Mulhem Ali, Alsulaiman Abdul Masih, Hammersen Stefanie, Kantelhardt Sven
Department of Neurosurgery Vivantes Klinikum im Friedrichshain Berlin Germany.
DPhil Program in Evidence-Based Health Care, Department of Continuing Education University of Oxford Oxford UK.
Clin Case Rep. 2023 Nov 28;11(12):e8234. doi: 10.1002/ccr3.8234. eCollection 2023 Dec.
Fourth ventricle outlet obstruction (FVOO) is a rare cause of hydrocephalus. In the last century, the standard treatment was the suboccipital craniotomy with magendieplasty or ventriculoperitoneal shunt (VP shunt). Since the beginning of the 21st century, the endoscopic third ventriculostomy (ETV) has been considered a less invasive alternative. The medical literature lacks sufficient reports of FVOO cases and strong evidence about ETV's efficacy in treating this condition. We report two cases of FVOO treated with ETV and review published similar cases. Clinical and radiological findings of two FVOO cases with outcomes after ETV were presented. Moreover, we conducted a systematic review after protocol registration in PROSPERO (CRD42021281474). MEDLINE, Embase, Scopus, and Web of Science were searched from inception till December 31, 2022. Studies were included if they reported cases of FVOO treated initially with ETV. Cases with Chiari malformation, Dandy-Walker malformation, tuberous sclerosis, trapped fourth ventricle, or space-occupying lesions were excluded. Two reviewers independently examined title/abstract records in the first stage and full-text publications in the second for eligibility. The primary outcome was the recurrence rate, defined by the need for re-ETV or other invasive treatments (e.g., VP shunt or magendieplasty). Other outcomes included clinical state at follow-up and mortality. Two cases, a 3-year-old male and 3.5-year-old female, with FVOO, were treated with ETV in our department by the same neurosurgeon (SH) in 2013 and 2021. Both cases improved significantly after ETV, and there was no recurrence through the follow-up. Besides the present cases, we found 57 other cases of FVOO treated with ETV reported in 17 studies between 2001 and 2021. The median age was 26 years, with an IQR from 2.4 to 59 years, and 56% of cases were females. The recurrence rate was 32% in the sample (19 out of 59), with a 95% CI from 21% to 46%. The median time to recure was 2 months with IQR from 1.25 to 26. A VP shunt was the treatment for recurrence in 68% and a re-ETV in 32%. At the follow-up (41 ± 29 months), only one case died, and one deteriorated clinically. FVOO is a rare cause of hydrocephalus encountered mainly in the first or sixth decades of life. ETV provides the first reasonable treatment. Despite the moderate recurrence rate, the outcomes are favorable.
第四脑室出口梗阻（FVOO）是脑积水的一种罕见病因。在上个世纪，标准治疗方法是枕下开颅术联合马让迪孔成形术或脑室腹腔分流术（VP分流术）。自21世纪初以来，内镜下第三脑室造瘘术（ETV）被认为是一种侵入性较小的替代方法。医学文献中缺乏足够的FVOO病例报告以及关于ETV治疗这种疾病疗效的有力证据。我们报告两例接受ETV治疗的FVOO病例，并回顾已发表的类似病例。介绍了两例FVOO病例的临床和影像学检查结果以及ETV治疗后的结局。此外，我们在PROSPERO（CRD42021281474）进行方案注册后进行了系统评价。检索了MEDLINE、Embase、Scopus和Web of Science数据库，检索时间从建库至2022年12月31日。纳入的研究需报告最初采用ETV治疗的FVOO病例。排除患有Chiari畸形、Dandy-Walker畸形、结节性硬化症、被困第四脑室或占位性病变的病例。两名研究者在第一阶段独立检查标题/摘要记录，在第二阶段独立检查全文出版物以确定是否符合纳入标准。主要结局是复发率，定义为是否需要再次进行ETV或其他侵入性治疗（如VP分流术或马让迪孔成形术）。其他结局包括随访时的临床状态和死亡率。2013年和2021年，我们科室的同一位神经外科医生（SH）对两例FVOO患者（一名3岁男性和一名3.5岁女性）进行了ETV治疗。两例患者在ETV治疗后均有显著改善，随访期间均未复发。除了本病例外，我们还在2001年至2021年期间的17项研究中发现了另外57例接受ETV治疗的FVOO病例。中位年龄为26岁，四分位间距为2.4至59岁，56%的病例为女性。样本中的复发率为32%（59例中有19例），95%置信区间为21%至46%。复发的中位时间为2个月，四分位间距为1.25至26个月。68%的复发患者接受了VP分流术治疗，32%接受了再次ETV治疗。在随访（41±29个月）时，只有1例死亡，1例临床症状恶化。FVOO是脑积水的一种罕见病因，主要发生在生命的第一个或第六个十年。ETV是首选的合理治疗方法。尽管复发率中等，但结局良好。
