Rhombencephalosynapsis
etiology:
Rhombencephalosynapsis consists of
Rhombencephalosynapsis consists of
- congenital fusion of cerebellar hemispheres, dentate nucleus and superior cerebellar peduncles .
-This is associated with vermian agenesis or hypogenesis. Rhombencephalosynapsis differs from Dandy-Walker malformation in that the anterior vermis is absent and posterior vermis is deficient .
-Transverse diameter of cerebellum is reduced.
-This may be accompanied by corpus callosal dysgenesis, aqueductal stenosis leadig to hydrocephalus, septo-optic dysplasia or holoprosencephaly.
Figure 10: Rhombencephalosynapsis. A, Sagittal T1 spin-echo image shows abnormal cerebellar vermis. B, Axial T2 spin-echo image shows continuity of the cerebral hemispheres across the midline without a midline cerebellar vermis. The cerebral hemispheres are abnormal, with reduced white matter and inward folding of the cortex as a result of ventriculoperitoneal shunting.
Figure 10: Rhombencephalosynapsis. A, Sagittal T1 spin-echo image shows abnormal cerebellar vermis. B, Axial T2 spin-echo image shows continuity of the cerebral hemispheres across the midline without a midline cerebellar vermis. The cerebral hemispheres are abnormal, with reduced white matter and inward folding of the cortex as a result of ventriculoperitoneal shunting.
(A)
(B)Reference:The Internet Journal of Radiology™ ISSN: 1528-8404
