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Virtual Pediatric Hospital: Paediapaedia: Omphalocele Paediapaedia: Gastrointestinal Diseases

Omphalocele

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Newborn with an anterior abdominal wall defect with a covering hernia sac with the umbilical cord attaching at the apex of the hernia sac.

Etiology/Pathophysiology:
Due to anomalous development and failure of fusion of the embryonic ventral folds (cephalic, paired lateral, and caudal) which form the anterior abdominal wall. Omphalocele results from failure to form the lateral folds and produces a complete defect in the anterior abdominal wall with a sac consisting of amnion and peritoneum covering the herniated bowel and the umbilical cord inserting at the apex of the hernia sac. Sixty-six percent have associated congenital defects including malrotation, intestinal atresias, imperforate anus, cardiovascular and genitourinary anomalies.

Pathology:
Not applicable

Imaging Findings:
Abdominal film shows a mass of extra-abdominal bowel loops protruding from the anterior abdominal wall with a covering membrane.

Since the diagnosis is made clinically, the radiological evaluation is important to determine if there are associated congenital abnormalities.

DDX:

References:

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