Morning glory disc anomaly associated with large facial infantile hemangioma as the presenting signs of PHACE syndrome / Anomalia de Morning Glory associada à extenso hemangioma infantil como apresentação da Síndrome PHACE

ABSTRACT Infantile hemangioma, the most common benign tumor in infancy, is usually an isolated condition occurring in many different locations in the body. However, large infantile hemangioma may be associated with other systemic malformations, including central nervous system, cerebrovascular, cardiac, and ophthalmology abnormalities, a condition termed PHACE syndrome. In this paper, we describe a case of PHACE syndrome that was presented with the unique association of a large facial infantile hemangioma and morning glory anomaly.

RESUMO O hemangioma infantil é a causa mais comum de tumor benigno na infância e usualmente é uma condição isolada podendo ocorrer em diferentes regiões do corpo. No entanto, hemangiomas infantil extensos podem ser associados com outras malformações sistêmicas incluindo anomalias no sistema nervoso central, cerebrovasculares, cardíacas e oftalmológicas, uma condição denominada síndrome PHACE. Neste trabalho, descrevemos o caso de um paciente com síndrome PHACE que se apresentou com um extenso hemangioma facial e anomalia de "morning glory".

Complicaciones tardías de la radioterapia ocular / Late complications of the ocular radiotherapy

La radioterapia es uno de los pilares terapéuticos utilizados en la oncología, no exenta de producir daño ocular, lo que constituye un fenómeno bien establecido. La aparición de complicaciones continúa siendo la principal preocupación de la aplicación de la radioterapia, a pesar del empleo de nuevas estrategias que buscan reducir las complicaciones y mejorar el índice terapéutico. Se presenta una paciente femenina de 63 años con diagnóstico de carcinoma epidermoide de la conjuntiva, moderadamente diferenciado G-II, invasor (T3N0M0) en ojo izquierdo, que llevó tratamiento quirúrgico y radioterapia superficial complementaria. Como complicación tardía, la paciente presentó catarata radiógena, que recibió tratamiento quirúrgico, además de aneurismas conjuntivales en el área tratada(AU)

Radiotherapy is one of the therapeutic pillars used in oncology, but ocular damage may occur and this is a well-established phenomenon. The occurrence of complications remains the main concern for the application of radiotherapy, despite the use of new strategies to reduce complications and to improve the therapeutic rate. Here is a 63 years old female patient, who was diagnosed with epidermoid carcinoma of the conjunctiva, moderately differentiated G-II invader (T3N0M0) in the left eye. She was surgically treated and received supplementary superficial radiotherapy As a late complication, the patient presented with radiogenic cataract treated by surgery, in addition to conjunctival aneurysms in the treated area as well(AU)

Solar elastosis in conjunctival squamous cell neoplasm.

Aim: To study the role of solar elastosis in the patient's with Conjunctival Squamous Cell Neoplasia (CSCN). Materials and Methods: Paraffin embedded 30 Conjunctival Squamous Cell Neoplasia tissues were studied for the presence of solar elastosis by Verhoeff's stain. Nineteen Squamous Cell Carcinoma (SCC), 6 Carcinoma In Situ (CIS) and 5 Conjunctival Intraepithelial Neoplasia (CIN) specimens were included in the study. Disease free conjunctiva and pterygium tissues were used as negative and positive control respectively. Results: Solar elastosis was found to be present in 19 of 30 (63.3%) Conjunctival Squamous Cell neoplasia specimens. Conclusion: Our study showed the presence of solar elastosis in conjunctival squamous cell neoplasia. Poor socioeconomic condition is an important factor as most of the elastosis positive patients were farmers.

Primary orbital aspergilloma of the exenterated orbit in an immunocompromized patient.

Aspergilloma is a fungal ball that usually forms in a preformed stationary cavity, mostly in lung and paranasal sinuses. We report a rare case of primary orbital Aspergilloma following exenteration for an invasive ocular surface squamous neoplasia, clinically mimicking a recurrence of the tumor. The fungal ball showed the presence of conidiophores with a globular head and a complete row of uni and biserrate phialides, suggestive of Aspergillus flavus species. The exposure to air in the orbit, possibly promoted the formation of conidiophores, which are normally seen when the organism is located in air cavities.

A Case of Intramuscular Hemangioma Presenting with Large-angle Hypertropia

PURPOSE: To report the case of a patient with large-angle hypertropia of an intramuscular hemangioma of the right superior rectus muscle (SR). METHODS: A 63-year-old man with progressive vertical deviation of the right eye for the past 6 months visited our strabismus department; his condition was not painful. An examination indicated that he had 60PD of right hypertropia at distance and near in primary gaze. Additionally, a significant limitation of his downgaze was noted. The right eye appeared mildly proptotic, and the upper and lower eyelids were slightly edematous. Corrected vision was 20/20 in both eyes. RESULTS: Orbital magnetic resonance imaging (MRI) studies revealed fusiform enlargement of the right superior rectus muscle, with prominent but irregular enhancement following gadolinium administration. Incisional biopsy revealed an intramuscular hemangioma in the superior rectus muscle with cavernous-type vessels. CONCLUSIONS: This case demonstrates that intramuscular hemangioma should be considered in the differential diagnosis of isolated extraocular muscle enlargement and unusual strabismus.

The additive influence of nevus flammeus and the nevus of Ota on congenital glaucoma.

Nevus flammeus and nevus of Ota are commonly encountered dermatological conditions, but both individually, are only occasionally associated with glaucoma. The occurrence of isolated nevus flammeus along with nevus of Ota in the same patient is also extremely rare. We are presenting two unusual cases, both having a nevus of Ota, together with nevus flammeus and severe congenital glaucoma.

Ocular manifestations of xeroderma pigmentosa in Iraq

We reviewed a total of 34 patients with xeroderma pigmentosa. Twelve [35%] had major ocular complications related to malignant tumors. The most common malignant tumors of the eye were squamous cell carcinoma

Orbital myiasis--a case report.

A case of basal cell carcinoma presenting in an unusual manner as orbital myiasis is reported. Orbit was full of maggots. Histopathology examination from the margin of the ulcerative area confirmed the diagnosis of basal cell carcinoma, which was infested secondarily with larvae of flies. Turpentine oil packs were used to remove the maggots.

Gliosis masiva de la retina / Massive retinal gliosis

En un período de cuatro años se revisan 80 casos de leucocoria, encontrándose dos casos anatomopatológicos de gliosis masiva de retina. Se efectúa estudios de microscopía electrónica y estudio inmunohistoquímico. Los hallazgos demostraron que la masa pseudotumoral se debía a proliferación de células fusiformes de citoplasma eosinofílico fibrilar y que el método de la peroxidasa antiperoxidasa confirmó como tejido glial. La microscopía electrónica demostró un patrón similar al presentado por las células de Muller

Evaluación de 47 casos de retinoblastoma (1982-1986) / Evaluation of 47 cases of retinoblastoma (1982-1986)

We have reviewed medical records of 47 patients of retinoblastoma treated between 1982-1986 in our section of retinoblastoma of the Department of Ophthalmology of the Hospital Universitario de Caracas, this section is constituted by Services of ophalmology, pediatrics radiotherapy and heamatology and was founded in 1979 by one of us(A, B). The age most affected was between 2-3 years (49.00%). We did not found difference between sexes. 57.40%had unilateral form and 42.60%bilateral. Two patients (4.25%) had family history of retinoblastoma. The most important symptoms were leucocoria (76.60%) and proptosis (10.60%). Almost half (47.00%) came in advanced state. The general mortality followed in 24 months was 77%

Sindrome de sjögren y enfermedades hematopoyeticas / Sjögren syndrome and hematopoyetic diseases

Se presentan 18 casos de pacientes con trastornos del Sistema Hematopoyético: 15 Linfomas Malignos, 2 Macroglobulinemias de Waldeström y 1 Púrpura Hipergammaglobulinémica de Waldeström estudiados en el Servicio de Oftalmología del Hospital "A. Loayza", quienes tuvieron asociado el Sindrome de Sjögren en el curso de su enfermedad de fondo. Todos los casos tuvieron algún grado de hiposecreción lagrimal: 1 leve, 4 moderados y 13 severas. Se demostró el SS diádico en 10 de los casos. Tres pacientes presentaron solo queratopatía tipo Sjögren y 5 solo hiposecreción lagrimal aislada. La discusión se orienta a reconocer en el SS una entidad que potencialmente puede evolucionar hacia el desarrollo de una enfermedad del Sistema Hematopoyético, principalmente aquellas que involucran la estirpe linfoide. Esta hipótesis aún precisa una demostración más fehaciente, pero queda anticipado por el presente trabajo que la coincidencia de estas entidades existe y que la sospecha de SS en un paciente amerita un plan diagnóstico exhaustivo y un seguimiento de la evolución de la enfermedad a largo plazo

Retinoblastoma: retrato da nossa realidade; uma proposta de trabalho em prevençäo da cegueira / Retinoblastoma: picture of our reality; a work proposal in blindness prevention

Analisa-se o estadiamento do retinoblastoma ao diagnóstico pelo oftalmologista, mostrando que em nosso meio, é alto o percentual de casos avançados. Apontam-se as principais causas que levam ao diagnóstico tardio, com a consequente piora do prognóstico. Propöe-se a divulgaçäo desta patologia, principalmente entre pediatras e oftalmologistas, através de literatura, inclusäo obrigatória do tema nos cursos de graduaçäo médica e especializaçäo em oftalmologia e pediatria, e, alerta-se ainda para a necessidade da orientaçäo familiar em se tratando de patologia hereditária