Adult Onset Best Vitelliform Macular Dystrophy: A Case Report

  • Sharad Gupta Sagarmatha Choudhary Eye Hospital, Siraha, Lahan, Nepal https://orcid.org/0000-0001-8654-5172
  • Hari Sharma Dristi Eye Hospital, Birgunj , Nepal
  • Reena Yadav Sagarmatha Choudhary Eye Hospital, Siraha, Lahan, Nepal
  • Sanjay Kumar Singh Sagarmatha Choudhary Eye Hospital, Siraha, Lahan, Nepal
  • Sushma Chaudhary Sagarmatha Choudhary Eye Hospital, Siraha, Lahan, Nepal
Keywords: Best disease, Fundus fluorescence angiography, Optical coherence tomography

Abstract

Introduction: Best disease or vitelliform macular dystrophy is a rare autosomal dominant disorder with bilateral presentation characterized by subretinal accumulation of yellowish material in the macular area. Macular findings range from a small yellow spot, multiple vitelliform or atrophic lesions to a chorio-retinal scar. Case report: A 35 years male presented to our outpatient department with chief complaint of blurring of vision of both eyes for the past three months. His visual acuity was 6/12 in both right and left eyes. On slit-lamp bio-examination anterior segments of both eyes were normal. On fundus examination, both eyes revealed a single, circular, yellow-opaque egg yolk-like macular lesion with no other abnormalities. Optical coherence tomography of both eyes revealed deposits of homogenous hyper- reflective material beneath retinal pigment epithelium at fovea. Fundus fluorescence angiography showed blocked fluorescence at the site of vitelliform lesion of both eyes. Conclusion: Best vitelliform macular dystrophy is a rare genetic disorder with incomplete penetrance. Optical coherence tomography and fundus fluorescence angiography support the diagnosis.

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Author Biographies

Sharad Gupta, Sagarmatha Choudhary Eye Hospital, Siraha, Lahan, Nepal

Consultant Ophthalmologist,

Sagarmatha Choudhary Eye Hospital.

Hari Sharma, Dristi Eye Hospital, Birgunj , Nepal

Consultant Ophthalmologist,

Dristi Eye Hospital, Birgunj.

Reena Yadav, Sagarmatha Choudhary Eye Hospital, Siraha, Lahan, Nepal

Consultant Ophthalmologist,

Sagarmatha Choudhary Eye Hospital.

Sanjay Kumar Singh, Sagarmatha Choudhary Eye Hospital, Siraha, Lahan, Nepal

Consultant Ophthalmologist,

Sagarmatha Choudhary Eye Hospital.

Sushma Chaudhary, Sagarmatha Choudhary Eye Hospital, Siraha, Lahan, Nepal

Optometrist,

Sagarmatha Choudhary Eye Hospital.

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Published
2020-05-21
How to Cite
1.
Gupta S, Sharma H, Yadav R, Singh S, Chaudhary S. Adult Onset Best Vitelliform Macular Dystrophy: A Case Report. J Lumbini Med Coll [Internet]. 21May2020 [cited 25Sep.2020];8(1):89-1. Available from: https://jlmc.edu.np/index.php/JLMC/article/view/316
Section
Case Report