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Radiology
Omphalocele
Built from Obstetric Imaging
What’s inside
10 sections · 101 slides
Overview
- The eight questions this topic answers
- The idea in one slide
What omphalocele actually is
The defect, the sac, and the cord that gives it away
- Definition, in plain words
- What is missing at the defect
- What the covering sac is made of
- Where the cord goes in
- Liver outside the abdomen at 21 weeks
- The same defect seen along the long axis
- Umbilical vessels running through the sac
- Cord vessels through the sac at 13 weeks
- When the sac tears
How common, and in whom
Incidence, registries and the risk factors that shift it
- How often it happens
- What the big registries found
- Who is at higher risk
- Race, ethnicity and survival
- Does it run in families?
Why it happens
Four weeks of embryonic folding, and the two ways it can go wrong
- The belly wall is built by folding
- The normal journey of the midgut
- Physiologic midgut herniation
- Two proposed developmental errors
- What each error produces
- Heart and belly wall are built together
- A mixed group, not one disease
What travels with it
Extra anomalies, the heart, the chromosomes and the syndromes
- The company it keeps
- What the four big series report
- Prevalence of Associated Anomalies
- Why the karyotype rate varies so much
- Heart defects: the commonest partner
- The tilted heart
- Heart rotated to the left at 29 weeks
- Look system by system
- Anomalies Associated With Omphalocele
- Which chromosome problems
- Chromosome Anomalies Associated With Omphalocele
- Syndromes that include an omphalocele
- Short List of Syndromes Associated With Omphalocele
- Beckwith-Wiedemann syndrome
- Spotting the overgrowth syndrome before birth
How it shows up, and how we image it
From a raised blood marker to the classic scan picture
- The blood test clue
- How high is high
- Most cases are found on the scan
- The classic ultrasound picture
- How early it can be seen
- Omphalocele at 11 weeks
- Size of the defect
- Measuring a 2.23 cm ventral wall defect
- A 4.23 cm defect with stomach in the sac
- A small defect with the liver still inside
- Large omphalocele containing ascites
- Fluid inside the sac
- Liver in the sac, or liver not
- What the liver split means for counselling
- When the membrane ruptures before birth
- Probably ruptured sac at 23 weeks
- Free-floating bowel after rupture
- Liver and bowel outside with no covering membrane
- What may still be hidden
- The work-up once you see it
- Where MRI fits
- The classic signs, gathered
What else it could be
Ten look-alikes, and the two features that settle it
- Why the differential matters
- Omphalocele against gastroschisis
- Normal for now: physiologic gut herniation
- Congenital hernia of the umbilical cord
- Limb-body wall complex
- Bladder and cloacal exstrophy
- Ectopia cordis and pentalogy of Cantrell
- The last three on the list
- Cord cyst beside an omphalocele
What to do about it
Before birth, at delivery, and in the first days of life
- Nothing to fix before birth
- How to deliver
- The giant omphalocele exception
- The first minutes after birth
- Why the newborn gets cold and dry
- If the sac has ruptured
- Giant omphalocele before and after serial decompression
- Newborn with an intact covering sac
- Newborn after the sac tore during delivery
- No rush to operate
- Four ways the defect gets closed
- Using a silo
- The slow route for large defects
How these babies do
Survival, growth and what actually predicts trouble
- What decides the outcome
- 67.9%
- Reading those numbers
- What predicts a bad neonatal outcome
- Growth runs both ways
- When these babies are born
- Amniotic fluid can swing either way
The points to carry away
Everything above, compressed
- Key points: the defect
- Key points: how common, and with what
- Key points: finding it
- Key points: look-alikes and management
- References
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- Suggested reading
- Obstetric Imaging: Fetal Diagnosis and Care, 2nd Edition