Adult Onset Best Vitelliform Macular Dystrophy: A Case Report

Authors

  • 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

Hari Sharma, Dristi Eye Hospital, Birgunj , Nepal

Consultant Ophthalmologist

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

Consultant Ophthalmologist

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

Consultant Ophthalmologist

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

Optometrist

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Published

2020-05-21

How to Cite

Gupta, S., Sharma, H., Yadav, R., Singh, S. K., & Chaudhary, S. (2020). Adult Onset Best Vitelliform Macular Dystrophy: A Case Report. Journal of Lumbini Medical College, 8(1), 89–91. Retrieved from https://nepjol.info/index.php/JLMC/article/view/40717

Issue

Vol. 8 No. 1 (2020)

Section

Case Reports

License

Copyright (c) 2020 Sharad Gupta, Hari Sharma, Reena Yadav, Sanjay Kumar Singh, Sushma Chaudhary

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