Ophthalmic complications of spina bifida and hydrocephalus

The article reviews the ophthalmic literature on spina bifida and hydrocephalus and presents the findings of a six-year survey of 322 children. Ophthalmic complications were found to occur very frequently. Forty-two per cent had a manifest squint, 29% lateral rectus palsy or musculoparetic nystagmus, 14% papilloedema and 17% optic atrophy. Only 27% definitely had normal visual function. Seventy per cent of proven episodes of shunt dysfunctions had positive ophthalmological evidence of raised intracranial pressure. The sudden appearance of a squint, other ocular motility disorder or papilloedema usually denotes uncontrolled hydrocephalus. Shunt surgery is the first priority but may not restore normal ocular motility and visual function. Subsequent treatment should be tailored to the special needs of these children. Regular ophthalmic supervision of these children saves life and sight.