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[Benign concentric annular macular dystrophy: two cases].

M J Pérez Alvarez1, F Clement Fernández

  • 1Hospital Universitario de La Princesa, Madrid, España. mjperezalv@hotmail.com

Archivos De La Sociedad Espanola De Oftalmologia
|September 5, 2003
PubMed
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Two sisters diagnosed with benign concentric annular macular dystrophy showed decreased vision and a characteristic bull's eye pattern on fluorescein angiography. Accurate diagnosis is crucial for good prognosis, distinguishing it from similar conditions.

Area of Science:

  • Ophthalmology
  • Medical Genetics

Background:

  • Benign concentric annular macular dystrophy (BCAMD) is a rare inherited retinal disorder.
  • Early diagnosis is essential for appropriate patient management and genetic counseling.

Observation:

  • Two sisters, aged 16 and 22, presented with reduced visual acuity.
  • Both exhibited annular atrophy of the macular retinal pigment epithelium.
  • Fluorescein angiography revealed a distinct "bull's eye" maculopathy pattern.

Findings:

  • Electrophysiologic tests, including electroretinogram and electrooculogram, were normal in both cases.
  • The clinical presentation and diagnostic findings are consistent with BCAMD.

Implications:

  • Accurate differentiation of BCAMD from other macular dystrophies with similar angiographic findings is critical.

Related Experiment Videos

  • Fluorescein angiography and electrophysiologic testing are valuable tools for diagnosing BCAMD.
  • The generally good prognosis for BCAMD underscores the importance of precise diagnosis.