Resumen

BERDUGO-MESA, Sara; GRACIA, Sydney Goldfeder de; OCAMPO-RAMIREZ, Sara  y  VARGAS-OLMOS, Andrea. Vitelliform macular dystrophy and its association with choroidal neovascular membrane in pediatric age. CES Med. [online]. 2019, vol.33, n.2, pp.134-143. ISSN 0120-8705.  https://doi.org/10.21615/cesmedicina.33.2.7.

Best’s disease, also called vitelliform macular dystrophy is an autosomal dominant maculopathy, which is caused by mutations in the BEST 1 gene, located in the retina pigment epithelium. Is part of the five retina degenerative diseases clinically distinguished as bestrophinopathies. Affected individuals, in the early disease stages may present normal visual acuity; however, in most patients there is a progressive loss of this material in the deposit of subfoveal yellow material. Some patients may also present choroidal neovascularization, an uncommon complication. In pediatric population, early disease treatment associated with choroidal neovascularization with ranibizumab has shown a prognosis improvement. Although more studies are needed to assess its efficacy in this condition and good response to treatment with other medications, this still being the first choice. We present a patient who at thirteen years was diagnosed with VMD and was successfully treated with ranibizumab for two years.

Palabras clave : Bestrophins; Viteliform macular dystrophy; Choroidal Neovascularization; Antibodies, Monoclonal; Ranibizumab.

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