Initial misdiagnosis of Vogt-Koyanagi-Harada disease

Abstract

PurposeTo report the initial misdiagnosis of patients with Vogt–Koyanagi–Harada (VKH) disease.MethodsThe medical records of 76 consecutive patients diagnosed with VKH disease were reviewed retrospectively at The Eye Center, Riyadh, Saudi Arabia. Patients were referred to The Eye Center from Saudi Arabia and other Middle Eastern countries. The initial diagnosis was made by an ophthalmologist or neurologist. The main outcome measure was to evaluate cases with VKH disease who were initially misdiagnosed.ResultsIn 7 (9.2%) out of the 76 patients the initial diagnosis was incorrect. Patients were initially misdiagnosed as optic neuritis (1.3%), intracranial hypertension (1.3%), brain tumor (1.3%), Susac disease (1.3%), migraine (1.3%), rhegmatogenous retinal detachment (1.3%) or anterior granulomatous uveitis of unknown etiology (1.3%). Patients underwent unnecessary tests including MRI and invasive procedures including CSF analysis and anterior chamber paracentesis.ConclusionThe initial diagnosis of patients with VKH disease was incorrect in 9 % of the cases. Delay in the diagnosis of VKH disease may lead to delay in management and may cause irreversible damage to the photoreceptors with poor visual outcome.

Evolution of the acute uveitic phase in Vogt-Koyanagi-Harada Syndrome / Evolução da fase uveítica aguda na Síndrome de Vogt-Koyanagi-Harada

“…Several weeks later, the disease progresses to the convalescent phase, which is characterized by depigmentation of the choroid ''sunset glow fundus''. In this phase, the optic disc may appear pale 3,11 . We report a case of women in acute uveitis phase: headache, hyperemic optic discs, serous retinal detachment and tinnitus.…”

Vogt-Koyanagi-Harada syndrome

“…In the study by Shoughya and Tabbaraa, 9,2 % (7 out of 76) of patients with VGH had incorrect initial diagnosis. The initial diagnosis included optic neuritis, intracranial hypertension, brain tumour, Susac disease, migraine, rhegmatogenous retinal detachment or anterior granulomatous uveitis of unknown etiology [5] . Even though the ophthalmologist suspected VGH disease after examining our patient, after MRI and CSF serology, the initial diagnosis was first optic neuritis and afterwards neuroborreliosis.…”

Vogt-Koyanagi-Harada disease diagnosed in members of the same family

“…This stage can sometimes be associated with neurological manifestations such as meningismus and headache, cranial nerve palsy, hemiparesis, transverse myelitis, and optic neuritis. Eye symptoms such as photophobia and tearing may occur after systemic symptoms [3][4][5][6][7][8][14][15][16][17].  Acute uveitic stage: This stage follows the prodromal phase and lasts for several weeks.…”

“…Thus, it is mandatory in suspected cases of unilateral manifestations and should be carefully evaluated for signs and symptoms in the adjacent eye. Uveitis commonly presents with multiple severe retinal detachments, optic nerve head hyperemia and swelling, and posterior choroidal thickening with elevation of the peripapillary retinochoroid layer [3][4][5][6][7][8][14][15][16][17].  Chronic (Convalescent) Stage: This stage can last for months or even years, resulting in depigmentation that can be tegumentary and/or uveal.…”

“…VKH disease is characterized by chronic onset and shows bilateral granulomatous uveitis with extraocular central nervous system manifestations such as cerebrospinal fluid (CSF) pleocytosis, dysacousia, tinnitus, vertigo, and in some cases, integumentary system vitiligo, poliosis, and alopecia. The ocular symptoms of the disease are characterized by multifocal serous retinal detachment, choroidal edema, and optic disc hyperemia in the acute phase [2,7,8,[11][12][13][14].…”