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Dandy-Walker Syndrome (Dandy Walker Malformation)

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; Syndrome, Dandy-Walker; Dandy-Walker Syndrome, Familial; Dandy Walker Syndrome; Dandy Walker Syndrome, Familial; Familial Dandy-Walker Syndrome; Malformation, Dandy-Walker; Syndrome, Familial Dandy-Walker; Dandy-Walker Malformation
Networked: 161 relevant articles (0 outcomes, 6 trials/studies)

Disease Context: Research Results

Related Diseases

1. Cysts
2. Hydrocephalus (Hydrocephaly)
3. Chromosome Aberrations (Chromosome Abnormalities)
4. Arachnoid Cysts (Arachnoid Cyst)
5. Cleft Palate (Palate, Cleft)

Experts

1. Volpe, P: 4 articles (02/2014 - 05/2006)
2. Contro, E: 3 articles (02/2014 - 06/2012)
3. Pilu, G: 3 articles (02/2014 - 06/2012)
4. Ghi, T: 3 articles (02/2014 - 06/2012)
5. Tonni, Gabriele: 2 articles (12/2014 - 09/2013)
6. Chitayat, David: 2 articles (12/2014 - 06/2012)
7. Dobyns, William B: 2 articles (03/2014 - 11/2010)
8. De Musso, F: 2 articles (02/2014 - 06/2012)
9. Volpe, G: 2 articles (06/2012 - 06/2012)
10. Unal, Ozkan: 2 articles (03/2010 - 01/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Dandy-Walker Syndrome:
1. Dandy Walker variantIBA
2. Neurocutaneous melanosisIBA
3. tranilast (N 5')IBA
4. Renal hepatic pancreatic dysplasia Dandy Walker cystIBA
5. tetrasomy 9p Chromosome 9IBA
6. Molybdenum cofactor deficiencyIBA
7. trisomy Chromosome 9IBA
8. Keratitis, Ichthyosis, and Deafness (KID) SyndromeIBA
9. Meckel syndrome type 1IBA
10. Warfarin (Coumadin)FDA LinkGeneric

Therapies and Procedures

1. Colonic Pouches (S Pouch)
02/01/2014 - "The study group of 11 fetuses included seven with Dandy-Walker malformation and four with Blake's pouch cyst. "
11/01/2014 - "Fetuses were grouped, according to malformation, into eight categories: cobblestone malformation complex (CMC, n = 3), Chiari-II malformation (C-II, n = 7), pontocerebellar hypoplasia (PCH, n = 2), rhombencephalosynapsis (RES, n = 4), Dandy-Walker malformation (n = 8), vermian dysgenesis (VD, n = 7), persistent Blake's pouch cyst (n = 6) and megacisterna magna (n = 6). "
02/01/2014 - "However, fetuses with Blake's pouch cyst and normal fetuses with an open fourth ventricle had strikingly similar sonograms: the BV angle was between 20° and 37° and the fourth ventricle appeared open only when viewed using a more steeply angulated scanning plane than that of the standard transcerebellar plane; in fetuses with Dandy-Walker malformation the fourth ventricle was widely open posteriorly, even in the standard transcerebellar view, and the BV angle was > 45°, significantly increased compared both with that in normal fetuses (P < 0.0001) and with that in fetuses with Blake's pouch cyst (P = 0.004). "
06/01/2012 - "From mid gestation, a BV angle > 45° is strongly suggestive of a Dandy-Walker malformation, while a measurement < 30° favors the diagnosis of a Blake's pouch cyst."
06/01/2012 - "The BV angle was significantly increased in each of the three subgroups of anomalies: Blake's pouch cyst (23 ± 2.8°; range, 19-26°), vermian hypoplasia (34.9 ± 5.4°; range, 24-40°) and Dandy-Walker malformation (63.5 ± 17.6°; range, 45-112°), the angle increasing with increasing severity of the condition. "
2. Ventriculoperitoneal Shunt
3. Ventriculostomy
4. Catheters
5. Decompression