Dandy-Walker malformation : a review of cases diagnosed by prenatal ultrasonography
Journal Title: Journal of the Indian Medical Association - Year 2018, Vol 116, Issue 3
Abstract
Dandy-Walker (DW) malformation is characterised by agenesis of cerebellar vermis, enlarged posterior fossa and elevation of tentorium. It may be isolated or associated with other systemic anomalies. The purpose of this case series was to observe abnormalities diagnosed by prenatal ultrasonography (USG) and study the clinical outcomes of fetuses. We reviewed seven cases of Dandy-Walker malformation, all of whom presented differently. A proportion of parents were consanguineous (two cases). Four had severe early onset intrauterine growth restriction (UGR), two with associated oligohydramnios. Routine scan prior to 20 weeks enabled detection in two cases. Interestingly we had one case of recurrent DW. A posterior fossa cyst with defective or absent vermis was the sonographic feature in all cases. Two cases had extracranial malformations. Fetal karyotype was normal. Excluding three terminated pregnancies, two were neonatal deaths. Autopsy confirmation was possible in two cases. One surviving infant is being followed up for mild developmental delay. When diagnosed in utero, it can be extremely difficult to ascertain the extent of associated malformations and prognosticate the postnatal outcome. Fetal karyotype and echocardiogram (ECHO) can be useful. Diagnosis prior to 20 weeks enables termination of pregnancy since neurological outcome and prognosis remain guarded in such cases.
Authors and Affiliations
Nimisha Agarwal
Keywords
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