Jejunoileal Atresia

Jejunoileal atresias occur as a result of an ischemic insult during pregnancy. The ischemic insult can be due to intussusception Intussusception Intussusception is telescoping of one portion of the intestine (intussusceptum) into an adjacent segment (intussuscipiens), causing intestinal obstruction and sometimes intestinal ischemia.... read more , perforation Acute Perforation of the Gastrointestinal Tract Any part of the gastrointestinal tract may become perforated, releasing gastric or intestinal contents into the peritoneal space. Causes vary. Symptoms develop suddenly, with severe pain followed... read more , volvulus, intestinal strangulation via a hernia, or thromboembolism. Maternal smoking and cocaine use have been associated with intestinal atresia. There is an estimated incidence of about 1 in 10,000 live births. This disorder affects both sexes equally. Jejunoileal atresias are equally distributed between the jejunum and ileum (1 Etiology reference Jejunoileal atresia is incomplete formation of part of the small intestine. Diagnosis is by abdominal x-ray. Treatment is surgical repair. (Also see Overview of Congenital Gastrointestinal Anomalies... read more ).

Associated congenital anomalies are less common with jejunoileal atresia than duodenal atresia Duodenal atresia The duodenum can be obstructed by atresia, stenosis, and pressure due to an extrinsic mass. (See also Overview of Congenital Gastrointestinal Anomalies.) This anomaly is the 2nd most common... read more . The most common associated conditions are cystic fibrosis Cystic Fibrosis Cystic fibrosis is an inherited disease of the exocrine glands affecting primarily the gastrointestinal and respiratory systems. It leads to chronic lung disease, exocrine pancreatic insufficiency... read more , malrotation Malrotation of the Bowel Malrotation of the bowel is failure of the bowel to assume its normal place in the abdomen during intrauterine development. Diagnosis is by abdominal x-ray. Treatment is surgical repair. (See... read more , and gastroschisis Gastroschisis Gastroschisis is protrusion of the abdominal viscera through a full-thickness abdominal wall defect, usually to the right of the umbilical cord insertion. (See also Overview of Congenital Gastrointestinal... read more , each of which is present in about 10% of cases. Peritoneal calcifications suggest the presence of meconium peritonitis, which is a sign of intrauterine intestinal perforation and can be seen in about 10% of cases. The presence of meconium peritonitis should raise suspicion of meconium ileus Meconium Ileus Meconium ileus is obstruction of the terminal ileum by abnormally tenacious meconium; it most often occurs in neonates with cystic fibrosis. Meconium ileus accounts for up to 33% of neonatal... read more and cystic fibrosis (1 Etiology reference Jejunoileal atresia is incomplete formation of part of the small intestine. Diagnosis is by abdominal x-ray. Treatment is surgical repair. (Also see Overview of Congenital Gastrointestinal Anomalies... read more ).

There are 5 major types of jejunoileal atresia:

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 cystic fibrosis Diagnosis Cystic fibrosis is an inherited disease of the exocrine glands affecting primarily the gastrointestinal and respiratory systems. It leads to chronic lung disease, exocrine pancreatic insufficiency... read more should be done.

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 Treatment references Jejunoileal atresia is incomplete formation of part of the small intestine. Diagnosis is by abdominal x-ray. Treatment is surgical repair. (Also see Overview of Congenital Gastrointestinal Anomalies... read more ).

The prognosis for infants with jejunoileal atresia is very good with > 90% survival. Prognosis is based on the length of remaining small bowel and the presence of the ileocecal valve. Infants who subsequently develop short bowel syndrome Short Bowel Syndrome Short bowel syndrome is malabsorption resulting from extensive resection of the small bowel (usually more than two thirds the length of the small intestine). Symptoms depend on the length and... read more require total parenteral nutrition (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. Prognosis for infants with ultrashort bowel syndrome has improved significantly because of newer surgical techniques including bowel-lengthening procedures (eg, serial transverse enteroplasty procedure or STEP), improved medical care, and the ability to do small bowel transplantation (2 Treatment references Jejunoileal atresia is incomplete formation of part of the small intestine. Diagnosis is by abdominal x-ray. Treatment is surgical repair. (Also see Overview of Congenital Gastrointestinal Anomalies... read more , 3 Treatment references Jejunoileal atresia is incomplete formation of part of the small intestine. Diagnosis is by abdominal x-ray. Treatment is surgical repair. (Also see Overview of Congenital Gastrointestinal Anomalies... read more , 4 Treatment references Jejunoileal atresia is incomplete formation of part of the small intestine. Diagnosis is by abdominal x-ray. Treatment is surgical repair. (Also see Overview of Congenital Gastrointestinal Anomalies... read more ).