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
PY - 2011/12/1
Y1 - 2011/12/1
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
VL - 46
SP - 2239
EP - 2243
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 12
ER -