Management of hip subluxation using hip bracing in patients with Fukuyama congenital muscular dystrophy: A pilot study
Ju Seok Ryu, Won-Jae Jo, Hyun Jin Kim, Hyunji Choi, Jee Hyun SuhBackground:
Fukuyama congenital muscular dystrophy (FCMD) is a rare neuromuscular disorder associated with severe muscle weakness and a high risk of hip displacement. Surgical management of hip dislocation is challenging because of cardiopulmonary risks, and the role of hip bracing in FCMD has not been established.
Objective:
To explore the feasibility and radiographic outcomes of hip bracing in nonambulatory children with FCMD.
Study Design:
Case series.
Methods:
Three nonambulatory children with genetically confirmed FCMD underwent hip bracing for 6 months. Radiographic parameters, including Reimer migration percentage (MP), femoral head-shaft angle, and acetabular index, were evaluated at baseline and follow-up. For contextual reference, radiographic changes in 3 age- and sex-matched historical FCMD cases without bracing were descriptively reviewed.
Results:
Individual responses to hip bracing were heterogeneous. Two patients demonstrated stabilization or slight improvement in MP, whereas 1 patient showed progression. In contrast, all historical nonbraced cases showed worsening of MP over a comparable follow-up period.
Conclusions:
This case series suggests that hip bracing is feasible in nonambulatory children with FCMD and may be associated with stabilization of hip displacement in selected cases. However, responses were variable, and effectiveness cannot be determined from this small series. Larger prospective studies are needed to clarify clinical relevance and identify patients most likely to benefit.