Surgical treatment for symptomatic ventriculus terminalis: case series and a literature review

BACKGROUND: 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. 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. METHODS: A 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. RESULTS: Fourteen 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. CONCLUSIONS: Surgery 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.