Exxcellence pearls: fetal abdominal wall defects Flashcards
Which is more likely to have bowel covered by amnion and peritoneum, omphalocele are gastroschisis?
Which is more likely associated with chromosome abnormalities?
Omphalocele for both.
When is physiologic herniation present in the fetus?
Between the seventh and nine weeks. Resolves by the 10th week of development.
What is the differential diagnosis for fetal ventral abdominal wall defects?
Gastroschisis, omphalocele, ectopia cordis, limb-body wall complex, cloacal exstrophy, urachal cyst
Discuss delivery of fetuses with omphalocele?
Less than 5 cm can be delivered vaginally while those greater than 5 cm may be delivered by cesarean.
What other anomalies are associated with omphalocele?
What percentage of infants with omphalocele will have other anomalies?
Amniotic band syndrome, pentalogy of Cantrell, Beckwith-Wiedemann syndrome, OEIS syndrome.
50% of omphalocele’s are associated with cardiac defects, GI, GU, CNS anomalies.
Which is associated with higher AFP, omphalocele or gastroschisis?
Gastroschisis
How is omphalocele treated?
Primary closure for small omphalocele; larger defects required silos, elastic bandages and epithelialization with delayed surgical repair.
What are possible complications of gastroschisis?
Infection, hypothermia and acidosis
How is gastroschisis treated?
After vaginal delivery primary closure is possible in 80% of cases. Otherwise a silo is used.
What is the suspected pathogenesis of gastroschisis?
Vascular accident of the umbilical vein or omphalomesenteric artery. All layers of the abdominal wall are absent.
What is ectopia cordis?
What is the prognosis?
Defect where in the heart is partly or completely positioned outside of the thorax; associated with pulmonary hypoplasia.
Neonatal mortality is high.
What is pentalogy of Cantrell?
- Median supraumbilical abdominal wall defect
- Defect in the sternum
- Diaphragmatic pericardial deficiency
- Anterior diaphragmatic deficiency
- Intracardiac defects.
Due to failure of cephalic folding.
May also be associated with cystic hygroma, cranial defects and chromosome abnormalities.
What is body stalk anomaly?
Failure of fusion of the four body folds; associated with cranial defects, facial midline clefts, thoracic and/or abdominal wall and limb defects. Abdominal contents may be attached directly to the placenta with an absent or short umbilical cord. This is generally lethal.
What is cloacal exstrophy?
The presence of what organ on ultrasound excludes this diagnosis?
Exstrophy refers to inversion of an organ and in this case refers to the urinary bladder, bowels, and omphalocele. It is also associated with anal atresia, hypoplasia of the colon, and anomalous genitalia.
Bladder.
What fetal abdominal wall defect is suggested by umbilical cord insertion at the apex?
To the side?
Below?
Above?
Omphalocele.
Gastroschisis.
Ectopia Cordis.
Cloacal exstrophy.
