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

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Short bowel syndrome Dr. Henrik Csaba Horvath

Bible class February 20, 2013

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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

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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

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Major causes of SBS in adults?

Postoperative complications Irradiaton/cancer Mesenteric valvular disease (mesenteric ischemia) Crohn`s disease Trauma

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Which factors are associated with worse prognosis in patients with SBS?

Total parenteral nutrition < 50 cm intact bowel Enterostomy Radiation or ischemic enteritis

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Which factors have impact on the outcome of SBS?

Presence/abscence of ileocaecal valve

Outcome of SBS

Length of the remaining intestine

Segment of intact bowel (jejunum vs. ileum, colon continuity)

Absorptive quality of the remnant bowel

Presence of residual underlying disease

(e.g.Crohn`s)

Age/BMI of the patient

State of other digestive organs

Efficacy of nutrition support

Pharmacologic therapy

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How does affect the remnant length the risk of developing SBS?

Anastomosis PLUS length of residual small intestine

Duodenostomy or jejunoileal < 35 cm

Jejunocolic or ileocolic < 60 cm

End jejunostomy < 115 cm

Patients at highest risk of developing SBS are

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Which impact has on absorption the resection of…

Loss of the major intestinal absorption area Loss of digestive enzymes Loss of GI feedback hormones (gastric emptying)

Jejunum:

Ileum:

Loss of the absorption of jejunal secretion Loss of absorption of vitamin B12

Loss of absorption of bile salts (fat malabsorption)

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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

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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

Up to 70% can do without TPN due to these mechanisms

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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

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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)

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Two major groups of complications of SBS?

dehydration electrolyte derangements (Mg, Ca, K)

Diagnosis: urinary electrolyte levels (plasma can be normal!) Treatment: sustained correction due to slow cellular uptake

TPN-related bacterial overgrowth micronutrient deficiency metabolic

Early complications:

Late complications:

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TPN-related late complications of SBS are…

gallstones, cirrhosis (IFALD)

end-stage liver cirrhosis in 15% of pts after one year TPN 100% mortality rate within 2 yrs

infection: one-third of deaths in 50% 5-yr-mortality rate in SBS thrombosis (v. cava superior): 0.2/1000 catheter days

1. Due to bypass first pass liver metabolism:

2. Catheter-associated complications:

steatosis cholestasis

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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

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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 As needed

Selenium 60-100 µg

Zinc 220-440 mg

Bicarbonate As needed

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The most common metabolic complications of small-bowel syndrome?

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)

1.  Metabolic acidosis 2.  Hyperoxaliuria (nephrolithiasis, chronic renal failure) 3.  Hyperammoniaemia 4.  Metabolic bone disease (osteoporosis, osteomalacia)

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3 key points of management? 1.  Nutrition / Supplementation of micronutrients 2.  Maintaining fluid, electrolytes and acid/base balance 3.  Avoid complications

Calories to be supplied:

25-30 kcal/kg/day

Proteins to be supplied: 1.0-1.5 g/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.

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Pharmacologic adjuncts in the management?

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

1. glucagon-like peptide-2 (teglutide): promotion of adaption

Cheng TT et al :Clinical and Experimental Gastroenterology 2011:4 189–196

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Two categories of surgical treatment options?

2. transplant 1. non- transplant

1.  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)

Aims of non-transplant surgical treatments?

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Surgical treatment of short bowel syndrome

Indications for intestinal transplants?

1.  Impending or overt liver failure (ESLD) 2.  Thrombosis of major central venous channels 3.  Frequent central line-related sepsis (>2 episodes/year) 4.  Frequent severe dehydration 5.  Diffuse mesenteric venous thrombosis with complications

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Intestinal transplantation for SBS

Which effect has the combined transplant on the rejection rate of intestinal transplants?

Major complications and cause of death after intestinal transplantation?

Acute and chronic rejection rate is lower in combined transplants (liver+intestine, multivisceral)

Sepsis, MOF, rejection