Anterior cervical discectomy and fusion for the treatment of pediatric Hirayama disease

Authors

Marc Prablek, Department of Neurosurgery, Baylor College of Medicine at Texas Children's Hospital, Houston, TX, 77030, USA. prablek@bcm.edu.
Gabriel Reyes, Department of Neurosurgery, Baylor College of Medicine at Texas Children's Hospital, Houston, TX, 77030, USA.
Varun Kannan, Department of Pediatrics, Division of Pediatric Neurology, Emory School of Medicine, Children's Healthcare of Atlanta, Atlanta, Georgia.
Charles T. Gay, Department of Pediatrics, Section of Neurology and Developmental Neuroscience, Baylor College of Medicine at Texas Children's Hospital, Houston, TX, USA.
Timothy E. Lotze, Department of Pediatrics, Section of Neurology and Developmental Neuroscience, Baylor College of Medicine at Texas Children's Hospital, Houston, TX, USA.
Daniel A. Donoho, Division of Neurosurgery, Center for Neuroscience at Children's National Hospital, Department of Neurosurgery, George Washington University School of Medicine and Health Sciences, Washington, DC, USA.
David F. Bauer, Department of Neurosurgery, Baylor College of Medicine at Texas Children's Hospital, Houston, TX, 77030, USA.

Document Type

Journal Article

Publication Date

1-17-2024

Journal

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery

DOI

10.1007/s00381-024-06281-3

Keywords

Anterior cervical fusion; Hirayama disease; Pediatric spinal surgery

Abstract

PURPOSE: Hirayama disease, a rare cervical myelopathy in children and young adults, leads to progressive upper limb weakness and muscle loss. Non-invasive external cervical orthosis has been shown to prevent further neurologic decline; however, this treatment modality has not been successful at restoring neurologic and motor function, especially in long standing cases with significant weakness. The pathophysiology remains not entirely understood, complicating standardized operative guidelines; however, some studies report favorable outcomes with internal fixation. We report a successful surgically treated case of pediatric Hirayama disease, supplemented by a systematic review and collation of reported cases in the literature. METHODS: A review of the literature was performed by searching PubMed, Embase, and Web of Science. Full-length articles were included if they reported clinical data regarding the treatment of at least one patient with Hirayama disease and the neurologic outcome of that treatment. Articles were excluded if they did not provide information on treatment outcomes, were abstract-only publications, or were published in languages other than English. RESULTS: Of the fifteen articles reviewed, 63 patients were described, with 59 undergoing surgery. This encompassed both anterior and posterior spinal procedures and 1 hand tendon transfer. Fifty-five patients, including one from our institution, showed improvement post-treatment. Eleven of these patients were under 18 years old. CONCLUSION: Hirayama disease is an infrequent yet impactful cervical myelopathy with limited high-quality evidence available for optimal treatment. The current literature supports surgical decompression and stabilization as promising interventions. However, comprehensive research is crucial for evolving diagnosis and treatment paradigms.

APA Citation

Prablek, Marc; Reyes, Gabriel; Kannan, Varun; Gay, Charles T.; Lotze, Timothy E.; Donoho, Daniel A.; and Bauer, David F., "Anterior cervical discectomy and fusion for the treatment of pediatric Hirayama disease" (2024). GW Authored Works. Paper 4168.
https://hsrc.himmelfarb.gwu.edu/gwhpubs/4168

Department

Neurological Surgery