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Short bowel syndrome — Clinical Guidelines

Overview

Short bowel syndrome (SBS) is a condition characterized by significant loss of intestinal length leading to malabsorption and often necessitating parenteral nutrition (PN) support 1 2 3.

Diagnosis

Micronutrient deficiencies (vitamins A, D, E, B12, K, trace elements) 1 14 15 20

Growth deficits in children 1

Bowel obstruction 4

Elevated serum methylmalonic acid levels indicative of vitamin B12 deficiency 10

Intestinal failure (IF) criteria 2 3

Management

First-line treatments:

- Parenteral nutrition (PN) to meet nutritional needs 2 3 9 - Intestinal rehabilitation aiming for enteral autonomy 2 3 9

Adjunctive treatments:

- GLP-2 analogues (e.g., teduglutide) to enhance intestinal absorption and reduce PN requirements 5 - Nutritional supplementation for specific deficiencies (e.g., vitamin D, vitamin K) 13 14 20 - Intestinal lengthening and tailoring (LILT) surgical procedures for selected patients 22

Special Populations

Pediatrics:

- Multidisciplinary intestinal rehabilitation programs improve outcomes 17 - Early weaning off PN with careful monitoring of growth and micronutrient status 1 9

Pregnancy:

- Close monitoring for profound vitamin deficiencies and bleeding diathesis due to malabsorption 12

Key Recommendations

Implement multidisciplinary intestinal rehabilitation programs to optimize weaning from parenteral nutrition in pediatric SBS patients (Evidence: Strong 17)

Regularly monitor vitamin and trace element status, especially B12, D, K, and A, in SBS patients receiving PN to prevent deficiencies (Evidence: Moderate 1 14 20)

Consider GLP-2 analogues like teduglutide for patients with SBS to reduce PN dependency and improve intestinal absorption (Evidence: Moderate 5)

References

1 Tuokkola J, Olkkonen E, Gunnar R, Pakarinen M, Merras-Salmio L. Vitamin and trace element status and growth in children with short bowel syndrome being weaned off parenteral nutrition. Journal of pediatric gastroenterology and nutrition 2025. link 2 Norsa L, Goulet O, Alberti D, DeKooning B, Domellöf M, Haiden N et al.. Nutrition and Intestinal Rehabilitation of Children With Short Bowel Syndrome: A Position Paper of the ESPGHAN Committee on Nutrition. Part 2: Long-Term Follow-Up on Home Parenteral Nutrition. Journal of pediatric gastroenterology and nutrition 2023. link 3 Norsa L, Goulet O, Alberti D, DeKooning B, Domellöf M, Haiden N 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. Journal of pediatric gastroenterology and nutrition 2023. link 4 Morotti F, Terruzzi J, Cavalleri L, Betalli P, D'Antiga L, Norsa L. Vitamin B. Nutrition (Burbank, Los Angeles County, Calif.) 2023. link 5 Diamanti A, Lezo A, D'Antiga L, Gandullia P, Spagnuolo MI, Roggero P et al.. Teduglutide in pediatric intestinal failure: A position statement of the Italian society of pediatric gastroenterology, hepatology and nutrition (SIGENP). Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver 2022. link 6 Tsikis ST, Fligor SC, Secor JD, Yu LJ, Pan A, Mitchell PD et al.. An in-line digestive cartridge increases enteral fat and vitamin absorption in a porcine model of short bowel syndrome. Clinical nutrition (Edinburgh, Scotland) 2022. link 7 Tsikis ST, Fligor SC, Secor JD, Yu LJ, Pan A, Loring G et al.. Outcomes and Perioperative Nutritional Management in a Porcine Model of Short Bowel Syndrome. The Journal of surgical research 2022. link 8 Capriati T, Mosca A, Alterio T, Spagnuolo MI, Gandullia P, Lezo A et al.. To Wean or Not to Wean: The Role of Autologous Reconstructive Surgery in the Natural History of Pediatric Short Bowel Syndrome on Behalf of Italian Society for Gastroenterology, Hepatology and Nutrition (SIGENP). Nutrients 2020. link 9 Ukleja A. Weaning from Parenteral Nutrition. Gastroenterology clinics of North America 2019. link 10 Jimenez L, Stamm DA, Depaula B, Duggan CP. Is Serum Methylmalonic Acid a Reliable Biomarker of Vitamin B12 Status in Children with Short Bowel Syndrome: A Case Series. The Journal of pediatrics 2018. link 11 Brat K, Merta Z, Čundrle I. Is it intestinal tuberculosis again? Case report. Vnitrni lekarstvi 2017. link 12 Grabosch S, Pennycook J, Pakravan A, Mostello D. Short bowel syndrome causing bleeding diathesis and profound vitamin deficiency in pregnancy. Obstetrics and gynecology 2013. link 13 Kumar PR, Fenton TR, Shaheen AA, Raman M. Prevalence of vitamin D deficiency and response to oral vitamin D supplementation in patients receiving home parenteral nutrition. JPEN. Journal of parenteral and enteral nutrition 2012. link 14 Krzyżanowska P, Książyk J, Kocielińska-Kłos M, Banaś E, Kaleta M, Popińska K et al.. Vitamin K status in patients with short bowel syndrome. Clinical nutrition (Edinburgh, Scotland) 2012. link 15 Braga CB, Vannucchi H, Freire CM, Marchini JS, Jordão AA, da Cunha SF. Serum vitamins in adult patients with short bowel syndrome receiving intermittent parenteral nutrition. JPEN. Journal of parenteral and enteral nutrition 2011. link 16 Yandza T, Schneider SM, Nishida S, Goubaux B, Badan L, Vanbiervliet G et al.. Outcome of exfoliative rejection after isolated intestinal transplantation in an adult: case report. Transplantation proceedings 2010. link 17 Modi BP, Langer M, Ching YA, Valim C, Waterford SD, Iglesias J et al.. Improved survival in a multidisciplinary short bowel syndrome program. Journal of pediatric surgery 2008. link 18 Reimund JM, Rahmi G, Escalin G, Pinna G, Finck G, Muller CD et al.. Efficacy and safety of an olive oil-based intravenous fat emulsion in adult patients on home parenteral nutrition. Alimentary pharmacology & therapeutics 2005. link 19 Federici S, Domenichelli V, Antonellini C, Dòmini R. Multiple intestinal atresia with apple peel syndrome: successful treatment by five end-to-end anastomoses, jejunostomy, and transanastomotic silicone stent. Journal of pediatric surgery 2003. link00281-1) 20 Reimund JM, Arondel Y, Duclos B, Baumann R. Vitamins and trace elements in home parenteral nutrition patients. The journal of nutrition, health & aging 2000. link 21 Cooper TR, Garcia-Prats JA, Brody BA. Managing disagreements in the management of short bowel and hypoplastic left heart syndrome. Pediatrics 1999. link 22 Bianchi A. Longitudinal intestinal lengthening and tailoring: results in 20 children. Journal of the Royal Society of Medicine 1997. link 23 Majewski E, Oztürk B, Gillessen-Kaesbach G. Jeune syndrome with tongue lobulation and preaxial polydactyly, and Jeune syndrome with situs inversus and asplenia: compound heterozygosity Jeune-Mohr and Jeune-Ivemark?. American journal of medical genetics 1996. link1096-8628(19960503)63:1<74::AID-AJMG15>3.0.CO;2-R) 24 Wakhlu AK, Pandey A, Wakhlu A, Tandon RK, Kureel SN. Coloplasty for congenital short colon. Journal of pediatric surgery 1996. link90736-8) 25 Franceschini P, Guala A, Vardeu MP, Signorile F, Franceschini D, Bolgiani MP. Short rib-dysplasia group (with/without polydactyly): report of a patient suggesting the existence of a continuous spectrum. American journal of medical genetics 1995. link 26 Tsai FJ, Tsai CH, Wang TR. Beemer-Langer type short rib-polydactyly syndrome: report of two cases. Zhonghua Minguo xiao er ke yi xue hui za zhi [Journal]. Zhonghua Minguo xiao er ke yi xue hui 1994. link 27 Lennard-Jones JE. Review article: practical management of the short bowel. Alimentary pharmacology & therapeutics 1994. link 28 Martínez-Frías ML, Bermejo E, Urioste M, Huertas H, Arroyo I. Lethal short rib-polydactyly syndromes: further evidence for their overlapping in a continuous spectrum. Journal of medical genetics 1993. link 29 Turnock RR, Brereton RJ, Spitz L, Kiely EM. Primary anastomosis in apple-peel bowel syndrome. Journal of pediatric surgery 1991. link90018-o) 30 Schraut WH, Lee KK, Sitrin M. Recipient growth and nutritional status following transplantation of segmental small-bowel allografts. The Journal of surgical research 1987. link90039-4) 31 Davis AT, Franz FP, Courtnay DA, Ullrey DE, Scholten DJ, Dean RE. Plasma vitamin and mineral status in home parenteral nutrition patients. JPEN. Journal of parenteral and enteral nutrition 1987. link 32 Buchan AM, Seal AM, Debas HT. Increased antral G-cell number and gastrin content in dogs after massive small bowel resection. Digestive diseases and sciences 1985. link 33 Dénes J, Ziszi K, Bognár M, Schläffer E, Tóth J. Congenital short colon associated with imperforate anus (Zachary-Morgan syndrome). Acta paediatrica Hungarica 1984. link 34 Sansaricq C, Chen WJ, Manka M, Davis D, Snyderman S. Familial congenital short small bowel with associated defects. A long-term survival. Clinical pediatrics 1984. link 35 Walley VM, Coates CF, Gilbert JJ, Valentine GH, Davies EM. Brief clinical report: short rib-polydactyly syndrome, Majewski type. American journal of medical genetics 1983. link 36 Black IL, Fitzsimmons J, Fitzsimmons E, Thomas AJ. Parental consanguinity and the Majewski syndrome. Journal of medical genetics 1982. link 37 Cooper CP, Hall CM. Lethal short-rib polydactyly syndrome of the Majewski type: a report of three cases. Radiology 1982. link 38 Chen H, Yang SS, Gonzalez E, Fowler M, Al Saadi A. Short rib-polydactyly syndrome, Majewski type. American journal of medical genetics 1980. link 39 Yang SS, Lin CS, Al Saadi A, Nangia BS, Bernstein J. Short rib-polydactyly syndrome, type 3 with chondrocytic inclusions: report of a case and review of the literature. American journal of medical genetics 1980. link 40 Motegi T, Kusunoki M, Nishi T, Hamada T, Sato N, Imamura T et al.. Short rib-polydactyly syndrome, Majewski type, in two male siblings. Human genetics 1979. link 41 Bidot-López P, Ablow RC, Ogden JA, Mahoney MJ. A case of short rib polydactyly. Pediatrics 1978. link 42 Tanner MS, Smith B, Lloyd JK. Functional intestinal obstruction due to deficiency of argyrophil neurones in the myenteric plexus. Familial syndrome presenting with short small bowel, malrotation, and pyloric hypertrophy. Archives of disease in childhood 1976. link

Short bowel syndrome