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Jejunoileal Atresia

by William J. Cochran, MD

Jejunoileal atresia is incomplete formation of the jejunum, usually caused by an ischemic insult.

There are 5 major types of jejunoileal atresia:

  • Type I consists of a membrane completely occluding the lumen with the intestine intact.

  • Type II is a gap in the intestine with a fibrous cord between the proximal and distal segments of intestine.

  • Type IIIA is a mesenteric gap without any connection between the segments.

  • Type IIIB is jejunal atresia with absence of the distal superior mesenteric artery; the distal small bowel is coiled like an apple peel, and the gut is short.

  • Type IV consists of multiple atretic segments (resembling a string of sausages).

Neonates with jejunoileal atresia usually present late during day 1 or on day 2 with increasing abdominal distention, failure to pass stools, emesis, and feeding problems.

Plain abdominal x-rays are done; they may reveal dilated loops of small bowel with air-fluid levels and a paucity of air in the colon and rectum. A barium enema reveals a microcolon (due to disuse). Because about 10% of patients also have cystic fibrosis (nearly 100% if meconium ileus is also present), testing for that disease (see Cystic Fibrosis) should be done.

Treatment

  • Surgical repair

Preoperative management consists of placing an NGT, giving nothing by mouth, and providing IV fluids. Surgical repair is the definitive therapy. During surgery, the entire intestine should be inspected for multiple areas of atresia. The atretic portion is resected, usually with a primary anastomosis. If the proximal portion of the ileum is extremely dilated and difficult to anastomose to the distal, unused part of the intestine, it is sometimes safer to do a double-barreled ileostomy and defer anastomosis until the caliber of the distended proximal intestine has diminished.

Prognosis is based on the length of remaining small bowel and the presence of the ileocecal valve. Infants who subsequently develop short bowel syndrome require TPN for extended periods. They should be provided continuous enteral feedings to promote gut adaptation, maximize absorption, and minimize the use of TPN. Infants should also be provided small amounts of nutrition by mouth to maintain sucking and swallowing.

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