Dandy Walker malformation (variant): late presentation with childhood blindness

Abstract

We present a rare case of Dandy Walker Syndrome in a child who presents with complaints of frontal headache, neck pain, fever, progressive visual impairment and multiple generalized tonic clonic seizures, inability to stand and urinary incontinence. Given the clinical and neuro-imaging findings, the diagnosis of Dandy Walker Variant was made. Post surgical recovery (Ventriculo peritoneal shunt) was remarkable. Unfortunately he never regained sight. The purpose of presenting this case report is to highlight the distinctive manifestation of Dandy walker syndrome to enhance early diagnosis, prompt intervention and better outcomeKeywords: Dandy Walker syndrome; congenital brain malformation; childhood blindness; hydrocephalus