<unk>  occurs in utero due to disruption in the development of the cerebellar vermis. It is characterized by the presence of hypoplastic cerebellar vermis, hydrocephalus, and cystic dilation of fourth ventricle. It usually presents itself in the first year of life due to symptoms caused by hydrocephalus. MRI is the diagnostic study of choice and surgical placement of shunt is the mainstay of therapy to reduce the hydrocephalus and to minimize brain damage.
Dandy-Walker syndrome
Robinow syndrome
Walker-Warburg syndrome
Foster Kennedy syndrome
Cantu syndrome
Krabbe disease
Cleidocranial dysplasia
Tolosa-Hunt syndrome