Abstract

Ventriculus terminalis (VT) 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 neurologic symptoms. The pathogenesis remains unclear but is thought to be related to failed embryonic regression with other proposed possible etiologies including vascular disturbances. We present an intriguing case of a slow-growing VT in a woman with progressive neurologic symptoms who experiences symptomatic relief following thoracic laminectomy and fenestration. Our case is the first to present a unique association with polyarteritis nodosa and only the third to report a case of documented enlargement of the VT over time successfully treated with surgical fenestration.

Highlights

  • The ventriculus terminalis (VT) or “fifth ventricle” is a small ependymal-lined residual lumen in continuity with the central canal of the conus medullaris

  • We present an intriguing case of a slowgrowing Ventriculus terminalis (VT) in a woman with progressive neurologic symptoms who experiences symptomatic relief following thoracic laminectomy and fenestration

  • Our case is the first to present a unique association with polyarteritis nodosa and only the third to report a case of documented enlargement of the VT over time successfully treated with surgical fenestration

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Summary

Introduction

The ventriculus terminalis (VT) or “fifth ventricle” is a small ependymal-lined residual lumen in continuity with the central canal of the conus medullaris. A 57-year-old female with a past medical history significant for polyarteritis nodosa on immunosuppression with Azathioprine presented to the neurosurgery clinic with progressive right lower extremity shooting pain, numbness and tingling over the past six months. On MRI thoracic spine with and without contrast at the time of her subsequent presentation in 2020, she was found to have interval enlargement (Figure 1) of the previously imaged conus medullaris cystic mass (Figure 2) which had enlarged over the course of the past nine years. Based on the interval increase in the size of the conus medullaris lesion in the setting of normal EMG and NCV, it was felt the etiology of her current symptomatology and neurologic examination findings were related to the cystic conus medullaris lesion She subsequently underwent a thoracic-11 to thoracic-12 laminectomy with cyst fenestration.

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