Dandy Walker malformation and hypertrophic cardiomyopathy. Unusual fatal association.
Dandy Walker malformation (DWM) is a rare congenital brain anomaly characterized by cystic dilation of the fourth ventricle and hypoplasia of the cerebellar vermis. Other extracranial anomalies can be associated, including cardiac defects. We report a rare patient with DWM associated with progressive heart failure secondary to hypertrophic cardiomyopathy. He was diagnosed at 2 months of age and died 5 months later. We conclude that hypertrophic cardiomyopathy can be associated with DWM with poor prognosis. A careful cardiac evaluation is needed in all infants with DWM for early recognition of such potentially serious associated cardiac malformations.
Department of Neurosciences, King Faisal Specialist Hospital & Research Center, Jeddah, Kingdom of Saudi Arabia.
Maher E
ME
Kurdi
Mohammed A
MA
Chamsi-Pasha
Saleh S
SS
Baeesa
Mohammed M
MM
Jan