Treating Dandy-Walker syndrome–related hydrocephalus (DWSH) involves either a CSF shunt-based or endoscopic third ventriculostomy (ETV)–based procedure. However, comparative investigations are lacking. This study aimed to compare shunt-based and ETV-based treatment strategies utilizing archival data from the Hydrocephalus Clinical Research Network (HCRN) registry.

A retrospective review of prospectively collected and maintained data on children with DWSH, available from the HCRN registry (14 sites, 2008–2018), was performed. The primary outcome was revision-free survival of the initial surgical intervention. The primary exposure was either shunt-based (i.e., cystoperitoneal shunt [CPS], ventriculoperitoneal shunt [VPS], and/or dual-compartment) or ETV-based (i.e., ETV alone or with choroid plexus cauterization [CPC]) initial surgical treatment. Primary analysis included multivariable Cox proportional hazards models.

Of 8400 HCRN patients, 151 (1.8%) had DWSH. Among these, the 102 patients who underwent shunt placement (79 VPSs, 16 CPSs, 3 other, and 4 multiple proximal catheter) were younger (6.6 vs 18.8 months, p < 0.001) and more frequently had 1 or more comorbidities (37.3% vs 14.3%, p = 0.005) than the 49 ETV-treated children (28 ETV-CPC). Fifty percent of the shunt-based and 51% of the ETV-based treatments failed. Notably, 100% (4/4) of the dual-compartment shunts failed. Adjusting for age, baseline ventricular size, and comorbidities, ETV-based treatment was not significantly associated with earlier failure compared with shunt-based treatment (HR for failure 1.32, 95% CI 0.77–2.26; p = 0.321). Complication rates were low: 4.9% and 6.1% (p = 0.715) for shunt- and ETV-based procedures, respectively. There was no difference in survival between ETV-CPC– and ETV-based treatment when adjusting for age (HR for failure 0.86, 95% CI 0.29–2.55, p = 0.783).

In this North American, multicenter, prospective database review, shunt-based and ETV-based primary treatment strategies of DWSH appear similarly durable. Pediatric neurosurgeons can reasonably consider ETV-based initial treatment given the similar durability and the low complication rate. However, given the observational nature of this study, the treating surgeon might need to consider subgroups that were too small for a separate analysis. Very young children with comorbidities were more commonly treated with shunts, and older children with fewer comorbidities were offered ETV-based treatment. Future studies may determine preoperative characteristics associated with ETV treatment success in this population.

治疗 Dandy-Walker 综合征相关脑积水 (DWSH) 包括基于 CSF 分流术或基于内窥镜第三脑室造口术 (ETV) 的手术。但是，缺乏比较研究。本研究旨在利用脑积水临床研究网络 (HCRN) 登记处的档案数据，比较基于分流和 ETV 的治疗策略。

对从 HCRN 登记处（14 个地点，2008-2018 年）获得的 DWSH 儿童的前瞻性收集和维护数据进行了回顾性审查。主要结果是初始手术干预的无翻修生存率。主要暴露是基于分流（即，膀胱腹膜分流 [CPS]、脑室腹腔分流 [VPS] 和/或双室）或基于 ETV（即单独 ETV 或脉络丛烧灼术 [CPC]）的初始手术治疗。主要分析包括多变量 Cox 比例风险模型。

在 8400 名 HCRN 患者中，151 名 (1.8%) 患有 DWSH。其中，102 名接受分流术的患者（79 名 VPS、16 名 CPS、3 名其他和 4 名多路近端导管）更年轻（6.6 个月 vs 18.8 个月，p < 0.001），并且更频繁地有 1 个或更多的合并症（37.3% vs 14.3%，p = 0.005）比 49 名接受 ETV 治疗的儿童（28 名 ETV-CPC）。50% 的基于分流的治疗和 51% 的 ETV 治疗失败。值得注意的是，100% (4/4) 的双室分流器失败。调整年龄、基线心室大小和合并症后，与基于分流的治疗相比，基于 ETV 的治疗与早期失败无显着相关性（失败的 HR 1.32，95% CI 0.77–2.26；p = 0.321）。并发症发生率低：基于分流和 ETV 的手术分别为 4.9% 和 6.1%（p = 0.715）。

在这项北美、多中心、前瞻性数据库审查中，基于分流和基于 ETV 的 DWSH 主要治疗策略似乎同样持久。鉴于类似的耐久性和低并发症发生率，小儿神经外科医生可以合理地考虑基于 ETV 的初始治疗。然而，鉴于本研究的观察性质，主治外科医生可能需要考虑太小而无法单独分析的亚组。患有合并症的非常年幼的儿童更常接受分流术治疗，而合并症较少的年龄较大的儿童则接受基于 ETV 的治疗。未来的研究可能会确定与该人群 ETV 治疗成功相关的术前特征。