Management of pediatric Vogt-Koyanagi-Harada (VKH)-associated panuveitis

Purpose: To assess the clinical course, determine the efficacy and safety of oral methotrexate in the control of intraocular inflammation, and evaluate the outcomes of lensectomy-vitrectomy and goniosynechiolysis on pediatric VKH-associated panuveitis. Design: Retrospective non-comparative interventional case series. Participants: Ten patients ( 20 eyes) with pediatric VKH-associated panuveitis ( onset of disease at age 14 years or younger). Intervention: Stepwise medical treatment consisted of oral prednisolone (0.5 - 1 mg/kg), supplemented by oral methotrexate ( 5 - 7.5 mg/wk) in refractory cases. Surgical intervention was performed for complications such as visually significant cataracts and secondary glaucoma and included pars plana lensectomy-vitrectomy and peripheral anterior synechiolysis in a one-stage procedure. Main outcome measures: Control of inflammation, corticosteroid requirement, visual acuity, and intraocular pressure (IOP). Results: Ten consecutive patients with a minimum follow-up duration of six months were analyzed. All patients were initially treated with oral corticosteroids; methotrexate was additionally required in six subjects. In all eyes, inflammation decreased and vision was preserved or improved. Side effects of methotrexate were mild and transient. Nine eyes of five patients underwent combined lensectomy and vitrectomy. The procedure was successful in all eyes without any exacerbation of inflammation. Eight eyes had elevated IOP, six of which underwent peripheral anterior synechiolysis at the time of lensectomy-vitrectomy. IOP was controlled after the procedure in all eyes with timolol only. Overall, final visual acuity was >= 20/40 in 30% of the eyes and < 20/ 200 in only 20%. Conclusions: Pediatric VKH-associated panuveitis seems to follow an aggressive course; the development rate of cataracts and secondary glaucoma seems to be high. Oral methotrexate is a safe and effective adjunct and displays steroid-sparing properties in the control of inflammation. Surgical interventions including peripheral anterior synechiolysis in addition to pars plana lensectomy and vitrectomy may be effective in controlling IOP in eyes with cataract and glaucoma. Precis: Vision may be preserved in pediatric VKH panuveitis using steroids and methotrexate for the control of inflammation, lensectomy-vitrectomy for visual improvement, and synechiolysis for IOP control.