Pediatrics international : official journal of the Japan Pediatric Society
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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. ⋯ 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.