Short bowel syndrome and intestinal transplantation in children.
OBJECTIVE
This review summarizes recent knowledge and clinical practice for pediatric patients suffering extensive intestinal resection causing short bowel syndrome. This condition requires the use of parenteral nutrition, as long as intestinal failure persists, and may be, in some selected cases, an indication for intestinal transplantation.


RESULTS
Biological evaluation of intestinal failure is becoming possible with the use of plasma citrulline as a marker of intestinal mass. Few epidemiological data are available; some indicate an increased incidence of short bowel syndrome-related gastroschisis and persistent high incidence of necrotizing enterocolitis. Morbidity and mortality data in pediatric patients with short bowel syndrome are limited, while long-term outcome is better documented from recently reported cohorts. Non-transplant surgery is one of the best options for patients with unadapted short bowel syndrome. Isolated liver transplantation may be avoided. The use of trophic factors for enhancing mucosal hyperplasia still remains disappointing.


CONCLUSIONS
The management should include therapies adapted to each stage of intestinal failure, based on a multidisciplinary approach in centers involving pediatric surgery, pediatric gastroenterology, parenteral nutrition expertise, home-parenteral nutrition program, and liver-intestinal transplantation experience. If managed appropriately, the prognosis of short bowel syndrome is excellent, with limited indications for intestinal and/or liver transplantation. Timing for patient referral in specialized centers remains an issue.
Goulet
Olivier
O
Integrated Program of Intestinal Failure, Home Parenteral Nutrition, and Intestinal Transplantation, National Reference Center for Rare Digestive Diseases, Necker Hospital for Sick Children, University of Paris, France. olivier.goulet@nck.aphp.fr
Sauvat
Frédérique
F
D015415:Biomarkers / Q000097:blood; D002648:Child; D002956:Citrulline / Q000097:blood; D006801:Humans; D007421:Intestine, Small / Q000601:surgery / Q000637:transplantation*; D008107:Liver Diseases / Q000209:etiology / Q000517:prevention & control* / Q000628:therapy; D016031:Liver Transplantation; D010288:Parenteral Nutrition; D011379:Prognosis; D012778:Short Bowel Syndrome / Q000097:blood / Q000453:epidemiology / Q000601:surgery / Q000628:therapy*; D016896:Treatment Outcome
D015415:Biomarkers; D002956:Citrulline