S5961 An Unexpected Turn: Jejunal Varices in Post-Transplant Alagille Syndrome

Introduction: Alagille syndrome is a rare genetic disorder (∼1 in 70,000) affecting multiple organs; hepatic involvement can manifest as cholestasis from bile duct paucity. Symptoms may include conjugated hyperbilirubinemia, xanthomas, and pruritus, and up to 15% develop end-stage liver disease. Additionally, jejunal varices may form. These rare occurrences arise from prior radiation, portal and/or splanchnic vein thrombosis, or surgery, and can cause hemorrhagic shock. Case Description/Methods: A 37-year-old African American man with Alagille syndrome with deceased donor liver transplant (DDLT) at age 4 on tacrolimus presented with hemorrhagic shock from a gastrointestinal bleed. History included biliary anastomotic stricture with hepaticojejunostomy, percutaneous cholecystostomy, allograft cirrhosis with portal hypertension, multiple varices, splenomegaly post splenic artery embolization, chronic portal, mesenteric, and splenic vein thrombosis, and neuroendocrine tumor with mesenteric metastasis s/p bowel resection. The patient had multiple prior admissions for GIB. CTA showed a 15 cm cluster of jejunal varices at the anastomotic site and esophageal varices (EV), which were confirmed alongside duodenal varices on esophagogastroduodenoscopy. This time he had a hemoglobin of 7 (baseline 9) and hematochezia. He was transfused; bleeding resolved spontaneously, per usual. Model for End-Stage Liver Disease was 13 (international normalized ratio low 1, creatine mid 1, platelets 30k, sodium normal, all baseline), with his alkaline phosphatase and aspartate aminotransferase chronically elevated in the 300s and 50s, respectively. Alanine aminotransferase was normal. Discussion: This case is unique as there are no reports of jejunal varices after DDLT in Alagille syndrome. His ectopic varices are likely related to prior hepaticojejunostomy and graft cirrhosis (thus portal thrombosis). Treatments in literature include endoscopic sclerotherapy, coil embolization, small bowel resection (SBR), and transjugular intrahepatic portosystemic shunt (TIPS). Decisions must be individualized. TIPS was not feasible due to extensive thrombosis in our patient. IR-guided embolization and re-transplantation was also limited by vasculature due to thrombosis. SBR required creating a new anastomosis, with high recurrence risk without treating underlying liver disease. Multivisceral transplant was contemplated but limited due to high mortality and thrombosis. Double-balloon sclerotherapy would be technically challenging because of depth and would only be temporizing; also, it may injure the varices. The patient continues with hepatology follow-up, with treatment pending further discussion.