Findings: There is demonstration of a soft tissue mass with herniation of small and large bowel within the mass. There are multiple segmentation anomalies including hemivertebrae, butterfly vertebrae and rib anomalies of the tenth and eleventh rib, which are widely spaced and appear hypoplastic.

Also noted is diastasis of the symphysis pubis which is consistent with patient's history of bladder exstrophy.

Diagnosis: Omphalocele with associated segmentation anomalies including hemivertebrae, butterfly vertebrae and left sided rib anomalies. Also noted is diastasis of the pubic symphysis.

Discussion: 80% are born prematurely. There is a male predominance with an incidence of 1 in 5,000 live births. Omphalocele is a midline abdominal wall defect which is the result of maldevelopment of the lateral folds. Abdominal organs may herniate into the defect. The peritoneal and amnion sac cover the herniated viscera into the apex of the umbilical cord inserts. Two-thirds of neonates have associated anomalies, most often involving the cardiovascular system. A consequence of omphalocele is malrotation. Some patients develop volvulus.

References:
Blickman H. Pediatric Radiology: The Requisites., 2nd Edition.
Mosby, Inc.; 1998(4):107-108.

Kirks DR. Practical Pediatric Imaging: Diagnostic Radiology of Infants and Children,
3rd Edition. Lippincott-Raven Publishers; 1998(8):838-840.

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