Long-term results of topical mitomycin C 0.02% for primary and recurrent conjunctival-corneal intraepithelial neoplasia.

PURPOSE: To evaluate the efficacy, recurrence rate, and long-term complications of topical mitomycin C (MMC) 0.02% for conjunctival-corneal intraepithelial neoplasia (CCIN). METHODS: A prospective, nonrandomized, noncontrolled study was conducted of patients with primary or recurrent CCIN treated with topical MMC 0.02%, four times per day, for 28 consecutive days. The main outcome measures were complete resolution of the neoplasia by slit-lamp examination and cytology 1 month after treatment, tumor recurrence, and long-term complications. RESULTS: Between June 1999 and September 2005, 23 patients were included. Eighteen had primary CCIN (group 1) and 5 had recurrent CCIN (group 2). The mean follow-up was 46 months in group 1 and 54 months in group 2. All patients were treated with MMC 0.02% for 28 consecutive days. Complete resolution of the lesion was achieved in all patients after 1 month of treatment. Recurrence occurred in 1 patient (4.3%) after 24 months of treatment. Four patients developed corneal erosion (17.4%), 2 of them with primary CCIN and 2 with recurrent CCIN. Corneal erosion occurred 4 to 24 months after treatment and was treated successfully. The probability for corneal erosions by the log-rank test was equal for both groups (p = 0.1705). CONCLUSIONS: The use of topical MMC 0.02% for 28 consecutive days to treat primary or recurrent CCIN was effective and showed a low recurrence rate. Corneal erosion occurred in 17.4% of cases and can occur as late as 24 months after treatment.

A new device for ocular globe enucleation.

It is important to perfect the enucleation technique, especially in cases of intraocular malignancy, to obtain an adequate amount of tissue for histologic analysis and to cause a minimum amount of trauma to the remaining orbital structures. This study describes the use of a new device for ocular globe enucleation. The new instrument has been employed in 16 consecutive enucleation procedures with no complications. The new device has promoted good division of the optic nerve and allowed severing of the optic nerve stump at a length of more than 10 mm.

Ultrasound biomicroscopy features of iris and ciliary body melanomas before and after brachytherapy.

BACKGROUND AND OBJECTIVE: To determine ultrasound biomicroscopy (UBM) features of iris and ciliary body melanomas before and after brachytherapy. PATIENTS AND METHODS: Four uveal anterior melanoma cases undergoing brachytherapy were retrospectively studied. All cases were examined by UBM prior to treatment and repeatedly after treatment. RESULTS: Before brachytherapy, UBM examination showed a solid mass in the iris, ciliary body, or both in all four cases and allowed its characterization, sizing, and positioning. Two cases had a pigmented scleral lesion corresponding to the tumor location, but UBM did not detect any scleral infiltration at those sites. After brachytherapy, all lesions showed progressive decrease in size and progressive attenuation of their limits. Internal reflectivity was variable. Complications related to brachytherapy were demonstrated, including cataract, peripheral anterior synechiae at tumor location, and secondary scleral thinning. CONCLUSION: UBM played an important role as a complementary diagnostic method for anterior uveal melanomas, particularly ciliary body melanomas, but also allowed therapeutic planning (brachytherapy or surgery) and follow-up after treatment.

Relationship between histopathological features of chemotherapy treated retinoblastoma and P-glycoprotein expression.

BACKGROUND: P-glycoprotein (P-gp) has been identified as a possible mediator of chemoresistance in retinoblastoma. The aim of this study was to determine the expression of P-gp in retinoblastoma treated with chemotherapy prior to enucleation. METHODS: Seventeen enucleated specimens of retinoblastoma from 16 patients were studied. Nine had been treated with chemotherapy alone, and eight had been treated with chemotherapy and other forms of local treatment. Tumour differentiation as well as choroidal and optic nerve invasion were assessed. P-gp immunohistochemical staining was performed and evaluated as negative, low or high. RESULTS: Histopathological assessment of the cases showed that 14 of 17 eyes (82.3%) had viable retinoblastoma cells. Nine retinoblastomas were considered regressed with a well-differentiated component, five regressed retinoblastomas had viable cells with poor differentiation and three retinoblastomas had regressed leaving no viable cells. Sixteen of 17 retinoblastomas were P-gp positive. In the one case with optic nerve invasion and the three cases with massive choroidal invasion, P-gp expression was found in invading retinoblastoma cells. CONCLUSION: Almost all retinoblastomas expressed P-gp. High levels of P-gp expression might play a role in chemotherapy resistance of retinoblastoma or, conversely, chemotherapy might induce P-gp expression. These results might have an impact on management of bilateral retinoblastoma.

Fordyce nodules in a buccal membrane graft to the ocular surface.

PURPOSE: To present a case of epibulbar Fordyce nodules, with a referral diagnosis of primary tumor. METHODS: Case report. RESULTS: A 38-year-old woman was referred for ocular oncology consultation because of a conjunctival lesion in the right eye. She had had a buccal mucous graft to treat recurrent pterygium 18 years earlier. The lesion consisted of multiple small, yellow granules over a pink, thickened mucosa from the 12 to 3 o'clock meridians. Excisional biopsy revealed multiple subepithelial sebaceous glands consistent with Fordyce nodules. CONCLUSIONS: Fordyce nodules are a possible late benign complication of buccal mucous grafts.

Estudo da etiologia genética do ceratocone / Genetics of keratoconus

Introduçäo: para se estudar a teoria genética da etiologia do ceratocone, este trabalho apresenta a avaliaçäo clínica e da topografia corneana dos indivíduos de 12 famílias nas quais o ceratocone estava presente. Pacientes e métodos: 87 familiares de 12 famílias nas quais o ceratocone era encontrado, foram submetidas a avaliaçäo oftalmológica clínica e análise da topografia corneana. Resultados: o diagnóstico de ceratocone foi confirmado em 15 pacientes, 12 (14 por cento) apresentaram ceratocone subclínico forem incluídos, esta incidência aumenta para mais de 33 por cento. Conclusöes: a análise dos heredogramas das famílias sugeriram um padräo de herança autossômico dominante com expressividade variável em 8 famílias. Entretanto a herança multifatorial näo pôde ser excluída. Este estudo acrescenta o fator alérgico como um possível fator ambiental, e ressalta a importância da avaliaçäo das crianças dessas famílias

A model for the natural history of retinoblastoma according to its cellular differentiation

Objetivo: Estudar a correlação entre a diferenciação celular do retinoblastoma, sua origem genética, estadiamento, idade do paciente e demora no tratamento. Métodos: O padrão histopatológico de 282 casos (271 pacientes) com retinoblastoma intra-ocular e extra-ocular enucleados (como primeiro tratamento) foi retrospectivamente revisado. Os casos bem diferenciados tiveram rosetas de Flexner-Wintersteiner e Homer-Wright, os pouco diferenciados somente rosetas de Homer-Wright e os indiferenciados nenhuma roseta. Os hereditários germinais tiveram história familiar ou apresentação bilateral-multifocal. O não hereditários-esporádicos foram unilaterais. Foram realizadas análises multifactoriais com os testes Chi2, G e Kruskal-Wallis. Resultados: Houve 116 (41 por dento) casos germinais, 166 (59 por cento) esporádicos; 184 (65 por cento) intra-oculares, 94 (33 por cento) extra-oculares; 59 (21 por cento) bem diferenciados, 55 (20 por cento) pouco diferenciados, 141 (50 por cento) indiferenciados. Origem, estadiamento e diferenciação celular foram dependentes entre si (p<0,0001). A probabilidade de encontrar rosetas Flexner-Wintersteiner foi maior nos germinais (p<0,0001) e nos intra-oculares (p<0,001). A idade média dos primeiros sinais (8,66 meses, p<0,0001) e da enucleação (14,02 meses, p<0,0001) foi menor nos bem diferenciados que nos indiferenciados (22,66 e 32,81 meses, respectivamente). Os bem diferenciados (5,7 meses) foram tratados antes (p=0,0089) que os indiferenciados (9,87 meses). Antes dos seis meses de idade, 75 por cento dos casos foram bem diferenciados, (p<0,0001). Após os três anos, 75 por cento foram indiferenciados. Conclusão: O padrão bem diferenciado é mais frequente nas crianças mais novas, nos casos hereditários e nos intra-oculares. O indiferenciado predomina nos mais velhos, nos esporádicos e nos extra-oculares. Na evolução do RB aconteceria uma transformação fenotípica tumoral que influenciaria a frequência dos padrões clinico-histopatológico encontrados às diferentes idades.

Retinoblastoma x exame do fundo do olho na criança / Retinoblastoma x fundus examination in children

Em que idade devemos fazer o exame do fundo do olho na criança com vistas a diagnosticar precocemente um retinoblastoma? Para responder essa pergunta säo revisados 244 casos de retinoblastoma atendidos, no Serviço de Oftalmologia do Hospital A.C. Camargo da Fundaçäo Antonio Prudente em Säo Paulo, no período de 1 de janeiro de 1975 a 31 dezembro de 1989. Foi analizada a relaçäo entre a idade dos pacientes, o início do tratamento e o estágio de evoluçäo da doença nesse momento. Verificou-se que 88.6 p/cento dos casos tratados até os 18 meses de idade, apresentavam doença intra-ocular, compatível com a cura pela enucleaçäo simples ou até passíveis de tratamento com conservaçäo do globo ocular. Os autores propöem que se incentive o exame do fundo do olho em toda criança até a idade de 18 meses, como forma de diagnóstico precoce dessa doença

Polidactilia pós-axial e miopia progressiva / Postaxial polydactyly and progressive myopia

O presente trabalho apresenta três casos de associaçäo de miopia progressiva com polidactilia pós-axial, sendo que dois säo irmäos do sexo masculino, filhos de casal näo consanguíneo e o terceiro caso é de uma menina filha de pais consanguíneos. Verificou-se na literatura apenas um relato, de Czeizel e Brooser, em 1986, em que é descrita a mesma associaçäo de defeitos, transmitindo-se em quatro geraçöes de forma autossômica dominante. Acreditamos que nossos pacientes apresentam a mesma síndrome, porém nestas duas famílias existem indícios de que a transmissäo ocorra de forma autossômica recessiva.