Conus Medullaris Arteriovenous Malformation Presenting with Acute Dysphagia and Intractable Hiccups

Conus medullaris arteriovenous malformations (AVMs) are rare spinal vascular malformations presenting most frequently with features of myelopathy (Foix-Alajounine syndrome), radiculopathy, bowel/bladder dysfunction, or acute spinal hemorrhage (Coup de poignard of Michon) causing profound neurological deficit. Here we present the case of a young patient with progressive dysphagia and intractable hiccups as a rare first presentation symptom of later verified conus medullaris AVM.

A 21-year-old male patient presented with acute onset of dysphagia and persistent hiccups. His magnetic resonance imaging of the spine demonstrated a lesion at the T11 and T12 levels with an associated holocord syrinx and syringobulbia to the level of the medulla. The patient underwent a decompressive suboccipital craniectomy and C1 (atlas) laminectomy with wide myelotomy of the medulla followed by T11 and T12 laminectomy and AVM reduction. Two days after partial AVM occlusion the patient developed transient worsening of his symptoms. Repeat magnetic resonance imaging showed recurrence of dilatation of the central canal. A syringo-subarachnoid shunt was sited at the level of the previous myelotomy of the medulla, after which his neurological symptoms resolved completely.

This is the first case report in the English literature to date of a conus AVM presenting with intractable hiccups. These are extremely rare sporadic vascular malformations, and although their natural history is poorly understood, symptomatic patients generally deteriorate, culminating in severe disability. Management requires a multimodality approach including combined endovascular and microsurgical treatment. The patient in our case made a full recovery confirmed at 2-year follow-up.