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.
Keywords: ISSVA classification; coagulopathy; high-output cardiac failure; infantile hepatic hemangioma.
© 2014 Japan Pediatric Society.