Cervical laminectomy and micro resection of the posterior venous plexus in Hirayama disease.

Abstract

Introduction. Hirayama disease is a rare cervical myelopathy predominantly affecting young adults andmainly found in Asia. It results in a pure motor distal lesion of the upper limbs with slow progression.Dynamic magnetic resonance imaging (MRI), which allows the diagnosis to be made, shows a typical appearance of anterior compression of the cervical spinal cord associated with enlargement of the poste-rior epidural spaces due to a dilated venous plexus. Surgery is considered when conservative treatmenthas failed. However, the type of surgery is not well standardized in this compressive myelopathy.Methods. – We report on three patients with Hirayama disease operated using an original method: cer-vical decompressive laminectomy and coagulation of the posterior epidural plexus without fixation. The clinical, radiological and surgical data of these three patients were analyzed. Each patient underwent postoperative MR imaging.Results. – The mean age at diagnosis was 18.6 years (16–20 years) with a history of progressive symptomslasting 1 to 4 years before treatment. Follow-up was 21 to 66 months after surgery. Neurological and electrophysiological improvement was noted in two patients; the third had stabilized. Post operative MRI confirmed normalization of flexion imaging on MRI. None of the three patients complained of disablingneck pain.Conclusion. – Posterior cervical decompression with coagulation of epidural venous plexus is a technique that seems effective in Hirayama disease in young subjects. It effectively treats patients by avoiding permanent cervical fixation.