Jejunoileal Atresia

Jejunoileal atresias occur as a result of an ischemic insult during pregnancy. The ischemic insult can be due to intussusception, perforation, volvulus, intestinal strangulation via a hernia, or thromboembolism. Maternal smoking and cocaine use have been associated with intestinal atresia (1).

The estimated incidence is approximately 1 in 10,000 live births. Jejunoileal atresias are equally distributed between the jejunum and ileum (2–5).

Associated congenital anomalies are seen in up to half of cases (6). The most common are urogenital anomalies.

The most common associated conditions are congenital heart disease, cystic fibrosis, malrotation, and gastroschisis.

Peritoneal calcifications suggest the presence of meconium peritonitis, which is a sign of intrauterine intestinal perforation and can be seen in approximately 10% of cases. The presence of meconium peritonitis should raise suspicion of meconium ileus and cystic fibrosis (5).

There are 5 major types of jejunoileal atresia (1):

• 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).

• Prenatal ultrasound or fetal MRI

• Abdominal radiograph

Prenatally, ultrasound showing dilated loops of small bowel and polyhydramnios, or fetal MRI may suggest or confirm the diagnosis.

Postnatally, a plain abdominal radiograph is performed; it 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).

Jejunal Atresia

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This radiograph of an infant with proximal jejunal atresia shows large dilated loops in the upper abdomen with no distal gas.

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Because 7 to 10% of patients also have cystic fibrosis (nearly 100% if meconium ileus is also present), testing for cystic fibrosis should be performed (1, 2). Screening for associated anomalies should also be considered.

• Surgical repair

Preoperative management of jejunoileal atresia consists of placing a nasogastric tube, 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. A procedure that tapers the dilated proximal loop is also a consideration (1, 2).

The prognosis for infants with jejunoileal atresia is very good with > 90% survival (3, 4). 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 parenteral nutrition (PN) for extended periods (5). They should be provided continuous enteral feedings to promote gut adaptation, maximize absorption, and minimize the use of PN. Infants should also be provided small amounts of nutrition by mouth to maintain sucking and swallowing. Prognosis for infants with ultrashort bowel syndrome has improved significantly because of surgical techniques including bowel-lengthening procedures (eg, serial transverse enteroplasty procedure or STEP), improved medical care, and the ability to perform small bowel transplantation (6–8).