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  1. 1. Short Bowel Syndrome
  2. 2. Objectives <ul><li>Definition </li></ul><ul><li>Etiology </li></ul><ul><li>Clinical manifestation </li></ul><ul><li>Management </li></ul><ul><li>prognosis </li></ul><ul><li>Complication </li></ul>
  3. 3. Definition <ul><li>It is a malabsorpative state that may follow massive resection of small intestine . </li></ul><ul><li>There is no specific intestinal length at which SBS well clinically present. </li></ul><ul><li>The small intestine of the neonate is about 250 cm in length, 750 cm in adult. </li></ul><ul><li>Infants have more favorable long term prognosis . </li></ul>
  4. 4. <ul><li>Factors that influence the length of time until child independent of TPN </li></ul><ul><ul><li>Extent/ location of resection. </li></ul></ul><ul><ul><li>Presence or absence of colon </li></ul></ul><ul><ul><li>Presence /Absence of ICV. </li></ul></ul><ul><ul><li>Degree of adaptation in remaining bowel. </li></ul></ul><ul><ul><li>Extent of residual bowel disease or complications e.g. adhesions, strictures </li></ul></ul>
  5. 5. Etiology <ul><li>Congenital anomalies : </li></ul><ul><ul><li>Intestinal Atresia . </li></ul></ul><ul><ul><li>Gastroschisis </li></ul></ul><ul><ul><li>omphalocele </li></ul></ul><ul><ul><li>Hirschsprung’s disease , </li></ul></ul><ul><li>Acquired : </li></ul><ul><ul><li>Resection of bowel: </li></ul></ul><ul><ul><li>NEC , </li></ul></ul><ul><ul><li>Crohn’s disease </li></ul></ul><ul><ul><li>volulus, </li></ul></ul><ul><ul><li>tumor , </li></ul></ul><ul><ul><li>radiation enteritis, </li></ul></ul><ul><ul><li>ischemic injury </li></ul></ul>
  6. 6. Manifestation <ul><li>Fluid & electlytes imbalance. </li></ul><ul><li>Steatorrhea </li></ul><ul><li>Wt loss and malnutrition. </li></ul><ul><li>Minerals def: Ca, Mg, Iron, zinc, B12, fat soluble vit. </li></ul><ul><li>Malabsorption of CHO and protein. </li></ul><ul><li>Metabolic acidosis. </li></ul><ul><li>Gastric acid hypersecretion. </li></ul><ul><li>Cholelithiasis. </li></ul><ul><li>Liver disease, cholestasis. </li></ul><ul><li>Bone disease. </li></ul><ul><li>Complications related to TPN. </li></ul><ul><li>Reduction of functioning bowel mass to below min necessary to balance supply & demand of essential body needs leading to intestinal failure manifested as: </li></ul>
  7. 7. Manifestation related to site of resection <ul><li>Duodenal resection </li></ul><ul><li>Jejunal resection </li></ul><ul><li>Ileal resection </li></ul><ul><li>Loss of the ileocecal valve </li></ul><ul><li>Colon </li></ul>
  8. 8. Duodenal resection <ul><li>Protein , CHO, fat maldigestion </li></ul><ul><li>Ca, mg, iron, folate malabsorption </li></ul><ul><li>Fat soluble vit deficiency </li></ul>
  9. 9. Jejunal resection <ul><li>CHO Malabsorption. </li></ul><ul><li>Water soluble vit defiency . </li></ul><ul><li>Malabsorption is transient (ileal adaptation). </li></ul>
  10. 10. Ileal resection <ul><li>Steatorrhea as bile salts not absorbed. </li></ul><ul><li>Cholesterol stones secondary to loss of bile acids. </li></ul><ul><li>Fat soluble vit def. </li></ul><ul><li>B12 def </li></ul><ul><li>Loss of ileal brake, decrease transit time causing diarrhea. </li></ul>
  11. 11. Loss of the ileocecal valve <ul><li>Bacterial overgrowth: allows bacteria to flux into ilium </li></ul><ul><li>Rapid transit time that exacerbate malabsorption. </li></ul>
  12. 12. Colon <ul><li>Role of The Colon: </li></ul><ul><li>water absorption. </li></ul><ul><li>It gives additional length, it slows transit time and slows gasteric empting, </li></ul><ul><li>But </li></ul><ul><ul><li>Deconjugation of bile acids by colonic bacteria & secondary secretory diarrhea. </li></ul></ul><ul><ul><li>lactic acidosis : conversion of CHO by lactobacillus to D-lactic acid lead to high AG metabolic acidosis, </li></ul></ul>
  13. 13. Intestinal adaptation <ul><li>Starts 24-48 hrs post-op, ( enteral feeds as early as possible). </li></ul><ul><li>Lasts up to 11-12 years . </li></ul><ul><li>Change in morphorogy and functional capacity. </li></ul>
  14. 14. Change in morphorogy <ul><li>Macroscopic </li></ul><ul><ul><li>Increase in length </li></ul></ul><ul><li>Microscopic </li></ul><ul><ul><li>Villus: increase height and diameter </li></ul></ul><ul><ul><li>Crypt: elongation </li></ul></ul><ul><ul><li>Epithelial cell life cycle: increase proliferation </li></ul></ul><ul><ul><li>decrease apoptosis. </li></ul></ul>
  15. 15. Change in functional capacity <ul><li>Increase absorption per unit length </li></ul><ul><li>Upregulation of sodium glucose transporter. </li></ul>
  16. 16. Lab investigation <ul><li>Blood </li></ul><ul><ul><li>U&E, bone profile, & mg, PRN then biweekly </li></ul></ul><ul><ul><li>CBC, triglycerides, cholesterol Weekly </li></ul></ul><ul><ul><li>Folate, vit B12, copper, zinc, Monthly </li></ul></ul><ul><ul><li>Blood gas and AG for suspected lactic acidosis. </li></ul></ul>
  17. 17. Microbiology <ul><li>If sepsis suspected; blood & urine c/s </li></ul><ul><li>Cultures from both the central and peripheral sites. </li></ul><ul><li>Consider opportunistic infections, so search for fungal infection . </li></ul>
  18. 18. Imaging Studies <ul><li>To assess for potential complications, </li></ul><ul><li>Infection </li></ul><ul><ul><li>Abdominal ultrasonography to look for fungal balls in the kidney </li></ul></ul><ul><li>Bowel obstruction </li></ul><ul><ul><li>Plain radiography. </li></ul></ul><ul><ul><li>Barium imaging of the bowel </li></ul></ul><ul><li>Liver disease </li></ul><ul><ul><li>Abdominal US to study the liver, biliary tract, & presence of ascites . </li></ul></ul>
  19. 19. Management <ul><li>During early period after intestinal resection, TPN to prevent fluid and electrolytes imblance. </li></ul><ul><li>Stomal & fecal losses replaced q 2 hrs with solution separate from TPN. </li></ul><ul><li>May develop gastric hypersecretion so give H2 blocker </li></ul>
  20. 20. Management <ul><li>The goals of nutritional therapy </li></ul><ul><ul><li>1.Maintain adequate nutrition </li></ul></ul><ul><ul><li>2.Promote intestinal adaptation </li></ul></ul><ul><ul><li>3.Avoid complications </li></ul></ul>
  21. 21. <ul><li>TPN for the first 7-10 days </li></ul><ul><li>TPN :30 kcal/kg/day </li></ul><ul><li>Enteral feeding when hemodynamic stable and fluid management stable. </li></ul><ul><li>Continuous enteral feeds: to prevent osmotic diarrhea. </li></ul><ul><li>Bolus feeds less well tolerated. </li></ul><ul><li>Formula osmolality should be < 310 mosm/kg. </li></ul>
  22. 22. Composition <ul><li>Protien hydrolysate or elemental diets </li></ul><ul><li>Complex carbohydrate is better than simple carbohydrate </li></ul><ul><li>Oxalate restriction in patient with an intact colon and fat malabsorption to avoid stone formation. </li></ul>
  23. 23. <ul><li>Lipid </li></ul><ul><li>Medium-chain triglycerides </li></ul><ul><ul><li>Better absorbed in the presence of bile acid or pancreatic insufficiency. </li></ul></ul><ul><li>Long-chain triglycerides : more effective in stimulating intestinal adaptation </li></ul><ul><li>Mix MCT + LCT </li></ul>
  24. 24. <ul><li>Indications for continued parental nutrition </li></ul><ul><ul><li>Poor weight gain or loss of maintenance weight. </li></ul></ul><ul><ul><li>Extensive stomal fluid and electrolyte losses which cannot be replaced orally . </li></ul></ul>
  25. 25. Pharmacologic therapy <ul><li>Decrease stomal secretory losses </li></ul><ul><ul><li>H2 blockers, PPI & octreotide </li></ul></ul><ul><ul><li>??loperamide </li></ul></ul><ul><li>Ursodeoxycholic acid: Improves bile acid–dependent bile flow. </li></ul><ul><li>Antibiotics used to prevent small-bowel overgrowth. </li></ul><ul><li>Insufficient data regarding -glutamine affects clinical outcomes in infants. </li></ul><ul><li>GH in children with SBS may have some benefit in those with low or limited GH responsiveness. </li></ul>
  26. 26. Surgical Care <ul><li>Surgical care is related to venous access (ie, central line placement to provide TPN). </li></ul><ul><li>Gastrostomy tube placement to provide for enteral access. </li></ul>
  27. 27. Nontransplantation procedures <ul><li>To improve the surface area or to slow transit emptying time. </li></ul><ul><li>Bianchi procedure (intestinal tapering or lengthening) </li></ul><ul><li>Indicated in small bowel with bacterial overgrowth ,dilated bowel and continued malabsorption </li></ul><ul><li>Cutting bowel longitudinally, and create a segment of bowel twice length, half diameter without loss of mucosal surface area. </li></ul>
  28. 28. Tapering Bowel lengthening
  29. 29. <ul><li>Indications </li></ul><ul><ul><li>Impending or overt liver failure </li></ul></ul><ul><ul><li>IV access loss </li></ul></ul><ul><ul><li>Frequent central line related sepsis </li></ul></ul><ul><ul><li>Intestinal failure </li></ul></ul>Small bowel transplantation
  30. 30. Prognosis <ul><li>Ultimately patient with SBS may be successfully wean from TPN although the entire process may take several years. </li></ul><ul><li>Intestinal transplantation should be consider as a last resort. </li></ul>
  31. 31. Complications <ul><li>Early complications </li></ul><ul><ul><li>Catheter related complication </li></ul></ul><ul><li>chronic complications </li></ul><ul><ul><li>liver & biliary disease </li></ul></ul><ul><ul><li>Bacterial overgrowth </li></ul></ul><ul><ul><li>D-lactic acidosis, </li></ul></ul><ul><ul><li>Nutritional def, </li></ul></ul>
  32. 32. Bacterial overgrowth <ul><li>Defined as increased bacterial content in the small intestine </li></ul><ul><li>Occurs if no Ileum , dilated bowel loops with hypomotility segment & strictures. </li></ul><ul><li>Clinically: N, V, distension, FTT, increase hepatic injury from TPN , GI blood loss </li></ul><ul><li>Also common cause of clinical deterioration in a previously stable patient with SBS. </li></ul><ul><li>Diagnosed duodenal fluid analysis, culture, stool c/s, H2 breath test . </li></ul>
  33. 33. <ul><li>This well leads to CHO malabsorption, worsening of osmotic diarrhea, and increased risk of metabolic acidosis and dehydration. </li></ul><ul><li>Treatment with antibiotic , including administration of metronidazole alternating with oral gentamicin. </li></ul><ul><li>Should be cycled on a weekly or biweekly basis. </li></ul>
  34. 34. Conclusion <ul><li>Early management of SBS replacement of fluid and electrolytes. </li></ul><ul><li>Enteral feeding should begin once the patient stabilizes. </li></ul><ul><li>Continuous enteral feeding or is preferred. </li></ul><ul><li>For enteral feedings, hypoallergenic protein hydrolysate formulas or breast milk are usually best tolerated </li></ul><ul><li>Several pharmacological approaches have been tested to enhance intestinal adaptation and improve feeding tolerance. None of these approaches are proven to be helpful, but studies are ongoing. </li></ul>
  35. 35. References <ul><li>1- . Management of the short bowel syndrome in children , September 2009 </li></ul><ul><li>2- www.emdicine .com , Short Bowel Syndrome, Carmen .C. Apr 2009 </li></ul><ul><li>3- Dr. Siham </li></ul>
  36. 36. <ul><li>Thanks </li></ul>