‘Congenital anomalies of craniovertebral junction presenting after 50 years of age’: An oxymoron or an unusual variation?

Publication date: Available online 21 December 2017
Source:Clinical Neurology and Neurosurgery
Author(s): Pravin Salunke, Madhivanan Karthigeyan, Narasimhaiah Sunil, Vasundhara Rangan
ObjectivesManifestation 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 methodsClinico-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.ResultsThree 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.ConclusionCongenital 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.



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