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SHORT BOWEL SYNDROME. Wong Wui Bun Tuen Mun Hospital. Short bowel syndrome. Overview Pathophysiology Intestinal rehabilitation program Medical treatment Operative treatment Transplantation. 1. OVERVIEW. Heterogeneous disease. 1. Overview. Definition:
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SHORT BOWEL SYNDROME Wong Wui Bun Tuen Mun Hospital
Short bowel syndrome • Overview • Pathophysiology • Intestinal rehabilitation program • Medical treatment • Operative treatment • Transplantation
1. OVERVIEW Heterogeneous disease
1. Overview • Definition: • Malabsorptive state that is associated with extensive resection of small bowel as well as a range of congenital conditions (American College of Surgeons) • Heterogeneous group of patients • Spectrum of disease severity • Reduced survival • (2 year 86%, 5 year 75%) • Significant morbidity http://www.nejm.org/na101/home/literatum/publisher/mms/journals/content/nejm/2011/nejm_2011.364.issue-14/nejmicm1001885/production/images/large/nejmicm1001885_f1.jpeg
1. Overview - causes Mesenteric ischaemia is the most common cause in adult
2. PATHOPHSIOLOGY Anatomy is the key
2. Pathophysiology • Fluid, electrolyte and nutritional deficiencies • Dysregulation of enteric hormone • Disturbance in bowel motility • Change in bowel flora • Catheter related complications • Intestinal failure related liver failure • Bone resorption, gallstone and renal stones
Surgical considerations • Limit resection • Use of second look operation • Prevention of stoma/ early closure • Preservation of ileocaecal valve • Post-operative care • Early establishment of central venous assess • Early involvement of multi-disciplinary team
3. INTESTINAL REHAB Planned multidisciplinary care
3. Intestinal rehabilitation program • Multidisciplinary, protocolized care • Combination of enteric nutrition +/- hormonal stimulation • Workload hypothesis • Oral nutrition stimulate intestinal adaptation Morphological: Epithelial hyperplasia Increased villi height Increased crypt depth Remodeling of bowel Functional: Up-regulation of transport molecule and brush border activity http://surgery.med.umich.edu/pediatric/chirp/clinical/mm/pathophysiology.shtml
3. Intestinal rehabilitation prgram • Systemic review 2013 by Stanger et.al. • Historical control (n=103) vs IRP (n=130) • Reduction in septic episodes (0.3 vs. 0.5 event/month; p=0.01) • Increase in overall patient survival (22% to 42%) • Weaning from PN (RR=1.05, 0.88-1.25, p=0.62) • Incidence of IFALD (RR=0.2, 0-17.25, p=0.48 • Relative risk of liver transplantation (3.99, 0.75-21.3, p=0.11).
Intestinal failure • Predictors: • Bowel length <100 cm • End jejunostomy/ jejunocolic anastomosis • Reduced Citrulline level (<20umol/L correlated with PN dependence) Definition: Failure of intestine to adequately meet the body’s requirement for fluid, macronutrients and micronutrients Long term parenteral nutrition required
4. MEDICAL TREATMENT Nutrition is backbone, what is more…
Medical treatment • Bacterial overgrowth • Increase parenteral nutrition requirement • D- lactic acidosis, mucositis, worsen diarrhoea • More common if ileocaecal valve absent • Empirical treatment with antibiotics
Medical treatment • Symptomatic care • Control of bowel motility and secretions • Lomotil, Imodium • Atropine • Proton pump inhibitor
5. OPERATIONS In selected patients
Autologus intestinal reconstruction • Indicated in intestinal failure with complications • Intestinal tapering • Longitudinal intestinal lengthening and tailoring (LILT) • Serial transverse enteroplasty (STEP) • Antiperistaltic segment • Colonic interposition
Choice of procedure • Preservation of absorptive surface • Dilated segment has impaired peristalsis • Technical difficulty • Feasibility of procedure: • Any bowel dilatation? • Any previous procedure? • Problem with transit time? • Antiperistaltic segment • Patient with a dilated bowel? • STEP/ LILT
Role of operation • Improve bowel autonomy • Decrease PN requirement • Decrease need for transplantation • Reverse liver disease up to 80% • Complications: • Intestinal obstruction • Anastomotic leakage • Bowel ischaemia • Mortality ~ 10%
6. TRANSPLANTATION Graft and patient survival problem
Transplantation • Intestinal transplant • Combined liver-intestinal transplant • Indications: • 1. Presence of PN-associated liver disease • 2. Loss of central venous access • 3. Recurrent catheter-related sepsis or a single episode of fungal sepsis • 4. Recurrent bouts of severe dehydration or metabolic abnormalities • (US Centers for Medicare and Medicaid Services) • ?Better catheter care • ?Improved parenteral nutrition • ?Quality of life • Early referral to specialist centre
Bring home message • Limit bowel resection • Early stoma closure • Intestinal rehabilitation program • STEP vs LILT • Considerations for transplant
Reference • Modern treatment of short bowel syndrome. Jeppesen PB. Curr Opin Clin Nutr Metab Care. 2013 Sep;16(5):582-7. doi: 10.1097/MCO.0b013e328363bce4. Review. • Short bowel syndrome – surgical perspectives and outcomes. Nicola Smith, Rachel Harwood, Sarah Almond. Paediatrics and Child Health Volume 24, Issue 11, November 2014, Pages 513–518 • Serial transverse enteroplasty (STEP) for patients with short bowel syndrome (SBS). American College of Surgeons. • Management of short bowel syndrome. Jason P. SulkowskixJason P. Sulkowski. Pathophysiology. February 2014. Volume 21, Issue 1, Pages 111–118 • Surgical management of short bowel syndrome. Iyer KR1. JPEN J Parenter Enteral Nutr. 2014 May;38(1 Suppl):53S-59S. doi: 10.1177/0148607114529446. Epub 2014 Mar 25. • Long-term outcome of short bowel syndrome in adult and pediatric patients. Wasa M1, Takagi Y, Sando K, Harada T, Okada A. JPEN J Parenter Enteral Nutr. 1999 Sep-Oct;23(5 Suppl):S110-2. • Effect of growth hormone, glutamine, and enteral nutrition on intestinal adaptation in patients with short bowel syndrome. Guo M, Li Y, Li J. Turk J Gastroenterol. 2013;24(6):463-8. • Short bowel syndrome: highlights of patient management, quality of life, and survival. Kelly DG1, Tappenden KA, Winkler MF. JPEN J Parenter Enteral Nutr. 2014 May;38(4):427-37. doi: 10.1177/0148607113512678. Epub 2013 Nov 18. • Overview of short bowel syndrome: clinical features, pathophysiology, impact, and management. Storch KJ1. JPEN J Parenter Enteral Nutr. 2014 May;38(1 Suppl):5S-7S. doi: 10.1177/0148607114525805. Epub 2014 Mar 6. • Pathophysiology of short bowel syndrome: considerations of resected and residual anatomy. Tappenden KA1. JPEN J Parenter Enteral Nutr. 2014 May;38(1 Suppl):14S-22S. doi: 10.1177/0148607113520005. Epub 2014 Feb 5. • Teduglutide: A Review of its Use in the Treatment of Patients with Short Bowel Syndrome. Celeste B. Burness, Paul L. McCormack. Drugs. June 2013, Volume 73, Issue 9, pp 935-947 • Are plasma citrulline and glutamine biomarkers of intestinal absorptive function in patients with short bowel syndrome? Luo M1, Fernández-Estívariz C, Manatunga AK, Bazargan N, Gu LH, Jones DP, Klapproth JM, Sitaraman SV, Leader LM, Galloway JR, Ziegler TR. JPEN J Parenter Enteral Nutr. 2007 Jan-Feb;31(1):1-7. • Intestinal dysbiosis in children with short bowel syndrome is associated with impaired outcome. Engstrand Lilja H, Wefer H, Nyström N, Finkel Y, Engstrand L. Microbiome. 2015 May 4;3:18. doi: 10.1186/s40168-015-0084-7.
THANK YOU! Questions welcomed
Elemental diet • Peptamen • Vivomax • Monosaccharides • Disaccharides • Medium chain fatty acid • Amino acids • Vitamins • Minerals
Parenteral nutrition • >50% carbohydrates • 30-40 % fat emulsion • Amino acids • Electrolytes • Additives: • Vitalipid: Vitamin A, D2, E, K1 • Soluvit: Vitamin C, Vitamin H, Vitamin Bs, folic acid • Addamel: trace elements
Options of venous access • Considerations: • Venous thrombosis rate per 1000 catheter day • Sepsis rate per 1000 catheter day • Reusability • Tunneled central venous access • Peripheral inserted central catheter (PICC) • Aseptic technique • 70% ethanol block • Heparin solution flush
Benefit of stoma closure • Recruit of distal bowel for adaptation • Resumption of enterohepatic circulation of bile salt • Production of short chain fatty acid (SCFA) in colon • Activation of L cell for enteric hormone production
Transplantation • 5 hours cold ischaemic time • Higher level of immunosuppression • Higher risk of graft rejection • Higher risk of drug toxicity • Higher risk of lymphoproliferative disease