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Dandy–Walker malformation (DWM), also known as Dandy–Walker syndrome (DWS), is a rare congenital brain malformation in which the part joining the two hemispheres of the cerebellum (the cerebellar vermis) does not fully form, and the fourth ventricle and space behind the cerebellum (the posterior fossa) are enlarged with cerebrospinal fluid.
Craniosynostosis-Dandy-Walker malformation-hydrocephalus syndrome (HCDPH1, also known as Sagittal craniosynostosis, Dandy-Walker malformation and hydrocephalus, Dandy-Walker malformation with sagittal craniosynostosis and hydrocephalus, Braddock-Jones-Superneau syndrome, or simply Hydrocephalus, autosomal dominant) is an autosomal dominant syndrome characterized by sagittal craniosynostosis ...
Developmental disorders including neural-tube defects, arachnoid cysts, Dandy–Walker syndrome, and Arnold–Chiari malformation can also cause primary hydrocephalus. About 80–90% of fetuses or newborn infants with the neural-tube defect spina bifida —often associated with meningocele or myelomeningocele —develop hydrocephalus.
Dandy Walker malformation is a relatively common congenital brain malformation with a prevalence of 1:30,000 live births. [17] Dandy Walker malformation is characterized by enlarged posterior fossa and in which the cerebellar vermis is completely absent, or present in a rudimentary form, sometimes rotated accompanied by an elevation of the ...
All patients showed cerebellar and/or vermal dysfunction and, on formal psychometric testing, cognitive abilities ranged from normal to moderately retarded. Abnormalities on CT scan ranged from prominent valleculla to an enlarged cisterna magna with hypoplasia of the cerebellar hemispheres and vermis.
The abnormalities of the leptomeninges during fetal development due to neurocutaneous melanosis may be the cause of this increased incidence of the Dandy-Walker malformation. The development of hydrocephalus is the most common symptom associated with a combination of neurocutaneous melanosis and a Dandy-Walker malformation, occurring in about ...
Agenesis of the corpus callosum (ACC) is a rare birth defect in which there is a complete or partial absence of the corpus callosum.It occurs when the development of the corpus callosum, the band of white matter connecting the two hemispheres in the brain, in the embryo is disrupted.
Dandy-Walker malformation is also occasionally seen in Ellis–Van Creveld syndrome, which is characterized by heart defects and malformed alveolar ridge. [5] Many disorders include the Dandy–Walker phenotype and thus it is not pathognomonic for 3C syndrome. [10] CHARGE syndrome can also be misdiagnosed. This is because both CHARGE syndrome ...