A Case of Posterior Polar Hemispheric Choroidal Dystrophy Successfully Diagnosed With Ultra-Widefield Fundus Autofluorescence and Optical Coherence Tomography Angiography

Cureus. 2024 Mar 10;16(3):e55878. doi: 10.7759/cureus.55878. eCollection 2024 Mar.

Abstract

We report a case of a 78-year-old man presenting with uncertain visual field loss, ultimately identified as posterior polar hemispheric choroidal dystrophy (PPHCD) using ultra-widefield fundus autofluorescence (FAF) and optical coherence tomography angiography (OCTA). The patient initially reported blurred vision in the left eye and had a previous diagnosis of suspected bilateral normal tension glaucoma based on optic nerve head excavation and static perimetry measurements. Detailed examination revealed suspicious retinal atrophy. Notably, the patient had a tigroid fundus, which complicated the correlation between visual field defect and chorioretinal atrophy. Ultra-widefield FAF highlighted mosaic/patchy hypofluorescent areas, emphasizing this atrophy. OCTA images confirmed choriocapillaris loss in the hemispheric choroidal atrophy and parafoveal atrophy. The combination of these imaging techniques enabled a definitive diagnosis of PPHCD. Long-term follow-up and continued investigation with these imaging modalities may hold promise for a better understanding of disease progression and management in similar cases.

Keywords: hemispheric peripapillary chorioretinal atrophy; hypofluorescence; optical coherence tomography angiography; retinal pigment epithelium; ultra-wide field fundus autofluorescence.

Publication types

  • Case Reports