fetal anterior fetal abdomen abdominal wall defects - … ·  · 2016-10-12abdominal wall defects...

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1 Fetal Anterior Abdominal Wall Defects Mani Montazemi, RDMS Director of Ultrasound Education & Quality Assurance Baylor College of Medicine Division of Maternal-Fetal Medicine Maternal Fetal Center Imaging Manager Texas Children’s Hospital, Pavilion for Women Houston Texas & Clinical Instructor Thomas Jefferson University Hospital - Radiology Department Philadelphia, Pennsylvania Fetal Abdomen Fetal Abdomen Fetal Abdomen Fetal Abdomen Fetal Abdomen Ventral Wall Defects Relation to Umbilicus Above umbilicus Consider pentalogy of cantrell At umbilicus Consider gastroschisis or omphalocele Below umbilicus Consider exstrophy of bladder or cloaca Difficult to tell because of size Consider body stalk anomaly Fetal Abdomen Ventral Wall Defects Relation to Umbilicus Above umbilicus Consider pentalogy of cantrell At umbilicus Consider gastroschisis or omphalocele Below umbilicus Consider exstrophy of bladder or cloaca Difficult to tell because of size Consider body stalk anomaly Fetal Abdomen Ventral Wall Defects Omphalocele Central defect Associated anomalies are common High risk of aneuploidy Gastroschisis Periumbilical defect Associated anomalies are uncommon Little to no risk of aneuploidy High rate of bowel related complications Associated with substance abuse & medications

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Page 1: Fetal Anterior Fetal Abdomen Abdominal Wall Defects - … ·  · 2016-10-12Abdominal Wall Defects Mani Montazemi, ... •Non-visible bladder in fetal pelvis •Bulging mass protruding

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Fetal Abdomen

Fetal Anterior

Abdominal Wall Defects

Mani Montazemi, RDMSDirector of Ultrasound Education & Quality Assurance

Baylor College of Medicine

Division of Maternal-Fetal Medicine

Maternal Fetal Center Imaging Manager

Texas Children’s Hospital, Pavilion for Women

Houston Texas

&

Clinical Instructor

Thomas Jefferson University Hospital - Radiology Department

Philadelphia, PennsylvaniaFetal Abdomen

Fetal Abdomen

Fetal Abdomen

Fetal Abdomen

Fetal Abdomen

Ventral Wall Defects

Relation to Umbilicus

• Above umbilicus

– Consider pentalogy of cantrell

• At umbilicus

– Consider gastroschisis or omphalocele

• Below umbilicus

– Consider exstrophy of bladder or cloaca

• Difficult to tell because of size

– Consider body stalk anomaly

Fetal Abdomen

Ventral Wall Defects

Relation to Umbilicus

• Above umbilicus

– Consider pentalogy of cantrell

• At umbilicus

– Consider gastroschisis or omphalocele

• Below umbilicus

– Consider exstrophy of bladder or cloaca

• Difficult to tell because of size

– Consider body stalk anomaly

Fetal Abdomen

Ventral Wall Defects

Omphalocele– Central defect

– Associated anomalies are common

– High risk of aneuploidy

Gastroschisis– Periumbilical defect

– Associated anomalies are uncommon

– Little to no risk of aneuploidy

– High rate of bowel related complications

– Associated with substance abuse & medications

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Fetal Abdomen

• Herniation of intraabdominal contents

into the base of the cord

Omphalocele

• ALWAYS covered by a membrane

Fetal Abdomen

Omphalocele

• Umbilical cord inserts onto membrane– In large defects may be displaced eccentrically

• The herniated bowel is NOT directly exposed to

amniotic fluid

• Bowel usually does not thicken or dilate

Fetal Abdomen

Is This an Omphalocele?

Fetal Abdomen

Pseudo-Omphalocele

Caused by scanning oblique or

by excessive transducer pressure

Fetal Abdomen

OmphaloceleCan be a small or large wall defect

Fetal Abdomen

Omphalocele

• Small

– Failure of normal midgut rotation

– Cord midpositioned

– Usually contains only bowel

– Associated with chromosomal

anomalies

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Fetal Abdomen

Omphalocele

• Large

– Failure of anterior abdominal

wall closure

– Cord eccentric

– May contain organs

– Scoliosis

– Associated with structural

anomalies

Fetal Abdomen

Omphalocele

Measurements of AC

inaccurate and should

be excluded from

biometric calculations

Fetal Abdomen

Omphalocele

• IUGR has been reported in 20% of patients

• 50% have associated anomalies (20% cardiac)

• 30% are associated with trisomy 13 & 18

Fetal AbdomenJane J.K. Burns, RDMS

Diagnostic Challenge

Fetal AbdomenJane J.K. Burns, RDMS

Omphalocele

• Omphalocele and cord cyst may co-exist

– Whartons jelly cyst – mucoid degeneration of Wharton Jelly

– Allantoic cyst – always near insertion site

– Omphalomesenteric duct cyst – associated with intraabdominal

mesenteric cysts

Fetal Abdomen

Diagnostic Challenge

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Fetal Abdomen

Cystic Ventral Mass

Arising at the Umbilicus

The umbilical cord inserted into the

membranous covering of the mass

Fetal Abdomen

Diagnostic Challenge

Separate from but contiguous with the bladder

Fetal Abdomen

Umbilical Cord

Allantoic Stalk

Yolk Stalk

Cloaca

Patent Urachus With Allantoic Cyst

Urachus is an embryological remnant of the

allantois which runs from the apex of the bladder

through the umbilical ring to terminate in the

proximal umbilical cord

Fetal Abdomen

Small defect to right of cord insertion

No membrane over bowel

Gastroschisis

Fetal Abdomen

Gastroschisis

• Incidence is 1 in 3000 births

– Varies considerably with

maternal age

– Strong association reported

among younger patients

• Less likely for organ

herniation

• Variable amounts of bowel

herniated

• Bowel floats within

amniotic fluid

Fetal Abdomen

Gastroschisis

• Hepatic herniation is less frequent with

gastroschisis than with omphaloceles

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Fetal Abdomen

Gastroschisis

• Small defect (2-4cm)

Fetal Abdomen

Gastroschisis

• Associated anomalies in about < 10% of fetuses

• IUGR in up to 50%

• No chromosomal abnormalities

Fetal Abdomen

Gastroschisis

• Oligohydramnios

– More common than polyhydramnios

– Suggest fetal distress

• Polyhydramnios

– Suggest bowel obstruction or atresia

Fetal Abdomen

Gastroschisis

• Marked bowel dilatation, which may be either

external or internal to the abdominal cavity, suggests

bowel obstruction and/or ischemia

Fetal Abdomen

Gastroschisis

Fetal Abdomen

Gastroschisis

• Bowel can twist and cut off blood supply

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Fetal Abdomen

Gastroschisis

Fetal Abdomen

Gastroschisis

Fetal Abdomen

Gastroschisis

Fetal Abdomen

20 y

G1 P0

Viability

Gastroschisis

Fetal Abdomen Fetal Abdomen

Omphalocele @11 Weeks ?

Potential Pitfall

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Fetal Abdomen

Normal Midgut Herniation

• Fetal bowel normally herniates into the base

of the umbilical cord at approx. the 7-8 weeks

MA

• Detected sonographically from 9-11 wks

• Should not be visible by 12 week

Fetal Abdomen

Normal Midgut Herniation

• This appearance should not be mistaken for a

ventral wall defect

Fetal Abdomen Fetal Abdomen

Fetal Abdomen Fetal Abdomen

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Fetal Abdomen Fetal Abdomen

Fetal Abdomen Fetal Abdomen

Pentalogy of Cantrell

A term used to describe the association of 5 anomalies:

1. Midline supraumbilical abdominal defect

2. Defect of the lower sternum

3. Defect of the diaphragmatic pericardium

4. Anterior diaphragmatic hernia

5. Intracardiac abnormalities

Fetal Abdomen

Pentalogy of Cantrell – US Findings

• Midline anterior wall defect usually upper abdomen

• Ectopic heart

• Pericardial or pleural effusion

• Craniofacial anomalies

• Ascites

• Two vessel cord

Fetal Abdomen

Ectopic Cordis

• Rare malformation

• Protrusion of heart through chest wall

• Association - Pentalogy of Cantrell

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Fetal AbdomenJane J.K. Burns, RDMS

Limb-Body Wall Complex

• Also known as “body stalk” anomaly

• Failure of ventral abdominal wall to close

– Often left sided

Fetal Abdomen

Limb-Body Wall Complex

• Abdominal organs lie in a sac outside the abdominal cavity

• Short or absent umbilical cord

• Fetus lies directly on placenta*

• Universally fatal

Fetal AbdomenJane J.K. Burns, RDMS

Limb-Body Wall Complex

• Amniotic bands attached broadly to the fetus &

placenta

• Large thoraco-abdominal wall defect

– no covering membrane

• Distorted body axis

Fetal Abdomen

Limb-Body Wall Complex

• Severe scoliosis – prominent feature

• Limb defects common

• Complex array of multiple malformations

– Craniofacial & Internal organ anomalies

Fetal Abdomen Fetal Abdomen

Bladder Exstrophy

• A very rare congenital malformation

• Failure of closure of lower abdominal wall resulting in

exposed bladder into the amniotic cavity

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Fetal Abdomen

Bladder Exstrophy – Caution

• Do not confuse cystic pelvic structures with bladder

• Normal bladder fills & empties repeatedly during scan

Fetal Abdomen

Bladder Exstrophy – US Findings

• Non-visible bladder in fetal pelvis

• Bulging mass protruding from the lower abdominal

wall

– Large infraumbilical anterior wall defect

– Irregular and lobulated surface

• Normal kidneys

• Normal amniotic fluid volume

Fetal Abdomen

Bladder Exstrophy – US Findings

• Inferiorly displaced umbilicus

• Small penis with anteriorly displaced scrotum

• Splayed iliac bones

Fetal Abdomen

Bladder Exstrophy – Caution

• Determine the site of the defect with respect to

the umbilicus

Fetal Abdomen

Anatomy

• Umbilical arteries arise from the EIAs and run

on each side of the bladder before entering the

umbilical cord

Fetal Abdomen

Bladder Exstrophy – Caution

“Look for a landmark”

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Fetal Abdomen

Cloacal Exstrophy

Defect extends inferiorly and the bowel loops extrude between the two bladder halves

Omphalocele & bladder exstrophy

Prolapsed ileum

Fetal Abdomen

Cloacal Exstrophy

• Large infraumbilical anterior

wall defect

• Absent bladder

• Neural tube defects

• 2-vessel cord

• Malformation of the genitalia

• Imperforate anus

Fetal Abdomen

Ventral Wall Defects

Relation to Umbilicus

• Above umbilicus

– Consider pentalogy of cantrell

• At umbilicus

– Consider gastroschisis or omphalocele

• Below umbilicus

– Consider exstrophy of bladder or cloaca

• Difficult to tell because of size

– Consider body stalk anomaly

Fetal Abdomen

Thank You