Combined ocular involvement in juvenile glaucoma and Best disease

PURPOSE. Monitoring of a patient with a rare combined pathology of juvenile open-angle glaucoma (JOAG) and Best disease (Best vitelliform macular dystrophy, BVMD). METHODS. Visometry, autorefractometry, tonometry, gonioscopy, biomicroscopy, ophthalmoscopy, ocular ultrasound, digital fundus photography, optical coherence tomography angiography (OCT-A). RESULTS. A 32-year-old male patient reported a long-standing fog before his right eye, a spot, progressive visual impairment in both eyes (more pronounced in the right eye), eye redness, and poor tolerance to hypotensive eye drops. He was previously diagnosed with Best disease and JOAG in both eyes. Fundus photography and OCT-A revealed retinal changes characteristic of JOAG. The patient exhibited poor tolerance to topical glaucoma medications, experiencing significant conjunctival hyperemia and ocular pain. A well-tolerated antiglaucoma regimen was established, consisting of a fixed combination of bimatoprost 0.3 mg/ml and timolol 5 mg/ml (BIMOKKO-SZ); dorzolamide 20 mg/ml (Dorzolamide-SZ); and brimonidine 2 mg/ml (Brimonidine-SZ) (all manufactured by NAO Severnaya Zvezda, Russia). Vision, intraocular pressure, and OCT-A parameters stabilized.During the follow-up, retinal thickening exceeding 700 µm was observed, with a risk of inner retinal layer rupture and macular hole formation. Emergence of new choroidal vessels and active choroidal neovascularization were oserved. Intravitreal aflibercept injection was administered, resulting in disease stabilization. CONCLUSION. The combination of JOAG and Best disease requires personalized monitoring and treatment selection, including conservative, laser, surgical, and antiangiogenic therapies as needed. Patient follow-up is ongoing.