title: Critical infantile hepatic hemangioma: results of a nationwide survey by the Japanese Infantile Hepatic Hemangioma Study Group.
abstract: BACKGROUND
  The current survey aimed to describe the clinical features of critical infantile hepatic hemangioma (IHH) and the implications of recent treatments.
  
  
  METHODS
  A nationwide survey of critical IHH patients treated between 2005 and 2010 was performed in all 117 registered pediatric surgical hospitals in Japan. As a result, 19 patients were identified and reviewed using a statistical analysis.
  
  
  RESULTS
  Abdominal distention (47.4%), high-output cardiac failure (47.4%), coagulopathy (42.1%), and respiratory distress (31.6%) were the major symptoms. Three patients died (1 of coagulopathy, 1 of cardiac failure, and 1 of both). An accompanying portovenous shunt was also highlighted. Infantile hepatic hemangioma was totally insensitive to steroid treatment in 3 (23.1%) of the 13 patients, and 9 (47.4%) of the 19 patients required other treatments. Surgical resection and β-blocker improved the hematologic data, whereas hepatic arterial ligation and embolization seemed to produce a limited effect. Among the dead patients, several hematologic parameters were significantly worse: the thrombocyte count (pretherapeutic: 73,000 vs 300,000/mm(3), dead vs survivor, respectively [P < .03]; posttherapeutic: 66,000 vs 388,700/mm(3) [P < .003]) and the prothrombin time (posttherapeutic, 35.0 vs 12.1 seconds [P < .0001], dead vs survivor, respectively).
  
  
  CONCLUSIONS
  For critical IHH cases with steroid-insensitive hematologic disorders, alternative treatments including β-blocker therapy, surgery, and liver transplantation should be considered.
authors.affiliation: Department of Surgery, National Center for Child Health and Development, Tokyo, Japan. kuroda-t@z8.keio.jp
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Kuroda
authors.forename: Masaaki
authors.initials: M
authors.lastname: Kumagai
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Nosaka
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Takimoto
authors.forename: Ken
authors.initials: K
authors.lastname: Hoshino
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001778:Blood Coagulation Disorders / Q000209:etiology; D016534:Cardiac Output, High / Q000209:etiology; D016638:Critical Illness; D019008:Drug Resistance, Neoplasm; D004621:Embolization, Therapeutic; D006390:Hemangioendothelioma / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology / Q000601:surgery; D006391:Hemangioma / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology* / Q000601:surgery; D006392:Hemangioma, Cavernous / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology / Q000601:surgery; D006498:Hepatectomy; D006499:Hepatic Artery; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008026:Ligation; D017093:Liver Failure / Q000209:etiology; D008113:Liver Neoplasms / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology* / Q000601:surgery; D016031:Liver Transplantation; D011239:Prednisolone / Q000627:therapeutic use; D012131:Respiratory Insufficiency / Q000209:etiology
chemical_list: D000319:Adrenergic beta-Antagonists; D000970:Antineoplastic Agents; D011239:Prednisolone