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Short bowel syndrome and intestinal transplantation in children
Olivier Goulet1, Frédérique Sauvat
1Integrated 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
Insights
Pediatric short bowel syndrome management requires a multidisciplinary approach. With appropriate care, including parenteral nutrition and tailored therapies, the prognosis for children with short bowel syndrome is excellent.
Area of Science:
- Pediatric Gastroenterology
- Intestinal Failure Research
- Surgical Innovation
Background:
- Short bowel syndrome (SBS) in children results from extensive intestinal resection, necessitating parenteral nutrition.
- The condition poses significant challenges in pediatric care, impacting growth and development.
Purpose of the Study:
- To review current knowledge and clinical practices for managing pediatric SBS.
- To highlight advancements in diagnosis, treatment, and long-term outcomes.
Main Methods:
- Comprehensive literature review of recent studies on pediatric SBS.
- Analysis of epidemiological data, diagnostic markers, and treatment modalities.
- Evaluation of surgical and transplant outcomes.
Main Results:
- Plasma citrulline emerges as a marker for intestinal mass in intestinal failure.
- Increased incidence of SBS-related gastroschisis and persistent necrotizing enterocolitis noted.
- Non-transplant surgery offers a favorable option; isolated liver transplantation may be avoidable.
Conclusions:
- Management necessitates a multidisciplinary approach tailored to intestinal failure stages.
- Prognosis for SBS is excellent with appropriate care, limiting transplant indications.
- Timely referral to specialized centers is crucial for optimal patient outcomes.
Purpose Of Review:
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.
Recent Findings:
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.
Summary:
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.
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