Spadaro A, Ambrosio D, Moraci A, Conforti R, Albanese V
J Neurosurg Sci. 1986 Jan-Jun;30(1-2):87-93.
The Authors report three cases of non tumoral aqueductal stenosis associated with von Recklinghausen disease in children. Moreover, 16 similar cases collected from the literature are illustrated. The clinical features are evaluated in light of literature's data. Among all 19 cases, the median age was 19 years (range 6-46 years) and 9 patients were under the age of 13 years. In this latter group, the most represented clinical symptoms were headache and gait disturbances. As regards the neuroradiological procedures, CT scan represented the best tool in the evaluation of the ventricular size. Our own three cases underwent to ventriculo-atrial shunt procedures, followed by clinical recovery (follow-up: 3 months-5 years). In conclusion it is felt that, among all the pathological events usually described in patients affected by neurofibromatosis, aqueductal stenosis seems to belong to the natural history of this disease.
作者报告了3例儿童中与冯·雷克林豪森病相关的非肿瘤性导水管狭窄病例。此外，还展示了从文献中收集的16例类似病例。根据文献数据对临床特征进行了评估。在所有19例病例中，中位年龄为19岁（范围6 - 46岁），9例患者年龄在13岁以下。在后一组中，最常见的临床症状是头痛和步态障碍。关于神经放射学检查方法，CT扫描是评估脑室大小的最佳工具。我们自己的3例病例接受了脑室 - 心房分流手术，随后临床症状缓解（随访：3个月至5年）。总之，人们认为，在神经纤维瘤病患者通常描述的所有病理事件中，导水管狭窄似乎属于这种疾病的自然病程。
