Pedro Oliveira João, Ramos João, Campos Mário, Calvão-Pires Pedro, Cunha Pedro
Neurosurgery, Centro Hospitalar de Lisboa Ocidental, Lisbon, PRT.
Neuroradiology, Centro Hospitalar de Lisboa Ocidental, Lisboa, PRT.
Cureus. 2024 Nov 12;16(11):e73557. doi: 10.7759/cureus.73557. eCollection 2024 Nov.
Hydrocephalus is the disruption of cerebral spinal fluid homeostasis, representing a common neurosurgical illness. Up to 10% have no identifiable cause, with fourth ventricle outflow obstruction (FVOO) being an extremely rare subtype. A 31-year-old male with a history of idiopathic hydrocephalus for over 10 years with the need for a ventriculoperitoneal shunt had shown progressively enlarged tetraventricular ventriculomegaly. There was no history of meningitis, traumatic injuries, or surgeries besides the shunt placement. Given clinical worsening with diplopia and gait impairment and imaging suggestive of shunt failure, the patient underwent surgery for a replacement. In the following days, the patient worsened and became lethargic. Bilateral frontal external ventricular drainages were placed for acute hydrocephalus due to tetraventricular haemorrhage, although ventricular enlargement kept progressing. Brain magnetic resonance imaging (MRI) showed a membrane in the Magendie foramen region with an apparently unobstructed aqueduct and no transependymal edema. The surgical team opted for a suboccipital craniectomy for membrane fenestration. Post-operative computed tomography (CT) scans showed a stark reduction of the ventricles. Within one week, the patient had an almost complete recovery and was discharged. One year later, the patient is asymptomatic with no need for acetazolamide or shunt. Hydrocephalus is a rather common neurosurgical pathology presenting serious comorbidity. It is amenable to very effective and potentially curative treatment, and therefore efforts should be made to find its cause. Here, we reported a rather challenging referral of an acute decompensation of a chronic hydrocephalus with no previous cause identified. Ultimately, in the midst of this chronically altered cerebrospinal fluid (CSF) dynamics, the use of advanced high-resolution imaging proved crucial and allowed adequate treatment for the true cause of the hydrocephalus. We hope to raise awareness of the possible existence of membranes in the foramen of Magendie and its unusual presentation.
脑积水是脑脊液稳态的破坏，是一种常见的神经外科疾病。高达10%的患者病因不明，第四脑室流出道梗阻（FVOO）是一种极其罕见的亚型。一名31岁男性，有特发性脑积水病史超过10年，需要进行脑室腹腔分流术，其第四脑室脑室扩大呈进行性加重。除了分流术外，无脑膜炎、外伤或手术史。鉴于出现复视和步态障碍导致临床症状恶化，且影像学提示分流失败，患者接受了分流管置换手术。在接下来的几天里，患者病情恶化，变得嗜睡。因第四脑室出血导致急性脑积水，放置了双侧额部外引流管，尽管脑室仍在继续扩大。脑部磁共振成像（MRI）显示马让迪孔区域有一层膜，导水管明显通畅，无室管膜下水肿。手术团队选择枕下颅骨切除术进行膜开窗。术后计算机断层扫描（CT）显示脑室明显缩小。一周内，患者几乎完全康复并出院。一年后，患者无症状，无需使用乙酰唑胺或分流管。脑积水是一种相当常见的神经外科疾病，伴有严重的合并症。它适合非常有效的潜在治愈性治疗，因此应努力寻找其病因。在此，我们报告了一例具有挑战性的转诊病例，一名慢性脑积水急性失代偿患者，此前未发现病因。最终，在这种长期改变的脑脊液（CSF）动力学情况下，使用先进的高分辨率成像被证明至关重要，并能针对脑积水的真正病因进行充分治疗。我们希望提高对马让迪孔膜可能存在及其不寻常表现的认识。
