Intestinal Atresia or Stenosis

Occasionally, the diagnosis of jejunoileal atresia (blockage of the small intestine) may be suspected when polyhydramnios (excess fluid around the baby and enlarged loops of intestine are observed on ultrasound on the fetus. Typically, however, a newborn with jejunoileal atresia presents with green/yellow vomiting, distention of the abdomen, and failure to have a bowel movement on the first day of life. Distention of the abdomen may vary depending on the level of the blockage. Although atypical, passage of stool may occur in the setting of a jejunoileal atresia which has developed later in gestation. X-rays of the abdomen often demonstrate a single large loop of enlarged, air-filled intestine just upstream from the atresia. An X-ray with dye placed into the rectum eliminates the possibility of blockage of the large intestine and typically an unusually small colon unless the blockage occurred late in gestation.

Three different types of jejunoileal atresia are observed: Type I (19%) consists of a single, thin web or diaphragm which blocks the intestine; type II (31%) a cord of scar between the upstream and downstream ends; and type III (50%) is an atresia in which a portion of the intestine is completely missing. All are treated similarly. A timely, but not emergent, operation is typically performed. An incision with upper abdomen typically reveals a very large upstream bowel and a small downstream portion of the intestine. Some of the upstream portion must be removed because it will not work correctly. The two ends will still be different in size and, therefore, a special "end-to-back" joining of the two ends must be performed. Since between 6-20% of newborns may have more than one atresia, downstream small intestine must be insufflated with water or air and examined carefully for the presence of another blocked area of intestine. Blockage of the colon is rarely observed, but the treatment is similar to that of jejunoileal atresia.

Survival or complications are generally affected by associated problems such as prematurity, heart disease, or insufficient intestine (short gut syndrome). Problems where the intestinal ends are joined, such as leak or stricture (narrowing), occur in approximately 5% of cases. It commonly will require a number of days to weeks before feeding may be tolerated. Most infants with more than 15 cm of remaining small intestine and an ileocecal valve (where the small intestine meets the large intestine) or more than 40 cm of small intestine if the ileocecal valve has been removed will survive.

This information is provided by the University of Michigan Department of Surgery, Section of Pediatric Surgery and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. For additional health information, please contact your health care provider or our offices.