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Visual failure caused by raised intracranial pressure in craniosynostosis
P Stavrou1, S Sgouros, H E Willshaw
1Department of Craniofacial Surgery, Birmingham Children's Hospital, UK.
Insights
Craniosynostosis can cause vision loss due to increased intracranial pressure (ICP). Early detection and treatment are crucial, as delayed presentation and recurrent cases are linked to poor visual outcomes.
Area of Science:
- Pediatric Neurosurgery
- Ophthalmology
- Craniofacial Surgery
Background:
- Craniosynostosis involves premature fusion of cranial sutures, potentially leading to increased intracranial pressure (ICP).
- Elevated ICP can cause visual impairment, a serious complication in affected children.
- This study examines visual outcomes in children with craniosynostosis and ICP-related visual failure.
Purpose of the Study:
- To investigate the incidence and visual outcomes of visual failure associated with raised intracranial pressure in craniosynostosis.
- To identify factors associated with poor visual prognosis in this patient group.
Main Methods:
- Retrospective review of 290 children treated for craniosynostosis between 1978 and 1995.
- Analysis of visual acuity, funduscopic findings (papilledema, optic atrophy), and surgical outcomes in 9 patients with visual failure attributed to raised ICP.
- Correlation of visual outcomes with age at presentation and history of recurrent craniosynostosis.
Main Results:
- Nine out of 290 patients (3.1%) presented with visual failure due to raised ICP.
- Patients with visual failure often presented late and had a higher incidence of recurrent craniosynostosis (5 out of 9).
- Good visual outcomes were observed in 4/5 patients with papilledema post-surgery, whereas all patients with optic atrophy had poor outcomes.
Conclusions:
- Visual failure is a significant complication of craniosynostosis, often linked to delayed presentation and recurrent disease.
- Prompt diagnosis and surgical intervention are vital, particularly in cases of papilledema.
- Optic atrophy at presentation indicates a poorer visual prognosis, underscoring the importance of early detection of ICP elevation.
Abstract:
Craniosynostosis, the premature fusion of one or multiple cranial 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 between 1978 and 1995 for craniosynostosis, 9 were found to have defective 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 patients presented significantly later than usual, and 5 of them developed recurrent craniosynostosis. At the initial examination bilateral papilloedema was seen in 4 patients, unilateral disc oedema in 1 patient, bilateral optic atrophy in 3 patients and unilateral optic atrophy in 1 patient. Following decompressive craniofacial surgery, the visual outcome was good in 4 out of 5 patients with papilloedema and poor in all patients with optic atrophy. visual failure resulting from raised ICP in craniosynostosis is a devastating complication, which appears to be associated with late presentation and recurrent craniosynostosis.