Journal of Child Neurology

 

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Journal of Child Neurology, Vol. 19, No. 6, 459-462 (2004)
DOI: 10.1177/088307380401900613

Syringomyelia in Twin Brothers Discordant for Chiari I Malformation: Case Report

R. Shane Tubbs

Department of Cell Biology University of Alabama at Birmircgham Birmingham, Alabama, and Pediatric Neurosurgery Children's Hospital Birmingham, Alabama, richard.tubbs{at}ccc.uab.edu

John C. Wellons

Pediatric Neurosurgery Children's Hospital Birmingham, Alabama

Jeffrey P. Blount

Pediatric Neurosurgery Children's Hospital Birmingham, Alabama

W. Jerry Oakes

Pediatric Neurosurgery Children's Hospital Birmingham, Alabama

Familial syringomyelia outside of trauma, tumor, or infection has been reported. Cases are presented that highlight the possible connection between familial syringomyelia and the Chiari 0 malformation. We report on 11-year-old twin brothers both with syringomyelia. Magnetic resonance imaging further revealed that one brother had Chiari I malformation and the other had Chiari 0 malformation. Both underwent posterior fossa decompression with radiologic improvement of their syringes. These case reports lend credence to earlier reports of improvement in syringomyelia following posterior fossa decompression in the absence of Chiari I malformation, the so-called Chiari 0 malformation. In addition, these case reports should influence the manner in which familial syringomyelia without tonsillar ectopia is defined and addressed, that is, posterior fossa decompression versus shunting of the syrinx, thereby addressing the potential cause of the syringomyelia and not only the enlarged cavity itself. (J Child Neurol 2004; 19:459-462).


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