Department of Neurological Surgery, Children's Hospital of Pittsburgh, Magee Women's Hospital, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.
Department of Neurological Surgery, Allegheny Health Network, Pittsburgh, Pennsylvania, USA.
World Neurosurg. 2018 Jun;114:e976-e981. doi: 10.1016/j.wneu.2018.03.123. Epub 2018 Mar 26.
Hydrocephalus due to congenital aqueductal stenosis (CAS) has significant long-term clinical implications. Previous reports on outcomes after treatment of congenital hydrocephalus are heterogenous and lack specificity for the subgroup of patients with isolated aqueductal stenosis.
An institutional surgical database was queried for the time period of 2005-2013 for patients with the diagnosis of isolated CAS and >2 years of clinical follow-up. Medical history and neurodevelopmental outcomes were recorded.
The institutional cohort consisted of 41 patients with isolated CAS who underwent cerebrospinal fluid diversion. At a mean follow-up of 5.9 years, 48.8% carried a diagnosis of epilepsy and 68% were developmentally delayed. Four patients were diagnosed with cerebral palsy (9.8%). In total, 78% of patients were shunt-dependent, and the remainder had patent third ventriculostomies. Only 32% of patients in our cohort were neurologically normal after long-term follow-up despite contemporary management.
Regardless of the initial treatment strategy, the age at diagnosis, or the timing of cerebrospinal fluid diversion after birth, patients with aqueductal stenosis have high rates of epilepsy, neurodevelopmental delay, and educational difficulties, and few are neurologically normal despite contemporary management. Investigation into in utero identification and correction of hydrocephalus may result in improved outcomes and warrants further investigation.
由先天性导水管狭窄（CAS）引起的脑积水具有显著的长期临床意义。既往关于先天性脑积水治疗后结局的报道存在异质性，且缺乏针对单纯性导水管狭窄患者亚组的特异性。
在 2005 年至 2013 年期间，通过机构手术数据库对诊断为单纯性 CAS 且具有 >2 年临床随访的患者进行查询。记录患者的病史和神经发育结局。
机构队列包括 41 例接受脑脊液分流术的单纯性 CAS 患者。平均随访 5.9 年后，48.8%的患者被诊断为癫痫，68%的患者存在发育迟缓。4 例患者被诊断为脑瘫（9.8%）。共有 78%的患者需要依赖分流管，其余患者存在第三脑室造瘘术通畅。尽管采用了现代治疗方法，但在长期随访中，仍有 78%的患者存在神经功能异常。
无论初始治疗策略、诊断时的年龄或出生后脑脊液分流的时间如何，导水管狭窄患者的癫痫、神经发育迟缓以及教育困难发生率均较高，且尽管采用了现代治疗方法，仍很少有患者的神经功能正常。对胎儿期脑积水的识别和纠正的研究可能会带来更好的结局，值得进一步研究。
