Natural history of ventriculomegaly in adults: a cluster analysis

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OBJECTIVE

Chronic ventriculomegaly in the absence of raised intracranial pressure (ICP) is a known entity in adult hydrocephalus practice. The natural history and indication for treatment is, however, poorly defined. A highly heterogeneous group, some adults with ventriculomegaly are asymptomatic, while others have life-threatening deteriorations. The authors hypothesized that the various presentations can be subtyped and represent different stages of decompensation. A cluster analysis was performed on a cohort of patients with chronic ventriculomegaly with the aim of elucidating typical clinical characteristics and outcomes in chronic ventriculomegaly in adults.

METHODS

Data were collected from 79 patients with chronic ventriculomegaly referred to a single center, including demographics, presenting symptoms, and 24-hour ICP monitoring (ICPM). A statistical cluster analysis was performed to determine the presence of subgroups.

RESULTS

Four main subgroups and one highly dissimilar group were identified. Patients with ventriculomegaly commonly have a perinatal event followed by one of four main presentations: 1) incidental ventriculomegaly with or without headache; 2) highly symptomatic presentation (including reduced consciousness) and raised ICP; 3) early presenting with symptoms of headache and nausea (with abnormal pulsatility); and 4) late presenting with features common to normal pressure hydrocephalus. Each symptomatic group has characteristic radiological features, ICPM, and responses to treatment.

CONCLUSIONS

Cluster analysis has identified subgroups of adult patients with ventriculomegaly. Such groups may represent various degrees of decompensation. Surgical interventions may not be equally effective across the subgroups, presenting an avenue for further research. The identified subtypes provide further insight into the natural history of this lesser studied form of hydrocephalus.

ABBREVIATIONS EI = Evans’ index; ETV = endoscopic third ventriculostomy; GCS = Glasgow Coma Scale; ICP = intracranial pressure; ICPM = ICP monitoring; LOVA = long-standing overt ventriculomegaly in adults; NPH = normal pressure hydrocephalus; OSA = obstructive sleep apnea; SDH = subdural hematoma; STV = spontaneous third ventriculostomy; VP = ventriculoperitoneal.

Article Information

Correspondence Claudia L. Craven: National Hospital for Neurology and Neurosurgery, London, United Kingdom. claudia.craven@gmail.com.

INCLUDE WHEN CITING Published online February 8, 2019; DOI: 10.3171/2018.8.JNS18119.

Disclosures Dr. D’Antona reports receiving a research fellowship sponsored by B. Braun Medical Ltd. Dr. Watkins has received honoraria from and served on advisory boards for Medtronic, Codman, and B. Braun.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    MR image showing the typical appearance of ventriculomegaly in LOVA.

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    Hierarchical clustering dendogram showing four major clusters and a fifth highly dissimilar cluster. Numbers at the base represent anonymized individual patients. Figure is available in color online only.

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    STV on MRI. Left: Sagittal sequence (inverse) showing a defect in the floor of the third ventricle. Right: CSF flow sequence demonstrating flow across the floor of the third ventricle in the same patient.

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    Flowchart summary of the four main subtypes, clinical features, and suggested management options (excluding panventriculomegaly). We emphasize the potential for various types to progress to other types (dashed line). CISS = constructive interference in steady state.

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