Progressive Hydrocephalus after Endoscopic Third Ventriculostomy in Pediatric Patients with Blake’s Pouch Cyst

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.