Congenital atresias can occur at any level of the gastrointestinal (GI) tract, according to specialists at the University Center for Fetal Medicine. Duodenal atresia (DA) is the most common congenital intestinal obstruction. Over 50 percent of DA cases are associated with other anomalies. Jejunoileal atresia (JA) is usually seen in the proximal jejunum or distal ileum, and on prenatal MRI, it may appear as dilated proximal bowel loops filled with meconium. JA may be associated with cystic fibrosis. Less than 10 percent of all intestinal atresias are colonic atresia (CA). CA is only rarely associated with other anomalies. Pyloric atresia (PA) is exceedingly rare.
Prenatal care includes serial ultrasounds and routine obstetrical visits to assess fetal and maternal well-being.
Fetal MRI may be used to assess structural abnormalities and further assessment of the bowel. Pictured (right) is a MRI of a fetus with duodenal atresia. The "double bubble" sign is indicated with arrows. Increased amniotic fluid also is seen, and it is consistent with polyhydramnios and intestinal obstruction.
In addition to imaging studies, amniocentesis is offered to rule out chromosomal abnormalities. Fetal echocardiogram may be offered to rule out cardiac anomalies. Genetic testing and counseling are offered for family planning purposes.
Delivery should occur at a tertiary care center with pediatric surgery available for intervention. Immediately following delivery, the neonate will be transferred to the neonatal intensive care unit (NICU), where nasogastric (NG) decompression and intravenous (IV) fluids will immediately be started. Central venous access for parenteral nutrition may be necessary. Radiographs (X-rays) will be taken of the abdomen to confirm NG placement and diagnosis obstruction. Other studies that may be performed are abdominal ultrasound, echocardiogram and CT scan.
Corrective surgery should be performed as soon as the neonate is stable enough to undergo the procedure. Following surgery, the neonate will return to the NICU, where status is monitored by an interdisciplinary team of neonatalogists and pediatric surgeons.
Feeds will begin when bowel function has returned. Feeds will be very small at first, but will increase slowly over time until goal has been met. Length of hospital stay varies as discharge from NICU to home is based on several variables, including tolerance of feeds and adequate weight gain and growth.