History of Management of Dandy-Walker Syndrome in Children

Walter Edward Dandy, M.D.:

Arthur Earl Walker, M.D.:

Understanding of Disease

• First described in 1887: The condition now known as Dandy-Walker malformation was first described in 1887 by Sutton (4).
• Dandy and Blackfan described clinically in 1914: The first clinical description was provided by Dandy and Blackfan in 1914 (3). In the monograph they highlighted the now standard characteristics of hypoplasia of the cerebellar vermis and hydrocephalus associated with atresia of the foramina of Luschka and Magendie.
• Taggart and Walker expanded description in 1942: Taggart and Walker expanded on Dandy and Blackfan’s description in 1942 (5). They also spoke of the hypoplastic vermis and atretic foramina of Luschka and Magendie with associated hydrocephalus.
• Current pathogenic model described in 1954: Benda introduced a modified theory in 1954 that suggested that the congenital malformation of the cerebellar vermis results in cyst formation and fourth ventricular hydrocephalus (1).

Technological Development

• Shunts: Treatment of the Dandy-Walker syndrome has been advanced as the technology of shunting materials has improved.

Surgical Technique

• Shunts: Improved surgical technique for the insertion of shunts has improved the outcome in management of Dandy-Walker syndrome.
• Fenestration: The introduction of microsurgery and the advent of endoscopic surgery have progressively diminished the risk of morbidity associated with fenestration of the fourth ventricular cyst in the Dandy-Walker syndrome.