Rhombencephalosynapsis and a Chiari II malformation

Rhombencephalosynapsis is an anomaly of the hindbrain characteristically presenting with cerebellar fusion and absence of cerebellar vermis on magnetic resonance imaging. Its association with spinal anomalies has not been reported previously. We report a unique case, a 22-year-old man with cerebella...

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Veröffentlicht in:Journal of computer assisted tomography 2003-03, Vol.27 (2), p.257-259
Hauptverfasser: SENER, R. N, DZELZITE, S
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description Rhombencephalosynapsis is an anomaly of the hindbrain characteristically presenting with cerebellar fusion and absence of cerebellar vermis on magnetic resonance imaging. Its association with spinal anomalies has not been reported previously. We report a unique case, a 22-year-old man with cerebellar fusion associated with a cervicothoracic meningomyelocele, diastematomyelia, tethering of the spinal cord, and dorsal dermal sinuses. In addition, cerebellar tonsillar herniation and tectal beaking similar to that seen in Chiari II malformations were present. These findings suggest that rhombencephalosynapsis can be associated with spinal malformations and, furthermore, that cases with the common features of rhombencephalosynapsis and a Chiari II malformation can exist. Such an association likely represents a new anomaly of the hindbrain and spine.
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subjects Adult
Arnold-Chiari Malformation - complications
Arnold-Chiari Malformation - diagnosis
Biological and medical sciences
Cerebellum - abnormalities
Cerebellum - diagnostic imaging
Encephalocele - complications
Encephalocele - diagnosis
Humans
Magnetic Resonance Imaging
Male
Malformations of the nervous system
Medical sciences
Meningocele - complications
Meningocele - diagnosis
Neurology
Radiography
Rhombencephalon - abnormalities
Rhombencephalon - diagnostic imaging
title Rhombencephalosynapsis and a Chiari II malformation
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