Andrey Kopylov* and Anna Korolenko

Pediatric Ophthalmology Department, Irkutsk Branch of S.N. Fyodorov "Eye Microsurgery" Federal State Institution, Ministry of Health of Russian Federation, Russia

*Corresponding Author: Andrey Kopylov, Pediatric Ophthalmology Department, Irkutsk Branch of S.N. Fyodorov "Eye Microsurgery" Federal State Institution, Ministry of Health of Russian Federation, Russia.

Received: June 29, 2022; Published: February 06, 2023

Abstract

Purpose: To present a case report of diagnosis and management of a child’s macular neovascularization (MNV) associated with Best's vitelliform macular dystrophy.

Material and Methods: A 15-year-old patient with decreased vision in both eyes (more left than right). The vision decrease was first detected during an examination, including the determination of the best corrected visual acuity (BCVA), the vision field, and the collection of a genealogical history; OCT and OCTA revealed macular degeneration and active MNV.

Results and Discussion: Stabilization of the pathological process in the form of adhering of the neuroepithelium detachment over the MNV was achieved only in the right eye after the 1^st ranibizumab injection. Relapses and continued activity of newly formed vessels were noted in the left eye.

The lack of sufficient suppression of de novo vessels can be explained by 1 MNV type, deposition of lipofuscin-like material and presence of a large adductor vessel - a branch of the posterior short ciliary artery, which in general are prognostically unfavorable factors determining possible relapses and progression of pathological retinal changes. At the same time, during the natural disease course, the vitelliform material was displaced by gravity into the lower segments of the macular region and its further resorption, while the active adductor vessel was preserved.

Conclusion: Kids’ diagnosis of Best's vitelliform macular dystrophy is based on a complex of studies, including the collection of genealogical history, OCT, OCTA, autofluorescence. It is possible to verify the disease at early stages of the pathological process, to conduct timely therapy and adequate monitoring with an assessment of the activity degree of newly formed vessels and structural retinal changes. The key factors determining the need for antiangiogenic therapy in children with Best's disease are not only the active form of MNV, but also structural retinal changes and the state of visual functions.

Keywords: Best's Disease; Macular Neovascularization (MNV); Vitelliform Macular Dystrophy; Bestrophin; Antiangiogenic Therapy

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Citation

Citation: Andrey Kopylov and Anna Korolenko. “Best's Disease: Diagnostic Criteria and Antiangiogenic Therapy Efficacy - A Case Report".Acta Scientific Ophthalmology 6.3 (2023): 07-13.

Copyright

Copyright: © 2022 Andrey Kopylov and Anna Korolenko. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.