OS odontoideum in an achondroplastic adult – A case report and review

Background: Achondroplasia is a rare genetic disorder affecting bone development, resulting in disproportionate short stature. The coexistence of os odontoideum (OO) in individuals with achondroplasia is exceptionally uncommon. Case Description: We present the case of a 29-year-old female with achondroplasia who experienced progressive weakness in the extremities, ultimately leading to difficulty in ambulation. Radiographic evaluation revealed an atlantoaxial dislocation secondary to an unstable OO. Surgical stabilization of C1–C2 resulted in gradual yet significant neurological recovery. Conclusion: Since the first reported case in 1974, only a limited number of achondroplastic individuals with atlantoaxial dislocation due to OO have been documented.

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Publication Details

Journal
Surgical Neurology International
Published
2026-05-22
DOI
https://doi.org/10.25259/sni_219_2026
Primary Topic
Connective tissue disorders research
Type
article
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article

OS odontoideum in an achondroplastic adult – A case report and review

Abolfazl Rahimizadeh, Reza Mollahouseini, Walter Wiliamson
Surgical Neurology International
Connective tissue disorders research
article

OS odontoideum in an achondroplastic adult – A case report and review

Abolfazl Rahimizadeh, Reza Mollahouseini, Walter Wiliamson
article en

Abstract

Background: Achondroplasia is a rare genetic disorder affecting bone development, resulting in disproportionate short stature. The coexistence of os odontoideum (OO) in individuals with achondroplasia is exceptionally uncommon. Case Description: We present the case of a 29-year-old female with achondroplasia who experienced progressive weakness in the extremities, ultimately leading to difficulty in ambulation. Radiographic evaluation revealed an atlantoaxial dislocation secondary to an unstable OO. Surgical stabilization of C1–C2 resulted in gradual yet significant neurological recovery. Conclusion: Since the first reported case in 1974, only a limited number of achondroplastic individuals with atlantoaxial dislocation due to OO have been documented.

Surgical Neurology InternationalVol. 17
Iran University of Medical Sciences (IR), Baylor College of Medicine (US), Firoozgar General Hospital (IR)
Gender equality
Openalex Percentile: Top 5%
Connective tissue disorders research
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