Cleidocranial dysostosis and syringomyelia. Review of the literature and case report.
A 34-year-old woman with congenital cleidocranial dysostosis had scoliosis diagnosed at age 13. The scoliosis was treated during adolescence with spinal orthoses but continued to progress after skeletal maturation. She was diagnosed as having syringomyelia at age 34. The possibility of syringomyelia should be considered in patients with cleidocranial dysostosis, especially those with neurologic symptoms, scoliosis not responding to conservative therapy, or progressive scoliosis after skeletal maturation. Although only two previous patients have been reported in the literature, the condition undiagnosed could be a more common problem than generally supposed.
D000328:Adult; D002973:Cleidocranial Dysplasia / Q000150:complications*; D005260:Female; D006801:Humans; D012600:Scoliosis / Q000150:complications; D013595:Syringomyelia / Q000150:complications* / Q000000981:diagnostic imaging; D013997:Time Factors; D014057:Tomography, X-Ray Computed