Critical hepatic hemangioma in infants: Recent nationwide survey in Japan

摘要: The International Society for the Study of Vascular Anomalies (ISSVA) classification divides vascular lesions into two major entities: neoplasms originating from endothelium and malformations. Although this concept has been widely accepted, little established regarding in deep organs, such as infantile hepatic hemangioma (IHH). current nationwide survey identified 19 critical hemangiomas during most recent 5 years. On histopathology all examined were neoplastic, but portovenos shunt was found histologically or clinically some cases. High-output cardiac failure, consumption coagulopathy, respiratory distress symptoms, treatment-resistant coagulopathy seemed to be reliable predictor fatal outcome. steroid gold standard treatment these lesions, 25% patients totally insensitive steroids, whereas propranolol had a prompt effect one case. For IHH with steroid-insensitive thrombocytopenia prothrombin time prolongation, novel therapeutic options including beta-blocker therapy, surgery, liver transplantation should urgently considered alterative treatment. present review summarizes results survey.