Dandy-Walker syndrome

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A variant of Arnold-Chiari syndrome characterized by a congenital brain malformation involving the fourth ventricle and cerebellum.

Description

A variant of Arnold-Chiari syndrome. A congenital brain malformation involving the fourth ventricle and cerebellum, marked by partial or complete absence of the cerebellar vermis, and posterior fossa cyst continuous with the fourth ventricle. Congenital hydrocephalus is caused by blockage of the foramina of Magendie and Luschka (the foramina of the fourth ventricle of the brain), through which the cerebrospinal fluid passes. Because of the blockage, the cerebrospinal fluid cannot pass into the subarachnoid space and in consequence accumulates in the ventricles.

Symptoms are vomiting, hyperirritability, convulsion. There is progressive enlargement of the head, congested veins in the scalp, bulging of anterior fontanelle, separated cranial sutures. Other complications may include papilledema, blepharoptosis, bradycardia, bradypnea and sixth cranial nerve paralysis. Develops in utero.

Aetiology uncertain. In some rare cases the disease is a dominant or recessive inheritable trait.

Dandy and Kenneth Daniel Blackfan (1883-1941) in 1914 described a 13 months old child with the combination of hydrocephalus, cysts in the posterior of the skull, and hypoplasia of the vermis of the cerebellum. Based on previous cases in the literature, they considered this to be a malformation triad caused by atresia of the foramina of the fourth ventricle of the brain, the foramina of Luschka and Magendie. Dandy in 1921 described the clinical picture. In February 1942, Walker and John Taggart participated in a meeting of the Chicago Neurological Society and gave a presentation entitled Congenital Atresia of the Foramens of Luschka and Magendie. In this talk, which was published as a comprehensive review in the Archives of Neurology and Psychiatry, they mentioned six cases in the literature, including the child documented by Dandy and Blackfan in 1914, and added three more cases from their own experience and defined it as an entity. The term "Dandy-Walker" was introduced by Benda in 1954. It is now recognised that the anatomical abnormality of the foramina that leads to internal hydrocephalus can be a component of several distinct syndromic entities.

The foraminas are named for Hubert von Luschka, German anatomist, 1820-1875; and Fran├žois Magendie, French physiologist, 1783-1855.

Bibliography

  • W. E. Dandy, K. D. Blackfan:
    Internal hydrocephalus: an experimental, clinical and pathological study.
    American Journal of Diseases of Children, Chicago, 1914, 8: 406-482.
    American Journal of Diseases of Children, Chicago, 1917, 14: 424-443.
  • W. E. Dandy:
    The diagnosis and treatment of hydrocephalus due to occlusion of the foramina of Magendie and Luschka.
    Surgery, Gynecology and Obstetrics, Chicago, 1921, 32: 112-124.
  • J. K. Taggart, A. E. Walker:
    Congenital atresia of the foramens of Luschka and Magendie.
    Archives of Neurology and Psychiatry, Chicago, 1942, 48: 583-612.
  • A. E. A. Walker:
    A case of congenital atresia of the foramina of Luschka and Magendie: Surgical cure.
    Journal of Neuropathology and Experimental Neurology, Lawrence, Kansas, 1944, 3: 368-373.
  • C. E. Benda:
    The Dandy-Walker syndrome or the so-called atresia of the foramen of Magendie.
    Journal of Neuropathology and Experimental Neurology, Lawrence, Kansas, 1954, 13: 14-39.
  • M. N. Hart, N. Malamud:
    The Dandy-Walker syndrome.
    Neurology, Minneapolis, 1972, 22: 771-780.

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