• Eur Spine J · Dec 2022

    Review

    Juvenile muscular atrophy of the distal upper extremity (Hirayama syndrome): a systematic review.

    • Henrik C Bäcker, Jacob Bock, Peter Turner, Michael A Johnson, John Cunningham, Patrick Chan, and Richard Gerraty.
    • Department of Orthopaedic Surgery, Royal Melbourne Hospital, 300 Grattan Street, Parkville, VIC, 3050, Australia. Henrik.baecker@sports-med.org.
    • Eur Spine J. 2022 Dec 1; 31 (12): 329633073296-3307.

    IntroductionHirayama syndrome is likely caused by a forward displacement of the posterior dura during cervical flexion leading to changes in the muscles of the fingers and wrist. The aim of this systematic review was to document the number of reported cases, the necessity of dynamic MRI of the cervical spine and the subsequent treatment.Methods And MaterialsA systematic review was conducted and the Pubmed/Medbase, Cochrane, Google, Embase and Ovid database were searched for (Hirayama) AND ((disease) OR (syndrome)). A total of 42 studies were included for analysis reporting 2311 patients.ResultsThe mean age was 20.2 ± 2.26 years and predominantly males (92.8%) were identified. On MRI the "snake eyes" appearance of the spinal cord was present in 27.8% and the typical time between onset of symptoms and diagnosis was 41.5 ± 16.4 months. A variety of different treatments have been reported, although there is no substantial evidence that any of them are superior to observation.ConclusionThe delay in diagnosis from initial presentation of symptoms shows that this condition may be underdiagnosed in a variety of cases. Further, this study shows the necessity of either a dynamic MRI in flexion or a static MRI scan in neutral position and in flexion, to identify functional spinal and/or foraminal stenosis for a prompt diagnosis and subsequent treatment.© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.

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