Dandy-Walker Syndrome

A congenital abnormality of the central nervous system marked by failure of the midline structures of the cerebellum to develop, dilation of the fourth ventricle, and upward displacement of the transverse sinuses, tentorium, and torcula. Clinical features include occipital bossing, progressive head enlargement, bulging of anterior fontanelle, papilledema, ataxia, gait disturbances, nystagmus, and intellectual compromise. (From Menkes, Textbook of Child Neurology, 5th ed, pp294-5)

Also Known As:

Dandy Walker Malformation; Dandy-Walker Complex; Dandy-Walker Cyst; Dandy-Walker Deformity; Dandy-Walker Syndrome, Familial; Hydrocephalus, Internal, Dandy-Walker Type; Hydrocephalus, Noncommunicating, Dandy-Walker Type; Luschka-Magendie Foramina Atresia; Cyst, Dandy-Walker; Cysts, Dandy-Walker; Dandy Walker Complex; Dandy Walker Deformity; Dandy Walker Syndrome; Dandy Walker Syndrome, Familial; Dandy-Walker Complices; Dandy-Walker Cysts; Dandy-Walker Deformities; Familial Dandy-Walker Syndrome; Luschka Magendie Foramina Atresia; Malformation, Dandy-Walker; Dandy-Walker Malformation

Nervous System Diseases: 14737
- Central Nervous System Diseases: 4371
  - Brain Diseases: 16141
    - Hydrocephalus: 7081
      - Dandy-Walker Syndrome: 122
    - Cerebellar Diseases: 647
      - Dandy-Walker Syndrome: 122
- Nervous System Malformations: 36
  - Dandy-Walker Syndrome: 122
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 960
- Congenital Abnormalities: 26404
  - Nervous System Malformations: 36
    - Dandy-Walker Syndrome: 122

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