顔面に巨大血腫を形成した神経線維腫症例

Neurofibromatosis with Massive Facial Hematoma

I．はじめに

　神経線維腫症は，皮膚のcafé-au-lait spotと神経線維腫を主病変に，多彩な症候を呈する全身性疾患である。本症の合併症として，骨病変や中枢性神経腫瘍の発生頻度は高いが，出血に関してはあまり知られていない。

　今回われわれは，神経線維腫症の小児において外傷により顔面，頸部に巨大な血腫を形成し，進行性の気道狭窄をきたし，気管内挿管による気道確保ののちに，動脈塞栓術と外頸動脈結紮を行い止血し得た症例を経験したので，若干の文献的考察を加えて報告する。

　We repot a rare case of massive facial hematoma by trauma in a child with neurofibromatosis. A 9-year-old male was struck his left temple and face by his friend. He had neurofibromatosis in his left temple extending to the skull base. He complained of his left facial swelling and dyspnea after this injury. MRI and echo showed intra-tumoral hemorrhage of temporal neurofibromatosis. We performed angiography and found that the bleeding was from the left external carotid artery branches. We controlled bleeding by embolization of the intra-tumoral artery and ligatation of the left external carotid artery.