Posterior Polar Central Choroidal Dystrophy: A Case Report

Area of Science:

• Ophthalmology
• Retinal Diseases
• Choroidal Dystrophies

Background:

• Posterior polar central choroidal dystrophy is a rare condition causing progressive vision loss.
• Characterized by bilateral, symmetric atrophy of the retinal pigment epithelium and choriocapillaris.
• Diagnosis often requires a combination of clinical examination and specialized imaging.

Purpose of the Study:

• To present a comprehensive diagnostic workup of a patient with posterior polar central choroidal dystrophy.
• To illustrate the utility of fluorescein angiography, fundus autofluorescence, and optical coherence tomography in diagnosing this rare condition.
• To document the electrophysiological findings in a case of posterior polar central choroidal dystrophy.

Main Methods:

• Fundus examination revealed bilateral symmetric atrophy in the posterior pole.
• Fluorescein angiography demonstrated visible large choroidal vessels.
• Fundus autofluorescence showed distinct hypoautofluorescent areas.
• Optical coherence tomography confirmed atrophy of the choriocapillaris and outer retina.
• Electrophysiological tests included flash, pattern, and multifocal electroretinograms (ERG).

Main Results:

• The patient exhibited decreased visual acuity and characteristic fundoscopic findings.
• Imaging revealed extensive atrophy of the retinal pigment epithelium and choriocapillaris.
• Electrophysiology showed subnormal and minimal responses across various ERG tests.
• These findings were consistent with a diagnosis of posterior polar central choroidal dystrophy.

Conclusions:

• Posterior polar central choroidal dystrophy presents with specific patterns of retinal and choroidal atrophy.
• Multimodal imaging (FA, FAF, OCT) and ERG are crucial for diagnosis.
• This case underscores the importance of advanced diagnostic tools in identifying rare retinal dystrophies.