Melinda Bechtell
CMI 352
13, May, 2016
Dandy walker syndrome
Dandy Walker syndrome (DWS) is a congenital brain defect that occurs in the cerebellum and the brain of the fourth ventricle during the first trimester. It is a genetic disorder and affects infants and children. Reason for the Dandy-Walker syndrome child's presence is teratogenic factor, which adversely affect fetal development. There are, at present, three identified types of Dandy–Walker complexes. These represent closely associated forms of the disorder: DWS malformation, DWS mega cisterna magna and DWS variant.
DWS malformation is one of the most severe forms of DWS. Although, it is uncommon, and appears in 1 – 25,000/30,000 pregnancy cases. According to Carol …show more content…
It communicates with the fourth ventricle via the foramina of Magendie and Luschka. Measurement of the anteroposterior diameter of the cisterna magna can be useful in diagnosing posterior fossa abnormalities. Mega Cisterna Magna is characterized by an enlarged posterior fossa despite normal or very nearly normal size of the cerebellum. The increased size, more than 10mm, is associated with an enlarged fluid collection beneath and often behind the cerebellum. This has been reported as a normal variant, but emerging experience suggests that it may be associated with developmental disabilities although these are usually less severe than seen in DWS malformation. If Mega Cisterna Magna has been discovered, it is highly recommended to look closer for other anomalies. Although, it should not be confused with arachnoid cyst, which is benign cerebrospinal fluid collection within arachnoid space. Spyros S. Kollias at al. mentioned in their embriologic analysis that “They do not communicate with the ventricular system or the subarachnoid space, which can help distinguish them from a Dandy-Walker malformation. Usually the vermis and cerebellum appear intact. They can be associated with hydrocephalus and a mass effect. They are usually sporadic. Clinical manifestations depend on the size of the cyst and the degree of hydrocephalus”