Dandy-Walker syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Zehra Malik, M.B.B.S[2]

Synonyms and keywords: Dandy-Walker Malformation, Dandy-Walker Deformity

Historical Perspective

• The term Dandy-Walker Syndrome was introduced in 1954 by a German psychiatrist Clemens Benda in the light of the following discoveries,
  • In 1914, American neurosurgeon Walter Dandy and American pediatrician Kenneth Blackfan recognized the association between the partial or complete absence of cerebellar vermis, hydrocephalus, and fourth ventricular enlargement.
  • In 1942, Canadian-American neurosurgeon Arthur Earl Walker and American physician John Taggart contributed by highlighting the possible cause to be the maldevelopment of the foramen of Lushka and Magendie.
• An English surgeon named John Bland Sutton was the first to describe the association of underdeveloped cerebellar vermis, hydrocephalus, and an enlarged posterior fossa in 1887.

Classification

• There is no established system for the classification of Dandy-Walker Syndrome.

Pathophysiology

• It is thought that Dandy-Walker Syndrome is the result of disruptions that occur in the development of cerebellar vermis leading to a fourth ventricle that is in continuation with the posterior fossa subarachnoid space.
• Embryonic development of cerebellum starts at week 5, it forms from the top part of metencephalon. The cerebellar hemisphere is formed from the forward surface of fourth ventricle. The lack of midline fusion of the cerebellar hemisphere by the 15th week of embryonic development results into underdeveloped cerebellar vermis.