Hydrocephalus has been commonly observed at necropsy of patients with systemic mucopolysaccharidoses. Clinical confirmation of papilledema and communicating hydrocephalus has been obtained in a child with the Maroteaux-Lamy syndrome (mucopolysaccharidosis, Type VI). The hydrocephalus and papilledema were relieved by successful shunting of cerebrospinal fluid. Corneal clouding is usually the most obvious cause of reduced visual acuity in systemic mucopolysaccharidoses. However, decreased visual functioning in these disorders may also result from either ascending or descending optic atrophy.
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