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. 2025 Jul 10;41(1):229.
doi: 10.1007/s00381-025-06860-y.

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

John P Avendano  1 , Tej D Azad  2 , William ElNemer  1 , Paul D Sponseller  3 , Mari L Groves  2
Affiliations

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

John P Avendano et al. Childs Nerv Syst. .

Abstract

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.

Keywords: Achondroplasia; Barthel index; Cervical fusion; Occipitocervical fusion; Osteogenesis imperfecta; Skeletal dysplasia.

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Conflict of interest statement

Declarations. Ethics Approval: This study was performed in line with the principes of the Declaration of Helsinki. It was approved by our institutional review board (approval no. NA_00043838). Financial Interest: Paul D. Sponseller receives IP royalties from DePuy and Globus Medical; publishing royalties and financial or material support from the Journal of Bone & Joint Surgery American; other financial or material support from Orthopediatrics; and is a paid consultant and receives research support from DePuy. No other authors have anything to disclose. Non-financial Interest: Paul D. Sponseller is on the editorial board of the Journal of Bone & Joint Surgery American and is a board or committee member of the Scoliosis Research Society. No other authors have non-financial interests to disclose. Consent to Participate: Informed consent was obtained from legal guardians. Institutional review board statement: This study was approved by the Johns Hopkins institutional review board, no. NA_00043838.

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