Famously happened to Louis Braille (the inventor of braille writing) after an injury to one eye
About
- An autoimmune, type 4 hypersensitivity reaction
- Following penetrating trauma or surgery to one eye
- Patients may develop autoimmune inflammation of the fellow eye.
Aetiology
- A rare granulomatous inflammatory reaction to melanin containing structures such as the photoreceptor outer segments
- Develops after penetrating trauma or surgery to the fellow eye
Clinical
- A history of eye trauma. Patients present with a florid pan-uveitis in the fellow eye
- Usually occurs approximately three months after initial injury, but may be many years later
- Significant reduction in vision, pain, photophobia, conjunctival injection
- Mutton fat keratitic precipitates
- Dalen-fuchs nodules – clusters of epithelioid cells between the RPE and Bruch’s membrane
Investigations
- A clinical diagnosis based on a history of ocular trauma and the clinical findings listed above
- OCT scan can be helpful in assessing any retinal complications such as retinal detachment or macular oedema
- Fluorescein angiography may be helpful
Management
- In the acute phase, high dose IV methylprednisolone should be initiated immediately
- Immunosuppression with agents such as azathioprine, ciclosporin, or cyclophosphamide
- Enucleation may be considered for a painful, non-seeing eye
Prognosis
- Vision loss can be severe and complete loss of sight is common
- In the majority of cases, prompt treatment with steroids and systemic immunosuppression can help preserve some degree of visual acuity
References