Cervical spine arteriovenous fistula associated with hereditary haemorrhagic telangiectasia

Abstract

Reported is a case of a man aged 55 years who presented with progressive spastic paraparesis. Examination demonstrated multiple cutaneous telangiectases. Subsequent development of upper limb weakness, acute urinary retention and eventual respiratory compromise resulted in the requirement for intensive care unit admission and mechanical ventilation. MRI spine revealed diffuse T2 hyperintensity in the cervical cord with enhancement and cord expansion. Immunomodulatory therapy for a presumed diagnosis of transverse myelitis yielded no response, so a vascular aetiology was suspected. Spinal angiography demonstrated an arteriovenous fistula involving the upper cervical cord. Endovascular embolisation was successfully performed and a marked clinical improvement was achieved. Cervical arteriovenous fistulas can cause progressive myelopathy, subarachnoid haemorrhage and brainstem dysfunction. Management typically comprises endovascular embolisation or surgical interruption. A clinical diagnosis of hereditary haemorrhagic telangiectasia was also made in this case, and spinal arteriovenous fistula formation has been associated with this condition.

Figure 1

Cutaneous vascular papules (A, B) visible on the hands and face and telangiectases (C) on the trunk.

Figure 2

Sagittal T2-weighted (A) and T1-weighted post intravenous gadolinium (B) MRI cervical spine demonstrating expansion of the spinal cord and lower medulla. Diffuse central T2 signal hyperintensity is seen extending from the medulla to the level of the inferior vertebral body endplate of C7 with diffuse ill-defined enhancement most marked in the superior aspect of the lesion in the lower brainstem. Multiple flow voids are noted along the posterior surface of the cervical cord, particularly at the C5/C6 levels (arrows).

Figure 3

Spinal angiogram (A) performed via the right vertebral artery (*) demonstrating abnormal vessels descending through the spinal canal (arrows) supplied via the intracranial (V4) segment of the vertebral artery. Following embolisation (B), there is no evidence of the fistula, with the absence of the previously evident arterialised veins in the cervical spinal canal.

Figure 4

Selective angiogram of the right vertebral artery (A) and later superselective angiogram of the feeder artery (B) prior to embolisation. The catheter is visible in the right vertebral artery (*) and the fistula and emissary veins are clearly demonstrated.

Figure 5

Spinal angiography anteroposterior view (A) demonstrating the presence of an abnormal tortuous vessel arising from the right vertebral artery (*) and descending emissary veins (arrows). Following embolisation (B), there is complete disappearance of these abnormal vessels.

Figure 6

Sagittal T2-weighted MRI cervical spine demonstrating an interval decrease in the size of the hyperintense lesion and the extent of cord expansion.