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Jejunoileal atresia is usually treated by excision, including the dilated proximal bowel, and primary anastomosis1. Morbidity is high during infancy1,2,3 but there are few reports of continued morbidity into teenage and adult life4,5,6.
A man aged 31 had chronic malabsorption syndrome. Soon after birth he had had extensive proximal jejunal resection for multiple jejunoileal atresias, with primary anastomosis. The consequent short-gut syndrome led to failure to thrive in the first year of life, but for the next 14 years he made satisfactory progress. Then at 15 he required multiple admissions for malabsorption syndromes, culminating in a laparotomy at which a grossly dilated small bowel was resected with end-to-end anastomosis. Three months later, he had a third laparotomy because of persistent symptoms; again a grossly dilated bowel was found and this was plicated. At the age of 21, after a year of recurrent admissions for malabsorption due to megajejunum, he had a fourth laparotomy. An entero-enterostomy was fashioned between dilated and non-dilated parts of his small bowel. There was no evidence of obstruction. From the age of 25 he again had multiple admissions for malabsorption syndrome secondary to recurrent megajejunum, which was managed conservatively. However, at the age of 31 he had his fifth laparotomy when the entero-enterostomy was revised. He did not get better (Figure 1), and a sixth laparotomy was undertaken four weeks later.
At laparotomy there was a megajejunum with about 25 cm of normal-calibre bowel distal to it. An excisional jejunoplasty was performed by several firings of a 75 mm linear stapler-cutter on the antimesenteric border up to the duodeno-jejunal flexure. The staple line was oversewn, a small segment of ischaemic bowel devascularized by mobilization was resected, and an end-to-end anastomosis was fashioned. Histological examination of the specimen showed no evidence of intrinsic disease. A barium follow-through, four months after surgery, showed normal small bowel (Figure 2). Fifteen months after surgery he is well, has put on weight and leads an active and normal life.
In a multivariate analysis of megaduodenum, the presence of duodenal dilatation postoperatively was associated with delayed transit7. Low contraction amplitude was the most distinguishing manometric finding and this causes ineffective peristalsis4,7. Massive dilatation of the bowel leads to bacterial overgrowth, vomiting and malabsorption that is resistant to conservative management4,5,6. Distal small-bowel transit is normal in these patients5—a finding that rules out a global motility disorder. Most of these patients are treated conservatively to avoid the short-bowel syndrome that can follow surgery.
In the patient reported above and in those described by Ein et al.4 with megaduodenum, massive dilatation recurred after plication. The mechanism is not well studied but a possible explanation is failure of surgery rather than true recurrence. However, enteroplasty, either by plication or preferably by excision, is regarded as the treatment of choice4,7 because it improves transit by increasing luminal pressure7. Incomplete resection of the dilated part may contribute to recurrence, since excision of the atretic part alone is associated with persistent obstructive symptoms8. Some workers have reported deficiency in mucosal enzymes and muscle adenosine triphosphatase for a distance of 10-20 cm, and hyperplasia of ganglion cells in the dilated parts9,10. Others, like us, have detected no histological abnormalities in the dilated parts6, other than the lipofuscinosis (brown bowel syndrome) that can result from fat malabsorption5. There is a suggestion that anastomotic dysfunction can be avoided by sacrificing as much as 15-20 cm of proximal dilated bowel. Enteroenterostomy, however, is doomed to failure3,4 because the flaccid bag of bowel is still present.
These sporadic cases of massive bowel dilatation, in the absence of obstruction or histological abnormality, in older children and adults years after surgery for intestinal atresia, raise questions about initial management. Are these a failure of surgical management? In infancy, the ideal procedure is said to be excision of the atresia and excisional enteroplasty where indicated1,2,3. The role for plication is not clear. The present case shows that excisional enteroplasty can be successful in an adult.