Best Disease Treatment & Management

Updated: Oct 01, 2018
  • Author: Michael Altaweel, MD, FRCSC; Chief Editor: Donny W Suh, MD, FAAP  more...
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Treatment

Medical Care

No treatment exists for vitelliform macular dystrophy (Best disease). Secondary choroidal neovascularization (CNVM) can be managed with direct laser treatment or photodynamic therapy [32, 33] ; however, treatment with anti–vascular endothelial growth factor (VEGF) therapy, including intravitreal injection of bevacizumab, has been reported more recently. [34, 5] CNVMs may spontaneously resolve without treatment, but vision outcomes are better with anti-VEGF than with observation alone. [34]

Evaluation of family members is important to identify carriers and individuals with vitelliform macular dystrophy. Both genetic counseling and career counseling are provided.

Future directions for research may include gene therapy targeting BEST1. [35]

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Consultations

Consult a vitreoretinal disease specialist for the initial diagnosis, electrophysiology testing, and family assessment, as well as for the long-term follow-up care of patients to monitor disease progression and choroidal neovascularization.

Consult a low vision specialist who can provide specialized equipment to assist individuals who have significant deterioration in visual acuity in both eyes.

Occupational counseling is important. Although most patients retain reading vision in at least 1 eye throughout life, visual deterioration can occur, particularly beyond age 40 years. This knowledge may influence the choice of career.

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Diet

Diet is not known to influence the progression of Best disease.

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Activity

Physical activity does not influence the progression of Best disease.

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Long-Term Monitoring

Examination of visual acuity and fundus lesions should be performed on a schedule dictated by the current stage of the disease. If visual changes occur at any stage, then an earlier visit should be scheduled, as follows:

  • Previtelliform stage - Yearly

  • Vitelliform/pseudohypopyon stage - Every 6 months

  • Scrambled egg stage - Every 6 months

  • Atrophic stage - Every 6 months to yearly

Patients in the atrophic stage should routinely use an Amsler grid. Changes in the central visual field should prompt an early visit to evaluate for choroidal neovascularization.

The electrophysiology test is usually only necessary once to establish the diagnosis. Initial results remain fairly stable during disease progression.

Fluorescein angiography should be performed at any visit if choroidal neovascularization is suspected.

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