Spotlight Case: What is the Meaning of all This Fluid?
R. Rishi Gupta, MD, FRCSC
Sweta Tarigopula, MD
John Chen, MD, FRCSC
A 28-year-old, nonpregnant, mildly myopic, Caucasian female presented with a 4-day history of progressive worsening of visual acuity, sequentially in the left eye (OS), and then right eye (OD). Her past medical history and past ocular history were unremarkable, and she did not take any medications. A review of systems was negative.
On examination, best-corrected visual acuity (VA) was counting fingers OD and hand motion OS. Intraocular pressures, extraocular movements, and visual field testing by confrontation were normal, and there was no relative afferent pupillary defect. Slit lamp examination of the anterior segment revealed ½+ cell and ½+ flare in both eyes (OU), with no keratic precipitates and no vitreous cell observed. Dilated fundus examination showed mild disc hyperemia, but no obvious edema. The posterior pole of each eye had multiple circumscribed areas of subretinal fluid, with no evidence of hemorrhage or vasculitis.
What’s your diagnosis?
Upon further questioning, the patient did not have a recent illness or dysacusis, however had experienced a headache over the past four days. She was not of Asian or Native descent. There was no evidence on examination of vitiligo or poliosis. Her blood pressure in clinic was normal.
Laboratory investigations revealed a normal complete blood count and differential, as well as normal eletrolytes, creatinine, and liver function tests. A lumbar puncture was organized and demonstrated pleocytosis.
A diagnosis of Vogt-Koyanagi-Harada (VKH) was made based on her clinical findings and the patient was started on oral prednisone at 1.5mg/kg per day. One week later her vision in both eyes had improved to 20/60, with marked reduction of subretinal fluid as shown below on OCT.
VKH is a granulomatous inflammatory condition with auditory, cutaneous, meningeal, and ocular manifestations. Ocular manifestations include granulomatous inflammation and multifocal serous detachments. Historically, the diagnostic criteria as established by the American Uveitis Society in 1978 defined a patient with VKH as having no history of prior ocular trauma or surgery and three of the following 4 signs: bilateral chronic iridocyclitis; posterior uveitis with multifocal serous retinal or RPE detachments, disc hyperemia or edema, or “sunset glow fundus;” neurologic signs including tinnitus, neck stiffness, CNS symptoms, or CSF pleocytosis; or cutaneous findings of alopecia, poliosis, or vitiligo. The revised definition of VKH encompasses an expanded diagnostic criteria in which patients with complete VKH disease have all 5 of the following: no history of penetrating ocular trauma or surgery; no clinical or laboratory evidence of other ocular disease entities; bilateral ocular involvement of either early or late manifestations of the disease; neurological or auditory findings; and cutaneous findings which do not precede CNS or ocular disease.
The 4 clinical stages of VKH are the prodromic, uveitic, convalescent, and recurrent. While VKH is primarily a clinical diagnosis, the use of imaging can help support the diagnosis and monitor treatment. During the uveitic stage, the predominant angiographic features are optic disc hyperfluoresence and disseminated choroidal hyperfluoresence. OCT can show intraretinal and subretinal cysts, demonstrated in the photo above, which form during the inflammatory process. ICG shows early choroidal stromal hyperfluorescence and leakage, hypofluorescent dark dots, a fuzzy vascular pattern of large stromal vessels, and disc hyperfluoresence.
Clinical presentation is key to diagnosing this condition, as is ruling out mimicking entities of bilateral granulomatous panuveitis or bilateral serous detachments such as sympathetic ophthalmia, sarcoidosis, syphilitic uveitis, tuberculous uveitis, Lyme disease, posterior scleritis, intraocular lymphoma, central serous choroiditis, and uveal effusion syndrome.
Take home points:
- VKH is a granulomatous inflammatory disorder that can cause panuveitis and granulomatous inflammation and multifocal serous detachments. It is often associated with cutaneous and neurological manifestations.
- Diagnosis is based on characteristic clinical findings and characteristic OCT, FA, and ICG findings
- Early and aggressive treatment with systemic corticosteroids is essential, and second-line immunosuppressive agents can be used in conjunction.
- With treatment, patients generally do well, with 50-90% of treated patients obtaining visual acuity of >20/40. Complications leading to visual loss include cataracts, glaucoma, and subretinal neovascular membranes.
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Dr. Gupta - None.
Dr. Tarigopula - None.
Dr. Chen - None.