Arianna Bertocchini1, Giuseppe d'Ambrosio2, Chiara Grimaldi2, Laura del Prete2, Fabrizio di Francesco2, Piergiorgio Falappa3, Lidia Monti4, Jean de Ville de Goyet2. 1. Hepato-biliary and Transplant Surgery Unit, Department of Surgery and Transplantation Centre, Bambino Gesù Childrens Hospital, Roma, Italy. Electronic address: arianna.bertocchini@opbg.net. 2. Hepato-biliary and Transplant Surgery Unit, Department of Surgery and Transplantation Centre, Bambino Gesù Childrens Hospital, Roma, Italy. 3. Interventional Radiology Unit, Department of Surgery and Transplantation Centre, Bambino Gesù Childrens Hospital, Roma, Italy. 4. Hepato-biliary Radiology Unit, Department of Imaging, Bambino Gesù Childrens Hospital, Roma, Italy.
Abstract
INTRODUCTION: Portal vein aneurysms (PVAs) are usually located at the vein trunk or at its bifurcation, rarely intra-hepatic, or at the umbilical portion. Etiology remains unclear. METHODS: Three children with PVA were identified over a 5-year period. PVA anatomy was assessed by Doppler Ultrasound, Angio CT/MRI, and trans-jugular retrograde portography. RESULTS: Three children with intrahepatic PVA (including the umbilical portion) were identified during assessment for pre-hepatic portal hypertension: all had splenomegaly and hypersplenism. One presented with massive variceal bleeding. In two cases, a portal vein cavernoma was found, and in the third a severe stricture at the portal bifurcation was observed. Restoration of portal venous flow was achieved by a meso-Rex bypass in two cases and transposing the PV into the Rex in one. High hepatopetal portal flow was restored immediately, with follow-up confirming long-term patency and resolution of signs of portal hypertension with time. CONCLUSIONS: These original observations suggest a common initial malformative pattern consisting of a portal venous stricture/web causing a post-stenotic aneurysmal dilatation of the intrahepatic portal branches complicated by thrombosis and cavernomatous transformation of the portal vein trunk. Importantly, the Meso-Rex bypass allows restoring a normal portal flow and cures the portal hypertension.
INTRODUCTION: Portal vein aneurysms (PVAs) are usually located at the vein trunk or at its bifurcation, rarely intra-hepatic, or at the umbilical portion. Etiology remains unclear. METHODS: Three children with PVA were identified over a 5-year period. PVA anatomy was assessed by Doppler Ultrasound, Angio CT/MRI, and trans-jugular retrograde portography. RESULTS: Three children with intrahepatic PVA (including the umbilical portion) were identified during assessment for pre-hepatic portal hypertension: all had splenomegaly and hypersplenism. One presented with massive variceal bleeding. In two cases, a portal vein cavernoma was found, and in the third a severe stricture at the portal bifurcation was observed. Restoration of portal venous flow was achieved by a meso-Rex bypass in two cases and transposing the PV into the Rex in one. High hepatopetal portal flow was restored immediately, with follow-up confirming long-term patency and resolution of signs of portal hypertension with time. CONCLUSIONS: These original observations suggest a common initial malformative pattern consisting of a portal venous stricture/web causing a post-stenotic aneurysmal dilatation of the intrahepatic portal branches complicated by thrombosis and cavernomatous transformation of the portal vein trunk. Importantly, the Meso-Rex bypass allows restoring a normal portal flow and cures the portal hypertension.