Asymptomatic Dandy Walker variant in adult

Abstract

Introduction Dandy-Walker Malformation is a rare congenital malformation which is characterized by the agenesis or hypoplasia of vermis cerebellum, cyst dilation of 4th ventricle and enlargement of posterior fossa. The incidence of Dandy-Walker Malformation (DWM) in USA is one case over 25.000-35.000 in every birth rate, with 40% of them are females and 60% are males. Meanwhile, there is still no definite incidence of Dandy-Walker Malformation in Indonesia. There are 11 articles reported asymptomatic DWM. Case report A 23 years old female presented with moderate and persistent headache, with no aura or hallucination since 1 year before admission. There were no symptoms of increased intrakranial pressure. There is no history head trauma and no history of developmental disorders while childhood. The neurological physical examination, fundus photos, OCT and humphrey from the eye part within normal limits. The EEG shows a normal EEG. The head CT-Scan and head MRI of the contras that revealed agenesis of cerebellar vermis and extensive CSF cysts at the cysterna magna with the enlarged fourth ventricle suggestive of Dandy-Walker malformation without hydrocephalus. In these patients managed conservative therapy and follow-up were carried out without surgical therapy. It seems that in the case of patients with asymptomatic DWS, CSF dynamics compensate for malformations. Conclusion There are important aspects should be followed up in asymptomatic Dandy Walker Malformation patients, there are head MRI to determine the present of hydrocephalus, high ICP signs, and cerebellum symptoms examination.