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Year : 2007  |  Volume : 2  |  Issue : 1  |  Page : 10-15

Aqueductal stenosis and mental retardation associated with O S odontoideum, "hypermobile" atlantoaxial dislocation and congenital stenosis of atlas leading to spastic quadriparesis

Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India

Correspondence Address:
Sanjay Behari
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Rae Bareli Road, Lucknow - 226 014, UP
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.31999

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Background: The authors report a unique concomitant occurrence of mental retardation, hydrocephalus due to aqueductal stenosis, a freely floating atlas (having anterior, posterior and transverse atlantoaxial dislocation [AAD]) consequent to an orthotopic os odontoideum and stenosis of atlantal ring. There were no features of coexisting chondroskeletal dysplasias or other systemic diseases. To the best of the authors' knowledge, this is the first reported case of its type in the literature. Case Report: This 17-year-old girl with delayed milestones had enlarging head with vomiting at 1 year of age. Her CT scan revealed hydrocephalus with aqueductal stenosis, and she underwent a ventriculoperitoneal shunt. She subsequently had progressive quadriparesis. A minor fall led to transient unconsciousness and aggravation of her symptoms. Radiology of the craniovertebral junction revealed os odontoideum with anterior, posterior and transverse C1-2 subluxation and atlantal ring stenosis with marked cervical compression. A C1 laminectomy with occipitocervical contoured rod fusion with onlay autologous bone graft under guidance of intraoperative image intensifier was performed. Conclusion: The concomitant presence of hydrocephalus due to aqueductal stenosis, mental retardation and congenital stenosis of the atlantal ring points towards a congenital origin for the os odontoideum. The free floating atlantal ring on the axis led to anteroposterior and transverse AAD, necessitating intubation and occipitocervical stabilization in absolutely neutral position of the neck since both flexion as well as extension movements would have been deleterious. Congenital stenosis of atlas is an extremely rare entity; it contributed to cervical canal compromise even in neutral position of the cervical spine when the AAD had been adequately reduced, requiring an additional C1 laminectomy. The simultaneous presence of all these anomalies merited unique management considerations.


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