Introduction Craniosynostosis, the premature fusion of one or multiple cranial sutures, results in compromise of skull growth and cosmetic deformity. Ocular complications are common and are usually secondary to derangement of the surrounding bony structures, because the skull base and facial skeleton are frequently involved [1, 11]. They include: proptosis, corneal exposure, strabismus, ocular motility disorders and nasolacrimal anomalies [1–4, 8–10, 12]. Elevated intracranial pressure (ICP) is not uncommon, and has been reported in 10% of patients with single su- ture craniosynostosis and 50% of patients with multiple su- ture craniosynostosis [14]. It has also been reported that in these patients, ICP reaches maximum levels at the age of 6 years and decreases thereafter [14]. Other reports have suggested that patients with craniosynostosis are at risk of complications attributable to raised ICP throughout in- fancy and adulthood, despite surgical attempts to increase cranial capacity in infancy [5]. The clinical significance of raised ICP in many patients with craniosynostosis remains unclear however [5, 14]. Optic nerve dysfunction caused by raised ICP has been poorly documented and appears to be a rare complication of craniosynostosis [8, 12]. It was recognised as early as 1913, however, when Larsen reported that 21% of the boys at the Royal Institute for the Blind in Copenhagen had cran- iosynostosis [10]. Furthermore, clinical presentation with visual failure caused by raised ICP secondary to undiag- nosed or recurrent craniosynostosis is unusual. The aim of this study was to assess the visual outcome in children who presented with or were found to have vis- ual failure as a result of raised ICP secondary to craniosy- nostosis. Child s Nerv Syst (1997) 13:64 – 67 © Springer-Verlag 1997 ORIGINAL PAPER Visual failure caused by raised intracranial pressure in craniosynostosis P. Stavrou S. Sgouros H. E. Willshaw J. H. Goldin A. D. Hockley M. J. C. Wake Abstract Craniosynostosis, the pre- mature fusion of one or multiple cra- nial sutures, can be complicated by visual failure resulting from raised intracranial pressure (ICP). Of the 290 children operated on at the Birmingham Children’s Hospital be- tween 1978 and 1995 for craniosy- nostosis, 9 were found to have defec- tive visual acuity attributable to raised ICP. Mean age at presentation was 3.11 years (range: 1–6 years) and mean follow-up, 7.33 years (range: 1.5–16 years). All these pa- tients presented significantly later than usual, and 5 of them developed recurrent craniosynostosis. At the in- itial examination bilateral papillo- edema was seen in 4 patients, unilat- eral disc oedema in 1 patient, bilat- eral optic atrophy in 3 patients and unilateral optic atrophy in 1 patient. Following decompressive craniofa- cial surgery, the visual outcome was good in 4 out of 5 patients with pa- pilloedema and poor in all patients with optic atrophy. Visual failure re- sulting from raised ICP in craniosy- nostosis is a devastating complica- tion, which appears to be associated with late presentation and recurrent craniosynostosis. Key words Craniosynostosis · Papilloedema · Optic atrophy · Vision · Intracranial pressure Received: 16 August 1996 P. Stavrou · S. Sgouros () 1 · J. H. Goldin A. D. Hockley · M. J. C. Wake Department of Craniofacial Surgery, Birmingham Children’s Hospital, Birmingham, UK H. E. Willshaw Department of Ophthalmology, Birmingham Children’s Hospital, Birmingham, UK Mailing address: 1 Craniofacial Surgery Unit, Queen Elizabeth Hospital, Birmingham B15 2TH, UK Tel.: (44) 121-62 72 303 Fax: (44) 121-62 72 302