1. Short Bowel Syndrome Anne Aspin 2010

2. Definition Rickham (1967) – an extensive resection to maximum of 75cm Rickham (1967) – an extensive resection to maximum of 75cm Kuffer (1972) – 15cm with ileocaecal valve Kuffer (1972) – 15cm with ileocaecal valve - 38cm without ileocaecal valve - 38cm without ileocaecal valve Dorney (1985) – 11cm with I/C valve or 25cm without I/C valve Dorney (1985) – 11cm with I/C valve or 25cm without I/C valve

3. Introduction Most common cause of intestinal failure. Most common cause of intestinal failure. NEC, Congenital atresia, Gastroschisis and volvulus. NEC, Congenital atresia, Gastroschisis and volvulus. Promote adaptive response through enteral feeding and careful management of TPN. Promote adaptive response through enteral feeding and careful management of TPN.

4. The Digestive System Digestion starts in the mouth Digestion starts in the mouth Moisten by saliva (contains Pytalin), begins to turn starch to sugar. Moisten by saliva (contains Pytalin), begins to turn starch to sugar. In stomach food churned mixes with gastric juices. In stomach food churned mixes with gastric juices.

5. Gastric juices Acid reaction Acid reaction Kills bacteria Kills bacteria Controls pylorus Controls pylorus

6. Gastric juices: Gastric juices: - Rennin coagulates milk - Hydrochloric Acid – Converts Pepsinogen to Pepsin. - Pepsin turns protein to peptone

7. Food is released in small amounts by relaxation of the sphincter passing onto Duodenum. Food is released in small amounts by relaxation of the sphincter passing onto Duodenum. Food further digested by Trypsin, Amylase and Lipase. Food further digested by Trypsin, Amylase and Lipase. Digestion completed in small intestine. Digestion completed in small intestine.

8. Intestinal juices. Enterokinase – pancreatic trypsinogen Enterokinase – pancreatic trypsinogen Peptidase – polypeptide to amino acid Peptidase – polypeptide to amino acid Maltase - maltose} Maltase - maltose} Sucrase – sucrose} to glucose Sucrase – sucrose} to glucose Lactase – Lactose} Lactase – Lactose} Lipase – Fats to fatty acids and glycerol Lipase – Fats to fatty acids and glycerol

9. Onto large intestine where fluids and nutrients are re absorbed. Onto large intestine where fluids and nutrients are re absorbed. Waste fluids taken by blood stream to kidneys to be filtered Waste fluids taken by blood stream to kidneys to be filtered

10. Small intestine Convoluted tube from pyloric sphincter to the junction of ileo – caecal valve Convoluted tube from pyloric sphincter to the junction of ileo – caecal valve Mucus membrane –has circular folds to increase surface area for absorption. Mucus membrane –has circular folds to increase surface area for absorption. Villi which contain blood and lymph vessel. Villi which contain blood and lymph vessel. Supplied with tubular glands secreting intestinal juice. Supplied with tubular glands secreting intestinal juice.

12. Absorption Proteins, Carbohydrates and Fats through villi in small intestine. Proteins, Carbohydrates and Fats through villi in small intestine. Fats in the form of fatty acids and glycerol are absorbed by cells covering villi. Pass into lymph within villi drained by lymphatic capillaries. Fats in the form of fatty acids and glycerol are absorbed by cells covering villi. Pass into lymph within villi drained by lymphatic capillaries.

13. Ileo Caecal valve. The Caecum lies in the right ileac fossa. The Caecum lies in the right ileac fossa. The Ileum opens into the Caecum through the Ileo-Caecal valve. The Ileum opens into the Caecum through the Ileo-Caecal valve. This is a sphincter which prevents the IC contents passing back into the Ileum. This is a sphincter which prevents the IC contents passing back into the Ileum.

15. What is SBS Reduced bowel surface area for absorption of nutrients together with rapid transit of intestinal contents. Reduced bowel surface area for absorption of nutrients together with rapid transit of intestinal contents. TPN reduced as enteral feeds are introduced. TPN reduced as enteral feeds are introduced. Need to promote intestinal adaptation. Need to promote intestinal adaptation.

16. Motility The IC valve and colon is important to slow intestinal transit. The IC valve and colon is important to slow intestinal transit. Proteins, Fats and Carbohydrates are absorbed almost completely within first 150cm of small bowel. Proteins, Fats and Carbohydrates are absorbed almost completely within first 150cm of small bowel.

17. Jejunum – most of electrolyte absorption Jejunum – most of electrolyte absorption Ileum is the only site for absorption of Vit B12 and bile salts. Ileum is the only site for absorption of Vit B12 and bile salts.

18. After resection. Increase gastric emptying. Increase gastric emptying. Ileal resection, increased transit time Ileal resection, increased transit time An intact IC valve prolongs gut transit, loss of this causes an increase. An intact IC valve prolongs gut transit, loss of this causes an increase. If colon resected transit increases. If colon resected transit increases.

19. Duodenal resection – malabsorption of Iron, Calcium and Folic Acid. Duodenal resection – malabsorption of Iron, Calcium and Folic Acid. Jejunal resection – If extensive resection, lactose intolerence Jejunal resection – If extensive resection, lactose intolerence Ileal resection – Some diarrhoea due to bile salts being incompletely absorbed. Ileal resection – Some diarrhoea due to bile salts being incompletely absorbed.

20. Gastric Hypersecretion After abdominal surgery, gastric hyper- secretion occurs in 50% cases. After abdominal surgery, gastric hyper- secretion occurs in 50% cases. This impairs digestion of lipids by lowering intraluminal PH and inactivating the pancreatic enzymes. This impairs digestion of lipids by lowering intraluminal PH and inactivating the pancreatic enzymes. Also stimulates peristalsis. Also stimulates peristalsis.

21. How does the bowel adapt? Cellular hyperplasia Cellular hyperplasia Villous hypertrophy Villous hypertrophy Intestinal lengthening Intestinal lengthening Altered motility Altered motility Hormonal changes Hormonal changes Takes approx 2 years to reach max effect. Takes approx 2 years to reach max effect.

22. Management of SBS. Total TPN Total TPN Gradual introduction of enteral feeding. Gradual introduction of enteral feeding. Fluid and electrolyte balance Fluid and electrolyte balance Fluid replacement if stool, gastric aspirate or ostomy losses are high Fluid replacement if stool, gastric aspirate or ostomy losses are high Reducing substances above1% contra indicate increasing enteral feeds. Reducing substances above1% contra indicate increasing enteral feeds.

23. Weaning off TPN Cycling – one hour off, line lock with Gentamycin. Build up to off all day. Cycling – one hour off, line lock with Gentamycin. Build up to off all day.

24. Complications. Bacterial overgrowth Bacterial overgrowth Anaemia Anaemia Bile salt depletion Bile salt depletion Bone disease Bone disease Cholestasis Cholestasis Diarrhoea Diarrhoea Hypocalcaemia Hypocalcaemia

25. Complications (cont) Hypomagnesaemia Hypomagnesaemia Liver fibrosis Liver fibrosis Renal stones Renal stones Protein malnutrition Protein malnutrition Trace mineral deficiency Trace mineral deficiency Vitamin deficiency, A, D, E, K, B12 Vitamin deficiency, A, D, E, K, B12

26. Central line complications Infection Infection Thrombosis Thrombosis Break in catheter Break in catheter Air embolus Air embolus Tissue necrosis Tissue necrosis Malposition Malposition Cardiac tamponade Cardiac tamponade

27. Bacterial Overgrowth Bloating, cramps, diarrhoea, gastrointestinal blood loss. Bloating, cramps, diarrhoea, gastrointestinal blood loss. Treat with sugar free Metronidazole and Trimethoprim Treat with sugar free Metronidazole and Trimethoprim

28. Watery diarrhoea Loperamide Loperamide Malabsorption of bile acids. Malabsorption of bile acids. Pectin Pectin

29. Surgery Further resection might be avoided by tapering, strictureplasty or serosal patching. Further resection might be avoided by tapering, strictureplasty or serosal patching. Patients with dilated segments proximal to tight anastomosis – resect and taper improves bacterial overgrowth by improving flow. Patients with dilated segments proximal to tight anastomosis – resect and taper improves bacterial overgrowth by improving flow.

30. Tapering

31. Bowel lengthening Cutting bowel longitudinally, preserve blood supply to both sides and create a segment of bowel twice length, half diameter without loss of mucosal surface area. Cutting bowel longitudinally, preserve blood supply to both sides and create a segment of bowel twice length, half diameter without loss of mucosal surface area.

32. Bowel lengthening

33. Antiperistaltic small intestine segment

34. Colonic interposition

35. Medical management Pectin (water sol, non cellulose dietary fibre which promotes intestinal adaptation) Pectin (water sol, non cellulose dietary fibre which promotes intestinal adaptation) Ranitidine (PH > 4) Ranitidine (PH > 4) Loperamide (slow gut transit time) Loperamide (slow gut transit time) Cholestyramine (binds bile salts) Cholestyramine (binds bile salts)

36. It takes approximately two years to achieve some normal diet

37. Thank you

38. References Bentley D, Lifschitz C, Lawson M (2001). Necrotising Entercolitis Bentley D, Lifschitz C, Lawson M (2001). Necrotising Entercolitis And Short Bowel Syndrome. http://www.naspghan.org/wmspage.cfm?porm1=130 And Short Bowel Syndrome. http://www.naspghan.org/wmspage.cfm?porm1=130http://www.naspghan.org/wmspage.cfm?porm1=130 Koglmeier J, Day C, Puntis J (2008). Clinical outcome in patients from a single region who were dependent on parenteral nutrition for 28 days or more. Archives of Disease in Childhood. 93 (4) : 300 - 302 Koglmeier J, Day C, Puntis J (2008). Clinical outcome in patients from a single region who were dependent on parenteral nutrition for 28 days or more. Archives of Disease in Childhood. 93 (4) : 300 - 302 Martin G, Wallace L and Sigalet D (2004). Glucagon – like Peptide -2 Induces Intestinal Adaptation in Parenterally Fed Rats with Short Bowel Syndrome. American Journal of Physiology. Gastro-intestinal and Liver Physiology. 286: G964-G972 Martin G, Wallace L and Sigalet D (2004). Glucagon – like Peptide -2 Induces Intestinal Adaptation in Parenterally Fed Rats with Short Bowel Syndrome. American Journal of Physiology. Gastro-intestinal and Liver Physiology. 286: G964-G972 McMahon M, Leviller J and Chescheir N (1996). Prenatal Ultrasonographic Findings Associated with Short Bowel Syndrome in Two Fetuses with Gastroschisis. Obstetrics and Gynaecology. 88: 676-678 McMahon M, Leviller J and Chescheir N (1996). Prenatal Ultrasonographic Findings Associated with Short Bowel Syndrome in Two Fetuses with Gastroschisis. Obstetrics and Gynaecology. 88: 676-678 Seidner D and Matarese L (2003). Selected topics in Seidner D and Matarese L (2003). Selected topics in Gastrotherapy. Case 2: Short Bowel Syndrome : Etiology, Gastrotherapy. Case 2: Short Bowel Syndrome : Etiology, Pathophysiology and Management. The Cleveland Clinic Center for Continuing Education Pathophysiology and Management. The Cleveland Clinic Center for Continuing Education Sinden A, Sutphen S (2003) Nutritional Management of Paediatric Short Bowel Syndrome. Nutrition Issues in Gastroenterology. Series #12 p28-48 Sinden A, Sutphen S (2003) Nutritional Management of Paediatric Short Bowel Syndrome. Nutrition Issues in Gastroenterology. Series #12 p28-48 Warner B, Vanderhoof J and Rayes J (2000). What’s New In The Management of Short Gut Syndrome in Children. Division of Paediatric Surgery. Department of Surgery. American College of Surgeons. p725-736 Warner B, Vanderhoof J and Rayes J (2000). What’s New In The Management of Short Gut Syndrome in Children. Division of Paediatric Surgery. Department of Surgery. American College of Surgeons. p725-736