Variable Presentations of Peripheral Exudative Hemorrhagic Chorioretinopathy.

Three cases of peripheral exudative hemorrhagic chorioretinopathy (PEHCR), which mimicked other retinal pathologies, were reported. Different preliminary diagnoses were made initially, but thorough examination combined with the appropriate investigations led to the final diagnosis of PEHCR. Despite the rare occurrence of PEHCR, it must be included in the differential diagnosis for peripheral retinal diseases. Copyright:

Introduction

Peripheral exudative hemorrhagic chorioretinopathy (PEHCR) is a rare disease, manifesting in elderly patients as a consequence of the degenerative process affecting the retinal periphery.[12] It was first described by Reese and Jones,[3] who described 34 eyes showing subretinal pigment epithelial hematoma that resembled the changes associated with age-related macular degeneration. However, these changes were seen at the periphery in four eyes. In 1976, Silva and Brockhurst[4] reported similar findings in six patients, which they described as “hemorrhagic detachment of the peripheral retinal pigment epithelium (RPE).” The term “PEHCR” was first reported by Annesley,[5] which is still used for describing these peripheral degenerative changes. The disease presents with a variety of features, including subretinal hemorrhage, subretinal exudation, retinal pigment epithelial detachment, or even vitreous hemorrhage.[2] PEHCR is classified as one of the diseases that mimic the appearance of uveal melanoma (pseudomelanoma), due to the striking similarities between the two pathologies, which require differentiation based on a thorough assessment.[12367] Herein, we report three cases of PEHCR with varying presentations, all of which were given inaccurate preliminary diagnoses but had the same pathology.

Case Reports

Case 1

An 81-year-old male, who was prescribed oral antihyperglycemic drugs for diabetes, had multiple sessions of panretinal photocoagulation for proliferative diabetic retinopathy. The patient was administered three intravitreal injections of antivascular endothelial growth factor (ranibizumab) in the right eye for subfoveal choroidal neovascular membranes that led to cicatrization. The patient presented to the emergency department complaining of a sudden decrease in vision of the left eye, associated with floaters, for 1 week. On examination, the best-corrected visual acuity (BCVA) was 2/200 in the right eye and 5/200 in the left eye. Intraocular pressure was 11 mmHg in both eyes. The anterior segment examination of both eyes showed no abnormality. Fundus examination of the right eye revealed involuted diabetic retinopathy, with cicatrized choroidal neovascular membranes, but the left eye showed diffuse vitreous hemorrhage with an area of subretinal hemorrhage in the superotemporal quadrant [Figure 1]. Brightness-scan (B-scan) ultrasound revealed a dense, vitreous opacity with posterior vitreous detachment. Bullous retinal detachment was detected at the superotemporal quadrant. Pars plana vitrectomy was performed to aspirate the blood from the vitreous cavity. Subsequent fundus examination showed a collection of subretinal blood, with no retinal breaks or other abnormalities. Subretinal blood showed regression on follow-up visits and macular spectral-domain optical coherence tomography (SD-OCT) showed no involvement of the macular area.

Figure 1

(a) B-scan ultrasound for the left eye shows dense vitreous opacity with associated bilobular retinal elevation and detachment by subretinal opacities (b) Fundus photo of the left eye after performing pars planar vitrectomy to clear the VH shows subretinal blood with laser scars from previous pan-retinal photocoagulation (c) Fundus photograph of the right eye shows disciform changes in the macular area associated with dispersed hard exudates without macular edema and peripheral retinal scars from previous pan-retinal photocoagulation (d) Fundus photograph of the left eye after 2 years of surgery shows the resolution of the subretinal blood from areas with retinal pigment epithelium changes and hyperpigmentation and an area of fibrotic scar at the periphery

Case 2

An 80-year-old female, with a long-standing history of diabetes mellitus and hypertension, was on medication for the same. The patient had no history of ocular interventions and she was referred from another institute, with a diagnosis of choroidal melanoma in the left eye, which was discovered during routine follow-up for retinal assessment. On examination, the BCVA was 20/80 for both eyes. Intraocular pressure was 17 mmHg in the right eye and 19 mmHg in the left eye. Anterior segment examination of both eyes revealed no abnormality. Fundus examination of the right eye showed a normal posterior pole with scattered areas of depigmentation in the superotemporal quadrant. Fundus examination of the left eye revealed a normal posterior pole and a well-circumscribed area of hyperpigmentation in the subretinal area resembling subretinal blood temporally, at the far periphery, with an adjacent streak of blood extending inferiorly, with an area of subretinal exudation in the inferotemporal quadrant [Figure 2]. B-scan ultrasound for the assessment of the suspected lesion in the left eye showed cystic elevation with a diameter of 2.73 mm and height of 1.94 mm. Intravenous fluorescein angiography (IVFA) revealed persistent fluorescence blockage by blood, and no internal circulation was observed within the lesion, which is usually seen in choroidal melanoma. SD-OCT showed preserved foveal contour, with macular involvement of the lesion.

Figure 2

(a) Fundus photograph of the left eye shows a small area of peripheral subretinal hemorrhage with an adjacent area with a blood streak, in addition to the area with exudation (b) Fundus photograph of the right eye shows area of retinal pigment epithelium changes with hypopigmentation (c) Fluorescein angiography of the left eye in the late phase shows masked fluorescence due to blockage by subretinal blood (d) Fluorescein angiography of the right eye shows an hyperfluorescence resembling a window defect due to retinal pigment epithelium alterations (e) B-scan ultrasound for the left eye shows cystic lesion with documented medium reflectivity

Case 3

A 74-year-old female, with a history of clotting disorders, was prescribed anticoagulants for the same. She had a history of uneventful cataract extraction with implantation of intraocular lenses in both eyes. The patient presented with floaters in the right eye for 1 month and was initially diagnosed with vasoproliferative tumor. On examination, the BCVA was 20/100 in the right eye and 20/25 in the left eye. Intraocular pressure was 16 mmHg for both eyes. Anterior segment examination of both eyes and fundus examination of the right eye showed no abnormalities. Fundus examination of the right eye showed mild vitreous haze, subretinal elevation with blood, and extensive exudation in the inferotemporal quadrant surrounded by an area of retinal pigment epithelial changes. However, no dilated or telangiectatic blood vessels were detected on the lesion. IVFA showed persistent hypofluorescence due to masking effect with the adjacent area of hyperfluorescence, which corresponded to the area of pigment epithelial changes. However, no leakage was detected, which occurs typically in vasoproliferative tumors [Figure 3]. The patient was kept under observation and the condition was stable for over a year of follow-up.

Figure 3

(a) Fundus photograph of the right eye shows mild vitreous haze with inferotemporal retinal elevation with extensive exudation surrounded by alternating areas of retinal pigment epithelium hyperplasia and retinal pigment epithelium atrophy. (b) Fundus photograph of the left eye shows normal anatomical retinal structure with no apparent pathology. (c) Fluorescein angiography of the right eye in shows hypofluorescent lesion corresponding with the area of elevation and the area of retinal pigment epithelium atrophy surrounded by irregular hyperfluorescence due to window defect no dilated feeder vessels or leakage effect noted. (d) Fluorescein angiography of the left eye is normal

Discussion

PEHCR is a rare retinal disease that occurs due to the degenerative processes that affect the retina, which typically affects elderly individuals and manifests with subretinal hemorrhage and exudation.[12] Advanced age is an important criterion for diagnosing this disease. In a study by Shields et al.[2] that included 146 patients, the mean age was 80 years, with a range of 57–97 years. The patients reported in this study are all above 70 years of age, which is approximately the mean age reported in the literature.[128] The disease is predominant in women.[12589] Two of the patients in this study were women, and though the sex-based difference is considered to be negligible due to the small sample size, it corresponds to the findings reported in the literature. All the patients had associated chronic conditions, including diabetes mellitus, hypertension, or the need for the administration of anticoagulants for a clotting disorder. Shield et al.[2] reported the association of PEHCR with diabetes mellitus, hypertension, and use of anticoagulants to be 14%, 51%, and 44%, respectively. Hypertension is the most persistent finding among the medical conditions associated with PEHCR in the literature, with the prevalence as high as 44%[5] and 55%.[1]

Temporal quadrant involvement of the disease was noted in all the patients in this study, in line with the findings of other reports, with respect to the unexplained tendency for temporal involvement in a majority of cases.[125] All the cases presented with subretinal hemorrhage, which is known to be the most common retinal finding in PEHCR, observed in more than 70% of the cases.[128] Subretinal exudation is a common finding of the disease. Although its presence is less common than subretinal hemorrhage, it is extremely helpful in differentiating PEHCR from choroidal melanoma.[25] Bilateral involvement is considered to be an important characteristic of PEHCR. The involvement of the contralateral eye can be symmetrical or asymmetrical and macular or extramacular in nature. Two of the patients in this study showed contralateral retinal findings. Case 1 had disciform choroidal neovascular membranes, and Case 2 had peripheral RPE changes. The bilaterality can be attributed to the degenerative process affecting the retina, and the asymmetrical presentation could be attributed to the difference in the stage of the disease in the contralateral eye. This feature is very helpful in excluding uveal melanoma from the differential diagnosis in these cases.[12] Moreover, the examination of the periphery of the retina in patients with age-related macular degeneration should be performed to prevent any confusion during the final diagnosis stage.[8]

The principal etiology of decrease in vision in PEHCR is due to vitreous hemorrhage, especially the dense type, which obscures the view to the retina.[2] This association is better understood by taking into account the effect of vitreous hemorrhage on central vision, which is not affected by the other findings observed peripherally. PEHCR should be suspected in any patient with vitreous hemorrhage with bullous retinal detachment, especially if the contralateral eye shows any retinal degenerative manifestations.

Advances in multimodal imaging in the retina, especially after the introduction of ultra-widefield retinal imaging, have an important role in the diagnosis, documentation, and follow-up of PEHCR cases. Therefore, ancillary testing should be performed for patients with PEHCR for the confirmation and differentiation from other pathologies. Compared to previously documented PEHCR cases in Shields’ paper,[2] this case series has the advantage of using more advanced technological imaging modalities in documenting the cases.

Shields et al.[2] presented the features of PEHCR in their study. The principal results were that no patients had shown intrinsic pulsation or mushroom-shaped growth appearance. The high internal reflectivity and retraction cleft were the factors distinguishing PEHCR from choroidal melanoma but were not present in all participants. Fundus fluorescein angiography typically shows fluorescence blockage due to subretinal hemorrhage, with irregular hyperfluorescence due to the window defect corresponding with RPE alterations. Late hyperfluorescence on fluorescein angiography due to leakage has been reported rarely, which is attributed to choroidal neovascular membranes. The absence of internal circulation is an important feature that eliminates choroidal melanoma.[2]

The third patient in this study has been diagnoses preliminarily with vasoproliferative tumor. Vasoproliferative tumors share some characteristics with PEHCR, including the tendency to involve the inferotemporal quadrant and the fundus appearance of hemorrhage, exudation, RPE changes, and vitreous hemorrhage. Fundus fluorescein angiography can be very helpful in establishing the final diagnosis.[10] Vasoproliferative tumors usually present with dilated feeder vessels over the mass with early hyperfluorescence due to filling effect, which increases in the late stages due to leakage, reflecting the abnormal vascular component near the mass, which was absent in our patient.[11]

The study's limitations include its retrospective nature, the small number of subjects, and lack of indocyanine green angiography testing.

PEHCR should always be included in the differential diagnosis for any peripheral retinal pathology. Examination of the retinal periphery should be always be considered for a patient with macular degeneration to document these degenerative lesions, if present.

Conclusion

Despite the rare presentation of PEHCR, it should always be included in the differential diagnosis of peripheral retinal diseases.

Ethical consideration

This case series that includes 3 patients was approved by the Internal Review Board (IRB)/Ethical Committee at King Khaled Eye Specialist Hospital (KKESH), Riyadh, Saudi Arabia, and adhered to the tenets of the Declaration of Helsinki.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.