von Recklinghausen病に合併した肋間動脈瘤破裂の1例

Rupture of Intercostal Aneurysm Associated with von Recklinghausen’s Disease：Report of a Case

はじめに　von Recklinghausen病（神経線維腫症1型）は約3,000人に1人の割合で生じる常染色顕性の遺伝性疾患で，本邦の患者数は約40,000人と推定されている1）．症候としては皮膚のカフェオレ斑や神経線維腫がよく知られているが，まれに動脈瘤，血管狭窄，動静脈奇形といった血管病変を合併することがある2，3）．われわれはvon Recklinghausen病に合併した肋間動脈瘤破裂に対して経皮的コイル塞栓術を施行した1例を経験したので報告する．

Vascular lesions, including aneurysms, are rare but well-documented complications of von Recklinghausen’s disease. Although aneurysms associated with this condition are often asymptomatic, rupture can lead to life-threatening events such as hemothorax. We report a 63-year-old male with von Recklinghausen’s disease who presented with sudden onset of chest pain. Imaging revealed left-side hemothorax, and contrast-enhanced computed tomography （CT） suggested rupture of the left 10th intercostal artery. Given his hemodynamic stability, percutaneous coil embolization was performed. Angiography revealed two adjacent aneurysms at the origin of the left 10th intercostal artery, both of which were successfully embolized. Postoperative course was uneventful, and the patient was discharged on postoperative day 6. This case highlights the importance of considering vascular complications in von Recklinghausen’s disease and supports the efficacy and safety of coil embolization in managing ruptured intercostal artery aneurysms.