Bilateral Acute Angle-Closure Glaucoma in a Patient with Vogt-Koyanagi-Harada Syndrome: A Case Report

Dear Editor,

Vogt-Koyanagi-Harada (VKH) disease is a rare multi-systemic inflammatory autoimmune disorder characterized by panuveitis often accompanied by neurologic and cutaneous manifestations, such as headache, tinnitus, dysacusia, poliosis, and vitiligo [1]. Generally, extraocular manifestations, including neurologic and auditory symptoms, precede the ocular and cutaneous findings. Bilateral acute angle-closure glaucoma (ACG) as an initial manifestation of VKH can be considered very rare [2]. Here, we report a rare instance of bilateral ACG occurring alongside VKH, which could occur with secondary drug-induced ACG (SDACG). Written informed consent was obtained from the patient.

A 49-year-old woman with decreased vision in both eyes, and a headache of 7 days’ duration visited a local clinic. Her intraocular pressure (IOP) was 40 mmHg in both eyes with shallow anterior chambers, and bilateral conjunctival injection was observed. She was thus diagnosed as suffering from bilateral primary angle-closure glaucoma and was treated with topical pilocarpine and a Cosopt (Merck & Co, Inc). Upon referral to our hospital for further evaluation and treatment, she had no previous uveitis history but we discovered her remarkable medical history. She had suffered from hypochondria and body shape, so she was usually taking several oral medications for diet and health supplements every day. Examination revealed best corrected visual acuity of 20 / 30 (−1.5 sphere) in the right eye and 20 / 60 (−1.25 sphere) in the left eye. IOP was 25 mmHg in the right eye and 40 mmHg in the left eye. Conjunctival injection and shallow anterior chambers were noted, but there were no cells or flare. The pupils were constricted due to the use of pilocarpine, and the fundus could not be examined in detail. Pentacam showed shallow anterior chambers and forward movement of the lens-iris diaphragm in both eyes (Fig. 1A, 1B). Initially diagnosed with SDACG based on young age, medical history, and initial findings, she was prescribed topical brimonidine and prednisolone, and advised to stop all oral medications. 2 weeks later, her vision decreased further to 20 / 400 in both, although IOP was normalized. Therefore, several additional examinations were done. Wide-field fundus showed huge and multiple exudative retinal detachments with optic disc swelling (Fig. 1C, 1D) and fluorescein angiography revealed severe dye leakage from the optic disc, along with multiple mild leakage from the peripheral retinal vessels (Fig. 1E, 1F). The optical coherence tomography demonstrated exudative retinal detachment with choroidal thickening in both eyes (Fig. 1G, 1H). She was subsequently diagnosed with incomplete VKH disease with bilateral ACG. She was prescribed intravenous prednisolone (1 mg/kg/day) for 3 days and tapered down to oral steroid for 1 month. A follow-up visit after 1 month, showed significant improvement in her symptoms, with 20 / 20 visual acuity and −0.25 sphere in both eye and resolved retinal lesions.

Fig. 1

Auxiliary multimodal imaging tests of both eyes at presentation and follow-up. (A, B) Pentacam images showed bilateral very shallow anterior chambers without sign of iris bombe. (C, D) Wide-field fundus photos revealed bilateral severe exudative retinal detachment and optic disc swelling. (E, F) Fluorescein angiography showed severe dye leakage from the optic disc and multiple large areas of hypofluorescence in the retina caused by the exudative retinal detachment, along with multiple mild leakages from the peripheral retinal vessels. (G, H) Optical coherence tomography demonstrated exudative retinal detachment with choroidal thickening in both eyes. (A, C, E, G) Right eye. (B, D, F, H) Left eye.

The mechanism of glaucoma in VKH includes steroid-induced glaucoma, trabeculitis, pupillary block, synechial angle closure, and uveal effusion [3]. Therefore, VKH can initially rarely present as ACG, and the presumed mechanism of VKH with ACG is that the swelling of the ciliary body and rotation of ciliary processes may put the ciliary body forward, lead to a shallow anterior chamber and angle closure with myopia. Moreover, this is seen mainly in relatively young adults and occurs bilaterally [3]. The mechanism and clinical manifestation of this is similar to SDACG. The mechanism of SDACG is that ciliary body edema causes relaxation of the zonules, which allowing lens thickening. Anterolateral rotation of the ciliary body about its attachment leads to anterior displacement of the lens and iris and concomitant bilateral shallowing of the anterior chamber with myopia. Treatment of SDACG includes cease of sulfa drugs, using topical cycloplegia, and topical corticosteroids [4]. In our case, she was also a relatively young adult, who had bilateral shallow anterior chambers with myopia and several oral medication histories. Considering the medication history and the complex nature of the case, it is plausible to consider the possibility that either SDACG or VKH disease could contribute to the ACG presentation in this patient. However, in cases where a relatively young patient presents with acute bilateral shallow anterior chambers, myopia, sudden onset of severe visual disturbances, high IOP, and no symptom improvement after cease of oral medications and treatment of topical corticosteroids, VKH should be considered as a potential diagnosis. A thorough fundus examination is necessary to differentiate the cause of secondary ACG and make an accurate diagnosis.