Short Bowel Syndrome
- 5 Downloads
Purpose of review
Short gut syndrome is life-altering and life-threatening disease resulting most often from massive small bowel resection. Recent advances in understanding of the perturbed physiology in these patients have translated into improved care and outcomes. This paper seeks to review the advances of care in SBS patients.
Anatomic considerations still predominate the early care of SBS patients, including aggressive preservation of bowel and documentation of remnant bowel length and quality. Intestinal adaptation is the process by which remnant bowel changes to fit the physiologic needs of the patient. Grossly, the bowel dilates and elongates to increase intestinal weight and protein content. Architectural changes are noted, such as villus lengthening and deepening of crypts. In addition, gene expression changes occur that function to maximize nutrient uptake and fluid preservation. Management is aimed at understanding these physiologic changes and augmenting them whenever possible in an effort to gain enteral autonomy. Complication mitigation is key, including avoidance of catheter complications, bloodstream infections, cholestasis, and nutrient deficiencies.
Multidisciplinary teams working together towards intestinal rehabilitation have shown improved outcomes. Today’s practitioner needs a current understanding of the ever-evolving care of these patients in order to promote enteral autonomy, recognize complications, and counsel patients and families appropriately.
KeywordsShort bowel syndrome Intestinal failure Intestinal adaptation Parenteral nutrition IFALD
Compliance with Ethical Standards
Conflict of Interest
Dr. Brad W. Warner reports grants from the National Institutes of Health and from serves on the Data, Safety, and Monitoring Board of Shire Pharmaceuticals Inc., during the conduct of the study.
Baddr A. Shakhsheer declares that there is no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
References and Recommended Reading
Papers of particular interest, published recently, have been highlighted as: • Of importance
- 9.• Seiler KM, Waye SE, Kong W, Kamimoto K, Bajinting A, Goo WH, et al. Single-cell analysis reveals regional reprogramming during adaptation to massive small bowel resection in mice. Cell Mol Gastroenterol Hepatol. 2019;10;8(3):407–426. This study used single-cell RNA sequencing after massive small bowel resection in mice to evaluate changes in cell identity resulting intestinal adaptation. Enterocytes assumed more proximal (i.e., jejunal) identities and upregulated lipid metabolism and oxidative stress gene expression.PubMedPubMedCentralCrossRefGoogle Scholar
- 20.• Warner BW. The pathogenesis of resection-associated intestinal adaptation. Cell Mol Gastroenterol Hepatol. 2016;2(4):429–38. This study provides a comprehensive review of what is currently known of the molecular pathways responsible for intestinal adaptation.PubMedPubMedCentralCrossRefGoogle Scholar
- 35.• Neelis E, de Koning B, Rings E, Wijnen R, Nichols B, Hulst J, et al. The gut microbiome in patients with intestinal failure: current evidence and implications for clinical practice. JPEN J Parenter enteral Nutr. 2019;43(2):194. This study provides a current review of the studies available on the microbiome of short gut patients and summarizes nicely how it contributes to the pathogenesis of their septic episodes.PubMedCrossRefGoogle Scholar
- 39.Cole CR, Frem JC, Schmotzer B, Gewirtz AT, Meddings JB, Gold BD, et al. The rate of bloodstream infection is high in infants with short bowel syndrome: relationship with small bowel bacterial overgrowth, enteral feeding, and inflammatory and immune responses. J Pediatr. 2010;156(6):941–947.e1.PubMedPubMedCentralCrossRefGoogle Scholar
- 42.• Merritt RJ, Cohran V, Raphael BP, Sentongo T, Volpert D, Warner BW, et al. Intestinal rehabilitation programs in the management of pediatric intestinal failure and short bowel syndrome. J Pediatr Gastroenterol Nutr. 2017;65(5):588. This study provides an outline of the current participants in a successful intestinal failure program that leads to improved patient outcomes.PubMedCrossRefGoogle Scholar
- 46.• Eisenberg M, Monuteaux MC, Fell G, Goldberg V, Puder M, Hudgins J. Central line-associated bloodstream infection among children with intestinal failure presenting to the emergency department with fever. J Pediatr. 2018;196:237. This study reviews infectious complications of intestinal failure patients and concludes that fevers in patients with CVCs have a high likelihood of representing a central line infection.PubMedCrossRefGoogle Scholar
- 48.Wong T, Clifford V, McCallum Z, Shalley H, Peterkin M, Paxton G, et al. Central venous catheter thrombosis associated with 70% ethanol locks in pediatric intestinal failure patients on home parenteral nutrition: a case series. JPEN J Parenter Enteral Nutr. 2012;36(3):358–60.PubMedCrossRefGoogle Scholar
- 50.Duro D, Mitchell PD, Kalish LA, Martin C, McCarthy M, Jaksic T, et al. Risk factors for parenteral nutrition–associated liver disease following surgical therapy for necrotizing enterocolitis: a Glaser Pediatric Research Network Study [corrected]. J Pediatr Gastroenterol Nutr. 2011;52(5):595–600.PubMedPubMedCentralCrossRefGoogle Scholar
- 55.• Barron L, Courtney C, Bao J, Onufer E, Panni RZ, Aladegbami B, et al. Intestinal resection-associated metabolic syndrome. J Pediatr Surg. 2018;53(6):1142–7. This study reviews known metabolic consequences of massive small bowel resection and demonstrates that many of the metabolic consequences are independent of parenteral nutrition.PubMedPubMedCentralCrossRefGoogle Scholar
- 60.• Neelis E, Olieman J, Rizopoulos D, Wijnen R, Rings E, de Koning B, et al. Growth, body composition, and micronutrient abnormalities during and after weaning off home parenteral nutrition. J Pediatr Gastroenterol Nutr. 2018;67(5):e95. This study reviews the growth abnormalities of SBS patients and the consequences of their micronutrient deficiencies.PubMedCrossRefPubMedCentralGoogle Scholar
- 67.Pereira-Fantini PM, Thomas SL, Taylor RG, Nagy E, Sourial M, Fuller PJ, et al. Colostrum supplementation restores insulin-like growth factor-1 levels and alters muscle morphology following massive small bowel resection. JPEN J Parenter Enteral Nutr. 2008;32(3):266–75.PubMedCrossRefPubMedCentralGoogle Scholar
- 68.Kulkarni S, Mercado V, Rios M, Arboleda R, Gomara R, Muinos W, et al. Breast milk is better than formula milk in preventing parenteral nutrition-associated liver disease in infants receiving prolonged parenteral nutrition. J Pediatr Gastroenterol Nutr. 2013;57(3):383–8.PubMedCrossRefPubMedCentralGoogle Scholar
- 87.• Pet GC, McAdams RM, Melzer L, Oron AP, Horslen SP, Goldin A, et al. Attitudes surrounding the management of neonates with severe necrotizing enterocolitis. J Pediatr. 2018;199:186. This study reviews the attitudes of neonatologists and surgeons towards redirection in cases of massive small bowel loss. Many recommendations may be given without understanding of current outcomes in small bowel syndrome.PubMedPubMedCentralCrossRefGoogle Scholar
- 90.Schall KA, Holoyda KA, Grant CN, Levin DE, Torres ER, Maxwell A, et al. Adult zebrafish intestine resection: a novel model of short bowel syndrome, adaptation, and intestinal stem cell regeneration. Am J Physiol Gastrointest Liver Physiol. 2015;309(3):G135–45.PubMedPubMedCentralCrossRefGoogle Scholar