Lorenzo Norsa1, Solene Artru2, Cecile Lambe2, Cecile Talbotec2, Benedicte Pigneur2, Frank Ruemmele3, Virginie Colomb2, Carmen Capito4, Christophe Chardot5, Florence Lacaille2, Olivier Goulet3. 1. Department of Pediatric Gastroenterology, Hepatology and Nutrition, Intestinal Failure Rehabilitation Center, Hôpital Universitaire Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France. Electronic address: lonorsa@hotmail.com. 2. Department of Pediatric Gastroenterology, Hepatology and Nutrition, Intestinal Failure Rehabilitation Center, Hôpital Universitaire Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France. 3. Department of Pediatric Gastroenterology, Hepatology and Nutrition, Intestinal Failure Rehabilitation Center, Hôpital Universitaire Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France; Faculté de Médecine, Universitè of Sorbonne-Paris-Cité, Paris Descartes, 15 Rue de l'École de Médecine, 75006 Paris, France. 4. Department of Pediatric Surgery and Transplantation, Hôpital Universitaire Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France. 5. Department of Pediatric Surgery and Transplantation, Hôpital Universitaire Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France; Faculté de Médecine, Universitè of Sorbonne-Paris-Cité, Paris Descartes, 15 Rue de l'École de Médecine, 75006 Paris, France.
Abstract
BACKGROUND & AIMS: Intestinal rehabilitation is the preferred treatment for children with short bowel syndrome (SBS) whatever the residual bowel length, and depends on the accurate management of long-term parenteral nutrition (PN). If nutritional failure develops, intestinal transplantation (ITx) should be discussed and may be life-saving. This study aimed to evaluate survival, PN dependency and nutritional status in children with neonatal very SBS on PN or after ITx, in order to define indications and timing of both treatments. PATIENTS AND METHODS: This retrospective cross-sectional study enrolled 36 children with very SBS (<40 cm) who entered our intestinal rehabilitation program from 1987 to 2007. RESULTS: All the children on long-term PN (n = 16) survived with a follow-up of 17 years (9-20). Six of them were eventually weaned off PN. Twenty children underwent ITx: eight children died (40%) 29 months (0-127) after Tx. The others 12 patients were weaned off PN 73 days (13-330) after Tx. Follow-up after transplantation was 14 years (6-28). Seven out of 8 (88%) patients with a history of gastroschisis required ITx. Patients who required ITx had longer stoma duration. CONCLUSION: Survival rate of children with very short bowel was excellent if no life-threatening complications requiring transplantation developed. Gastroschisis and delayed ostomy closure are confirmed as risk factor for nutritional failure. Intestinal rehabilitation may allow a total weaning of PN before adulthood. A follow-up by a multidisciplinary team is necessary to avoid PN complications in order to minimize indications for ITx.
BACKGROUND & AIMS: Intestinal rehabilitation is the preferred treatment for children with short bowel syndrome (SBS) whatever the residual bowel length, and depends on the accurate management of long-term parenteral nutrition (PN). If nutritional failure develops, intestinal transplantation (ITx) should be discussed and may be life-saving. This study aimed to evaluate survival, PN dependency and nutritional status in children with neonatal very SBS on PN or after ITx, in order to define indications and timing of both treatments. PATIENTS AND METHODS: This retrospective cross-sectional study enrolled 36 children with very SBS (<40 cm) who entered our intestinal rehabilitation program from 1987 to 2007. RESULTS: All the children on long-term PN (n = 16) survived with a follow-up of 17 years (9-20). Six of them were eventually weaned off PN. Twenty children underwent ITx: eight children died (40%) 29 months (0-127) after Tx. The others 12 patients were weaned off PN 73 days (13-330) after Tx. Follow-up after transplantation was 14 years (6-28). Seven out of 8 (88%) patients with a history of gastroschisis required ITx. Patients who required ITx had longer stoma duration. CONCLUSION: Survival rate of children with very short bowel was excellent if no life-threatening complications requiring transplantation developed. Gastroschisis and delayed ostomy closure are confirmed as risk factor for nutritional failure. Intestinal rehabilitation may allow a total weaning of PN before adulthood. A follow-up by a multidisciplinary team is necessary to avoid PN complications in order to minimize indications for ITx.
Authors: Helena A S Goldani; Marilia R Ceza; Liege L Godoy; Juliana M Giesta; Simone Beier; Juliana G Oliveira; Daltro L Nunes; Leticia Feldens; Iara R S Lucena; Adriano N R Taniguchi; Silvia C Hallberg; Daiane Durant; Simone Boettcher; Marcia A Schneider; Patricia P Mello; Mariana G L Riberg; Alana V Signorini; Cristina Miller; Berenice L Santos; Claudete O Silveira; Maira C M Morais; Terezinha V Laggazio; Carla C Costa; Carlos O Kieling Journal: J Pediatr Gastroenterol Nutr Date: 2022-05-17 Impact factor: 3.288