A description of the management and outcomes of infants with short bowel syndrome in a South African context
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Abstract
Background. Intestinal failure because of congenital or acquired massive bowel loss is an extremely difficult problem to manage and has traditionally been associated with a very poor outcome.
Objective. To describe the current management of short bowel syndrome (SBS) and the factors associated with survival and achievement of enteral autonomy in a South African context.
Methods. A retrospective chart review of children managed with intestinal failure due to SBS was conducted at Inkosi Albert Luthuli Central Hospital, South Africa, from November 2015 to February 2023.
Results. A total of 22 patients with intestinal failure were managed during the study period. The diagnoses included: type 4 jejunal atresia with 3b component (n=5; 22.7%); type 3b jejunal atresia (n=5; 22.7%); type 4 jejunal atresia without 3b component (n=3; 13.6%) and malrotation with volvulus (n=3; 13.6%). One patient each had necrotising enterocolits, gastroschisis with atresia, intussusception, type 1, jejunal atresia with volvulus, type 3a jejunal atresia and volvulus around an ileostomy. The median bowel length was 36.5 cm and average length of stay was 122 days. Enteral autonomy was achieved in 10 patients (45%) and survival in 9 patients (41%). Surgical complication requiring early re-operation (odds ratio 18) and bowel length ≤20 cm were associated with non-survival.
Conclusion. A substantial proportion of children with intestinal failure can achieve enteral autonomy and survival in our context. The treatment process is, however, resource intensive. Avoiding early re-operation and bowel length >20 cm are associated with better survival.
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References
Ziegler MM. Short bowel syndrome in infancy: Etiology and management. Clin Perinatol 1986;13(1):163-173. https://doi.org/10.1016/S0095-5108(18)30844-3.
Andorsky DJ, Lund DP, Lillehei CW, et al. Nutritional and other postoperative
management of neonates with short bowel syndrome correlates with clinical outcomes. J Pediatr 2001;139(1):27-33. https://doi.org/10.1067/ mpd.2001.114481.
Modi BP, Langer M, Avery Ching Y, et al. Improved survival in a multidisciplinary short bowel syndrome program. J Pediatr Surg 2008;43(1):20-24. https://doi. org/10.1016/j.jpedsurg.2007.09.014.
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. https://doi.org/10.1097/MPG.0b013e31805905f9.
Nucci A, Burns C, Armah T. Interdisciplinary management of pediatric intestinal failure: A 10-year review of rehabilitation and transplantation. J Gastrointest Surg 2008;12(3):429-436. https://doi.org/10.1007/s11605-007-0444-0.
Hess RA, Welch KB, Brown PI. 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. https://doi.org/10.1016/j. jss.2011.03.037.
Stanger JD, Oliveira C, Blackmore C. 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 2013;48(5):983- 992. https://doi.org/10.1016/j.jpedsurg.2013.02.070.
Saayman BD, Millar AJW, van Niekerk E. Nutritional interventions and outcomes of children with short bowel syndrome in a tertiary hospital setting in South Africa. S Afr J Clin Nutr 2022;35(2):1-8. https://doi.org/10.1080/160 70658.2022.2114404.
Wilmore DW. Factors correlating with a successful outcome following extensive intestinal resection in newborn infants. J Pediatr 1972;80(1):88-95. https://doi. org/10.1016/s0022-3476(72)80459-1.
Spencer AU, Neaya A, West B. Pediatric short bowel syndrome redefining predictors of success. Ann Surg 2005;242(3):102-110. https://doi.org/10.1097/01. sla.0000179647.24046.03.
Khan FA, Squire RH, Litman HJ. Predictors of enteral autonomy in children with intestinal failure: A multicenter cohort study. J Pediatr 2015;167(1):29-34. https://doi.org/10.1016/j.jpeds.2015.03.040.
Struijs MC, Diamond IR, de Silva, Wales PW. Establishing norms for intestinal length in children. J Pediatr Surg 2009;44(5):933- 938. https://doi.org/10.1016/j. jpedsurg.2009.01.031.
Harper L, Michel J-L, de Napoli-Cocci, et al. One-step management of apple-peel atresia. Acta Chir Belg 2009;109(6):775-777. https://doi.org/10.1080/00015458.2 009.11680535.
Ogle SB, Nichol PF, Ostlie DJ. Chapter 30: Duodenal and intestinal atresia and stenosis. In: Holcomb GW III, Murphy JP, St Peter SD, eds. Holcomb and Ashcraft’s Pediatric Surgery. Amsterdam: Elsevier, 2020:489-506.
16.
18. 19.
25.
28.
31.
Stollman TH, de Blaauw I, Wijnen MH, et al. Decreased mortality but increased morbidity in neonates with jejunoileal atresia: A study of 114 cases over a 34-year period. J Pediatr Surg 2009;44(1):217-212. https://doi.org/10.1016/j. jpedsurg.2008.10.043.
Mangray H, Ghimenton F, Aldous C, et al. Jejuno-ileal atresia: Its characteristics and peculiarities concerning apple peel atresia, focused on its treatment and outcomes as experienced in one of the leading South African academic centres. Pediatr Surg Int 2019;36(2):201-207. https://doi. org/10.1007/s00383-019-04594-y.
Wales PW, de Silva N, Kim J, et al. Neonatal short bowel syndrome: Population- based estimates of incidence and mortality rates. J Pediatr Surg 2004;39(5):690- 695. https://doi.org/10.1016/j.jpedsurg.2004.01.036.
Javid PJ, Malone FR, Reyes J, et al. The experience of a regional pediatric intestinal failure program: Successful outcomes from intestinal rehabilitation. Am J Surg 2010;199(5):676-679. https://doi.org/10.1016/j.amjsurg.2010.01.013. Diamond IR, de Silva N, Pencharz PB, et al. Neonatal short bowel syndrome outcomes after the establishment of the first Canadian multidisciplinary intestinal rehabilitation program: Preliminary experience. J Pediatr Surg 2007;42(5):806-811. https://doi.org/10.1016/j.jpedsurg.2006.12.033.
Koehler AN, Yaworski JA, Gardner M, et al. Coordinated interdisciplinary management of pediatric intestinal failure: A 2-year review. J Pediatr Surg 2000;35(2):380-385. https://doi.org/10.1016/s0022-3468(00)90045-9. Banieghbal B, Schoeman L, Kalk F, et al. Surgical indications and strategies for necrotizing enterocolitis in low-income countries. World J Surg 2002;26(4):444- 447. https://doi.org/10.1007/s00268-001-0246-6.
Motsisi S, Ballot DE. A review of necrotising enterocolitis in very low birth weight babies in a tertiary hospital in Johannesburg. Afr J Paediatr Surg 2023;20(1):59-66. https://doi.org/10.4103/ajps.ajps_156_21.
Arnold M, Moore SW, Sidler D, Kirsten GF. Long-term outcome of surgically managed necrotizing enterocolitis in a developing country. Pediatr Surg Int 2010;26(4):355-360. https://doi.org/10.1007/s00383-010-2583-8.
Wales PW, de Silva N, Kim JH. Neonatal short bowel syndrome A cohort study. J Pediatr Surg 2005;40(5):755-762. https://doi.org/10.1016/j.jpedsurg.2005.01.037. Khalil BA, Ba’ath ME, Aziz A, et al. Intestinal rehabilitation and bowel reconstructive surgery: Improved outcomes in children with short bowel syndrome. J Pediatr Gastroenterol Nutr 2012;54(4):505-509. https://doi. org/10.1097/MPG.0b13e318230c27e.
Cowles R, Ventura K, Martinez M. Reversal of intestinal failure–associated liver disease in infants and children on parenteral nutrition: experience with 93 patients at a referral center for intestinal rehabilitation. J Pediatr Surg 2010;45(1):84-88. https://doi.org/10.1016/j.jpedsurg.2009.10.014.
Gupte GL, Beath SV, Protheroe S, et al. Improved outcome of referrals for intestinal transplantation in the UK. Arch Dis Child 2007;92(2):147-152. https://doi.org/10.1136/adc.2005.090068.
Marras-Salmio L, Mutanen A, Ylinen E, et al. Pediatric intestinal failure: The key outcomes for the first 100 patients treated in a national tertiary referral center during 1984 - 2017. J Parenteral Enteral Nutr 2018;42(8):1304-1313. https://doi.org/10.1002/jpen.1164.
Cruzeiro PCF, Camargos PAM, Miranda ME. Central venous catheter placement in children: A prospective study of complications in a Brazilian public hospital. Pediatr Surg Int 2006;22(6):536-540. https://doi.org/10.1007/ s00383-006-1671-2.
Johnson EM, Saltzman DA, Suh G. Complications and risks of central venous catheter placement in children. Surgery 1998;124(5):911-916.
Duesing LA, Fawley JA, Wagner AJ. Central venous access in the pediatric population with emphasis on complications and prevention strategies. Nutr Clin Pract 2016;31(4):490-501. https://doi.org/10.1177/0884533616640454. Milford K, von Delft D, Majola N, Cox S. Long-term vascular access in differently resourced settings: A review of indications, devices, techniques, and complications. Pediatric Surg Int 2020;36(5):551-562. https://doi.org/10.1007/ s00383-020-04640-0.