POSTERIOR POLAR ANNULAR CHOROIDAL DYSTROPHY: A CASE SERIES

Area of Science:

• Ophthalmology
• Medical Imaging
• Genetics

Background:

• Posterior polar annular choroidal dystrophy (PPACD) is a rare chorioretinal disorder.
• Limited information exists on its characteristic imaging findings.

Observation:

• Two patients with PPACD were analyzed retrospectively.
• Multimodal imaging included spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), and fluorescein angiography (FA).
• Electroretinography (ERG) was also performed.

Findings:

• Bilateral peripapillary atrophy with foveal sparing was observed.
• SD-OCT revealed outer retinal atrophy with foveal sparing.
• FA showed window defects with late staining.
• FAF demonstrated central hypoautofluorescence and leading-edge hyperautofluorescence.
• ERG indicated cone loss with rod preservation.

Implications:

• This study details the multimodal imaging characteristics of PPACD.
• Findings can guide clinicians in evaluating and managing this rare condition.
• Enhanced understanding of PPACD imaging aids in differential diagnosis.