title: Critical hepatic hemangioma in infants: recent nationwide survey in Japan.
abstract: The International Society for the Study of Vascular Anomalies (ISSVA) classification divides vascular lesions into two major entities: neoplasms originating from the vascular endothelium and vascular malformations. Although this concept has been widely accepted, little has been established regarding vascular lesions in deep organs, such as infantile hepatic hemangioma (IHH). The current nationwide survey identified 19 critical infantile hemangiomas during the most recent 5 years. On histopathology all the lesions examined were neoplastic, but portovenos shunt was found histologically or clinically in some cases. High-output cardiac failure, consumption coagulopathy, and respiratory distress were the major symptoms, and treatment-resistant coagulopathy seemed to be the most reliable predictor of fatal outcome. Although steroid has been the gold standard treatment for these lesions, 25% of the patients were totally insensitive to steroids, whereas propranolol had a prompt effect in one case. For critical IHH with steroid-insensitive thrombocytopenia and prothrombin time prolongation, novel therapeutic options including beta-blocker therapy, surgery, and liver transplantation should be urgently considered as alterative treatment. The present review summarizes the results of the survey.
authors.affiliation: Infantile Hepatic Hemangioma Study Group, Department of Pediatric Surgery, Keio University, School of Medicine, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Kuroda
authors.forename: Ken
authors.initials: K
authors.lastname: Hoshino
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Nosaka
authors.forename: Yohko
authors.initials: Y
authors.lastname: Shiota
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Takimoto
mesh_terms: D003625:Data Collection; D005260:Female; D006391:Hemangioma / Q000453:epidemiology / Q000628:therapy; D006801:Humans; D007223:Infant; D007564:Japan / Q000453:epidemiology; D008113:Liver Neoplasms / Q000453:epidemiology / Q000628:therapy; D008297:Male
keywords: ISSVA classification; coagulopathy; high-output cardiac failure; infantile hepatic hemangioma