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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 ...
These symptoms seem to be present regardless of the malignancy of the melanin deposits within the central nervous system. [7] Approximately 10% of patient with neurocutaneous melanosis also present the Dandy–Walker syndrome and associated Dandy-Walker malformation.
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 ...
Developmental disorders including neural-tube defects, arachnoid cysts, Dandy–Walker malformations, and Arnold–Chiari malformations can cause primary hydrocephalus. Dandy-walker malformations and Arnold-Chiari malformations lead to structural abnormalities in the brain, which disrupts the flow of CSF and causes hydrocephalus. [26] [27]
A variety of brain abnormalities are also associated with 13q deletion. They can include epilepsy, craniosynostosis (premature closing of the skull bones), spastic diplegia, cerebral hypotrophy, underdevelopment or agenesis of the corpus callosum, cerebellar hypoplasia, deafness, and, rarely, hydrocephalus, Dandy–Walker syndrome, and spina ...
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[4] [5] 75% of children with 3C syndrome have Dandy-Walker malformation and hydrocephalus. [6] Signs and symptoms in other body systems are also associated with 3C syndrome. In the skeletal system, ribs may be absent, and hemivertebrae, syndactyly (fusion of fingers together), and clinodactyly (curvature of the fifth finger) may be present.