Occipital meningoceles in patients with the Dandy-Walker syndrome

Occipital cephaloceles in the Dandy-Walker syndrome are not rare. To date, 28 cases have been reported. We report on 8 cases of associated occipital meningocele, totalling 16% of all patients with the Dandy-Walker syndrome in our series. In all patients, communication existed between the posterior fossa cyst and the occipital meningocele. In 2 patients, the occipital meningocele disappeared after cerebrospinal fluid shunting and never required surgical repair. It appears that the cranial defect had ossified and resulted in self-closure. Poor intellectual development in persons with the Dandy-Walker syndrome is associated with the presence of other central nervous system and systemic abnormalities and not with occipital meningoceles alone.