Fetal intestinal atresia is a congenital defect in which a part of the intestines fail to form properly resulting in an obstruction or blockage. See the normal stomach and small intestines to the right.
There are several types of intestinal atresia, but the most common are duodenal and jejunoileal. The small intestine is made up of several parts: the duodenum, the first section that the stomach empties into; the jejunum, the middle section; and the ileum, the lower section. Newborns with duodenal atresia generally vomit soon after birth, and their abdomens appear swollen. It is very uncommon to have atresia of the large intestine (colon). The appearance of the fetal ultrasound often gives clues as to the area of blockage.
When the first part of the small bowel is blocked, there is only a small amount of dilated or "ballooned" intestine seen (illustration to the right). When the blockage is lower, multiple loops of dilated intestine are seen (illustration below).
Babies with jejunoileal atresia suffer from an obstruction of the jejunum or ileum resulting in a large amount of swelling that blocks nutrients from moving through the digestive tract normally.
It is estimated that one in every 1,500 live births is affected by intestinal atresia. About half of these babies are born prematurely. Fifty to 75% of babies with this condition are born with other birth defects including congenital heart disease, kidney problems or gastrointestinal abnormalities, and about 30% have Down syndrome.
Other conditions that can mimic intestinal atresia include cystic fibrosis and meconium plugs in the fetal bowel.
Fetal intestinal atresia can be diagnosed by ultrasound (sonogram) examination prior to birth. Evaluation of the bowels is part of the routine ultrasound examination done by many obstetricians as part of their routine prenatal care around the 20th week of pregnancy.
If fetal intestinal atresia is diagnosed before birth, the staff at Lurie Children’s Institute for Fetal Health counsels the parents and prepares them for what to expect when their baby is born. Plans are made to assure that the baby is delivered in a hospital that has a neonatal intensive care unit (NICU) and a neonatologist present at the delivery.
After birth, the baby is stabilized and then transferred via ambulance to Lurie Children's to begin treatment. All intestinal atresia conditions require surgery. In most cases, the part of the intestine that has the obstruction is removed, and the healthy segments of the intestine are reconnected. Surgery becomes more complex if there are multiple obstructions or damage to multiple segments of the intestine. Children who undergo this type of surgery usually remain in the hospital for one to several weeks until they can tolerate regular feedings. In carefully selected cases, the surgery can be completed using 'minimally invasive surgery' which shortens the recovery time and minimizes skin incisions.
The long-term outlook for these children depends on how much of their intestines surgeons were forced to remove. Another contributing factor to their long-term health is whether they are born with other birth defects associated with this condition. Many babies need regular follow-up care after they return home due to the other defects and to monitor their progress.