Restoration of Global Sagittal Alignment After Surgical Correction of Cervical Hyperlordosis in a Patient with Emery-Dreifuss Muscular Dystrophy

Abstract

Case: We report a rare cervical hyperlordotic deformity in a 19-year-old woman with Emery-Dreifuss muscular dystrophy and concomitant scoliosis. After standard posterolateral instrumentation and fusion of C2-T1 and extensive soft-tissue release, her neck pain improved and unassisted maintenance of cervical alignment and horizontal gaze were preserved through an 8-year follow-up. More importantly, she exhibited reciprocal correction of compensatory global sagittal malalignment, including lumbar lordosis. Conclusions: This case highlights the importance of full-spine analysis for all patients with spinal deformity to identify and differentiate primary driver(s) of deformity from compensatory mechanisms to individualize treatment toward what truly drives the patient's disability.

Laminopathies’ Treatments Systematic Review: A Contribution Towards a ‘Treatabolome’

“…However, a frequent question asked by physicians following these patients pertains the management of joint contractures. Early joint contractures observed in the LMNA -related Emery-Dreifuss disease, which are not necessarily linked to muscle deficit, may benefit from direct surgical procedures when severe or responsible for high disability [ 24 ]. The most common joint contractures localizations are Achilles tendons, elbows and post-cervical muscles.…”

“…The most common joint contractures localizations are Achilles tendons, elbows and post-cervical muscles. There is published evidence on the surgical management of severe extension deformity of the cervical spine associated or not with scoliosis [ 24, 25 ] and also of severe upper extremity contractures. These were treated successfully with contracture release and musculotendinous lengthening [ 26 ] that improved range of motion without a significant sacrifice of strength.…”

“…procedures when severe or responsible for high disability[24]. The most common joint contractures localizations are Achilles tendons, elbows and post-cervical muscles.…”

“…The most common joint contractures localizations are Achilles tendons, elbows and post-cervical muscles. There is published evidence on the surgical management of severe extension deformity of the cervical spine associated or not with scoliosis[24,25] and also of severe upper extrem-…”

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

“…5,6 This rare cervical hyperlordotic deformity is a feature of EDMD which impedes the horizontal gaze and normal gait of patients. 1,5 Moreover, cervical deformity can cause severe dysphagia and/or dyspnea.…”