[Cavernous transformation of portal vein].

Introduction: In Cavernous transformation of portal vein (CTPV), therapeutic options are limited; however the restoration of circulation by mesenteric-portal bypass (MPB) is an option in selected cases. CASE REPORT 1: 13-year-old female with polymalformative syndrome. Admission at 4 months of age to Intensive Care Unit due to severe pneumonia with hemodynamic instability. Follow up due to thrombocytopenia and splenomegalia she was diagnosed CTPV at7-years old. At 13y-old she was submitted to MPB. CASE REPORT 2: 9-years-old male; severe neonatal Rh isoimmunization treated with exsanguinations. Followed-up since 6-months ofage due to thrombocytopenia and splenomegalia, and at 3 years of age he was diagnosed CTPV. Due to disease progression he was proposed as candidate to MBP which was contraindicated due to vascular thrombosis of the Rex recess.
Comments: MBP presents as one of the few therapeutic options to CTPV in children; due to its specificity and rigid requirements it is vital the close follow up and early diagnosis.