Omphalocele- Omphalocele
Information, picture
Omphalocele Information
The three most common abdominal wall defect in newborns are umbilical hernia, gastroschisis and
omphalocele. Omphalocele is a milder form of primary abdominoschisis since during the embryonic
folding process the outgrowth at the umbilical ring is insufficient (shortage in apoptotic cell death).
Bowel and/or viscera remains in the umbilical cord causing a large abdominal wall defect. In Omphalocele Defect may
have liver, spleen, stomach, and bowel in the sac while the abdominal cavity remains nderdeveloped in
size. The sac is composed of chorium, Wharton's jelly and peritoneum. The defect is centrally localized
and measures 4-10 cm in diameter. A small defect of less than 2 cm with bowel inside is referred as a
hernia of the umbilical cord.
There is a high incidence of Omphalocele (30-60%)of associated anomalies in patients with
omphalocele. Epigastric localized omphalocele are associated with sternal and intracardiac defects (i.e.,
Pentalogy of Cantrell), and hypogastric omphalocele have a high association with genito-urinary
defects (i.e., Cloacal Exstrophy). All have malrotation. Cardiac, neurogenic, genitourinary, skeletal and
chromosomal changes and syndromes are the cornerstones of mortality.
Antenatal diagnosis of Omphalocele may
affect management by stimulating search for associated anomalies and changing the site, mode or
timing of delivery. Cesarean section is warranted in large omphaloceles to avoid liver damage and
dystocia. After initial stabilization management requires consideration of the size of defect, prematurity
and associated anomalies. Primary closure with correction of the malrotation should be attempted
whenever possible.
Omphalocele Treatment
If this is not possible, then a plastic mesh/silastic chimney is fashioned around the
defect to cover the intestinal contents and the contents slowly reduced over 5-14 days. Antibiotics and
nutritional support are mandatory. Manage control centers around sepsis, respiratory status, liver and
bowel dysfunction from increased intraabdominal pressure.
If you like this page, please post it on:
|
