1. Craniocervical instability in an infant with partial sacral agenesis.
- Author
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Thiryayi WA, Alakandy LM, Leach PA, and Cowie RA
- Subjects
- Abnormalities, Multiple diagnostic imaging, Atlanto-Axial Joint pathology, Atlanto-Axial Joint surgery, Bone Transplantation, Cervical Vertebrae pathology, Cervical Vertebrae surgery, Child, Preschool, Craniofacial Abnormalities diagnosis, Decompression, Surgical, Female, Follow-Up Studies, Humans, Image Processing, Computer-Assisted, Infant, Joint Dislocations diagnosis, Joint Dislocations surgery, Joint Instability diagnosis, Joint Instability surgery, Magnetic Resonance Imaging, Nervous System Malformations diagnosis, Occipital Bone pathology, Occipital Bone surgery, Odontoid Process pathology, Odontoid Process surgery, Sacrum pathology, Spinal Cord Compression diagnosis, Spinal Cord Compression surgery, Tomography, X-Ray Computed, Abnormalities, Multiple surgery, Atlanto-Axial Joint abnormalities, Cervical Vertebrae abnormalities, Joint Dislocations congenital, Joint Instability congenital, Nervous System Malformations surgery, Odontoid Process abnormalities, Sacrum abnormalities, Spinal Fusion
- Abstract
Unlabelled: Sacral agenesis is an uncommon condition characterised by total or partial absence of the sacrum. The association of this condition with craniocervical junction abnormalities is extremely rare., Case Report: We describe a 3-year-old girl who, at the age of 8 months, while being investigated for short stature, was found to have significant craniocervical instability associated with anomalies of the odontoid peg. In addition to this she had partial sacral agenesis but genetic tests showed a normal karyotype. Due to the inherent difficulty of surgical fixation and immobilisation in an infant of her age, she was managed conservatively in a soft cervical orthosis. At the age of three years, surgery for decompression and stabilisation was deemed necessary due to the onset of neurological morbidity., Conclusion: The authors describe this extremely rare association and discuss the difficulties faced while deciding the optimum surgical strategy for managing such young children with craniocervical instability.
- Published
- 2007
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