Surgical treatment of long-standing overt ventriculomegaly in adults (LOVA)

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.

Longstanding overt ventriculomegaly in adults (LOVA) with patent aqueduct: surgical outcome and etiopathogenesis of a possibly distinct form of chronic hydrocephalus

“…As reported in previous studies [14,23,25,26], the clinical onset of LOVA could occur at any age of adulthood, mainly after the fourth and the fifth decades. This feature clearly reflects the "longstanding" clinical course of LOVA, starting from early childhood before the closure of cranial sutures (therefore allowing the skull to abnormally grow and resulting in macrocephaly in adult life) and insidiously passing in adulthood with late decompensation [25].…”

“…The following criteria were required to be included in the study: (1) age > 18 years; (2) clinical and radiological characteristics compatible with the diagnosis of LOVA according to Ved’s criteria (Table 1 ) [ 26 ]; (3) evidence of CSF flow through aqueduct in phase-contrast (PC)–MR sequences with turbulence void signal in T2-weighted images; (4) no previous neurosurgical procedures; (5) availability of complete preoperative and follow-up imaging and clinical data.…”

“…Table 1 Clinical and radiological criteria used to confirm the diagnosis of LOVA (from Ved et al [26])…”

“…The grades in each domain are added up to obtain a single score [16] Grade Definition 3-or 4-Fogarty balloon. Any secondary membrane found in the prepontine cistern was fenestrated as well [26]. Similarly, VPS procedures were also performed in a standardized fashion.…”

“…The correct surgical treatment for LOVA is also disputed. Some authors, such as Oi and Ved [ 23 , 26 ], claim endoscopic third ventriculostomy (ETV) as the first treatment option for LOVA. Others [ 14 ], in the view that LOVA represents a form of communicating hydrocephalus, proposed a primary role of cerebrospinal fluid (CSF) diversion surgery (i.e., ventriculoperitoneal shunt (VPS)).…”