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Congenital atlanto-occipital dislocation in a patient with Down syndrome: a case report.
- Source :
-
Skeletal radiology [Skeletal Radiol] 2023 Sep; Vol. 52 (9), pp. 1785-1789. Date of Electronic Publication: 2023 Feb 11. - Publication Year :
- 2023
-
Abstract
- Down syndrome, also known as trisomy 21, is associated with congenital cervical spine abnormalities, including atlantoaxial instability with or without os odontoideum, atlanto-occipital instability, and hypoplasia of the atlas. Herein, we report a case of Down syndrome complicated by congenital atlanto-occipital dislocation. The patient presented with severe cervical myelopathy at 13 years of age after a 10-year follow-up. Radiography and computed tomography revealed os odontoideum protruding into the foramen magnum and congenital anterior atlanto-occipital dislocation. Additionally, a bifurcated internal occipital crest with a thinned central portion of the occipital bone was noted. Magnetic resonance imaging revealed kyphotic alignment of the spinal cord with severe compression at the foramen magnum level. As the neurological impairment was partially improved by halo vest immobilization, we performed in situ O-C2 fusion with an iliac autograft and decompression of the foramen magnum and posterior arch of C1. An improvement was observed immediately after surgery. Two years after surgery, radiography and computed tomography showed solid O-C2 segment fusion. The accumulation of similar cases is essential for determining the prognosis or optimal treatment for this rare congenital condition.<br /> (© 2023. The Author(s).)
- Subjects :
- Humans
Radiography
Down Syndrome complications
Joint Dislocations complications
Joint Dislocations diagnostic imaging
Joint Dislocations surgery
Joint Instability diagnostic imaging
Joint Instability surgery
Joint Instability etiology
Spinal Cord Diseases
Spinal Diseases
Spinal Fusion
Atlanto-Axial Joint diagnostic imaging
Atlanto-Axial Joint surgery
Subjects
Details
- Language :
- English
- ISSN :
- 1432-2161
- Volume :
- 52
- Issue :
- 9
- Database :
- MEDLINE
- Journal :
- Skeletal radiology
- Publication Type :
- Academic Journal
- Accession number :
- 36773086
- Full Text :
- https://doi.org/10.1007/s00256-023-04297-5