The mainstay of surgical treatment for this type of lesion is resection of the atretic or stenotic segment and primary anastomosis, with closure of the mesenteric defect. The proximal intestinal segment is usually dilated and functionally abnormal with absent or ineffective peristalsis. This dilated proximal segment is excised along with a short segment distal to the stenosis or atresia. It is essential to establish patency of the distal bowel by irrigation or wash-out of the intestine and subsequently a primary anastomosis is performed. There is a balance to be struck between the length of the dilated proximal segment resected and the risk of leaving the infant with a short length of small bowel. It is almost inevitable that the caliber of the proximal bowel will be greater than that of the distal intestine, and a number of techniques including fishtailing and tapering exist to assist the anastomosis in such circumstances.
Outcome following intestinal atresia is dependent primarily on the length of remaining intestine and the presence of the ileocecal valve. Short bowel syndrome is defined as the presence of less than 75 cm of the small intestine or 30% of the predicted intestinal length in a premature infant.15,16 Outcomes following short bowel syndrome vary and there is a high level of dependence on parenteral nutrition. However, intestinal adaptation can occur such that more than 80% of babies with short bowel syndrome do eventually become entirely enterally nourished.17
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