Intestinal Rehabilitation and Transplantation

Transcription

1 Intestinal Rehabilitation and Transplantation Joel Lim, MD Associate Professor of Pediatrics Children s Mercy Hospital University of Missouri in Kansas City

2 Objective: Intestinal Failure/Short Bowel Syndrome Medical Management - Enteral Nutrition - Parenteral Nutrition - Pharmacologic Therapy Surgical Management - Lengthening procedure - Intestinal transplantation

3 Intestinal Failure/Short Bowel Syndrome The reduction of gut function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes such that intravenous supplementation is required to maintain health and/or growth. Pironi L, et al. Clin. Nutr 2015;34:171-80

4 Etiology: Anatomic short gut NEC and congenital malformations Functional- Pseudo- obstruction and congenital diarrhea syndromes Mixed - long segment hirschsprung s disease - Gastroschisis- atresia syndrome

5 iron carbohydrates, protein, fats, vitamins, minerals, trace elements water, electrolyte, vitamin B 12, bile salt, fat, fat soluble vitamins water, electrolytes, SCFA, oxalates

6 Evaluation of Length and Site Surgical measurement Radiologic evaluation UGI- SBFT Citrulline level

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8 Nutritional Assessment Weight, height and head circumference Weight values may be underestimated due to fluid shift, ascites, ostomy output and organomegaly Mid- arm circumference and triceps skin fold thickness is important

9 MEDICAL MANAGEMENT Nutritional Pharmacologic

10 Nutritional Management Enteral Feeding - Initiated as soon as possible drip vs. bolus feeds What formula? - breast milk is still the most optimal Cow s milk/soy based formula Hydrolyzed formula Amino Acid based formula

11 When to Feed As soon as postoperative ileus resolves Prompt initiation of enteral feeding increases the rate of enteral autonomy and decrease duration of hospitalization 11

12 Oral vs Tube Feeding Macronutrient absorption in percent was significantly increased in Enteral tube feeding (ETF), and Oral continuous feeding (OCEF) vs. Oral feeding (oral feeding) Francisca J Gastroenterology 2009;136:

13 Nutritional Management Fiber Insoluble fiber (wheat bran and psyllium) - gelatinize stool but no effect on bowel adaptation Soluble fiber (pectin and guar gum) - converted to short chain fatty acid (butyrate) - can cause osmotic diarrhea

14 Nutritional Management Monitoring - Iron deficiency - Zinc deficiency - Iodine deficiency - Calcium, chromium, copper, manganese, and selenium - Fat soluble vitamins - Water soluble vitamins

15 Parenteral Nutrition Life saving therapy Associated with complications - Intestinal Failure Associated Liver Disease (IFALD) - Central line infection - Vascular thrombosis

16 Predictors of ending PN Small bowel length - >40 cm - > or equal to 10% of expected small bowel length for gestational age - colon length greater than 50% - Retention of ICV ( after 1 year) - Combination of SB >20%+intact colon+icv - NEC and intestinal atresia best prognosis gastroschisis- atresia worst prognosis

17 Parenteral Nutrition Management of IFALD - Ursodeoxycholic acid - Limiting PN caloric intake to kcal/kg - Cycling PN - Limiting the fat - Aggressively treating infection - Advancing enteral nutrition - Fish oil (Omega 3 fatty acid)

18 Parenteral Nutrition Omegaven (Fish Oil) - Omega 3 fatty acids - Improved triglyceride clearance - Anti- inflammatory effect SMOF (Soy, MCT, Olive oil and Fish oil) - adequate balance of omega- 3 and omega- 6 fatty acids

19 Central Line Access (catheters) Central Line - Broviac - Hickmann - PICC line - Port- a- cath

20 Central Line Access (catheters) Placed by skilled team Proper position Smaller size Single lumen

21 Catheter Associated Complications Infection - prevalence is 1.4/1000 days - poor line care - bacterial translocation - Short gut patients have a 6 fold increase in infection - Catheter salvage rate is 42-52%

22 Catheter Associated Complications Prevention of Infection - Good line care - Appropriate antibiotic treatment - Antibiotic coated catheter - Antibiotic lock therapy - Ethanol lock therapy

23 Ethanol Lock Therapy 70% ethanol, Silicone lines 63% reduction in infection rates Adverse events are rare which include thrombosis Increase in catheter repair rate with ethanol

24 Catheter Associated Complications Thromboses - prevalence of 0.2/1000 days - Coagulation defect work- up - Anti- coagulation therapy?

25 Catheter Associated Complications Imaging Studies - Doppler Ultrasound - Standard Venography - CT Angiography - MRI with Feraheme

26 Thromboses

27 Pharmacologic Approach Antimicrobials - bacterial translocation - bacterial overgrowth - short courses (rotational) of oral antimicrobials - rifaximin, metronidazole, and oral gentamicin Lactobacillus rhamnosus GG (probiotics contraindicated with central lines) Pancreatic Enzymes H2 blockers and Proton pump inhibitors

28 Glucagon Like Peptide - 2 (GLP- 2) Synthesis confined to the ileum and right colon Inhibits apoptosis and stimulates proliferation of the small intestine Improves fluid and electrolytes absorption and citrulline levels Effects were only transient 12 week safety trial in pediatric population (2017)

29 Oral Insulin Present in breast milk Insulin receptors expressed in small bowel Increase cell proliferation Decrease enterocyte loss Multi- center study in Europe and North America

30 Surgical Management

31 Surgical Management Prevention and retention of intestinal length Restoration of Intestinal Continuity Lengthening procedures

32 LILT- Bianchi

33 Serial Transverse Enteroplasty Kim. J Pediatr Surg 2003

34 Tissue Bioengineering Intestinal lengthening is feasible In continuity expanders

35 Intestinal/Multivisceral Transplantation

36 History of Intestinal Transplantation Early 1950 s first reported transplantation in dogs 1990 s first successful transplantation due to discovery of more potent immunosuppression 2000 s Rapid increase in the number of intestinal transplantation 2010 s 25% decline in transplantation due to successful rehabilitation

37 Intestinal Transplantation (types)

38 Indication for Transplantation Severe intestinal failure associated liver disease Loss of venous access Recurrent central line infection (life threatening) Complete Mesenteric Thrombosis Extremely short residual bowel

39 Intestinal Transplants Performed Number of Transplants Year of Transplant Children Adults

40 Pediatric Patient Survival by Transplant Type ( )

41 Transplantation Annual IT case volumes have leveled after a steep decline and seem to be increasing There is a trend to include the colon with the intestine graft, with evidence that this practice improves outcomes. There have been no major advances in IT graft survival rates for the past 5 years.

42 Interdisciplinary team Improves the outcome of patients with short gut syndrome (intestinal failure) Early referral is imperative before cholestasis and other complications set in

43 Take Home Message Aggressive early small bowel rehabilitation is needed (multidisciplinary team) Surgical lengthening should be considered Intestinal transplant is an emerging treatment for patients with intestinal failure

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45 Intestinal Rehabilitation and Transplantation Association Encouraging membership of non- transplant programs Worldwide Pediatric Intestinal Failure Registry Looking for a center to represent Asia jdlim@cmh.edu