Daneman A, Grattan-Smith P
Pediatr Radiol. 1976 Apr 14;4(3):161-6. doi: 10.1007/BF00975350.
Three children with neurofibromatosis involving the lower urinary tract are reported and their clinical, radiological and pathological findings are described. Lower urinary tract involvement in neurofibromatosis has previously been reported in 17 children, 12 of whom had other stigmata of von Recklinghausens disease. Lower urinary tract involvement may be asymptomatic and can be found incidentally. Every case with neurofibromatosis presenting with what may appear to be even only insignificant urinary symptoms should be thoroughly investigated urologically. Symptoms are usually related to urinary tract obstruction or neurogenic bladder dysfunction due to the involvement of the nerves supplying the bladder. Urinary diversion is the treatment of choice as surgical removal of the extensive tumour is seldom feasible. Following treatment the prognosis for survival appears to be good.
报告了3例患有累及下尿路的神经纤维瘤病的儿童，并描述了他们的临床、放射学和病理学发现。先前已有17例儿童被报道患有累及下尿路的神经纤维瘤病，其中12例有冯·雷克林豪森病的其他体征。下尿路受累可能无症状，可偶然发现。每例患有神经纤维瘤病且出现哪怕看似微不足道的泌尿系统症状的患者，都应接受全面的泌尿外科检查。症状通常与尿路梗阻或由于供应膀胱的神经受累导致的神经源性膀胱功能障碍有关。尿流改道是首选的治疗方法，因为很少能够通过手术切除广泛的肿瘤。治疗后生存预后似乎良好。
