Dandy–Walker syndrome associated with syringomyelia in an adult: a case report and literature review

Abstract

Dandy–Walker syndrome associated with syringomyelia is a rare condition, with few reports of adult cases. We describe an adult case of Dandy–Walker syndrome with concomitant syringomyelia. A 33-year-old man presented with a 3-month history of walking instability, numbness in the hands, memory deterioration, and urinary incontinence. A physical examination showed a positive Romberg sign. Brain computed tomography and magnetic resonance imaging showed hydrocephalus, a cyst in the posterior fossa, absence of the cerebellar vermis, hypoplasia of the corpus callosum and cerebella, and syringomyelia. All of these symptoms were consistent with the diagnosis of Dandy–Walker syndrome. Surgery involving arachnoid adhesiolysis and endoscopic third ventriculostomy was performed. At the 6-month follow-up, the symptoms were completely relieved. Magnetic resonance imaging showed that syringomyelia was greatly reduced and the hydrocephalus remained unchanged. Dandy–Walker syndrome with concomitant syringomyelia in adults is exceedingly rare. Early diagnosis and appropriate surgical treatment of this condition should be highlighted. Combined arachnoid adhesiolysis and endoscopic third ventriculostomy may be an effective approach.