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Keywords:

  • Best's disease;
  • macular hole

An eight-year-old boy presented with decreased near and distance vision in both eyes. The right ocular fundus showed a giant macular hole surrounded by a vitelliform ring. Spectral domain optical coherence tomography (SD-OCT) revealed a full-thickness macular hole with cystic spaces at the edges. The left fundus had an egg-yolk like appearance of 1.5-disc diameter at the macular region. SD-OCT showed an optically empty space between retinal pigment epithelium and the neurosensory retina. Fundus autofluorescence confirmed the presence of lipofuscin in both eyes. The Arden ratio was 1.38 for the right eye and 1.61 for the left. There was no surgical intervention because of the poor prognosis due to large size of the hole. Environmental modification and contrast-enhancing measures were explained to the patient and his parents.