Abstract

BackgroundVentriculus terminalis is a cystic embryological remnant within the conus medullaris that normally regresses after birth. In rare cases, it may persist into adulthood and give rise to neurological symptoms, for which the optimal treatment remains uncertain. The aim of this study was to present our experience from a population-based cohort of patients with ventriculus terminalis and discuss our management strategy as compared to the existing literature.MethodsA retrospective review was conducted of all adult (≥ 15 years) patients with ventriculus terminalis who were referred to the Karolinska University Hospital between 2010 and 2018.ResultsFourteen patients were included. All patients were symptomatic at the time of referral, and the most common symptom was lower limb weakness (n = 9). Microsurgical cyst fenestration was offered to all patients and performed in thirteen. Postoperative imaging confirmed cyst size reduction in all surgically treated patients. No surgical complications were reported. Eleven of the surgically treated patients showed clinical improvement at long-term follow-up. One patient declined surgery, with progression of the cyst size and clinical deterioration observed at follow-up.ConclusionsSurgery for ventriculus terminalis seems to be a safe and effective option for relief of symptoms. We propose that surgery should be offered to all patients with symptomatic ventriculus terminalis.

Highlights

  • Ventriculus terminalis, known as the fifth ventricle, is an ependymal lined cerebrospinal fluid (CSF)-filled cavity within the conus medullaris

  • In an effort to facilitate this, de Moura Batista and colleagues established a clinical classification system based on the available literature (“cystic lesion of the ventriculus terminalis classification” (CLVT) [10], later revised by Ganau et al based on their cohort of 13 patients [2], in which patients are categorized into Cystic lesion of the ventriculus terminalis classification (CLVT) type Ia, type Ib, type II, and type III

  • 14 patients were referred for MRIverified ventriculus terminalis

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Summary

Introduction

Ventriculus terminalis, known as the fifth ventricle, is an ependymal lined cerebrospinal fluid (CSF)-filled cavity within the conus medullaris. It is formed between the 43rd and the 48th day of embryogenesis, via canalization and retrogressive differentiation of the caudal end of the developing spinal cord and typically regresses completely after birth [1]. The treatment for symptomatic ventriculus terminalis, whether conservative or surgical, remains uncertain. Ventriculus terminalis is a cystic embryological remnant within the conus medullaris that normally regresses after birth In rare cases, it may persist into adulthood and give rise to neurological symptoms, for which the optimal treatment remains uncertain. We propose that surgery should be offered to all patients with symptomatic ventriculus terminalis

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