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Abstract：
Background Although idiopathic aqueductal stenosis is a congenital disorder, some patients present in adulthood. Many theories have tried to account for the late-onset presentation; however, the aetiology remains uncertain. This study aimed to investigate the clinical presentation, management, and outcomes of patients with late-onset idiopathic aqueductal stenosis (LIAS) managed at our centre. Methodology A retrospective study of patients with a diagnosis of LIAS managed at our centre between 1996 and 2018 was undertaken. Data on clinical presentation, imaging, management, and outcomes were retrieved from patient records and radiology reports. Results A total of 20 patients were diagnosed with LIAS during the study period. Endoscopic third ventriculostomy (ETV) was the initial modality of treatment for nine patients, ventriculoperitoneal shunt (VPS) for four patients, and conservative management in seven patients, in four of them intracranial pressure (ICP) was found to be normal following a period of ICP monitoring. The median follow-up period was three years (1 month to 24 years). One patient was lost to follow-up. One ETV failed in the first six months necessitating VPS insertion. Two cases that were initially managed conservatively required a VPS three and nine years following the initial presentation. Of the patients undergoing VPS insertion, all subsequently required valve adjustment or surgical revision. Conclusions The majority of patients with LIAS undergoing ETV were managed successfully, whereas VPS insertion was associated with a high rate of revision surgery in this cohort. ETV should be considered as the treatment of choice to avoid the long-term complications of shunting for patients with LIAS.
摘要：
背景虽然特发性导水管狭窄是一种先天性疾病,一些患者出现在成年期。许多理论试图解释迟发性表现；然而，病因仍不确定。本研究旨在调查临床表现，管理,和在我们中心治疗的晚发性特发性导水管狭窄(LIAS)患者的结局.方法对1996年至2018年在我们中心治疗的诊断为LIAS的患者进行回顾性研究。临床表现数据，成像,管理,从患者记录和放射学报告中检索结果.结果在研究期间共有20例患者被诊断为LIAS。内镜下第三脑室造瘘术（ETV）是9例患者的初始治疗方式，四例患者的脑室-腹腔分流术(VPS),对7名患者进行保守治疗，其中4例颅内压（ICP）在经过一段时间的ICP监测后被发现正常。中位随访期为3年（1个月至24年）。一名患者失去了随访。一个ETV在前六个月失败，需要插入VPS。最初保守管理的两个案例在初次陈述后需要三年和九年的VPS。在接受VPS插入的患者中，所有随后需要的瓣膜调整或手术翻修。结论大多数接受ETV的LIAS患者得到了成功的治疗，而VPS插入与该队列中的高翻修手术率相关。ETV应被视为治疗的选择，以避免LIAS患者分流的长期并发症。
