Gastroschisis and Omphalocele

Posted by e-Medical PPT Thursday, March 8, 2012
Defect in abdominal wall, usually to right of intact umbilical cord
Results in herniaton of free bowel loops into amniotic fluid
No sac covering
1/10,000 live births
bowel shortened
thick, inflammatory peel
25% association w/ intestinal atresias
Malrotation, meckel’s diverticulum, low birth weight also associated

Unknown cause, many theories
Failure of abdominal wall to form properly
Vascular disruption at abd wall
In utero disruption of omphalocele

Prenatal management
Vaginal delivery OK
Prognosis good d/t low associated anomalies
Immediate Surgical Repair
Delays d/t institutional transfers costly

Initially IVF, gastric decompression, Broad spectrum antibiotics, coverage of bowel
Intraop - ? midgut volvulus, intestinal perforation
75% of cases, reduction is successful
Monitor intraabdominal pressures

 Short gut syndrome
 Developmental delay
 Necrotizing Enterocolitis
 Cholestatic liver disease from prolonged TPN

Umbilical cord inserts onto the membrane
Large defects can extend from umbilicus to costal margin – contain liver, small and large bowel
1/4000 live births
Failure of abdominal wall to fuse at umbilical ring
Unlike gastroschisis, bowel is not significantly injured, normal motility and function

Associated anomalies
40% have chromosomal abnormalities (Trisomy 13, 18, 21, Turner’s and Klinefelter synd)
60-70% infants have associated malformations
Cardiovascular, genitourinary, CNS
Beckwith-Wiedman syndrome
Pentalogy of Cantrell
Prognosis based on associated anomalies

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