Bilateral acute depigmentation of the iris: a case report / Despigmentação aguda bilateral da íris: um relato de caso

ABSTRACT Bilateral acute depigmentation of the iris (BADI) is a new clinical entity of unknown etiology and is characterized by bilateral, symmetrical, and simultaneous depigmentation of the iris with focal or diffuse stromal atrophy; this condition generally has a good prognosis. We present a case of a 26-year-old woman who noted a spontaneous change in the iris color in both eyes in the last 2 months. The ophthalmological findings were atrophy of the iris stroma and pigmentation of the trabecular meshwork, without affecting the pigmented epithelium of the iris. Her intraocular pressure was normal and the visual acuity was 20/20 in both eyes.

RESUMO A despigmentação aguda bilateral da íris (DABI) é uma nova entidade clínica caracterizada pela despigmentação bilateral, simétrica e simultânea da íris, com atrofia focal ou difusa do seu estroma, geralmente com bom prognóstico. Apresentamos o caso de uma mulher de 26 anos de idade que procurou atendimento médico em nosso serviço com queixa de mudança espontânea na cor da íris de ambos os olhos nos últimos dois meses. Os achados oftalmológicos observados durante o exame clínico foram atrofia do estroma da íris e pigmentação da malha trabecular, sem afetar o epitélio pigmentado da íris. A pressão intraocular era normal e acuidade visual de 20/20 em ambos os olhos.

Coroidite serpiginosa macular: relato de caso / Macular serpiginous choroiditis: case report

A coroidite serpiginosa é uma doença rara, que pode causar perda visual irreversível quando a mácula é atingida. Este artigo relata um caso de coroidite serpiginosa com acometimento macular isolado, submetido a tratamento com injeções subtenoniana e intravítrea de triancinolona. Os aspectos clínicos e diagnósticos diferenciais desta doença ocular são discutidos, assim como, os achados de angiografia fluoresceínica e tomografia de coerência óptica de alta resolução.

Serpiginous choroiditis is a rare ocular inflammatory disease that can lead to permanent vision loss due to macular involvement. This article reports a case of a patient with macular serpiginous choroiditis submitted to subtenon and intravitreal triamcinolone injections. The clinical aspects and differential diagnosis of this ocular disease, including fluorescein angiogram and high-resolution optical coherence tomography are discussed.

A Case of Myotonic Dystrophy With Pigmentary Retinal Changes

A 46-year-old man presented with visual disturbances in both eyes. His best corrected visual acuity was 0.7 (both eyes). Ptosis and limitation of ocular movement in every direction were observed. Slit lamp examination showed a bilateral iridescent cataract. Fundus examination showed peripheral depigmentation of the retinal pigment epithelium and pigmentary clumping in both eyes that agreed with blocked fluorescence and widow defects on fluorescein angiography. The amplitude of b-wave was decreased on electroretinography. Fourteen months later, the patient's best corrected visual acuity decreased to 0.3 due to increased lens opacity. Phacoemulsification and intraocular lens implantation were performed on both eyes. At the patient's final visit, retinal findings were stable with a best corrected visual acuity of 0.7 in both eyes. In conclusion, the visual disturbance could have been caused by both cataracts and retinal degeneration, meaning the fundus should be examined carefully in patients with myotonic dystrophy.

Recurrent Occlusion of Laser Iridotomy Sites After Posterior Chamber Phakic IOL Implantation

We report a case of recurrent occlusion of laser iridotomy (LI) sites after a Visian ICL (Implantable contact lens version 4, Staar Surgical AG, Nidau, Switzerland) implantation. A 45-year-old woman had bilateral ICL implantation after placement of two peripheral LI sites in each eye to prevent pupillary block. At one month after the operation, severe narrowing or occlusion of four LI sites occurred. After this, although she received four additional LIs at postoperative months 1, 6, 9 and 10 in both eyes, the narrowing or occlusion recurred. Mild chronic anterior chamber inflammation was observed intermittently throughout the follow-up period. We performed clear lens extraction in both eyes (at postoperative month 11 in the left eye and month 26 in the right eye) due to recurrent occlusion of the LI sites and excess trabecular meshwork pigment deposition presumably caused by the four repeated LIs. Recurrent obstruction of LI sites can occur after ICL implantation. These problems were unresolvable despite four repeated laser iridotomies. The risks associated with anterior uveitis must be considered when planning an ICL implantation.

Retinal pigment epithelial tears after intravitreal bevacizumab injection for exudative age-related macular degeneration.

We report a series of retinal pigment epithelial (RPE) tears after intravitreal bevacizumab therapy for choroidal neovascularization associated with age-related macular degeneration (ARMD). Retinal pigment epithelial tears were estimated to occur at an incidence of 1.6% in this patient population at our institution. Ophthalmologists should be aware of this rare but serious finding associated with exudative macular degeneration therapy.

Avaliação da perimetria macular em pacientes com degeneração macular relacionada à idade por meio do oftalmoscópio de rastreamento a laser / Evaluation of macular perimetry in patients with age-related macular degeneration using the scanning laser ophthalmoscope

OBJETIVOS: Verificar a relação entre alterações anatômicas (drusas duras, drusas moles, hiperpigmentação, neovasos, descolamento do epitélio pigmentado da retina, hipopigmentação e atrofia coriorretiniana) e a sensibilidade à luz em pacientes com degeneração macular relacionada à idade (DMRI); analisar a sensibilidade macular em áreas com ausência de lesões anatômicas nos pacientes com DMRI comparando-as ao grupo de controles, para avaliar a existência ou não de lesão funcional em área sem lesão anatômica. MÉTODOS: Estudo comparativo, descritivo e analítico, de corte transversal. O grupo de casos foi formado por 31 indivíduos portadores de DMRI com idade entre 51 e 88 anos. O grupo de controles ficou composto por 31 indivíduos considerados "sadios", não portadores de DMRI com idade entre 61 e 80 anos. Os grupos foram pareados por sexo e idade. Realizou-se a perimetria macular estática, vermelho-vermelho, com o oftalmoscópio de rastreamento a laser (ORL). Os resultados da perimetria macular foram correlacionados à lesão anatômica identificada no local correspondente pelo laser infravermelho e fotografias coloridas. RESULTADOS: As áreas com neovasos ou atrofia apresentaram sensibilidade significantemente diferente em relação às áreas com ausência de lesões anatômicas nos pacientes com DMRI. Houve perda funcional significativa em áreas com ausência de lesões anatômicas nos pacientes com DMRI em relação ao grupo de controles. CONCLUSÕES: Áreas com neovasos ou atrofia podem ser fatores individuais de piora da sensibilidade macular localizada. Pode ocorrer perda funcional mesmo sem lesão anatômica aparente nos pacientes com DMRI.

PURPOSES: To evaluate the correlation between anatomical changes (hard druses, soft druses, hyperpigmentation, new vessels, detachment of retinal pigment epithelium, hypopigmentation and chorioretinal atrophy) and light sensitivity in patients with age-related macular degeneration (ARMD); analyze macular sensitivity in areas with no anatomical lesions in patients with ARMD and compared them to the control group in order to detect if there was any functional lesion in areas with no anatomical changes. METHODS: A cross-sectional, comparative, descriptive and analytic study was performed. The case group consisted of 31 subjects with ARMD aged between 51 and 88 years. The control group consisted of 31 "healthy" subjects, without ARMD aged between 61 and 80 years. The groups were matched for gender and age. We performed static macular perimetry, red-red, using a scanning laser ophthalmoscope (SLO). Results of macular perimetry were correlated with the anatomic lesion identified in the same site by infrared laser and color photographs. RESULTS: Areas with new vessels or atrophy showed a significantly different sensitivity in relation to areas without anatomical lesions in patients with ARMD. There was significant functional loss in areas with no anatomical lesions in patients with ARMD in relation to the control group. CONCLUSIONS: Areas with new vessels or atrophy could be distinct factors for worsening of the localized macular sensitivity. There might be functional loss even in areas with no apparent anatomical changes in ARMD patients.

Ocular masquerade syndrome due to intraocular lymphoma - two forms of retinal pigment epithelium involvement: case reports

Ocular masquerade syndrome was diagnosted in two patients with chronic uveitis. The patients presented non-Hodgkin's lymphoma as the final diagnosis two forms of intraocular retinal pigment epithelium involvement was seen. One case was flecks of the retinal pigment epithelium and another case was a solid retinal pigment epithelium detachment. These unusual presentations of non-Hodgkin's lymphoma is an alert to all involved in lymphoma care.

Os autores apresentam dois casos de pacientes com linfoma não-Hodgkin, diagnosticados inicialmente como uveite crônica de etiologia não determinada. Os exames posteriores a partir da presença de outros sinais e sintomas demonstraram a presença de linfoma não-Hodgkin nestes pacientes. Um dos pacientes apresentava "flecks" ao nível do epitélio pigmentar da retina e o outro paciente apresentava descolamento sólido do epitélio pigmentar da retina. Estas formas de envolvimento do epitélio pigmentar da retina não são usuais em linfoma não-Hodgkin.

The white dot syndromes

Several entities must be considered when a patient presents with a white dot syndrome. In most cases these can be distinguished from one another based on the appearance or distribution of the lesions, the clinical course, or patient variables such as age, sex, laterality, and functional and image examinations. In this paper we review the distinctive and shared features of the white dot syndromes, highlighting the clinical findings, diagnostic test results, proposed etiologies, treatment, and prognosis.

Várias doenças devem ser consideradas quando nos deparamos com paciente com uma entidade clínica incluída no grupo das "síndromes dos pontos brancos retinianos". O diagnóstico diferencial na maioria das vezes é baseado na aparência e/ou na distribuição das lesões, no curso clínico, ou por algumas variáveis relacionadas ao paciente, tais como idade, sexo, lateralidade, bem como por meio de exames funcionais e de imagem. O presente artigo revisa os achados clínicos das doenças que fazem parte do grupo das "síndromes dos pontos brancos retinianos", enfatizando as similaridades e as diferenças entre essas entidades. Os exames complementares, bem como a etiologia, o tratamento e o prognóstico de cada uma delas são descritos e comentados.

Pigmented epiretinal membranes caused by RPE migration: OCT-based observational case reports.

Epiretinal membranes are cellular sheets on the retinal surface that are formed due to varied etiologies. We present two observational case reports to demonstrate the transretinal migration of the retinal pigment epithelium in pigmented idiopathic epiretinal membranes using optical coherence tomography.

Cancer-associated Nummular Loss of the Retinal Pigment Epithelium

PURPOSE: To report a case of cancer-associated nummular loss of the retinal pigment epithelium. METHODS: A 47-year-old man with a history of hepatocellular carcinoma presented with three weeks of bilateral visual loss. His best-corrected visual acuity was 20/40 in each eye. He had multiple round confluent grayish-brown patches at the level of retinal pigment epithelium, and no pigmented choroidal lesions. Fluorescein angiography showed circular areas of transmission defect and indocyanine green angiography showed early hyperfluorescence, corresponding with the multiple round confluent patches. CONCLUSIONS: We report a case of visual paraneoplastic syndrome which showed nummular loss of the pigment epithelial cells which distinguishes the clinical component of BDUMP syndrome.

Atypical manifestations of acute posterior multifocal placoid pigment epitheliopathy.

We report four patients with acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with atypical features which were unilaterality, serous detachment of the neurosensory retina, retinal vasculitis and papillitis. All patients responded well to oral corticosteroids. We suggest that systemic corticosteroids could be useful in selected cases of APMPPE with atypical clinical features.

Ocular disorders in renal transplant patients

To evaluate patients with renal transplantation for abnormal ocular findings and to determine the association of these findings with pretreatment dialysis duration, immunosuppressive regimen and underlying disease causing renal failure. This cross-sectional study was performed on 150 patients who had undergone kidney transplantation for at least three months and with serum creatinin < 3 mg/dl. All patients underwent ophthalmologic examination including visual acuity, slit lamp biomicroscopy and dilated funduscopy. This study included 91 male and 59 female subjects with mean age of 39 years. No abnormal ocular findings was found in 10.7%; there was at least one ocular disorder in 89.3% including visual acuity

Butterfly-shaped macular dystrophy

To report a case of butterfly-shaped macular dystrophy. A 34-year-old woman presented with metamorphopsia in both eyes from 2 years ago. Visual acuity was 9/10 in right eye and 10/10 in left eye. There was no pathologic finding on slit lamp biomicroscopy and funduscopy other than mild macular retinal pigment epithelium mottling. Visual fields and electroretinogram were normal but electrooculogram showed sub-normal results. Fluorescein angiography showed black, non-fluorescent butterfly-shaped macular structures. Butterfly-shaped macular dystrophy occurs in middle aged subjects and presents with mild visual loss and metamorphopsia. It is slowly progressive and leads to marked visual acuity reduction in older ages. Considering the paucity of clinical findings, fluorescein angiography and/ or electrophysiological tests are recommended for making a correct diagnosis

Optic disc morphometry with optical coherence tomography: comparison with planimetry of fundus photographs and influence of parapapillary atrophy and pigmentary conus.

PURPOSE: Optical coherence tomography (OCT) is an established method for visualising macular pathology and for measuring the thickness of parapapillary retinal nerve fibre layer. The purpose of the present study was to compare OCT measurements of the optic disc with those obtained by planimetry of fundus photographs and to investigate whether OCT measurements are influenced by the amount of parapapillary atrophy or pigmentary conus at the disc margin. MATERIALS AND METHODS: Eighty-two eyes of 41 randomly selected Asian-Indian subjects were included.The mean age was 44.2+/-14.3 years (mean+/-SD). All eyes underwent optic disc assessment by OCT (fast optical disc protocol) and digital 20 degree disc photos.. Planimetry of the discs were performed by outlining the disc and the cup. The amount of parapapillary atrophy (zone beta) was quantified as the number of clock-hours around the disc with adjacent parapapillary atrophy. The same procedure was performed to quantify the amount of pigmentary conus around the disc. RESULTS: Mean+/-SD disc size and mean+/-SD cup size in OCT measurements (2.37+/-0.51, 1.29+/-0.55) were significantly smaller than in photographic planimetry (2.83+/-0.62, 1.56+/-0.5: P<0.001, P<0.001). Optic discs with parapapillary atrophy (P=0.2) in their relative difference of disc sizes between OCT and planimetry. Similarly, optic discs with pigmentary conus (n=12) and discs without pigmentary conus (n=70) did not differ significantly (P=0.65). The relative difference in disc size between the two measurement modalities did not correlate with the amount of parapapillary atrophy (r=-0.17, P=0.29) or with the amount of pigmentary conus (r=-0.04, P=0.9). CONCLUSION: OCT analysis of the optic disc produces significantly smaller parameters, compared to the established method of optic disc planimetry. Neither presence, nor extent of parapapillary atrophy zone beta and pigmentary conus seems to produce a systematic error in measurements of disc size with the OCT.

Role of photodynamic therapy in polypoidal choroidal vasculopathy.

PURPOSE: To evaluate photodynamic Therapy (PDT) with Verteporfin for polypoidal choroidal vasculopathy (PCV) involving the fovea in Indian eyes, through a retrospective interventional case series. MATERIALS AND METHODS: We retrospectively reviewed the records of 9 patients (9 eyes) diagnosed to have PCV with foveal involvement between September 2001 and October 2002. RESULTS: Nine eyes underwent PDT for PCV. Follow-up ranged from 12 to 16 months. Initial visual acuity (VA) ranged from 1/60 to 6/12 and final VA varied from 1/60 to 6/9 at the end of follow-up. VA improved in 4/9 eyes (44.4%) by one line and remained unchanged in 5/9 eyes (55.6%), hence it was considered stabilized in all eyes. No adverse effects or events were observed during or after treatment with verteporfin. CONCLUSION: PDT may be beneficial for PCV with foveal involvement. Its long-term efficacy requires to be evaluated.

RPE atrophy following photodynamic therapy in type 2A idiopathic parafoveal telangiectasis.

We report a case of Type 2A idiopathic parafoveal telangiectasis treated by Photodynamic Therapy (PDT), resulting in retinal pigment epithelial (RPE) atrophy corresponding to the size of the laser spot used. This raises a doubt regarding the safety of photodynamic therapy in CNVM secondary to type 2A parafoveal telangiectasis.

Intraocular osseous metaplasia. A clinico-pathological study.

PURPOSE: To evaluate the clinico-pathologic features of intraocular osseous metaplasia. METHODS: Pathology specimens of enucleated eyes submitted to the ophthalmic pathology service at a tertiary eye-care referral center between January 1995 and June 1999 were studied for intraocular osseous metaplasia. Specific histopathologic features noted in specimens with osseous metaplasia were the presence of retinal detachment, gliosis, retinal pigment epithelial hyperplasia, drusen, epiretinal membrane, fibrovascular proliferation and inflammation. Immunohistochemistry using monoclonal antibody against glial fibrillary acidic protein was performed to assess the glial component within the membranes and the proliferative vitreoretinal mass. Clinical records were reviewed and correlated with histopathologic findings. RESULTS: Osseous metaplasiaS was noted in 8 of 151 (5.2%) eyes examined. Clinical diagnosis in these was phthisis bulbi, staphyloma, absolute glaucoma and microphthalmos. Enucleation was performed for relief of symptoms (in painful blind eyes) or for cosmesis, and in an eye inciting sympathetic ophthalmia. Retinal detachment, gliosis and retinal pigment epithelial hyperplasia were noted in all the cases. Drusen with calcification or ossification (5 of 8), fibrovascular proliferation in the vitreous (5 of 8) and active inflammation (4 of 8) were the other associated histologic features. Location of ossification was subretinal in 3 cases, preretinal (ora serrata) in 1 case and in both locations in 4 cases. The eyes with subretinal osseous metaplasia had associated calcified drusen, while preretinal ossification was seen within the fibrovascular membranes. CONCLUSION: Chronic retinal detachment, hyperplasia and transdifferentiation of retinal pigment epithelium appear to be a few of the prerequisites for intraocular osseous metaplasia. Ossification can occur at isolated subretinal and preretinal locations or can involve both. Though a larger study is required to postulate the chronology of events, in this small series, isolated subretinal ossification appears to be initiated by calcification and ossification of drusen, while in the pre-retinal region it is associated with vitreoretinal proliferation.

Macular serpiginous choroiditis.

PURPOSE: To report a variant form of serpiginous choroiditis, that initially or predominantly involved the macular area. METHODS: Nine eyes of 6 patients with the macular form of serpiginous choroiditis were evaluated clinically and angiographically in a longitudinal fashion for a period of 12-36 months. The active stage and the recurrences were treated by oral and periocular cortico steroids; and two patients were supplemented with oral azathioprine. Most of these patients were referred to our center with varied diagnoses. RESULTS: In this group, 4 were male and 2 were female with an average age of 30.5 years. Three patients had bilateral macular lesions, two had typical serpiginous choroiditis in the fellow eye and the remaining one had unilateral macular involvement alone. The initial visual acuity was 6/60 or less in 60% eyes whereas the final visual acuity was 6/18 or better in 66% eyes. Angiographic findings were typical of serpiginous choroiditis characterised by early hypofluorescence followed by leakage and staining of the borders and the lesion itself without any evidence of choroidal ischaemia or retinal vascular abnormalities. CONCLUSION: The macular variant of serpiginous choroiditis can mimic many other macular pathologic lesions, thus posing a diagnostic dilemma. Because of its relentless destructive course, early diagnosis and prompt treatment is required to prevent sight-threatening complications.