Tetraventricular hydrocephalus with aqueductal flow void: an overlooked entity having consistent improvement following endoscopic third ventriculostomy.

Background: Tetraventricular Hydrocephalus (TetHCP) is a heterogeneous group of cerebrospinal fluid (CSF) flow disorders having varying success rates with Endoscopic third ventriculostomy (ETV). This is report on the efficacy and rationale of ETV in a specific subset of primary TetHCP with aqueductal CSF flow voids.

Methods: Patients of primary acquired TetHCP presenting with increasing head size and/or headache having aqueductal CSF flow void on sagittal Magnetic Resonance Imaging (MRI) were included in this study. All of them underwent ETV. All patients were evaluated for clinical improvement & MRI at 3 months, and need for any additional procedure, in contrast to those without CSF flow void. The pathophysiology of hyperdynamic CSF circulation and its correlation to ETV was further reviewed.

Results: Eleven patients had tetraventricular hydrocephalus and aqueductal flow void, with age ranging from 10 months to 59 years. Two patients who could undergo quantitative flow study confirmed the hyperdynamic flow across the aqueduct. Following ETV, all showed clinical improvement. MRI at 3 months showed CSF flow void across the third ventricular stoma in addition to across the aqueduct. None of these patients required any redo procedures for a mean follow-up of 39.2 months. In contrast, there was 30% failure rate after ETV among 10 patients of tetraventricular hydrocephalus without aqueductal flow void.

Conclusion: Tetraventricular hydrocephalus with aqueductal CSF flow void may be a unique entity with hyperdynamic CSF circulation and relative resistance at fourth ventricular outlets. ETV is highly efficacious in these patients, resulting in consistent clinico-radiological improvement.