Center for Advanced Intestinal Rehabilitation | Research and Innovation

The Center for Advanced Intestinal Rehabilitation (CAIR) at Boston Children’s Hospital is committed to moving patient care forward through research. The group recently published three pioneering studies evaluating outcomes in children with short bowel syndrome (SBS) who achieve enteral autonomy, or who fully wean from parental nutrition (PN).

Children with SBS require PN to grow and develop properly, but its long-term use is associated with liver failure and other life-threatening complications. Although the advent of liver-protective drugs such as Omegaven is associated with improved mortality, enteral autonomy remains the ultimate goal of care for children with SBS. These recent findings promise to advance the treatment of SBS patients.

Most common cause of SBS associated with weaning success

A group of investigators from the CAIR program reviewed data from 109 patients treated over a 12-year period to evaluate predictors of enteral autonomy. They found that children with the most common cause of SBS, necrotizing enterocolitis (NEC), were more likely to wean from PN in comparison to those with other primary causes of SBS. Specifically, 64.9 percent of NEC patients achieved enteral autonomy in the study period versus 29.2 percent of those with other underlying diagnoses. These findings suggest that short bowel patients with NEC may have particularly favorable outcomes of intestinal rehabilitation.

Intestinal rehabilitation improves odds of enteral autonomy

Another review conducted by our group examined outcomes for 63 children with SBS who were followed in our institution. They found that the likelihood of achieving enteral autonomy was 96 percent after 24 months in patients with at least 50 centimeters of small intestine, and 71 percent after 57 months in patients with less than 50 cm of small intestine.

Entirety of care within our institution was significantly and independently associated with weaning from PN. These findings suggest that intestinal rehabilitation can take time, but is eventually successful in a large number of patients, even those with very short residual intestinal length. Follow-up in a specialty program such as CAIR may improve outcomes.

Enteral autonomy improves transplant-free survival

Despite a history of cirrhosis diagnosed on liver biopsy, the CAIR team has observed that some patients remain stable for prolonged periods of time. To further explore this observation, they examined transplant-free survival outcomes in 313 patients followed over a 12-year period. The researchers found no association between transplant-free survival and a diagnosis of cirrhosis in this group. Furthermore, overall 1-year survival outcomes were 98.2 percent in patients who achieved enteral autonomy and 90.3 percent for those who didn’t. Although transplant is indicated for some patients with SBS and associated complications, these findings indicate that the isolated finding of cirrhosis on liver biopsy may not require automatic referral for transplant and highlights the importance of intestinal rehabilitation.

The CAIR team is committed to doing everything they can to mitigate the risks and common complications of SBS, and to help patients achieve enteral autonomy whenever possible. In the event that transplant is indicated or desired, CAIR also has a collaborative relationship with the Boston Children’s transplant program and can provide access to this type of care as well.

Serial Transverse Enteroplasty (STEP) Procedure

In 2002, Boston Children's doctors performed the world's first serial transverse enteroplasty (STEP) procedure, a surgical technique developed by Tom Jaksic, MD and Heung Bae Kim, MD, which lengthens the bowels of children with short bowel syndrome.

Since its invention, Boston Children's doctors have performed 19 STEP procedures with increasing success, establishing Boston Children's Hospital as one of the preeminent hospitals in the world for the treatment of short bowel syndrome.

Finally, watch and listen to Drs. Kim, Jaksic and Duggan below as they describe the history and benefits of STEP.

How STEP works