title: Dandy-walker malformation and down syndrome association: good developmental outcome and successful endoscopic treatment of hydrocephalus.
abstract: 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.
authors.affiliation: Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Flavio
authors.initials: F
authors.lastname: Nigri
authors.affiliation: Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Isaias Fiuza
authors.initials: IF
authors.lastname: Cabral
authors.affiliation: Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Raquel Tavares Boy
authors.initials: RT
authors.lastname: da Silva
authors.affiliation: Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Heloisa Viscaíno
authors.initials: HV
authors.lastname: Pereira
authors.affiliation: Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Carlos Roberto Telles
authors.initials: CR
authors.lastname: Ribeiro
keywords: Dandy-Walker malformation; Down syndrome; Prenatal diagnosis