A 7-month-old female baby weighing 7.4 kg presented with abdominal distension, bilious vomiting, low urine output and fever for two days. Examination revealed pallor, dehydration and feeble peripheral pulses. The abdomen was distended and tender. Plain abdominal X-ray showed multiple air-fluid levels. Ultrasound scan of the abdomen showed some free fluid and there was no intussusception. Hemoglobin was low and renal functions were normal. Clinical setting of shock with tender abdomen suggested a bowel catastrophe. Emergency laparotomy after adequate resuscitation revealed 70 mL of hemorrhagic fluid in the peritoneal cavity, and on evisceration and simply splaying the intestines, we found two large ‘V’ shaped mesenteric defects in the jejunal mesentery approximately 30 cm from duodeno-jejunal flexure, each measuring 10x12cm with a short strip of intact intervening mesentery containing a feeding vessels in between the two defects (Fig. 1). The jejunum overlying the defects had multiple impending perforations and necrotic patches. There was no marginal vascular arcade along both the jejunal mesenteric defects. Another small mesenteric defect measuring 2x4 cm was noted in the ileal mesentery but the overlying ileum was normal. There was no evidence of malrotation. Rest of the bowel was normal. Fifty centimeters of necrotic jejunum overlying the defects was excised and jejuno-ileal anastomosis was performed. Jejunal and ileal mesenteric defects were closed. Post-operative recovery was uneventful. The histopathology of the resected jejunum showed scattered areas of mucosal ulceration, hyperemia and villous atrophy with hemorrhagic necrosis and leucocytic infiltration suggestive of ischemic enteritis. There was no evidence of fibrosis or stricture.

Fig. 1 Intraoperative photograph showing two large jejunal mesenteric defects with an intervening strip of intact mesentery with a feeding vessel (thick arrow). Absence of marginal vascular arcade along both the defects and distribution of necrotic patches in the mid portion of the jejunal segment overlying each defect (white arrows). Few Impending perforations are sealed by omentum, and a very pink segment of jejunum being supplied by feeding vessel (double head arrow).

Common causes of bowel gangrene in infants are intussusception and midgut volvulus. Congenital mesenteric defects (CMDs) are usually accompanied by corresponding bowel atresia and present acutely at birth. CMD not associated with bowel atresia are rare and present with intestinal obstruction or bowel gangrene due to internal herniation or volvulus at a later age with or without bowel gangrene [1]. First reported in 1960 [2] CMDs are rare but an important cause of intestinal obstruction in all age groups; they may be asymptomatic, can present with shock or can be a cause of unexpected death [3]. CMD can also present with recurrent constipation, recurrent abdominal pain or recurrent intestinal obstruction due to intermittent internal herniation, those are often misdiagnosed [3,4]. CMDs can be either segmental defects or basilar defects, and mostly located in the ileocecal mesentery [5]. Rarely, segmental defects can occur both in jejunal and ileal mesentery as in our index case. Most CMDs are 2 to 3 cm wide and usually have a marginal vessel, unlike this case where defects were quite large and marginal vessels were conspicuous by their absence. Adjacent bowel might get trapped in the defect resulting in strangulation, or bowel overlying the defect might twist on its supplying vessel leading to volvulus, both causing occlusive ischemia leading to bowel gangrene. However, contrary to the above fact, CMD in our case were too large to trap adjacent bowel and there was no volvulus found intraoperatively, however the possibility of a spontaneously reduced volvulus cannot be ignored. Large CMD with absent marginal vessels and long segment of growing bowel overlying the CMDs runs a significant risk of vascular insufficiency. In the absence of occlusive phenomenon, a non-occlusive ischemic trigger (like infections especially viral, dehydration or shock) to a bowel, which already has a diminished blood supply, can lead to enterocolitis. However, the exact etiology of the necrosis in our case is not known and it could be due to either an infection, dehydration, shock or a spontaneously reduced volvulus.

1. Murphy DA. Intestinal hernias in infancy and childhood. Surgery. 1964;55:311-6.

2. Blandy JP. Neonatal intestinal obstruction from a congenital hole in the mesentery. Br J Surg. 1960;48:133-5.

3. Byaud RW, Wick R. Congenital mesenteric defects and unexpected death-a rare finding at autopsy. Pediatr Dev Pathol. 2008;11:245-8.

4. Page MP, Ricca RL, Resnick AS, Puder M, Fishman SJ. Newborn and toddler intestinal obstruction owing to congenital mesenteric defects. J Pediatr Surg. 2008;43:755-8.