Diego A Gomez, Anna D Lee, Jacob B Hammond, Skyler K Palmer, Brooke French, Jason W Yu, Phuong D Nguyen, David W Mathes, David Y Khechoyan
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Early-Onset Facial Weakness: Evaluation and Surgical Treatment of Facioscapulohumeral Muscular Disease.
Facioscapulohumeral muscular dystrophy (FSHD) is the third most common inherited muscular dystrophy, following Duchenne and Becker muscular dystrophy. Early-onset FSHD presents unique challenges, particularly in the context of managing early-acquired facial weakness, which significantly impacts functional and psychosocial well-being. Despite its clinical significance, the surgical outcomes of facial reanimation in this population have not been reported, and a standardized treatment protocol is lacking. This review seeks to address this gap by proposing a standardized diagnostic and management pathway for pediatric patients with early-onset FSHD presenting with facial paralysis. Our multidisciplinary diagnostic protocol includes comprehensive genetic testing, magnetic resonance imaging, and electromyography, with assessments conducted by a collaborative team comprising Pediatricians, Neurologists, Geneticists, and Ophthalmologists. We discuss surgical strategies for facial reanimation, emphasizing their critical role in preserving psychosocial health and quality of life. By consolidating current knowledge and proposing a structured approach, this review aims to serve as a reference for clinicians and surgeons, guiding improved diagnosis, management, and outcomes in early-onset FSHD.
期刊介绍:
The Journal of Craniofacial Surgery serves as a forum of communication for all those involved in craniofacial surgery, maxillofacial surgery and pediatric plastic surgery. Coverage ranges from practical aspects of craniofacial surgery to the basic science that underlies surgical practice. The journal publishes original articles, scientific reviews, editorials and invited commentary, abstracts and selected articles from international journals, and occasional international bibliographies in craniofacial surgery.