Forbes K A
J Urol. 1979 Jan;121(1):100-2. doi: 10.1016/s0022-5347(17)56680-x.
Neurofibromatosis is a neural disease of hereditary nature affecting both sexes of all races. Visceral and central nervous system involvement can cause serious interference with normal function of affected structures. Under these circumstances, lifelong observation and individualized treatment of the patient are essential to proper management. This report is an account of nearly 2 decades of conservative management of neurofibromatosis of the pelvis in a young woman. Progressive, bilateral ureteral obstruction developed but normal function of the urinary tract has been maintained for the last 10 years with a sigmoid conduit cystoplasty. It is anticipated that continued progression of the disease will require cutaneous transfer of the sigmoid conduit. Also, a colostomy might become necessary because of recent evidence of rectal obstruction noted on computed tomography.
神经纤维瘤病是一种具有遗传性质的神经疾病，影响所有种族的男女。内脏和中枢神经系统受累可严重干扰受影响结构的正常功能。在这种情况下，对患者进行终身观察和个体化治疗对于妥善管理至关重要。本报告讲述了一名年轻女性骨盆神经纤维瘤病近20年的保守治疗情况。出现了进行性双侧输尿管梗阻，但通过乙状结肠导管膀胱成形术，尿路在过去10年一直保持正常功能。预计疾病的持续进展将需要乙状结肠导管的皮肤转移。此外，由于最近计算机断层扫描显示有直肠梗阻的迹象，可能需要进行结肠造口术。
