Dandy-walker malformation associated with syringomyelia: an unusual presentation

ABSTRACT

We are reporting a patients with a Dandy-Walker malformation who underwent insertion of a cysto-peritoneal shunt as a neonate. At the age of 17, she presented with difficulty in walking and black-out attacks for 6 months before she was rushed to the hospital in a coma. Following an urgent CT scan, the shunt was revised and was found to be partially obstructed by omentum. After recovery, the patients demonstrated newly developed cerebellar and pyramidal signs. MRI showed evidence of cervical syringomyelia with no brain stem involvement. A second MRI carried out 2 weeks after shunt revision, showed collapse of the syringomyelia that correlated well with the patients clinical improvement. In addition to the rare association of Dandy-Walker malformation and syringomyelia, this case also demonstracts an unusual presentation of shunt malfunction. Revision of the shunt resulted in both clinical and radiological improvement. We believe that cerebrospinal fluid shunting should be the initial step in the management of those cases