1. SHORT BOWEL SYNDROME SHORT BOWEL SYNDROME Abdulwahab Telmesani Abdulwahab Telmesani Associate Professor Of Pediatrics Associate Professor Of Pediatrics Umm Al-Qura University Umm Al-Qura University

2. Definition Malabsorption Malabsorption + + Shortened Bowel Shortened Bowel

3. Etiology Primary: (Abnormal anatomically) Primary: (Abnormal anatomically) Born with short bowel Congenital anomalies e.g. Multiple Artesia's, Gastroschisis Secondary: Secondary:NEC Hirschsprung disease IschemiaRadiationTumors Crohns’s Crohns’s

5. Jejunum Long villi → Large absorptive surface area Long villi → Large absorptive surface area High concentration of enzymes and High concentration of enzymes and transport carrier transport carrier Large tight junction → Porous to large Large tight junction → Porous to large molecule molecule

6. Ileum Short villi → Less absorptive capacity Short villi → Less absorptive capacity Small tight junction → Less porous and Small tight junction → Less porous and increased absorption for fluid & electrolyte increased absorption for fluid & electrolyte Specific function of absorption of B12 and Specific function of absorption of B12 and bile salts (specific receptors) bile salts (specific receptors) Synthesis of hormones e.g.Enteroglucagon & Synthesis of hormones e.g.Enteroglucagon & negative gastrin feedback negative gastrin feedback

7. Ileocecal valve Stops reflux of bacteria Stops reflux of bacteria Regulate fluid and nutrient exit Regulate fluid and nutrient exit

8. Adaptation Ileum > Jejunum Ileum > Jejunum

9. Adaptation Hyperplasia, Increased crypts and villi Hyperplasia, Increased crypts and villi Dilatation Dilatation Increased Absorption capacity Increased Absorption capacity

10. Adaptive factors Entral feed: Entral feed: Direct stimulation Upper GI secretion Upper GI secretion Trophic GI hormone Trophic GI hormone Hormonal regulation: Hormonal regulation: Enteroglucagon, Neurotensin, Secretin Cholicystokinin,Epidermal growth factors(EGF) IGF-I + GH

11. Adaptive factors(Contd.) Prostaglandin Prostaglandin Polyamines Polyamines Intracellular regulation (Genetic) Intracellular regulation (Genetic)

12. Nutrients stimulate adaptation better Nutrients stimulate adaptation better Long chain fatty acid Long chain fatty acid 3-Omega fatty acids (fish oil) 3-Omega fatty acids (fish oil) Fibers Fibers Glutamine Glutamine

13. Management

15. Management TPN TPN Replace losses (Electrolyte & fluid) Replace losses (Electrolyte & fluid) Introduction of Enteral feed Introduction of Enteral feed

16. TPN Gradual introduction with monitoring of blood chemistry,LFT &lipids

17. Replace losses Losses from : Losses from : NG, gastrostomy, diarrhea, Ostomy Appropriate fluid (based on lost electrolyte) Appropriate fluid (based on lost electrolyte) Replace losses through separate infusion pump Replace losses through separate infusion pump

18. Enteral feeding Continuous enteral infusion Continuous enteral infusion (can use portable) Breast Milk/Predigested formulas as a start Breast Milk/Predigested formulas as a start Progress to solids Progress to solids Wean TPN as enteral feeding increases Wean TPN as enteral feeding increases

19. Guide to advancing enteral feeding Diarrhea not getting worse Diarrhea not getting worse > 50% or 40ml/kg/day Stool reducing substances (pH < 5.5) Stool reducing substances (pH < 5.5)

20. Complications Bacterial overgrowth Bacterial overgrowth Diarrhea Diarrhea Nutritional deficiency Nutritional deficiency TPN related liver disease TPN related liver disease Catheter related Catheter related

21. Bacterial overgrowth More that 10 5 bacterial growth in upper intestine (facultative bacteria & anaerobes) More that 10 5 bacterial growth in upper intestine (facultative bacteria & anaerobes) Causes: Causes: stagnation, dilatation, lost ileocaecal valve stagnation, dilatation, lost ileocaecal valve

22. Bacterial overgrowth cont. Manifestations: Manifestations: Deconjugation of bile → Malabsorption Deconjugation of bile → Malabsorption Lactose intolerance → Diarrhea, bloating, cramps Lactose intolerance → Diarrhea, bloating, cramps Lactic acidosis → CNS symptoms Lactic acidosis → CNS symptoms Inflammation → Ileitis, colitis Inflammation → Ileitis, colitis

23. Bacterial overgrowth(Contd.) Diagnosis: Aspiration of intestine for C&S Diagnosis: Aspiration of intestine for C&S Breath hydrogen test Breath hydrogen test Treatment: Treatment: Antibiotics: Commonly TMP-SMX + Metronidazole Frequent defecation Frequent defecationSurgical

24. Diarrhea Causes: Causes: Osmotic load Osmotic load Elevated gastrin Elevated gastrin Treatment: Treatment: Revise enteral feeds Cholestyramine H2 blockers

25. Nutritional deficiency Fat soluble vitamin A,D,E,K Fat soluble vitamin A,D,E,K Zinc (low serum alkaline phosphatase) Zinc (low serum alkaline phosphatase) Trace elements Trace elements B12 B12 Micronutrients e.g. carnitine, choline, taurine Micronutrients e.g. carnitine, choline, taurine

26. TPN liver disease Hepatocellular damage Hepatocellular damage Cholestasis,Cholelethiasis Cholestasis,Cholelethiasis Sepsis Sepsis

27. Catheter related complication Sepsis Sepsis Improper catheter care Improper catheter care bacterial overgrowth → bacteremia bacterial overgrowth → bacteremia Thrombosis Thrombosis

28. Surgical management Treat anastomosis strictures: Treat anastomosis strictures: Tapering enteroplasty, stricturoplasty Increased length: Increased length: Intestinal transection (Bianchi procedure)

29. Surgical management cont. Increased intestinal transit: Increased intestinal transit: -Colon interposition -Creation of valve Transplantation Transplantation

30. Transplantation  Getting better survival  Not yet a standard procedure  Problem with rejection  Post-immunosuppressant lymphoproliferative disorders

31. Transplantation cont. Indicated when You are against the wall Indicated when You are against the wall -Major liver disease secondary to TPN -Intolerance to feeding -Catheter sepsis, thrombosis and no site for insertion for insertion

32. SBS In the Neonates

34. Correlates of good outcome Use of breast milk Use of breast milk Use of Amino acid based formulas Use of Amino acid based formulas Percentage of enteral calories at 6 WKs Percentage of enteral calories at 6 WKs Residual small bowel length at Sx Residual small bowel length at Sx The year of Sx The year of Sx Androsky et al 2001 Androsky et al 2001

35. Correlates with low Peak Bilirubin Level Early Closure of Ostomy Early Closure of Ostomy Enteral Calories at 6 WKs Enteral Calories at 6 WKs Less Gm +ve Infections Less Gm +ve Infections Use of Casein hydrolysate formulas Use of Casein hydrolysate formulas Androsky et al 2001 Androsky et al 2001

36. Correlations Between: Early enteral feeding and the subsequent weaning of TPN Sondheimer et al 1998 Correlations Between: Early enteral feeding and the subsequent weaning of TPN Sondheimer et al 1998 Length of the small bowel at Sx and discontiuation of TPN

37. A correlation between amino acid based formula and weaning from TPN was observed Bines et al 1999 Bines et al 1999

38. Conclusions Early introduction of enteral feeding Early introduction of enteral feeding Use of BREAST MILK / Hydrolysate formulas Use of BREAST MILK / Hydrolysate formulas Early closure of the OSTOMIES Early closure of the OSTOMIES Use of improved types of TPN solutions Use of improved types of TPN solutions Stringent care of the TPN catheters Stringent care of the TPN catheters Watch and treat bacterial over growth Watch and treat bacterial over growth

39. DONE