and in mild cases the
only treatment is a course of antibiotics
given before surgical or dental procedures.
In moderate cases, children can
accumulate excess body fluid, and this is
usually controlled wi th tablets .
Surgery to c lose the defect is largely
reserved for the severe case which does
not respond to medication. This is best
done as a single operation, during which
the function of the baby's heart and
lungs are taken over by a machine.
There are many other heart defects
which may occur in isolation or
accompany a VSD.
The TOF Child
Tracheo-Oesophageal Fistula with Oesophageal Atresia
Symptoms and treatment do not
differ from other TOFs.
Renal Anomalies
These fall into two main categories;
total failure of one or both kidneys to form,
and 'other' anomalies.
Absence of both kidneys has sinister
implications and may be detected ante
natally by ultrasound. A single absent
kidney is however entirely symptomless,
because the remaining kidney can fully
compensate for the deficiency.
The 'other' category is vast, including
abnormally small (` hypoplastic') kidneys,
abnormally shaped kidneys (e.g.
'horseshoe kidney'), abnormally placed
kidneys (e.g. 'pelvic kidney'), abnormally
arranged kidney cells (e.g. `polycystic
kidney') and abnormal structures which
communicate with the kidney (e.g. an
abnormal blood s up p l y , o r an
a bn or ma l s ha pe o r number of the
tubes (ureters) which run between the
kidney and bladder).
These are not always associated
with malfunction, but problems arise
The TOF Child
fall into two main categories;
total failure of one or both kidneys to form,
Absence of both kidneys has sinister
implications and may be detected ante-
natally by ultrasound. A single absent
kidney is however entirely symptomless,
cause the remaining kidney can fully
The 'other' category is vast, including
kidneys,
'horseshoe kidney'), abnormally placed
kidneys (e.g. 'pelvic kidney'), abnormally
arranged kidney cells (e.g. `polycystic
structures which
the kidney (e.g. an
r of the
run between the
These are not always associated
with malfunction, but problems arise
when the kidney cells do not work
efficiently, either because they never
developed properly or because they have
been damaged. The latter is commonly
caused by an obstruction to urine flow,
particularly at the junction of the kidney
and ureter. The resultant backlog of urine
is often associated with infection and the
baby may have diarrhoea, a fever,
vomiting and be slow to gain weight.
Antibiotics are prescribed to prevent or
treat infection a n d t h e o b s t r u c t i o n
i s r e l i e v e d surgically. If only one
kidney is involved the other will always
compensate.
Left kidney
Ureters
Bladder
Normal kidneys Line of rib
cage
Right k i d n e y '
Spine
Pelvis —
The TOF Child
developed properly or because they have
commonly
urine flow,
of the kidney
backlog of urine
with infection and the
prescribed to prevent or
a n d t h e o b s t r u c t i o n
kidney is involved the other will always
kidney
Ureters
Bladder
101
Renal abnormalities
Ureteric duplication
Crossed renal ectopia
Hypo plastic Horseshoe kidney kidney
102
Unilateral agenesis
(one kidney)
Pelvic kidney
The TOF Child
Below:
Radiograph of a grossly enlarged kidney
filled with contrast. Urine is produced by
this kidney, but cannot leave via the ureter
because of an obstruction.
Polycystic kidney
Radiograph of a grossly enlarged kidney
filled with contrast. Urine is produced by
this kidney, but cannot leave via the ureter
The TOF Child
Limb Abnormalities
The forearm (the part of the arm
between the elbow and wrist) contains
two bones, the ulna and radius. Partial
or total failure of the radius (and the
muscles which attach to it) to develop
is frequently associated with VACTERL.
Characteristically this causes the hand
to lie at a right-angle to the forearm
and usually the thumb is also malformed
or absent. Considerable deformity and
disability may result.
Initially, the affected arm is passively
corrected with a splint. This is followed
by an operation between the age of 6
and 12 months to realign the hand and
forearm. If the thumb is absent a second
procedure, called 'pollicization', is
used to create a surrogate thumb by
rotating the index finger. Physiotherapy
is an important part of the rehabilitation
process and a considerable degree of hand
function can be restored.
Further details about these problems are
given in the following chapter.
Below:
Radial club hand, a characteristic limb
anomaly in VACTERL children.
The VACTERL Child
Hospitals are a second home for
VACTERL children. They receive
intensive medical attention, both to treat
the immediate problems and to monitor
their progress. This involves frequent
consultations with a variety of specialist
doctors, in addition to intensive general
health surveillance, such as regular growth
checks. The impact of such prolonged
hospitalisation should not be
underestimated. Although VACTERL
children have comparable intelligence to
their peers, their social integration and
development is almost inevitably affected.
Ultimately, the future of an affected
child will be determined by the success
of their treatment. Residual problems may
cause long-term disability, the severity of
which will vary between individuals and
depends on the number and type of
defects. In the majority of cases,
continued medical support will be
required, but this does not prevent a
VACTERL chi ld f rom leading an
active, independent and fulfilled life.
References
1. Bayne L.G. (1993). Radial Club Hand
(Radial Deficiencies) in
` O p e r a t i v e H a n d S u r g e r y , '
Churchill Livingstone, New York.
2. Beals R.K. and Rolfe B. (1989).
`Current Concepts Review VATER
Association. A Unifying Concept of
Multiple Anomalies.' The Journal of
Bone and Joint Surgery 71-A: 948-
950.
3. Buck-Gramcko D. (1985).
aadialisation as a New Treatment
for Radial Club Hand.'
The Journal of Hand Surgery
10A: 964-968.
103
4. Chen J.M., Schloss M.D. and Laberge J. (1991). 'Extensive Upper Aerodigestive Tract Anomalies in `VACTERL' Association.' Archives of Otolaryngology, Head and Neck Surgery 117: 1407-1410.
5. Jablonski S. (1991). Jablonski's Dictionary of Syndromes and Eponymous Diseases, Krieger Publishing Co.: 624.
6. Khoury M.J., Cordero J.F., Greenberg F., James L.M. and Erickson J.D. (1983).
`A Population Study of the VACTERL Association: Evidence for Its Etiologic Heterogeneity.' Paediatrics 71: 815-820.
7. Merlob P. and Naor N. (1994). `Drug Induced VATER Association: Is Dibenzepin a Possible Cause?' [Letter]. J Med Genet 31: 423.
8. Morris P.J. and Matt R.A. (1994). `Oxford Textbook of Surgery,' Oxford University Press.
104
The TOF Child
9. Sadler T.W. (1990). `Langman's Medical Embryology,' Williams and Wilkins, Baltimore.
10. Say B. and Gerald P. (1968). `A New Polydactyly/Imperforate-Anus/Vertebral-Anomalies Syndrome?' [Letter].
The Lancet 2: 688.
11. Strickland J.W. and Kleinman W.B. (1993). 'Thumb Reconstruction for Congenital Absence' in 'Operative Hand Surgery,' Churchill Livingstone, New York: 2044-2073.
12. Weaver D.D., Mapstone C.L. and Yu P. (1986). 'The VATER Association. Analysis of 46 Patients.' Am J Dis Child 140: 225-229.
13. Wiedemann H.R., Kunze J., Dibbern H. (1992). NATER Association,' in 'An Atlas of Clinical Syndromes. A Visual Aid to Diagnosis,' Wolfe Publishing Ltd.: 548.
Ben Gordon, VACTERL child
Ben was born with TOF/OA, radial club left hand and a vertebra missing. Left; Ben aged 14 months.
Below; his 4th birthday, after surgery on his arm; Ben is in the middle near the
base of the photograph.
The TOF Child
The TOF Child
Useful Resources and Support Groups for VACTERL
Information about VACTERL can be gained from TOFS office: additional contact
details may also be available from your hospital.
Association for Children with Heart Disorders Postal contact through umbrella group,
Children's Heart Foundation (see below)
Tel: 01706 213632 (Helpline)
Email: [email protected]
Web site: www.heartchild.info
British Kidney Patient Foundation
Bordon, Hants GU35 9JZ
Tel: 01420 472021/2
Web site: www.britishkidney-pa.co.uk
Children's Heart Foundation
52, Kennington Oval, London SE1 1 5SW
T e l : 0 80 8 80 8 5 00 0 (F re ep ho ne
9.30am – 9.30pm)
Email: [email protected]
Web site: www.childrens-heart-fed.org.uk
Contact a Family
(source of contact details for other medical
support groups and charities) 209-211
City Road, London EC1V 1JN Tel:
0808 808 3555 (Helpline open loam-4pm,
Mon-Fri).
On-line helpline through web site
Web site: www.cafamily.org.uk
NASPCS (National Advisory Service to Parents of Children with a Stoma) 51 Anderson Drive, Darvel,
Ayrshire KA17 ODE
Tel: 01560 322024
Email: [email protected]
Web site: www.naspcs.co.uk
REACH: The Association for Children with Hand or Arm Deficiency PO Box 54, Helston,
Cornwall TR13 8WD
Tel: 0845 1306 225
Email: [email protected]
Web site: www.reach.org.uk
Sacral Agenesis Contact Group
15 Elizabeth Gardens, Dibden Purlieu,
Southampton, Hants SO5 4NF
Tel: 023 8084 2661
STEPS
Association for People with Lower
Limb Abnormalities
Lymm Court, 11 Eagle Brow, Lymm,
Cheshire WA 13 OLP
Tel: 0871 717 0044 (Helpline open
Monday-Friday, 9.30am - 4.00pm)
Email:[email protected] Web
site:www.steps-charity.org.uk
Tracheo-Oesophageal Fistula Support (TOFS)
St. George's Centre, 91 Victoria Road,
Netherfield, Nottingham NG4 2NN
Tel: 0115 961 3092
Email: [email protected]
Web site: www.tofs.org.uk
105
The TOF Child
Radial Deformities in VACTERL By Simon P.J. Kay, FRCS, FRCS (Plastic Surgery), Consultant Plastic Surgeon,
St James University Hospital, Leeds.
The VACTERL anomaly includes the
component 'radial.' This refers to radial
dysplasias which affect the forearm and
hand. In order to understand these
conditions, one must be familiar with
the basic anatomy of the forearm.
Nomenclature
The forearm is made up of two bones;
the ulna which hinges with the upper arm
humerus, and the radius which swivels
around the ulna as the hand rotates.
Because the hand can rotate from the
elbow so that the palm faces forward or
backwards, the borders of the forearm are
not named inner and outer but are named
after the underlying bone; radial or ulnar.
The thumb therefore lies on the radial
border of the hand while the little finger
lies on the ulnar border.
Forearm Viewed from the Front
`Dysplasia' simply means abnormal
development. In radial dysplasia there
is a variable degree of abnormal
development on the radial border of
the forearm and hand. Radial dysplasia
may occur in one or both arms, and is
particularly commonly associated with
the VACTERL anomaly, but may occur
on its own or in association with other
syndromes.
The resulting deficiency is best
considered in terms of the forearm
deformity and the hand defonnity. These
do not necessarily correspond; a major
abnormality of the thumb may be
present with a minor abnormality of
the forearm (or vice versa).
The Forearm Anomaly: Radial Club Hand.
The normal hand articulates with both
the radius and the ulna at the wrist.
The articulation with the ulna is relatively
small and the radius provides more of the
support for the hand.
In radial dysplasia the soft tissues
and flesh of the forearm are affected as
well as the bone. The arrangement of
muscles and nerves may be abnormal
and some are commonly absent.
The degree to which the radius is
abnormal is variable; in the most
Left:
Arrangement of forearm bones. Note how
the radius rotates around the ulna and
each border of the hand is named after the
underlying bone when the palm is facing
forwards and the elbow is by the side (the
so-called anatomical position).
106
severe cases the radius is completely
absent and the elbow joint may be
disturbed or even fused with no
movement possible. In the mildest of
cases the radius is merely slightly smaller
than the other side and there is minimal
deviation at the wrist. Between these two
extremes there may be a small remnant of
radius, or the radius may be only partially
formed.
In any case where the distal part (hand
end) of the radius is absent, there may be
an abnormal bar of gristle connecting the
remnants of the radius to the hand. This
gristle remnant is known by the German
word `anlage' and has a very limited
ability to grow. It is attached to the radial
border of the hand and wrist; as the ulna
grows in the mother's womb, lack of
growth in the radial anlage draws the
hand into a deviated "club" position.
Anlage Results in Deviation ofthe Hand with Growth...
The degree to which the hand and wrist
deviate at birth and thereafter depends on
the degree to which support is lacking (i.e.
the nature of the remaining bony
platfoiin on which the hand sits) and the
degree to which the
The TOF Child
movement possible. In the mildest of
cases the radius is merely slightly smaller
than the other side and there is minimal
deviation at the wrist. Between these two
extremes there may be a small remnant of
us, or the radius may be only partially
In any case where the distal part (hand
end) of the radius is absent, there may be
an abnormal bar of gristle connecting the
radius to the hand. This
gristle remnant is known by the German
and has a very limited
It is attached to the radial
hand and wrist; as the ulna
the mother's womb, lack of
the radial anlage draws the
Results in Deviation of
The degree to which the hand and wrist
deviate at birth and thereafter depends on
the degree to which support is lacking (i.e.
hand sits) and the
anlage tethers the wrist, meaning that
the ulna pushes the wrist further and
further into deviation as it grows in length.
Another important component of the
deviating force is muscle action. Once the
wrist has begun to deviate, muscle
activity will tend to exacerbate the
deviation as the wrist is levered about its
fulcrum or point of contact with the
ulna.
Muscle Pull Causing Hand Deviation
Treatment of the Forearm
Treatment of the wrist and forearm may
not be seen as a priority at the time
of birth. However, by the time the child
reaches adolescence, many of the other
anomalies in the VACTERL syndrome
will either have been successfully
addressed or be of little consequence.
The wrist and hand may then be the
most significant and enduring stigma of
the condition and may be the only part
that is causing any form of disability.
Surgical treatment is by no means
automatically indicated and there are
complex decisions to be made along
the way before deciding what
treatment may be appropriate.
Remnant of radius
Fulcrum
Muscle pull
The TOF Child
anlage tethers the wrist, meaning that
length.
of the
deviating force is muscle action. Once the
about its
Muscle Pull Causing Hand Deviation
may
time
of birth. However, by the time the child
reaches adolescence, many of the other
anomalies in the VACTERL syndrome
quence.
be the
stigma of
only part
Surgical treatment is by no means
automatically indicated and there are
complex decisions to be made along
107
The TOF Child
The Immediate Neonatal Period
It has been shown that splinting and
passive stretching exercises for the
wrist and elbow can not only maintain
whatever motion is already available,
but can also improve an initially poor
range of motion. Such exercises may
also lessen the degree of deviation at
the wrist.
The application of splints to a
neonate's arm is extremely difficult
and can be very trying for both the
physiotherapist and the mother. For
this reason many surgeons prefer to
guide their patients in gentle but firm
and frequently repeated passive
stretching exercises in the wrist and
elbow. If these are begun early in a
child's life they will be taken as normal
and will not provoke distress.
Most children with radial dysplasia have
good or normal flexion and extension
(bending and straightening movements) in
the elbow. In patients where the range of
motion is restricted, any improvement
as a result of therapy is welcome, even
if the child is unable to move the elbow
voluntarily. Once the flexibility of the
joint has been regained, the ability to
move the joint can be restored by
surgery, provided the joint remains
supple and mobile.
P h ys i o t h e r a p y a l o n e w i l l n o t
resolve the wrist contracture and
d e v i a t i o n b u t t h i s s h o u l d n o t
discourage parents from an exercise
regimen. Any improvement in the
range of movement in the joint will make
subsequent surgery and splinting more
effective and less complex.
Surgical Treatment of the Elbow
If the elbow is completely rigid,
treatment may not be possible.
In some cases it is possible to
surgically release the structures at the
back of the elbow or any bony bridge
between the forearm and the upper arm
(synostosis) in such a way as to allow
some assisted or passive motion to be
restored. Thereafter voluntary movement
and physical strength may be restored
to the elbow by transferring a muscle
from the wall of the chest. This can be
very successful in restoring to the child
the ability to bend and sometimes to
straighten the elbow. However, it is not
indicated in all cases, depending on the
initial condition of the elbow.
Many surgeons prefer to delay this
surgery until the age of three or four years
when the structures in the region of the
elbow are more mature, while others
prefer to restore elbow bending at as
early an age as possible in order to
facilitate subsequent decisions about
the management of the wrist.
Surgical Treatment of the Wrist
Much of the surgical management
of the wrist was pioneered during the
Thalidomide epidemic of the 1960s.
Not all children should have the wrist
straightened; in particular those children
with VACTERL anomaly having rigid,
straight elbows should only have wrist
surgery under unusual circumstances and
after careful discussion. If the elbow is
unable to bend, the presence of a bent
wrist and short forearm can be an
advantage in personal hygiene and
feeding.
T h i s k i n d o f s u r g e r y i s a l s o
relatively contraindicated in cases with
other serious complications.
108
Surgical endeavours to straighten
wrist have undergone many advances and
changes. In principle, the tight structures
which are tending to deviate the wrist (the
skin, the radial anlage, the abnormal
muscles, and the tight joint capsule and
lining of the joint) are divided or
lengthened and the wrist is therefore
able to rotate straight on the end of the
ulna.
In practice, the wrist can be very tight
and difficult to free up. Several solutions
to this problem have been found,
including removing bone from the wrist
so that the hand can sit straight on the end
of the ulna, or removing a slot of bone
from the wrist so that the wrist fits like
a cap over the end of the ulna.
The latter procedure is known as
centralisation and is preferable because
removing bone from the ulna could affect
growth. The end of the ulna is where its
longitudinal growth occurs; removal of
or damage to the end of the ulna may
therefore result in an even shorter
forearm than would normally be the case.
The surgeon may be
unable to straighten the
wrist...
Centralisation
The TOF Child
Surgical endeavours to straighten the
advances and
tight structures
which are tending to deviate the wrist (the
anlage, the abnormal
tight joint capsule and
the wrist is therefore
able to rotate straight on the end of the
In practice, the wrist can be very tight
and difficult to free up. Several solutions
the wrist
t straight on the end
removing a slot of bone
so that the wrist fits like
and is preferable because
removing bone from the ulna could affect
th. The end of the ulna is where its
occurs; removal of
or damage to the end of the ulna may
forearm than would normally be the case.
The sensitive 'growth zone' at the
of the ulna can also be damaged by
excessive pressure; for this reason it is
better to remove bone from the wrist
than to force the wrist bone over the
ulna under great compression.
In recent years there has been a move
towards distraction lengthening in
which a frame is placed on the hand
and forearm in such a way that pins
through the ulna and pins through the
hand can be moved apart gradually (1mm
a day maximum) so as to gently stretch
and 'grow' the hand into a straighter
position prior to surgery. The result of this
is that there is little or no compression
when the hand is finally surgically
straightened on the wrist.
This procedure has proved very
effective although in many units use of
the technique is only in its infancy and
surgeons are still learning the risks and
benefits of the procedure.
Below.
Radiograph of a child with a distraction
device on both sides of the wrist.
Sometimes these are only put on one side.
The TOF Child
The sensitive 'growth zone' at the tip
excessive pressure; for this reason it is
better to remove bone from the wrist
than to force the wrist bone over the
move
in
n the hand
hand can be moved apart gradually (1mm
a day maximum) so as to gently stretch
position prior to surgery. The result of this
here is little or no compression
effective although in many units use of
the technique is only in its infancy and
surgeons are still learning the risks and
Radiograph of a child with a distraction
Sometimes these are only put on one side.
109
The TOF Child
I f l i t t le or no bone has been removed in order to accommodate the hand and wrist on the end of the ulna, then the hand may be pushed slightly further across towards the ulnar border of the forearm and the tight muscles of the wrist reorganised so as to balance the hand on the end of the ulna. This
procedure is known as radialisation and has the theoretical benefits over centralisation of resecting less bone, being less likely to interfere with growth, and allowing more mobility at the wrist (because the muscles are restored and the ulna is not placed in the slot within the wrist).
Prot racted spl in t ing may be
necessary after centralisation or
radialisation. At the time of surgery a
pin is placed through the wrist and the
ulna and this serves as an 'internal
splint' which may remain in place for
a year or even more after surgery and
may be augmented by an external
splint. It may be necessary to continue
splinting for many months or even years
before the ulna gradually broadens and
becomes a more stable platform on
which the wrist may balance.
In practice it has proved very difficult
to obtain reliable and reproducible
results with radialisation. In both
centralisation and radialisation further
surgery for rebalancing may be
necessary even quite early after the
first procedure and certainly is not
unusual at least once during the growth
of the child.
At the time of radialisation or
centralisation there is a deficit of skin
on the radial border of the wrist and an
excess of skin on the ulnar border of
the wrist. Various operations have been
devised to redress the balance between
these two areas but all the procedures
result in scarring on the back of the
wrist and in some cases may give rise
to slow healing in at least one part of
the wound. This is not usually painful
or disturbing for the child.
At the time of radialisation or centralisation the surgeon will be able
to inspect the tendons which pass from the forearm to the hand as well as the muscles and soft tissue structures in the hand itself. This may be important in planning surgery to the hand later.
110
The TOF Child
Surgical Treatment of the Forearm Bones
Frequently the restrictions causing
hand deviation also affect the growth
of the ulna bone.
When the radius is partially or
completely absent and there is an
unyielding anlage connecting the elbow
to the wrist, then not only will the wrist
deviate but the ulna may be forced to grow
in a curve (i.e. to 'bow').
This bowing can become extreme and
result in a 90° bend in the forearm. It
may also result in a twist in the forearm
because of the complex arrangement of the
anlage. These abnormalities may lead to
an apparently shorter forearm than is
truly the case.
At the time of the operation for
radialisation and centralisation of the
wrist, the surgeon may choose to
straighten the forearm bones by
rearranging wedges of bone within the
middle of the arc and the ulnar. These
will be held in place by the same pin
that holds the wrist.
There has been some discussion about
the pros and cons of distraction
lengthening of the foreatm after the
wrist has been centralised (this is in
distinction to the distraction lengthening
mentioned previously, which serves to
straighten the wrist prior to surgery and
which may be undertaken from a very
early age –even in the neonatal period).
Distraction lengthening of the forearm
bones is generally considered to be an
option for later in the child's life and the
benefits of this may, in some patients
eyes, be outweighed by the
disadvantages. These need to be discussed
carefully with the surgeon undertaking the
procedure, and include minor infections,
pain, discomfort and scarring as well as
the possibility of poor healing and
function.
It should be recognised that any
distraction lengthening may need to be
repeated a number of times while the
child is still growing.
The Hand Anomaly
In radial dysplasia, the major anomaly in
the hand is a variable degree of hypoplasia
of the thumb and abnormalities in the long
tendons which pass to the remaining
fingers. Hypoplasia simply means
undergrowth and hypoplasia of the
thumb has been classified by the German
surgeon Blauth as follows:
Type I: slight reduction in size but all
structures normal.
Type II: small thumb with abnormalities
of the muscles and tendons, instability
(wobbliness) of the middle joinfof the
thumb and a tight restricted web space
between the thumb and index finger.
111
Type III: small skeleton of the thumb,
abnormalities of many if not all of the
muscles, abnormal stiffness in all of the
thumb joints and a highly abnormal first
web space. The position of the thumb may
also be abnormal.
Type IV: a 'floating' thumb attached
by only a thread.
Type V: total absence of the thumb.
The abnotnialities of the thumb are
surprisingly often overlooked when they
are mild at birth. The decision whether or
not to treat the abnormality when it is
found is not always straightforward.
Treatment of the Hand
Type I
Type I hypoplasia needs no treatment
since function is virtually normal.
Type II
Type II hypoplasia often requires three
elements in treatment, all of which may be
undertaken at one stage.
Firstly, the tight web space between
the thumb and index finger is released
with skin grafts or the transposition of
skin from an adjacent area.
Secondly, the wobbliness or instab
ility of the middle joint (the metacarpo
phalangeal or MCP joint) of the thumb
is addressed. This is either through
ligament reconstruction or, in more severe
cases, by making this joint rigid. The
latter may sound drastic, however the
main functional ability of the thumb
comes from the joint at the base of the
thumb and rigidity at even one or even
both of the other joints is functionally
preferable to floppiness or instability.
Finally, the abnormality in the
muscles of the thumb is addressed.
Many surgeons believe that these
abnormalities should be corrected at
the same time in order to prevent
112
The TOF Child
the
web space. The position of the thumb may
are mild at birth. The decision whether or
Type II hypoplasia often requires three
elements in treatment, all of which may be
Firstly, the tight web space between
the thumb and index finger is released
with skin grafts or the transposition of
Secondly, the wobbliness or instab-
ility of the middle joint (the metacarpo-
phalangeal or MCP joint) of the thumb
ligament reconstruction or, in more severe
Above:
Type III thumb.
Above:
Type IV thumb.
Above:
Type V thumb.
The TOF Child
recurrence of the tightness in the first
web space, which might otherwise occur if
the thumb is not sufficiently active to
'exercise' the skin on the hand.
Some surgeons prefer to rearrange
the long tendons of the forearm to
allow the thumb to be moved away from
the palm or to rotate across the palm while
other surgeons prefer to move one of the
small muscles from the little finger
border of the hand in order to provide this
function. On rare occasions, it is
necessary to restore the long tendon that
allows the thumb to bend; this is usually
best left until such time as the child is
able to cooperate with rehabilitation.
Types III, IV and V
In Type III the widespread nature of
the abnormalities is such that one has
to consider complete reconstruction of
the thumb.
Type IV and V patients have no
option other than to do nothing or
undergo such surgery.
Many surgeons have sought to
avoid this and have subdivided Type
III in order to identify those cases in
which the existing inadequate thumb
can be preserved in large part and
elements of the skeleton and the joints
replaced by microsurgical transplant-
ation from the foot. Some of these
pioneering works have proved successful
but it is still to early to make widespread
recommendations about these techniques.
Instead, in appropriate cases,
reconstruction of a very functional and
aesthetically pleasing thumb can be
completed by transferring the index
finger. This operation is known as
pollicisation. It may seem extraordinarily
severe because it sacrifices an apparently
normal index finger in order to construct a
thumb,
however i t is an operation which is
very well understood and has proven
to be very reliable and reproducible. In
cases where the index finger is normal
and functional, a very good thumb results;
the choice is therefore between a four
fingered hand without a thumb or a
three fingered hand with a thumb. So
crucial is the function of the thumb to
the function of the hand as a whole that
most surgeons believe that the child
is better served by the creation of a three
fingered hand with a thumb.
Cosmetically most surgeons also find
such a hand less bizarre and noticeable.
Above:
The Type IV case shown on the previous
page, following pollicisation.
Timing of Surgery
Much has been written about the pros
and cons of timing of the various stages
of surgery. What is not in doubt in
syndromes such as VACTERL and Holt-
Oram anomaly is that life-threatening
conditions take precedence.
Under the best possible conditions many
surgeons prefer to complete radialisation or
centralisation of the wrist and straightening
of the forearm (assuming a normal elbow)
by six to eight months of age, leaving
the child free to have a pollicisation
procedure if appropriate (see below) at
about one year to eighteen months of age.
113
The TOF Child
Where the wrist does not need
centralisation or radialisation, the
pollicisation procedure may be
brought forward.
Some surgeons have indicated that there
is a penalty for delaying this surgical
timetable. Others increasingly feel that
such an opinion is based on assumptions
rather than fact and that only the
progressive deformity of the wrist needs to
be treated early, after which the non-
progressive abnormality of the thumb
may be treated at a more c o n s i d e r e d
t i m e p r o v i d e d i t i s completed well
before school years.
Contraindications to pollicisation
Not all children with a Type III or
worse thumb should undergo a
pollicisation procedure.
Parental attitudes should be considered
carefully and their wishes taken into
account. Children with a normal hand on
one side have a less imperative need for
reconstruction on the opposite side.
This is not to say that reconstruction
should not be offered to those children;
many experienced surgeons feel that cases
in which only one hand i s
a f f ec ted benefit just as much from
surgery as those in which two hands are
affected.
More importantly however, the
condition of the index finger and the
other fingers is pivotal in deciding
whether to reconstruct the thumb.
The final functional quality of the
pollicised thumb is determined mostly
by the ini t ia l qual i ty of the index
finger. In some cases the fingers are
incapable of independent movement, have
abnormalities including duplications or
deviations within the structure of the
finger, or have stiff joints and poor
movement. In these cases a poor thumb
will result and pollicisation may not be
indicated.
Some severe anomalies of the forearm
muscles and tendons noted at wrist
surgery may also make the surgeon
decide against pollicisation.
An important relative contraindication is
the 'occasional' or inexperienced
surgeon. Pollicisation is an operation in
which experience is of enormous benefit
since the procedure is quite complex.
Parents may feel reassured by seeing
examples of other cases treated at the
surgical centre.
Pollicisation is contraindicated in
the uncorrected wrist. This is because
the radial border (thumb border) of the
hand in the uncorrected wrist is not the
side which first approaches an object.
Such children almost universally have
a prehension (grasping) pattern which
uses the little finger (which is in their
case the leading edge of the hand) as the
thumb, meaning that objects are usually
held between the little finger and ring
finger. If the wrist is corrected relatively
late in the child's development (perhaps
because of late correction of the elbow)
then the child may have become so
accustomed to this pattern of grasp that
after pollicisation the pattern is hard or
slow to break and the child neglects the
new thumb. The decision whether or not to
offer pollicisation requires experience and
judgement on the part of the surgeon.
Dogmatic opinions about absolute
contraindications to surgery in these cases
are rarely valid and provided a good thumb
is reconstructed following correction of the
wrist and elbow most children of an
appropriate age will revert to using the
thumb rather than the little finger.
Some children make use of both
patterns of prehension; this is perfectly
reasonable since it adds rather than detracts
from the functional repertoire of the hand
and also improving its cosmetic
appearance.
114
The TOF Child
Late Reconstruction and
Secondary Surgery Late reconstruction and secondary
surgery are very common in radial
dysplasia. Each element of the
dysplastic forearm may need further
help as the child grows.
The elbow may need further help
with bending, the forearm may need
straightening or distraction
lengthening later in the growth period,
the wrist may need revision of
centralisation or radialisation and, in about
25% of cases following pollicisation, the
thumb may require muscle transfer to help
with the opposition movement (rolling
across the palm) of the thumb. The latter
movement will never be as fluid and
functional as in the normal thumb but
can be very satisfactorily restored at
pollicisation; even when restoration at
the initial operation is not complete,
subsequent muscle transfers may greatly
improve this movement.
Summary
Radial dysplasia as part of the
VACTERL anomaly may seem the least
of the child's worries at birth when
tracheo-oesophageal fistula, cardiac
anomalies or renal anomalies may be
urgently requiring attention.
After the other anomalies have been
corrected, the radial dysplasia may how-
ever be more of a restriction for a child.
Treatment of this condition is specialised
and is best conducted in a surgical unit
where the whole repertoire of forearm
and hand reconstruction is available.
Staged and repeated operations are often
necessary, in combination with specialised
physiotherapy (much of which can be
done at home) and splinting.
The functional and cosmetic outcomes
are frequently rewarding, however depend
not only the skill, experience and devotion
of the whole team caring for the child
(including the parents) but also the extent
and
severity of the original deformity.
115
The TOF Child
The Role of the Professional;
Who to Ask for Help by Suzanne Goodley SRN SCM, Family Care Sister, Nottingham Neonatal Service.
During pregnancy and maybe before, you
will already be aware of a number of
health professionals. The health
professionals most of us know about are
members of the primary health care team,
which comprises the following (listed in
alphabetical order):-
Community midwife
General practitioner
Health visitor
Practice nurse
When a baby is sick, premature or
requiring surgery and therefore needing
admission to a Neonatal Intensive Care
Unit (NICU), additional professionals
will be involved in the care of the baby.
The following will be added to the above
list (again in alphabetical order, so as not
to indicate any one as being more
important that another):-
Chaplains of various denominations
Dietician
Geneticist
H o s p i t a l m i d w i f e
Liaison health visitor
Neonatal nurse / advanced
neonatal nurse practitioner
Neonatologist or paediatrician
O p h t h a l m o l o g i s t
Paediatric surgeon
P h y s i o t h e r a p i s t
Radiologist
Radiographer
Social worker
Speech therapist
The roles of these medical
professionals are now explained in
more detail.
Community Midwife
The community midwife is trained to
care for mothers and babies pre-natally,
ante-natally and post-natally up to 28
days after delivery. During the course of
the pregnancy parents will have come to
know and trust the midwife; even where
the baby needs to be moved onto a
neonatal unit the midwife should still be
aware of progress and will often remain
in close contact.
General Practitioner (GP)
This is your family doctor who you
should already know. A GP cares for
family health needs from his/her local
practice.
Health Visitor
The health visitor is a qualified nurse
who has had extra training to practise as a
health visitor and is responsible for you
and your children until they reach
school age. Their responsibilities include
helping with health
promotion/preventative medicine,
running well baby clinics, dealing with
social, emotional and environmental
issues, and performing regular child
assessments to monitor a child's progress
with reference to recognised
developmental milestones. Usually the
health visitor will
introduce themselves to a family ante-
natally – if not already known to you
through previous children – but will
otherwise be in contact either soon after
delivery or once the baby is home.
116
The TOF Child
Practice Nurse
A general practitioner usually has at
least one nurse attached to their practice
who is responsible for nursing duties
within the practice and may therefore
be known to many of the patients. The
practice nurse's involvements include well
woman and man clinics, wound dressings,
removal of sutures, immunisations and
innoculations.
Chaplains of Various Denominations
Most hospitals have a chaplain
a t t a c h e d t o t h e m , i f n o t a f u l l
chaplaincy service. They are there to
give spiritual, emotional and practical
support and are always on hand, day or
night. If you wish to have your baby
baptised they will be able to perform a
simple service for you.
Dietician
A dietician is trained in nutrition. Advice
is often sought from them in caring for sick
babies regarding the correct type and
amount of feed to give to enable them to
grow. Some babies have unique
requirements after surgery and therefore
need special diets.
Geneticist
This is a doctor who specialises in
genetics, the study of the genes and
chromosomes in the many cells which
make up each individual. Sometimes
having a baby with an abnormality
such as TOF can have genetic
implications for you and your children,
and therefore you will be referred to one
of these specialists if either you make a
request for this, or if the doctor believes it
to be advisable.
Hospital Midwife
The hospital midwife is trained in
the same way as your community
midwife, but works in a hospital
setting. She will be responsible for the
mother's post-natal care while she is
resident in the hospital. Postnatally a
mother needs to be seen daily by a
midwife for the first ten days, and then
at less frequent intervals for up to twenty-
eight days afterwards, depending on
circumstances.
Liaison Health Visitor
This is a health visitor based within a
hospital whose role is to communicate
with other health professionals. They
will normally inform the health
visitors in the parents' local area that a
baby on their case load is a patient on
a neonatal unit, and then keep them
informed and up to date on the baby's
and family's well being. In some units
they also act as a support person for
the family, instead of the role of a family
care sister.
Neonatal Nurse
The nurses on a neonatal intensive care
unit are trained nurses who have taken
on additional training to be able to
specialise in caring for premature
and sick babies, and are therefore well
qualified to care for your baby.
Most neonatal intensive care units use
what is called a 'named nurse' or `primary
nurse' system. This means that, within a
short time of your baby being admitted
to the unit, a nurse will be named as the
person with some increased responsibility
for their care. Obviously this nurse
cannot be on duty al l the t ime, but
wi l l identi fy the baby's and the
family's needs, write an appropriate
nursing care plan and ensure that this is
carried out, sharing care with other
nurses in her absence. Units work shift
systems which vary from unit to unit,
but you no doubt will soon become
familiar with the system employed.
117
Apart f rom the nurses actual ly
caring for your baby on a day to day basis,
some units have other specialist nurses to
help care for you and your baby during you
stay. These may be introduced as a
'family care sister,' a title which is self
explanatory; the sister cares for the whole
family, encompassing the entire
scenario of having a sick baby, and
all the emotions that entails. Other titles
used for such a nurse might be 'paediatric
community sister' or `liaison neonatal
nurse,' but the aim of them all is the same.
They will sometimes start their relationship
with parents antenatally, introducing
families to a neonatal intensive care unit
prior to delivery, and will follow the whole
family through to discharge home,
ensuring that the transition from a
Above:
Lesley Brown, 1 year, who had a long gap
OA and spent a long time in hospital; the
nurses became very fond of her ...
118
The TOF Child
caring for your baby on a day to day basis,
some units have other specialist nurses to
help care for you and your baby during you
title which is self
explanatory; the sister cares for the whole
be 'paediatric
nurse,' but the aim of them all is the same.
start their relationship
prior to delivery, and will follow the whole
Lesley Brown, 1 year, who had a long gap
OA and spent a long time in hospital; the
stressful neonatal intensive care unit to
the hopefully relaxed atmosphere of
home goes as smoothly as possible.
In some neonatal intensive care
units all babies automatically have
their hearing tested (and it is hoped
that in the near future universal testing
of all babies born will be carried out.)
The person performing this test is
usually another neonatal nurse, who
has undertaken further training to be
able to test babies' hearing. This test is
not harmful to the babies. Special
equipment measures the response
from the auditory (hearing) nerve to
quiet sounds played into their ears.
The test is done when the baby is asleep.
Advanced Neonatal Nurse Practitioner (ANNP or NP)
This is a neonatal nurse who has
several years experience and has
undertaken further extensive training.
This enables them to carry extra
responsibilities, although how these
are expressed varies between units.
Not all units have ANNPs. Most
commonly they are on the unit to give
expert continuing clinical care of babies,
often in a role very similar to that of
the junior doctors.
They may attend deliveries of sick
babies to provide resuscitation, perform
routine baby examinations on the
postnatal word or work on the
neonatal unit where they are able to
provide advanced care for sick babies
often in place of or alongside junior
doctors.
Neonatologist or Paediatrician
A neonatologist is a doctor who
specialises in the new-born infant.
A paediatrician is a doctor who
specialises in all children, from newborn
to adulthood.
There will be a team of doctors on a
Neonatal Unit led by a consultant –
The TOF Child
either a consultant neonatologist of
consultant paediatrician – and they are
in overall charge of the baby's care. They
usually make daily ward rounds and are
often on the unit during normal working
hours and when on call.
Next in line are the senior
registrars/registrars; these are senior
paediatricians who are working towards
becoming a consultant.
The senior house officers (SHOs) are
the junior doctors who, along with the
nurses, are responsible for the day to
day work and running of the unit.
Al though j unior , they a re usual ly
trained in the speciality of neonatology
or paediatrics.
Medical students do not work in
neonatal intensive care units, but may
be present from time to time, just as
observers.
Ophthalmologist
This is a doctor who specialises in
e ye s . B a b i e s t ha t h ave r e q u i r e d
neonatal intensive care and needed
oxygen therapy as part of their treatment
will have their eyes checked routinely by
an ophthalmologist.
Paediatric Surgeon
A paediatric surgeon is a consultant
who specialises in the childhood
conditions which require operations,
and will be the doctor who performs
the surgery on your baby. If the condition
was diagnosed before delivery, hopefully
you will have been introduced and the
condition and operation explained.
Physiotherapist
A person trained in the body's
movement by natural forces, who may
be required to help with your baby's
breathing post-operatively, and also with
general development later.
Radiologist
This is a consultant doctor who
specialises in the interpretation of
radiographs (X-ray pictures) and scans
e.g. ultrasonographs, CAT (Computer
Assisted Tomography) scans, NMR
(Nuclear Magnetic Resonance, sometimes
called MRI or Magnetic Resonance
Imaging) scans, and will probably need
to examine radiographs of your baby
before and after surgery.
Any special scanning procedure
that your baby requires will be
explained to you by a doctor.
Radiographer
This is a person trained to perform
radiographs and ultrasound scans.
Social Worker
Social workers in a neonatal setting
are able to help families with any practical
issues that arise during the hospitalisation
period – such as travel expenses,
accommodation and care of other children
– as well as offering emotional support
and advice on benefits and family
entitlements which may be available.
In some units it is the social worker
who takes on the role of support person
for the family.
Speech Therapist
This person is trained to help with
speech and language development
problems. They are also often very
useful with babies with feeding
problems because they have expertise
in dealing with such difficulties.
Other Medical Professionals
The professions listed above are those
most often involved in neonatal intensive
care, but there may be others who you
may come into contact with. Do not be
afraid to ask who they are and what they
do if you are unsure; they are there for
your benefit, so make use of them!
119
The TOF Child
Parents' Worries JWL Puntis BM DM FRCP FRCPCH, Consultant Paediatric Gastroenterologist,
The General Infirmary at Leeds, also Senior Lecturer in Paediatrics and
Child Health, University of Leeds.
X-rays
Children with OA/TOF often require
repeated X-rays in the early weeks of
life, both before and after surgery. Both
parents and doctors worry about the risk
of X-rays, particularly whether they
will increase the chance of cancer
developing in childhood or later life.
However, these risks are extremely low.
In a recent study of small babies in a
neonatal unit,' many of whom had a
considerable number of X-rays, the
calculated dose of radiation received
was reassuringly small. Even in the child
who had the largest number of X-rays (far
more than most TOFs need), the amount
of radiation was estimated to be
equivalent to three months of the natural
background radiation to which we are
all exposed in our daily life. The increased
risk of cancer in this child was worked out
as being 1 in 60,000.
Despite this low risk, doctors are taught
to carefully consider whether or not any
X-ray examination is really necessary
before making a request, and the
smallest dose of radiation possible is
used. Overall, the benefits of any X-
rays in terns of treating the child far
outweigh any potential radiation risks.
Antibiotics and Anaesthetics
TOF children may also need a number
of anaesthetics and frequent courses of
antibiotics. The situation is very similar
to that of X-rays in that there are risks
involved, but they are very small and
greatly outweighed by the risks of not
having an anaesthetic or failing to treat
an infection.
Once again it is important for doctors to
be certain that a procedure requiring an
anaesthetic is really necessary (usually
there is little doubt about this) or that
there is a bacterial infection demanding
treatment. Children operated on for TOF
will almost certainly be anaesthetised
by a very experienced senior
anaesthetist who has special expertise
in dealing with young children.
The most likely risk from antibiotics
is a reaction to the drug, for example a
rash, or diarrhoea. These usually resolve
when the antibiotic is stopped and are
rarely life threatening.
Parents may worry that children
will 'get used to' antibiotics, so that
their effectiveness wanes over time. This
is not the case, although very frequent
(almost continuous) antibiotic treatment
may encourage the development of
germs in the body that become resistant
to the effects of the antibiotic. This
would be an unusual situation in the
child with TOF who requires intermittent
treatment, and in fact is rarely a problem
in those children with other diseases
such as cystic fibrosis who need
intermittent courses of antibiotics for their
whole life.
References
1. Sutton P.M., Arthur R.J.,
Taylor C., Stringer M.D. (1998).
Ionising radiation from diagnostic
x rays in very low birthweight babies.
Arch Dis Child: 78: F227-F229.
120
The TOF Child
Paediatric Resuscitation David A Needham GIFireE, REMT(p), DipAdEd, Medical Rescue, Nottingham
Every parent or carer of a child worries
about the possibility of their child choking
or being involved in some other mishap
that stops them breathing. Anyone with a
TOF child will almost certainly worry
about this kind of thing even more.
A respiratory arrest (inability to breathe)
catches some TOF parents completely
unawares because they have not been given
the slightest hint from medical staff that
this may occur. Medical staff sometimes
withhold information from the parents of a
sick child in the (often misguided) belief
that the truth will terrify them. If
however the risk of respiratory arrest is
not explained, parents can then find
themselves in a critical situation, the
outcome of which is largely dependent
upon them, for which they have not had
the opportunity to confront in their
minds – let alone train for.
Some TOF children have what is
known as a "floppy trachea" and a few
may need to undergo corrective surgery
for this problem. Whilst these children
will have a slightly increased risk of
choking, it does not automatically follow
that they will definitely have a fatal
choking episode.
The number of children who die of
asphyxia in the UK is very small.
Respiratory arrest in children is usually
recognised quickly and action is taken
before it degenerates into cardiac arrest
as well. Whilst most Accident and
Emergency Department staff are unaware
of the complexities of the TOF child's
problems, and some may not even have
heard of the condition, they are well
trained to deal with the acute
crisis of a non-breathing child, whatever
the cause may be.
Rather than becoming continually fearful
about their TOF child suffering respiratory
arrest – even making themselves ill
with worry – it is far more useful
for parents to channel energy into
working out an action plan so that
everyone knows what action to take if the
occasion should arise. Having some
notion of what to do makes the risk of
respiratory arrest much less of a concern;
parents cope much better once they are
confident about what to do. Many parents
have successfully resuscitated their child;
some have done it more that once. Parents
should realise that the outcome does
not have to be bad; you can make a
difference by preparing now in case the
crisis occurs.
Resuscitation is a skill and skills are best
learned through doing. Most TOF parents
leaving paediatric units will be taught
how to resuscitate their baby using a
mannikin. Failing this, the most
a p p r o p r i a t e w a y t o p r e p a re i s b y
a t t e n d i n g a t r a i n i n g s e s s i o n i n
r e s u s c i t a t i on ( o r ga n i s a t i on s a n d
addresses can be found at the end of this
chapter). Additionally, inside the front
and back covers of this book you will find
charts on resuscitation (one for infants, the
other for children) which you can refer to,
if the need should ever arise.
A lot of misinformation about
resuscitation is often revealed at
resuscitation classes and this needs to be
clarified. The following pages provide
a n s w e r s t o s o me o f t h e c o mmo n
questions that people ask (or sometimes are
too self-conscious to ask).
121
The TOF Child
What should I do if my child
is choking and coughing?
The fact that your child is coughing is a
good sign; it shows that they still have
some control over their airway and they
are trying to eject the foreign matter. In
order to cough, the child has t o t a k e
a i r i n t o t h e i r l u n g s a n d therefore
they are still breathing. However, this
threat to the airway may deteriorate to a
complete obstruction and then cessation
of breathing, so some supervision is
required.
Calmly encourage the child to cough,
incline the child forward so that gravity
is helping the object to fall out of the
mouth. If this does not help, slap the child
on the back between the shoulder blades
with the heel of the hand to try to
dislodge the foreign matter. If the foreign
body still remains stuck, then you must
resort to abdominal or chest thrusts to
dislodge it.
How much air should I blow
into the child when I am performing mouth to mouth?
This obviously depends on the size of
the child or infant.
As a general rule, the amount of air
that you could hold in your mouth with
your cheeks puffed out is about right
for small infants. Larger children will
obviously need more air to ventilate their
lungs and it is important that you look
at the child's chest when blowing into
them. When the chest rises, you have
given enough air.
If you continue to force air in after the
chest has fully risen then you may
cause damage to the lungs, but what is
more likely is that the air will follow
the route of least resistance and end up
in the child's stomach. This may cause
vomiting, or more seriously it may fill
the stomach so full of air that the lungs
cannot expand properly; this would
eventually lead to you not being able
to blow any air into the child's lungs.
Your lungs can easily expel 1.5 litres
of air in one long breath. A child's lungs
will only take 0.5 litres, or possibly
less. Remember this and do not be
over-zealous.
I have heard about mouth to
nose resuscitation; is this more
effective than mouth to mouth?
The short answer is no. Mouth to mouth
and mouth to nose are equally efficient,
but sometimes circumstances make it
difficult for people to get an effective seal
around the child or infant's mouth. In that
case, do not hesitate to use mouth to nose.
In the past, the teaching has been to carry
out mouth to mouth-and-nose when
resuscitating infants. Recent research
shows that mothers do not have a
sufficiently large mouth to form an
adequate seal over both the mouth and
nose of their infant. My advice to people
is to ensure that a good seal is made to
connect with the child or infant's airway,
even if that means only doing mouth to
nose.
When I did a first aid course,
we always checked for a pulse at the neck, and not the
wrist. I have since then heard
that you should check an infant's pulse on the upper
arm. Which is correct?
A pulse can indeed be obtained at all these
sites, however the best to use varies with
the individual situation and what you
are trying to achieve.
Checking for a pulse at the wrist is OK
in some circumstances, but is never
acceptable if someone has stopped
breathing and you need to detect whether
their heart has stopped.
122
The TOF Child
In infants, the pulse in the neck is often
difficult to locate due to their chubby
necks. You may find it easier to detect
a pulse on the inside of the upper arm,
behind the biceps muscle.
NB When measuring the pulse rate in beats
per minute, don't spend a whole minute
doing it! Count the beats for 15 seconds;
double this figure, then double it again
(i.e. multiply it by four; doubling it twice
is easier when under pressure!) to give
the pulse rate for a minute. Practise this
when your child is well and sleeping or
sitting on your knee.
What if I can detect a pulse,
but it is very slow and weak, and my child is unconscious?
Because the pulse is slow and weak,
this means that the heartbeat is slow and
weak. This in turn means that the blood
supply to the brain will not be adequate –
and in fact this is probably the reason
that the child is unconscious.
You must begin chest compressions on
any infant or child who has a pulse rate
of less than 60 per minute. This will
increase the blood output from the heart
and will therefore increase blood flow
to the brain, which is critical to survival.
What should I do if my child stops breathing, but still has a
fast strong pulse?
If you encounter these circumstances,
then you have caught the problem at a very
early stage, before respiratory arrest
deteriorates to cardiac arrest. If you take
the correct action promptly, the outcome of
the episode is likely to be good.
Give mouth to mouth (or mouth to
nose) at a rate of 1 breath every 5 seconds,
and continue to monitor the pulse. If the
pulse stops or drops below 60 per
minute, then commence chest
compressions as well as mouth to mouth.
What if I am not completely
sure that my child has stopped breathing — can I do
harm by beginning mouth to
mouth?
If it is such a fine judgement about
whether a child is breathing or not, then
start mouth to mouth without delay. In
practical terms, you will not cause any
harm. If the child makes respiratory
efforts (i.e. tries to breathe) then you
should try to synchronise your breaths
with this respiratory effort.
I have heard that if someone has not been breathing for 4
minutes, irreversible brain damage will o c c u r . B e a r i n g
t h i s i n m i n d , should I stop
mouth to mouth after four minutes if the person is not
breathing for themselves?
Absolutely not! You are providing the
person with oxygen through your mouth
to mouth ventilation, and you are
circulating this oxygen round the body in
the bloodstream by the chest
compressions you are doing. You are
the only thing that is keeping their brain
alive. By doing mouth to mouth (and
chest compressions when necessary)
you are "buying time" for the
ambulance crew to arrive and commence
advanced emergency care techniques. Do
not stop!
Resuscitation courses
Medical Rescue
Contact David Needham; address
available from the TOFS office.
St John Ambulance
Contact the local division or the
County Office. In Scotland, contact the
Director General in Glasgow or your local
Committee Secretary.
British Red Cross
Contact your local branch headquarters. 123
The TOF Child
Glossary
Vicki Martin BSc BVSc and Sue Goodley, Nottingham Neonatal Service.
amniocentesis: a test whereby amnotic fluid
is sampled by insertion of a needle. Tests
on this fluid can give information about the
baby's health.
amniotic fluid: water around the baby in the
womb.
anastomosis: 'join' — as in oesophageal
anastomosis, where the two ends of the
oesophagus are stitched together in
oesophageal atresia.
antibiotic: medication used to treat bacterial
infections.
anus: external opening of the rectum
(backpassage)... anal: relating to the anus.
aortopexy: surgical procedure used in the
management of severe tracheomalacia, in
which the aorta (a major artery which lies in
front of the trachea) is anchored to the sternum
(breast bone). This has the effect of opening
up the trachea.
asthma: respiratory disease often associated
with difficulty in breathing and a cough.
atresia: term taken from ancient Greek, meaning 'no
passage / no way through.' For example, in
oesophageal atresia there is a break in the
continuity of the oesophagus.
bacteria: cellular agent causing disease...
distinct from a virus.
barium: is often used as a contrast agent in
radiography: it shows up on radiographs (`X-
rays')... a barium meal can be swallowed to
show up the gastro-intestinal tract... a barium swallow shows up the oesophagus... a barium study is a sequence of radiographs taken
over a period of time to show the passage of
material through the gastro-intestinal tract.
bowel: intestines... the small bowel is the small
intestine (upper intestinal tract, that which
follows after the stomach): the large bowel is the large intestine (lower intestinal tract,
between the small intestine and the rectum).
bronchi, bronchiole: the branching airways
after the splitting of the trachea (bronchi
then bronchioles).
bronchoscopy: endoscopic examination of
the airways.
cardiac: related to the heart.
CHARGE: acronym describing a group of
anomalies which are sometimes associated
with TOF/OA. The letters stand for: Coloboma
(defects affecting the pupil of the eye), Heart
disease, Atresia of the choanae (the passages at
the back of the nose, Retarded growth, Genital
hypoplasia (underdevelopment of the genital
organs) and Ear anomalies/deafness.
congenital: deformities or diseases which are
either present at birth or, having been transmitted
direct from parents to offspring, become obvious
some time after birth.
contra-indication: term which relates to a
medicine or procedure... a contra-indication
describes a situation in which that medicine
or procedure should not be administered or
carried out. For example, asthma is a contra-
indication for the drug ibuprofen.
CPAP: Continuous Positive Airway Pressure
—the application of a continuous positive
pressure to the airways, used (for example) in
the management of tracheomalacia to keep the
airways open.
dilatation: term used to describe a procedure
undertaken to widen a narrowing (stricture).
distal: the end furthest away (cf. proximal:
the closest end).
duodenum: the first part of the small
i n t e s t i n e , j u s t a f t e r t h e s t o m a c h .
electrolytes : medical usage o f th i s t erm
relates to the salts found in body fluids, for example
sodium, potassium, chloride, calcium etc...
electrolyte imbalance: abnormality of the
relative concentrations of body salts.
endoscope: medical instrument with a tube-
like structure, through which the inside of the
body can be examined. Endoscopes can be
rigid or flexible tubes incorporating either
fibreoptic or video technologies: the image is
seen either via an eyepiece or on a television
screen. -
fistula: term from the Latin meaning `pipe...'
an abnormal connection running either between
two tubes or between a tube and a surface. In
tracheo-oesophageal fistula it runs between the
trachea and the oesophagus.
124
The TOF Child
fluids: medical usage of this term relates to
the administration of fluids into the blood stream, via an intravenous drip or pump.
foreign body: medical usage of this term
relates to the abnormal presence of a
particle or object... an oesophageal foreign
body is a swallowed item which is
abnormally present in the oesophagus.
gastric: relating to the stomach.
gastrostomy: surgically-created connection
between the inside of the stomach and the
body wall.
gastro-oesophageal reflux: the involuntary
return of gastric (stomach) contents to the
lower end of the oesophagus.
gullet (see oesophagus).
haematology and biochemistry: the study of
the blood components... haematology relates
to the cellular content, biochemistry to the
chemical components.
hypoplasia: decreased (less-than-normal)
growth.
immunity: resistance to disease... immune
system: the body's armoury of defences
producing resistance to disease. immunisation: the artificial creation of immunity against specific diseases.
incision: surgical cut.
inflammation: the body's reaction to injury
(caused by e.g. trauma or disease). anti-
infiammatories: drugs given to reduce
inflammation.
inpatient: patient receiving medical attention
while resident in the hospital (cf. outpatient:
patient seen during a short visit to hospital).
kyphosis: abnormal curvature of the spine to
give the appearance of a 'rounded back.'
ligate: surgical term — to 'tie off' with a knot.
medical: non-surgical management of disease
(vs. surgical, involving physical intervention
i.e. an operative procedure).
mucus: moist fluid which is found (for
example) in the normal airways where it
helps to trap small particles e.g. dust.
nebuliser: device which administers a drug to
the airways.
neonatal: newly born.
oesophagus: gullet, the tube taking food from
the mouth to the stomach.
oesophageal incoordination: lack of
coordination of the muscular activity of the
oesophagus.
oesophageal substitution procedure:
replacement of (a part of) the oesophagus
with another part of the gastro-intestinal
tract.
oesophagostomy: surgical creation of a
connection between the oesophagus and the
body wall (skin)... cervical oesophagostomy:
connection of the oesophagus to the neck.
oral: relating to the mouth.
outpatient: patient seen during a short visit to
the hospital (cf. inpatient: patient receiving
medical attention whilst resident in hospital).
p a e d i a t r i c : r e l a t i n g t o c h i l d r e n .
paediatrician: doctor who has specialised in
the treatment of childhood disorders.
peristalsis: wave of muscular activity, in
e.g. the oesophagus or intestine, which
transports the contents along the gut. pH:
acidity... pH monitoring: the monitoring of
pH (performed in the diagnosis of gastro-
oesophageal reflux, in which case the monitor is
positioned at the base of the oesophagus, j us t
b e fo r e i t e n t e r s t h e s t o ma c h) .
pneumonia: inflammation of the lungs.
aspiration pneumonia is caused by the
entrance of fluids into the respiratory tract.
pollicization: the surgical reconstruction of
thumb by transferring the index finger.
polyhydramnios: condition in which an
abnormally large amount of fluid is present in
the womb: often associated with TOF/OA in the
baby. Also known as simply 'hydramnios.'
prenatal: before birth.
primary health care team: the team of medical
professionals who are involved in local health
care... includes (e.g.) the general practitioner,
practice nurse, health visitor etc.
progeny: offspring (sons and daughters).
proximal: the closest end (cf. distal: the end
furthest away).
radiograph: an X-ray image... radiography:
the taking of X-rays.
radiology: the study/interpretation of
radiographs.
radial dysplasia/aplasia: abnormality of
development of the radius, a bone in the
forearm: this is a condition affecting some
VACTERL children.
rectum: backpassage, the last part of the
gastro-intestinal tract.
reflux (see gastro-oesophageal reflux).
renal: relating to the kidneys.
125
The TOF Child
respiration: breathing... respirator: a machine
which 'breathes for' the patient...
respiratory distress syndrome: complex
condition affecting newborn babies, in which
respiration does not occur normally.
resuscitation: life-saving procedure(s) involving
bringing an unconscious person back to
consciousness by stimulation of the respiratory
and/or circulatory systems.
scoliosis: abnormal curvature of the spine in a side-
to-side direction.
sham feeding: procedure carried out with
babies who have not received an anastomosis
(join-up operation) on their oesophagus, but
have received a cervical oesophagostomy and
gastrostomy. Food is given by mouth, but exits
at the neck through an oesophagostomy (a
surgically created opening of the upper
oesophagus at the neck). Simultaneously,
food is given straight into the stomach via
the gastrostomy tube (a surgically-created
opening of the stomach at the body wall). In
this way the child learns to eat and swallow,
and to associate this with the feeling of
`being ful l , ' in preparat ion for later l i fe
when their mouth has been surgically reconnected
to their stomach and normal feeding can begin.
stricture: narrowing... most often - but not
always — referring to a narrowing of the
oesophagus at the anastomosis (join-up) site.
Can also be congenital (present at birth) or
subsequent to gastro-oesophageal reflux.
sutures: surgical stitches.
symptom: the problem reported by the patient.
TOF cough: characteristic 'barking' cough
often exhibited by TOF children, caused by
a degree of tracheomalacia.
trachea: windpipe... the main airway from
the mouth/nose to the branching bronchi
and bronchioles which lead into the lungs.
tracheomalacia: 'floppiness' of the trachea.
tracheopexy: surgical procedure used in the
management of tracheomalacia, in which the
trachea is anchored to the sternum (breast
bone) in order to open up the airway.
tracheostomy: the surgical creation of a
connection between the trachea and the
skin at the neck, and the insertion of a tube
into the trachea to preserve a clear airway.
Used in the management of severe
tracheomalacia.
transanastomotic tube: a tube which is
placed in the oesophagus and runs across the
site of an anastomosis (join-up) into the stomach.
This enables the baby to be fed milk while the
operation site heals.
ulna: bone in the forearm: the upper end of
the ulna is the so-called 'funny bone.'
ultrasonography: diagnostic imaging procedure
in which an ultrasound scan is performed which
produces 'real-time' images of the internal
body structures using sound waves.
VATER: acronym for a group of disorders
often occurring together. The letters stand for
Vertebral (spine), Anal (backpassage), Tracheal,
Esophageal (from the American spelling; the UK
spelling is `oesophageal'), Renal (related to
the kidney) and Radial (the radius bone in the
forearm). VACTERL: more correct/up-to-date
acronym, in place of VATER (above): the extra
letters in VACTERL stand for Cardiac (affecting
the heart) and Limb (since there are o ther
l imb abnormali t ies other than those
involving only the radius).
ventricular septal defect (VSD): defect in
t h e w a l l d i v i d i n g t h e l e f t a n d r i g h t
ventricles (the lower chambers in the heart)
m e a n i n g t h a t o x y g e n a t e d a n d d e -
oxygenated blood mixes and the transportation
of oxygen from the lungs to the tissues is
inefficient. The circulation in the lungs is also
subject to abnormally high pressures, which can be
damaging.
ventilation: medical usage generally refers to
mechanical ventilation, in which a machine
`breathes' for the patient (the machine is
called a ventilator, which is the same as a
respirator).
vertebra: each of the bones which makes up
the spine is called a vertebra.
virus: infective agent causing disease... distinct
from a bacteria and not susceptible to antibiotics.
vital functions: term often used to refer to the
basic bodily functions monitored during
patient care, e.g. breathing, circulation and
temperature.
vomiting: 'being sick.'
VSD (see ventricular septal defect).
weaning: introducing solid food as well as
milk into the baby's diet.
windpipe (see trachea).
X-ray (see radiograph).
126
The TOF Child
Index A acid lowering drugs 68 advanced neonatal
nurse practitioner 118 Aerobec 79 Aerolin 79 alpha feto protein 10 amniocentesis 10 anaesthetics 120 anastomosis 22 anlage 107 anorectal atresia 100 antibiotics 77, 120
for renal anomalies 101 antireflux medicines 68 antireflux surgery 69 aortopexy 77 aspiration pneumonia 32 asthma 78 atresia. See oesophageal atresia
Atrovent 79
B Barrett's oesophagitis 29 B e c k , J M 3 7 B e c o t i d e 7 9 Bland , David 5 b l u e d o ' s 4 0 bonding 16 Bricanyl 79 bronchodilators 79 Brown, Leslie 60, 118 Buckler Tanner chart 82 B u i c k , R G 3 0 Butcher, Eleanor 32
C cardiac abnormalities 100 Carobel 68 centralisation 109 cervical oesophagostomy 26 chaplain 117
C H A R G E 1 4 chest drain 24, 33 chest infections 47, 77, 95 Chetcuti, PAJ 82, 95 C i m e t i d i n e 6 8 Cisapride 68 Clamp, Janita 6 Clamp-Gray, Freddie 59 cleft lip 98
club hand 107 colon interposition 27 colostomy 100 community midwife 116
Dickson, JAS 7 dietician 117 d i l a t a t i o n 6 3 , 9 5 distraction lengthening 109 D o b b s , B e n 2 0 Dodd, Emma Louise 29 d o m p e r i d o n e 6 8 Downs syndrome 49 dumping 28, 70
E ear abnormalities 98 education 95 Edward's syndrome 49, 99 endoscopy
for diagnosis of OA 12 for diagnosis of reflux 67 for diagnosis of stricture 62 for diagnosis of TOF 12 for stricture 63 for tracheomalacia 76
exercise 95
F feed thickeners 68 feeding 52-57
breast feeding 52 oral feeding 39 post-operatively 33 problems 54 reluctance to feed 54 sha m feed in g 4 4 solid foods 52 troublesome foods 55 tube feeding 40 weaning 52
Firth, Helen 49 fistula. See tracheo-oesophageal fistula
friends 91
G gas bloat 70
gas tr ic t ransposi t ion 28 gastric tube oesophagoplasty 28 gastro-oesophageal reflux
35, 40, 44, 55, 64, 67, 72 diagnosis 66-67 surgery 69 symptoms 65
gastrostomy 24, 41, 69
care of 43 for reflux 69
Gaviscon 68 general practitioner 116 genetic counselling 49 geneticist 117
genital anomalies 98
Gibson, Thomas 7
Goodley, Sue 6, 116
Gordon, Ben 104
grandparents 87
growth 82-85
growth hormone 83
H haematemesis 65
health visitor 116
Holt-Oram anomaly 113
h o r s e s h o e k i d n e y 1 0 1
hospital liaison visitor 39, 85
hospital midwife 117
hydramnios. See polyhydramnios
hypoplastic kidneys 101
I
imperforate anus 100
Intal 79
Intensive Care Unit 30
Intensive Therapy Unit 30
ipratropium 79
J
jaundice 35
jejunostomy 29, 42
K Kapila, L 98
Kaufman syndrome 98
Kay, Simon PJ 106
Kluth, Dietrich 8
kyphosis 99
L Lander, AD 30
laryngeal cleft 8 Lawton, Sandra Kay 97 Lee, R 98 liaison health visitor 117
limb abnormalities 103
Ludman, Lorraine 15
M MacFadyen, Una M 71 Martin, Vicki 4 Mayo, Jacob 31, 35
medications 46 MedicAlert 6 Micolaud, Christine 97
milk scans 67
monitoring 30
motility drugs 68
Motilium 68
127
The TOF Child
N
nasogastric tube 41 care of 42
Needham, David A 121 neonatal nurse 117 neonatologist 118 Nestargel 68 Nissen fundoplication 69
0
oesophageal atresia 8 conditions occurring with 14 diagnosis 12 long-gap 24 pre-natal diagnosis 10, 18 with lower pouch fistula 8 with upper pouch fistula 8 without fistula 8
oesophageal incoordination 55 oesophageal obstruction 55, 62 oesophagostomy. See cervical
oesophagostomy
ophthalmologist 119
P
paediatric surgeon 119 paediatrician 118 Palmer, Craig 80 parents 15, 86 Parkinson, Glenys 86 pelvic kidney 101 pH Study 66 physiotherapist 119 physiotherapy 47
for radial dysplasia 108 PIAVA 98 Pierce, Anthony 40 PIV 98 play therapy 89 pollicisation 113 polycystic kidney 101 polyhydramnios 10, 51 Pouncey, Benjamin 5 practice nurse 117 Prepulsid 68 preventers 79 Pulmicort 79
Puntis, JWL 52, 120
R
radial dysplasias 106
the hand anomaly 111-112 treatment of the elbow 108 treatment of the forearm 107-111 treatment of the hand 112 treatment of the wrist 108-111
radialisation 110 radiographer 119 radiography
for OA 12 for reflux 66 for stricture 62, 64 for tracheomalacia 76
r a d i o l o g i s t 1 1 9 r a n i t i d i n e 6 8 r e g i s t r a r 1 1 9 r e l i e v e r s 7 9 r e n a l a n o m a l i e s 1 0 1 respiratory arrest 121 respiratory distress syndrome 21 respiratory function tests 76 resuscitation 38, 121
S
salmeterol 79 Sancto, Hannah and Rebecca 51 Say-Gerald Syndrome 98 school 92, 96 scoliosis 99 senior house officer 119 Serevent 79 Seymour, Penny 37 Shore, Celia 52 siblings 89 single umbilical artery 98 s o c i a l w o r k e r 1 1 9 sodium cromoglycate 79 Special Care Baby Unit 30 speech therapist 41, 119 S p e n d e r , K r i s 3 4 Spitz, Prof L 21, 26 Sprigg, Alan 11 stricture 55, 61
anastomotic 61 and reflux 61 congenital 61 diagnosis 62 surgery 64 symptoms of 61 treatment 63-64
Stringer, Mark D 61 surgery for TOF/OA 21-25
discharge from hospital 37 follow up after 48
leaks after 34 long term outcome 95-96
oesophageal substitution p r o c e d u r e s 2 6 post-operative care 30-35 pre-operative management 21 primary anastomosis 22 surgical outreach nurse 39
T
Tagamet 68
Thal fundoplication 69 theophylline 79 Thick and Easy 68 Thixo-D 68 thumb abnormali t ies 98 TOF cough 45 , 73 , 95 total parenteral nutrition 33 tracheo-oesophageal fistula 8
conditions occurring with 14 diagnosis 12 double fistula 8 H fistula 8 without atresia 8
tracheomalacia 34, 45, 72 tests for 76
treatment 77 tracheopexy 77 tracheostomy 77 transanastomotic tube 24, 31 transfer to another hospital
14, 15, 21
Treacher-Collins syndrome 49 Trump, Dorothy 49
U
Uniphyllin 79
V
vaccinations 37 VACTERL 98-104
antenatal diagnosis 99 VATER 98 ventilator 32 Ventolin 79 Ventricular septal defect
(VSD) 100 vertebral abnormalities 99 vomiting 65, 95
w Wharfe, James 57, 85 wheeze 78, 95 Wood, Andrew 5
X
X-rays 120
z
Zantac 68
128