Best Disease Follow-up

Updated: Oct 10, 2016
  • Author: Michael Altaweel, MD, FRCSC; Chief Editor: Hampton Roy, Sr, MD  more...
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Follow-up

Further Outpatient Care

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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Complications

Although uncommon, choroidal neovascularization can occur following the atrophic stage, and it can be responsible for further deterioration in visual acuity. A disciform scar may result.

Plaques of white subretinal fibrous tissue can develop in conjunction with the atrophic stage. Visual acuity is often reduced to 20/100 or worse with this appearance.

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Prognosis

Prognosis for Best disease is mixed. Some carriers will never phenotypically express the disorder. Some individuals will never have progression beyond the earliest stages of the disease and will maintain better than 20/40 vision in both eyes. In general, most people will maintain reading vision in at least 1 eye throughout life. In one study, 88% of patients retained 20/40 or better visual acuity, and only 4% of them had 20/200 or worse visual acuity in the better eye. The deterioration of vision usually is very slow and is not significant in most individuals until after age 40 years. [8, 32, 19]

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Patient Education

Genetic inheritance: Provide an explanation of autosomal dominant inheritance to the patient and family members. In genetic counseling, discuss carrier state, variable penetrance and expressivity, and implications for offspring. Recommend familial evaluation.

Occupational counseling: Discuss the patient's prognosis and the possible implications on career direction.

Routine examination: Emphasize regular examinations because changes in fundus appearance over time may elucidate the eventual prognosis. Conduct evaluation for choroidal neovascularization.

Amsler grid: Teach use of this tool to identify central visual field changes.

Low vision aids: Assistive devices may be necessary if visual acuity deteriorates. Refer to a low vision specialist or organization.

For excellent patient education resources, visit eMedicineHealth's Eye and Vision Center. Also, see eMedicineHealth's patient education article Macular Degeneration.

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