RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164067470 T1 Dandy-Walker Malformation T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accesspediatrics.mhmedical.com/content.aspx?aid=1164067470 RD 2024/10/03 AB It is classified as a rare congenital human brain anomaly of the cerebellum and fourth ventricle. It is characterized by hypoplasia of the cerebellum, absence of the cerebellar vermis, and consequently noncommunicating hydrocephalus that is caused by cystic expansion of the fourth ventricle in the posterior fossa. The clinical features include abnormal motor coordination, raised intracranial pressure due to accumulation of the cerebrospinal fluid, and other multiple brain function problems. It is typically a lifelong disorder. Other systemic malformations are reported between 47 and 81% of DWS patients and include urogenital, renal, cardiac, intestinal, and craniofacial defects.