Objectives: Manifestation of congenital anomalies of cranio-vertebral junction (CVJ) in the later half of life is unusual and intriguing. Coexisting cervical spondylotic changes with multilevel compression, poorer bone quality as well as less smooth post-surgical recuperation make management of elderly Congenital Atlantoaxial Dislocation/ Basilar Invagination (CAAD/BI) challenging. The clinico-radiological presentation, pathogenesis and outcome are analysed here.
Patients and methods: Clinico-radiological data of 20 patients of CAAD/BI (with markers of congenital anomalies) presenting after 50 years of age, the challenges faced and outcomes after C1-C2 fusion have been analysed.
Results: Three distinct groups were identified. Seven patients with Os-odontoideum had reducible AAD (Type I). Seven patients had assimilated C1, C2-3 fusion and deformed C1-2 joints with irreducible AAD/BI (Type II). In type III, 4 patients had similar segmentation defects but with compression at both cervico-medullary junction and subaxial spine, although clinical localisation pointed to the CVJ. Spastic quadriparesis was the commonest presentation. All underwent C1-2 fusion alone. There was significant improvement in 18, including those with compression at additional level. Bony fusion was documented in all patients followed up beyond one year.
Conclusion: Congenital CVJ anomalies may present in later half of life, though attempts at reasoning remain speculative. These patients improve after multiplanar realignment and C1-2 fusion. Careful clinico-radiological evaluation is required in those with additional subaxial compression. Bone quality in elderly is not a deterrent for instrumentation. Fusion eventually occurs in most.
Keywords: Atlantoaxial dislocation; C1-2 fusion; Congenital; Old age; Outcome.
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