Orthopaedic surgery · 2022
Surgical Treatment for Severe Cervical Hyperlordosis and Thoracolumar Kyphoscoliosis with Emery-Dreifuss Muscular Dystrophy: A Case Report and Literature Review.
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Tang Ziyang, Hu Zongshan, Qin Xiaodong, Zhu Zezhang, Liu Zhen
Аннотация
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.
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.