[A case of nonrhegmatogenous retinal detachment in Dandy-Walker Syndrome]

A 2-month-old female presented with nonrhegmatogenous retinal detachment in Dandy-Walker syndrome. At the fist examination, coloboma involving the optic disc in both eyes was detected. The left eye showed microphthalmos with sclerocornea, persistent pupillary membrane, hypoplasia of the iris stroma, and bullous retinal detachment near the optic disc. Chromosomal analysis revealed a mosaic pattern: 46, XX/47, XXX. Increased intracranial pressure associated with Dandy-Walker syndrome was detected by a neurosurgeon at the age of 3 months. The patient was followed for several weeks, and then nonrhegmatogenous retinal detachment appeared in the right eye. Subretinal fluid alternately increased and decreased in both eyes. A ventriculo-peritoneal shunt was performed at the age of 6 months, and the retinal detachment was remarkably reduced in both eyes after lowering of intracranial pressure. Coloboma involving the optic disc, sclerocornea, persistent pupillary membrane, hypoplasia of iris stroma, and Dandy-Walker syndrome were thought to be caused by the abnormal development of neural crest cells. We surmised that the retinal detachment in this case might have resulted from a communicating pathway between the subarachnoid space and the subretinal space. We concluded that the etiology of retinal detachment associated with optic disc anomaly should be investigated to determine adequate treatment.