Terapia fotodinâmica com verteporfina associada à injeção intravítrea de antifator de crescimento endotelial vascular para tratamento de hemangioma circunscrito de coroide / Photodynamic therapy with verteporfin associated with intravitreal injection of vascular endothelial growth factor for the treatment of circumscribed choroidal hemangioma

RESUMO O hemangioma de coroide é um tumor vascular benigno, de coloração vermelho-alaranjada, bem delimitado, caracterizado por uma placa elevada. É um tumor raro, com prevalência de um caso a cada 40 tumores de coroide. O diagnóstico pode ser feito por meio da clínica associada à avaliação biomicroscópica e a exames complementares para diferenciação de outros tumores. O tratamento pode ser expectante nos casos assintomáticos. Para os casos sintomáticos ou com presença de fluido sub-retiniano, existem diversas terapias. O objetivo deste estudo foi relatar um caso de hemangioma circunscrito de coroide submetido a tratamento combinado de terapia fotodinâmica com verteporfina e injeção intravítrea de antiangiogênico (bevacizumabe). A decisão de tratar um hemangioma de coroide deve ser individualizada com base nos sintomas, na perda visual e em qualquer potencial de sua recuperação. O exame oftalmológico completo é necessário, mesmo em casos assintomáticos, para rastreamento precoce de doenças oculares.

ABSTRACT Choroid hemangioma is a benign, well-delimited orange-red, vascular tumor characterized by an elevated plaque. It is a rare tumor with a prevalence of one case in every 40 choroidal tumors. It can be diagnosed by the clinic associated with biomicroscopic evaluation and complementary tests to differentiate from other tumors. Treatment can be expectant in asymptomatic cases. For symptomatic cases or those with the presence of subretinal fluid, there are several therapies. The objective of this study was to report a case of circumscribed choroidal hemangioma submitted to combined treatment of photodynamic therapy with verteporfin and intravitreal injection of an antiangiogenic agent (bevacizumab). The decision to treat choroidal hemangioma must be individualized based on symptoms, visual loss, and any potential for recovery. A complete eye examination is necessary, even in asymptomatic cases, for early screening for eye diseases.

Hemangioma de coroide e o desafio do diagnóstico diferencial / Choroidal hemangioma and the challenge of differential diagnosis

Resumo O hemangioma de coroide é um tumor benigno relativamente raro, que se apresenta de forma circunscrita ou difusa, sendo esta última normalmente associada à Síndrome de Sturge-Weber. Os tumores circunscritos manifestam-se de forma insidiosa, com o diagnóstico realizado comumente após o aparecimento de sintomas secundários. Apresentam como diagnóstico diferencial lesões graves e potencialmente letais, como melanoma de coroide e doença metastática. Neste relato descrevemos o caso de um hemangioma intraocular nodular avançado associado a descolamento hemorrágico da retina, evidenciando o desafio do diagnóstico diferencial devido às semelhanças clínicas e radiológicas compartilhadas pelos tumores.

Abstract Choroidal hemangioma is a fairly rare benign vascular tumor that can manifest in either circumscribed or diffuse type; the latter one is usually related to Sturge-Weber Syndrome. The circumscribed tumors have an insidious presentation and diagnosis is commonly made after the onset of secondary symptoms. Serious and potentially lethal lesions, such as choroidal melanoma and metastatic disease, may represent a differential diagnosis. In this report, we describe an advanced case of nodular hemangioma associated with hemorrhagic retinal detachment. This case highlights the challenge of differential diagnosis in intraocular tumors, due to their similar clinical and radiologic features.

Baixa visão secundária a osteoma de coroide / Reduced vision caused by choroid osteoma

RESUMO Relatamos um caso de osteoma de coroide em uma paciente feminina de 25 anos. Apresentava uma lesão amarelada e elevada no polo posterior, característica da lesão tumoral. O diagnóstico foi confirmado com a ultrassonografia ocular. A paciente apresentava baixa acuidade visual e edema de mácula, que melhorou após injeção intravítrea de bevacizumab.

ABSTRACT The authors present a case of choroidal osteoma diagnosed in a 25-year-old female patient. A well-defined and slightly elevated yellow lesion located in the posterior pole of the right eye was suspected to be a tumor. Confirmation of diagnosis was obtained with A and B ecography. The patient had low vision and macular edema, which improved after intraocular injection of bevacizumab .

Idiopathic polypoidal choroidal vasculopathy masquerading as choroidal tumors: one year follow-up of a peripheral lesion / Vasculopatia polipoidal idiopática da coroide simulando tumores da coroide: um ano de seguimento de uma lesão periférica

ABSTRACT This case report describes peripheral idiopathic polypoidal choroidal vasculopathy (IPCV) with a collection of small aneurysmal dilations that masqueraded as choroidal tumors in an elderly patient. A 68-year-old African American woman was referred to us with a suspected diagnosis of asymptomatic vascular choroidal tumor and choroidal capillary hemangioma, affecting the temporal peripheral fundus. Upon examination, optical coherence tomography (OCT) revealed two large hemorrhagic pigment epithelium detachments (PED), and indocyanine green angiography (ICG) confirmed the diagnosis of IPCV. One year later, there was reduction in the hemorrhagic pigment epithelium detachments and the lesion took on a different appearance, resembling a choroidal osteoma. No treatment was necessary despite the presence of multiple polyps. IPCV is a rare condition that can resemble other choroidal diseases depending on the stage of presentation. OCT is the best tool to determine the characteristics of the lesions, and indocyanine green angiography should be used to confirm the diagnosis. Not all cases require treatment.

RESUMO Relato de um caso de vasculopatia polipoidal idiopática da coroide (IPCV) com múltiplas dilatações aneurismáticas em região temporal periférica da retina, em uma paciente idosa que assemelhou-se com alguns tumores de coroide no seguimento de um ano. Paciente de 68 anos da raça negra, assintomática, foi encaminhada com a hipótese diagnóstica de um tumor vascular de coroide e hemangioma capilar da coroide, em região temporal inferior periférica da retina. Ao exame de tomografia de coerência óptica (OCT) era observado dois grande descolamentos de epitélio pigmentado (DEP), sendo confirmado o diagnóstico de vasculopatia polipoidal idiopática da coroide pela angiografia com indocianina verde (ICG). Após um ano, houve absorção do descolamento do epitélio pigmentado hemorrágico assemelhando-se assim ao osteoma de coroide. Nenhum tratamento foi necessário apesar da quantidade dos pólipos. A vasculopatia polipoidal idiopática da coroide é uma doença rara que, dependendo do estágio da apresentação, pode se assemelhar com algumas doenças da coroide. A tomografia de coerência óptica pode ilustrar melhor as características das lesões e a ICG confirma o diagnóstico. Nem todos os casos necessitam ser tratados.

Enhanced depth imaging optical coherence tomography and fundus autofluorescence findings in bilateral choroidal osteoma: a case report / Tomografia de coerência óptica com profundidade de imagem aprimorada e resultados autofluorescência de fundo em osteoma de coroide bilateral: relato de caso

The authors present enhanced depth imaging optical coherence tomography (EDI OCT) and fundus autofluorescence (FAF) characteristics of a patient with bilateral choroidal osteoma and try to make a correlation between two imaging techniques. Two eyes of a patient with choroidal osteoma underwent complete ophthalmic examination. Enhanced depth imaging optical coherence tomography revealed a cage-like pattern, which corresponded to the calcified region of the tumor. Fundus autofluorescence imaging of the same area showed slight hyperautofluorescence. Three different reflectivity patterns in the decalcified area were defined. In the areas of subretinal fluid, outer segment elongations similar to central serous chorioretinopathy were observed. Hyperautofluorescent spots were evident in fundus autofluorescence in the same area. Calcified and decalcified portions of choroidal osteoma as well as the atrophy of choriocapillaris demonstrated different patterns with enhanced depth imaging and fundus autofluorescence imaging. Both techniques were found to be beneficial in the diagnosis and follow-up of choroidal osteoma.

Os autores apresentam tomografia de coerência óptica com profundidade de imagem aprimorada (EDI OCT) e autofluorescência de fundo (FAF) características de um paciente com osteoma de coroide bilateral e tentam correlacionar as duas técnicas de imagem. Dois olhos de um paciente com osteoma de coroide foram submetidos a exame oftalmológico completo. Tomografia de coerência óptica com profundidade de imagem aprimorada revelou padrão em gaiola, correspondente à região de calcificação do tumor. Imagens de autofluorescência de fundo da mesma área mostraram ligeira autofluorescência positiva. Três padrões de refletividade diferentes foram definidos na área descalcificada. Nas áreas com fluido sub-retiniano, foram observados prolongamentos dos segmentos externos semelhantes aos da coroidorretinopatia serosa central. Manchas autofluorescentes positivas foram evidentes em autofluorescência de fundo na mesma área. Porções calcificadas e descalcificadas do osteoma de coroide, bem como a atrofia da camada coriocapilar, demonstraram diferentes padrões de tomografia de coerência óptica com profundidade de imagem aprimorada e de imagens de autofluorescência de fundo. Ambas as técnicas se mostraram úteis no diagnóstico e acompanhamento de osteoma de coroide.

Metástasis coroidea de cáncer pulmonar: reporte de un caso / Choroidal metastasis from lung cancer: case report

Introducción: El compromiso ocular en el cáncer sistémico es diverso y ampliamente conocido en la literatura internacional, pero en la nacional hay carencia de información al respecto. La coroides es la estructura ocular donde con mayor frecuencia se encuentran metástasis tumorales dentro del ojo, siendo el cáncer de pulmón el más comúnmente diagnosticado en pacientes que debutan con metástasis coroidea. En esta localización, los síntomas más frecuentes son la visión borrosa y dolor ocular, siendo la presentación asintomática infrecuente. Este tipo de lesiones se produce en la fase final de la enfermedad, donde la supervivencia no supera los 6 meses. Presentación del caso: Paciente portador de cáncer pulmonar, que debutó con metástasis coroidea, siendo la primera sintomatología compatible con desprendimiento de retina. Las claves para el diagnóstico fueron la ultrasonografía ocular, resonancia nuclear magnética, y la biopsia realizada tras la enucleación del ojo afectado que es informada como adenocarcinoma tubular y papilar. De acuerdo a este resultado, se estudia con tomografía computada (TAC) de tórax, abdomen y pelvis, cintigrama óseo, revelándose la imagen de un nódulo pulmonar sospechoso y metástasis pulmonares y extrapulmonares. Tras 8 meses desde el hallazgo de la metástasis coroidea, y 6 meses desde el diagnóstico de cáncer pulmonar, el paciente fallece. Finalmente, se revisó la bibliografía pertinente acerca de la clínica, aproximación diagnóstica, imágenes y consideraciones sobre el tratamiento de las metástasis coroideas. Discusión: Es importante conocer la aproximación diagnóstica de las metástasis coroideas de tumores primarios para tener un alto índice de sospecha.

Introduction: Ocular involvement in systemic cancer is diverse and widely known in the international literature, but in our country there is lack of information. The choroid is the structure of the eye where they most often metastatic tumor within the eye are found, being lung cancer the most commonly diagnosed in patients presenting with choroidal metastasis. At this location, the most common symptoms are blurred vision and eye pain. Asymptomatic presentation is uncommon. This type of injury occurs in the final stage of the disease, where survival does not exceed 6 months. Case report: Patient with lung cancer, which debuted with choroidal metastasis, whose first symptoms consistent in retinal detachment. The keys to diagnosis were ocular ultrasonography, magnetic resonance imaging, and biopsy performed after enucleation of the affected eye, which was reported as tubular and papillary adenocarcinoma. According to this result, he was studied with chest, abdomen and pelvis CT, bone scintigraphy, revealing the image of a suspicious lung nodule and pulmonary and extrapulmonary metastases. After 8months since the finding of choroidal metastases, and 6 months after diagnosis of lung cancer, the patient dies. Reviewed the relevant literature on the clinical diagnostic approach, images and considerations on the treatment of choroidal metastases. Discussion: It is important to know the approach to diagnosis of choroidal metastases from primary tumors to have a high index of suspicion.

Cutaneous metastasis in choroidal melanoma / Metástases cutâneas em melanoma de coróide

Metastasis in choroidal melanoma is frequent on advanced diseases, involving mainly the liver, lungs and central nervous system. We report a case of cutaneous metastasis in choroidal melanoma because is an unusual condition, even in advanced disease.

A ocorrência de metástases do melanoma de coróide é frequente na doença avançada, acometendo principalmente fígado, pulmões e sistema nervoso central. Relatamos um caso de melanoma de coróide com metástases cutâneas, por se tratar de acometimento raro, mesmo em casos avançados da doença.

Combination therapy of low-fluence photodynamic therapy and intravitreal ranibizumab for choroidal neovascular membrane in choroidal osteoma.

Choroidal osteoma is an unusual form of intraocular calcification seen in otherwise healthy eyes. It is a benign idiopathic osseous tumor of the choroid, typically seen in young females. Choroidal neovascular membrane (CNVM) is a complication seen in one-third of these patients and carries a poor visual outcome. We report a case of a 25-year-old hyperthyroid female with choroidal osteoma and subfoveal CNVM in her left eye which was successfully treated using low-fluence photodynamic therapy (PDT) with verteporfin followed by a single injection of intravitreal ranibizumab.

Intravitreal bevacizumab (avastin) for circumscribed choroidal hemangioma.

Circumscribed choroidal hemangiomas are rare ophthalmic entities that cause diminution in vision due to accumulation of subretinal and/or intraretinal fluid in the macular area. Various treatment options ranging from conventional laser to photodynamic therapy have been employed to destroy the tumor and reduce the exudation; however, either the inability to penetrate through the exudative fluid or the collateral retinal damage induced by these treatment modalities make them unsuitable for lesions within the macula. We evaluated the role of intravitreal bevacizumab, a pan-vascular endothelial growth factor (VEGF) inhibitor, in reducing the sub- and intraretinal fluid in three patients with circumscribed choroidal hemangiomas. All the patients had complete resolution of the serous retinal detachment that was maintained till at least 12 months after the first injection. Intravitreal bevacizumab may be used in combination with thermal laser or photodynamic therapy in treating circumscribed choroidal hemangiomas with subretinal fluid.

Subretinal lipid exudation associated with untreated choroidal melanoma.

Subretinal lipid exudation in an untreated choroidal melanoma is very rare. It is seen following plaque radiotherapy in choroidal melanoma. There is only one case report of untreated choroidal melanoma with massive lipid exudation in a patient with metastatic hypernephroma. We report here a rare case of untreated choroidal melanoma with lipid exudation. Subretinal exudation that is rarely seen following plaque brachytherapy was noted at the borders of this untreated tumor. Lipid exudation partially resolved following brachytherapy.

Melanoma de corpo ciliar e coróide: relato de caso / Choroidal and ciliary body melanoma: case report

Melanomas oculares correspondem a 5 por cento de todos os melanomas e 85 por cento deles têm origem no trato uveal. Melanoma uveal é o tumor maligno intraocular primário mais comum no adulto. Relatamos neste artigo um caso de melanoma uveal em paciente, sexo feminino, 31 anos, com quadro de fotopsia, hiperemia e baixa da acuidade visual no olho esquerdo com evolução de quatro meses. Apresentava ao exame oftalmológico acuidade visual menor que 20/400, grande massa tumoral na região nasal retroiriana, com deslocamento anterior do cristalino, estreitamento da câmara anterior e descolamento seroso da retina. A ecografia sugeriu tratar-se de grande massa tumoral suspeita de melanoma de coróide com invasão do corpo ciliar. A confirmação diagnóstica foi possível por meio do exame anatomopatológico.

Ocular melanomas correspond to 5 percent of all melanomas and 85 percent of them have its origin in the uveal tract. Uveal melanoma is the most commom primary intraocular malignant tumor in the adult. In this article, a case of uveal melanoma in a 31 year-old female patient, with photopsia, hyperemia and low visual acuity in the left eye with evolution of 4 months is presented. In the ophthalmologic examination, visual acuity was lower than 20/400, a large tumoral mass was noted at the nasal region behind the iris with anterior lens displacement, anterior chamber narrowing and serous retinal detachment. The ocular echography suggested a large tumoral mass as a choroidal melanoma extending to the ciliary body. The confirmation diagnosis was possible through the histopathologic examination.

Small choroidal melanoma with monosomy 3

To report a patient with small juxtapapillary choroidal melanoma with chromosome 3 monosomy treated with I[125] plaque and transpupillary thermotherapy [TTT]. A 64-year-old Caucasian male presented with painless blurred vision of the left eye. Ocular examination disclosed a small juxtapapillary choroidal melanocytic tumor with overlying subretinal fluid and orange pigment. Ultrasound showed an elevated choroidal mass of 2 mm thickness with low reflectivity on A-scan and hollowness on B scan, consistent with a small choroidal melanoma. The patient was treated with plaque I[125] radiotherapy combined with one session of TTT. Genetic testing of the tumor cells obtained by fine needle aspiration biopsy showed chromosome 3 monosomy. At 1 year after treatment, the tumor was regressed with resolution of subretinal fluid and 20/40 visual acuity. A small choroidal melanoma can manifest monosomy of chromosome 3, a known predictive factor for the development of systemic metastasis

Uveal melanoma presenting as cataract and staphyloma.

Blind eyes can harbor a choroidal melanoma. We report a case of uveal melanoma presenting as staphyloma and complicated cataract in a 45-year-old female. The left eye was blind for six months. She underwent comprehensive ocular examination but fundus examination was precluded due to total cataract. The ultrasound of the eye showed a large mass filling the superior, nasal and inferonasal vitreous cavity with high surface reflectivity and low to moderate internal reflectivity. Magnetic resonance imaging (MRI) confirmed the diagnosis of choroidal melanoma. The patient underwent extended enucleation and histopathology was consistent with uveal melanoma.

Application of Intravitreal Bevacizumab for Circumscribed Choroidal Hemangioma

We report 3 cases of circumscribed choroidal hemangioma (CCH) effectively managed with intravitreal bevacizumab. One patient (case 1) who had recurrent CCH (1.6 mm in thickness) with prior laser photocoagulation was treated with intravitreal bevacizumab alone. Two patients (case 2 and 3) who had CCH (2.4 mm and 2.2 mm in thickness, respectively) with recent visual impairment were treated with bevacizumab followed by photodynamic therapy (PDT). Ophthalmic evaluations included visual acuity, ophthalmoscopic examination, fluorescein angiography, ultrasonography, and optical coherence tomography. Patients were followed up for 6-9 months. After therapy, all patients showed improved visual acuity due to complete resorption of subretinal fluid and macular edema. Ultrasonography demonstrated a reduction of the thickness of CCH in case 1 and complete regression of the lesions in case 2 and 3. No patient showed tumor recurrence. Intravitreal bevacizumab, alone or in combination therapy with PDT, may be a useful alternative for the treatment of symptomatic CCH with subretinal fluid.

A Choroidal Schwannoma Confirmed by Surgical Excision

Schwannomas rarely present as intraocular tumors and are often misdiagnosed as malignant melanoma. We describe a choroidal schwannoma confirmed by sclerouvectomy. A 30-year-old woman presented with a large nonpigmented intraocular mass of the choroid in the right eye and underwent surgical excision by sclerouvectomy. Histologically, the tumor was composed of a mixture of cellular solid components (Antoni A) and loose myxoid components (Antoni B). The tumor was eventually diagnosed as a schwannoma. Currently available ancillary studies are still of little value in definitively differentiating schwannomas from other choroidal tumors. In the case of atypical findings for a malignant melanoma, a benign neoplasm should be included in the differential diagnosis. This patient avoided enucleation by first having the mass excised. We are unaware of previous reports in which a choroidal schwannoma was diagnosed by surgical excision.

Metástase de coróide simulando síndrome de efusão uveal: relato de caso / Choroidal metastasis simulating uveal effusion syndrome: case report

Descrição de um caso de efusão uveal secundária a metástase de coróide e revisão dos principais diagnósticos diferenciais. Uma paciente com baixa da acuidade visual, no olho esquerdo, apresentou nódulos ao nível da coróide, dispersão pigmentar retiniana e descolamento seroso da retina. O exame angiofluoresceinográfico demonstrou padrão "leopard spots", semelhante ao encontrado nos casos de efusão uveal. A ecografia ocular demonstrou a presença do descolamento seroso da retina, presença de espessamento e nódulos na coróide e a tomografia de coerência óptica evidenciou ruptura do epitélio pigmentado da retina, presença de nodulações coroidianas hiperrefletivas e áreas de descolamento de retina seroso. Após ser submetido a exames diagnósticos de imagem, constatou-se a presença de nódulo único no ápice pulmonar esquerdo. As metástases de coróide podem levar a um quadro similar à síndrome de efusão uveal. Esta possibilidade deve sempre ser pensada diante de um quadro de efusão uveal atípica e avaliação sistêmica para pesquisa de carcinoma deverá ser solicitada.

We report a case of choroidal metastasis simulating uveal effusion syndrome. This patient with uveal metastasis from lung carcinoma presented with decreased vision in the left eye. Fundoscopic examination revealed pigmentary clumping, multiple choroidal nodules and serous retinal detachment. Fluorescein angioraphy revealed a leopard spots pattern similar to uveal effusion syndrome. Ultrasound revealed the presence of choroidal nodules with moderate internal reflectivity, choroidal thickening and serous retinal detachment. Optical coherence tomography showed retinal pigment epithelium tear and hyper-reflective nodules within the choroid and areas of serous retinal detachment. Image investigation demonstrated the presence of a solitary pulmonary nodule at the left apical lung. Choroidal metastasis can lead to a scenario that simulates uveal effusion syndrome. This possibility must be considered when having an atypical uveal effusion symptomatoloy and a systemic screening for systemic cancer should be performed.

Bilateral choroidal metastasis from carcinoma of the submandibular gland.

Metastatic tumor is the most common uveal malignancy. However, choroidal metastasis from a salivary gland neoplasm is extremely rare. We report a case of bilateral, multifocal choroidal metastasis from carcinoma of the submandibular gland.