Dandy-Walker Malformation and Down Syndrome Association: Good Developmental Outcome and Successful Endoscopic Treatment of Hydrocephalus

The association of Down syndrome (DS) with Dandy Walker malformation (DWM) is extremely rare, with only 3 cases reported to date. All cases reported have shown a bad life expectancy and a bad developmental outcome. The present case reveals the possibility of a good prognosis. A 19-month-old male pat...

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Bibliographic Details
Published inCase reports in neurology Vol. 6; no. 2; pp. 156 - 160
Main Authors Nigri, Flavio, Fiuza Cabral, Isaias, Boy da Silva, Raquel Tavares, Viscaíno Pereira, Heloisa, Telles Ribeiro, Carlos Roberto
Format Journal Article
LanguageEnglish
Published Basel, Switzerland S. Karger AG 14.05.2014
Karger Publishers
Subjects
Dandy-Walker malformation
Down syndrome
Prenatal diagnosis
Published: May 2014
Dandy-Walker malformation
Prenatal diagnosis
Down syndrome
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Summary:The association of Down syndrome (DS) with Dandy Walker malformation (DWM) is extremely rare, with only 3 cases reported to date. All cases reported have shown a bad life expectancy and a bad developmental outcome. The present case reveals the possibility of a good prognosis. A 19-month-old male patient had successful endoscopic hydrocephalus treatment and a good developmental outcome. He probably had a better outcome because of good DS and DWM prognostic parameters. Our patient suffered from a DWM with vermis identification of 2 fissures and 3 lobes and a DS with a well-preserved tonus, which was not associated with other congenital systemic defects. We may conclude that the prognosis of DS-DWM association may separately depend on the degree of clinical and neurological involvement of each malformation.
ISSN:1662-680X
1662-680X
DOI:10.1159/000363179