1993 Diagnosis and Management of Porto-Porto Varices in a Patient With Prior Orthotopic Liver Transplant

Abstract

INTRODUCTION: Gastrointestinal variceal bleeding is a frequent complication in patients with liver disease. Small bowel varices represent a less common source of bleeding, and are often challenging to manage due to their location. We describe a case of bleeding small bowel porto-porto varices in a young patient status-post orthotopic liver transplant. CASE DESCRIPTION/METHODS: An 18-year-old-female patient presented with melanotic stools and a presyncopal episode. Her medical history was significant for iron deficiency anemia and biliary atresia. Associated surgical history included hepatoportoenterostomy in infancy and orthotopic liver transplantation at age two. Admission labs were significant for hemoglobin (Hgb) of 4.0 g/dL. Hepatic enzymes, INR and platelets were within normal limits. Initial colonoscopy and EGD were revealing for hematin in the cecum and colon. CT enterography showed smaller collateral veins draining into a large superior mesenteric collateral vein. This finding was confirmed by triple phase CT angiography, which also demonstrated chronic occlusion of the portal vein. The collaterals provided bypass of the thrombosed portal vein, and also included jejunal submucosal porto-porto varices. As the patient’s hemoglobin continued to drop despite blood transfusions, recanalization of the portal vein was attempted by Interventional Radiology (IR). The portal vein was stented, resulting in reduced flow through collateral vessels. The patient was taken back to the IR suite three days later as she had recurrent melena and Hgb drop. The stent had thrombosed, and a combination of chemical and mechanical thrombolysis was used to restore flow. Coil embolization of three collateral vessels from the SMV was also performed. A contrasted CT scan of the abdomen three days later revealed a patent stent with significant improvement in jejunal porto-porto varices. At her last clinic appointment two months after discharge, the patient denied further bleeding. DISCUSSION: This case illustrates the diagnostic and therapeutic challenges in patients with small bowel varies. This case is noteworthy as it highlights a type of collateralization, porto-porto varices, which have not been well described. Furthermore, this case occurred in a population not frequently studied: patients who previously underwent orthotopic liver transplant for biliary atresia. Recognition and appropriate treatment of associated liver conditions is crucial to increasing survival in this group.