Rosa Magno Rocha Freitas, Cruz Thainá Zanon, Magalhães Junior Eduardo Vasconcelos, Nigri Flavio
Department of Surgical Specialties and Neurosurgery, Pedro Ernesto University Hospital, Rio de Janeiro, Brazil.
Surg Neurol Int. 2021 Oct 19;12:519. doi: 10.25259/SNI_635_2021. eCollection 2021.
Tetraventricular hydrocephalus is a common presentation of communicating hydrocephalus. Conversely, cases with noncommunicating etiology impose a diagnostic challenge and are often neglected and underdiagnosed. Herein, we present a review of literature for clinical, diagnostic, and surgical aspects regarding noncommunicating tetrahydrocephalus caused by primary fourth ventricle outlet obstruction (FVOO), illustrating with a case from our service.
We performed a research on PubMed database crossing the terms "FVOO," "tetraventriculomegaly," and "hydrocephalus" in English. Fifteen articles (a total of 34 cases of primary FVOO) matched our criteria and were, therefore, included in this study besides our own case.
Most cases presented in adulthood (47%), equally divided between male and female. Clinical presentation was unspecific, commonly including headache, nausea, and dizziness as symptoms (35.29%, 21.57%, and 9.80%, respectively), with ataxic gait (65%) and papilledema (40%) being the most frequent signs. MRI and CT were the imaging modalities of choice (11 patients each), often associated with CSF flow studies, such as cine MRI and CT ventriculogram. Endoscopic third ventriculostomy (ETV) was both the most popular and effective surgical approach (50.85% of cases, with 18.91% of recurrence) followed by ventricle-peritoneal shunt (16.95% of patients, 23.0% of recurrence).
FVOO stands for a poorly understood etiology of noncommunicating tetrahydrocephalus. With the use of ETV, these cases, once hopeless, had its morbimortality and recurrence reduced greatly. Therefore, its suspicion and differentiation from other forms of tetrahydrocephalus can improve its natural course, reinforcing the importance of its acknowledgment.
四脑室脑积水是交通性脑积水的常见表现。相反，非交通性病因的病例带来诊断挑战，常被忽视和漏诊。在此，我们对由原发性第四脑室出口梗阻（FVOO）引起的非交通性四脑室脑积水的临床、诊断和手术方面进行文献综述，并结合我们科室的一个病例进行说明。
我们在PubMed数据库中用英文交叉检索了“FVOO”“四脑室扩大”和“脑积水”等术语。除我们自己的病例外，有15篇文章（共34例原发性FVOO）符合我们的标准，因此被纳入本研究。
大多数病例在成年期出现（47%），男女比例相等。临床表现无特异性，常见症状包括头痛、恶心和头晕（分别为35.29%、21.57%和9.80%），共济失调步态（65%）和视乳头水肿（40%）是最常见的体征。MRI和CT是首选的影像学检查方法（各11例患者），常结合脑脊液流动研究，如电影MRI和CT脑室造影。内镜下第三脑室造瘘术（ETV）是最常用且有效的手术方法（50.85%的病例，复发率为18.91%），其次是脑室-腹腔分流术（16.95%的患者，复发率为23.0%）。
FVOO是一种病因尚不清楚的非交通性四脑室脑积水。通过使用ETV，这些曾经无望的病例的病死率和复发率大大降低。因此，对其进行怀疑并与其他形式的四脑室脑积水进行鉴别可以改善其自然病程，强化认识它的重要性。
