PACHYCHOROID PIGMENT EPITHELIOPATHY

In Brief Purpose: To report nine cases of pachychoroid pigment epitheliopathy. Methods: An observational case series of nine patients who underwent comprehensive ophthalmic examination, fundus photography, fundus autofluorescence, spectral-domain optical coherence tomography, and enhanced depth imaging optical coherence tomography. Results: Eighteen eyes of 9 patients, aged 27 years to 89 years, were diagnosed with pachychoroid pigment epitheliopathy based on the characteristic funduscopic appearance of reduced fundus tessellation with overlying retinal pigment epithelial changes in one or both eyes, fundus autofluorescence abnormalities, and increased subfoveal choroidal thickness confirmed by enhanced depth imaging optical coherence tomography (mean, 460.2 μm). The five older patients had been previously diagnosed with age-related macular degeneration, while the four younger subjects were referred for possible inflammatory chorioretinitis, pattern dystrophy, or nonspecific drusen. No subjects had a history of or subsequently developed subretinal fluid. Conclusion: Pachychoroid pigment epitheliopathy falls within a spectrum of diseases associated with choroidal thickening that includes central serous chorioretinopathy and polypoidal choroidal vasculopathy, and it should be suspected in eyes with a characteristic fundus appearance related to choroidal thickening and associated retinal pigment epithelial abnormalities but no history of subretinal fluid. Enhanced depth imaging optical coherence tomography confirming an abnormally thick choroid and characteristic retinal pigment epithelial changes on fundus autofluorescence support the diagnosis. Because these patients are frequently misdiagnosed, the recognition of pachychoroid pigment epitheliopathy may avoid unnecessary diagnostic testing and interventions. A unique entity termed pachychoroid pigment epitheliopathy characterized by reduced fundus tessellation related to increased choroidal thickness, overlying retinal pigment epithelial abnormalities, fundus autofluorescence changes, and an absence of subretinal fluid is described. Pachychoroid pigment epitheliopathy appears to fall within a pathophysiologic spectrum of diseases related to choroidal thickening, which includes central serous chorioretinopathy and polypoidal choroidal vasculopathy.