Published online Feb 26, 2019. doi: 10.12998/wjcc.v7.i4.532
Peer-review started: November 5, 2018
First decision: January 5, 2019
Revised: January 21, 2019
Accepted: January 26, 2019
Article in press: January 26, 2019
Published online: February 26, 2019
Spinal deformities in Ehlers-Danlos syndrome (EDS; type VI) are generally progressive and severe. Surgical treatment has been described for kyphoscoliosis in the thoracolumbar spine. However, there are few studies describing the consequences of an anterior approach in cervical kyphosis. An anterior approach may not be able to fully decompress the spinal canal and restore the normal curvature of the cervical spine. Therefore, the anterior approach for cervical kyphosis in young children is hard. We describe the first case in an EDS girl with cervical kyphosis who received satisfactory anterior cervical corpectomy decompression and fusion.
The chief complaints of a 16-year-old girl with EDS were double upper limb weakness for 7 years and double lower limb walking instability for 2 years. Moreover, the imaging results revealed that the degree of kyphosis from cervical vertebra 2 to 4 accompanying with spinal cord compression was 30°. An anterior cervical corpectomy involving cervical vertebra 3 and a titanium mesh implant were performed with internal fixation. The results at 3 mo after surgery demonstrated that the anterior fusion was solid, and the kyphosis of the cervical spine was corrected. Additionally, the power of all four extremities was significantly improved.
The incidence rate of cervical kyphosis in EDS is rare. The surgical treatment for these patients, especially an anterior approach, is challenging. Therefore, to develop safer and more effective strategies to treat cervical kyphosis in EDS, there is still much work to do.
Core tip: Ehlers-Danlos syndrome (ESD) is a rare inherited and clinically heterogeneous group of connective tissue disorders. We report a case diagnosed with ESD that was treated by anterior cervical surgery. The clinical outcome is satisfactory.