We present our experience with the treatment of 13 patients with Dandy-Walker syndrome. The common presenting symptom and associated central nervous system anomaly were enlargement of head and occipital encephalocele, respectively. Eleven out of 13 patients were treated surgically after stabilization of systemic medical status. Two patients could not be operated because of poor medical condition. In 6 patients with an opened passage between posterior fossa cyst and lateral ventricle, cystoperitoneal shunt system with medium pressure valve was the treatment of choice. In 5 patients with no relation between cyst and ventricle, cystoperitoneal and ventriculoperitoneal shunting with ‘Y’ connectors were applied separately. Another patient with a shunt infection was treated by shunt system renewal combined with parenteral antibiotics. One patient died 7 months after the operation due to recurrent meningitis.
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