title: Prehepatic portal hypertension with aneurysm of the portal vein: unusual but treatable malformative pattern.
abstract: BACKGROUND
  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.
authors.affiliation: Hepato-biliary and Transplant Surgery Unit, Department of Surgery and Transplantation Centre, Bambino Gesù Childrens Hospital, Roma, Italy. Electronic address: arianna.bertocchini@opbg.net.
authors.forename: Arianna
authors.initials: A
authors.lastname: Bertocchini
authors.affiliation: Hepato-biliary and Transplant Surgery Unit, Department of Surgery and Transplantation Centre, Bambino Gesù Childrens Hospital, Roma, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: d'Ambrosio
authors.affiliation: Hepato-biliary and Transplant Surgery Unit, Department of Surgery and Transplantation Centre, Bambino Gesù Childrens Hospital, Roma, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Grimaldi
authors.affiliation: Hepato-biliary and Transplant Surgery Unit, Department of Surgery and Transplantation Centre, Bambino Gesù Childrens Hospital, Roma, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: del Prete
authors.affiliation: Hepato-biliary and Transplant Surgery Unit, Department of Surgery and Transplantation Centre, Bambino Gesù Childrens Hospital, Roma, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: di Francesco
authors.affiliation: Interventional Radiology Unit, Department of Surgery and Transplantation Centre, Bambino Gesù Childrens Hospital, Roma, Italy.
authors.forename: Piergiorgio
authors.initials: P
authors.lastname: Falappa
authors.affiliation: Hepato-biliary Radiology Unit, Department of Imaging, Bambino Gesù Childrens Hospital, Roma, Italy.
authors.forename: Lidia
authors.initials: L
authors.lastname: Monti
authors.affiliation: Hepato-biliary and Transplant Surgery Unit, Department of Surgery and Transplantation Centre, Bambino Gesù Childrens Hospital, Roma, Italy.
authors.forename: Jean
authors.initials: J
authors.lastname: de Ville de Goyet
mesh_terms: D000293:Adolescent; D000714:Anastomosis, Surgical; D000783:Aneurysm / Q000209:etiology* / Q000601:surgery; D001783:Blood Flow Velocity; D019917:Blood Vessel Prosthesis Implantation; D002675:Child, Preschool; D003952:Diagnostic Imaging; D004932:Esophageal and Gastric Varices / Q000209:etiology; D005260:Female; D006392:Hemangioma, Cavernous / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology*; D007601:Jugular Veins / Q000637:transplantation; D008113:Liver Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D011169:Portal Vein / Q000002:abnormalities* / Q000196:embryology / Q000601:surgery; D011172:Portography; D013163:Splenomegaly / Q000209:etiology; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000379:methods*; D020246:Venous Thrombosis / Q000209:etiology
keywords: Extrahepatic portal hypertension; Intrahepatic portal vein; Meso Rex bypass; Rex recessus; Vein Aneurysm