Abstract

BackgroundLongstanding overt ventriculomegaly in adults (LOVA) is characterised by chronic hydrocephalus presumed to begin during infancy, but arresting before becoming clinically detectable. Later in life clinical features of hydrocephalus ensue, typically in the 5th or 6th decades. Only a relatively small number of LOVA case series have been published, and ambiguity remains regarding optimal management. This case series describes a series of patients with LOVA treated successfully at a single neurosurgical institution using endoscopic third ventriculostomy (ETV).MethodsA series of 14 patients were diagnosed with LOVA using established clinical and radiological criteria. All patients underwent an ETV and their clinical conditions were followed up for up to 5 years post-operatively.ResultsFourteen patients (100 %) reported either improvement or halt of progression in their presenting symptoms 3 months after ETV; 93 % of patients (n = 13) did not require any further surgical intervention. One patient (7 %) reported deterioration in symptoms beyond 3 months post-operatively, which necessitated further surgery (ventriculoperitoneal shunt). These promising outcomes after ETV are mirrored in numerous other LOVA case series. Other works have analysed the value of CSF shunting procedures in LOVA, with mixed results. A direct, prospective comparison of outcomes after shunt procedures and ETV, with a specific focus on LOVA patients, is yet to be completed. A minority of patients fail to respond, or develop recurrence of symptoms, months or years after initial surgical intervention.ConclusionsETV is an attractive option for surgical treatment of LOVA. After surgical treatment for LOVA, long-term follow-up should be considered to screen for late recurrence of the condition.

Highlights

  • endoscopic third ventriculostomy (ETV) is an attractive option for surgical treatment of Longstanding overt ventriculomegaly in adults (LOVA)

  • After surgical treatment for LOVA, long-term follow-up should be considered to screen for late recurrence of the condition

  • The term BLOVA^ was first used by Oi and colleagues [1] to define a cohort of adult patients with symptoms of chronic hydrocephalus, a head circumference of more than 2 standard deviations above the 98th percentile and overt triventriculomegaly on neuroimaging, in the absence of a secondary cause for aqueductal stenosis in adulthood

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Summary

Introduction

The term BLOVA^ was first used by Oi and colleagues [1] to define a cohort of adult patients with symptoms of chronic hydrocephalus, a head circumference of more than 2 standard deviations above the 98th percentile and overt triventriculomegaly on neuroimaging, in the absence of a secondary cause for aqueductal stenosis in adulthood The mechanism for this phenomenon remains unclear [2]. It is hypothesised that there is a full or partial obstruction of CSF flow through the aqueduct of Sylvius before fusion of cranial sutures (i.e. before age 24 months) followed by restoration of CSF flow before clinical symptoms can manifest in childhood This theory explains the large head circumference and initial asymptomatic period of patients with true LOVA. This case series collates outcome data from LOVA patients treated using endoscopic third ventriculostomy (ETV) at our institution and reviews current data published about the proposed pathophysiology, diagnosis, treatment options and patient outcomes for LOVA

Methods
Results
Discussion
SDH—one necessitating operative drainage
F Headaches
Compliance with ethical standards
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