Multiorgan Transplantation as a Viable Treatment Option in Infantile Juvenile Polyposis Syndrome.

e86 CASE REPORT I nfantile juvenile polyposis syndrome (IJPS) is a rare and aggressive form of juvenile polyposis syndrome (JPS) that usually presents very early in the first year of life. It generally has poor prognosis due to chronic gastrointestinal (GI) bleeding, proteinlosing enteropathy, subsequent malnutrition, and immune deficiency. We herein describe a child with IJPS who underwent multiorgan transplantation. The patient was born at full term and her perinatal period was uneventful. By the age of 9 months, she presented with multiple episodes of intussusception. Abdominal magnetic resonance imaging and subsequent endoscopy revealed multiple small bowel and colonic polyps. She was initially managed with endoscopic polypectomies. Pathology confirmed juvenile polyps, with no dysplasia (Fig. 1). Her genetic workup using targeted microarray analysis revealed heterozygous deletion of BMPR1A gene. Due to high polyp burden unmanageable by endoscopy, she underwent a total colectomy with an end ileostomy at the age of 16 months. She, however, continued to have regrowth of polyps in her stomach and remaining small bowel, with recurrent bleeding episodes, leading to severe anemia (hemoglobin of 5.4 g/dL) requiring frequent blood transfusions, and hypoalbuminemia (albumin <1 g/dL). She even had obstruction of her ileostomy due to regrowth of polyps. Once her nutrition and blood indices were optimized, she underwent an isolated small bowel transplant at the age of 2 years, with a jejunojejunostomy and end ileostomy. Her native small bowel explant revealed several polyps up to 1.6 cm in size, with no dysplasia. She, however, developed severe acute cellular intestinal rejection 6 weeks posttransplant, complicated by acute renal failure and hemodynamic instability requiring explantation of her graft. She recovered well and was re-listed for multiorgan transplant while being maintained on total parental nutrition. Subsequently she underwent a liver, small bowel, pancreas, and colon transplant at the age of 3.5 years. She had a roux-en-Y gastrojejunostomy, a loop ileostomy, and an end colostomy with rectal stump closure. She also had a splenectomy as it is routinely performed in patients receiving multiorgan transplant at our center. She received standard immunosuppression protocol, which