S6001 Multisystem Complications in Hollow Visceral Myopathy With Dysmotility Managed by Small Intestine Transplantation

Introduction: We present an unusual case of visceral myopathy with dysmotility managed by small intestine transplantation, complicated by rejection and graft failure. Case Description/Methods: A 32-year-old woman with hollow visceral myopathy first presents to the emergency department (ED) at age 18 with persistent nausea, emesis, and a 25-pound weight loss. Computed tomography imaging reveals retained stool. Despite normal stools in the ED, she remains unable to tolerate oral intake. She has no prior gastrointestinal history. Esophagogastroduodenoscopy and a gastric emptying study reveal severe gastroparesis and alkaline reflux gastritis. Multiple prokinetics offer minimal relief. By age 20, she requires a jejunostomy tube (J-tube) and total parenteral nutrition (TPN) due to oral and enteral intolerance. A venting gastrostomy tube is placed to reduce nausea. Attempts to wean from TPN fail due to J-tube pain, infection, frequent sepsis, chronic constipation, rectal bleeding, and upper extremity thromboses requiring anticoagulation. Small bowel biopsies confirm visceral myopathy by showing absent actin fibers. In April 2017, she undergoes isolated intestinal transplantation for intestinal failure. Initially, she gains weight and tolerates oral and enteral intake. However, she soon develops acute cellular rejection, treated with high-dose steroids and Thymoglobulin. Symptoms of emesis, diarrhea, and weight loss return. Persistent gastropareses, dysmotility, and poor absorption require TPN, complicated by central line infections, MRSA bacteremia, and access issues. She later develops severe antibody-mediated rejection, leading to frequent hospitalizations. Additional complications include duodenal perforation, nephrolithiasis, stage 3b chronic kidney disease, and cytomegalovirus, Epstein-Barr virus, adenovirus, and C.difficile infections. Immunosuppression includes tacrolimus, prednisone, intravenous immunoglobulin, and antimicrobials. By 2025, graft biopsies show rejection and chronic graft injury. Computed tomography and endoscopy show enterocolitis and inflammation near anastomoses. She remains TPN-dependent with failure to thrive and poor oral tolerance. Evaluation for re-transplantation is underway. Discussion: Intestinal transplant for severe motility disorders is rare, typically performed at specialized centers when TPN fails. While function may be restored, outcomes are limited by rejection risk, persistent dysmotility, and the need for lifelong immunosuppression. Chronic graft failure may necessitate re-transplantation.