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:
- MRI – This has been noted as one of the best diagnostic exam for neurological or CNS related conditions.
- 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.
- Angiography –This can demonstrate or provide the features of malformations of Dandy Walker syndrome.
- Ultrasonography – The malformation can be identified through the antenatal ultrasound.