Dr. Henrik Csaba Horvath Short bowel syndrome Dr. Henrik Csaba Horvath Bible class February 20, 2013

Definition of short-bowel syndrome Management of gastric polyps Definition of short-bowel syndrome Loss of intestinal absorption from surgical resection, congenital defect or diseases characterized by the inability to maintain protein-energy, fluid, electrolyte, or micronutrient balances when on a conventionally accepted, normal diet Bible class 31.10.2012

Which conditions can lead to a short-bowel syndrome? Management of gastric polyps Which conditions can lead to a short-bowel syndrome? Physical loss of portions of intestine Loss of function c Obstruction Dysmotility Surgical resection (volvulus) Congenital defect Disease-associated loss of absorption (Crohn`s, postirradiation) Loss of bowel or enterocyte mass (trauma, infarction) SBS-associated intestinal failure Surgical resection: volvulus, intestinal pseudoobstruction Bible class 31.10.2012

Major causes of SBS in adults? Management of gastric polyps Major causes of SBS in adults? Postoperative complications Irradiaton/cancer Mesenteric valvular disease (mesenteric ischemia) Crohn`s disease Trauma Functional improvement can take up to 2 years Bible class 31.10.2012

Management of gastric polyps Which factors are associated with worse prognosis in patients with SBS? Total parenteral nutrition < 50 cm intact bowel Enterostomy Radiation or ischemic enteritis Bible class 31.10.2012

Which factors have impact on the outcome of SBS? Management of gastric polyps Which factors have impact on the outcome of SBS? Length of the remaining intestine Segment of intact bowel (jejunum vs. ileum, colon continuity) Presence/abscence of ileocaecal valve Absorptive quality of the remnant bowel Outcome of SBS Presence of residual underlying disease (e.g.Crohn`s) State of other digestive organs Efficacy of nutrition support Pharmacologic therapy Age/BMI of the patient Bible class 31.10.2012

How does affect the remnant length the risk of developing SBS? Management of gastric polyps How does affect the remnant length the risk of developing SBS? Patients at highest risk of developing SBS are Anastomosis PLUS length of residual small intestine Duodenostomy or jejunoileal < 35 cm Jejunocolic or ileocolic < 60 cm End jejunostomy < 115 cm Functional improvement can take up to 2 years Bible class 31.10.2012

Which impact has on absorption the resection of… Management of gastric polyps Which impact has on absorption the resection of… Jejunum: Loss of the major intestinal absorption area Loss of digestive enzymes Loss of GI feedback hormones (gastric emptying) Ileum: Loss of the absorption of jejunal secretion Loss of absorption of vitamin B12 Loss of absorption of bile salts (fat malabsorption) Functional improvement can take up to 2 years Bible class 31.10.2012

Which impact has the loss of ileocaecal valve? Management of gastric polyps Which impact has the loss of ileocaecal valve? Dilatation of the small intestine Slower motility Bacterial overgrowth of the small intestine Competition for nutrients, inflammation, GI bleeding, bacterial translocation ± endotoxaemia, liver injury, D-lactic acidosis Bible class 31.10.2012

Which adaptive mechanisms occur in the residual intestine/ GI tract? Management of gastric polyps Which adaptive mechanisms occur in the residual intestine/ GI tract? Mucosal hyperplasia (due to fat stimulated glucagon-like peptide receptor II) Increased mucosal blood flow Improved segmental absorption Gastric hypersecretion Increased pancreatobiliary secretions Functional improvement can take up to 2 years Up to 70% can do without TPN due to these mechanisms Bible class 31.10.2012

Importance of colon in compensation for the lack of intestine? Management of gastric polyps Importance of colon in compensation for the lack of intestine? Increase reabsorption of water, electrolytes, short-chain fatty acids and GI secretions Slow down the intestinal transit and stimulate intestinal adaptation by hormonal regulation Fermentation of malabsorbed carbohydrates by colonic bacteria Functional improvement can take up to 2 years Bible class 31.10.2012

Measurement of the functional capacity of the small intestine? Management of gastric polyps Measurement of the functional capacity of the small intestine? 1. 48-hour nutritional balance test analysis of daily absorption rate (intake-output) prediction of intestinal failure: <1,4 kg wet weight/day 1170 kcal/day of energy (Difficulties with duplicate food portions and accurate stool collections) 2. Fasting plasma citrulline concentration (>5 μmol/L) (is synthetized by the small intestine, best practical measure of enterocyte function) Citrullin : not-proteinogenic alpha amino-acid (in rind of watermelon) Bible class 31.10.2012

Two major groups of complications of SBS? Management of gastric polyps Two major groups of complications of SBS? Early complications: dehydration electrolyte derangements (Mg, Ca, K) Diagnosis: urinary electrolyte levels (plasma can be normal!) Treatment: sustained correction due to slow cellular uptake Late complications: TPN-related bacterial overgrowth micronutrient deficiency metabolic Bible class 31.10.2012

TPN-related late complications of SBS are… Management of gastric polyps TPN-related late complications of SBS are… 1. Due to bypass first pass liver metabolism: steatosis cholestasis gallstones, cirrhosis (IFALD) end-stage liver cirrhosis in 15% of pts after one year TPN 100% mortality rate within 2 yrs IFALD: intestinal failure-associated liver disease 2. Catheter-associated complications: infection: one-third of deaths in 50% 5-yr-mortality rate in SBS thrombosis (v. cava superior): 0.2/1000 catheter days Bible class 31.10.2012

Bacterial overgrowth-related late complications of SBS are… Management of gastric polyps Bacterial overgrowth-related late complications of SBS are… 1. carbohydrate malabsorption 2. sepsis due to bacterial localisation 3. decreased absorption of fatty acids due to interference with chilomicron formation 4. loss of absorptive capacity due to inflammatory response Bible class 31.10.2012

Management of gastric polyps Which are the most common micronutritient deficiencies as late complications of SBS? 2. fat-soluble vitamins (A,D,E,K) 3. vitamin B12 4. folate (if >60 cm of terminal ileum resected) (if proximal jejunum resected) 1. Mg, Ca, Zn, Se Micronutrients Recommended dose /day Vitamin A 10.000-50.000 units Vitamin B12 300 μg/month Vitamin C 200-500mg Vitamin D 1600 U Vitamin E 30 IU Vitamin K 10 mg/week Calcium 800-1200 mg Magnesium As needed Iron Selenium 60-100 μg Zinc 220-440 mg Bicarbonate Bible class 31.10.2012

The most common metabolic complications of small-bowel syndrome? Management of gastric polyps The most common metabolic complications of small-bowel syndrome? Metabolic acidosis Hyperoxaliuria (nephrolithiasis, chronic renal failure) Hyperammoniaemia Metabolic bone disease (osteoporosis, osteomalacia) gram-positive colonic bacteria ferment carbohydrate to D-lactic acid Acidic environment short-chain fatty acids proliferation of the flora Metabolic acidosis (encephalopathy, headaches, ataxia, dysarthria) Bible class 31.10.2012

3 key points of management? Management of gastric polyps 3 key points of management? Nutrition / Supplementation of micronutrients Maintaining fluid, electrolytes and acid/base balance Avoid complications Calories to be supplied: Proteins to be supplied: 1.0-1.5 g/kg/day 25-30 kcal/kg/day 40-50% carbohydrates 20-30% proteins 20-40% lipids Continous PN Cyclic/discontinous PN Continous enteral Bolus enteral Intravenous fluid Oral rehidration fluid (glucose-polymer based with sodium) ORS ± oral electrolyte suppl. Bible class 31.10.2012

Pharmacologic adjuncts in the management? Management of gastric polyps Pharmacologic adjuncts in the management? 1. glucagon-like peptide-2 (teglutide): promotion of adaption 2. loperamid, diphenoxylate, codein: anti-motility agents 3. octreotid: increasing the small bowel transit time (but also inhibits pancreatic secretions) only if > 3 L of iv. fluid intake is required 4. cholestyramine: binding bile salts in steatorrhea secondary to bile acid malabsorption Cheng TT et al :Clinical and Experimental Gastroenterology 2011:4 189–196 Bible class 31.10.2012

Two categories of surgical treatment options? Management of gastric polyps Two categories of surgical treatment options? 1. non- transplant 2. transplant Aims of non-transplant surgical treatments? Preserve intestinal remnant minimize resrection, restore intestinal continuity, recruit additional intestine 2. Slow intestinal transit (segmental reversal of intestine, colonic interposition) 3. Increase intestinal surface (LILT = longitudinal intestinal lengthening and tailoring with longitudinal devision of intestine and blood supply at the mesenteric border) STEP = serial transverse enteroplasty) Bible class 31.10.2012

Surgical treatment of short bowel syndrome Management of gastric polyps Surgical treatment of short bowel syndrome Indications for intestinal transplants? Impending or overt liver failure (ESLD) Thrombosis of major central venous channels Frequent central line-related sepsis (>2 episodes/year) Frequent severe dehydration Diffuse mesenteric venous thrombosis with complications 3-year survival rate is better in combined transplantation Bible class 31.10.2012

Intestinal transplantation for SBS Management of gastric polyps Intestinal transplantation for SBS Which effect has the combined transplant on the rejection rate of intestinal transplants? Acute and chronic rejection rate is lower in combined transplants (liver+intestine, multivisceral) Major complications and cause of death after intestinal transplantation? Sepsis, MOF, rejection 3-year survival rate is better in combined transplantation Bible class 31.10.2012