Neurologic outcomes in patients with skeletal dysplasias undergoing cervical fusion and occipitocervical fusion.

Purpose: To assess neurologic function after cervical or occipitocervical spinal decompression and fusion in patients with skeletal dysplasias, and to compare the incidence of reoperation between cervical vs. occipitocervical fusion and between postoperative bracing vs. no postoperative bracing.

Methods: We retrospectively reviewed data from 15 patients (mean age, 27 ± 22 years) with skeletal dysplasias (achondroplasia, chondrodysplasia punctata, osteogenesis imperfecta, Morquio syndrome, Hurler syndrome) who underwent cervical (n = 12) or occipitocervical (n = 3) decompression and fusion from 1997 to 2021 and had minimum 2-year follow-up (mean, 5.4 years). We assessed radiographic evidence of fusion, reoperation, and neurologic function (measured by the Barthel Index for Activities of Daily Living, which ranges from 0 [completely dependent] to 100 [fully independent]). Chi-squared tests compared outcomes between cervical vs. occipitocervical fusion and postoperative bracing vs. no bracing.

Results: Among the 9 patients with Barthel Index values, mean values were 49 ± 29 preoperatively, 51 ± 29 postoperatively, and 53 ± 27 at medium-term follow-up, reflecting a lack of worsening in neurologic function. Five of 15 patients underwent reoperation. We found no difference in the incidence of reoperation between cervical and occipitocervical fusion (p = .49) or between braced and non-braced patients (p = .85).

Conclusion: For patients with skeletal dysplasia, cervical and occipitocervical decompression and fusion can prevent worsening of neurological function. Fusion to the occiput and postoperative bracing were not associated with differences in the incidence of reoperation. Cervical spine abnormalities in patients with skeletal dysplasias, and the stenosis and instability they can cause, can be addressed with rigid internal fixation.