This thesis represents an attempt to further our knowledge of the ophthalmic complications of spina bifida and hydrocephalus by means of literature review and a long term clinical study, and to determine whether regular ophthalmic supervision can assist in the general management of affected children. The ophthalmic complications of spina bifida have often been reported in the literature and thought to merit regular supervision of affected children, yet few centres currently offer this service. In this study 322 children attending one regional centre were examined repeatedly over a six year period by one ophthalmologist. Ophthalmic complications were found to be very common. They frequently provided evidence of raised intracranial pressure due to shunt dysfunction even when other objective evidence was lacking. Every spina bifida and hydrocephalus clinic should have an ophthalmalogist in its medical team. Preservation of visual function and early diagnosis of raised intracranial pressure in these children should result from this arrangement.