Congenital malformations - Edocr

250 PART VI GASTROINTESTINAL MALFORMATIONS
minimal pressure with closure of the abdominal
wall defect is the goal of repair and depends primarily
on the size of the abdominal cavity. Associated
bowel atresia and stenosis will require
identification and repair but may be precluded
by severe matting of the bowel and peel formation.
Staged closure is performed if an infant
cannot tolerate primary repair.
With current advances in neonatal care,
long-tem survival for gastroschisis has improved
dramatically over the years to a nearly 90–95%
survival rate. The size of the abdominal wall defect
and contents of herniated viscera do not affect
the outcome but bowel wall thickening of
>3 mm and dilatation of bowel in >17 mm at birth
have been associated with a poor outcome. Intestinal
atresia and necrosis have also been associated
with increased morbidity and mortality. 12
Infants with gastroschisis often have a prolonged
ileus and require parenteral nutrition support for
longer periods compared to infants with omphalocele.
The overall long-term outcome is good
since they have few associated anomalies. Longterm
complications include short gut syndrome
and postoperative intraabdominal adhesions.
GENETIC COUNSELING
Recurrence risk in nonfamilial cases is extremely
low (