Surgical Innovations

Intestinal Rehabilitation and Transplantation:
Hope for Children with Short Bowel Syndrome

In patients with short bowel syndrome, absorption of nutrients is significantly altered, leading to electrolyte and mineral imbalances and inadequate delivery of calories. art by Nancy Heim

It's all in the details. For children with intestinal failure, the difference between needing organ transplantation or not—or even between life and death—may depend on changes as subtle as adjustments in the balance of their nutrients.

A series of small changes in strategy made a world of difference for one baby girl, an overweight, highly jaundiced nine-month-old with a surgically foreshortened small intestine whose liver was failing as a result of continuous IV nutrition. "The child's skin was virtually green and glowing when she arrived at NewYork-Presbyterian," recalled Steven J. Lobritto, MD, Medical Director, Pediatric Liver Transplantation. He decreased her total caloric load, cycled her intravenous nutrition, increased the nutrition she received via gastric tube, initiated occupational therapy to teach her to take food by mouth, and adjusted the balance between the lipids and other components of her nutrition solution. "Her bilirubin dropped from a high of 17 to a normal level of one, her liver was allowed to heal, and she is now walking and talking like a normal 1^1/2-year-old should," says Dr. Lobritto. "This was a child who had been headed for both liver and intestinal transplant, but is now functioning normally, is no longer hooked to a tube 24-7, and is beginning to take food orally."

At Morgan Stanley Children's Hospital of NewYork-Presbyterian, the Intestinal Rehabilitation and Transplant team has the expertise to provide the most advanced care available to such children. Formed in 2004, the team is one of just several in the U.S. to provide comprehensive, multidisciplinary treatment of intestinal failure. In addition to expertise in the full spectrum of medical therapies, the team specializes in protecting, and when necessary, transplanting the liver and/or bowel.

"Patients with intestinal failure are difficult to treat because of the complexity of their conditions," says Robert A. Cowles, MD, Assistant Professor of Surgery. "Taking nutrition by mouth may not be possible for a long time; they may even need total parenteral nutrition or TPN (nutrition delivered via an intravenous catheter) for the rest of their lives. "While long-term IV nutrition can save patients' lives, recurrent infections and liver toxicity present ongoing challenges. Moreover, blood stream infections, often from intestinal bacteria, can have detrimental effects on the liver. "The combined effects of IV nutrition and these infections can ultimately lead to liver failure. Some children have intestinal failure first, and then go on to have liver failure," says Dr. Cowles.

Surgical procedures such as Serial Transverse Enteroplasty (STEP) can lengthen the bowel in some patients with short bowel syndrome. As a last resort, segments of small bowel may be transplanted into select patients (above). art by Nancy Heim

Intestinal failure, a rare but serious illness affecting primarily newborns and young children, is most commonly caused by diseases resulting in short bowel syndrome. These include necrotizing enterocolitis, intestinal atresia, volvulus, and gastroschisis, intestinal dysmotilities (such as Hirschsprung's disease or intestinal pseudo-obstruction), and congenital enteropathies (such as Microvillous inclusion disease and Tufting syndrome). The loss of intestinal functions leaves patients unable to digest food or grow properly, and susceptible to serious infection related to artificial nutrition. Treatments for short bowel syndrome may include:

• tube feedings (through IV lines, nasogastric or gastric tubes),
• antibiotic therapy, and
• surgery to remove diseased portions of the intestine, elongate healthy intestinal tissue, or connect the bowel to an opening in the abdomen (create a diverting ostomy).

For children with short bowel syndrome, says Dr. Lobritto, there are several critical goals of treatment. "First, we aim to deliver as many calories as possible through oral feeding, because chewing and swallowing enhance the growth of the intestines and nerves. Second, we try to provide optimal balance of calories overall, whether delivered by mouth, feeding tube, or intravenously. Third, we try to protect the liver from damage while awaiting restoration of intestinal sufficiency." Meanwhile, preventing and treating recurrent infections—both major and minor—requires constant vigilance.

"Intestinal transplantation may be an option when TPN has failed due to liver failure, major vein thrombosis, or frequent line-related sepsis," says Dominique M. Jan MD, Professor of Surgery and one of the world's foremost experts in intestinal transplantation. Over 1000 intestinal transplantations have been performed around the world, enabling some patients to resume a normal diet and survive long-term. Nevertheless, transplantation (and the subsequent need for immunosuppression) remains complex, and is considered a last resort. "Whenever possible, we maximize the intestine that the patient has left, and give the bowel a chance to adapt," explains Dr. Cowles. "In this way, we help patients avoid the need for transplantation."

Because most medical centers treat intestinal failure only rarely, unfamiliarity with the nuances of treatments can dramatically affect patients' quality of life and outcomes. "In many cases, it is possible to transform a child's condition through management of their nutritional balance," explains Dr. Lobritto. Adjustments in the ratio between lipids, proteins and glucose can transform some patients from being very sick to very functional. Replacing an intravenous or nasogastric tube with a gastric feeding tube also improves their quality of life. Portable feeding pumps and concealed gastric tubes now allow children to function more normally during the day and take nutrition at night during sleep, and these tubes are not prone to infection to the same degree as IV lines.

While the Intestinal Rehabilitation team is already making a world of difference for patients like the young girl, it is researching multiple ways to improve current treatment options even further. The team is studying the small bowel in order to target bowel disease at earlier stages; it is developing methods of leaving more intestinal length intact when surgery is necessary; and it is investigating key strategies to reduce complications associated with bowel transplantation.