Sudden Quadriparesis Due to a Ventral Cervical Neurenteric Cyst: A Rare but Reversible Cause of Acute Myelopathy

Abstract

Introduction

Neurenteric cysts are rare congenital lesions of endodermal origin that typically present with slowly progressive myelopathy. Accounting for less than 2% of all spinal tumors, they most often occur in the cervical and upper thoracic spine. Acute neurological deterioration due to a neurenteric cyst is uncommon and represents a clinical emergency.

Case Presentation

We describe an 18-year-old woman with no prior medical history who developed sudden-onset neck pain, rapidly progressive quadriparesis, and respiratory compromise. Neurological examination revealed upper motor neuron signs with a C4 sensory level. Cervical magnetic resonance imaging showed a ventral intradural extramedullary cystic lesion at the C2–C3 level, compressing the spinal cord and producing cord edema. Emergent posterior C2–C3 laminectomy was performed, and a tense mucin-filled cyst was completely excised. Histopathology demonstrated a columnar mucinous epithelium with goblet cells, confirming the diagnosis of a neurenteric cyst. Postoperatively, the patient exhibited substantial neurological recovery, regaining independent ambulation within three months. Follow-up imaging at six months showed no recurrence.

Discussion

This case underscores two important lessons. First, although neurenteric cysts are classically indolent, they may present with abrupt, life-threatening neurological decline, even in the absence of associated congenital vertebral anomalies. Second, prompt surgical decompression with gross total resection remains the cornerstone of management, offering excellent potential for recovery. Given the risk of recurrence, radiological surveillance is warranted. This report adds to the limited literature on cervical neurenteric cysts with hyperacute presentations and emphasizes the need for early recognition and timely intervention to optimize outcomes.