von Recklinghausen病に合併した右鎖骨下動脈分枝の動脈瘤破裂による血胸

Hemothorax Due to Rupture of an Aneurysm of a Branch of the Right Subclavian Artery with von Recklinghausen’s Disease

von Recklinghausen病は皮膚にカフェ・オ・レ斑や神経線維腫を生じる遺伝性疾患であり，本邦では約40,000人の患者数と推定されている1）．動脈狭窄や動脈瘤，自然動脈破裂，動静脈瘻などの血管病変の合併は約3.6％との報告があり，まれである2）．われわれは，von Recklinghausen病に合併した右鎖骨下動脈分枝の動脈瘤破裂により血胸をきたした症例を経験したので報告する．

A 52-year-old man with a history of von Recklinghausen’s disease presented to our hospital with abrupt onset of right chest and back pain. A contrast-enhanced chest computed tomography （CT） revealed massive right hemothorax, extravasation of the contrast medium at a branch of the right subclavian artery, and a tumor surrounding the second right dorsal rib. Based on findings from emergent angiography, hemothorax secondary to the rupture of an aneurysm of a branch of the right subclavian artery was diagnosed, and transcatheter arterial embolization （TAE） was performed. After hemostasis, intrathoracic hematoma was removed by surgery, and a recurrence of hemothorax did not occur until at least 8 months after the initial TAE procedure.