Cavernous Transformation of Portal Vein

• Autor(a) principal: Mascarenhas, Maria Inês
• Data de Publicação: 2012
• Outros Autores: Carneiro de Moura, Marta, Sande Lemos, Piedade
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/296

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 at 7-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 of age 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.