Objective: Recent data have demonstrated improved survival in children with intestinal failure. We hypothesized that this trend would also be observed in children with ultrashort bowel syndrome.
Methods: A prospective database from Seattle Children's Intestinal Failure Program was used to evaluate outcomes and morbidities of consecutive patients with 10 cm or less of small bowel enrolled in the program since 2005. Data are listed as median (range).
Results: Five patients were identified with a bowel length of 6 (1–10) cm and follow-up of 54 (43–61) months. All children have survived and are currently between 3.5 and 5.5 years of age. One patient underwent isolated intestinal transplantation and one patient is currently listed for intestinal transplantation. The transplanted child is fully enterally fed. The other patients remain at least partially dependent on parenteral nutrition. None of these patients have current evidence of parenteral nutrition-associated liver disease. Patients have required extensive care after referral to our program, including 18 (15–32) visits to the emergency room, 152 (114–273) days of inpatient care, and 6 (5–9) central line–associated blood stream infections.
Conclusions: Long-term survival in children with ultrashort bowel length is possible after referral to an intestinal failure program, although extensive medical management is required. These children may be reasonable candidates for long-standing intestinal rehabilitation as a bridge to intestinal transplantation.