DANDY WALKER MALFORMATION - ANTENATAL ULTRASOUND

 

A 31 year old female with 9 months of amenorrhoea came to emergency for antenatal scan. No previous ultrasound scan was available.

                                                
     

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Dandy-Walker malformation (DWM) is characterized classically by the triad of cystic dilatation of the fourth ventricle, cerebellar vermis hypoplasia, and hydrocephalus.
DWM is commonly associated with an enlarged posterior fossa, high confluence of the dural venous sinuses (torcular herophili), and higher displacement of the cerebellar hemispheres.
DWM has an incidence of 1 in every 35,000 live births.
Hydrocephalus was considered an essential diagnostic element of DWM, but more recent evidence suggests that it is not present at birth in most patients, though it may develop later in life. In 75 % cases, it develops by 3 months of age.
DWM is associated with a high incidence of motor dysfunction and mental retardation (40%). Most of the symptoms and signs of DWM are related to the hydrocephalus complications.
Hypoplasia/aplasia of the cerebellar vermis with marked dilatation of the fourth ventricle causing a “keyhole configuration” .