A 21 year old male was referred to South West Eye Surgery by his GP with blurred vision and a central visual field defect (a blurred patch) in his right eye. He had awoken 2 days previously with a headache, fatigue and bloodshot eyes.
On examination, visual acuities were Right 6/12 and Left 6/5. He had bilateral conjunctival hyperaemia (red eyes), but little anterior or posterior uveitis (internal eye inflammation) of note.
The ultra wide field fundus picture showed subtle grey/white lesions inferior and temporal to (below and to the next to) the macula in the right but also in the left eye. There was associated elevation (lift or detachment) of the retina below the macula on the right.
There were no autofluorescence features of note, other than the overlying retinal folds consistent with a neurosensory (a layer of the retina) detachment beneath and involving the right macula.
Optical Coherence Tomography revealed sub retinal fluid (fluid under the retina) on the right, causing a serous neuroretinal (the nerve layer of retina) detachment.
Intravenous fluorescein angiography showed early hyper fluorescent lesions that later enhanced and leaked fluorescein, associated with an intensely hyperfluorescent left optic disc (injected dye leaked into the areas of the eye affected).
The patient was immediately admitted to hospital and commenced on intravenous methyl prednisolone. He made a rapid recovery and by day 3 of his hospital stay, his right vision had improved back to 6/5 and repeat scans showed steady resolution of the fundal lesions and sub retinal fluid.
The diagnosis is Vogt-Koyanagi-Harada disease - a systemic illness resulting from inflammation around melanocytes (the pigment producing cells of the body), melanin (the body's pigment material) and retinal pigment epithelium (a support layer of the retina). It is thought to be an auto-immune disease in which some of the body's white cells (T-cells) attack areas in which these cells and material exist. Therefore, patients develop problems in their skin (vitiligo, alopecia), ears (hearing loss) and eyes (inflammation, and vision loss). Urgent treatment with immune-suppressant agents is required to prevent permanent loss of vision.
The patient is now on a reducing dose of steroids over the next few months and is looking forward to a full recovery, but may require long term immune suppression.
Fore more information on V-K-H disease, click the button below:
Dr Vincent Lee,