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Upper Extremity Surgery in Hirayama Disease: A Modification of the Current Algorithm.

Elad Y Holzer1, Tod A Clark2, Jennifer L Giuffre1

  • 1From the Section of Plastic Surgery, Department of Surgery.

Annals of Plastic Surgery
|April 24, 2025
PubMed
Summary

Hirayama disease (HD) can be effectively treated with upper extremity surgery, including nerve and tendon transfers. This study presents the largest case series and literature review of these successful interventions for HD patients.

Keywords:
AIN to UMN transferHirayama diseaseanterior interosseus nerve to ulnar motor nerve transferbenign focal amyotrophy nerve transfersjoint fusionjuvenile muscular monomelic amyotrophymonomelic amyotrophytendon transfers

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Area of Science:

  • Neurology
  • Orthopedic Surgery

Background:

  • Hirayama disease (HD) is a rare, nonfamilial amyotrophy causing forearm and hand muscle atrophy and weakness.
  • Current treatments for HD include conservative measures and spinal surgery, but upper extremity surgical options are not well-established.

Purpose of the Study:

  • To present a case series of HD patients treated with nerve and tendon transfers and joint fusions.
  • To conduct a literature review of upper extremity surgical interventions for HD.

Main Methods:

  • Retrospective review of three HD patients (four limbs) treated with nerve and tendon transfers and fusion.
  • Literature search on PubMed for studies on surgical treatment of HD using terms like "Hirayama disease" and "upper extremity surgery."

Main Results:

  • Three HD patients (four limbs) with intrinsic muscle and thenar/hypothenar atrophy underwent surgical treatment.
  • Procedures included anterior interosseous (AIN) to ulnar motor nerve transfer and thumb metacarpophalangeal joint fusion with Zancolli lasso.
  • All patients showed subjective and objective functional improvements postoperatively.

Conclusions:

  • Upper extremity surgery, including nerve transfers, offers a successful treatment option for Hirayama disease.
  • This study represents the largest case series and first literature review of such interventions in HD.