Intractable Rare Dis Res. 2022;11(4):173-179. (DOI: 10.5582/irdr.2022.01105)

Do patients with Hirayama disease require surgical treatment? A review of the literature

Gao Y, Sun C, Ma X, Lu F, Jiang J, Wang H


The main clinic characteristic of Hirayama disease (HD) is atrophy of the distal muscles in the upper limbs. Recently, an increasing number of HD cases have been reported. Many HD patients have persistently progressive symptoms and conservative treatments failed. This article aims to review the current status of the field and summarizes the main surgical treatment options for patients with HD. A comprehensive search of the PubMed and the Web of Science databases was conducted from their inception to September 15th, 2022. Search terms included "juvenile muscular atrophy of upper extremity", "Hirayama disease" and "surgery". A total of 169 relevant publications were identified and 29 articles were finally reviewed. Current surgical treatments for HD are either anterior cervical surgery or posterior cervical surgery. The two approaches can effectively stop the disease. However, no studies have compared the advantages and limitations of the two surgical methods. The previous view that HD can be improved with conservative treatment has been challenged. In many studies, surgical treatment has been shown to improve the hand function in patients with HD. However, there is still controversy about the methods of anterior and posterior cervical surgery. Future research could focus on exploring the advantages and limitations of different surgeries.

KEYWORDS: Hirayama disease, posterior cervical surgery, anterior cervical surgery

Full Text: