Intestinal atresia and stenosis Pediatric Research and Clinical Trials

Boston Children's Hospital is home to the world’s most extensive research enterprise at a pediatric hospital. We also have many partnerships with research, biotech and health care organizations, and we work together to find innovative ways to improve kids’ health.

A new surgery

Problem: The small intestine is a very important part of the digestive tract – it’s where we absorb most of the nutrients from the food we eat. But sometimes children may not be able to absorb all the nutrients their growing bodies require. This could happen because their intestines don’t work as they should, or because they don’t have enough small intestine, often as the result of surgery to correct congenital defects including intestinal atresia and stenosis. 

When the child can’t absorb enough nutrition from her short intestines to meet her nutritional requirements, she has short bowel syndrome and is at risk for serious malnutrition, with all of its complications. 

If a child doesn’t have enough small intestine, the remaining part tries to fix the problem on its own. It puffs up like a balloon, creating more surface area to draw in nutrients, just as we might set out more buckets if we wanted to collect more rainwater. But this has an unfortunate side effect: the wider the intestine, the longer it takes for the body to move nutrients through it. (Imagine holding a tube with water flowing through it. Now imagine squeezing the tube – the water flows faster.) More time in the intestines means more time for the bacteria that would normally be swept promptly along to multiply, increasing your child’s chance of infection.

Solution: Children’s physicians Heung Bae Kim, MD, surgical director of the Pediatric Transplant Center, and Tom Jaksic, MD, PhD, surgical director of CAIR, figured out a way to solve both problems at once. The procedure is called serial transverse enteroplasty (STEP).

STEP solves both problems:

1. It exposes more usable surface area, similar to the way that sharpening a pencil exposes more lead.

2. It creates a narrower space, which keeps food moving through your child’s digestive tract at an appropriate pace, carrying bacteria out of her body along with the rest of the body’s waste.

Watch a video of an actual STEP procedure.

Results: The STEP procedure is now being used worldwide, allowing some children to wean completely from IV nutrition and avoid the need for intestinal transplantation.  In the spring of 2004, the STEP Data Registry was started to track outcomes of children who have had the STEP procedure, so that we can better understand its effectiveness compared to other bowel-lengthening procedures.

 Learn more about the STEP procedure.

Smarter nutrition

Problem: Some children with intestinal failure can only receive nutrients intravenously (through an IV). But prolonged use of IV feedings often damages the liver, which can lead to liver failure and the need for a liver transplant.

Solution: Children’s researchers Mark Puder, MD, Jenna Garza, MD, and Kathy Gura, PharmD discovered that the then-standard formula included a fat mixture that made fat accumulate in the liver, which may contribute to liver disease. They discovered that switching from the old fat to Omegavan, a fat mixture made from fish oil, (which has been shown to prevent fat accumulation) resulted in a formula that’s far less damaging to the liver.  

Result: This discovery has caused a worldwide shift in treatment. Puder and colleagues are now conducting a formal clinical trial to learn how it can be used even more effectively.