Ultrashort Bowel Syndrome Outcome in Children Treated in a Multidisciplinary Intestinal Rehabilitation Unit

Abstract

Aim Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in the pediatric population. Our aim was to review long-term outcome of ultrashort bowel syndrome (USBS) in an Intestinal Rehabilitation Unit (IRU).

Patients and Methods Retrospective study of patients with USBS (defined as < 10 cm of remnant small bowel) treated between 2000 and 2015. Demographic data, clinical, and treatment variables including parenteral nutrition (PN), surgical techniques, and intestinal transplantation (IT) were analyzed.

Results Out of 250 children, 30 referred to the IRU met inclusion criteria. Upon first assessment, patients had a median age of 3 (1–217) months and had undergone 3 (1–6) previous laparotomies that left 5 (0–9) cm of remnant small bowel. The main cause of USBS was neonatal midgut volvulus (50%). Follow-up was 28 (4–175) months. Advanced IF-associated liver disease (IFALD) was documented in 63%. None of the patients achieved digestive autonomy and was consequently considered for IT. One patient was excluded, five died before IT, and three are still on the waiting list. Six patients received an isolated IT, 6 a combined liver IT, and 18 a multivisceral graft. Digestive autonomy was achieved in 71% after 31 (14–715) days after IT and currently 62% are alive and off total PN. A significant drop in IFALD progression prior to IT was observed with the introduction of new lipid emulsions in 2010 (SMOF or Soy oil MCT (mid-chain triglycerides) Olive oil Fish oil).

Conclusion A multidisciplinary IRU including an IT program offers a comprehensive approach for patients with IF and is crucial to improve survival rate of USBS. New PN lipid emulsions had an impact on IFALD progression and may eventually reduce overall mortality.

Keywords

intestinal transplantation - ultrashort bowel syndrome - intestinal failure

• References

• 1 Infantino BJ, Mercer DF, Hobson BD , et al. Successful rehabilitation in pediatric ultrashort small bowel syndrome. J Pediatr 2013; 163 (5) 1361-1366
• 2 DeLegge M, Alsolaiman MM, Barbour E, Bassas S, Siddiqi MF, Moore NM. Short bowel syndrome: parenteral nutrition versus intestinal transplantation. Where are we today?. Dig Dis Sci 2007; 52 (4) 876-892
• 3 Demehri FR, Stephens L, Herrman E , et al. Enteral autonomy in pediatric short bowel syndrome: predictive factors one year after diagnosis. J Pediatr Surg 2015; 50 (1) 131-135
• 4 Squires RH, Duggan C, Teitelbaum DH , et al; Pediatric Intestinal Failure Consortium. Natural history of pediatric intestinal failure: initial report from the Pediatric Intestinal Failure Consortium. J Pediatr 2012; 161 (4) 723-8.e2
• 5 Fallon EM, Mitchell PD, Nehra D , et al. Neonates with short bowel syndrome: an optimistic future for parenteral nutrition independence. JAMA Surg 2014; 149 (7) 663-670
• 6 Sanchez SE, Javid PJ, Healey PJ, Reyes J, Horslen SP. Ultrashort bowel syndrome in children. J Pediatr Gastroenterol Nutr 2013; 56 (1) 36-39
• 7 Goulet O, Baglin-Gobet S, Talbotec C , et al. Outcome and long-term growth after extensive small bowel resection in the neonatal period: a survey of 87 children. Eur J Pediatr Surg 2005; 15 (2) 95-101
• 8 Abu-Wasel B, Molinari M. Liver disease secondary to intestinal failure. BioMed Res Int 2014; 2014: 968357
• 9 Kaufman SS, Matsumoto CS. Management of pediatric intestinal failure. Minerva Pediatr 2015; 67 (4) 321-340
• 10 Coletta R, Khalil BA, Morabito A. Short bowel syndrome in children: surgical and medical perspectives. Semin Pediatr Surg 2014; 23 (5) 291-297
• 11 Pakarinen MP. Autologous intestinal reconstruction surgery as part of comprehensive management of intestinal failure. Pediatr Surg Int 2015; 31 (5) 453-464
• 12 Dore M, Junco PT, Andres AM , et al. Surgical rehabilitation techniques in children with poor prognosis short bowel syndrome. Eur J Pediatr Surg 2016; 26 (1) 112-116
• 13 Beath SV, Woodward JM. Intestinal failure-associated liver disease. In: Lanhas AN, Goulet O, Quigley EMM, Tappenden KA, , eds. Intestinal Failures: Diagnosis, Management and Transplantation, 1st ed. Massachusetts: Blackwell Publishing; 2008: 191-200
• 14 Khan FA, Squires RH, Litman HJ , et al; Pediatric Intestinal Failure Consortium. Predictors of enteral autonomy in children with intestinal failure: a multicenter cohort study. J Pediatr 2015; 167 (1) 29-34.e1
• 15 Avitzur Y, Wang JY, de Silva NT , et al. Impact of intestinal rehabilitation program and its innovative therapies on the outcome of intestinal transplant candidates. J Pediatr Gastroenterol Nutr 2015; 61 (1) 18-23
• 16 Sigalet D, Boctor D, Robertson M , et al. Improved outcomes in paediatric intestinal failure with aggressive prevention of liver disease. Eur J Pediatr Surg 2009; 19 (6) 348-353
• 17 Schalamon J, Mayr JM, Höllwarth ME. Mortality and economics in short bowel syndrome. Best Pract Res Clin Gastroenterol 2003; 17 (6) 931-942
• 18 Sudan D, DiBaise J, Torres C , et al. A multidisciplinary approach to the treatment of intestinal failure. J Gastrointest Surg 2005; 9 (2) 165-176 , discussion 176–177
• 19 Torres C, Sudan D, Vanderhoof J , et al. Role of an intestinal rehabilitation program in the treatment of advanced intestinal failure. J Pediatr Gastroenterol Nutr 2007; 45 (2) 204-212
• 20 Hess RA, Welch KB, Brown PI, Teitelbaum DH. Survival outcomes of pediatric intestinal failure patients: analysis of factors contributing to improved survival over the past two decades. J Surg Res 2011; 170 (1) 27-31
• 21 Modi BP, Langer M, Ching YA , et al. Improved survival in a multidisciplinary short bowel syndrome program. J Pediatr Surg 2008; 43 (1) 20-24