A case of probable Vogt-Koyanagi-Harada disease in a 3-year-old girl

Abstract

Background: Vogt-Koyanagi-Harada (VKH) disease is a T-cell-mediated autoimmune disorder characterized by bilateral granulomatous panuveitis with various systemic manifestations. Although VKH disease rarely occurs in the pediatric population, the clinical course tends to be aggressive, and the visual prognosis is worse than that in adult patients due to severe ocular complications secondary to recurrent inflammation.

Case presentation: A 3-year-old girl with probable VKH was referred to Kobe University Hospital. She had severe bilateral panuveitis with posterior synechiae of the iris, marked optic disk swelling, and serous retinal detachment in both eyes, and her best corrected visual acuities (BCVAs) were 20/200 OD and 20/125 OS. A third course of therapy was administered because serous retinal detachment remained after two courses of therapy. She was treated with three courses of high-dose intravenous corticosteroid therapy, followed by slow tapering of oral corticosteroids. Her BCVAs recovered to 20/16 OU, and relapse of ocular inflammation and side effect of treatment were not observed during the 1.5-year follow-up period.

Conclusions: We experienced a pediatric patient with probable VKH disease who was treated with three courses of high-dose intravenous corticosteroid therapy. With the favorable clinical course in our patient, initial treatment with repeated high-dose intravenous corticosteroid therapy might be beneficial in pediatric VKH disease.

Keywords: Child; Corticosteroid therapy; Uveitis; Vogt–Koyanagi–Harada disease.

Fig. 1

Color fundus photographs at presentation and changes of OCT images after steroid therapy (a, b). Fundus images at presentation. Optic disk swelling and tortuous retinal vessels are observed in both eyes (c, d). OCT images at presentation. Bilateral marked serous retinal detachment is shown (e, f). OCT images after two courses of high-dose intravenous corticosteroid therapy. Serous retinal detachment is still present (g, h). OCT images after three courses of high-dose intravenous corticosteroid therapy. No subretinal fluid is seen, but the integrity of EZ is lost in both eyes

Fig. 2

Ultra-widefield color fundus photographs and OCT images 1 year after presentation (a, b). Ultra-widefield color fundus imaging shows normal results in the right eye and a limited depigmented area around the optic disk in the left eye (c, d). OCT imaging demonstrates a normal macular morphology with restored EZ in both eyes