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Short Bowel Syndrome

Short Bowel Syndrome. Ricardo A. Caicedo, M.D. Pediatric Gastroenterology. Definitions. Short bowel syndrome (SBS) Functional impairment resulting from critical reduction in intestinal length Includes malnutrition, chronic diarrhea, malabsorption, growth failure The most common cause of…

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Short Bowel Syndrome

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  1. Short Bowel Syndrome Ricardo A. Caicedo, M.D. Pediatric Gastroenterology

  2. Definitions • Short bowel syndrome (SBS) • Functional impairment resulting from critical reduction in intestinal length • Includes malnutrition, chronic diarrhea, malabsorption, growth failure • The most common cause of… • Intestinal failure • Severely compromised intestinal function of any etiology (irrespective of bowel length) • Total parenteral nutrition (TPN)-dependent

  3. Etiology: Neonates • - Gastroschisis • - Omphalocele • - SB atresias • Volvulus • Malrotation • Other • Thrombosis • Aganglionosis About 20 % of infants with NEC requiring resection develop SBS

  4. Etiology: Children/Adolescents • Volvulus • Trauma • Intra-abdominal cancer • Vascular anomalies • Radiation enteropathy Overall Incidence 1:500,000

  5. Prognostic Factors • SB length • Normal FT infant: 200-250 cm • Loss of >70% (residual SB length < 70 cm in infants) portends SBS • Absorptive function of residual SB • Proportion of daily calories tolerated enterally • Intestinal adaptation • Compensatory events stimulated by massive bowel resection • Anatomy of resection • Loss of segment-specific functions • Loss of ileocecal valve

  6. Anatomic Considerations • Loss of segment-specific functions • Jejunum: primary site of digestion and absorption • Ileum: B12 and bile acid absorption • Colon: water absorption and adjunctive carbohydrate absorption • Ileo-cecal valve • Regulation of intestinal transit • Prevention of bacterial reflux into SB

  7. Management: Postoperative Period • Maintenance of fluid/electrolyte balance • Maintenance TPN • Separate IVF for ostomy output replacement • H2-blocker to tx gastric hypersecretion • Await resolution of postoperative ileus

  8. Enteral Feeding • Initiated when postop. Ileus resolved, UGI tract decompressed, and F/E/N status stable • Advancement of EN and reduction/elimination of PN is the goal • Promotes intestinal adaptation • Follow hydration status, stool output, body growth, labs (albumin, prealbumin, Hb, BUN) • Continuous tube feeds: decrease SB osmotic load and absorptive workload per unit of time

  9. Enteral Feeding Composition • Protein • Breast milk: promotes adaptation • Hydrolysate (semi-elemental): peptides more easily assimilated in short gut • Amino acid (elemental): prevention of milk protein hypersensitivity • Higher osmolarity • Fats • MCT do not required bile acids/micelle formation for absorption • Excess of MCT can lead to EFA deficiency • Carbohydrate • CHO malabsorption is the rate-limiting factor in advancing EN • Glucose polymers digested more efficiently than lactose • Start with dilute formula • increasing in volume before increasing energy density OR • Increasing in concentration before increasing rate • Monitor stool output, pH, reducing substances

  10. Advancement of EN • Supplements • Vitamins ADEK, B12 • Electrolytes: Na/K citrate, Ca, Mg • Elements: Fe, Zn • Cycle/window and wean TPN • Increase EN at avg. rate of 0.5-1.0 cal/kg q 1-3 days • Non-nutritive sucking and PO feeding to prevent oral feeding aversion • Intolerance of EN but stable: home TPN

  11. Predicting Ability to Wean TPN • Length of residual SB • Percent daily enteral intake • Earlier restoration of intestinal continuity • Fewer complications • Biomarkers: experimental stage • Urine 3-0-methylglucose after PO feeding (rat model) • Plasma citrulline levels (adult SBS pts) • < 20 umol/L predict permanent intestinal failure

  12. Complications • TPN-related • Cholestasis/hepatopathy • Calcium-bilirubinate gallstone disease • CVL-related • Infection- bacterial or fungal sepsis • Thrombus/thromboembolism • SB bacterial overgrowth • Nutrient deficiencies • Enterocolitis • Mechanical or pseudo-obstruction (dysmotility) • Pancreatitis • Nephrolithiasis (oxalate stones)

  13. Prevention of TPN Liver Disease • Reduce TPN/advance EN • Cycle TPN (run < 18 hrs/d) • Monitor TPN composition • Glucose infusion rate < 8-12 mg/kg/min • Keep AA at 2.5 g/kg/d • Keep lipids at < 40% of total caloric intake • Reduce trace elements (Mn, Cu) to 25% RDA once cholestasis develops • Manage CVL infection and SBBO • Start UDCA at 15-30 mg/kg/d

  14. SB Bacterial Overgrowth • Factors • Dysmotility/poor peristalsis leading to stasis of enteral contents • Loss of ICV • Symptoms • Feeding intolerance, abd. distention, weight loss, blood in stools • D-lactic acidemia: lethargy, ataxia, WAGMA • Diagnosis • Duodenal fluid aspirate and culture (> 105 col/ml) • Breath hydrogen test • Often made on clinical grounds • Treatment • Empiric antibiotics (metronidazole, TMP-SMX, PO gentamicin, amox-clavulonate, rifaximin) • Adjunctive probiotics: little evidence, and a safety concern in SBS pts esp. those with CVL

  15. Medical Therapies • Anti-motility agents • Loperamide, diphenoxylate/atropine • Slow transit time • Improve fluid absorption and reduce fecal fluid loss • Bile acid binder (cholestyramine) • Prevents bile acid malabsorption and bile acid-induced secretory diarrhea • Octreotide: reduces output from proximal enterostomies • Glutamine/GH • Mostly theoretical/animal or transient benefit • GLP-2: may stimulate mucosal growth and reduce secretions; still experimental

  16. Surgical Interventions • Longitudinal tapering • Bianchi lengthening operation • Doubles length, reduces diameter by 0.5 • preserves effective surface area

  17. Serial Transverse Enteroplasty (STEP) • Creates a maze-like tunnel within dilated segment • Kim HB et al, J. Pediatr Surg. 2003; 38:881-5.

  18. SB Transplantation • Indications (Am. Soc. Transplantation, 2001) • Progressive/irreversible TPN liver disease (~ 4% of infants w/SBS) • Cholestasis beyond 3-4 mos of age • Features of PORTAL HTN • Impaired synthetic function (albumin, INR) • Recurrent sepsis • Threatened loss of central venous access • Contraindications • Absolute: AIDS, overwhelming sepsis • Relative: weight < 5 kg, multiple previous abdominal operations

  19. Survival • Overall: 80-94% • Mortality higher in patients with NEC • Very long term (up to 25 y) TPN: 94% survival • Transplantation • Mortality is center-specific • Largest experience: U. Pittsburgh (Reyes J, Semin Pediatr Surg 2001) • 1-yr survival rate: 70% • 3-yr, all ages combined: 55% • Mortality greater in • Patients under 2 y of age • Combined SB-liver tx • Higher risk of PTLD in SB transplantation

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