Key result
Von Recklinghausen's disease-associated thoracic aneurysms and arterial ruptures had a mortality rate of 32.3% (8/22), the highest among all regions in a review of 57 Japanese cases.
Thoracic aneurysms associated with von Recklinghausen's disease carry a high mortality rate (32.3%), highlighting the need for prompt diagnosis and surgical intervention.
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May warrant vigilance for thoracic vascular events in von Recklinghausen's disease; leaves open optimal management strategies.
症例は58歳, 女性. 14歳頃より皮膚結節が出現し, von Recklinghausen病と診断されていた. 突然の左胸痛にて発症し, 胸部X線写真にて左胸水を指摘され, 胸腔穿刺にて血性胸水が認められた. 胸部CT, 血管造影検査によって, 左第8肋間動脈瘤が認められた. 瘤破裂による血胸と診断し, 開胸にて動脈瘤切除術を施行した. von Recklinghausen病に合併する動脈瘤及び動脈断裂の本邦報告例は自験例を含め57例であった. 胸部領域では他の領域に比べ死亡率が32.3% (8/22) と最も高かった.
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Yoshii et al. (2005) studied this question. Von Recklinghausen's disease-associated thoracic aneurysms and arterial ruptures had a mortality rate of 32.3% (8/22), the highest among all regions in a review of 57 Japanese cases.
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