Dandy-Walker malformation: a review of 78 cases diagnosed by prenatal sonography

HAS R., ERMIS H., YUKSEL A., IBRAHIMOGLU L. E., Yildirim A., SEZER H., ...Daha Fazla

FETAL DIAGNOSIS AND THERAPY, cilt.19, sa.4, ss.342-347, 2004 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 19 Sayı: 4
  • Basım Tarihi: 2004
  • Doi Numarası: 10.1159/000077963
  • Dergi Adı: FETAL DIAGNOSIS AND THERAPY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.342-347
  • Anahtar Kelimeler: Dandy-Walker malformation, Dandy-Walker variant, prenatal diagnosis, outcome of Dandy-Walker malformation, ENLARGED CISTERNA MAGNA, ABNORMALITIES
  • İstanbul Üniversitesi Adresli: Evet

Özet

Objective: The purpose of this study was to determine the associated abnormalities and clinical outcomes of fetuses with Dandy-Walker malformations. Methods: Sonograms and medical reports of 78 cases were reviewed and information regarding each outcome was collected from autopsy records, hospital charts and specialists caring for the surviving infants. Results: We identified 64 fetuses with classic Dandy-Walker malformation (DW) and 14 fetuses with Dandy-Walker variant (DWV). A high proportion (44.8%) of the parents were consanguineous. The spectrum and proportion of central nervous system (67.1 vs. 71.4%; p = 1.0) and other malformations (43.7 vs. 64.2%; p = 0.57) associated with DW and DWV were similar. Chromosome abnormalities were found in 9 of the 51 (17.6%) fetuses that underwent karyotype analysis. Only 4 of 64 (6.2%) DW and 3 of 14 (21.4%) DWV infants survived (p 0.14), and all surviving infants with DW or DWV had neurological disorders. Conclusions: DW and DWV cases show so many similarities that a clear-cut distinction is difficult. There was no significant difference in the spectrum of associated anomalies and postnatal prognosis between DW and DWV cases. Copyright (C) 2004 S. Karger AG, Basel.