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Short bowel syndrome. Dr. Henrik Csaba Horvath. Bible class February 20, 2013. Definition of short-bowel syndrome. Loss of intestinal absorption from surgical resection , congenital defect or diseases characterized by the inability to maintain
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Short bowelsyndrome Dr. Henrik Csaba Horvath BibleclassFebruary 20, 2013
Definition ofshort-bowelsyndrome Loss of intestinal absorptionfromsurgicalresection, congenitaldefectordiseasescharacterizedbytheinabilitytomaintain protein-energy, fluid, electrolyte, ormicronutrientbalanceswhen on a conventionallyaccepted, normal diet
Whichconditionscanleadto a short-bowelsyndrome? c Physicallossof portionsofintestine Loss offunction Surgical resection (volvulus) Obstruction Disease-associated lossofabsorption (Crohn`s, postirradiation) Loss ofbowel orenterocyte mass (trauma, infarction) Dysmotility Congenital defect SBS-associated intestinal failure
Major causesof SBS in adults? Postoperative complications Irradiaton/cancer Mesentericvalvulardisease (mesentericischemia) Crohn`sdisease Trauma
Whichfactorsareassociatedwithworseprognosis in patientswithSBS? Total parenteral nutrition < 50 cm intactbowel Enterostomy Radiation orischemicenteritis
Whichfactorshaveimpact on theoutcomeof SBS? Presence/abscenceofileocaecalvalve • Lengthofthe • remainingintestine • Segment ofintactbowel • (jejunum vs. ileum, coloncontinuity) • Absorptive qualityof • theremnantbowel Outcomeof SBS • Presence of residual • underlyingdisease • (e.g.Crohn`s) • State ofother digestive organs • Efficacyof • nutritionsupport • Pharmacologictherapy • Age/BMI of • thepatient
Howdoesaffecttheremnantlengththeriskofdeveloping SBS? Patientsathighestriskofdeveloping SBS are
Whichimpacthas on absorptiontheresectionof… Loss ofthemajor intestinal absorptionarea Loss of digestive enzymes Loss of GI feedbackhormones (gastricemptying) Jejunum: Ileum: Loss oftheabsorptionofjejunalsecretion Loss ofabsorptionofvitamin B12 Loss ofabsorptionofbilesalts (fatmalabsorption)
Whichimpacthasthelossofileocaecalvalve? Dilatation ofthesmallintestine Slowermotility Bacterialovergrowthofthe small intestine Competitionfornutrients, inflammation, GI bleeding, bacterialtranslocation± endotoxaemia, liverinjury, D-lacticacidosis
Which adaptive mechanismsoccur in the residual intestine/ GI tract? Mucosalhyperplasia (due tofatstimulatedglucagon-likepeptidereceptor II) Increasedmucosalbloodflow Improved segmental absorption Gastrichypersecretion Increasedpancreatobiliarysecretions • Upto 70% can do without TPN due tothesemechanisms
Importanceofcolonin compensationforthe lack ofintestine? Increasereabsorptionofwater, electrolytes, short-chainfattyacidsand GI secretions Slow down the intestinal transitandstimulate intestinal adaptationby hormonal regulation Fermentation ofmalabsorbedcarbohydratesbycolonicbacteria
Measurement ofthefunctionalcapacityofthesmallintestine? 1. 48-hour nutritional balancetest analysisofdailyabsorption rate (intake-output) predictionof intestinal failure: <1,4 kg wetweight/day 1170 kcal/dayofenergy (Difficultieswithduplicatefoodportionsandaccuratestoolcollections) 2. Fastingplasmacitrullineconcentration (>5 μmol/L) (issynthetizedbythesmallintestine, bestpracticalmeasureofenterocytefunction)
Twomajorgroupsofcomplicationsof SBS? dehydration electrolytederangements (Mg, Ca, K) • Early complications: Diagnosis: • urinaryelectrolytelevels (plasmacanbe normal!) Treatment: • sustainedcorrection due toslowcellularuptake • Latecomplications: TPN-related bacterialovergrowth micronutrientdeficiency metabolic
TPN-relatedlatecomplicationsof SBS are… gallstones, cirrhosis(IFALD) 1. Due tobypassfirst pass livermetabolism: steatosis cholestasis end-stagelivercirrhosis in 15% ofpts after oneyear TPN 100% mortality rate within 2 yrs • 2. Catheter-associatedcomplications: infection: one-thirdofdeaths in 50% 5-yr-mortality rate in SBSthrombosis (v. cavasuperior): 0.2/1000 catheterdays
Bacterialovergrowth-relatedlatecomplicationsof SBS are… 1. carbohydratemalabsorption 2. sepsis due tobacteriallocalisation 3. decreasedabsorptionoffattyacids due tointerferencewithchilomicronformation 4. lossof absorptive capacity due toinflammatoryresponse
Whicharethemostcommonmicronutritientdeficienciesaslatecomplicationsof SBS? 1. Mg, Ca, Zn, Se 2. fat-soluble vitamins (A,D,E,K) 3.vitamin B12 (if >60 cm of terminalileumresected) 4. folate (if proximal jejunumresected)
The mostcommonmetaboliccomplicationsofsmall-bowelsyndrome? Metabolicacidosis Hyperoxaliuria (nephrolithiasis, chronicrenalfailure) Hyperammoniaemia Metabolicbonedisease (osteoporosis, osteomalacia) gram-positive colonicbacteria fermentcarbohydrateto D-lacticacid proliferationoftheflora Acidicenvironment short-chainfattyacids Metabolicacidosis(encephalopathy, headaches, ataxia, dysarthria)
3 keypointsofmanagement? • Nutrition / Supplementation ofmicronutrients • Maintaining fluid, electrolytesandacid/basebalance • Avoidcomplications Caloriestobesupplied: Proteins tobesupplied: 1.0-1.5 g/kg/day 25-30 kcal/kg/day 40-50% carbohydrates 20-30% proteins 20-40% lipids Bolus enteral Continous enteral Cyclic/discontinous PN Oral rehidrationfluid (glucose-polymer basedwithsodium) ORS ± oral electrolytesuppl. Intravenous fluid Continous PN
Pharmacologicadjuncts in themanagement? 1. glucagon-like peptide-2 (teglutide): promotionofadaption 2. loperamid, diphenoxylate, codein: anti-motilityagents • 3.octreotid: increasingthesmallboweltransittime (but also inhibitspancreaticsecretions) onlyif > 3 L ofiv. fluid intakeisrequired • 4. cholestyramine: bindingbilesaltsin steatorrheasecondarytobileacidmalabsorption Cheng TT et al :Clinical and Experimental Gastroenterology 2011:4 189–196
Twocategoriesofsurgicaltreatmentoptions? 2. transplant 1. non- transplant Aimsof non-transplant surgicaltreatments? Preserve intestinal remnant minimizeresrection, restore intestinal continuity, recruit additional intestine 2. Slow intestinal transit (segmental reversalofintestine, colonicinterposition) 3. Increase intestinal surface (LILT = longitudinal intestinal lengtheningandtailoring withlongitudinal devisionofintestineandbloodsupplyatthemesentericborder) STEP = serialtransverseenteroplasty)
Surgicaltreatmentofshortbowelsyndrome Indicationsfor intestinal transplants? Impendingorovertliverfailure (ESLD) Thrombosisofmajorcentralvenouschannels Frequentcentralline-relatedsepsis (>2 episodes/year) Frequentseveredehydration Diffuse mesentericvenousthrombosiswithcomplications
Intestinal transplantationfor SBS Whicheffecthasthecombined transplant on therejection rate of intestinal transplants? Acuteandchronicrejection rate islower in combinedtransplants (liver+intestine, multivisceral) Major complicationsandcauseofdeath after intestinal transplantation? Sepsis, MOF, rejection