Coats disease associated with vasoproliferative tumor treated with cryotherapy and intravitreal injections of triamcinolone / Doença de Coats associada a tumor vasoproliferativo tratada com crioterapia e injeções intravítreas de triancinolona

Abstract Report of a case of Coats disease associated with retinal vasoproliferative tumor in a young female patient with two peripheral vascularized tumors and lipid exudation involving the macula and peripapillary region with serous retinal detachment areas and pre-papillary fibrous proliferation. The proposed and performed treatment was the intravitreal injection of triamcinolone acetonide to decrease the tumor exudation, followed by photocoagulation of the peripheral areas of telangiectasia without subretinal fluid and cryotherapy of the tumors. Despite that this is a rare and difficult to treat combination, in this case report, success was obtained in receding the tumor masses and reapplying the retina, leading to anatomic and visual stabilization.

Resumo Relato de um caso de Doença de Coats associada a tumor vasoproliferativo de retina em uma paciente jovem com duas tumorações vascularizadas periféricas e exsudação lipídica acometendo mácula e região peripapilar com áreas de descolamento de retina seroso e proliferação fibrosa pré-papilar. O tratamento proposto e realizado foi a injeção intra-vítrea de triancinolona para diminuir a exsudação do tumor, seguida de fotocoagulação periférica das áreas de telangiectasia sem fluido subretiniano e criocoagulação dos tumores. Apesar de se tratar de uma associação rara e de difícil tratamento, neste relato de caso, obteve-se êxito em regredir as massas tumorais e reaplicar a retina, levando à estabilização anatômica e visual.

Fluorescein Angiographic Abnormalities in the Contralateral Eye with Normal Fundus in Children with Unilateral Coats' Disease

PURPOSE: We report the detection of peripheral retinal vascular abnormalities in the fellow eye with normal fundus in children with unilateral Coats' disease. METHODS: The clinical records of patients diagnosed with Coats' disease were retrospectively reviewed. We recorded the subjects' characteristics and obtained fundus photography and fluorescein angiography (FA) images. The main outcome measure was peripheral vascular abnormalities in the contralateral eye with normal fundus in children with unilateral Coats' disease, observed with FA. RESULTS: Out of 47 patients with Coats' disease, two (4.3%) were diagnosed with clinically bilateral Coats' disease. Of the 45 patients with presumed unilateral Coats' disease, four (8.9%) had bilateral abnormal peripheral vasculature in FA. The mean age of these four patients was 6.4 ± 5.4 years (range, 1 to 14 years), and three patients were male (75%). All four had peripheral retinal nonperfusion, and two (50%) received laser photocoagulation due to peripheral leakage with telangiectatic vessels. CONCLUSIONS: Coats' disease may more often be a bilateral disease with asymmetry than previously thought. Patients with Coats' disease should undergo careful examination of the fellow eye with FA in order to detect and treat vascular abnormalities that are not visible clinically.

Pars Plana Vitrectomy for Vitreous Hemorrhage in Coats-Type Retinitis Pigmentosa

PURPOSE: For vitreous hemorrhage induced by coats-types retinitis pigmentosa, we report a case treated with pars plana vitrectomy and endolaser photocoagulation. CASE SUMMARY: A 24-year-old male who was diagnosed with retinitis pigmentosa in both eyes 6 years earlier presented with decreased visual acuity in his left eye for the last 7 months. Corrected visual acuity was measured at 0.06 in the left eye and fundus examination revealed a vitreous hemorrhage in the left eye as well as an exudative lesion in the right eye's peripheral retina, which suggested Coats-type retinitis pigmentosa. The left eye was treated with pars plana vitrectomy. After removal of the vitreous hemorrhage, endolaser photocoagulation was performed around the peripheral exudative lesion that caused the vitreous hemorrhage. One month later, the best-corrected visual acuity increased to 0.20 in the left eye, and there was an improvement in the vitreous hemorrhage and the exudative lesion. CONCLUSIONS: Pars plana vitrectomy and endolaser can be helpful in vitreous hemorrhage induced by coats-type retinitis pigmentosa.

A Case of Congenital Simple Hamartoma of the Retinal Pigment Epithelium and Coats' Disease in the Same Eye

No abstract available.

Demographic Features of Idiopathic Macular Telangiectasia in Korean Patients

PURPOSE: To investigate the clinical and demographic features of idiopathic macular telangiectasia (MacTel) in Korean patients since the introduction of spectral domain optical coherence tomography (SD-OCT). METHODS: We reviewed medical records of patients who were diagnosed with MacTel from 2009 to 2013. All patients underwent fluorescein angiography and SD-OCT and were classified as type 1 or type 2 according to the classification system proposed by Yannuzzi. RESULTS: Over a period of 5 years, 4 (18.2%) patients were diagnosed with type 1 MacTel and 18 (81.8%) patients were diagnosed with type 2 MacTel. All patients with type1 MacTel were male, and their mean age was 51 +/- 8.6 years. Among patients with type 2 MacTel, 3 (16.7%) were male, 15 (83.3%) were female, and the mean age was 60 +/- 13.6 years. Whereas all type 1 MacTel patients had either metamorphopsia or mild scotoma, of the 18 patients with type 2 MacTel, only 4 (22.2%) had those symptoms, 10 (55.6%) complained of only mild visual impairment, and the other 4 (22.2%) had no symptoms. Intraretinal cystoid spaces were observed in 26 (72.2%) of 36 eyes with type 2 MacTel by SD-OCT. These cystoid spaces had irregular boundaries and did not correspond to angiographic leakages. CONCLUSIONS: Type 2 MacTel was most common in the present study. The wider availability of SD-OCT may have contributed to the diagnosis of type 2 MacTel. Type 2 MacTel may be more prevalent than type 1 in Koreans, which corresponds to the results of Western countries.

Demographic Features of Idiopathic Macular Telangiectasia in Korean Patients

PURPOSE: To investigate the clinical and demographic features of idiopathic macular telangiectasia (MacTel) in Korean patients since the introduction of spectral domain optical coherence tomography (SD-OCT). METHODS: We reviewed medical records of patients who were diagnosed with MacTel from 2009 to 2013. All patients underwent fluorescein angiography and SD-OCT and were classified as type 1 or type 2 according to the classification system proposed by Yannuzzi. RESULTS: Over a period of 5 years, 4 (18.2%) patients were diagnosed with type 1 MacTel and 18 (81.8%) patients were diagnosed with type 2 MacTel. All patients with type1 MacTel were male, and their mean age was 51 +/- 8.6 years. Among patients with type 2 MacTel, 3 (16.7%) were male, 15 (83.3%) were female, and the mean age was 60 +/- 13.6 years. Whereas all type 1 MacTel patients had either metamorphopsia or mild scotoma, of the 18 patients with type 2 MacTel, only 4 (22.2%) had those symptoms, 10 (55.6%) complained of only mild visual impairment, and the other 4 (22.2%) had no symptoms. Intraretinal cystoid spaces were observed in 26 (72.2%) of 36 eyes with type 2 MacTel by SD-OCT. These cystoid spaces had irregular boundaries and did not correspond to angiographic leakages. CONCLUSIONS: Type 2 MacTel was most common in the present study. The wider availability of SD-OCT may have contributed to the diagnosis of type 2 MacTel. Type 2 MacTel may be more prevalent than type 1 in Koreans, which corresponds to the results of Western countries.

Epiretinal membrane formation associated with idiopathic macular telangiectasia: case report / Formação de membrana epirretiniana associada a telangectasia macular idiopática: relato de caso

A 46-year-old woman complained of blurred and distorted vision in both eyes. Ophthalmic examination showed that visual acuity was 20/200 for the right eye and counting fingers left eye. Fundoscopy revealed perimacular hemorrhages, aneurismal dilatation of the vessels in the posterior pole, and a white and elevated lesion adjacent to vascular changes. We report a case of idiopathic macular telangiectasia and epiretinal membrane that occurs concomitantly. To our knowledge, this is the first report that describes an association between idiopathic macular telangiectasia and epiretinal membrane formation.

Paciente feminina de 46 anos apresentando queixa de embaçamento visual e visão distorcida em ambos os olhos. Ao exame oftalmológico, sua acuidade visual era 20/200 no olho direito e conta dedos a 5 metros no olho esquerdo. A fundoscopia revelou hemorragias perimaculares, dilatação aneurismática dos vasos no polo posterior e uma lesão elevada e esbranquiçada ao lado das alterações vasculares. Relatamos um caso de telangectasia macular idiopática e membrana epirretiniana que ocorreram concomitantemente. Até o momento, não existem relatos de associação entre telangiectasia macular e membrana epirretiniana.

Vitreorretinopatia exsudativa familiar simulando doença de Coats: relato de caso / Familial exudative vitreoretinopathy simulating Coats disease: case report

O objetivo é relatar o caso de um paciente de sete anos, nascido a termo, sem intercorrências perinatais, encaminhado ao Setor de Retina/Vítreo para elucidação diagnóstica. Apresentava história de redução da acuidade visual à esquerda, de caráter insidioso/progressivo, há quatro anos. Ao exame, apresentava diminuição do diâmetro corneano e corectopia do olho direito (OD), sem alterações à biomicroscopia do olho esquerdo (OE). A fundoscopia do OD revelava descolamento de retina (DR) total e, do OE, inicialmente, mostrava alterações vasculares retinianas periféricas e exsudação retiniana, associado à tração vitreorretiniana no setor temporal. As tomografias e ressonâncias de crânio/órbitas não apresentavam anormalidades, com exceção de achados sugestivos de DR antigo no OD, confirmado pela ultrassonografia do globo ocular, que também demonstrou microftalmia. Diante disso, aventou-se a hipótese diagnóstica de vitreorretinopatia exsudativa familiar, doença rara de caráter autossômico dominante e relacionada com casamentos consanguíneos, inicialmente simulando doença de Coats. O paciente foi tratado com fotocoagulação a laser diodo na periferia temporal do OE, com melhora das áreas de tração vitreorretiniana.

We report the case of a seven year-old male patient, born at term without any perinatal complications, referred to the Retina/Vitreous Service for diagnostic elucidation. He had a history of progressive visual acuity loss on his left eye that started four years ago. On examination, he had decreased corneal diameter and corectopia of the right eye (OD), without any noteworthy findings on the biomicroscopy of the left eye (OS). The fundus of the OD revealed total retinal detachment, and the OS initially showed peripheral retinal vascular abnormalities and retinal exudation, associated with retinal vitreous traction on the temporal sector. The CT and MRI of the brain/orbits showed no abnormalities, except for findings suggestive of an old retinal detachment on the OD, confirmed by ultrasonography, which also showed microphthalmia of the OD. The diagnosis of familial exudative vitreoretinopathy, a rare disease of autosomal dominant inheritance and related to consanguineous marriages, that can initially simulate Coats disease, was proposed. The patient was treated with diode laser photocoagulation in the temporal periphery of the OS, with improvement in the areas of vitreoretinal traction.

Intravitreal bevacizumab for pediatric exudative retinal diseases

To detect if intravitreal bevacizumab can reduce retinal exudation, improve visual and anatomical outcomes, and facilitate the treatment in various pediatric exudative retinal diseases. Prospective, non-randomized, case series of nine eyes of pediatric exudative retinal diseases less than 18years old which included six eyes with juvenile diabetic retinopathy, two eyes in children with Coats' disease, and one eye with myopic choroidal neovascular membrane [CNV]. All eyes received only intravitreal bevacizumab injection 1.25mg/0.05ml as the primary treatment. The need for adjuvant ablative procedures, including laser photocoagulation or cryotherapy, were performed and recorded. The need for supplementary intravitreal bevacizumab injection was recorded. The changes in pre-and post-operative best-corrected visual acuity [BCVA] and central macular thickness [CMT] were recorded. Serial optical coherent tomography [OCT] and fundus flourescein angiography [FFA] were performed to follow treatment efficacy. The study included 19 eyes of 11 patients with age equal to or less than eighteen years with exudative retinal diseases including type I DM [n=sixteen eyes], Coats' disease [n=2 eyes], and due to myopic CNV [n=1 eye]. Mean pre-injection log MAR for all was 0.605 +/- 0.174 and mean post-injection for all log MAR was 0.284 +/- 0.247. While mean pre-injection log MAR for DR and myopic CNV patients was 0.576+0.152 SD and mean post-injection log MAR for DR and myopic CNV patients was 0.229+0.189 at one year. Serial OCT measurements showed that mean CMT for all eyes was 355.8 +/- 35.3 micro m SD at baseline, which was decreased to 222.42+26.2 micro m SD. The two eyes of Coats' disease needed another two supplementary intravitreal bevacizumab injections. No ocular or systemic complications related to bevacizumab were noted during the entire course of follow-up. Intravitreal bevacizumab appears to be a well-tolerated treatment for pediatric age group with various exudative retinal diseases. It has the potential as an adjuvant therapy for ablative procedures to improve final visual and anatomical outcome

A review of pseudoretinoblastoma cases at a tertiary hospital

Objective@#To report various intraocular conditions that mimic retinoblastoma.@*Methods@#A review was conducted of eyeballs enucleated for suspected retinoblastoma between 2003 and 2007, and referred for histopathological confirmation. The slides of cases not histopathologically consistent with the diagnosis of retinoblastoma were reexamined. Clinical records and results of neuroimaging studies were reviewed retrospectively@*Results@#Of the 197 eyeballs examined, 182 (92%) proved to be retinoblastoma on histological exam, while 15 (8%) from 13 patients were pseudoretinoblastomas. The age of patients ranged from 4 months to 9 years, with a mean of 35.5 months. The etiologies of the pseudoretinoblastomas were as follows: persistent primary hyperplastic vitreous (PHPV) in 5 eyeballs (33%); retinal dysplasia in 3 (20%); Coats’disease, phthisis bulbi, and vitreous hemorrhage with retinal detachment in 2 (13%) each; and granulomatous endophthalmitis in 1 (8%).@*Conclusion@#The 8% erroneous diagnosis was lower than the published rates of 10 to 20%. The common etiologies of pseudoretinoblastoma were similar to those reported.

Combined Photodynamic Therapy and Intravitreal Bevacizumab Injection for the Treatment of Adult Coats' Disease: A Case Report

A 68-year-old woman presented with a visual field defect in her right eye. The fundus of her right eye showed multiple telangiectatic vessels, retinal hemorrhages, and subretinal exudates in the inferior peripheral retina. Nine months later, the subretinal exudates extended to the fovea despite treatment with laser photocoagulation. Cryotherapy was not possible at the time because of the posterior location of the retinal telangiectatic vessels. She was treated with a combination of photodynamic therapy (PDT) and intravitreal bevacizumab injection: three injections were given at 2-month intervals. After this combined therapy, her right fundus revealed a significant regression of abnormal retinal vessels and subretinal exudates. A fluorescein angiography showed no leakage from the abnormal retinal vessels. At 9 months after the combined therapy, she was able to maintain a stable visual acuity and visual field. This is the first case report that demonstrates the efficacy of the combined treatment of PDT and intravitreal bevacizumab injection in Coats's disease. This combined therapy is a kind of treatment modality for adult Coats' disease in cases which cryotherapy cannot be employed and are refractory to laser photocoagulation.