Asia Pacific Journal of Pediatric and Child Health

Abstract Ref Number = APCP1152

Poster Presentation

HIGH OUTPUT PRODUCT OF POST-OPERATIVE ENTEROCUTANEOUS FISTULA IN PEUTZ-JEGHERS SYNDROME A CASE REPORT

WindaYanuarni Meye,Endy Paryanto Prawirohartono,Neti Nurani Department of Child Health Faculty of Medicine Publich Health and Nursing Universitas Gadjah Mada Dr Sardjito General Hospital Yogyakarta Indonesia Division of Pediatric Nutrition and Metabolic Disease, Department of Child Health, Faculty of Medicine Public Health and Nursing, Universitas Gadjah MadaDr Sardjito General Hospital, Yogyakarta, Indonesia

Background : Peutz-Jeghers Syndrome (PJS) is a rare autosomal dominant disorder with prevalence 1 in 8300 to 280.000 individuals. The possible conditions observed in patient with PJS are instussuception (46.9%), obstruction (42.8%), and bleeding (13.5%). These conditions require surgical intervention that lead to complications such as post-operative enterocutaneous fistula (ECF). Case Presentation Summary : A 15 year-old boy presented with severe malnutrition (BMI for age -5.4 SD of WHO curve), anemia, multiple mucocutaneous hyperpigmentation patch at the lower lips and signs of gastrointestinal obstruction. Pre-operative abdominal CT scan revealed ileo–ileal invagination. Explorative laparotomy procedure was performed, indicating multiple intraluminal polyps and ileo-ileal invagination. Anastomosis resection along ileo-ileal site and biopsy were performed with the result of polypoid hemangioma suggestive for PJS. Unfortunately, postoperative ECF size 25 x 7 cm was developed with high output product ranging from 910 to 2770 ml/day (31.3 to 95.5 ml/kgbw/day) during 26 consecutive days of observation. Nutritional supports were given with parenteral nutrition, elemental formula as initial feeding and micronutrient (zinc, folic acid, and cobalamin injection), including correction of hypoalbuminemia, electrolyte and fluid imbalance. Antibiotic and antifungal regimens were given according to the fecal and ECF culture result. However, due to progression of severe sepsis, surgical closure of the fistula defect was unable to be performed. Despite the adequate treatment, patient passed away after 2 months of hospitalization. Learning Points/Discussion : Burden in managing high output ECF product within this case is related with the complication of sepsis, malnutrition, electrolyte and fluid abnormalities which leads to chronic intestinal failure; thus surgical closure of ECF was not performed. The major risk of high output stoma product within this case is PJS as intrinsic intestinal disease and location of ECF that may involve small bowel.

Keywords: Peutz-Jeghers Syndrome enterocutaneous fistula high output product