Chronic complications of the short bowel syndrome in children
- Jon A Vanderhoof, MD
Jon A Vanderhoof, MD
- Lecturer of Pediatrics, Harvard Medical School
- Professor Emeritus, University of Nebraska Medical Center
- Rosemary J Pauley-Hunter, NP-C, MS, RN
Rosemary J Pauley-Hunter, NP-C, MS, RN
- Pediatric Gastroenterology Nurse Practitioner
- Boys Town National Research Hospital, Omaha, NE
- Section Editors
- Craig Jensen, MD
Craig Jensen, MD
- Section Editor — Pediatric Gastroenterology
- Associate Professor
- Baylor College of Medicine
- Kathleen J Motil, MD, PhD
Kathleen J Motil, MD, PhD
- Section Editor — Pediatric Nutrition
- Professor of Pediatric Nutrition
- Baylor College of Medicine
Short bowel syndrome (SBS) is a malabsorptive state that typically occurs following extensive resection of the small intestine. It is a functional definition, implying a significant amount of malabsorption of both macronutrients and micronutrients and is not necessarily dependent on the loss of bowel or a particular length of bowel. SBS is the most common cause of intestinal failure, a term which refers to the state when an individual’s gastrointestinal function is inadequate to maintain his or her nutrient and hydration status without intravenous or enteral supplementation . SBS in children usually results from necrotizing enterocolitis, gastroschisis, and congenital intestinal anomalies . In addition to SBS, causes of intestinal failure in children include diseases or congenital defects that cause severe malabsorption, bowel obstruction, and dysmotility (eg, pseudo-obstruction).
Complications of SBS are related to the site of resection, the process of ileal adaptation, and the need for parenteral nutrition over the short or long-term. Acute complications that can occur at any time include watery diarrhea, electrolyte disturbances, and catheter-related complications [3,4]. Chronic complications include liver and biliary disease related to parenteral nutrition, bacterial overgrowth (sometimes associated with arthritis, colitis, or D-lactic acidosis), nutritional deficiencies, thromboses associated with central catheters, and enteric hyperoxaluria. Patients with intractable complications, or on rare occasions those with loss of adequate central venous access, may be candidates for small bowel transplantation .
The chronic complications of SBS in children are reviewed here. An overview of the management of SBS and its pathogenesis are reviewed separately. (See "Management of the short bowel syndrome in children" and "Pathophysiology of the short bowel syndrome".)
This topic is also discussed in a position statement and technical review, issued by the American Gastroenterological Association (AGA). The AGA position statement for short bowel syndrome and intestinal transplantation , as well as other AGA guidelines, can be accessed through the AGA website at www.gastro.org/practice/medical-position-statements.
Watery diarrhea is the most common early complication of short bowel syndrome (SBS) in children, and may also be a chronic or recurrent problem. It is particularly likely to occur when enteral nutrition is advanced. Most often, it occurs when the osmotic load in the small intestine lumen exceeds the absorptive capacity. The volume load presented to the intestines also may be a contributing factor. Watery diarrhea also can be caused by malabsorbed bile salts in the colon (choleretic enteropathy, a secretory diarrhea), although this usually is not the primary cause.
- O'Keefe SJ, Buchman AL, Fishbein TM, et al. Short bowel syndrome and intestinal failure: consensus definitions and overview. Clin Gastroenterol Hepatol 2006; 4:6.
- Kirby RS, Marshall J, Tanner JP, et al. Prevalence and correlates of gastroschisis in 15 states, 1995 to 2005. Obstet Gynecol 2013; 122:275.
- Duro D, Kamin D, Duggan C. Overview of pediatric short bowel syndrome. J Pediatr Gastroenterol Nutr 2008; 47 Suppl 1:S33.
- Youssef NN, Mezoff AG, Carter BA, Cole CR. Medical update and potential advances in the treatment of pediatric intestinal failure. Curr Gastroenterol Rep 2012; 14:243.
- Nucci AM, Barksdale EM Jr, Beserock N, et al. Long-term nutritional outcome after pediatric intestinal transplantation. J Pediatr Surg 2002; 37:460.
- American Gastroenterological Association. American Gastroenterological Association medical position statement: short bowel syndrome and intestinal transplantation. Gastroenterology 2003; 124:1105.
- Joly F, Dray X, Corcos O, et al. Tube feeding improves intestinal absorption in short bowel syndrome patients. Gastroenterology 2009; 136:824.
- Allard JP, Jeejeebhoy KN. Nutritional support and therapy in the short bowel syndrome. Gastroenterol Clin North Am 1989; 18:589.
- Teduglutide injection (Gattex) for short bowel syndrome. Med Lett Drugs Ther 2013; 55:29.
- Tee CT, Wallis K, Gabe SM. Emerging treatment options for short bowel syndrome: potential role of teduglutide. Clin Exp Gastroenterol 2011; 4:189.
- Sadlier C. Intestinal failure and long-term parenteral nutrition in children. Paediatr Nurs 2008; 20:37.
- Cole CR, Frem JC, Schmotzer B, 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; 156:941.
- Cole CR, Hansen NI, Higgins RD, et al. Bloodstream infections in very low birth weight infants with intestinal failure. J Pediatr 2012; 160:54.
- Sondheimer JM, Asturias E, Cadnapaphornchai M. Infection and cholestasis in neonates with intestinal resection and long-term parenteral nutrition. J Pediatr Gastroenterol Nutr 1998; 27:131.
- Beath SV, Davies P, Papadopoulou A, et al. Parenteral nutrition-related cholestasis in postsurgical neonates: multivariate analysis of risk factors. J Pediatr Surg 1996; 31:604.
- Mohammed A, Grant FK, Zhao VM, et al. Characterization of posthospital bloodstream infections in children requiring home parenteral nutrition. JPEN J Parenter Enteral Nutr 2011; 35:581.
- Terra RM, Plopper C, Waitzberg DL, et al. Remaining small bowel length: association with catheter sepsis in patients receiving home total parenteral nutrition: evidence of bacterial translocation. World J Surg 2000; 24:1537.
- Wales PW, Kosar C, Carricato M, et al. Ethanol lock therapy to reduce the incidence of catheter-related bloodstream infections in home parenteral nutrition patients with intestinal failure: preliminary experience. J Pediatr Surg 2011; 46:951.
- Oliveira C, Nasr A, Brindle M, Wales PW. Ethanol locks to prevent catheter-related bloodstream infections in parenteral nutrition: a meta-analysis. Pediatrics 2012; 129:318.
- Huang EY, Chen C, Abdullah F, et al. Strategies for the prevention of central venous catheter infections: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee systematic review. J Pediatr Surg 2011; 46:2000.
- Wong T, Clifford V, McCallum Z, 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; 36:358.
- Kayton ML, Garmey EG, Ishill NM, et al. Preliminary results of a phase I trial of prophylactic ethanol-lock administration to prevent mediport catheter-related bloodstream infections. J Pediatr Surg 2010; 45:1961.
- Pittiruti M, Hamilton H, Biffi R, et al. ESPEN Guidelines on Parenteral Nutrition: central venous catheters (access, care, diagnosis and therapy of complications). Clin Nutr 2009; 28:365.
- Cober MP, Teitelbaum DH. Prevention of parenteral nutrition-associated liver disease: lipid minimization. Curr Opin Organ Transplant 2010; 15:330.
- Premkumar MH, Carter BA, Hawthorne KM, et al. High rates of resolution of cholestasis in parenteral nutrition-associated liver disease with fish oil-based lipid emulsion monotherapy. J Pediatr 2013; 162:793.
- D'Antiga L, Goulet O. Intestinal failure in children: the European view. J Pediatr Gastroenterol Nutr 2013; 56:118.
- Vanderhoof JA, Langnas AN. Short-bowel syndrome in children and adults. Gastroenterology 1997; 113:1767.
- Quigley EM, Marsh MN, Shaffer JL, Markin RS. Hepatobiliary complications of total parenteral nutrition. Gastroenterology 1993; 104:286.
- Nightingale JM, Lennard-Jones JE, Gertner DJ, et al. Colonic preservation reduces need for parenteral therapy, increases incidence of renal stones, but does not change high prevalence of gall stones in patients with a short bowel. Gut 1992; 33:1493.
- Teitelbaum DH, Han-Markey T, Drongowski RA, et al. Use of cholecystokinin to prevent the development of parenteral nutrition-associated cholestasis. JPEN J Parenter Enteral Nutr 1997; 21:100.
- Randall LH, Shaddy RE, Sturtevant JE, et al. Cholelithiasis in infants receiving furosemide: a prospective study of the incidence and one-year follow-up. J Perinatol 1992; 12:107.
- Radetti G, Gentili L, Paganini C, Messner H. Cholelithiasis in a newborn following treatment with the somatostatin analogue octreotide. Eur J Pediatr 2000; 159:550.
- Yetkin DO, Boysan SN, Tiryakioglu O, et al. Forty month follow-up of persistent and difficultly controlled acromegalic patients treated with depot long acting somatostatin analog octreotide. Endocr J 2007; 54:459.
- Kosters A, Karpen SJ. The role of inflammation in cholestasis: clinical and basic aspects. Semin Liver Dis 2010; 30:186.
- Thompson JS. The role of prophylactic cholecystectomy in the short-bowel syndrome. Arch Surg 1996; 131:556.
- Hyman PE, Everett SL, Harada T. Gastric acid hypersecretion in short bowel syndrome in infants: association with extent of resection and enteral feeding. J Pediatr Gastroenterol Nutr 1986; 5:191.
- Jeppesen PB, Staun M, Tjellesen L, Mortensen PB. Effect of intravenous ranitidine and omeprazole on intestinal absorption of water, sodium, and macronutrients in patients with intestinal resection. Gut 1998; 43:763.
- Kaufman SS, Loseke CA, Lupo JV, et al. Influence of bacterial overgrowth and intestinal inflammation on duration of parenteral nutrition in children with short bowel syndrome. J Pediatr 1997; 131:356.
- Taylor SF, Sondheimer JM, Sokol RJ, et al. Noninfectious colitis associated with short gut syndrome in infants. J Pediatr 1991; 119:24.
- Vanderhoof JA, Young RJ, Murray N, Kaufman SS. Treatment strategies for small bowel bacterial overgrowth in short bowel syndrome. J Pediatr Gastroenterol Nutr 1998; 27:155.
- Raphael BP, Nurko S, Jiang H, et al. Cisapride improves enteral tolerance in pediatric short-bowel syndrome with dysmotility. J Pediatr Gastroenterol Nutr 2011; 52:590.
- Andres AM, Thompson J, Grant W, et al. Repeat surgical bowel lengthening with the STEP procedure. Transplantation 2008; 85:1294.
- Halperin ML, Kamel KS. D-lactic acidosis: turning sugar into acids in the gastrointestinal tract. Kidney Int 1996; 49:1.
- Bongaerts G, Bakkeren J, Severijnen R, et al. Lactobacilli and acidosis in children with short small bowel. J Pediatr Gastroenterol Nutr 2000; 30:288.
- Bongaerts G, Tolboom J, Naber T, et al. D-lactic acidemia and aciduria in pediatric and adult patients with short bowel syndrome. Clin Chem 1995; 41:107.
- Goulet O, Colomb-Jung V, Joly F. Role of the colon in short bowel syndrome and intestinal transplantation. J Pediatr Gastroenterol Nutr 2009; 48 Suppl 2:S66.
- Yang CF, Duro D, Zurakowski D, et al. High prevalence of multiple micronutrient deficiencies in children with intestinal failure: a longitudinal study. J Pediatr 2011; 159:39.
- Van Gossum A, Cabre E, Hébuterne X, et al. ESPEN Guidelines on Parenteral Nutrition: gastroenterology. Clin Nutr 2009; 28:415.
- Matarese LE, Steiger E. Dietary and medical management of short bowel syndrome in adult patients. J Clin Gastroenterol 2006; 40 Suppl 2:S85.
- Jeppesen PB, Høy CE, Mortensen PB. Essential fatty acid deficiency in patients receiving home parenteral nutrition. Am J Clin Nutr 1998; 68:126.
- Compston JE, Ayers AB, Horton LW, et al. Osteomalacia after small-intestinal resection. Lancet 1978; 1:9.
- Khan FA, Fisher JG, Bairdain S, et al. Metabolic bone disease in pediatric intestinal failure patients: prevalence and risk factors. J Pediatr Surg 2015; 50:136.
- Dellert SF, Farrell MK, Specker BL, Heubi JE. Bone mineral content in children with short bowel syndrome after discontinuation of parental nutrition. J Pediatr 1998; 132:516.
- Appleman SS, Kalkwarf HJ, Dwivedi A, Heubi JE. Bone deficits in parenteral nutrition-dependent infants and children with intestinal failure are attenuated when accounting for slower growth. J Pediatr Gastroenterol Nutr 2013; 57:124.
- Tangpricha V, Luo M, Fernández-Estívariz C, et al. Growth hormone favorably affects bone turnover and bone mineral density in patients with short bowel syndrome undergoing intestinal rehabilitation. JPEN J Parenter Enteral Nutr 2006; 30:480.
- Williams HE. Oxalic acid and the hyperoxaluric syndromes. Kidney Int 1978; 13:410.
- Obialo CI, Clayman RV, Matts JP, et al. Pathogenesis of nephrolithiasis post-partial ileal bypass surgery: case-control study. The POSCH Group. Kidney Int 1991; 39:1249.
- Emmett M, Guirl MJ, Santa Ana CA, et al. Conjugated bile acid replacement therapy reduces urinary oxalate excretion in short bowel syndrome. Am J Kidney Dis 2003; 41:230.
- WATERY DIARRHEA
- CATHETER RELATED COMPLICATIONS
- Mechanical failure
- HEPATOBILIARY DISEASE
- Liver disease
- - Prevention
- ESOPHAGITIS/PEPTIC ULCER DISEASE
- ANASTOMOTIC ULCERS
- SMALL INTESTINE BACTERIAL OVERGROWTH
- D-lactic acidosis
- NUTRIENT DEFICIENCIES
- Common deficiencies
- Metabolic bone disease and rickets
- HYPEROXALURIA AND KIDNEY STONES
- SUMMARY AND RECOMMENDATIONS