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Pediatric Intestinal Failure

Wednesday Marie A. Sevilla, MD, MPH Jeffrey A. Rudolph, MD Children’s Hospital of Pittsburgh Reviewed by Jyoti Ramakrishna, MD of the Professional Education Committee. Pediatric Intestinal Failure. Patient Case #1. Infant born premature at 28 weeks via vaginal delivery

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Pediatric Intestinal Failure

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  1. Wednesday Marie A. Sevilla, MD, MPH Jeffrey A. Rudolph, MD Children’s Hospital of Pittsburgh Reviewed by Jyoti Ramakrishna, MD of the Professional Education Committee Pediatric Intestinal Failure

  2. Patient Case #1 • Infant born premature at 28 weeks via vaginal delivery • Developed abdominal distention and bloody stools at 2 weeks of age • Abdominal x-ray showed intestinal pneumatosis • Diagnosed with necrotizing enterocolitis (NEC)

  3. Patient Case #1 • Underwent serial abdominal surgeries resulting in the significant resection of gangrenous bowel • At the last surgery, patient had 15 cm of small bowel anastomosed to an intact colon with an ileocecal valve • Discharged at 5 months of life • 22-hour daily TPN • Enteral feeds with elemental formula, 10 ml by mouth as tolerated

  4. Patient Case #2 • Infant born full term at 37 weeks, delivered via vaginal delivery, Apgar scores 7-10 • Prenatal diagnosis of Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) • Started on total parenteral nutrition (TPN) at first day of life

  5. Patient Case #2 Upper GI and barium enema: • Atonic and malrotated small bowel • Malrotatedmicrocolon Voiding cystourethrogram (VCUG): • Enlarged atonic bladder

  6. Patient Case #2 • Patient underwent gastrostomy tube (g-tube) placement and decompressing ileostomy at 5 days of age • Surgical pathology sample of small bowel showed small bowel with focal defects of longitudinal muscle layer • Patient discharged at 3 weeks of age • TPN with lipids running 24 hours per day • intravenous ampicillin as urinary tract infection (UTI) prophylaxis • No scheduled feeds via g-tube

  7. Definition of Intestinal Failure Critical reduction of functional gut mass below the minimal amount necessary for adequate digestion and absorption to satisfy body nutrient and fluid requirements in adults or growth in children. Thompson, JS. Gastroenterology 2006;130:S3-S4

  8. Definition of Intestinal Failure • Describes a physiological state • Encompasses both anatomic and/or functional causes • Leads to alterations in the normal physiological function of the intestine • Implies a dependence upon total parenteral nutrition to maintain homeostasis

  9. Etiology of Intestinal Failure in Infants Squires RH et al, for the Pediatric Intestinal Failure Consortium. J Pediatr 2012; 161:723-8.

  10. Etiology of Intestinal Failure in Pediatrics: Anatomical vs. Functional Perspectives Autoimmune enteropathies Congenital enteropathies pseudoobstruction Ultra short gut full length intestine Surgical NEC Malrotation/volvulus Gastroschisis Long segment Hirschsprungs Intestinal atresias Adapted from: Rudolph JA, Children’s Hospital of Pittsburgh of UPMC, 2011

  11. “Short Bowel Syndrome”:A Subset of Intestinal Failure • Loss of function from anatomic loss: • congenital absence • Surgical resection after abdominal catastrophes • The common form of intestinal failure; considered the “classic” form of the disease • Incidence of 3 to 5 per 100,000 live births (Squires et al, 2012) Small Intestine Colon

  12. Etiology of Short Bowel Syndrome in Pediatrics Goulet O and Ruemmele F. Gastroenterology 2006;130:S16-S28.

  13. Management of Intestinal Failure: Intestinal Adaptation • The mechanism through which feeds can be advanced over time • Compensation for loss of enterocyte mass • Marked by increase in intestinal absorptive capacity • Measured as toleration to feeds or radiographic evidence of intestinal dilation

  14. Management of Intestinal Failure:Intestinal Adaptation • Ways to promote adaption • Nutritional: gradual introduction of enteral feeds • Medical: no current approved medical therapies that promote adaptation • Surgical: lengthening

  15. Triad of Nutritional Support in Intestinal Failure Parenteral Nutrition Goal for TPN Weaning Maintain fluid/electrolyte/nutrient balance as nutrition is converted from parenteral to enteral feeds. • Tools for enteral autonomy: • Adequate anatomy • Enteral feeding device or surgeon willing to place one • Caregiver willingness Enteral Nutrition Tools for oral autonomy: • Adequate anatomy and neuroanatomy • Oral feeding skills • Innate at birth • Learned afterwards • Willing/patient caregiver Oral Feeding Skills Adapted from: Rudolph JA, Children’s Hospital of Pittsburgh of UPMC, 2011

  16. Management of Intestinal Failure: Nutritional Therapy • Enteral stimulation by nutrients important for intestinal adaptation • Ideal for short gut: breast milk • Provides nucleotides, immunoglobulin A and growth factors • May shorten period of TPN use

  17. Management of Intestinal Failure:Nutritional Therapy • Consideration on macronutrients • Simple sugars can cause diarrhea due to relative disaccharidase deficiency • Mixture of long- and medium-chain triglycerides • Protein in breast milk and hydrolyzed or elemental formulas allow for faster weaning from TPN • Enteral feeding: • Need to balance amount given with stoma or stool output • Gradual increase as toleration is observed • Bolus vs. continuous feeds- no definite data in children available

  18. Management of Intestinal Failure:Monitoring Nutritional Deficiencies • High prevalence of zinc and vitamin D deficiency • Other deficiencies: fat soluble vitamins, copper, selenium and iron • Electrolyte depletion: sodium, potassium, bicarbonate • Steatorrhea from increased enteral intake: calcium, magnesium and zinc loss • Vitamin B12 deficiency- patients with ileal resection

  19. Management of Intestinal Failure: Medical Management • Decreasing motility to enhance adaptation • Anti-motility agents • Loperamide • Diphenoxylate Hydrochloride/Atropine • Increase transit time to increase functional load • Administration of soluble fiber • Used in patients with intact colon • Can enhance colonic absorption

  20. Management of Intestinal Failure: Small Bowel Bacterial Overgrowth (SBBO) • Due to poor intestinal motility, presence of strictures and absence of an ileo-cecal valve • Can increase risk for development of IF-related liver disease • Symptoms: diarrhea, bloating, gassiness, D-lactic acidosis • Empiric treatment with “cycled” antibiotics – metronidazole, vancomycin, neomycin

  21. Management of Intestinal Failure:Lengthening • Surgical lengthening to improve transit time and increase surface area absorption • Intestinal valves • Reverse intestinal segments • Longitudinal intestinal lengthening and tailoring (LILT) • Serial transverse enteroplasty (STEP) LILT Procedure STEP Procedure

  22. Management of Intestinal Failure:Catheter-related blood stream infections (CRBSI) • Placement and home care of central venous catheter (CVC) necessary for patients with IF • CRBSIs a significant cause for morbidity and mortality • Most common cause of hospital admission • Entails a course of antibiotics • Can lead to septic shock and death • External contamination of CVC is a common source of blood stream infection • Role of bacterial translocation

  23. Management of Intestinal Failure:Catheter-related blood stream infections (CRBSI) • Enteric flora most common organism isolated: Klebsiella, Coagulase-negative Staph, Enterobacter • Prevention of CRBSIs • Continued education of primary care providers on aseptic techniques • Strategic placement of ports: away from stomas, genital area • Use of prophylactic ethanol lock therapy

  24. Management of Intestinal Failure:IF-related liver disease • Most frequent and most severe complication in patients with IF • Multi-factorial • Role of TPN • Environmental factors • Possible genetic factors

  25. Management of Intestinal Failure:IF-related liver disease • Prevention and treatment of liver injury: • Stimulating the enterobiliary axis through enteral feeding • Treatment of intraluminal bacterial overgrowth • Administration of ursodeoxycholic acid • Avoidance of catheter-related blood stream infection • Continued modification of PN intake

  26. Management of Intestinal Failure:Intestinal +/- Liver Transplant • Indicated in patients with irreversible IF when rehabilitation is not possible • Ultra-short gut • Long segment Hirschsprung’s disease • Congenital enteropathy • Transplantation Criteria • Liver disease • Thrombosis of major venous access • Recurrent catheter-related sepsis • Uncontrollable water losses causing severe acute dehydration

  27. Management of Intestinal Failure:Intestinal +/- Liver Transplant • Combined liver-small bowel transplantation • Patients with short gut and IF-related liver disease • Isolated liver transplantation • Patients with IF-related liver disease with length of remnant small bowel sufficient to achieve PN weaning • Rarely done and only in select cases when enteral autonomy can be achieved

  28. Outcomes of Pediatric Intestinal Failure • 47% of patients with IF reach enteral autonomy • Highest probably of reaching autonomy is during the first 2 years of life Squires RH et al, for the Pediatric Intestinal Failure Consortium. J Pediatr 2012; 161:723-8.

  29. Outcomes ofPediatric Intestinal Failure • Multi-disciplinary management at each stage of IF improves survival with less IF-related complication • Multidisciplinary approach includes: • Pediatric gastroenterologists and surgeons • At times, intensive care specialists • Specialized nurses and pharmacists • Dietitians and speech/occupational therapists • Social workers and psychologists

  30. References Bianchi A and Morabito A. The dilated bowel: a liability and an asset. SemPediatrSurg2009;18:249-257. Drews B, Sanghavi R, Siegel JD, Metcalf P and Mittal N. Characteristics of catheter-related bloodstream infections in children with intestinal failure. GastroenterolNurs2009;32(6):385-390. Goulet O and Ruemmele F. Causes and management of intestinal failure in children. Gastroenterol 2006;130:S16-S28. Hess RA, Welch KB, Brown PI and Teitelbaum DH. Survival outcomes of pediatric intestinal failure patients: analysis of factors contributing to improved survival over the past two decades. J Surg Res 2011;170:27-31. Javid PJ, Malone FR, Reyes J, Healey PJ and Horslen SP. The experience of a regional pediatric intestinal failure program: successful outcomes from intestinal rehabilitation. Am J Surg 2010;199:676-697. Kim HB, Fauza D, Garza J, Oh JT, Nurko S and Jaksic T. Serial Transverse Enteroplasty (STEP): A novel bowel lengthening procedure. J PediatrSurg2003;38:425-429. Rudolph JA and Squires RH. Current concepts in the medical management of pediatric intestinal failure. Current Op Org Transpl 2010;15:324-329. Squires RH et al for the Pediatric Intestinal Failure Consortium. Natural history of pediatric intestinal failure: initial report from the pediatric intestinal failure consortium. J Pediatr2012;161:723-8. Thompson JS. Overview of etiology and management of intestinal failure. Gastroenterol 2006;130:S3-S4. Youssef NN, Mezoff AG, Carter BA and Cole CR. Medical update and potential advances in the treatment of pediatric intestinal failure. CurrGastroenterol Rep 2012;14:243-252. Wales PW, Kosra C, Carricato M, de Silva N, Lang K and Avitzur Y. Ethanol lock therapy to reduce the incidence of catheter-related bloodstream infections in home parenteral nutritional patients with intestinal failure: preliminary experience. J PediatrSurg2011;46:951-956.

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