Surgical Management of Hirayama Disease (Monomelic Amyotrophy): Systematic Review and Meta-Analysis of Patient-Level Data.

BACKGROUND

Hirayama disease or juvenile-onset monomelic amyotrophy is a clinical syndrome that disproportionately affects young males. Standard of care revolves around conservative management, but some patients experience disease progression that may benefit from surgical intervention.

METHODS

Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, a systematic review of previous reports of surgical treatment for Hirayama disease was performed. Studies were included if they provided individual patient-level data, described the clinical presentation and surgical intervention, and reported neurological improvement at last follow-up. Comparison between those who improved and those with stable symptoms at last follow-up was performed. Decision-tree analysis was used to identify the best predictors of neurological improvement by last follow-up.

RESULTS

Of 624 unique articles, 30 were included in the qualitative review and 23 in the meta-analysis. Among the 70 patients in the meta-analysis, mean age was 21.2 ± 6.3 years, 91% were male, and mean symptom duration at presentation was 43.3 ± 61.8 months. Fifty-nine patients (84.3%) had improvement in their neurological symptoms by last follow-up. Univariable analysis showed the only significant predictor of improvement in neurological symptoms by last follow-up was the use of stabilization-alone versus decompression with or without stabilization. Baseline clinical symptoms nor radiographic features predicted outcome. Decision-tree analysis showed surgical strategy (stabilization-alone vs. decompression ± stabilization), age (<20 vs. ≥20), and surgical approach (anterior-only vs. posterior-only or anterior-posterior) predicted a higher likelihood of neurological improvement by last follow-up.

CONCLUSIONS

Nearly 85% of patients experienced improvement in neurological symptoms. Improvement was best for those who underwent stabilization-alone, and decision-tree analysis suggested that the likelihood of improvement was also superior for patients under 20 years of age and those treated with an anterior versus posterior or staged approach.