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Asymptomatic peripheral elevated lesion found in 71-year-old man
The lesion was surrounded by exudate, subretinal and sub-RPE hemorrhage, and fibrosis.
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A 71-year-old man was referred for evaluation of a peripheral elevated retinal lesion in the left eye found incidentally on an annual exam. The patient was asymptomatic and denied distortion or loss of vision. His ocular history was significant for amblyopia in the right eye, and he had been treated with strabismus surgery as a child. He had no pertinent medical or family history.
Examination
The patient’s best corrected visual acuity was 20/50 in the right eye and 20/25 in the left eye. Pupils were round and reactive to light and accommodation without evidence of afferent pupillary defects. Extraocular movements and IOPs were within normal limits. On slit lamp biomicroscopy, the anterior segments were notable only for mild nuclear sclerotic cataracts in both eyes.
Posterior segment examination of the left eye revealed a peripheral elevated lesion with surrounding exudate, subretinal and sub-retinal pigment epithelium (RPE) hemorrhage, and fibrosis (Figure 1). The maculae were normal, as was the peripheral retina in the right eye. Fluorescein angiography of the lesion revealed both blockage and late leakage. Optical coherence tomography of the lesion demonstrated both intraretinal and sub-RPE hyporeflective areas as well as hyperreflective material in the outer retina (Figure 2). OCT through the maculae were within normal limits.
Figure 1. Color fundus photograph of the left eye demonstrates an inferior peripheral elevated gray lesion with a ring of exudate and whitening.
Images: Branchini L, Reichel E
Figure 2. Time-domain OCT through the peripheral lesion. Note the hyporeflective material in both the subretinal and sub-RPE spaces.
What is your diagnosis?
Peripheral elevated lesion
The diagnosis of peripheral exudative hemorrhagic chorioretinopathy, or PEHCR, can be made clinically based on funduscopic appearance and fluorescein angiography. Lesions may be bilateral and demonstrate hemorrhage involving subretinal and/or sub-RPE spaces. Lesions may also have associated exudate. Fluorescein angiogram typically demonstrates early hypofluorescence due to blockage from hemorrhage and late retinal vascular leakage.
A differential diagnosis of a peripheral elevated lesion with a hemorrhagic and/or exudative component includes metastatic tumor, choroidal hemangioma, retinal capillary hemangioma and macroaneurysm. In the absence of exudates, choroidal melanoma may be considered. In a younger patient, one may consider familial exudative vitreoretinopathy and Coats’ disease.
Discussion
PEHCR was first described by Reese and Jones in 1962. Since that period, it has been referred to by a variety of names, including eccentric disciform, extramacular disciform, peripheral age-related degeneration, hemorrhagic peripheral pigment epithelium disease, hemorrhagic detachment of the peripheral RPE and peripheral choroidal neovascularization. It is a peripheral retinal phenomena involving hemorrhage in both the subretinal and sub-RPE spaces with or without exudate. It is often, although not necessarily, bilateral.
The largest series of this entity published by Shields et al described the findings of 173 eyes of 146 patients. The average age of the cohort was 80 years, with a female predominance of 67%. Ninety-nine percent of subjects were Caucasian. Typical lesion features included temporal location in 77% of eyes. Lesion location between the equator and ora serrata was found in 90% of eyes. Bilateral lesions were found in 31% of subjects. Subretinal hemorrhage was found in 78% of eyes, serous RPE detachment in 28% of eyes, sub-RPE hemorrhage in 26% of eyes, subretinal fluid in 19% of eyes, vitreous hemorrhage in 24% of eyes and subretinal exudates in 21% of eyes.
Typical B-scan findings were an elevated lesion that was hollow (53%) or solid (47%). No lesion was observed to have vascular pulsations, which may be seen in choroidal melanoma. Fluorescein angiogram most often demonstrated blockage. Indocyanine green (ICG) angiography, when performed, most frequently showed “hot spot” hyperfluorescence with blockage. Of note, age-related macular degeneration was observed in the same or fellow eye. Specifically, RPE changes including hyperplasia or atrophy were observed in 75% of eyes and drusen in 60% of eyes.
The mean follow-up time was 15 months, with 89% of cases demonstrating regression or stability of the PEHCR lesion. Progression was documented in 11% of cases. Recurrent or new lesions were found in 17% of cases, 9% of which were found in the contralateral eye. Eight percent of cases developed AMD in the ipsilateral eye. Thirty-six eyes (21%) were observed to have decreased visual acuity. Of these 36 eyes, 24 had decrease in visual acuity attributable to vitreous hemorrhage. Subretinal hemorrhage extending into the macular region was the cause in eight eyes (5%). Subretinal fluid extending into the macular region was the cause in four eyes (2%).
There has been discussion in the literature about a link between polypoidal choroidal vasculopathy (PCV) and PEHCR. Goldman et al reported a series of 10 patients with ICG findings suggestive of PCV who had peripheral retinal lesions consistent with PEHCR. In 2013, Mantel et al described PCV-like lesions using a wide-field contact lens system and ICG in 48 eyes of 40 patients with PEHCR.
Follow-up
The patient was initially observed. Due to a decrease in visual acuity to 20/70 in the left eye secondary to extension of exudates and fluid on a subsequent visit, he was treated with intravitreal Avastin (bevacizumab, Genentech) on two occasions, which induced inactivity. He eventually developed bilateral disease (Figure 3).
Figure 3. Color fundus mosaic demonstrates bilateral peripheral lesions.