Abstract
Purpose
To report an unusual case of incomplete Vogt-Koyanagi-Harada (VKH) in a 14-Year-Old African American female.
Observations
Here we present a 14-Year-Old African American Female with incomplete VKH who presented to the emergency department with a one-month history of malaise, fever, bilateral decreased vision and temporal headaches. At the time of presentation, she was found to have bilateral anterior uveitis and disc edema. The brain and orbit MRI were unremarkable, CSF analysis showed lymphocytic pleocytosis with negative cultures and gram stain. An extensive lab workup was unrevealing.
Conclusion
This is an unusual case of incomplete VKH in a young African American female presenting with anterior uveitis and disc edema without serous retinal detachments. Initiation of high dose oral prednisone promptly after diagnosis resulted in rapid improvement of symptoms and improvement in visual acuity with the resolution of the disc edema. This case highlights the importance of considering VKH in the differential diagnosis of disc edema associated with uveitis, even in the absence of serous retinal detachment.
1
Introduction
Vogt-Koyanagi-Harada (VKH) disease is a multisystem disease with auditory, neurologic, ocular, and dermatologic manifestations. , Although the true pathophysiology of the disease is not well understood, current leading theories suggest an autoimmune T-cell mediated process against melanocytes. VKH classically presents as bilateral panuveitis as well as extraocular manifestations such as meningismus, alopecia, poliosis, vitiligo, and dysacousia. The disease mainly affects pigmented individuals. It has been reported to occur less frequently in African Americans when compared to other ethnicities, including Hispanics, Native Americans, Asians, and Middle Easterners. ,
We present an unusual case of a 14-year-old African American female with incomplete VKH manifesting as bilateral anterior uveitis and disc edema.
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Case report
A 14-year-old African-American female with no significant past medical history was referred to the emergency department (ED) for worsening headaches and vision loss. Her symptoms started one month prior to presentation with fevers (102.6 °F), sudden onset pounding bitemporal headache of 8/10 severity, body aches, paranasal sinus drainage and occasional cough. She was seen at an urgent care clinic and was started on amoxicillin/clavulanic acid. There was no lymphadenopathy or meningismus at that time. The patient underwent a CT scan of the head at an outside hospital, which was reported as normal. One week later, she started to notice bilateral eye redness. She was evaluated and found to have anterior uveitis and disc edema. She was started on topical steroids and cycloplegic eye drops. On follow-up visit, she was noted to have worsening disc edema and visual acuity and was referred to our ED.
On examination, visual acuity at distance was 20/40 in the right eye and 20/200–1 in the left eye with no improvement with pinhole. Pupils were equal, round, and sluggishly reactive to light with no afferent pupillary defect. Extraocular motility was full with intact visual fields to confrontation bilaterally. Intraocular pressures were 9 mmHg and 10 mmHg in the right and left eye, respectively. On slit-lamp examination, she had 1+ conjunctival injection in both eyes. The anterior chamber was deep with 3+ cell and inferior keratic precipitates in both eyes. She was noted to have bilateral posterior synechia with pigment on anterior lens capsule bilaterally. Dilated fundus exam showed bilateral disc edema, diffuse choroidal thickening, and fine macular exudates in the left eye ( Fig. 1 ). Her blood pressure was 100/78 mmHg and weight was 62.4 kg (BMI 19.92).
MRI of brain/orbit and MRV were performed in the ED and were unremarkable except for enhancement of the optic disc bilaterally. The patient was admitted for further workup. Laboratory testing including CBC, CMP, ACE, ANCA panel, QuantiFERON gold, HLA-B27, ANA, Bartonella, and syphilis antibody were negative. Lyme IgG was positive; however, confirmatory western plot testing came back negative. Lumbar puncture showed lymphocytic pleocytosis with negative gram stain and cultures with elevated CSF opening pressure of 27 cm H 2 O.
Optical coherence tomography showed intact foveal contour and significant peripapillary retinal nerve fiber layer edema in both eyes ( Fig. 2 ). The patient was diagnosed with incomplete VKH and started on 60 mg of oral prednisone daily and continued on topical steroids.
At the follow-up visit 3 weeks after the discharge, vision improved to 20/20 in the right eye and 20/25 in the left eye. Intraocular pressures were normal in both eyes. The anterior chamber was deep with 1+ cell bilaterally. The dilated fundus exam was notable for bilateral disc edema.
At the subsequent follow-up, 6 weeks after the discharge, vision remained 20/20 in the right eye and 20/25 in the left. The anterior chamber was deep and quiet in both eyes. The dilated fundus exam revealed blurred disc margins. The oral prednisone dose was decreased to 40mg. Topical steroids were reduced to twice daily use. The oral steroids were tapered slowly over the subsequent follow-up visits and methotrexate was started. At the 4 months follow up-visit, dilated fundus exam showed sharp and pink nerves without disc edema ( Fig. 3 ).
