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Dandy-Walker malformation diagnosed before 21 weeks of gestation: associated malformations and chromosomal abnormalities.

Abstract
This study examined rates of concomitant structural and chromosomal abnormalities in 14 fetuses with a diagnosis of Dandy-Walker malformation or Dandy-Walker variant before 21 weeks' gestational age, compared to 14 fetuses with a diagnosis of Dandy-Walker malformation or variant between 21 weeks' gestation and delivery. A total of 24 fetuses had Dandy-Walker malformation and four had Dandy-Walker variant. Eight of the fetuses with the malformation had ventriculomegaly: one of the fetuses with early diagnosis and seven with later diagnosis (p = 0.027). None of the fetuses with Dandy-Walker variant had ventriculomegaly. The overall prevalence of concomitant structural abnormalities was 13/28; 8/14 for fetuses with early prenatal diagnosis and 5/14 for fetuses with late prenatal diagnosis of the malformation or the variant. Chromosomal abnormality rates were significantly higher among fetuses with early prenatal diagnosis (7/14) than among those with later prenatal diagnosis (1/14; p = 0.032). Abnormal karyotypes were more prevalent among fetuses without ventriculomegaly (7/20), compared to fetuses with ventriculomegaly (1/8). We conclude that fetuses with an antenatal diagnosis of Dandy-Walker malformation or Dandy-Walker variant before 21 weeks' gestational age have worse prognosis than fetuses with a later prenatal diagnosis of the same defect.
AuthorsB Ulm, M R Ulm, J Deutinger, G Bernaschek
JournalUltrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology (Ultrasound Obstet Gynecol) Vol. 10 Issue 3 Pg. 167-70 (Sep 1997) ISSN: 0960-7692 [Print] England
PMID9339523 (Publication Type: Comparative Study, Journal Article)
Topics
  • Adult
  • Chromosome Aberrations (diagnostic imaging)
  • Chromosome Disorders
  • Chromosomes, Human, Pair 13
  • Chromosomes, Human, Pair 18
  • Confidence Intervals
  • Dandy-Walker Syndrome (diagnostic imaging)
  • Diagnosis, Differential
  • Female
  • Fetal Death (epidemiology)
  • Fetal Diseases (diagnostic imaging)
  • Gestational Age
  • Humans
  • Infant, Newborn
  • Intracranial Arteriovenous Malformations (diagnostic imaging)
  • Pregnancy
  • Pregnancy Outcome
  • Retrospective Studies
  • Sensitivity and Specificity
  • Trisomy
  • Ultrasonography, Prenatal

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