A Case Report Of A Dandy-Walker Syndrome In A Girl Baby

Dandy-Walker anomaly is a rare congenital disorder. This syndrome is characterized by complete or partial agenesis of the cerebellar vermis, cystic dilatation of the fourth ventricle and enlargement of the posterior cavity with or without hydrocephalus. This neurological syndrome is more common in girls than in boys. The most important abnormality is the presence of a large dilatation of the fourth ventricle, which has been cysts and has been covered by a neuroglial vascular membrane. Hydrocephalus is not seen at birth, often occurs at 3 months, and is proven at 1 year in 80% of cases. In addition to hydrocephalus, pale, ataxia, abnormal breathing, seizures and prominent occiput may be present. Mental retardation is common due to brain abnormalities in these infants; a major fetal defect in this disorder is still discussed. Pre-birth ultrasonography may show hydrocephalus and ventricular enlargement. Postpartum diagnosis is by CT scan and MRI. In the case of progressive hydrocephalus, the treatment is surgical.