Cervical kyphosis in Ehlers-Danlos syndrome

We see many patients with Ehlers-Danlos syndrome. For these patients, there can be a great challenge in having a successful surgery.  A  paper in the American Journal of Medical Genetics summarizes the challenges some patients with hypermobility syndromes such as hEDS may face.

“The prevalence of cervical and thoracic segmental instability in the population of patients with hypermobility syndromes has not been well established. However, discopathy (degenerative disc disease) and early degenerative spondylotic disease (degenerative disease associated with aging)  in hEDS and classical type EDS are well established. EDS is characterized by segmental instability, kyphosis, and scoliosis. Spondylosis, defined by the presence of non‐inflammatory disc degeneration, is usually preceded by mild segmental instability. As a consequence of cervical and thoracic instability, and discopathy in EDS, there is loss of the normal cervical lordosis and an increasing kyphosis, rendering EDS patients prone to progressive myelopathy (compression of the spinal cord), and mechanical neck and chest pain. (3)

Ehlers-Danlos Syndrome, Atlanto-axial instability, and Craniocervical instability

References

1 Henderson Sr FC, Austin C, Benzel E, Bolognese P, Ellenbogen R, Francomano CA, Ireton C, Klinge P, Koby M, Long D, Patel S. Neurological and spinal manifestations of the Ehlers–Danlos syndromes. In American Journal of Medical Genetics Part C: Seminars in Medical Genetics 2017 Mar (Vol. 175, No. 1, pp. 195-211). [Google Scholar]

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