VISUAL FAILURE CAUSED BY RAISED INTRACRANIAL-PRESSURE IN CRANIOSYNOSTOSIS

Craniosynostosis, the premature fusion of one or multiple cranial sutu res, can be complicated by visual failure resulting from raised intrac ranial pressure (ICP). Of the 290 children operated on at the Birmingh am Children's Hospital between 1978 and 1995 for craniosynostosis, 9 w ere found to have defective visual acuity attributable to raised ICP. Mean age at presentation was 3.11 years (range: 1-6 years) and mean fo llow-up, 7.33 years (range: 1.5-16 years). All these patients presente d significantly later than usual, and 5 of them developed recurrent cr aniosynostosis. At the initial examination bilateral papilloedema was seen in 4 patients, unilateral disc oedema in 1 patient, bilateral opt ic atrophy in 3 patients and unilateral optic atrophy in 1 patient. Fo llowing decompressive craniofacial surgery, the visual outcome was goo d in 4 out of 5 patients with papilloedema and poor in all patients wi th optic atrophy. Visual failure resulting from raised ICP in craniosy nostosis is a devastating complication, which appears to be associated with late presentation and recurrent craniosynostosis.