Small bowel atresia, also known as intestinal atresia, is a birth defect that affects a part of the small intestine, the tube that connects the stomach to the large intestine and helps digest food.
Depending on the extent of the blockage, the defect is classified as either atresia or stenosis.
Small bowel atresia is classified according to the location along the intestinal tract where the blockage or lack of continuity occurs. The opening between the stomach and the small intestine is called the pylorus. The small intestine is divided into three consecutive parts: the duodenum, jejunum and ileum.
Small bowel atresia is often diagnosed before birth by prenatal ultrasound or fetal MRI if available. Doctors may suspect a fetus has small bowel atresia if a pregnant woman has too much amniotic fluid (polyhydramnios).
Diagnosing fetal small bowel atresia is uncommon before 18 weeks gestation and difficult to detect up to 24 weeks. A prenatal diagnosis of intestinal atresia typically occurs during the third trimester when the bowel becomes easier to see on ultrasound.
The extra fluid in the uterus can make it very uncomfortable for the mother, as well as increase the risk of preterm delivery related to the size and capacity of the uterus.
Polyhydramnios can be associated with all types of small bowel atresia, but is more frequently seen in cases where the obstruction occurs closer to the stomach. Polyhydramnios accompanies duodenal atresia in 53 percent of cases (30-65 percent according to Pediatric Surgery, 7th Edition), and jejunoileal atresia in 25 percent of cases.
Because of the high incidence of associated birth defects, a complete prenatal evaluation and amniocentesis are recommended for mothers carrying babies with small bowel atresia. Related condtions include cystic fibrosis, Trisomy 21 (Down syndrome), congenital heart disease, bowel rotational abnormality, annular pancreas, esophageal atresia, anorectal atresia and genitourinary conditions.
If your baby has been diagnosed with small bowel atresia before birth, we recommend a prenatal pediatric surgical consultation to help explain the details of your diagnosis, discuss delivery options, and answer any questions you have about the surgical procedures and plans for treating your baby after birth.
Doctors may also diagnose a small bowel atresia on an abdominal ultrasound or X-ray after your baby is born. Newborns with small bowel atresia vomit frequently, which is also a sign that something is wrong.
Additional postnatal imaging tests can help to identify or confirm the diagnosis, as well as test for any related birth defects:
Here is an overview of the two more common types of small bowel atresia, including diagnostic indicators and associated conditions:
|1 in 10,000 live births||Most common: 1 in 3,000 live births|
|53% polyhydramnios||25% polyhydramnios|
|"Double bubble"||Vascular disruption|
|17-33% heart disease||10% cystic fibrosis|
|30% Trisomy 21|
There is currently no prenatal treatment option available for small bowel atresia. Close management and monitoring of the pregnancy plays an important role in the ability to offer highly specialized postnatal care and improve outcomes for your child.
Because of the risk of early delivery due to polyhydramnios and need for immediate surgical repair, babies prenatally diagnosed with small bowel atresia should be delivered in a tertiary care center with pediatric surgical and neonatal expertise readily available. If possible we recommend avoiding transport because of the associated physiological changes due to stress and fluid loss in infants with a congenital intestinal atresia.
At CHOP, babies with small bowel atresias have the option of being delivered in the Garbose Family Special Delivery Unit (SDU), allowing for the highest level of immediate care for your baby, as well as expert obstetric services — all within the same pediatric hospital. The world’s first birth facility designed exclusively for pregnancies complicated by birth defects, the SDU is a vital part of our team’s ability to provide comprehensive care. In the SDU, our delivery rooms feature adjacent neonatal stabilization rooms containing all of the equipment, medications and monitoring that your baby might need.
After delivery, your baby will be stabilized by a highly specialized team of pediatric surgeons, neonatologists, specially trained nurses and respiratory therapists, all with extensive experience in treating neonates with small bowel atresia.
If your baby is stable, we will bring her to you for a moment so that you can see her before she is passed through the access window that connects the delivery room to the stabilization room. This is where our multidisciplinary Neonatal Surgical Team will be waiting, ready to provide whatever care your baby needs.
This is what you can expect while your baby is being stabilized:
After your baby is stabilized in the SDU, she is brought down special elevators to the Newborn/Infant Intensive Care Unit (N/IICU), which is located directly below the SDU. From there, she will be prepared for the operating room. Our Neonatal Surgical Team can also perform surgery in the N/IICU, reducing risk of transport for high-risk neonates.
Surgical repair of a bowel obstruction is usually performed in the first days of life, as soon as your baby is stabilized.
During the operation, the surgical team will make an incision in your child's abdomen so that they can examine the stomach and intestines and locate the blocked part of the small intestine. They will open up the blocked end of the intestine and connect it to the area of small bowel just past the blockage. This is called resecting and anastomosing the bowel.
Minimally invasive surgical techniques are also available at CHOP, and this may be a potential option for your baby. Your surgeon will discuss all options with you.
After surgery, your child will be transported to the N/IICU for recovery. Post-surgical treatment is focused on getting your child’s bowel to regain function so that it can operate on its own.
Here's what else to expect after surgery:
Feedings will begin slowly. Breast milk, if available, will first be given through the feeding tube, increasing in volume over time as your baby tolerates feedings. She can begin to feed by mouth once she demonstrates tolerance to half of her goal feeding.
Putting baby to breast has many benefits. While we are waiting for your baby to increase feedings, your care team will work with you to do non-nutritive sucking, allowing your baby to suck without taking milk. Non-nutritive sucking gives your baby a chance to practice at the breast, encouraging sucking and weight gain. This is particularly important for sick or premature babies who haven’t been able to suck at the breast or from a bottle because of their condition.
CHOP’s Breastfeeding and Lactation Program can provide resources and support services to help you with your breastfeeding efforts.
The time from initial feeding until full feedings can be a long and challenging course for you and your baby. It may take many days, sometimes weeks, depending on the extent of the atresia, but our feeding and lactation teams are available to support you every step along the way.
Your child will be discharged from the hospital when she is able take in enough feeding volume to provide good nutrition, and is gaining weight.
Once your child comes home, she may have formula or breast milk.
Two weeks after discharge, you'll need to take your child for a follow-up appointment with her surgeon.
Please call your child's doctor (at Children's Hospital, call 215-590-2730) if:
Infants who are treated for small bowel atresia face several potential long-term complications, including mechanical bowel problems, feeding and absorption difficulties. For these reasons, your child may require specialized follow-up care.
Nutrition is a significant issue for children with small bowel atresia, so it is important for your child to see a nutritionist to track growth. Occasionally, if your child is not taking enough nutrition by mouth, we will send your child home with a temporary feeding tube though her nose. This ensures that your child will have adequate nutrition and allows you spend time safely with your child as a family at home.
Patients who undergo surgery for small bowel atresia may receive long-term care through the Division of Gastroenterology, Hepatology and Nutrition’s Intestinal Rehabilitation Program (IRP). This program provides specialized care for children with intestinal complications who are unable to absorb sufficient nutrients, fluid and electrolytes that are necessary to sustain life and support growth.
Your child will have access to inpatient and outpatient care from a multidisciplinary team that consists of physicians, surgeons, dietitians, social workers and a nurse coordinator. Your family will work closely with the team to formulate an integrated treatment plan to optimize care for your child.
Babies born with a small bowel atresia today have excellent long-term outcomes. Over the last 50 years, survival rates have increased to 90 percent, with almost all mortality related to associated anomalies.