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Surgical Treatment for Severe Cervical Hyperlordosis and Thoracolumar Kyphoscoliosis with Emery–Dreifuss Muscular Dystrophy: A Case Report and Literature Review

BACKGROUND: Emery–Dreifuss muscular dystrophy (EDMD) is an uncommon, gradually progressive X‐linked myopathy, and it could result in rigid spinal deformity. Only a few case reports have described surgical treatment of cervical hyperlordosis and thoracolumbar kyphoscoliosis secondary to EDMD. We repo...

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Detalles Bibliográficos
Autores principales: Tang, Ziyang, Hu, Zongshan, Qin, Xiaodong, Zhu, Zezhang, Liu, Zhen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons Australia, Ltd 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9732636/
https://www.ncbi.nlm.nih.gov/pubmed/36250567
http://dx.doi.org/10.1111/os.13526
Descripción
Sumario:BACKGROUND: Emery–Dreifuss muscular dystrophy (EDMD) is an uncommon, gradually progressive X‐linked myopathy, and it could result in rigid spinal deformity. Only a few case reports have described surgical treatment of cervical hyperlordosis and thoracolumbar kyphoscoliosis secondary to EDMD. We report a rare case of EDMD to present the surgical strategies of severe cervical hyperlordosis and thoracolumbar kyphoscoliosis. CASE PRESENTATION: The patient was a 22‐year‐old man with EDMD who had severe cervical hyperlordosis and thoracolumbar kyphoscoliosis. A posterior spinal fusion from T9‐S2 was performed to correct the thoracolumbar kyphoscoliosis at the age of 21 years. Six months later, with an anterior C7‐T1 closing wedge bone‐disc‐bone osteotomy and a posterior–anterior–posterior cervicothoracic fusion from C4‐T4, the cervical deformity was corrected, thus achieving a horizontal gaze. During 1.5‐year follow‐up, no loss of correction was observed. CONCLUSION: Cervical posterior–anterior–posterior closing‐wedge osteotomy combined with long fusion at thoracolumbar spine can be a reliable surgical technique to correct severe spine deformity in EDMD. This two‐stage revision surgical strategy can help restore a horizontal gaze on the basis of a balanced trunk. Cervical deformity in such patients should be corrected in the first stage considering its role as a “driver” of the global spine deformity.