A Severe Case of Hirayama Disease Successfully Treated by Posterior Cervical Fixation without Decompression and Fusion

World Neurosurg. 2019 Feb:122:326-330. doi: 10.1016/j.wneu.2018.10.157. Epub 2018 Nov 2.

Abstract

Background: Hirayama disease (HD) is a rare disease traditionally treated with nonsurgical procedures. Surgical treatment, which is performed by decompression and/or fusion of the cervical spine, is generally considered for rapidly progressive or advanced cases, and the choice of surgical technique is debatable. We describe the first severe case of HD that was successfully treated by posterior lateral mass screw fixation without decompression or fusion.

Case description: The patient was a 17-year-old adolescent boy who presented with progressive symptoms bilaterally with an unsatisfactory history of conservative treatment. We performed posterior lateral mass screw fixation without decompression or fusion and removed the internal fixation after 4 years of follow-up. Symptom remission and imaging performance improvement were achieved, and the stability and range of motion of the cervical spine were maintained.

Conclusions: Nondecompression and nonfusion surgery was successful and achieved satisfactory results in this case; therefore, it serves as a promising candidate for the surgical treatment of HD.

Keywords: Hirayama disease; Nondecompression; Nonfusion; Posterior cervical fixation; Surgical treatment.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Bone Screws*
  • Cervical Vertebrae / physiology
  • Cervical Vertebrae / surgery*
  • Hand Strength / physiology
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Neurologic Examination
  • Range of Motion, Articular / physiology
  • Spinal Muscular Atrophies of Childhood / physiopathology
  • Spinal Muscular Atrophies of Childhood / surgery*
  • Treatment Outcome

Supplementary concepts

  • Amyotrophy, monomelic