DISCOVERIES REPORTS (ISSN 2393249X), 2016, volume 2

CITATION: 

Stancu SM, Cirstoveanu CG. The Long-Term Treatment of Short Bowel Syndrome. Discoveries Reports 2016; 2: e6. DOI: 10.15190/drep.2016.2

 Submitted: July 3rd, 2016; Revised: August 2nd, 2016; Accepted: August 4th, 2016; Published: August 10th, 2016;

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The Long-Term Treatment of Short Bowel Syndrome

Samantha McKenzie Stancu (1,*), Catalin Gabriel Cirstoveanu (2,*)

(1) Carol Davila University of Medicine & Pharmacy, 37 Dionisie Lupu Street, District 1, Bucharest, Romania

(2) Marie S. Curie Children’s Emergency Hospital, 20 Constantin Brancoveanu Street, District 4, Bucharest, Romania; 

*Correspondence to: Catalin Gabriel Cirstoveanu MD, PhD, Chief of Neonatal Intensive Care Unit Marie S. Curie Children’s Emergency Hospital and Samantha McKenzie Stancu, Carol Davila University of Medicine & Pharmacy, 37 Dionisie Lupu Street, District 1, Bucharest, Romania; Emails: cirstoveanu@yahoo.com and samantha.mckenzie730@gmail.com

Abstract

Short bowel syndrome (SBS) is the most common cause of intestinal failure in the pediatric population. Functionally, SBS is defined as the spectrum of malabsorption features occurring after extensive surgical resection. Congenital SBS is a rare entity, with an estimated prevalence of 0.02-0.1% among all live births. The mainstay of treatment consists of parenteral nutrition and intestinal rehabilitation, comprising surgical reconstruction. The two main procedures are Longitudinal Intestinal Lengthening and Tailoring procedure (LILT) and Serial Transverse Enteroplasty Procedure (STEP). Transplantation is reserved as treatment of last resort, when intestinal rehabilitation has been unsuccessful. Short bowel syndrome is a debilitating disease, whose management stretches over the course of years. However, with the innovation of surgical techniques such as LILT and STEP, as well as advances in multidisciplinary intestinal rehabilitation centers, long-term survival rates are approaching 90%. In this review we discuss the current and future treatment options for SBS.

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References

1. Oliviera C, de Silva N, Wales, P. Five-year outcomes after serial transverse enteroplasty in children with short bowel syndrome. J of Pediatr Surg 2012; 47: 931-7. 

2. Modi P, Jaksic T. Pediatric intestinal failure and vascular access. Surg Clin  North Am 2012; 93: 729-43. 

3. Kocoshis S. Medical management of pediatric intestinal failure. Semin Pediatr Surg 2010; 19: 20-6. 

4. Wales P, de Silva N, Kim H, Lecce L, Sandhu A, Moore A. Neonatal short bowel syndrome- A cohort study. J Pediatr Surg 2005; 40: 755-62.  

5. Modi B, Langer M, Ching Y. Improved survival in a multidisciplinary short bowel syndrome program. J Pediatr Surg 2008; 43:20-4.  

6. Smith N, Harwood R, Almond S. Short bowel syndrome- surgical perspectives and outcomes. J Paediatr Child Health 2014; 24: 513-8. 

7. Pierro A, Nigel H. Surgical treatment of infants with necrotizing enterocolitis. Semin Neonatol 2003; 8: 223-32. 

8. De Legge M, Alsolaiman M, Barbour E, Bassas S, Siddiqi M, Moore N. Short bowel syndrome: Parenteral nutrition versus intestinal transplantation. Where are we today? Dig Dis and Sci 2007; 52: 876-92. 

9. Wales P, de Silva N, Kim J, Lecce L, To T, Moore A. Neonatal short bowel syndrome: population-based estimates of incidence and mortality rates. J  Pediatr Surg 2004; 39: 690-5. 

10. Frongia G, Kessler M, Weih S, Nickkholgh A, Mehrabi A, Cunz    S.  Comparison of LILT and STEP procedures in children with short bowel syndrome – A Systematic review of the literature. J Pediatr Surg 2013; 48: 1794-1805. 

11. Sulkowski J, Minneci P. Management of short bowel syndrome. Pathophysiology 2014; 21: 111-8. 

12. Raschperger E, Engstrom U, Pettersson RF, Fuxe J. CLMP, a Novel Member of the CTX Family and a New Component of Epithelial Tight Junctions. J Biol Chem. 2004;279(1):796–804.

13. Cserni T, Varga G, Erces D, Kaszaki J, Boros M, Laszlo A et al. Spiral intestinal lengthening and tailoring- first in vivo study. J Pediatr Surg 2013; 48: 1907-1913. 

14. Van Der Werf CS, Wabbersen TD, Hsiao N, Paredes J, Etchevers HC, Kroisel PM, et al. CLMP is required for intestinal development, and loss-of-function mutations cause congenital short-bowel syndrome. Gastroenterology 2012;142(3):453–62. 

15. Coletta R, Khalil BA, Morabito A. Short bowel syndrome in children: Surgical and medical perspectives. Semin Pediatr Surg 2014; 23: 291-7. 

16. Quiros-Tejeira M, Ament L, Reyen F, Herzog M, Merjanian N, Olivares-Serrano J et al. Long-term parenteral nutrition support and intestinal adaptation in children with short bowel syndrome: a 25 year experience. J  Pediatr 2004; 145: 157-63. 

17. Kelly, D.A. Failure-associated liver disease. What do we know today? Gastroenterol 2006; 30: 70-7. 

18. Yang C.F, Lee M, Valim C. Persistent alanine aminotransferase elevations in children with parenteral nutrition-associated liver disease. J Pediatr Surg 2009; 44: 1087-8. 

19. Andorsky D, Lund D, Lillehei C. Nutritional and other postoperative management of neonates with short bowel syndrome correlates with clinical outcomes. J Pediatr 2001; 139: 27-33. 

20. Gutierrez I, Kang K, Jaksic T. Neonatal short bowel syndrome. Semin Fetal Neonatal Med 2011; 16: 157-63. 

21. Lee O.K, Johnston L. A systematic review of effective management of central venous catheter infections in pediatric patients with intestinal failure. J Pediatr Surg 2010; 45: 1289-93. 

22. Jones BA, Hull MA, Richardson DS. Efficacy of ethanol locks in reducing central catheter venous catheter and catheter infections in pediatric patients with intestinal failure. J Pediatr Surg 2010; 45: 1287-93. 

23. Kaufman SS, Loseke CA, Lupo JV. Influence of bacterial overgrowth and intestinal inflammation on duration of parenteral nutrition in children with short bowel syndrome. J Pediatr 1997; 131: 356-61.

24. Wilmore D. Factors correlating with a successful outcome following extensive intestinal resection in newborn infants. J Pediatr1972; 80: 80-95.

25. Fitzgibbons S, Ching Y, Valim C. Relationship between serum citrulline levels and progression to parenteral nutrition independence in children with short bowel syndrome. J Pediatr Surg. 2009; 44(5): 928-32.

26. Perlmutter DH, Boyle JT, Campos JM. D-Lactic acidosis in children: an unusual metabolic complication of small bowel resection J Pediatr 1983; 104: 234-38.

27. Land MH, Rouster-Stevens K, Woods CR. Lactobacillus sepsis associated with probiotic therapy. Pediatrics 2005; 115: 178-81. 

28. Williamson R.C. Intestinal adaptation (one of two parts). Structural, functional and cytokinetic changes. N Engl J Med 1978; 298: 1393-1402. 

29. Williamson R.C. Intestinal adaptation (two of two parts). Mechanisms of control. N Engl J Med 1978; 298: 1444-50. 

30. Shin J, Namgung R, Park M.  Could lipid infusion be a risk for parenteral nutrition-associated cholestasis in low-birth weight neonates? Eur J Pediatr 2008: 167: 197-202. 

31. Pakarinen M, Kurvinen A, Koivusalo A, Iber T, Rintala R. Long- term controlled outcomes after autologous intestinal reconstruction surgery in treatment of severe short bowel syndrome. J Pediatr Surg; 2013. 48: 339-44. 

32. Dicken BJ, Sergi C, Rescorla FJ, Breckler F, Sigalet D. Medical management of motility disorders in patients with intestinal failure: a focus on necrotizing enterocolitits, gastroschisis and intestinal atresia. J Pediatr Surg 2011; 46: 1618-30. 

33. Ng, PC. Use of oral erythromycin for the treatment of gastrointestinal dysmotility in preterm infants. Neonatol 2009; 95: 97-104. 

34. Jeppesen PB, Sanguinetii EL, Buchman A. Teduglutide (ALX-0600), a dipepitdyl peptidase IV resistant glucagon-like peptide 2 analogue, improves intestinal function in short bowel syndrome patients. Gut 2005; 54: 1224-31. 

35. Jeppensen P, Hartmann B, Hansen B, Thulesen J, Holst J, Mortensen P. Impaired meal stimulated glucagon-like peptide 2 response in ileal resected short bowel patients with intestinal failure. Gut 1999; 45: 556-63. 

36. Bianchi A. Intestinal loop lengthening- a technique for increasing small intestinal length. J Pediatr Surg 1980; 15: 145-51.

37. Huskisson, LJ, Brereton RJ, Kiely EM, Spitz L. Problems with intestinal lengthening. J Pediatr Surg 1993; 28: 720-2. 

38. Kim HB, Lee PW, Garza C,  Oh JT, Nurko T, Jaksic T. Serial transverse enteroplasty (STEP): a novel lengthening bowel procedure. J Pediatric Surg 2003; 38: 425-9. 

39. Sudan DJ, Thompson J, Botha J. Comparison of intestinal lengthening procedures for patients with short bowel syndrome: a systematic review of the Bianchi and STEP procedures. Ann Surg 2007; 24: 593-604. 

40. Kaji T, Tanaka H, Wallace LE. Nutritional effects of the serial transverse enteroplasty procedure in experimental short bowel syndrome. J Pediatr Surg 2009; 44: 1552-9. 

41. Modi BP, David PJ, Jaksic T. First report of the international serial transverse enteroplasty data registry: indications, efficacy, and complications. J Am Coll Surg 2007; 204: 365-371. 

42. Mazariegos GV, Steffick DE, Horslen S, Farmer D, Grant D, Langnas A et al. Intestine transplantation in the United States, 1999-2008. Am J Transplant 2010; 10: 1020-1034. 

43. Grant D, Wall W, Mimemault R. Successful small-bowel/liver transplantation. Lancet 1990; 335: 181-4.

44. Kaufman SS, Atkinson JB, Bianchi A, Goulet OJ, Grant D, Langnas AN et al. Indications of pediatric intestinal transplantation: a position paper of the American Society of Transplantation. Pediatr Transpl 2001; 5:2: 80-87. 

45. Nayyar G, Mazariegos GV, Ranganathan, Soltys K, Bond G, Jaffe Q et al. Pediatric small bowel transplantation. Semin Pediatr Surg 2010; 19: 68-77. 

46. Hamilton J, Reilly B, Morecki R. Short small intestine associated with malrotation: a newly described congenital cause of intestinal malabsorption. Gastroenterology. 1969;56:124–36.

47. Erez I, Reish O, Kovalivker M, Lazar L, Raz A, Katz S. Congenital short-bowel and malrotation: clinical presentation and outcome of six affected offspring in three related families. Eur J Pediatr Surg. 2001;11:331–4.

48. Polette M, Mestdagt M, Bindels S, Nawrocki-Raby B, Hunziker W, Foidart JM, et al. β-catenin and ZO-1: Shuttle molecules involved in tumor invasion-associated epithelial-mesenchymal transition processes. Cells Tissues Organs. 2007;185(1-3):61–5. 

49. Vacanti JP, Morse MA, Saltzman WM, Domb AJ, Perez-Atyade A, Langer R. Selective cell transplantation using bioabsorbable artificial polymers as matrices. J Pediatr Surg 1988; 23: 3-9.

50. Choi RS, Vacanti JP. Preliminary study of tissue-engineered intestine using isolated epithelial units of tubular synthetic biodegradable scaffolds. Transplant Proc 1997; 29 (1-2): 848-851. 

51. Finkbeiner S, Freeman J, Wieck MM, El-Nachef F, Altcheim CH, Tsai YH et al. Generation of tissue-engineered small intestine using embryonic stem cell-derived human intestinal organoids. Biol Open 2015; 4:11: 1462-1472. 

52. Squires RH, Duggan C, Teitelbaum DH. Natural history of pediatric intestinal failure: initial report from the Pediatric Intestinal Failure Consortium. J Pediatr 2012; 161: 723-8.