Evaluation of corneal layers and anterior sclera in patients with primary Sjögren's syndrome / Avaliação das camadas da córnea e da esclera anterior na síndrome de Sjögren primária

ABSTRACT Purpose: We aimed to compare the thickness of anterior sclera, corneal layers, and pre-ocular tear film between patients with primary Sjögren's syndrome and healthy individuals. Methods: Fifty-one patients with primary Sjögren's syndrome and 41 healthy control participants were recruited in this cross-sectional and comparative study. The thickness of the pre-ocular tear film, corneal epithelium, Bowman's layer, stroma, Descemet's membrane, and endothelium were measured on the corneal apex. Anterior scleral thickness was measured at distances of 1 mm and 3 mm from the limbus. The anterior segment module of spectral-domain optical coherence tomography was used to measure thicknesses of pre-ocular tear film, corneal layers, and anterior sclera. Results: Tear film thickness, Schirmer's test, and tear break up time values were significantly lower in the Sjögren's disease group than in the healthy controls (p<0.05). The thickness measurements of corneal layers and sclera were similar between the groups. Tear film thickness was moderately correlated with the Schirmer's test results (r=0.34, p=0.001), but there was no correlation between the Schirmer's test results and tear break up time (r=0.18, p=0.09). Conclusions: Pre-ocular tear film, as measured by anterior segment optical coherence tomography, was thinner in patients with primary Sjögren's syndrome than in the healthy controls. The thicknesses of corneal layers and anterior sclera were similar between the groups.

RESUMO Propósito: Nosso objetivo foi comparar a espessura da esclera anterior, camadas da córnea e do filme lacrimal pré-ocular entre pacientes com síndrome de Sjögren primária e indivíduos saudáveis. Métodos: Cinquenta e um pacientes com síndrome de Sjögren primária e 41 controles saudáveis foram recrutados neste estudo comparativo e transversal. A espessura do filme lacrimal pré-ocular, epitélio corneal, camada de Bowman, estroma, membrana de Descemet e endotélio foram medidos no ápice corneal. A espessura da esclera anterior foi medida às distâncias de 1 mm e 3 mm do limbo. O módulo do segmento anterior da tomografia de coerência óptica de domínio espectral foi utilizado para mensurar as espessuras do filme lacrimal pré-ocular, camadas da córnea e esclera anterior. Resultados: A espessura do filme lacrimal, o teste de Schirmer e os valores do tempo de ruptura do filme lacrimal foram significativamente menores no grupo com síndrome de Sjögren do que nos controles saudáveis (p<0,05). As medidas de espessura das camadas corneais e da esclera foram similares entre os grupos. A espessura do filme lacrimal foi moderadamente correlacionada com os resultados do teste de Schirmer (r=0,34, p=0,001), mas não houve correlação entre os resultados do teste de Schirmer e tempo de ruptura (r=0,18, p=0,09). Conclusões: O filme lacrimal pré-ocular, medido pela tomografia de coerência óptica de segmento anterior, foi mais fino em pacientes com síndrome de Sjögren primária do que nos controles saudáveis. As espessuras das camadas da córnea e da esclera anterior foram semelhantes entre os grupos.

Scleral necrosis in patients with posterior uveal melanomas evaluated by transcleral fine needle aspiration biopsy and treated by 125I plaque / Necrose escleral em pacientes com melanoma da úvea posterior avaliados pela biópsia aspirativa com agulha fina e tratados com placa de Iodo 125

ABSTRACT Purpose: To evaluate the incidence, potential correlation with transcleral fine needle aspiration biopsy, and treatment of scleral necrosis in patients with posterior uveal melanomas treated by 125I plaque radiotherapy and assessed by transcleral fine needle aspiration biopsy. Methods: We per­formed a retrospective review of posterior uveal melanoma treated by 125I plaque radiotherapy at a single academic institution between July 2006 and July 2013. Consecutive patients diagnosed with a posterior uveal melanoma during the study period that had an anterior margin at or anterior to the equator who were evaluated by transcleral fine needle aspiration biopsy prior to 125I plaque radiotherapy were included. The main outcome measure was development of scleral necrosis, and the secondary outcome was treatment of this complication. Statistical analysis included computation of conventional descriptive statistics, cross-tabulation and chi-square tests of potential factors related to the development of scleral necrosis, and summarizing of treatment approaches and results. The incidence of treatment of scleral necrosis was calculated using the Kaplan-Meier method. Results: During the 7-year study period, 87 posterior uveal melanomas were evaluated by transcleral fine needle aspiration biopsy and treated by 125I plaque radiotherapy. The median largest basal diameter of the tumor was 13.3 mm, and the median thickness was 6.8 mm. Eight patients (9.2%) developed scleral necrosis during follow-up. Thicker tumors (> 6.5 mm) were more likely to develop scleral necrosis (n=7) than thinner tumors (p=0.05). The median interval between 125I plaque radiotherapy and detection of scleral necrosis was 19.1 months. The overall cumulative probability of scleral necrosis was 6.2% at 6 months and 14.3% at 24 months, subsequently remaining stable. For thicker tumors, the probability of scleral necrosis was 23.5% at 45.4 months. Five patients were treated by scleral patch graft (62.5%) and three by observation (37.5%). One patient underwent enucleation after two failed scleral patch attempts and recurrent scleral necrosis. The mean follow-up period for patients with scleral necrosis was 34.5 months. Conclusions: Thicker posterior uveal melanomas are more likely to develop scleral necrosis after 125I plaque radiotherapy and transcleral fine needle aspiration biopsy. While observation is sufficient for managing limited scleral necrosis, scleral patch graft is a viable alternative for eye preservation in extensive scleral necrosis.

RESUMO Objetivo: Avaliar incidência, possível correlação da biópsia aspirativa com agulha fina trans-escleral e manejo da necrose escleral em pacientes com melanoma da úvea posterior tratados com placa de Iodo-125 (PLACA) avaliados pela biópsia aspirativa com agulha fina trans-escleral. Métodos: Revisão retrospectiva de melanoma da úvea posterior tratados com placa de Iodo-125 entre 07/2006 e 07/2013 em uma única instituição acadêmica. Pacientes diagnosticados consecutivamente com melanoma da úvea posterior durante o intervalo desse estudo cuja margem anterior está no equador ou anterior ao mesmo e foram avaliados pela biópsia aspirativa com agulha fina trans-escleral antes do tratamento com PLACA foram incluídos. O principal desfecho avaliado foi desenvolvimento de necrose escleral e o desfecho secundário foi o manejo dessa complicação. Análise estatística incluiu computação de variáveis descritivas convencionais; tabulação e teste do Chi-quadrado de fatores potencialmente relacionados com o desenvolvimento de necrose escleral e sumarização do manejo dessa complicação. A incidência de necrose escleral foi calculada usando o método de Kaplan-Meier. Resultados: Durante o período de 7 anos desse estudo, 87 melanomas da úvea posterior foram avaliados pela biópsia aspirativa com agulha fina trans-escleral e tratados com placa. A mediana do maior diâmetro basal tumoral foi 13,3 mm e a mediana da espessura foi 6,8 mm. Oito pacientes (9,2%) desenvolveram necrose escleral durante o período de acompanhamento. Tumores mais espessos (> 6,5 mm) foram mais propensos a desenvolver necrose escleral (n=7) que tumores mais finos (p=0,05). O intervalo mediano entre PLACA e a detecção da necrose escleral foi 19,1 meses. Probabilidade cumulativa de desenvolvimento de necrose escleral foi 6,2% em 6 meses e 14,3% em 24 meses permanecendo estável subsequentemente. Em tumores espessos, a probabilidade de necrose escleral foi 23,5% em 45,4 meses. Cinco pacientes foram manejados com enxerto escleral (62,5%), 3 foram observados (37,5%). Um paciente foi enucleado após 2 enxertos esclerais com necrose escleral recidivada. Tempo de seguimento médio dos pacientes com necrose escleral foi 34,5 meses. Conclusões: Tumores espessos pareceram mais propensos a desenvolver necrose escleral após PLACA e biópsia aspirativa com agulha fina trans-escleral para melanoma da úvea posterior. Apesar de observação para necrose escleral limitada ser suficiente, enxerto de esclera é uma alternativa viável para preservação ocular em necrose escleral extensa.

Treatment of perforated cornea with an autologous lamellar scleral graft: histologic findings / Tratamento de córnea perfurada com enxerto autônomo de esclera lamelar: achados histológicos

ABSTRACT We report a case of central corneal perforation treated with an autologous lamellar scleral graft and histologic findings obtained after a subsequent penetrating keratoplasty. A corneal perforation within a large Pseudomonas ulcer in a 55-year-old male rigid gas permeable contact lens wearer was sealed by a lamellar scleral graft from the same eye, followed by an uneventful penetrating keratoplasty 6 months later. Histology of the excised button revealed that the well-apposed graft, which maintained the irregular arrangement of the scleral collagen fibers, was embedded in the corneal stroma over the deep blood vessels and a rupture in Descemet's membrane. The clinical and histologic findings showed that autologous lamellar scleral grafts can be successfully used for the emergency treatment of corneal perforation when a corneal transplant is not available. The distinctive scleral structure revealed by histology and the inadequate graft transparency indicate that visual rehabilitation of eyes with a central corneal perforation can be achieved only by a subsequent optic penetrating keratoplasty.

RESUMO Relatamos um caso de perfuração corneana central tratada com enxerto autólogo lamelar de esclera e os achados histológicos obtidos após ceratoplastia penetrante (CP) subsequente. Uma perfuração da córnea devido a uma grande úlcera por Pseudomonas em um usuário de lentes de contato rígidas gás permeável de 55 anos de idade foi selada por um enxerto escleral lamelar do mesmo olho, seguida de ceratoplastia penetrante, sem intercorrências, seis meses depois. A histologia do botão excisado revelou que um enxerto bem posicionado, que manteve o arranjo irregular das fibras de colágeno escleral, foi incorporado no estroma corneano sobre os vasos sanguíneos profundos e uma ruptura na membrana de Descemet. Os achados clínicos e histológicos demonstraram que o enxerto autônomo de esclerose lamelar pode ser usado com sucesso como tratamento de emergência da perfuração da córnea, quando o transplante de córnea não é possível. A estrutura escleral característica revelada pela histologia e a transparência inadequada do enxerto indicam que a reabilitação visual dos olhos com uma perfuração corneana central só pode ser alcançada através de uma ceratoplastia penetrante óptica subsequente.

Management of necrotizing scleritis after pterygium surgery with rituximab / Tratamento com rituximabe de esclerite necrosante após cirurgia de pterigeo

ABSTRACT The authors present a case of necrotizing scleritis after pterygium excision successfully treated with rituximab after attempts with high doses of corticosteroids and immunosuppressive drugs. A literature review revealed case reports and a phase I/II dose-ranging randomized clinical trial using rituximab for necrotizing scleritis with or without association with autoimmune disease. This is the only case report on rituximab treatment for necrotizing scleritis after pterygium surgery. In cases with refractoriness to immunosuppressive drugs, a CD20 antibody can be used.

RESUMO Os autores apresentam um caso de sucesso no tratamento com rituximabe de esclerite necrosante após cirurgia de pterígio refratário a altas doses de corticosteroides e drogas imunossupressoras. Uma revisão da literatura direcionada ao uso de rituximabe para tratamento de esclerites necrosantes revelou relatos de casos e um estudo clínico randomizando fase I/II. Este é o único caso descrito de rituximabe para o tratamento de esclerite necrosante pós cirúrgica. O uso de anticorpo anti-CD20 pode ser uma opção em casos refratários aos imunossupressores no tratamento da esclerite necrosante pós-cirúrgica.

Choroidal melanoma recurrence after episcleral brachytherapy and transpupillary thermotherapy / Recorrência melanoma de coroide após a braquiterapia episcleral e termoterapia transpupilar

ABSTRACT A 68-year-old man diagnosed with choroidal melanoma (CM) in the right eye underwent treatment with episcleral brachytherapy (I125) and transpupillary thermotherapy. Ultrasound, computed tomography, and magnetic resonance imaging were performed and revealed ocular recurrence of CM. Treatment with extended enucleation was performed. Macroscopic and microscopic examinations revealed extraocular extension and malignant cells, respectively. Immunohistochemistry demonstrated tumoral Melan-A and HMB-45 expression. No cytogenic abnormalities were detected with fluorescence in situhybridization of tumor cells using probes against chromosomes 3q27 and 8q24. The patient underwent adjuvant external beam radiotherapy for treatment of residual tumor tissue. This case represents the first reported case of recurrent CM with no cytogenetic abnormalities and the absence of metastatic disease, despite a number of the poorest prognostic factors.

RESUMO Um homem de 68 anos de idade com diagnóstico de melanoma de coroide no olho direito foi submetido a tratamento com braquiterapia episcleral (I125) e termoterapia transpupilar. Ultrassonografia, tomografia computadorizada e ressonância magnética foram realizadas para avaliar a presença de recorrência ocular ou doença sistêmica. Enucleação ampliada foi realizada para tratar a recorrência ocular. O exame macroscópico e microscópico revelou o tipo de célula tumoral e a extensão extraocular. Colorações por Melan-A e HMB-45 foram realizadas. A fluorescência por hibridização in situ com sondas para os cromossomos 3q27 e 8q24 não mostraram anormalidades citogenéticas. O paciente foi submetido a radioterapia externa adjuvante para o tratamento de tumor residual orbitário. Este caso representa a o primeiro relato de paciente sem anomalias citogenéticas e sem doença metastática, apesar de demonstrar alguns dos mais pobres fatores prognósticos.

Coexistence of posterior staphyloma, retinitis pigmentosa and moderate myopia

The authors report a 22-year patient presented with night-blindness and progressive visual loss. Ophthalmologic examination included slit lamp biomicroscopy, best-corrected distance visual acuity, cycloplegic refraction, A-mode and B-mode ultrasonography, and full-field flash electroretinogram [ERG]. Cycloplegic refractions were -6.00 [-1.25 x 175] dpt in the right eye and -7.00 [-1.50 x 165] dpt in the left eye. Slit lamp examination showed normal anterior segment. Fundoscopic examination revealed bone-spicule pigmentations, waxy optic disc, arteriolar narrowing and posterior staphyloma, with 5 optic disc in diameter in the right eye and 4 optic disc diameter in the left. Ocular ultrasonography confirmed staphyloma. Full-field ERG showed evidence of a generalized retinal dysfunction involving both rod and cone responses, supported the diagnosis of retinitis pigmentosa

Alkaptonuria - Case report

Alkaptonuria, also called endogenous ochronosis, is a rare metabolic autosomal recessive disorder. It occurs by complete inhibition of homogentisic acid oxidase enzyme having its deposition in various tissues. Male patient, 52 years old, sought medical help complaining about progressive appearance of hyperchromic papules on the lateral edge of the second finger of both hands for 02 years. He also complained about darkening of urine, sperm and underwear. Incisional biopsy of second hand finger and test for homogentisic acid in the urine results were positive. The findings are compatible with the diagnosis of alkaptonuria. Given these findings, treatment was initiated, followed-up by other specialties and he was advised to avoid certain foods.

Expression of TNF-α and IL-6 cytokines in the choroid and sclera of hypercholesterolemic rabbits / Expressão das citocinas TNF-α and IL-6 na coroide e esclera de coelhos hipercolesterolêmicos

Purpose: This study aimed to evaluate the expression of the inflammatory cytokines TNF-α and IL-6 in the sclera and choroid of hypercholesterolemic rabbits. Method: Twenty-one New Zealand male albino rabbits were divided into two groups: NG and HG. The NG group was fed a standard rabbit diet and the HG group was fed a cholesterol-enriched diet (1%). The serum total cholesterol, triglyceride, HDL cholesterol, and fasting blood glucose levels were determined at the beginning of the experiment and on the day of euthanasia. Euthanasia of animals in the NG and HG groups was performed at the end of the 4th and 8th week, respectively. The eyes were analyzed immunohistochemically using TNF-α and IL-6 antibodies. Results: At the time of euthanasia, the HG group showed a significant increase in total cholesterol and triglyceride when compared with the NG group (p<0.001). When compared with the NG group, there was a significant increase in the expression of TNF-α (p<0.001) and IL-6 (p=0.002) in the choroid and sclera of animals in the HG group. Conclusion: This study demonstrates that the hypercholesterolemic diet induces expression of TNF-α and IL-6 in the choroid and sclera of rabbits. .

Objetivo: Avaliar a expressão das citocinas inflamatórias TNF-α e IL-6 na esclera e coroide de coelhos hipercolesterolêmicos. Método: Coelhos New Zealand foram organizados em dois grupos: GN recebeu ração padrão para coelhos; GH recebeu dieta rica em colesterol a 1%. Foi realizada a dosagem sérica de colesterol total, triglicerídeos, HDL colesterol, glicemia de jejum no início do experimento e no momento da eutanásia. Ao final da 4ª semana para o GN e 8ª semana para o GH foi realizada a eutanásia dos animais. Os olhos foram submetidos à análise imuno-histoquímica com os anticorpos TNF-α e IL-6. Resultados: O GH manifestou significativo aumento do colesterol total e triglicerídeos em relação ao GN (p<0,001). Houve significativo aumento da expressão da TNF-α (p<0,001) e da IL-6 (p=0,002) na coroide e esclera dos animais do GH em relação ao GN. Conclusão: Este estudo demonstra que a dieta hipercolesterolêmica induz ao aumento da expressão das citocinas TNF-α e IL-6 na coroide e esclera de coelhos. .

Severe scleral dellen as an early complication of pterygium excision with simple conjunctival closure and review of the literature / "Dellen" escleral grave como complicação precoce de excisão de pterígio com fechamento conjuntival simples e revisão da literatura

We describe a patient with acute scleral dellen (SD) after pterygium excision with simple conjunctival closure. In addition, we present a PUBMED review on the medical literature on early SD after pterygium surgery. This case describes a 45-year-old man who presented with severe SD, 7 days after pterygium surgery with minimal cauterization of episcleral vessels and simple conjunctival closure. No other adjunctive therapy was used intraoperatively. The patient refused conjunctival flap coverage of the lesion. Therefore, medical treatment consisted of antibiotic ointment, patching, and daily follow-up. After 7 days, the patching was changed for intensive ocular lubrication. Five weeks later, the surrounding conjunctiva had completely covered the affected sclera. To the best of our knowledge, this is the first report of early SD following pterygium excision and simple conjunctival closure with no other adjunctive therapy. When performing pterygium excision with conjunctival coverage of the sclera, a close follow-up is recommended to rule out wound dehiscence and SD, even when surgical wound closure is considered to prevent SD. If this complication is detected, the treatment can be conservative.

Descrevemos um paciente com "dellen" escleral agudo (SD) após excisão de pterígio com fechamento conjuntival simples. Uma revisão adicional da literatura médica sobre SD precoce após a cirurgia de pterígio também é realizada. Este caso descreve um homem de 45 anos de idade, que apresentou SD grave, sete dias após a cirurgia de pterígio com cauterização mínima de vasos episclerais e fechamento conjuntival simples. Nenhuma outra terapia adjuvante foi utilizada no intraoperatório. O paciente recusou-se à cobertura de retalho conjuntival da lesão. Portanto, o tratamento médico consistiu em pomada antibiótica, oclusão e acompanhamento diário. Após sete dias, a oclusão foi mudada para a lubrificação ocular intensiva. Cinco semanas após, a conjuntiva cobriu completamente a esclera afetada. Ao melhor de nosso conhecimento, este é o primeiro relato de SD precoce após a excisão do pterígio e fechamento conjuntival simples com nenhuma outra terapia adjuvante. Ao realizar a excisão do pterígio com cobertura conjuntival da esclera, um acompanhamento frequente é recomendado para descartar a deiscência da ferida e SD. Se esta complicação for detectada, o tratamento pode ser conservador.

Aumento da imunorreatividade ao VEGFR-1 no complexo coroido-escleral em modelo experimental de hipercolesterolemia / Increased VEGFR-1 immunoreactivity in the choroid-scleral complex in hypercholesterolemia experimental model

OBJETIVO: O objetivo deste trabalho é investigar a expressão do fator de crescimento vascular endotelial (VEGF) na coroide e esclera, utilizando um modelo experimental de hipercolesterolemia. MÉTODO: Coelhos New Zealand foram organizados em dois grupos: O grupo dieta normal (GN), composto por 8 coelhos (8 olhos), recebeu ração padrão para coelhos, durante 4 semanas; e o grupo hipercolesterolêmico (GH), composto por 13 coelhos (13 olhos), recebeu dieta rica em colesterol a 1% por 8 semanas. Foi realizada a dosagem sérica de colesterol total, triglicerídeos, HDL colesterol, glicemia de jejum no início do experimento e no momento da eutanásia. Ao final da 8ª semana para o GH e 4ª semana para o GN foi realizada a eutanásia dos animais e os olhos foram submetidos à análise imuno-histoquímica com os anticorpos RAM-11 e VEGFR-1. RESULTADOS: Observou-se significativo aumento do colesterol total e triglicerídeos do GH em relação ao GN (p<0,001). Houve significativo aumento da expressão da RAM-11 e VEGFR-1 na coroide e esclera dos animais do GH em relação ao GN (p<0,001). CONCLUSÃO: Este estudo demonstra que a dieta hipercolesterolêmica em coelhos induz ao aumento da concentração de macrófagos e da imunorreatividade ao VEGFR-1 na coroide e esclera, expressando similaridade com a degeneração macular relacionada à idade (DMRI) humana.

PURPOSE: The aim of this study is to investigate the expression of vascular endothelial growth factor (VEGF) in the choroid and sclera using hypercholesterolemia experimental model. METHODS: New Zealand rabbits were divided into two groups: 8 rabbits (8 eyes), in the normal diet group (NG), were fed by a standard diet for 4 weeks; and 13 rabbits (13 eyes), in the hypercholesterolemic group (HG), were fed by a 1% cholesterol-enriched diet for 8 weeks. Total serum cholesterol, triglyceride, HDL cholesterol and fasting blood glucose exams were performed at the initiation of the experiment and at the euthanasia time. After hypercholesterolemic group 8th week and NG 4th week, animals were euthanized and their eyes underwent immunohistochemical analysis with the RAM-11 and VEGFR-1). RESULTS: The diet has induced a significant increase in total cholesterol and triglyceride levels in HG when compared with NG (p<0.001). There was a significant increase in the RAM-11 and VEGFR-1 expressions in hypercholesterolemic group choroid and sclera in relation to NG (p<0,001). CONCLUSION: This study has revealed that the hypercholesterolemic diet in rabbits induces an increase in the macrophage concentration and immunoreactivity to VEGFR-1 in the choroid and sclera, resembling human age-related macular degeneration (ARMD).

Papillary Thyroid Carcinoma: Bilateral Choroidal Metastases with Extrascleral Extension

Here, we present the case of a patient with bilateral choroidal metastases with extraocular extension in one eye. Metastasis of papillary thyroid carcinoma to the uvea is extremely rare, with only 6 patients reported in the literature. A 62-year-old man with a prior history of papillary thyroid carcinoma suffered the rapid loss of vision in his right eye. He had neovascular glaucoma, total retinal detachment, and a solitary choroidal mass. A month later, his left visual acuity also decreased because of a small macular choroidal mass. The right eye was enucleated and a nodular lesion over the sclera representing extraocular extension was observed. This tumor and the intraocular lesion were composed of papillary excrescences and cystic spaces and stained positively for thyroid transcription factor 1 and thyroglobulin, all confirming the diagnosis of metastatic papillary thyroid carcinoma. The tumor in the left eye was successfully treated with diode laser transpupillary thermotherapy. The patient expired within a month as a result of widespread pulmonary metastases. Papillary thyroid carcinoma may metastasize to the uvea bilaterally, cause rapid visual function loss, erode the sclera, and may extend outside the globe similar to choroidal melanoma. This aggressive ocular involvement was associated with a dismal prognosis in our patient.

Tectonic corneal graft for conjunctival rhinosporidiosis with scleral melt.

A 16 year old girl presented with irritation and watering of the right eye for 3 months. On examination, the superior perilimbal sclera was ectatic with incarcerated uveal tissue covered by conjunctiva. The conjunctiva showed discreet, yellow white mucoid spots. Excision biopsy of the conjunctiva showed subepithelial spherules of sporangia containing numerous endospores, suggestive of rhinosporidiosis. Diathermy was applied to flatten the staphyloma. The ectatic area was covered with a corneal patch graft. The patient was started on prednisolone acetate eye drops and oral dapsone for 6 months. Corneal graft was well incorporated and conjunctivalized by 3 months. Since the graft was not seen within the palpebral aperture, there was good cosmetic result. The corneal graft had the added advantage of transparency which allowed visualization of the underlying tissue to diagnose early recurrence. There was no recurrence at 6 months.

Preserved scleral patch graft for unexpected extreme scleral thinning found at the scleral buckling procedure: A case report.

Pre-existing scleral pathology is an important risk factor for globe rupture during scleral buckling procedures. We report here, the surgical management of an unexpected scleral pathology found at the scleral buckling procedure in a retinal detachment patient. A 77-year-old white female with retinal detachment underwent a scleral buckling procedure. The surgery was converted into a scleral graft procedure, as extreme scleral thinning was found intraoperatively. An alcohol-preserved donor sclera graft was used. The second surgery for definitive retinal alignment was performed two weeks later. The presented case of an unexpected scleral pathology in a retinal detachment patient was managed with a combination of scleral grafting and pars plana vitrectomy, without any major complications. The anatomical outcome was excellent and the scleral rupture was prevented; the visual outcome was satisfactory. A conversion of the scleral buckling procedure into a scleral graft procedure has proved to be safe and effective for unexpected scleral pathology.

Aspergillus fumigatus Scleritis Associated with Monoclonal Gammopathy of Undetermined Significance

A 68-year-old woman presented with pain in her left eye. Necrosis with calcium plaques was observed on the medial part of the sclera. Aspergillus fumigatus was isolated from the culture of the necrotic area. On systemic work-up including serum and urine electrophoresis studies, the serum monoclonal protein of immunoglobulin G was detected. The patient was diagnosed with monoclonal gammopathy of undetermined significance and fungal scleritis. Despite intensive treatment with topical and oral antifungal agents, scleral inflammation and ulceration progressed, and scleral perforation and endophthalmitis developed. Debridement, antifungal irrigation, and tectonic scleral grafting were performed. The patient underwent a combined pars plana vitrectomy with an intravitreal injection of an antifungal agent. However, scleral and intraocular inflammation progressed, and the eye was enucleated. Aspergillus fumigatus was isolated from the cultures of the eviscerated materials. Giemsa staining of the excised sclera showed numerous fungal hyphae.

Esclerite posterior: características clínicas, associação sistêmica, tratamento e evolução de 23 pacientes / Posterior scleritis: clinical features, systemic association, treatment and evolution of 23 patients

OBJETIVOS: Documentar características clínicas, associações sistêmicas, tratamento e evolução de 23 pacientes com esclerite posterior, examinados no serviço de uveítes da Universidade Federal de Minas Gerais. MÉTODOS: Revisão de todos os pacientes com esclerite atendidos neste serviço, de 1999 até 2006, para identificar aqueles com esclerite posterior. Identificados 23 pacientes, registrados e analisados os dados com relação aos sinais e sintomas oculares, visão, alterações na ecografia, manifestações sistêmicas, tratamento e evolução. RESULTADOS: Dezesseis pacientes do sexo feminino e 7 do sexo masculino com média de idade de 44,7 anos. Esclerite posterior ocorreu associada à esclerite anterior em 10 pacientes, envolvimento unilateral em 17 pacientes e, bilateral simultâneo, em 6 pacientes. Esclerite posterior associada à doença sistêmica ocorreu em 8 pacientes (síndrome de Cogan, tuberculose, granulomatose de Wegener, herpes simples e zoster, aspergilose, retocolite-ulcerativa e sarcoidose). A principal queixa foi dor ocular seguida de embaçamento visual e o sinal fundoscópico que predominou foi o descolamento seroso de retina. O achado mais comum na ecografia foi espessamento da parede escleral observado em 18 pacientes e a principal forma de tratamento, o uso de corticóide sistêmico. Somente 4 pacientes necessitaram de imunossupressor. CONCLUSÃO: Esclerite posterior é doença de difícil diagnóstico e pode ser potencialmente devastadora. Análises estatísticas são incapazes de revelar outras características específicas da esclerite posterior, características clínicas dos pacientes e evolução da doença que poderiam ajudar na identificação dos casos com maior risco de perda visual ou com maior probabilidade de doença sistêmica.

PURPOSE: To document the clinical features, systemic association, treatment and evolution of 23 patients with posterior scleritis evaluated in the Uveitis service of the Federal University of Minas Gerais. METHODS: 23 patients were identified with the diagnosis of posterior scleritis. Signals and symptoms, visual acuity, B-mode ultrasonography signals, systemic associations, treatment and evolution were described and analyzed. RESULTS: Sixteen patients were female and seven were male with mean age of 44,7 years. Posterior scleritis occurred in association with anterior scleritis in 10 patients, unilateral involvement in 17 patients and simultaneous bilateral involvement in 6 patients. Posterior scleritis in association with systemic disease occurred in 8 patients (Cogan's syndrome, TBC, Wegener, Herpes simplex and Zoster, Apergilosis, inflamatory bowel disease and Sarcoidosis). The main symptoms were ocular pain and decrease of visual acuity and the main signal was retinal serous detachment. Increase of thickness choroidal tissue was the main signal in B-mode ultrasonography in 18 patients and the principal kind of treatment was the use of systemic corticosteroids. Only 4 patients required systemic immunosuppressive drugs. CONCLUSIONS: Posterior scleritis still represents a diagnostic challenge and is often associated with life threatening systemic disease and vision threatening ocular complications. Knowledge of posterior scleritis may aid in determining timely and accurate diagnosis and treatment of both ocular and any systemic conditions associated, thus decreasing morbidity and mortality. Elevated suspicion rate is always required to detect this condition.

Avaliação das alterações precoces na coróide e esclera ocorridas em coelhos hipercolesterolêmicos: estudo histológico e histomorfométrico / Evaluation of choroid and sclera early alterations in hypercholesterolemic rabbits: histologic and histomorphometric study

OBJETIVO: Demonstrar experimentalmente, através de exames histológicos e histomorfométricos, as alterações degenerativas da esclera e coróide desencadeadas precocemente pela dieta hipercolesterolêmica. MÉTODOS: Coelhos New Zealand foram organizados em dois grupos: GC (grupo controle), composto por 6 coelhos (6 olhos), recebeu dieta normal por 6 semanas; G1, composto por 12 coelhos (12 olhos), tratado previamente com ração colesterol a 1 por cento (Sigma-Aldrich) por 2 semanas e a partir do 14º dia com ração colesterol a 0,5 por cento (Sigma-Aldrich). Os olhos foram submetidos à análise histológica, avaliados com corante de hematoxilina-eosina e ao exame morfométrico. A análise histomorfométrica foi realizada no setor posterior, adjacente ao disco óptico, e na periferia. RESULTADOS: O GC apresentou espessura média da esclera e coróide na periferia de 228,61 ± 31,71 micrômetros, enquanto na região posterior de aproximadamente 246,07 ± 25,66 micrômetros. No G1, observou-se espessura média da esclera e coróide na periferia de aproximadamente 303,56 ± 44,21 micrômetros, enquanto na região posterior de aproximadamente 295,59 ± 62,59. Houve diferença estatisticamente significativa da espessura da esclera e coróide entre os grupos na região periférica (p<0,001), não ocorrendo o mesmo no setor posterior (p=0,250). O aumento da espessura da parede de G1 em relação ao GC deve-se principalmente à quantidade elevada de histiócitos e fibras colágenas. CONCLUSÃO: Este estudo demonstrou que a dieta hipercolesterolêmica em coelhos induz rapidamente aumento da espessura da coróide e esclera, principalmente à custa de histiócitos e fibras colágenas.

PURPOSE: To demonstrate experimentally, by means of histological and histomorphometric examinations, the sclera and choroid degenerative alterations, which take place at an early stage due to a hypercholesterolemic diet. METHODS: New Zealand rabbits were divided into two groups: CG (control group) of 6 rabbits (6 eyes) received a regular diet for 6 weeks; G1, of 12 rabbits (12 eyes), was first fed a 1 percent cholesterol diet (Sigma-Aldrich) for 2 weeks and then from the 14th day on a 0.5 percent cholesterol diet (Sigma-Aldrich). The eyes underwent a histological analysis, stained with hematoxiline-eosine, and a morphometric examination. The histomorphometric analysis was performed in the posterior region, adjacent to the optic disk, and in the peripheral region. RESULTS: The CG presented a mean sclera and choroid thickness of 228.61 ± 31.71 micrometers in the peripheral region, while the thickness in the posterior region was approximately 246.07 ± 25.66 micrometers. In G1, these values were 303.56 ± 44.21 micrometers in the peripheral region and 295.59 ± 62.59 in the posterior region. There was a statistically significant difference in the sclera and choroid thickness between the groups in the peripheral region (p<0.001); however, this difference did not occur in the posterior region (p=0.250). The large number of histiocytes and collagen fibers accounted for the increase of G1 wall thickness in relation to CG. CONCLUSION: This study demonstrated that the hypercholesterolemic diet in rabbits induces a fast increase in the choroid and sclera thickness, mainly due to the increase in the number of histiocytes and collagen fibers.

Anomalous Scleral Insertion of Superior Oblique in Axenfeld-Rieger Syndrome

Axenfeld-Rieger syndrome (ARS) is associated with ocular and systemic anomalies. PITX2 is known to be a major controlling gene in the pathogenesis of ARS and is associated with differentiation in both the neural crest and mesoderm during eye development. A 4-year-old girl with bilateral ARS had 20 prism diopters (PD) of exotropia with 30PD of A- pattern deviation, more than 20PD of dissociated vertical deviation (DVD), and severe superior oblique overaction (SOOA). During surgery we observed that the SO inserted more posteriorly than normal. We believe this finding is one of the abnormal manifestations of the development of the extraocular muscles in ARS.

Tumor indiferenciado da lâmina fusca / Undifferentiated tumor of the lamina fusca

Na reunião da Associação Pan-americana de Patologia Oftálmica, realizada em Los Angeles CA, 10 de Outubro de 1991, no DOHENY EYE INSTITUTE, C.O. Degrazia propôs o nome de LOFONEUROGONIOMA para um tumor solitário intra-ocular, indiferenciado, originado nas células da lâmina fusca, com células indiferenciadas de expressiva diferenciação para a linhagem schwannocítica, melanocítica e neuroendócrina. Em face do trimorfismo, o patologista pode ser conduzido para os diagnósticos de melanoma, schwannoma maligno ou outro tipo de tumor. O exaustivo estudo do tumor apresentado, através da microscopia eletrônica, da histoquímica e da imunohistoquímica permitiu a formulação da seguinte hipótese: uma célula indiferenciada em repouso, a lofoneurogô- nia, segue as linhagens melanocítica, schwannocítica e neuroendócrina. A diversidade de células dentro de um tumor, o continuus intratumor, responsável pela estrutura em mosaico de muitas neoplasias, além da multiclonalidade resultante de mitoses atípicas, pode ser explicada por essa hipótese. É dessa maneira que se torna compreensível a classificação de Callender para os melanomas intra-oculares nos tipos celulares fusiforme A, fusiforme B, epitelióide, fasciculado e misto, num verdadeiro continuus intertumores. Para justificar a designação proposta, e para enquadrar o caso num grupo taxonômico, foram usadas duas bases classificatórias: 1o ­ histogênica, isto é, correlacionar as células do tumor com as células normalmente existentes nas membranas oculares, no caso, a lâmina fusca; 2o ­ embriogênica isto é, delimitar um grupo de tumores cuja base, no desenvolvimento do embrião, é a crista neural (AU)

In the meeting of the Panamerican Association of Ophtalmic Pathology, held in the Doheny Eye Institute in Los Angeles, CA on Oct 10 1991, C.O. Degrazia proposed the name LOPHONEUROGONIOMA for an undifferentiated intraocular solitary tumor, originating from the cells of the lamina fusca, with undifferentiated cells of expressive differentiation for the schwannian, melanocitic and neuroendocrine lines. Because of the trimorphism, the pathologist may be led to the diagnosis of melanoma, malignant schwannoma or other type of tumor. The exhaustive investigation of the presented tumor through electronmicroscopy, histochemistry, and immunohistochemistry allowed the formulation of the following hypothesis: an undifferentiated cell at rest, the lophoneurogonia, follows the melanocitic, schwannian, and neuroendocrine lines. The diversity of cells inside a tumor responsible for the mosaic structure of many neoplasias, besides the multiclonality resulting from atypical mitoses, can be explained by this hypothesis. This also elucidates Callender's classification of intraocular melanomas in cell types fusiform A, fusiform B, epithelioid, fasciculated and mixed, in a true intertumor continuum. In order to justify the proposed designation, and to fit the case into a taxonomic group, two classificatory bases were used: first, a histogenic one, correlating tumor cells with normally existing cells; and second, embriogenic which bases is the neural crest (AU)

Porcine pericardium as glaucoma implant tube coverage: an experimental study / Uso de pericárdio suíno no recobrimento de implantes de drenagem de glaucoma: um estudo experimental

PURPOSE: To evaluate the inflammatory response associated with the use of processed porcine pericardium and glycerin-preserved homologous sclera as silicone glaucoma drainage device coverage in a rabbit experimental model. METHODS: Eight New Zealand white rabbits' eyes received an equal-sized glycerin-preserved homologous scleral patch or a double-layered processed porcine pericardium that was sutured to the bare sclera covering a silicone tube. Conjunctival hyperemia was graded using the double-blind method during the immediate postoperative period and during the first, third, and seventh postoperative weeks. After the seventh week, the enucleated eyes were histopathologically examined. They were also evaluated for signs of patch graft melting, tube erosion and chemosis. RESULTS: There was no occurrence of graft melting or tube exposure, although porcine pericardium was associated with greater inflammation through clinical observation. Light microscopy revealed marked inflammation surrounding the porcine pericardium with foreign body granuloma formation. On the other hand, the sclera group presented milder inflammation with foreign body granulomas only around the sutures. CONCLUSIONS: Porcine pericardium is associated with significant inflammation when used as tube coverage in a rabbit model, at both histopathologic and clinical levels, when compared to glycerin-preserved homologous sclera during the seven-week follow-up period.

OBJETIVO: Avaliar a resposta inflamatória associada com o uso de pericárdio suíno processado e esclera homóloga preservada em glicerina no recobrimento de tubo de drenagem de silicone utilizado em cirurgia de glaucoma em modelo experimental. MÉTODOS: Oito olhos de 8 coelhos albinos da raça Nova Zelândia receberam enxertos do mesmo tamanho de esclera homóloga, preservada em glicerina ou pericárdio suíno processado, suturados à esclera recobrindo um tubo de silicone. Os olhos foram avaliados quanto à hiperemia conjuntival de modo mascarado na primeira, terceira e sétima semanas de pós-operatório. Após a sétima semana os olhos foram enucleados e submetidos a estudo histopatológico. Os olhos também foram avaliados quanto à presença de afinamento do enxerto, exposição do tubo e quemose. RESULTADOS: Não ocorreram casos de afinamento do enxerto ou exposição do tubo, porém o pericárdio suíno esteve associado clinicamente a uma maior inflamação. Achados à microscopia óptica incluíram intensa reação inflamatória em torno do enxerto de pericárdio com formação de granuloma do tipo corpo estranho. Por outro lado, no grupo que recebeu esclera, ocorreu formação de granulomas apenas em torno dos fios de sutura. CONCLUSÃO: O pericárdio suíno esteve associado a maior inflamação tanto à histopatologia quanto clinicamente, quando usado no recobrimento de tubo de silicone, em comparação à esclera homóloga preservada em glicerina, durante o período de acompanhamento de sete semanas.