Takeuchi Youhei, Kimiwada Tomomi, Shirane Reizo, Tominaga Teiji
Department of Neurosurgery, Miyagi Children's Hospital, Sendai, Miyagi, Japan.
Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.
NMC Case Rep J. 2020 Jun 26;7(3):141-145. doi: 10.2176/nmccrj.cr.2019-0263. eCollection 2020 Jul.
The pathophysiology and optimal treatment for hydrocephalus with Blake's pouch cyst (BPC) remain controversial. The authors present two pediatric cases of hydrocephalus associated with BPC, in which the patients' hydrocephalus progressed after endoscopic third ventriculostomy (ETV), despite a patent stoma of the third ventricular floor. Case 1: A 4-year-old girl with delayed gait development was diagnosed with BPC-associated hydrocephalus and received ETV. Postoperatively, the patient presented headaches and nausea. Computed tomography (CT) scans demonstrated larger ventricles than those observed on the preoperative images. Because phase-contrast cine magnetic resonance imaging (MRI) and constructive interference in steady state (CISS) MRI revealed patent cerebrospinal fluid (CSF) flow at the third ventricular floor level, a ventriculoperitoneal shunt (VPS) was placed using a programmable pressure valve to treat the hydrocephalus. Case 2: A 6-year-old girl with newly developed repeated convulsive seizures was diagnosed with BPC-associated hydrocephalus and received ETV. Phase-contrast cine MRI on the 5th postoperative day showed hyperdynamic CSF flow at the third ventricular floor level. She also developed vomiting and headache 6 weeks after ETV. CT scans demonstrated much larger tetraventricular hydrocephalus than that observed on the preoperative images. VPS placement improved her hydrocephalus. Referencing the previous literature, we discuss the CSF dynamics and the mechanism of BPC-associated hydrocephalus, focusing on the third ventricular floor bulging. We hope our experience will help elucidate the pathophysiology and treatment strategies for BPC-associated hydrocephalus.
伴Blake囊肿（BPC）的脑积水的病理生理学及最佳治疗方法仍存在争议。作者介绍了两例与BPC相关的小儿脑积水病例，尽管第三脑室底部造口通畅，但在内镜下第三脑室造瘘术（ETV）后，患者的脑积水仍有进展。病例1：一名4岁步态发育迟缓的女孩被诊断为BPC相关性脑积水并接受了ETV。术后，患者出现头痛和恶心。计算机断层扫描（CT）显示脑室比术前图像所见更大。由于相位对比电影磁共振成像（MRI）和稳态构成干扰（CISS）MRI显示第三脑室底部水平脑脊液（CSF）流动通畅，遂使用可编程压力阀置入脑室腹腔分流术（VPS）来治疗脑积水。病例2：一名6岁新出现反复惊厥发作的女孩被诊断为BPC相关性脑积水并接受了ETV。术后第5天的相位对比电影MRI显示第三脑室底部水平CSF流动活跃。ETV术后6周，她也出现了呕吐和头痛。CT扫描显示四脑室脑积水比术前图像所见大得多。VPS置入改善了她的脑积水。参考既往文献，我们讨论了CSF动力学及BPC相关性脑积水的机制，重点关注第三脑室底部膨出。我们希望我们的经验将有助于阐明BPC相关性脑积水的病理生理学及治疗策略。
