Short Bowel Syndrome Sang-Mo Kang, M.D. Division of Transplantation - PowerPoint PPT Presentation

Disaster Short Bowel Syndrome Sang-Mo Kang, M.D. Division of Transplantation Director, Intestinal Rehabilitation and Transplantation University of California, San Francisco Short Bowel Syndrome Disaster Normal Short Bowel Feldman’s GastroAtlas online 1

Overview Intestinal Failure: Definition • Definition/Incidence of Intestinal Failure • Intestinal Physiology • A condition in which inadequate digestion and/or absorption of • Etiology and Pathophysiology nutrients leads to malnutrition and/or • Intestinal adaptation dehydration • Medical Management –rehabilitation • Inability of the native gastrointestinal • Surgical Management tract to provide nutritional autonomy • Intestinal transplantation Nightingale J, ed. Intestinal Failure. 2001 Fishbein TM et al. Gastroenterology 2003;124:615 Incidence and Prevalence Causes of Intestinal Failure: Major Categories • 3-4/million in western countries eventually develop intestinal failure • Occurs in ~ 15% of pts undergoing intestinal resection • Loss of bowel length – ¾ occur from massive resection – ¼ from multiple sequential resections • Loss or absence of bowel • ~70 % pts with SBS are d/c from the hospital & ~ 70% of these function remain alive one year later • Improved Survival is due to ability to deliver long-term nutritional • Unresectable tumors support DiBaise JK et al. Am Gastro 2004; 99: 1386-95 Thompson JS. J Gastrointestinal Surg 2000; 4 :101-4. Messing B. et al Gastroenterology 1999; 117:1043-50 2

Pathophysiology Short Bowel Syndrome (SBS) INTESTINAL REMNANT LENGTH is the primary • determinant of outcome but quality also important • Defined as loss of � 2/3 of small bowel • Resection of up to ½ of the SB is usually well tolerated (remnant of <200 cm) • SBS is most likely to develop in patients losing > 2/3 length of SB. Wilmore DW, Best Pract Res Clin Gastroenterol 2003;17:895 • Adults likely to require long-term TPN: – <50 cm small bowel AND colon • “Functional” definition? (Fecal energy – <100 cm small bowel AND NO colon loss) • Children likely to require long-term TPN: – <30 cm small bowel • Most common condition resulting in • Presence of ileocecal valve is highly advantageous – Due to presence of ileum, prevention of bacterial reflux intestinal failure Carbonnel F et al JPEN 1996;20:275-80. DiBaise JK et al., Am J Gastroenterol 2004;99:1386 Normal Intestinal Function Intestinal Growth �������������������������������� • Small intestine ��� – Greatest growth velocity during ��� last trimester ���������� � ��� – Term ~275cm ��� • Colon ��� – 30-40cm at birth ��� � � �� �� – 1.5-2.0m in adult ��������� Feldman’s GastroAtlas online Weaver LT et al Gut 1991;32:1321-3 3

Stages Following Massive Resection Intestinal Adaptation • Muscular hypertropy • Large fluid/electrolyte losses (weeks) – Increased bowel diameter – Increased wall thickness • Fewer fluid and electrolyte problems; need for nutritional support (months/year) • Mucosal hyperplasia – TPN weaning? – Crypt cell proliferation – Increased number of enterocytes • Intestinal adaptation – Villous hyperplasia • Lengthening DiBaise JK et al. , Am J Gastroenterol 2004;99:1386 Sacks AI et al J Pediatr Gastroenterol Nutr 1995;21:158-64 �������������������������������� Intestinal Adaptation • Hormonal mediators • Dependent on enteral nutrients – Growth hormone • May take 1-2 years – Glucagon like peptides – Enteroglucagon – Neurotensin • Ileum adapts for macronutrient absorption – Peptide YY – Insulin-like growth factor • Blunted adaptation: Active Crohn’s, pubs.acs.org radiation enteritis, carcinoma, • Luminal factors pseudoobstruction – Glutamine – Polyamines – Epidermal growth factor – Trefoil peptides – Short chain fatty acids DiBaise JK et al. , Am J Gastroenterol 2004;99:1386 – Long chain fatty acids Buchman AL et al. Gastroenterology 2003;124:1111 4

SBS: Medical Management Medical Management • Fluid and electrolytes • Early management: Critically ill in post-op setting – Oral rehydration solution – Control of sepsis, maintenance of fluid and electrolyte balance – Antisecretory agents (PPI) – TPN is required early – Antimotility agents – Initiation of enteral feeds when possible • Lomotil, Imodium, tincture of opium – Fluid and electrolytes losses are high in post-op period management can be challenging – Supplemental IV fluids may be required in addition to TPN • For pts that survive the early phase, goals are to maintain adequate nutritional status and prevent complications • Micronutrients MAINTENANCE OF NUTRITIONAL STATUS • BECOMES THE PRIMARY GOAL ���������������������� Medical Management -Dietary Management- • Gastric acid hypersecretion • Pts should eat more than usual (hyperphagic) • Metabolic bone disease • Small meals throughout the day and/or tube feeds • Calcium deficiency • Pts with colonic continuity should eat complex CHO • Renal calculi with starch, non-starch polysaccharides and • Hyperoxaluria soluble fibers (not absorbed by SB). • Liver disease – Colon ferments these carbs � butyrate (fuel) • Cholelithiasis – 500-1000 Kcals can be absorbed from colocytes • Bacterial overgrowth – Amount of energy absorbed is proportional to the • D-lactic acidosis length of the residual colon and may increase with • Neurologic syndrome adaptive response to resection – Medium chain triglycerides can be absorbed in the colon 5

Glucagon like peptide 2 SBS: Pharmacologic Options • Proglucagon-derived peptides – Synthesized in L cells • Antisecretory, antimotility agents • Tissue specific post-translational processing of proglucagon in the intestine liberates PGDPs • Antibiotics for overgrowth • Growth hormone? • Glucagon-like peptide II (Gattex) • Glutamine supplementation of feeds • Highly localized expression of GLP-2 receptor in intestinal epithelium Glucagon like peptide 2 Teduglutide (Gattex) • Secreted in response to food ingestion • Promotes nutrient absorption by expansion of the mucosal epithelium • Stimulates crypt cell proliferation • Inhibitory effects on motility and secretion • Post-prandial GLP-2 secretion is impaired in patients without a terminal ileum or colon Jeppesen et al Gut. 2011;60:902-14 6

How to feed • CONTINUOUS ENTERAL FEEDINGS ARE ADVANTAGEOUS – Via NG or GT – Constant saturation of carrier transport proteins – Take full advantage of absorptive surface area available • Older children have better capacity to regulate gastric emptying Jeppesen et al Gut. 2011;60:902-14 How to feed Home Parental Nutrition • TPN should be compressed volume and time • ADVANCE SLOWLY of infusion. (preferably over night) – Concentration vs. volume • Tapered over 30-60 min to avoid • Small quantities of oral feedings hypoglycemia. – Scheduled at least 2-3 times per day • Complications; – Stimulate suck swallow – Avoid line sepsis (0.3/ year) – Minimize feeding aversion – Line thrombosis Woolf GM et al Gastroenterolgy 1983; 84;823-8 7

PNALD PN complications • Biochemical elevations in: – Serum aminotransferases – Alkaline phosphatase, GGT • Catheter related: • Metabolic: – Bilirubin – Liver disease • Histologic changes – Sepsis – Biliary stones – Steatosis – Access – Metabolic bone – Steatohepatitis disease – Venous thrombosis – Cholestasis – Trace element – Occlusion – Cirrhosis and/or vitamin – Migration deficiency • May improve with decreased lipid infusion and/or switch to Omega-3 enriched lipids SBS: Surgical Management Surgical Management • Ostomy closure • Dilated segments of bowel with ineffective • Restoration of bowel continuity peristalsis are associated with: • Bowel lengthening and tapering – bacterial overgrowth – secretory diarrhea procedures – mucosal inflammation – increased malabsorption – increase risk of liver disease • Aims: – Increase total length of small bowel, prevent stasis in dilated segment 8

Increasing Absorptive Surface Area Surgical Management Bianchi Procedure • Experimental animal models: – Contruction of intestinal valve or sphincter – Denervation of intestinal segments • Human Experience: – Reversing segments of intestine • Antiperistaltic “physiologic valve” – Bowel lengthening procedures: • Bianchi – longitutinal lengthening • STEP – serial transverse enteroplasty Bianchi A. J Ped Surg 1980;15:145 Increasing Absorptive Surface Area Serial Transverse Enteroplasty (STEP) • Pt selection- Dilated intestinal segment, bacterial overgrowth. • Stapler is from Alternating directions. • Less complicated than Bianchi Procedure. • Improves absorptive capacity in ~ 90% pts. • Complications: – Leak and obstruction ~20% Kim H et al. J Ped Surg 2003;38:425 9