Shimizu T, Yamazaki Y, Tomoe H, Nishino S, Toma H, Shibata T, Kobayashi M
Department of Urology, Tokyo Women's Medical College.
Nihon Hinyokika Gakkai Zasshi. 1998 Oct;89(10):846-9. doi: 10.5980/jpnjurol1989.89.846.
A 51-year-old male of von Recklinghausen's disease presented with huge retroperitoneal hematoma caused by spontaneous arterial rupture. He died of uncontrollable hemorrhage during the operation. Gross appearance of aorta, renal artery, celiac artery, pulmonary artery and lumbar artery were extremely fragile, and there was a complete rupture between aorta and lumbar artery. On the microscopic examination, significant medial dysplasia were demonstrated in these arteries. Arterial stenosis is well-known vascular complication in von Recklinghausen's disease. Spontaneous rupture of major artery, however, has been extremely uncommon. This is the 26th case reported in the world literature, and 21th case in the Japanese literature.
一名患有冯·雷克林霍增氏病的51岁男性，因自发性动脉破裂出现巨大的腹膜后血肿。他在手术过程中死于无法控制的出血。主动脉、肾动脉、腹腔动脉、肺动脉和腰动脉的大体外观极其脆弱，主动脉和腰动脉之间完全破裂。显微镜检查显示，这些动脉存在明显的中膜发育异常。动脉狭窄是冯·雷克林霍增氏病中众所周知的血管并发症。然而，大动脉的自发性破裂极为罕见。这是世界文献中报道的第26例，也是日本文献中的第21例。
