Closed Gastroschisis with Vanished Small Bowel and Jejunal Atresia

Pre-term (35 weeks) 1-day old (2.1kg) male child delivered by Caesarean section (in view of meconium stained liquor) presented with a fleshy red mass protruding from right side of umbilical cord since birth. Examination revealed soft mass (mummified mass of bowel) attached with abdominal wall just right to umbilical cord (Fig.1). Laboratory investigations were normal. Abdominal X-ray gave impression of proximal small bowel atresia and extra abdominal soft tissue mass with no calcification in it (Fig.2). Intra-operatively there was tiny defect in muscle just right to umbilicus. Matted (mummified) vanished small bowel was connected with cord like structure to atretic end of jejunum proximally and transverse colon distally (entry-exit atresia) without any lumen. After resection of the matted bowel, only 25cm of jejunum and micro transverse colon were left (Fig.3). Bishop Koop type stoma (jejuno-transverse) was fashioned. A central line (right internal jugular vein) was inserted. Post operatively child remained on total parental nutrition. Oral pre-digested feed was started on 9th post-operative day but child developed complications of short bowel syndrome like watery diarrhoea, electrolyte imbalance, weight loss and central line related sepsis. Although we have closed Bishop Koop stoma 3 weeks after first surgery but ultimately child succumbed within 4 weeks of admission. Figure 1: Showing dilated jejunum with atresia (big arrow) and distal transverse colon atresia (small arrow). Inset shows matted bowel mass exiting from a tiny hole in the muscle just right to the umbilical cord.