Purpose: Polypoidal choroidal vasculopathy (PCV) is characterized by polyp-like sub-retinal pigment epithelium vascular abnormalities predominantly found in the macula and peripapillary region. Less commonly, PCV can be found peripherally and be a cause of peripheral exudative hemorrhagic chorioretinopathy (PEHCR). We sought to further describe the clinical spectrum of this ill-defined subgroup of PEHCR.
Methods: A retrospective observational case series, of 10 eyes of 8 patients diagnosed with PEHCR caused by peripheral PCV, was conducted. In all cases, the presence of PCV was confirmed with indocyanine green angiography and/or fluorescein angiography and optical coherence tomography. The clinical presentation, natural history, and clinical outcomes with or without intervention were studied.
Results: Patients with PEHCR caused by peripheral PCV were most commonly men, white, asymptomatic, and had a concomitant diagnosis of age-related macular degeneration. The mean age was 70 years (range, 59-82 years) with a mean follow-up of 32.5 months (range, 4-91 months). Four patients had unilateral involvement with minimal subretinal hemorrhage that resolved spontaneously, one patient had unilateral involvement outside the macula that responded to anti-vascular endothelial growth factor therapy, one patient had unilateral involvement with subretinal hemorrhage threatening the macula that responded to anti-vascular endothelial growth factor therapy, and two patients had extensive bilateral subretinal hemorrhage requiring surgical intervention. Both patients with multiple lesions in one eye had bilateral lesions (two of eight patients). Lesions were most commonly located in the temporal periphery (8 of 10 eyes).
Conclusion: A new subclassification is proposed that includes both eyes with polyps and those without polyps within the spectrum of disease described previously as PEHCR. Within the spectrum of disease described previously as PEHCR exists a subgroup of lesions caused by peripheral PCV, which has not been well defined before this report. The largest case series to date of eyes with PEHCR due to peripheral PCV, a unique form of type 1 neovascularization, is further classified and described. These eyes have a spectrum of disease, including small, medium-sized, and large lesions. Although most eyes with PEHCR from peripheral PCV experience a benign course with spontaneous resolution, a subset of eyes may experience macula-threatening hemorrhage, requiring treatment with laser-based therapies, anti-vascular endothelial growth factor injections, or surgical intervention.