apil conference 2012
TRANSCRIPT
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High alimentary tract obstruction
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Topics
Development of GIT oesophageal atresia oesophageal stricture gastro-oesophageal reflux gastrostomy diaphragmatic hernia pyloric stenosis abdominal wall defects intestinal atresia intestinal perforation (NAI) X
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Overview
• What they are• Why they occur• How they present - signs and symptoms• Diagnosis• Treatment
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Development of the Alimentary Tract
alimentary tract 10 -12m
oral cavity
Anus
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Development of the Alimentary Tract
alimentary tract 10 -12m
oral cavityoesophagus 0.5m
stomach 0.3m
small intestine 7mduodenumjejunumileum
large intestine 1.5mcaecumcolon
rectum 0.1
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Development of the Alimentary Tract
stomach
duodenum
small intestinejejunum / ileum
oesophagus
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Development of the Alimentary Tract
• Intestinal tract– tube– runs from mouth to anus
• Oesophagus & Trachea are formed from the proximal gut– runs through chest cavity
mouth
anus
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Development of the Alimentary Tract
• Intestinal tract– tube– runs from mouth to anus
• Oesophagus & Trachea are formed from the proximal gut– runs through chest cavity
• Stomach distends asymmetrically– rotates anticlockwise
anterior posterior
right left
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Development of the Gastro-intestinal Tract (GIT)
• Intestinal tract– tube– runs from mouth to anus
• Oesophagus & Trachea are formed from the proximal gut– runs through chest cavity
• Stomach distends asymmetrically– rotates anticlockwise
• Small & Large intestine elongate– herniates through umbilicus
– rotate anticlockwise– returns to the abdomen
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Oesophageal Atresia
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oesophageal atresia
• oesophageal • esophagus (american)• gullet
➜ pharynx to stomach
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What is Oesophageal Atresia?
What is Oesophageal Atresia?• the proximal and distal parts of the oesophagus do not communicate
What is Tracheo-oesophageal Fistula?• a communication between the oesophagus and trachea
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Aetiology
INCIDENCE1 in 4000 live births
• Genetic factors • Vitamen deficiency• Drug and alcohol exposure• Viral and chemical influences• External physical events
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Anatomy
• anterior thoracic wall removed
• mediastinum– heart & great vessels– trachea bronchi– oesophagus
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Anatomy
• mediastinum– heart & great vessels
– trachea / bronchi
– oesophagus
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Pathogenesis
• Intestinal tract– tube– runs from mouth to anus
• Oesophagus & Trachea are formed from the proximal gut– runs through chest cavity
mouth
anus
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Pathogenesis
• Trachea & Oesophagus are formed from the proximal gut in upper chest
• 4th week of intrauterine growth
• Lateral mesodermal ridges form in the proximal gut
• Fuse in the midline separating the Oesophagus from Trachea
mouth
anus
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Pathogenesis
➜ Lateral mesodermal ridges form in the proximal gut
➜ Fuse in the midline separating the Oesophagus from Trachea
oesophagus trachea
This process fails to occur or occurs incompletely
This process fails to occur or
occurs incompletely
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Pathogenesis
• Trachea & Oesophagus are formed from the foregut
• 4th week of intrauterine growth• Lateral mesodermal ridges form in the
proximal foregut• Fuse in the midline separating the
Oesophagus from Trachea• Occurs before D34 probably D26• This process fails to occur or occurs
incompletely
ALSO abnormal• Trachea
• deficient cartilage rings• floppy trachea• respiratory difficulty• Tracheomalacia
• Oesophagus• proximal oesophagus
• very dilated • thick wall
• dysmotility• reflux
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Classification
5 different types and combinations
OA with Distal TOF Pure OA without a fistula ‘H’ Type without OA
OA; proximal & distal fistula OA with proximal fistula
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Classification
5 different types and combinations
• OA with Distal TOF 84%
• Isolated (Pure) OA without a fistula 8%
• ‘H’ Type without OA 4%
• OA with both proximal & distal fistula 3%
• OA with proximal fistula 1%
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Classification
In Oesophageal Atresia with distal TOF
• Proximal oesophagus blind ending dilated thick (hypertrophied) walled
• Distal segment
small diameter thin walled extends a variable distance above the diaphragm usually just above carina
OA with Distal TOF
85%
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Classification
In Pure Oesophageal Atresia without a fistula
• Proximal oesophagus blind ending dilated thick (hypertrophied) walled
• Distal segment
no fistula no air in stomach usually just above diaphragm long gap Primary repair not possible difficult repair
Pure OA without a fistula
8%
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Classification
In ‘H’ Type TOF
• NO Atresia
Can feed diagnosed later easier repair better prognosis
4%
‘H’ Type without OA
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Clinical Features & Diagnosis
Defect occurs 4th week of intrauterine growth
• Antenatal Diagnosis ?
not usually diagnosed antenatally occasionally dilated upper pouch seen
most common type – pure oesophageal atresia absence of a gastric bubble
Polyhydramnios should alertPolyhydramnios
• 1% ‘normal’ pregnancies•35 - 85 % TOF pregnancies
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Clinical Features & Diagnosis
• Postnatinatal Diagnosis
Usually term baby clinical suspicion – polyhydramnios drooling / excessive salivation ‘Mucosy’ – not coping with saliva On feeding – inability to swallow/not coping with feeds Pass a Naso-gastric tube. It must be a good size reasonably thick and rigid (10 FG) It will get stuck at about 10 cms (or curl up in upper pouch) Plain abdominal film. – tube seen in upper thorax AP and lateral
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Clinical Features & Diagnosis
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Clinical Features & Diagnosis
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Clinical Features & Diagnosis
• Postnatal Diagnosis
include abdomen confirm air in stomach
• not isolated OA
and small bowel • not duodenal atresia
• Barium rarely required– Risk of aspiration
• Endoscopic examination of trachea +/- upper pouch– exclude upper pouch TOF– identify position of upper pouch fistula
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Clinical Features & Diagnosis
• Postnatal Diagnosis
• suspected – other associated abnormalities
• occasionally late diagnosisfed
• orally• Naso-gastric tube
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Treatment
Initial Treatment• Arrange transfer to Paediatric Surgical Unit• Do not feed• Intravenous fluids• consider associated abnormalities• Nurse head flat / head down • Right lateral position• Continuous suction / intermittent aspiration of upper pouch
– Replocle Tube• Ventilate only if required
– Care not to distend stomach• Consent• Reassure mother – not her fault
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Treatment
Definitive Treatment• Surgical
– Difficult– Oesophagus inaccessible– Posterior mediastinum– behind the heart and lungs
• Oesophageal Atresia and Tracheo-oesophageal Fistula– Primary repair
• Isolated Oesophageal Atresia– Ends too far apart– Primary repair not possible– gastrostomy tube– Delayed primary repair– Different options– Oesophageal substitute
Detailed discussion of surgical issuesbeyond scope of this presentation
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Outlook
Surgical complications
• Anastomotic leak– May close spontaneously– Major early leak – May need re-repair
• Anastomotic Stricture– dilatation
• Gastro-oesophageal Reflux– drugs
• Tracheomalacia– occassionally operation– surgery
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Oesophageal Stricture
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Oesophageal Stricture
A narrowing of the wall of the oesophagus
Due to • Gastro-oesophageal Reflux• Oesophageal Atresia • Ingestion of Caustic• Congenital oesophageal stricture – very rare
Treatment• Treat the cause• Oesophageal dilatation• Resect Stricture• Replace oesophagus
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Oesophageal Stricture
Reflux stricture
Repeated dilatations
Rigid dilators
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Oesophageal Stricture
Reflux stricture
Repeated dilatations
Rigid dilators
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Oesophageal Stricture
Reflux stricture
Repeated dilatations
Rigid dilators
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Oesophageal Stricture
Reflux stricture
Repeated dilatations
Rigid dilators
Balloon dilators
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Gastro-oesophageal Reflux
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Gastro-oesophageal Reflux
Gastro-oesophageal reflux disease acid reflux
a chronic condition due to inflammation of the oesophagus caused by stomach acid coming up from the stomach into the oesophagus
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Gastro-oesophageal Reflux
Gastro-oesophageal reflux disease acid reflux
Gastro-oesophageal junction Hiatus hernia
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Gastro-oesophageal Reflux
Gastro-oesophageal reflux disease acid reflux
Gastro-oesophageal junction Hiatus hernia
Symptoms• Pain• Vomiting
Complications• Stricture• Aspiration
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Gastro-oesophageal Reflux
Gastro-oesophageal reflux disease
InvestigationBarium X-rayspH studyendoscopy
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Gastro-oesophageal Reflux
TreatmentMedicationSurgery– Anti-reflux surgery
Gastro-oesophageal junction Hiatus hernia
Anti-reflux procedure
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Gastrostomy
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Gastrostomy
➜ Opening into the stomach usually using a tube
➜ Initially performed more than 100 years ago, remains in use➜ Gauderer performed the first percutaneous endoscopic gastrostomy (PEG) in 1980
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Gastrostomy
➜ An opening into the stomach➜ Using a tube➜ permanent or long term➜ open operation➜ inserted with gastroscope➜ inserted with laperoscope
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Gastrostomy
➜ Reasons for gastrostomy
➜ Inability to swallow➜ Inability to consume adequate oral intake
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Gastrostomy
➜ Conditions requiring gastrostomy
➜ 365 children
➜ CNS lesions: 214➜ Inflammatory bowel disease: 25➜ Renal disease: 19➜ Cystic fibrosis: 15➜ Metabolic conditions: 13➜ Oropharyngeal/tracheal: 12➜ Others: 67
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Gastrostomy
➜ Usually placed endoscopically➜ General Anaesthetic
➜ Need a good Assistant
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Digital Indentation
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Complications
➜ Death➜ tension pneumoperitoneum ➜ bleeding➜ gastro-colic fistula➜ colo-cutaneous fistula➜ small bowel obstruction➜ perforation of the stomach➜ duodenal haematoma➜ peritonism➜ peritonitis
➜ pharyngeal injur y➜ oesophageal injury➜ tract infection➜ necrotising faciatis➜ surgical emphysema➜ catheter migration➜ catheter leakage
➜ up to 50% minor complications➜ up to 12% mod/severe complications
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Congenital Diaphragmatic Hernia
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What is Congenital Diaphragmatic Hernia
Congenital Diaphragmatic Hernia
is a defect or hole in the diaphragm that allows the abdominal contents to move into the chest cavity.
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What is Congenital Diaphragmatic Hernia
Congenital Diaphragmatic Hernia
is a defect or hole in the diaphragm that allows the abdominal contents to move into the chest cavity.
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What is Congenital Diaphragmatic Hernia
Congenital Diaphragmatic Hernia
Bochdalek HerniaDefect in Postero-lateral Diaphragm
Inferior vena cava
Oesophagus
Aorta
Postero-lateral defectBochdalek Hernia
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Epidemiology
Bochdalek HerniaDefect in Postero-lateral Diaphragm
1 in 3000 births 8% of all major congenital abnormalitiesrisk of recurrence 2% (nonsyndromic)Familial is rare (< 2% of all cases),
Mortalityhidden mortality – in utero 40% (1/3 terminated)
60% live born- high 50% live births
Present at birth• late presentors
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Pathophysiology
Development of the Diaphragm• Complex• 4th to 6th week of foetal life• complete partition of chest and abdominal cavities• Fusion
– Septum transversum– pleuro-peritoneal membranes– dorsal mesentery of oesophagus– body wall
• Right side closes slightly earlier than Left• The diaphragm separates the abdominal viscera
from the thoracic organs
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Pathophysiology
Development of the Diaphragm• Complex• 4th to 6th week of foetal life• complete partition of chest and abdominal cavities• Fusion
– Septum transversum– pleuro-peritoneal membranes– dorsal mesentery of oesophagus– body wall
• Right side closes slightly earlier than Left• The diaphragm separates the abdominal viscera
from the thoracic organs
Bochdalek Hernia – abdominal viscera in the chest cavity
Pulmonary hypoplasiaPulmonary hypertension
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Clinical - Antenatal Period
50% antenatal diagnosis
should prompt detailed foetal scanassociated abnormalities
Stomach
Heart
headchestabdomen
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Clinical - At Birth
Prognostic indicators
• Prenatal diagnosis
• Apgar scores
• Birth weight & maturity
• Side of lesion
• Presence of stomach in chest
• Other lesions
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Clinical - Postnatal
• 50% undiagnosed at birth
• Present with Respiratory Distress
• Scaphoid Abdomen
• Mediastinal Shift– trachea– heart
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Diagnosis
Chest X-ray
• absent diaphragmatic outline
• Loops of bowel in chest
• NG tube passed
Left Diaphragmatic
Hernia
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Diagnosis
Chest X-ray
• absent diaphragmatic outline
• Loops of bowel in chest
• NG tube passed
➜ mediastinal shift
LEFTMediastinal shift as
indicated by position of nasogastric tube
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Diagnosis
Chest X-ray
• absent diaphragmatic outline
• Loops of bowel in chest
• NG tube passed
➜ mediastinal shift
➜ tip may be in the chest
LEFT
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Diagnosis
Chest X-ray
• absent diaphragmatic outline
• Loops of bowel in chest
• NG tube passed
➜ mediastinal shift
➜ tip may be in the chest
Contrast occasionally necessary(cystadenomatoid lung malformation)
LEFT
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Diagnosis
LEFT
Chest X-ray
➜ Right sided Hernia
➜ Eventration of diaphragm
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Diagnosis
LEFT
Contrast Swallow X-ray
➜ Right sided Hernia
➜ Eventration of diaphragm
This was late presenting with chronic GI symptoms
Contrast occasionally necessary(cystadenomatoid lung malformation)
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Treatment
Immediate Treatment
Naso-gastric tube
Endo-tracheal tube – ventilate
Goaladequate tissue oxygenationuse low ventilation pressuresavoid barotrauma
sedation / paralyse
prevent acidosis
avoid pulmonary hypertension
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Treatment
Ventilation
• conventional ventilation
• High Frequency Oscillatory Ventilation
• Inhaled Nitric Oxide
• Extracorporeal Membrane Oxygenation
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Treatment
Surgery
Not an emergency
Abdominal approach
Reduce the hernia
Suture with interrupted non-absorbable sutures
‘Gortex’ patch sometimes required
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Treatment
LEFT
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Treatment
LEFT
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Treatment
RIGHT
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Infantile Pyloric Stenosis
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Infantile Pyloric Stenosis
• may be called• Infantile Hypertrophic Pyloric Stenosis
• sometimes called (incorrectly)• Congenital Pyloric Stenosis
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Epidemiology
• Age between 2 to 8 weeks – usually 4-6 weeks
• incidence – 2-3 per 1000 live births
• sex – M:F 4:1
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Aetiology
• Cause of Pyloric Stenosis• multifactorial
• family history
• more common in firstborn
• Affected mother– 20% risk for son, 7% risk for daughter• Affected father– 5% risk for son, 2.5% risk for daughter
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Pathogenesis
pylorus
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Pathogenesis
• Hypertrophy of the circular layer of smooth muscle surrounding the pyloric opening
• Narrowing of the pyloric lumen
• Obstructs the passage of food
• Severe projectile vomiting
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Clinical Presentation - History
• Effortless vomiting initially, becoming projectile
• Non-bilious
• ‘Well child’ - Hungry
Later• lost weight• dehydrated• lethargic
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Clinical Presentation - Examination
• Well Child / thin & dehydrated
• crying; hungry
• lethargic – semiconscious
• Abdomen
• Visible peristalsis
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Clinical Presentation - Examination
• Well Child / thin & dehydrated
• crying; hungry
• lethargic – semiconscious
• Abdomen
• Visible peristalsis
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Clinical Presentation - Examination
• Well Child / thin & dehydrated
• crying; hungry
• lethargic – semiconscious
• Abdomen
• Visible peristalsis
• Test feed– pyloric tumour (olive)
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Investigations
• Ultrasound– >4mm thickness, – >18mm long, – non-passage of fluid despite gastric peristalsis
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Management
Erect IV infusion
Check serum electrolytes– sodium– potassium– chloride
Rehydrate– when electrolytes normal
OPERATION– Ramstedt’s Pyloromyotomy
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Management
Erect IV infusion
Check serum electrolytes– sodium– potassium– chloride
Rehydrate– when normal
OPERATION– Ramstedt’s Pyloromyotomy
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Management
Erect IV infusion
Check serum electrolytes– sodium– potassium– chloride
Rehydrate– when normal
OPERATION– Ramstedt’s Pyloromyotomy
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Management
Erect IV infusion
Check serum electrolytes– sodium– potassium– chloride
Rehydrate– when normal
OPERATION– Ramstedt’s Pyloromyotomy
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Management
Erect IV infusion
Check serum electrolytes– sodium– potassium– chloride
Rehydrate– when normal
OPERATION– Ramstedt’s Pyloromyotomy
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Post-op
feed after a few hours
observe until feeding begins to be established
out-patient review is not required
Complicationsperforation of duodenal mucosa
duodenal fornix
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Duodenal Atresia
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Duodenal Atresia
• Failure of canalization
• Early – 8-10 weeks
• Jejunoileal Atresia
• Late event
• Mesenteric Vascular accidents• volvulus / malrotation • internal hernia,• intussusception, • bands, • gastroschisis
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What is Duodenal Atresia
Duodenal atresia is the congenital absence or complete closure of a portion of the lumen of the duodenum
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Anatomy
Duodenal atresia congenital absence or complete closure
of a portion of the lumen of the duodenum
• behind transverse colon
• Four parts
• retroperitoneal – except 1st part
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Anatomy
Duodenum
• behind transverse colon
• Four parts
• retroperitoneal – except 1st part
• Atresia in SECOND part
Bile & pancreatic juice
Ampulla of Vater
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Anatomy
Duodenum
• behind transverse colon
• Four parts
• retroperitoneal – except 1st part
• Atresia in SECOND part
duodenum
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Epidemiology
Duodenal Atresia
• 1 in every 5,000 live births• M = F• Race – no significant differences• Approximately 30% have Downs Syndrome• Approximately 8% all infants with Down syndrome have duodenal atresia
• Overall survival – 70%
• Mortality– Associated abnormalities– Prematurity– Low Birth Weight
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Associated Conditions
Duodenal Atresia
• Down’s- third• Cardiac- 20%• Malrotation – 20%• TOF – 10%• Genito-urinary – 10%• Ano-rectal – 5%
• Not associated with other small bowel atresias.
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Clinical - Antenatal
Antenatal Considerations– Polyhydramnios– Ultrasound scan
• double bubble
– 50% diagnosed– Persistent polyhydramnios
• Repeat scan
– Downs Syndrome• karyotyping
– Counselling– Planning delivery
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Clinical - Antenatal
Antenatal Considerations– Polyhydramnios– Ultrasound scan
• double bubble
– 50% diagnosed
– Counselling– Planning delivery
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Clinical - Postnatal
Duodenal Atresia
• vomiting – early– bile stained
• scaphoid abdomen
• epigastrium– fullness– mass
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Investigations
Duodenal Atresia
• Abdominal x-ray– Double bubble
• Contrast study – rarely required
DuodenumStomach
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Clinical - Postnatal
Operation
• Do not excise the obstruction• Bypass the obstruction
• Duodenoduodenostomy side-to-side anastomosis
• Complications uncommon
• Hospital 2-3 weeks
• Following discharge
• no long-term sequelae
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Atresia of the Small Bowel
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What is Small Bowel Atresia?
Jejunoileal atresia and stenosis
congenital obstruction
major causes of neonatal intestinal obstruction.
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Compare with Duodenal Atresia
Duodenal Atresia
• Failure of canalization
• Early – 8-10 weeks
• Jejunoileal Atresia
• Late event
• Mesenteric Vascular accidents• volvulus / malrotation • internal hernia,• intussusception, • bands, • gastroschisis
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Anatomy
Small intestine
• intra-abdominal
• from: duodeno-jejunal flexure• to: ileocaecal valve
• adults: 6 m• children 3 m
• jejunum 40%• ileum 60%
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Epidemiology
• 1 in 300 live births
• More common than Duodenal atresia
• Much more common that large bowel atresia
• not associated with Down’s syndrome
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Aetiology
• intrauterine vascular accident resulting in necrosis of the affected segment, with subsequent resorption.
• Meconium Ileus
• Mid-gut volvulus
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Types
Types
• Type I – in continuity; mesentery intact• Type II – band; mesentery intact (usually)• Type IIIa – ends separate; V shaped defect in mesentery• Type IIIb – Ends separate “apple peal”; Christmas “Tree deformity”• Type IV – multiple atresias
• Defect in mesentery– missing bowel– short gut
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Types
Types 4 Types
• Type I • Type II • Type IIIa • Type IIIb • Type IV
• Defect in mesentery– missing bowel– short gut
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Clinical - Antenatal
• Polyhydramnios
• Diagnosed on foetal ultrasound– dilated or obstructed foetal intestine
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Clinical - Postnatal
• features of intestinal obstruction Bilious vomiting Abdominal distension
• Diagnosed on day 1 – 2
• Distal obstruction more delayed
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Clinical - Postnatal
• features of intestinal obstruction Bilious vomiting Abdominal distension
• Diagnosed on day 1 – 2
• Distal obstruction more delayed
• Present with perforation
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Diagnosis
• Radiology
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Diagnosis
• Radiology
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Treatment
Surgery Type I
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Treatment
Surgery Type III
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Treatment
Surgery
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Treatment
Surgery
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Treatment
Surgery
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Treatment
Surgery
• Discrepancy in bowel diameter
• Short gut
Procedure
• anastomosis
• Resect very dilated segment
• Stoma
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Prognosis
Post-op
• Anastomotic leak• progress• difficult and slow• parenteral nutrition• dysmotility• re-operation common
• Survival• 80 – 90%
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Abdominal Wall Defects
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Overview
➜ Types➜ Epidemiology➜ Pathophysiology➜ Clinical➜ Treatment➜ Prognosis
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Types
➜ Exomphalos➜ Major ➜ Minor
➜ Gastroschisis
ExomphalosOmphalocele
Gastroschisis
sac
other congenital abnormalities
no other congenital abnormalities
no sac
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Types
➜ Exomphalos➜ Major ➜ Minor
ExomphalosOmphalocele
sac
other congenital abnormalities
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Types
➜ Gastroschisis Gastroschisis
no other congenital abnormalities
no sac
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Types
➜ Exomphalos➜ Major ➜ Minor
ExomphalosOmphalocele
Exomphalos MinorHernia of the cord
Exomphalos Major
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Epidemiology & Pathophysiology
➜ Incidence1 in 3000 births
Exomphalos ➜ Associated abnormalities 50%➜ Association with Trisomy 13 and 18
➜ Pathophysiology➜ not known➜ Wk 5 to 10 intestine lies in a peritoneal extension in the umbilical cord➜ ?? Gut does not return to abdominal cavity➜ Does Gastroschisis result from a ruptured Exomphalos?
➜ But Exomphalos is associated with other major early developmental abnormalities➜ Gastroshisis is not➜ In Exomphalos, in utero - liver appears before gut in sac
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Clinical - Antenatal
➜ Diagnosis➜ no problem at birth
➜ Antenatal diagnosis➜ major structural abnormalities➜ occur early in development
➜ Can tell➜ if membrane is present➜ if liver is outside abdomen
➜ If antenatal diagnosis made➜ Search for other abnormalities➜ Counsel parents➜ Plan delivery➜ C/S only for obstetric reasons➜ Repeated frequent scans especially in last trimester
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Clinical - Postnatal
➜ Delivery➜ At or near paediatric surgical centre➜ Diagnosis
➜ no problem at birth➜ cord clamped and cut !!
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Clinical - Postnatal
➜ Delivery➜ At or near paediatric surgical centre➜ Diagnosis
➜ no problem at birth➜ cord clamped and cut !!
➜ Naso-gastric tube ➜ IV fluids
➜ account for increased fluid loss➜ Protect exposed gut
➜ clean ➜ prevent evaporation ➜ prevent kinking of bowel
➜ Keep infant warn➜ significant increased risk of heat loss
➜ Look for other abnormalities
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Treatment
Exomphalos Minor➜ Hernia of the cord➜ Small defect < 4cms diameter➜ Contains only midgut➜ Midgut rotational abnormalities are common➜ Associated abnormalities are rare➜ Persistent vitello-intestinal duct frequent➜ Associated with other midline defects
➜ Pentalogy of Cantrell
Cantrell’s PentalogyThoraco-abdominal ectopia cordia
➜Cleft lower sternum➜anterior diaphragm defect➜absence of parietal pericardium➜exomphalos➜congenital heart defect
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Treatment
Exomphalos Minor➜ Hernia of the cord➜ Small defect < 4cms diameter➜ Contains only midgut➜ Midgut rotational abnormalities are common➜ Associated abnormalities are rare➜ Persistent vitello-intestinal duct frequent➜ Associated with other midline defects
➜ Pantalogy of Cantrell➜ Beckwith-Wiedemann syndrome
Beckwith-Wiedemann syndrome
➜Macroglossia➜Exomphalos➜visceromegaly➜giantism➜hypoglycaemia➜hemihypertrophy➜Renal tumours➜Beckwith-Wiedemann syndrome
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Treatment
Exomphalos Minor➜ Hernia of the cord➜ Small defect < 4cms diameter➜ Contains only midgut➜ Midgut rotational abnormalities are common➜ Associated abnormalities are rare➜ Persistent vitello-intestinal duct frequent➜ Associated with other midline defects
➜ Pantalogy of Cantrell➜ Beckwith-Wiedemann syndrome➜ cloacal exstrophy
➜cloacal exstrophy
➜Exomphalos➜bladder exstrophy➜vesical cleft➜intestinal fistula
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Treatment
Exomphalos Minor➜ Hernia of the cord➜ Small defect < 4cms diameter➜ Contains only midgut➜ Midgut rotational abnormalities are common➜ Associated abnormalities are rare➜ Persistent vitello-intestinal duct frequent➜ Associated with other midline defects
➜ Pantalogy of Cantrell➜ Beckwith-Wiedemann syndrome➜ cloacal exstrophy
➜ Primary closure usually possible
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Treatment
Exomphalos Minor
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Treatment
➜ Exomphalos Major➜ Large defect > 4 cms➜ in addition to midgut contains
➜ Liver➜ ? stomach➜ ? spleen
➜ Closure – difficult➜ Close skin only – leave abdominal wall➜ Close skin only – patch abdominal wall➜ leave to granulate
9 weeks
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Treatment
Gastroschisis➜ defect small usually < 4cms➜ contains
➜ midgut➜ ? stomach➜ ? bladder / uterus / ovaries / testes
➜ Bowel wall – thickened / matted➜ parts ischaemic / necrotic➜ Closure may increase intra-abdominal pressure➜ Complicate gastroschisis➜ Associated atresia
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Treatment
Gastroschisis
bowel matted fibrinous coat
necrotic bowel
stalk
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Treatment
Gastroschisis➜ primary closure➜ just close skin
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Treatment
Gastroschisis➜ primary closure➜ just close skin➜ Delayed primary closure
silo
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Treatment
Gastroschisis➜ primary closure➜ just close skin➜ Delayed primary closure➜ Abdominal wall patch abdominal wall patch
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Outcome
Gastroschisis➜ 90 % survive➜ most cosmetically acceptible
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Outcome
Gastroschisis➜ 90 % survive➜ most cosmetically acceptible