Intensive Care for Short Bowel Syndrome in Children: a Review

Cover Page

Cite item

Full Text

Abstract

Short bowel syndrome is one of the most challenging conditions in modern pediatric surgery and intensive care, leading to a significant decrease in quality of life, disability, and mortality. This review presents current insights into the pathogenesis, clinical manifestations, diagnosis, and treatment of the condition. Publications focused on the principles of intensive care for pediatric short bowel syndrome were analyzed. Scientific data searches were conducted in the PubMed, Cochrane Central Register of Controlled Trials, eLIBRARY.RU, and CyberLeninka databases for the period from 2001 to December 2024. The search was conducted using the following keywords: синдром короткой кишки / short bowel syndrome, дети/pediatric, интенсивная терапия / intensive care, парентеральное питание / parenteral nutrition, энтеральное питание / enteral nutrition, and осложнения/complication. Inclusion criteria were studies describing the definition, clinical course, and intensive care principles of short bowel syndrome in children. Publications focused solely on adult patients were excluded, except for guidelines illustrating general intensive care principles applicable regardless of age. A total of 62 articles were included. The review discusses the definition, epidemiology, etiology, key risk factors, and pathogenesis of short bowel syndrome, as well as its classification and clinical presentation depending on the extent of intestinal resection. Particular attention is given to intensive care measures and nutritional support, the use of antibiotics and medications for restoring gut microbiota, glucagon-like peptide-2 analog therapy, and chyme reinfusion techniques. The core principles of nutritional support, indications and contraindications for its administration, and complications of long-term parenteral nutrition are examined. Strong evidence is presented supporting early initiation of enteral feeding. Functional gastrointestinal assessment tools and food tolerance diagnostics are described, along with suggested algorithms for initiating enteral support and managing short bowel syndrome–associated diarrhea. The necessity of a multidisciplinary approach for the early rehabilitation of children with short bowel syndrome is emphasized, aimed at restoring physiological function and development.

About the authors

Ekaterina A. Plevako

St. Mary Magdalene Children’s Hospital No. 2

Email: katerondo@mail.ru
ORCID iD: 0009-0001-5804-5020
SPIN-code: 9744-9205
Russian Federation, Saint Petersburg

Konstantin V. Pshenisnov

Saint Petersburg State Pediatric Medical University

Author for correspondence.
Email: Psh_K@mail.ru
ORCID iD: 0000-0003-1113-5296
SPIN-code: 8423-4294

MD, Dr. Sci. (Medicine), Associate Professor

Russian Federation, Saint Petersburg

Yuri S. Alexandrovich

Saint Petersburg State Pediatric Medical University

Email: jalex1963@mail.ru
ORCID iD: 0000-0002-2131-4813
SPIN-code: 2225-1630

MD, Dr. Sci. (Medicine), Professor

Russian Federation, Saint Petersburg

Igor A. Komissarov

Saint Petersburg State Pediatric Medical University

Email: komissarov_i_a@mail.ru
ORCID iD: 0009-0000-5464-4627
SPIN-code: 3356-9330

MD, Dr. Sci. (Medicine), Professor

Russian Federation, Saint Petersburg

References

  1. Aver’ianova YuV, Razumovsky AYu, Makarov SP, et al. Current strategy for management of short bowel syndrome: a twelve-year single-center experience. Russian Journal of Anaesthesiology and Reanimatology. 2018;63(6):67–74. doi: 10.17116/anaesthesiology201806167 EDN: YURYZF.
  2. Kamalova AA, Podshivalin AA, Sageeva GI, Druzhkova EE. Regional experience in the treatment of children with short bowel syndrome. Russian Bulletin of Perinatology and Pediatrics. 2022;67(5):231–236. doi: 10.21508/1027-4065-2022-67-5-231-236 EDN: WOSSYY
  3. Luft VM, Demko AE, Leiderman IN, et al. Short bowel syndrome in adults: diagnosis and treatment. Saint Petersburg; 2023. 78 p. (In Russ.)
  4. Weih S, Kessler M, Fonouni H, et al. Current practice and future perspectives in the treatment of short bowel syndrome in children—a systematic review. Langenbecks Arch Surg. 2012;397(7):1043–1051. doi: 10.1007/s00423-011-0874-8
  5. Guillen B, Atherton NS. Short bowel syndrome. In: StatPearls. Treasure Island: StatPearls Publishing; 2023.
  6. Averyanova YuV, Vessel L, Erpulyova YuV, et al. Federal clinical recommendations "Treatment of children with the short bowel syndrome". Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care. 2014;4(4):92–108. doi: 10.17816/psaic88 EDN: RKUCKQ
  7. Caporilli C, Giannì G, Grassi F, Esposito S. An overview of short-bowel syndrome in pediatric patients: focus on clinical management and prevention of complications. Nutrients. 2023;15(10):2341. doi: 10.3390/nu15102341
  8. Pironi L. Definitions of intestinal failure and the short bowel syndrome. Best Pract Res Clin Gastroenterol. 2016;30(2):173–185. doi: 10.1016/j.bpg.2016.02.011
  9. Muff JL, Sokolovski F, Walsh-Korb Z, et al. Surgical treatment of short bowel syndrome-the past, the present and the future, a descriptive review of the literature. Children. 2022;9(7):1024. doi: 10.3390/children9071024
  10. Goulet O, Sauvat F. Short bowel syndrome and intestinal transplantation in children. Curr Opin Clin Nutr Metab Care. 2006;9(3):304–313. doi: 10.1097/01.mco.0000222116.68912.fc
  11. Koffeman GI, van Gemert WG, George EK, Veendaal RA. Classification, epidemiology and aetiology. Best Pract Res Clin Gastroenterol. 2003;17(6):879–893. doi: 10.1016/s1521-6918(03)00099-4
  12. Nikitin AM, Shmatova AA. Features of intensive care for children with short bowel syndrome. Young scientist. 2018;13(199):81–83. EDN: WBBFEL
  13. Wales PW, Christison-Lagay ER. Short bowel syndrome: epidemiology and etiology. Semin Pediatr Surg. 2010;19(1):3–9. doi: 10.1053/j.sempedsurg.2009.11.001
  14. 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. doi: 10.1016/j.jpedsurg.2004.01.036
  15. Burrin DG, Ng K, Stoll B, et al. Impact of new-generation lipid emulsions on cellular mechanisms of parenteral nutrition-associated liver disease. Adv Nutr. 20141;5(1):82–91. doi: 10.3945/an.113.004796
  16. Tazuke Y, Udagawa E, Mizushima T, et al. Real-world etiologies and treatments of pediatric short bowel syndrome in Japan. Pediatr Int. 2022;64(1):e15258. doi: 10.1111/ped.15258
  17. Puoti MG, Köglmeier J. Nutritional management of intestinal failure due to short bowel syndrome in children. Nutrients. 2022;15(1):62. doi: 10.3390/nu15010062
  18. Rozinov VM, Ampar FB, Samorokovskaya MV, Isakov MA. Organizational and clinical potential of the Federal Register of children with short bowel syndrome. Russian Journal of Pediatric Surgery. 2022;12(1):73–84. doi: 10.17816/psaic1229 EDN: CZYVFK
  19. Tappenden KA. Pathophysiology of short bowel syndrome: considerations of resected and residual anatomy. J Parenter Enteral Nutr. 2014;38(1):14S–22S. doi: 10.1177/0148607113520005
  20. Rossi L, Kadamba P, Hugosson C, et al. Pediatric short bowel syndrome: adaptation after massive small bowel resection. J Pediatr Gastroenterol Nutr. 2007;45(2):213–221. doi: 10.1097/MPG.0b013e31803c75e8
  21. Neelis E, de Koning B, Rings E, et al. The gut microbiome in patients with intestinal failure: current evidence and implications for clinical practice. J Parenter Enteral Nutr. 2019;43(2):194–205. doi: 10.1002/jpen.1423
  22. Averyanova YuV, Stepanov AE, Razumovsky AY. Effective diverse treatment of recurrent D-lactate acidosis in short bowel syndrome: a clinical case. Pediatric surgery. 2015;19(4):42–46. EDN: UFGSJB
  23. Goodman BE. Insights into digestion and absorption of major nutrients in humans. Adv Physiol Educ. 2010;34(2):44–53. doi: 10.1152/advan.00094.2009
  24. Jahan-Mihan A, Luhovyy BL, El Khoury D, Anderson GH. Dietary proteins as determinants of metabolic and physiologic functions of the gastrointestinal tract. Nutrients. 2011;3(5):574–603. doi: 10.3390/nu3050574
  25. Phan CT, Tso P. Intestinal lipid absorption and transport. Front Biosci. 2001;6(3):D299–D319. doi: 10.2741/phan
  26. Menge H, Gräfe M, Lorenz-Meyer H, Riecken EO. The influence of food intake on the development of structural and functional adaptation following ileal resection in the rat. Gut. 1975;16(6):468–472. doi: 10.1136/gut.16.6.468
  27. Eastwood GL. Small bowel morphology and epithelial proliferation in intravenously alimented rabbits. Surgery. 1977;82(5):613–620.
  28. Feldman EJ, Dowling RH, McNaughton J, Peters TJ. Effects of oral versus intravenous nutrition on intestinal adaptation after small bowel resection in the dog. Gastroenterology. 1976;70(5-1):712–719. doi: 10.1016/S0016-5085(76)80261-2
  29. Bering J, DiBaise JK. Short bowel syndrome: Complications and management. Nutr Clin Pract. 2023;38(S1):S46–S58. doi: 10.1002/ncp.10978
  30. Norsa L, Goulet O, Alberti D, et al. Nutrition and intestinal rehabilitation of children with short bowel syndrome: A position paper of the ESPGHAN committee on nutrition. Part 1: From intestinal resection to home discharge. J Pediatr Gastroenterol Nutr. 2023;77(2):281–297. doi: 10.1097/MPG.0000000000003849
  31. Modi BP, Galloway DP, Gura K, et al. ASPEN definitions in pediatric intestinal failure. J Parenter Enteral Nutr. 2022;46(1):42–59. doi: 10.1002/jpen.2232
  32. Moltu SJ, Bronsky J, Embleton N, et al. Nutritional management of the critically Ill neonate: A position paper of the ESPGHAN committee on nutrition. J Pediatr Gastroenterol Nutr. 2021;73(2):274–289. doi: 10.1097/MPG.0000000000003076
  33. Stultz JS, Fly JH, Bagga B, et al. How broad should gram-negative coverage be for febrile parenteral nutrition dependent short bowel syndrome patients? J Pediatr Gastroenterol Nutr. 2022;74(6):845–849. doi: 10.1097/MPG.0000000000003382
  34. Gelfand BR, Kiriyenko AI, Dibirov MD, Khachatryan NN. Abdominal infection and sepsis. Infections in surgery. 2017;15(3-4):1–27. (In Russ.) EDN: YPHXIL
  35. Pimentel M, Saad RJ, Long MD, et al. ACG clinical guideline: Small intestinal bacterial overgrowth. Am J Gastroenterol. 2020;115(2):165–178. doi: 10.14309/ajg.0000000000000501
  36. Ramos JT, Romero CA, Belda S, et al. Clinical practice update of antifungal prophylaxis in immunocompromised children. Rev Esp Quimioter. 2019;32(5):410–425.
  37. Pappas PG, Kauffman CA, Andes DR, et al. Clinical practice guideline for the management of candidiasis: 2016 update by the Infectious Diseases Society of America. Clin Infect Dis. 2016;62(4):e1–e50. doi: 10.1093/cid/civ933
  38. Gatta L, Scarpignato C. Systematic review with meta-analysis: rifaximin is effective and safe for the treatment of small intestine bacterial overgrowth. Aliment Pharmacol Ther. 2017;45(5):604–616. doi: 10.1111/apt.13928
  39. Rykov MYu, Petrash AA, Men TH. Venous access systems as medical care quality indicators: Comparative analysis of peripherally inserted central catheters and implantable venous port systems. Onkopediatria. 2017;4(2):123–130. doi: 10.15690/onco.v4i2.1706 EDN: YTDRSX
  40. Mihatsch WA, Braegger C, Bronsky J, et al. ESPGHAN/ESPEN/ESPR/CSPEN guidelines on pediatric parenteral nutrition. Clin Nutr. 2018;37(6B):2303–2305. doi: 10.1016/j.clnu.2018.05.029
  41. Secor JD, Yu L, Tsikis S, et al. Current strategies for managing intestinal failure-associated liver disease. Expert Opin Drug Saf. 2021;20(3):307–320. doi: 10.1080/14740338.2021.1867099
  42. Shaw D, Gohil K, Basson MD. Intestinal mucosal atrophy and adaptation. World J Gastroenterol. 2012;18(44):6357–6375. doi: 10.3748/wjg.v18.i44.6357
  43. Sukhotnik I, Levi R, Moran-Lev H. Impact of dietary protein on the management of pediatric short bowel syndrome. Nutrients. 2023;15(13):2826. doi: 10.3390/nu15132826
  44. Merras-Salmio L, Pakarinen MP. Infection prevention and management in pediatric short bowel syndrome. Front Pediatr. 2022;10:864397. doi: 10.3389/fped.2022.864397
  45. Dobson R, McGuckin C, Walker G, et al. Cycled enteral antibiotics reduce sepsis rates in paediatric patients on long-term parenteral nutrition for intestinal failure. Aliment Pharmacol Ther. 2011;34(8):1005–1011. doi: 10.1111/j.1365-2036.2011.04826.x
  46. Dieterich W, Schink M, Zopf Y. Microbiota in the gastrointestinal tract. Med Sci. 2018;6(4):116. doi: 10.3390/medsci6040116
  47. Lkhagva E, Chung H-J, Hong J, et al. The regional diversity of gut microbiome along the GI tract of male C57BL/6 mice. BMC Microbiol. 2021;21(1):44. doi: 10.1186/s12866-021-02099-0
  48. Lapthorne S, Pereira-Fantini PM, Fouhy F, et al. Gut microbial diversity is reduced and is associated with colonic inflammation in a piglet model of short bowel syndrome. Gut Microbes. 2013;4(3):212–221. doi: 10.4161/gmic.24372
  49. Devine AA, Gonzalez A, Speck KE, et al. Impact of ileocecal resection and concomitant antibiotics on the microbiome of the murine jejunum and colon. PLoS One. 2013;8(8):e73140. doi: 10.1371/journal.pone.0073140
  50. Nagpal R, Yadav H. Bacterial translocation from the gut to the distant organs: An overview. Ann Nutr Metab. 2017;71(S1):11–16. doi: 10.1159/000479918
  51. Cerdó T, García-Santos JA, Rodríguez-Pöhnlein A, et al. Impact of total parenteral nutrition on gut microbiota in pediatric population suffering intestinal disorders. Nutrients. 2022;14(21):4691. doi: 10.3390/nu14214691
  52. Piper HG, Coughlin LA, Hussain S, et al. The impact of lactobacillus probiotics on the gut microbiota in children with short bowel syndrome. J Surg Res. 2020;251:112–118. doi: 10.1016/j.jss.2020.01.024
  53. Tropina EP, Zmanovskaya VA. Successful experience in the use of teduglutide in children with short bowel syndrome. Russian Pediatric Journal. 2022;25(5):344–349. doi: 10.46563/1560-9561-2022-25-5-344-349 EDN: UINRLI
  54. Brubaker PL. Glucagon-like peptide-2 and the regulation of intestinal growth and function. Compr Physiol. 2018;8(3):1185–1210. doi: 10.1002/cphy.c170055
  55. Ampar FB, Rozinov VM, Chernobabova MM. The assessment of the effectiveness and safety of teduglutide in the treatment of children with chronic intestinal insufficiency. A systematic review. Russian Journal of Pediatric Surgery Anesthesia and Intensive Care. 2023;13(2):175–188. doi: 10.17816/psaic1513 EDN: UKKVEZ
  56. 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. J Parenter Enteral Nutr. 2020;44(4):621–631. doi: 10.1002/jpen.1690
  57. Chubarova AI, Kostomarova EA, Tumanova EL, et al. Clinical and morphological assessment of the effectiveness of teduglutide in children with short bowel syndrome: a pilot study in Russia. Issues of pediatric dietetics. 2024;22(1):29-39. doi: 10.20953/1727-5784-2024-1-29-39 EDN: IZUMTB
  58. Lam JR, Schneider JL, Zhao W, et al. Proton pump inhibitor and histamine 2 receptor antagonist use and vitamin B12 deficiency. JAMA. 2013;310(22):2435–2442. doi: 10.1001/jama.2013.280490
  59. Chandra R, Kesavan A. Current treatment paradigms in pediatric short bowel syndrome. Clin J Gastroenterol. 2018;11(2):103–112. doi: 10.1007/s12328-017-0811-7
  60. Kumpf VJ. Pharmacologic management of diarrhea in patients with short bowel syndrome. J Parenter Enteral Nutr. 2014;38(15):38S–44S. doi: 10.1177/0148607113520618
  61. Chan L-N. Opioid analgesics and the gastrointestinal tract. PractGastroenterol. 2008;32:37–50.
  62. Gause CD, Hayashi M, Haney C, et al. Mucous fistula refeeding decreases parenteral nutrition exposure in postsurgical premature neonates. J Pediatr Surg. 2016;51(11):1759–1765. doi: 10.1016/j.jpedsurg.2016.06.018

Supplementary files

Supplementary Files
Action
1. JATS XML
2. Fig. 1. Methodology of publication search and analysis.

Download (163KB)

Согласие на обработку персональных данных с помощью сервиса «Яндекс.Метрика»

1. Я (далее – «Пользователь» или «Субъект персональных данных»), осуществляя использование сайта https://journals.rcsi.science/ (далее – «Сайт»), подтверждая свою полную дееспособность даю согласие на обработку персональных данных с использованием средств автоматизации Оператору - федеральному государственному бюджетному учреждению «Российский центр научной информации» (РЦНИ), далее – «Оператор», расположенному по адресу: 119991, г. Москва, Ленинский просп., д.32А, со следующими условиями.

2. Категории обрабатываемых данных: файлы «cookies» (куки-файлы). Файлы «cookie» – это небольшой текстовый файл, который веб-сервер может хранить в браузере Пользователя. Данные файлы веб-сервер загружает на устройство Пользователя при посещении им Сайта. При каждом следующем посещении Пользователем Сайта «cookie» файлы отправляются на Сайт Оператора. Данные файлы позволяют Сайту распознавать устройство Пользователя. Содержимое такого файла может как относиться, так и не относиться к персональным данным, в зависимости от того, содержит ли такой файл персональные данные или содержит обезличенные технические данные.

3. Цель обработки персональных данных: анализ пользовательской активности с помощью сервиса «Яндекс.Метрика».

4. Категории субъектов персональных данных: все Пользователи Сайта, которые дали согласие на обработку файлов «cookie».

5. Способы обработки: сбор, запись, систематизация, накопление, хранение, уточнение (обновление, изменение), извлечение, использование, передача (доступ, предоставление), блокирование, удаление, уничтожение персональных данных.

6. Срок обработки и хранения: до получения от Субъекта персональных данных требования о прекращении обработки/отзыва согласия.

7. Способ отзыва: заявление об отзыве в письменном виде путём его направления на адрес электронной почты Оператора: info@rcsi.science или путем письменного обращения по юридическому адресу: 119991, г. Москва, Ленинский просп., д.32А

8. Субъект персональных данных вправе запретить своему оборудованию прием этих данных или ограничить прием этих данных. При отказе от получения таких данных или при ограничении приема данных некоторые функции Сайта могут работать некорректно. Субъект персональных данных обязуется сам настроить свое оборудование таким способом, чтобы оно обеспечивало адекватный его желаниям режим работы и уровень защиты данных файлов «cookie», Оператор не предоставляет технологических и правовых консультаций на темы подобного характера.

9. Порядок уничтожения персональных данных при достижении цели их обработки или при наступлении иных законных оснований определяется Оператором в соответствии с законодательством Российской Федерации.

10. Я согласен/согласна квалифицировать в качестве своей простой электронной подписи под настоящим Согласием и под Политикой обработки персональных данных выполнение мною следующего действия на сайте: https://journals.rcsi.science/ нажатие мною на интерфейсе с текстом: «Сайт использует сервис «Яндекс.Метрика» (который использует файлы «cookie») на элемент с текстом «Принять и продолжить».