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Dandy–Walker malformation: prenatal diagnosis and outcome

✍ Scribed by Nicole Kölble; Josef Wisser; Josef Kurmanavicius; Eugen Bolthauser; Thomas Stallmach; Albert Huch; Renate Huch

Publisher
John Wiley and Sons
Year
2000
Tongue
English
Weight
304 KB
Volume
20
Category
Article
ISSN
0197-3851

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✦ Synopsis

Prenatal ultrasound identi®ed Dandy±Walker malformation (DWM) in ten singleton pregnancies with concurrent central nervous system (CNS) anomalies and extra-CNS anomalies in eight cases. DWM was con®rmed by postnatal magnetic resonance imaging (MRI) or pathological examination in nine cases. Karyotypes were normal in the seven infants tested. Postnatal neurological and developmental testing in the ®ve survivors showed a spectrum of clinical outcome from minor defects to severe handicap. Postnatal investigation also disclosed additional CNS and extra-CNS ®ndings not detected on ultrasound, as did autopsy in the other ®ve infants. However, ultrasound diagnosis of DWM is accurate and is an indication for exhaustive screening for concurrent anomalies both within and outside the CNS and in chromosome structure and number, as the prognosis is heavily dependent on associated malformations and karyotype.

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