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Eur J Pediatr Surg 2021; 31(01): 008-013
DOI: 10.1055/s-0040-1721040
Original Article

European Pediatric Surgeon' Association Survey on the Management of Short-Bowel Syndrome

Anne Dariel
1   Department of Pediatric Surgery, Assistance Publique des Hôpitaux de Marseille, Hôpital Timone Enfants, Marseille, France
,
Alice Faure
1   Department of Pediatric Surgery, Assistance Publique des Hôpitaux de Marseille, Hôpital Timone Enfants, Marseille, France
,
Leopoldo Martinez
2   Department of Pediatric Surgery, Hospiltal Infantil La Paz, Madrid, Spain
,
Francesco Morini
3   Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy
,
Alessio Pini Prato 
4   Department of Pediatric Surgery, Alessandria Children Hospital, Alessandria, Italy
,
Florian Friedmacher
5   Department of Pediatric Surgery, University Hospital of Frankfurt, Frankfurt, Germany
,
Marie-Edith Coste
6   Department of Pediatrics, Intestinal Failure Program, Assistance Publique des Hôpitaux de Marseille, Marseille, France
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Abstract

Introduction The aim of this study was to assess the management of short-bowel syndrome (SBS) at the time of primary surgery, and the strategies used to facilitate enteral autonomy depending on the institutional expertise.

Materials and Methods An online questionnaire was sent in 2019 to members of The European Pediatric Surgeons' Association.

Results Among the 65 responding members (26 countries, 85% from university hospitals), 57% manage less than three new patients with SBS per year (group A), and 43% at least three patients (group B). The cut-off of three patients treated yearly used in our study was defined after statistical analysis of different cut-offs. A multidisciplinary intestinal rehabilitation program is significantly more frequent in group B than in group A (85 and 53%, respectively; p = 0.009). Considering the primary surgical management of multiple intestinal atresia and congenital ultra-short bowel with jejunal atresia, primary surgical strategies to optimize bowel length are more often used in group B than group A (p = 0.09 and p = 0.04, respectively). A minimum of one intestinal lengthening procedure every 2 to 3 years is significantly more frequent in group B than group A (95 and 45%, respectively; p = 0.0013). Among the strategies used to promote intestinal adaptation, group B (35%) uses significantly more often glucagon-like peptide 2 analogs than group A (10%) (p = 0.02).

Conclusion Based on our survey, a minimum number of SBS patients treated yearly is required to manage this challenging disease according to up-to-date medical and surgical strategies. However, whatever their level of expertise is in managing SBS, most of pediatric surgeons are involved in the primary surgery. Medical education programs about SBS should be more largely available to pediatric surgeons.

Keywords

short-bowel syndrome - surgery - multidisciplinary - intestinal adaptation - secondary serial transverse enteroplasty

Supplementary Material



Publication History

Received: 15 May 2020

Accepted: 09 October 2020

Article published online:
16 November 2020

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Mutanen A, Wales PW. Etiology and prognosis of pediatric short bowel syndrome. Semin Pediatr Surg 2018; 27 (04) 209-217
    CrossrefPubMedGoogle Scholar
  • 2 Goulet O, Abi Nader E, Pigneur B, Lambe C. Short bowel syndrome as the leading cause of intestinal failure in early life: some insights into the management. Pediatr Gastroenterol Hepatol Nutr 2019; 22 (04) 303-329
    CrossrefPubMedGoogle Scholar
  • 3 Oliveira SB, Cole CR. Insights into medical management of pediatric intestinal failure. Semin Pediatr Surg 2018; 27 (04) 256-260
    CrossrefPubMedGoogle Scholar
  • 4 Struijs MC, Diamond IR, de Silva N, Wales PW. Establishing norms for intestinal length in children. J Pediatr Surg 2009; 44 (05) 933-938
    CrossrefPubMedGoogle Scholar
  • 5 Wales PW, Jancelewicz T, Romao RL, Piper HG, de Silva NT, Avitzur Y. Delayed primary serial transverse enteroplasty as a novel management strategy for infants with congenital ultra-short bowel syndrome. J Pediatr Surg 2013; 48 (05) 993-999
    CrossrefPubMedGoogle Scholar
  • 6 Hatch Jr EI, Schaller Jr RT. Surgical management of multiple intestinal atresias. Am J Surg 1986; 151 (05) 550-552
    CrossrefPubMedGoogle Scholar
  • 7 Romão RLP, Ozgediz D, de Silva N, Chiu P, Langer J, Wales PW. Preserving bowel length with a transluminal stent in neonates with multiple intestinal anastomoses: a case series and review of the literature. J Pediatr Surg 2011; 46 (07) 1368-1372
    CrossrefPubMedGoogle Scholar
  • 8 Stanger JD, Oliveira C, Blackmore C, Avitzur Y, Wales PW. 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 (05) 983-992
    CrossrefPubMedGoogle Scholar
  • 9 Courtney CM, Onufer EJ, Seiler KM, Warner BW. An anatomic approach to understanding mechanisms of intestinal adaptation. Semin Pediatr Surg 2018; 27 (04) 229-236
    CrossrefPubMedGoogle Scholar
  • 10 Spencer AU, Neaga A, West B. et al. Pediatric short bowel syndrome: redefining predictors of success. Ann Surg 2005; 242 (03) 403-409
    CrossrefPubMedGoogle Scholar
  • 11 Merritt RJ, Cohran V, Raphael BP. et al. Nutrition Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Intestinal rehabilitation programs in the management of pediatric intestinal failure and short bowel syndrome. J Pediatr Gastroenterol Nutr 2017; 65 (05) 588-596
    CrossrefPubMedGoogle Scholar
  • 12 Javid PJ, Wendel D, Horslen SP. Organization and outcomes of multidisciplinary intestinal failure teams. Semin Pediatr Surg 2018; 27 (04) 218-222
    CrossrefPubMedGoogle Scholar
  • 13 Belza C, Wales PW. Multidisciplinary management in pediatric ultrashort bowel syndrome. J Multidiscip Healthc 2020; 13: 9-17
    CrossrefPubMedGoogle Scholar
  • 14 Tappenden KA. Pathophysiology of short bowel syndrome: considerations of resected and residual anatomy. JPEN J Parenter Enteral Nutr 2014; 38 (01) 14S-22S
    CrossrefPubMedGoogle Scholar
  • 15 Belza C, Fitzgerald K, de Silva N. et al. Predicting intestinal adaptation in pediatric intestinal failure: a retrospective cohort study. Ann Surg 2019; 269 (05) 988-993
    CrossrefPubMedGoogle Scholar
  • 16 Walker AA, Cole CR. Ultra-short bowel syndrome during infancy: improving outcomes and novel therapies. Curr Opin Pediatr 2019; 31 (02) 177-181
    CrossrefPubMedGoogle Scholar
  • 17 Norsa L, Lambe C, Abi Abboud S. et al. The colon as an energy salvage organ for children with short bowel syndrome. Am J Clin Nutr 2019; 109 (04) 1112-1118
    CrossrefPubMedGoogle Scholar
  • 18 Diamond IR, Wales PW. Advantages of the distal sigmoid colostomy in the management of infants with short bowel syndrome. J Pediatr Surg 2008; 43 (08) 1464-1467
    CrossrefPubMedGoogle Scholar
  • 19 Ramos-Gonzalez G, Kim HB. Autologous intestinal reconstruction surgery. Semin Pediatr Surg 2018; 27 (04) 261-266
    CrossrefPubMedGoogle Scholar
  • 20 Jones BA, Hull MA, Potanos KM. et al. International STEP Data Registry. Report of 111 consecutive patients enrolled in the International Serial Transverse Enteroplasty (STEP) Data Registry: a retrospective observational study. J Am Coll Surg 2013; 216 (03) 438-446
    CrossrefPubMedGoogle Scholar
  • 21 Fitzgerald K, Muto M, Belza C, De Silva N, Avitzur Y, Wales PW. The evolution of the serial transverse enteroplasty for pediatric short bowel syndrome at a single institution. J Pediatr Surg 2019; 54 (05) 993-998
    CrossrefPubMedGoogle Scholar
  • 22 Drucker DJ, Yusta B. Physiology and pharmacology of the enteroendocrine hormone glucagon-like peptide-2. Annu Rev Physiol 2014; 76: 561-583
    CrossrefPubMedGoogle Scholar
  • 23 Sigalet DL. Advances in glucagon like peptide-2 therapy. physiology, current indications and future directions. Semin Pediatr Surg 2018; 27 (04) 237-241
    CrossrefPubMedGoogle Scholar
  • 24 Carter BA, Cohran VC, Cole CR. et al. Outcomes from a 12-week, open-label, multicenter clinical trial of teduglutide in pediatric short bowel syndrome. J Pediatr 2017; 181: 102-111.e5
    CrossrefPubMedGoogle Scholar
  • 25 Kocoshis SA, Merritt RJ, Hill S. et al. Safety and efficacy of teduglutide in pediatric patients with intestinal failure due to short bowel syndrome: a 24-week, phase III study. JPEN J Parenter Enteral Nutr 2020; 44 (04) 621-631
    CrossrefPubMedGoogle Scholar