Jejunoileal Atresia

Jejunoileal atresia is the most common GI atresia; it occurs in approximately 1 in 2000 live births. It is thought to occur as a result of an intrauterine mesenteric vascular occlusion. Atresias occur slightly more frequently in the jejunum than in the ileum. Jejunoileal atresias are classified as type I, a mucosal web or diaphragm ( Fig. 67-10 A ), type II, with an atretic cord between two blind ends of bowel with intact mesentery, type IIIa, a complete separation of the blind ends of the bowel by a V-shaped mesenteric gap, and type IIIb, an apple peel or Christmas tree deformity with a large mesenteric gap (see Fig. 67-10 B), in which the distal bowel receives a retrograde blood supply from the ileocolic or right colic artery. This tenuous blood supply has implications for reanastomosis and the potential for ischemic necrosis caused by an antenatal volvulus. Thus, many of these infants with this type of atresia are born with reduced intestinal length. Finally, in type IV, there are multiple atresias, with a string of sausage appearance.

FIGURE 67-10 Jejunoileal atresia. A, Massively dilated jejunum with narrow distal bowel segment. B, Apple peel type of atresia with a large mesenteric gap.

Clinical Presentation
Infants present with bilious emesis, abdominal distention, and failure to pass meconium. Typically, the overall clinical presentation is dependent on the level of obstruction. In proximal atresia, abdominal distention is less, but significant bilious emesis is present. Plain abdominal radiographs show air-fluid levels with absent distal gas. With distal atresias,

abdominal distention is present more frequently. A barium enema demonstrates a small unused colon and may also be useful to exclude multiple atresias, which may be present in 10% to 15% of cases. Jejunoileal atresia is typically not associated with other anomalies except cystic fibrosis (CF) in approximately 10% of patients.

Treatment: Surgical Management

Infants are managed in a manner similar to that for other conditions of neonatal bowel obstruction. An orogastric tube is placed and appropriate IV fluid resuscitation is implemented. At operation, the main goal is to reestablish intestinal continuity while preserving as much intestinal length as possible. In multiple atresias, multiple anastomoses over an endoluminal stent may be necessary. If the proximal intestine is significantly dilated, prolonged dysmotility may persist and, therefore, a tapering enteroplasty of the dilated bowel should be considered. However, in cases of adequate bowel length, resection of the dilated bowel segment can result in faster recovery. The overall survival for infants with jejunoileal atresia is more than 90%.