Abstract
Short bowel syndrome (SBS) in neonates is an uncommon but highly morbid condition. As SBS survival increases, physiologic complications become more apparent. Few reports in the literature elucidate outcomes for adults with a pediatric history of SBS. We present a case report of a patient, born with complicated gastroschisis resulting in SBS at birth, who subsequently developed symptoms and pathologic changes of inflammatory bowel disease (IBD) as an adult. The patient lived from age 7, after a Bianchi intestinal lengthening procedure, to age 34 independent of parenteral nutrition (PN), but requiring hydration fluid via G-tube. He was then diagnosed with IBD, after presenting with weight loss, diarrhea, and malabsorption, which required resumption of PN and infliximab treatment. This report adds to a small body of the literature which points to a connection between SBS in neonates and subsequent diagnosis of IBD. Recent evidence suggests that SBS and IBD have shared features of mucosal immune dysfunction and altered intestinal microbiota. We review current treatment options for pediatric SBS as well as multidisciplinary and coordinated transition strategies. We conclude that there may be an etiologic connection between SBS and IBD and that this knowledge may impact outcomes and approaches to care.
References
Wales PW, de Silva N, Kim JH, Lecce L, Sandhu A, Moore AM (2005) Neonatal short bowel syndrome: a cohort study. J Pediatr Surg 40(5):755–762. doi:10.1016/j.jpedsurg.2005.01.037
Reddy VS, Patole SK, Rao S (2013) Role of probiotics in short bowel syndrome in infants and children–a systematic review. Nutrients 5(3):679–699. doi:10.3390/nu5030679
Quiros-Tejeira RE, Ament ME, Reyen L, Herzog F, Merjanian M, Olivares-Serrano N, Vargas JH (2004) Long-term parenteral nutritional support and intestinal adaptation in children with short bowel syndrome: a 25-year experience. J Pediatr 145(2):157–163. doi:10.1016/j.jpeds.2004.02.030
Squires RH, Duggan C, Teitelbaum DH, Wales PW, Balint J, Venick R, Rhee S, Sudan D, Mercer D, Martinez JA, Carter BA, Soden J, Horslen S, Rudolph JA, Kocoshis S, Superina R, Lawlor S, Haller T, Kurs-Lasky M, Belle SH, Pediatric Intestinal Failure C (2012) Natural history of pediatric intestinal failure: initial report from the Pediatric Intestinal Failure Consortium. J Pediatr 161 (4):723–728 e722. doi:10.1016/j.jpeds.2012.03.062
Ziegler TR, Cole CR (2007) Small bowel bacterial overgrowth in adults: a potential contributor to intestinal failure. Curr Gastroenterol Rep 9(6):463–467
Freeman JJ, Rabah R, Hirschl RB, Maspons A, Meier D, Teitelbaum DH (2015) Anti-TNF-alpha treatment for post-anastomotic ulcers and inflammatory bowel disease with Crohn’s-like pathologic changes following intestinal surgery in pediatric patients. Pediatr Surg Int 31(1):77–82. doi:10.1007/s00383-014-3633-4
Levin DN, Marcon MA, Rintala RJ, Jacobson D, Langer JC (2012) Inflammatory bowel disease manifesting after surgical treatment for Hirschsprung disease. J Pediatr Gastroenterol Nutr 55(3):272–277. doi:10.1097/MPG.0b013e31824f617a
Bianchi A (1980) Intestinal loop lengthening—a technique for increasing small intestinal length. J Pediatr Surg 15(2):145–151
Jeppesen PB, Sanguinetti EL, Buchman A, Howard L, Scolapio JS, Ziegler TR, Gregory J, Tappenden KA, Holst J, Mortensen PB (2005) Teduglutide (ALX-0600), a dipeptidyl peptidase IV resistant glucagon-like peptide 2 analogue, improves intestinal function in short bowel syndrome patients. Gut 54(9):1224–1231. doi:10.1136/gut.2004.061440
Jeppesen PB, Gilroy R, Pertkiewicz M, Allard JP, Messing B, O’Keefe SJ (2011) Randomised placebo-controlled trial of teduglutide in reducing parenteral nutrition and/or intravenous fluid requirements in patients with short bowel syndrome. Gut 60(7):902–914. doi:10.1136/gut.2010.218271
Knights D, Lassen KG, Xavier RJ (2013) Advances in inflammatory bowel disease pathogenesis: linking host genetics and the microbiome. Gut 62(10):1505–1510. doi:10.1136/gutjnl-2012-303954
Engstrand Lilja H, Wefer H, Nystrom N, Finkel Y, Engstrand L (2015) Intestinal dysbiosis in children with short bowel syndrome is associated with impaired outcome. Microbiome 3:18. doi:10.1186/s40168-015-0084-7
Carlisle EM, Morowitz MJ (2011) Pediatric surgery and the human microbiome. J Pediatr Surg 46(3):577–584. doi:10.1016/j.jpedsurg.2010.12.018
Till H, Castellani C, Moissl-Eichinger C, Gorkiewicz G, Singer G (2015) Disruptions of the intestinal microbiome in necrotizing enterocolitis, short bowel syndrome, and Hirschsprung’s associated enterocolitis. Front Microbiol 6:1154. doi:10.3389/fmicb.2015.01154
Kim HB, Lee PW, Garza J, Duggan C, Fauza D, Jaksic T (2003) Serial transverse enteroplasty for short bowel syndrome: a case report. J Pediatr Surg 38(6):881–885
Dore M, Junco PT, Andres AM, Sanchez-Galan A, Amesty MV, Ramos E, Prieto G, Hernandez F, Lopez Santamaria M (2016) Surgical Rehabilitation Techniques in Children with Poor Prognosis Short Bowel Syndrome. Eur J Pediatr Surg 26(1):112–116. doi:10.1055/s-0035-1567805
Oh PS, Fingeret AL, Shah MY, Ventura KA, Brodlie S, Ovchinsky N, Martinez M, Lobritto SJ, Cowles RA (2014) Improved tolerance for enteral nutrition after serial transverse enteroplasty (STEP) in infants and children with short bowel syndrome–a seven-year single-center experience. J Pediatr Surg 49(11):1589–1592. doi:10.1016/j.jpedsurg.2014.07.019
Khalil BA, Ba’ath ME, Aziz A, Forsythe L, Gozzini S, Murphy F, Carlson G, Bianchi A, Morabito A (2012) Intestinal rehabilitation and bowel reconstructive surgery: improved outcomes in children with short bowel syndrome. J Pediatr Gastroenterol Nutr 54(4):505–509. doi:10.1097/MPG.0b013e318230c27e
Javid PJ, Sanchez SE, Horslen SP, Healey PJ (2013) Intestinal lengthening and nutritional outcomes in children with short bowel syndrome. Am J Surg 205(5):576–580. doi:10.1016/j.amjsurg.2013.01.013
Matarese LE, O’Keefe SJ, Kandil HM, Bond G, Costa G, Abu-Elmagd K (2005) Short bowel syndrome: clinical guidelines for nutrition management. Nutr Clin Pract 20(5):493–502
Goulet O, Dabbas-Tyan M, Talbotec C, Kapel N, Rosilio M, Souberbielle JC, Corriol O, Ricour C, Colomb V (2010) Effect of recombinant human growth hormone on intestinal absorption and body composition in children with short bowel syndrome. JPEN J Parenter Enteral Nutr 34(5):513–520. doi:10.1177/0148607110362585
Youssef NN, Mezoff AG, Carter BA, Cole CR (2012) Medical update and potential advances in the treatment of pediatric intestinal failure. Curr Gastroenterol Rep 14(3):243–252. doi:10.1007/s11894-012-0262-8
Chan CF, Wu TC (2014) Recent advances in the management of pediatric intestinal failure. Pediatr Neonatol 55(6):426–430. doi:10.1016/j.pedneo.2013.11.005
Stanger JD, Oliveira C, Blackmore C, Avitzur Y, Wales PW (2013) The impact of multi-disciplinary intestinal rehabilitation programs on the outcome of pediatric patients with intestinal failure: a systematic review and meta-analysis. J Pediatr Surg 48(5):983–992. doi:10.1016/j.jpedsurg.2013.02.070
Callahan ST, Winitzer RF, Keenan P (2001) Transition from pediatric to adult-oriented health care: a challenge for patients with chronic disease. Curr Opin Pediatr 13(4):310–316
Cole R, Ashok D, Razack A, Azaz A, Sebastian S (2015) Evaluation of Outcomes in Adolescent Inflammatory Bowel Disease Patients Following Transfer From Pediatric to Adult Health Care Services: Case for Transition. J Adolesc Health 57(2):212–217. doi:10.1016/j.jadohealth.2015.04.012
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Baxter, K.J., Srinivasan, J.K., Ziegler, T.R. et al. Pediatric short bowel syndrome and subsequent development of inflammatory bowel disease: an illustrative case and literature review. Pediatr Surg Int 33, 731–736 (2017). https://doi.org/10.1007/s00383-017-4080-9
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00383-017-4080-9