Sturge-Weber syndrome is a neurocutaneous disorder characterised by facial port-wine stain, leptomeningeal vascular malformations, and ocular involvement. Glaucoma and choroidal haemangiomas are key eye concerns.
Ocular features
Eye changes may be present from infancy.
- Congenital or early-onset glaucoma, especially when the stain involves the upper lid
- Diffuse choroidal haemangioma causing ‘tomato ketchup’ fundus and serous retinal detachment
- Refractive errors and amblyopia related to asymmetry
- Visual field defects from associated brain involvement
Management
Care is multidisciplinary and long term.
- Regular glaucoma surveillance and medical or surgical treatment as needed
- Laser or radiotherapy for choroidal haemangioma in selected cases
- Amblyopia therapy, glasses, and low-vision support where required
- Neurology input for seizures and developmental assessment
