Dandy-Walker Syndrome or the so-called atresia of the foramen Magendie

Dandy-Walker malformation (DWM) was first used in 1954 to describe the combination of a cystic dilation of the fourth ventricle and a hypoplastic cerebellar vermis. Since that time, the eponym has undergone modifications.

The Dandy-Walker malformation (DWM) is a spectrum of posterior fossa abnormality (Dandy-Walker complex) the key feature of which is complete or partial agenesis of the cerebellar vermis.

DWM likely results from a defect in the embryologic development of loose connective tissue of the pia mater within the fourth ventricle, creating a dorsal out-pouching and variable vermian hypoplasia. The result is often an obstructive form of hydrocephalus secondary to inadequate fourth ventricle cerebrospinal fluid (CSF) drainage.

 Dandy-Walker malformation (DWM) :

• Complete absence of the cerebellar vermis, or severe unequivocal hypoplasia of the inferior vermis.
• A posterior fossa CSF collection in direct communication with the fourth ventricle.
• An unequivocally enlarged posterior fossa.

Dandy-Walker Malformation (DWM).

Dandy-Walker variant (DWV) :

• Inferior vermis hypoplasia.
• Posterior fossa CSF collection in direct communication with the fourth ventricle.
• No obvious enlargement of the posterior fossa.

Diagnosis :

Dandy Walker Syndrome can be diagnosed through the following examinations:

1. MRI – This has been noted as one of the best diagnostic exam for neurological or CNS related conditions.
2. CT scan – This procedure assists in the diagnosis of Dandy Walker syndrome. There are 3D studies that are good in the evaluation process of DWS.
3. Angiography –This can demonstrate or provide the features of malformations of Dandy Walker syndrome.
4. Ultrasonography – The malformation can be identified through the antenatal ultrasound.