TY - JOUR
T1 - Critical infantile hepatic hemangioma
T2 - Results of a nationwide survey by the Japanese Infantile Hepatic Hemangioma Study Group
AU - Kuroda, Tatsuo
AU - Kumagai, Masaaki
AU - Nosaka, Shunsuke
AU - Nakazawa, Atsuko
AU - Takimoto, Tetsuya
AU - Hoshino, Ken
N1 - Funding Information:
This study received a grant from the Rare Diseases Study Project, Ministry of Health and Labor, Japan (No. H22-Rare diseases-general-153). The authors deeply thank the following pediatric surgical institutions for cooperation for the current study: Iwate University, Niigata University, Gunma Children's Hospital, Jichi University, Nihon University, Kitasato University, Osaka University, Tokushima University, Kochi Medical Center, National Center for Child Health and Development, and Keio University.
PY - 2011/12
Y1 - 2011/12
N2 - Background: The current survey aimed to describe the clinical features of critical infantile hepatic hemangioma (IHH) and the implications of recent treatments. Materials and 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). Conclusion: For critical IHH cases with steroid-insensitive hematologic disorders, alternative treatments including β-blocker therapy, surgery, and liver transplantation should be considered.
AB - Background: The current survey aimed to describe the clinical features of critical infantile hepatic hemangioma (IHH) and the implications of recent treatments. Materials and 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). Conclusion: For critical IHH cases with steroid-insensitive hematologic disorders, alternative treatments including β-blocker therapy, surgery, and liver transplantation should be considered.
KW - Hemangioendothelioma
KW - Infantile hepatic hemangioma
KW - Liver transplantation
KW - β-blocker
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U2 - 10.1016/j.jpedsurg.2011.09.007
DO - 10.1016/j.jpedsurg.2011.09.007
M3 - Article
C2 - 22152857
AN - SCOPUS:83455187951
SN - 0022-3468
VL - 46
SP - 2239
EP - 2243
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
IS - 12
ER -