Peripheral Exudative Hemorrhagic Chorioretinopathy (PEHCR): Diagnostic and Therapeutic Challenges.

Background and Objectives: Peripheral exudative hemorrhagic chorioretinopathy (PEHCR) is a peripheral retinal vascular abnormality that is likely underreported. We review the differential diagnoses, etiology, and treatment options for PEHCR. Methods: We present a case of an asymptomatic 72-year-old female referred following left eye fundus photography finding of the peripheral lesion. Results: Fundus photography demonstrated a large temporal pigment epithelial detachment (PED) with adjacent fibrovascular membrane. Optical coherence tomography (OCT) confirmed the PED with trace subretinal fluid. Fluorescein angiography (FA) demonstrated early and late hypofluorescence of the PED with late leakage of the adjacent temporal fibrovascular membrane. Observation was elected, visual acuity remained unaffected, and the PED spontaneously resolved. Conclusions: Due to the peripheral location, patients often present as asymptomatic; however, vision loss can occur due to vitreous hemorrhage or extension of subretinal fluid, hemorrhage, or exudate to the macula. Commonly, these lesions are referred with concern for choroidal melanoma due to their large, dark, elevated presentation in the peripheral retina. Multimodal testing using B-scan, FA, and OCT is important in establishing the proper diagnosis. PEHCR lesions can often be observed without treatment, though intravitreal injection of anti-VEGF is increasingly used to prevent secondary causes of vision loss.

Características clínicas de la coriorretinopatía exudativa hemorrágica periférica en un centro de referencia en España / Clinical characteristics of peripheral exudative hemorrhagic chorioretinopathy in a referral center in Spain

Objetivo: Publicar las características clínicas de la coriorretinopatía exudativa hemorrágica periférica (CEHP) en la población española. Métodos: Estudio retrospectivo y análisis de los resultados en pacientes con diagnóstico de CEHP. Fueron recogidas las características clínicas, por OCT, el tratamiento utilizado y la evolución posterior al tratamiento. Resultados: Se evaluaron 39 ojos de 23 pacientes con CEHP. La edad promedio al diagnóstico fue de 79 años (66-94 años). El síntoma ocular principal fue la disminución de visión en 26 ojos (66,6%), siendo 11 ojos (28,2%) asintomáticos. El diagnóstico de referencia más frecuente fue sangrado/exudación en 24 ojos (61,5%), le siguió melanoma coroideo con 9 (23,1%). Las hemorragias intra o subretinianas fueron el tipo de lesión periférica más frecuentemente encontrado, en 24 ojos (61,5%). Veinticinco ojos (58,9%) recibieron algún tipo de tratamiento: A 15 ojos (60%) se les realizó inyección intravítrea (IIV) de antioangiogénicos (anti-VEGF); se realizó fotocoagulación con láser en 2 casos (8%), terapia fotodinámica en 2 casos (8%) y 6 casos (18,2%) precisaron vitrectomía vía pars plana (VPP) por hemorragia vítrea. No hubo cambios en la agudeza visual (AV) en los pacientes seguidos con observación entre el inicio 0,66±0,80 (0,04-2,82) y el fin de seguimiento 0,75±0,96 (0,00-2,82) (p=0,352), ni para los que recibieron algún tipo de tratamiento entre inicio 0,78±0,79 (0,04-2,30) y fin 1,22±1,01 (0,04-2,82) (p=0,157), posiblemente debido al gran componente de degeneración macular asociada con la edad (DMAE) atrófica o exudativa presente en ambos grupos (29 ojos presentaron DMAE atrófica o exudativa). Conclusiones: La CEHP es una enfermedad poco frecuente, asociada frecuentemente con DMAE, que se presenta típicamente como una masa periférica que se confunde frecuentemente con melanoma...(AU)

Objective: To publish the clinical characteristics of peripheral exudative hemorrhagic chorioretinopathy (PHEC) in the Spanish population. Methods: Retrospective study and analysis of results in patients diagnosed with PHEC. The clinical characteristics, by OCT, the treatment used and the evolution after treatment were collected. Results: 39 eyes of 23 patients with PHEC were evaluated. The average age at diagnosis was 79 years (66-94 years). The main ocular symptom was low vision in 26 eyes (66.6%); only 11 eyes (28.2%) were asymptomatic. The most frequent referred diagnosis was bleeding/exudation in 24 eyes (61.5%), followed by choroidal melanoma in 9 (23.1%). Intra or subretinal hemorrhages were the type of peripheral lesion most frequently found, in 24 eyes (61.5%). Twenty-five eyes (58.9%) received some type of treatment: 15 eyes (60%) underwent intravitreal injection (IIV) of antiangiogenic agents (anti-VEGF); Laser photocoagulation was performed in 2 cases (8%), Photodynamic therapy in 2 cases (8%) and 6 cases (18.2%) required pars plana vitrectomy (PPV) due to vitreous hemorrhage. There were no changes in visual acuity (VA) in patients followed with observation between baseline 0.66±0.80 (0.04-2.82) and end of follow-up 0.75±0.96 (0.00-2.82) (P=.352), nor for those who received some type of treatment between the beginning 0.78±0.79 (0.04-2.30) and the end 1.22±1.01 (0.04-2.82) (P=.157), possibly due to the large component of atrophic or exudative age-related macular degeneration (AMD) presented in both groups. (29 eyes presented atrophic or exudative AMD). Conclusions: PHEC is a rare pathology, frequently associated with AMD, which typically presents as a peripheral mass that is frequently confused with choroidal melanoma and other intraocular tumors and hence the importance of learning to identify it, making the correct differential diagnosis and avoid unnecessary treatments. Antiangiogenic therapy is effective in most patients with...(AU)

[PEHCR-Peripheral exudative hemorrhagic chorioretinopathy: Diagnosis and treatment]. / PEHCR ­ periphere exsudative hämorrhagische Chorioretinopathie: Erkennen und behandeln.

Peripheral exudative hemorrhagic chorioretinopathy (PEHCR) is a rare degenerative chorioretinal disease with subretinal or subepithelial hemorrhage and exudation in the fundus periphery in older patients. Even though it is the second most common entity among pseudomelanomas and its characteristic features simplify diagnosis, PEHCR is often not recognized and therefore misdiagnosed. In most cases stabilization or regression spontaneously occur. Therefore, treatment for PEHCR should be cautiously selected when function is good and surgical intervention should be preferably considered when visual loss is imminent.

Clinical characteristics of peripheral exudative hemorrhagic chorioretinopathy in a referral center in Spain.

OBJECTIVE: To publish the clinical characteristics of peripheral exudative hemorrhagic chorioretinopathy (PHEC) in the Spanish population. METHODS: Retrospective study and analysis of results in patients diagnosed with PHEC. The clinical characteristics, by OCT, the treatment used and the evolution after treatment were collected. RESULTS: 39 eyes of 23 patients with PHEC were evaluated. The average age at diagnosis was 79 years (66-94 years). The main ocular symptom was low vision in 26 eyes (66.6%); only 11 eyes (28.2%) were asymptomatic. The most frequent referred diagnosis was bleeding/exudation in 24 eyes (61.5%), followed by choroidal melanoma in 9 (23.1%). Intra or subretinal hemorrhages were the type of peripheral lesion most frequently found, in 24 eyes (61.5%). Twenty-five eyes (58.9%) received some type of treatment: 15 eyes (60%) underwent intravitreal injection (IIV) of antiangiogenic agents (anti-VEGF); Laser photocoagulation was performed in 2 cases (8%), Photodynamic therapy in 2 cases (8%) and 6 cases (18.2%) required pars plana vitrectomy (PPV) due to vitreous hemorrhage. There were no changes in visual acuity (VA) in patients followed with observation between baseline 0.66±0.80 (0.04-2.82) and end of follow-up 0.75±0.96 (0.00-2.82) (P=.352), nor for those who received some type of treatment between the beginning 0.78±0.79 (0.04-2.30) and the end 1.22±1.01 (0.04-2.82) (P=.157), possibly due to the large component of atrophic or exudative age-related macular degeneration (AMD) presented in both groups. (29 eyes presented atrophic or exudative AMD) CONCLUSIONS: PHEC is a rare pathology, frequently associated with AMD, which typically presents as a peripheral mass that is frequently confused with choroidal melanoma and other intraocular tumors and hence the importance of learning to identify it, making the correct differential diagnosis and avoid unnecessary treatments. Antiangiogenic therapy is effective in most patients with an active phase (exudative/hemorrhagic) into leading to fibrosis of peripheral hemorrhagic areas.

"Pseudotumors" in Dermatology.

In dermatology, "pseudo" is often used as a prefix for entities resembling another standard condition, either morphologically or histopathologically. Correspondingly, "pseudotumor" is a term encompassing dermatological conditions which are not true proliferations, but either have a clinical resemblance to a known tumor (e.g., Pseudokaposi's sarcoma is actually a non-neoplastic condition) or a histopathological resemblance to one (e.g., pseudo-myogenic hemangioendothelioma named due to a histopathological resemblance between myocytes and tumor cells). Often such a nomenclature can create confusion and unnecessary alarm for both the physicians and the patients. Through this article we attempt to summarise "pseudotumors" in dermatology and classify them into clinical and histopathological "pseudotumors", so as to produce a ready reckoner for this confusing nomenclature.

Photodynamic Therapy for the Treatment of Vascularized Intraretinal Tuberculoma.

PURPOSE: To report a case of intraretinal tubercular granuloma successfully treated with photodynamic therapy (PDT). METHODS: Retrospective case report. Multimodal imaging was performed at each follow-up visit. RESULTS: The tuberculoma did not regress and did not significantly reduce its exudation after anti-tubercular therapy (ATT), systemic steroid therapy and intravitreal anti-VEGF. Second line treatment with PDT was attempted. The lesion showed a regression with reduced sub-retinal fluid and intra-retinal exudates. A second PDT was performed for reactivation of the lesion 5 months after the first treatment. Further regression of the lesion was observed. CONCLUSION: PDT may be a valuable second-line therapeutic approach for vascularized intraretinal granulomas.

Nodular posterior scleritis - The great masquerader.

We aim to report the management of a patient who presented with a choroidal mass masquerading as an amelanotic choroidal melanoma. A 57-year-old male presented with defective vision in his right eye, which was associated with mild periocular pain. Fundus examination showed a large dome-shaped yellowish-orange subretinal mass in the macular region and exudative retinal detachment (RD). Magnetic resonance imaging (MRI) showed a 16 mm × 8 mm choroidal mass, which was hyperintense on T1-weighted images and hypointense on T2-weighted images. B-scan ultrasonography revealed a dome-shaped mass with homogeneous echogenicity, inferior RD, and fluid collection in the sub-Tenon space. There was no choroidal excavation. He was diagnosed as nodular posterior scleritis (NPS) with exudative RD in the right eye. The lesion regressed completely after treatment with oral steroids. Choroidal mass can pose a diagnostic dilemma to ophthalmologists. Atypical MRI features can further augment the confusion. Despite its low incidence, NPS should always be kept as a differential in the presence of an amelanotic choroidal mass.

Choroidal Effusion Mimicking Uveal Melanoma: A Novel Presentation of Idiopathic Systemic Capillary Leak Syndrome.

BACKGROUND: Idiopathic systemic capillary leak syndrome (ISCLS, also known as Clarkson's disease) is a rare medical condition characterized by episodes of capillary endothelial cell dysfunction with leakage of fluid into the interstitial space resulting in severe hypotension, hemoconcentration, hypoalbuminemia, and generalized edema. Each episode can result in multiorgan failure due to systemic hypoperfusion. CASE PRESENTATION: We report a case of uveal effusion, mimicking uveal melanoma, associated with ISCLS following viral infection. A 74-year-old white male was evaluated in our ocular tumor clinic for a large intraocular mass in the right eye concerning for choroidal melanoma. We completed a review of the literature and list clinical recommendations for these cases. ISCLS, although rare, was a significant diagnostic consideration in this patient. Due to the high mortality rate of this condition, accurate diagnosis and prompt treatment was critical. We hypothesize that the mechanism of choroidal effusion development was due to reduced oncotic pressure from rapid decrease in serum albumin. Increased permeability of choroidal capillaries may be an additional mechanism leading to uveal effusion. CONCLUSION: With treatment, the patient had complete resolution of his choroidal effusion with no recurrence of his ISCLS. Further research should be considered on the role of viral infections in the pathogenesis of ISCLS.

Updated Systematic Review and Clinical Spectrum of Peripheral Exudative Hemorrhagic Chorioretinopathy.

Peripheral exudative hemorrhagic chorioretinopathy (PEHCR) is a rare retinal vasculopathy that might cause subretinal and/or vitreous hemorrhages. Although the primary etiology is still unknown, choroidal neovascularization is mainly involved in the pathogenesis. The main risk factors are age and systemic hypertension. Ancillary testing such as fluorescein angiography, indocyanine green angiography and ultrasonography can be of great value for diagnosing this entity and distinguishing PEHCR from other lesions as choroidal melanoma and retinal vasoproliferative tumor. Various treatments have been reported including photocoagulation, cryotherapy, intravitreal injection of anti-vascular endothelial growth factor (Anti-VEGF) and surgical intervention as pars plana vitrectomy. This review handles an up-to-date perspective regarding PEHCR.

Not every pigmented tumour is melanoma: Adenoma of the ciliary body pigment epithelium. / No todo tumor hiperpigmentado es melanoma: adenoma del epitelio pigmentario del cuerpo ciliar.

Differential diagnosis between a melanoma and a pseudomelanoma requires many years of experience and ocular studies, and requires paying attention to the smallest details. The case is presented of a 57 year-old male with an adenoma of the ciliary body pigment epithelium that was treated with an enucleation of the eye due to a suspicion of a choroidal melanoma.

Melanocytoma of the optic disk: A review.

Melanocytoma is a deeply pigmented variant of melanocytic nevus that classically occurs in the optic disk, sometimes with contiguous involvement of the adjacent retina or choroid. Historically, this tumor was often confused with malignant melanoma both clinically and histopathologically. Today, however, it is generally recognized by its typical clinical features that differ from most melanomas and erroneous enucleation is rarely done. Histopathologically, melanocytoma is composed of intensely pigmented round to oval nevus cells with benign features. Although traditionally believed to be a relatively stationary lesion, it is now known to exhibit minor enlargement in 10--15% of cases and can cause minor visual loss by a variety of mechanisms. In rare instance, it can induce severe visual loss due to spontaneous necrosis of the lesion or compressive optic neuropathy. More importantly, it can exhibit malignant transformation into melanoma in 1--2% of cases. Ophthalmologists should be familiar with melanocytoma of the optic disk and affected patients should be followed periodically.

Suprachoroidal Hemorrhage Simulating Melanoma in Idiopathic Thrombocytopenic Purpura.

BACKGROUND: We report a case of suprachoroidal hemorrhage simulating choroidal melanoma in a patient with idiopathic thrombocytopenic purpura (ITP). METHODS: Case report. RESULTS: A 79-year-old white male with a history of lung adenocarcinoma, ITP, and mild anemia noted blurred vision with photopsia and floaters in the right eye (OD) that worsened over 2 months. He was found to have a pigmented choroidal mass and was referred for evaluation of possible choroidal melanoma. Visual acuity was 20/30 in the affected right eye and 20/25 in the left eye (OS). There was mild vitreous hemorrhage and 2 pigmented peripheral choroidal tumors in the temporal and nasal fundus OD. B-scan ultrasonography confirmed 2 acoustically hollow tumors, and transillumination demonstrated no shadowing. Optical coherence tomography over the lesions revealed choroidal elevation with suprachoroidal scalloped surface. Fluorescein and indocyanine green angiography revealed normal choroidal fluorescence and cyanescence with no "double circulation" of melanoma and no dye blockage, suggesting a suprachoroidal location. These findings were suggestive of suprachoroidal hemorrhage rather than melanoma. The patient was managed conservatively, and the hemorrhages demonstrated spontaneous resolution. CONCLUSION: Ocular manifestations of ITP are uncommon. Rarely, spontaneous suprachoroidal hemorrhage simulating melanoma can occur. A thorough clinical evaluation with multimodal imaging is critical to establishing the appropriate diagnosis.

Clinical and dermoscopic (in vivo and ex vivo) predictors of recurrent nevi.

Recurrent nevi occur following removal of melanocytic nevi mainly after shaving excision. Due to its differential diagnosis with melanoma, its recognition can be challenging. To evaluate clinical and dermoscopic predictors of recurrent nevi. Clinical data on 224 melanocytic nevi were collected, together with dermoscopic images. Shave biopsy was performed and the underside of the surgical specimen was examined using ex vivo dermoscopy. A total of 195 lesions were followed for six months. Recurrent nevi were observed in 59 lesions. The occurrence of recurrent nevi was inversely associated with increased patient age. Darker skin phototypes and the compound nevus type were more associated with recurrence. Regarding dermoscopic features, the presence of dark brown colour, multiple colours, dots, terminal hair, and an annular-distributed pigmentation around adnexal structures were associated with recurrent nevi. Ex vivo dermoscopy showed that visible hyperpigmentation on the underside of the surgical specimen was correlated with recurrence. Multivariable analysis showed that annular hyperpigmentation and age were the most relevant predictors of recurrence. Individuals with dark skin type and young adults are at increased risk of recurrent nevi. Dermoscopy of the primary lesion and the underside of the surgical specimen are helpful in predicting recurrence after shave biopsy.

Choroidal hematoma presenting as pseudo-uveal melanoma in a monocular 47-year-old Asian Indian lady with opaque media.

Pseudomelanoma is a condition that mimics a uveal melanoma. Various methods are used to differentiate between uveal melanoma and mimicking conditions, but none is definitive. We report a case of localized hemorrhagic choroidal detachment in monocular middle-aged Asian lady of Indian origin with opaque media secondary to failed corneal graft. An ultrasound scan revealed a mass lesion suspected to be choroidal melanoma. After 10 weeks of observation and follow-up, lesion showed complete regression as seen with serial ultrasound scans. This case is being presented for its rarity and unique presentation in a bilaterally blind patient with spontaneous resolution of pseudomelanoma documented with serial imaging.

The regenerating naevus.

The re-emergence of a melanocytic proliferation at the site of a previously excised pigmented lesion may not only cause great concern clinically but may also be amongst the most difficult of all melanocytic lesions for pathologists to assess. These lesions can adopt an appearance which may be impossible to confidently distinguish from a regressing or traumatised melanoma on histological grounds alone. For this reason, careful attention must be paid to the clinical context which has given rise to the lesion or a misdiagnosis may occur. In the absence of a corroborating history of prior surgery or trauma to the site, a diagnosis of a regenerating naevus may only be provisional. When considering a diagnosis of regenerating naevus, whenever possible, it is important to review and confirm the benign nature of the precursor lesion. Nevertheless, 50 years of research into this phenomenon has identified certain characteristic clinical features and histological patterns which provide clues both to clinicians and pathologists that will assist them to make the correct diagnosis and avoid over diagnosing as melanoma what is ultimately a benign process.

Giant leiomyoma of the ciliary body.

Leiomyoma is a rare intraocular tumor that arises from uveal smooth muscle. Herein, we report a large leiomyoma that occupied nearly 50% of the globe, closely resembling melanoma. A 40-year-old female presented with a 17 × 15 × 11 mm amelanotic ciliochoroidal mass causing visual defect in her right eye (OD). Based on transillumination features of tumor shadow and ultrasonographic features of acoustically solid mass, there was low clinical suspicion for leiomyoma or schwannoma, and a preliminary diagnosis of ciliochoroidal melanoma was rendered. Following enucleation, histopathology revealed a paucicellular tumor comprised of spindle cells, with positive immunostaining for smooth muscle actin and negative stains for melanoma markers (S-100 protein, HMB45, and MITF-2). These features were consistent with ciliochoroidal leiomyoma. Benign uveal leiomyoma can achieve an unusually large size and block light transmission on transillumination, features that simulate malignant melanoma.

Pseudomelanoma at a referral center in iran.

PURPOSE: To report the diagnoses of lesions initially misdiagnosed as ocular melanoma. METHODS: This retrospective study included all new patients who were referred with a presumptive diagnosis of choroidal melanoma to the ocular oncology clinic at Farabi Eye Hospital from January 2009 to December 2012. Each patient underwent a full ocular examination and B-scan ultrasonography by an ocular oncologist. The final diagnosis was made based on a combination of clinical features, fluorescein angiography, indocyanine green angiography, optical coherence tomography, neuroimaging and biopsy when necessary. RESULTS: Out of a total of 194 patients referred with a preliminary diagnosis of choroidal melanoma, 73 (37.6%) subjects actually had pseudomelanoma. Mean age in this subgroup was 46.5±23.1 (range, 1.5-85) years. The most common entities simulating a choroidal melanoma were vasoproliferative tumors (12 cases), choroidal metastasis (11 cases), peripheral exudative hemorrhagic chorioretinopathy (10 cases), lymphoproliferative infiltrative lesions (6 cases) and melanocytoma (5 cases). CONCLUSION: A wide range of lesions may mimic ocular melanoma; a correct diagnosis may be made by a combination of clinical examination and imaging modalities.