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

Overview

Historical Perspective

  • The term Dandy-Walker Syndrome was introduced in 1954 by a German psychiatrist Clemens Benda in the light of the following discoveries,[1]
    • 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.[2]
    • 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.[3]
  • 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.[4]

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 during the development of cerebellar vermis leading to a fourth ventricle that is in continuation with the posterior fossa subarachnoid space.[5]
  • 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. [5]
  • The exact pathogenesis of hydrocephalus in Dandy-Walker Syndrome is not fully understood. Several factors could play a role in development of hydrocephalus.
    • The initial hypothesis of atresia of foramen of Luschka and Magendie as a possible cause is not well supported. It was found later that these foramens are patent in a large number of patients with DWM and hydrocephalus is not present at birth in more than 80% of patients diagnosed with DWM. Moreover, the closure of one or two foramen can be compensated by presence of other foramens preventing collection of fluid.[6]
    • Another hypothesis was proposed suggesting the outflow impairment to be distal to fourth ventricle outlets, possibly caused by inflammation of arachnoid mater causing outflow obstruction. Excisions of these obstructions have not been able to show whether impaired arachnoid absorption is involved, since the subarachnoid space always takes days to weeks to fill up following excision. [5]
    • Stenosis of aqueduct of Sylvius once suggested, does not seem to be the causing factor in pathogenesis of hydrocephalus. Shunts placed in the posterior fossa cyst almost always drain all above ventricles. Occasionally when it is present, it is functional stenosis caused by herniation of posterior fossa content. [5]
    • Increased pressure in venous sinuses due to compression from the posterior fossa cyst could also contribute to the pathogenesis or worsening of hydrocephalus, no evidence has been found so far.[5]
    • The importance of understanding the pathogenesis of hydrocephalus in Dandy-Walker Syndrome forms the basis of choosing most appropriate treatment.

Causes

References=

  1. Benda, Clemens E. (1954). "The Dandy-Walker Syndrome or The So-Called Atresia of the Foramen Magendie*". Journal of Neuropathology & Experimental Neurology. 13 (1): 14–29. doi:10.1093/jnen/13.1.14. ISSN 1554-6578.
  2. Dandy, Walter E. (1914). "AN EXPERIMENTAL, CLINICAL AND PATHOLOGICAL STUDY". American Journal of Diseases of Children. VIII (6): 406. doi:10.1001/archpedi.1914.02180010416002. ISSN 0096-8994.
  3. Taggart, John K. (1942). "CONGENITAL ATRESIA OF THE FORAMENS OF LUSCHKA AND MAGENDIE". Archives of Neurology And Psychiatry. 48 (4): 583. doi:10.1001/archneurpsyc.1942.02290100083008. ISSN 0096-6754.
  4. Sutton, J. Bland (1886). "THE LATERAL RECESSES OF THE FOURTH VENTRICLE; THEIR RELATION TO CERTAIN CYSTS AND TUMOURS OF THE CEREBELLUM, AND TO OCCIPITAL MENINGOCELE". Brain. 9 (3): 352–361. doi:10.1093/brain/9.3.352. ISSN 0006-8950.
  5. 5.0 5.1 5.2 5.3 5.4 Spennato, Pietro; Mirone, Giuseppe; Nastro, Anna; Buonocore; Ruggiero, Claudio; Trischitta, Vincenzo; Aliberti, Ferdinando; Cinalli, Giuseppe (2011). "Hydrocephalus in Dandy–Walker malformation". Child's Nervous System. 27 (10): 1665–1681. doi:10.1007/s00381-011-1544-4. ISSN 0256-7040. Unknown parameter |sufirst4= ignored (help)
  6. Takami, Hirokazu; Shin, Masahiro; Kuroiwa, Masafumi; Isoo, Ayako; Takahashi, Kan; Saito, Nobuhito (2010). "Hydrocephalus associated with cystic dilation of the foramina of Magendie and Luschka". Journal of Neurosurgery: Pediatrics. 5 (4): 415–418. doi:10.3171/2009.10.PEDS09179. ISSN 1933-0707.

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