Blefaroespasmo essencial: revisão da literatura / Essential blepharospasm: literature review

O blefaroespasmo essencial é uma distonia focal caracterizada por contrações involuntárias, espasmódicas e bilaterais dos músculos orbicular da pálpebra, corrugador, prócerus e depressor do supercílio. Trata-se de uma doença rara, cujo diagnóstico é frequentemente tardio, o que permite a progressão dos sintomas até quadros de cegueira funcional. Nesse estudo, por meio de revisão da literatura, descreve-se as principais características da doença, incluindo os seguintes aspectos: histórico, epidemiologia, etiologia, quadro clí­nico, diagnóstico diferencial e tratamento.

Essential blepharospasm is a focal distonia characterized by involuntary, spasmodic, bilateral contractions of eyelid protractors. This is a rare disease, which diagnosis is frequently late and symptoms may progress until functional blindness. In this article, we perform a review and describe the most important features of the disease, including historical aspects, epidemiology, etiology, clinical findings, differential diagnosis and treatment.

[Essential blepharospasm: literature review]. / Blefaroespasmo essencial: revisão da literatura.

Essential blepharospasm is a focal distonia characterized by involuntary, spasmodic, bilateral contractions of eyelid protractors. This is a rare disease, which diagnosis is frequently late and symptoms may progress until functional blindness. In this article, we perform a review and describe the most important features of the disease, including historical aspects, epidemiology, etiology, clinical findings, differential diagnosis and treatment.

Treatment of eyelid xanthelasma with 70% trichloroacetic acid.

PURPOSE: To analyze the results of treatment of eyelid xanthelasma (EX) with 70% trichloroacetic acid (TCA) with regard to efficacy, cosmetic appearance, patient satisfaction, and recurrence rate. METHODS: Twenty-four patients with EX of up to one third of the affected palpebral area were treated with 70% TCA. All patients were photographed at the start of treatment and 3 months after the last application. The recurrence rate was analyzed at the end of 6 months of treatment. RESULTS: The average number of applications of 70% TCA until the xanthelasma resolved was 1.5. Eleven patients (45.8%) had an excellent result, 8 (33.3%) a good result, and 5 (20.8%) had a satisfactory result. The most common complication was hypopigmentation (33.3%). All patients reported an improved final cosmetic result. Six patients (25%) treated with 70% TCA had a recurrence 6 months after treatment. CONCLUSIONS: The results of prospective treatment of 24 patients with EX with 70% TCA indicated that this is a simple and effective method, achieves a satisfactory cosmetic result, has an acceptable recurrence rate, and high patient satisfaction.

Browlifting as an alternative procedure for apraxia of eyelid opening.

Essential blepharospasm is an idiopathic disorder that consists of spontaneous, spasmodic, and involuntary eyelid closure in the absence of ocular disease. Some patients develop an inability to open their eyelids in the absence of orbicularis spasms. These patients have essential blepharospasm combined with apraxia of eyelid opening. Botulinum toxin injections are the treatment of choice for blepharospasm but results may be insufficient, especially in cases associated with apraxia. Apraxia can be treated surgically by levator aponeurosis advancement, frontalis suspension, and upper myectomy. The authors report the first browlift using polypropylene suture to manage eyelid apraxia associated with blepharospasm as an alternative and minimally invasive procedure.

Patients with essential blepharospasm and glaucoma: case reports.

Essential blepharospasm is a facial dystonia characterized by spontaneous, spasmodic and involuntary contractions of the eyelid muscles. In advanced cases, blepharospasm patients develop severe eyelid spasms that render them functionally blind, socially reclusive, and unable to work or care for themselves. Oculoplastic surgeons frequently have to deal with patients with blepharospasm. The decrease in quality of life caused by this pathology drives all the attention to the resolution of the spasms. However, other conditions may be associated with them and must be kept in mind during the ophthalmological examination. Four patients with essential blepharospasm were diagnosed as glaucomatous during their follow-up at the Oculoplastic Service. All of them showed glaucomatous optic neuropathy and corresponding visual field defect and no clinically apparent secondary cause for their glaucoma. Forced eyelid closure may lead to intraocular pressure peaks. These patients with blepharospasm present repetitive and spasmodic eyelid contractions and the intraocular pressure rise observed during eyelid squeezing could be an additional risk factor for glaucomatous damage. Our case series suggest that patients with blepharospasm should be seriously evaluated for glaucoma.

Patients with essential blepharospasm and glaucoma: case reports / Pacientes com glaucoma e blefaroespasmo essencial: relato de casos

Essential blepharospasm is a facial dystonia characterized by spontaneous, spasmodic and involuntary contractions of the eyelid muscles. In advanced cases, blepharospasm patients develop severe eyelid spasms that render them functionally blind, socially reclusive, and unable to work or care for themselves. Oculoplastic surgeons frequently have to deal with patients with blepharospasm. The decrease in quality of life caused by this pathology drives all the attention to the resolution of the spasms. However, other conditions may be associated with them and must be kept in mind during the ophthalmological examination. Four patients with essential blepharospasm were diagnosed as glaucomatous during their follow-up at the Oculoplastic Service. All of them showed glaucomatous optic neuropathy and corresponding visual field defect and no clinically apparent secondary cause for their glaucoma. Forced eyelid closure may lead to intraocular pressure peaks. These patients with blepharospasm present repetitive and spasmodic eyelid contractions and the intraocular pressure rise observed during eyelid squeezing could be an additional risk factor for glaucomatous damage. Our case series suggest that patients with blepharospasm should be seriously evaluated for glaucoma.

Blefaroespasmo essencial é uma distonia facial caracterizada por contrações espontâneas, espasmódicas e involuntárias dos músculos palpebrais, podendo tornar os pacientes funcionalmente cegos. Tais pacientes são geralmente referidos aos médicos oculoplásticos para avaliação e tratamento. Devido à intensidade dos espasmos e ao comprometimento da qualidade de vida, toda a atenção é dirigida à sua resolução e outras condições oculares associadas podem passar despercebidas. Neste estudo, quatro pacientes com blefaroespasmo foram diagnosticados como glaucomatosos durante o seu seguimento no Departamento de Plástica Ocular. As quatro pacientes apresentavam neuropatia óptica glaucomatosa e defeito no campo visual compatível, sem que houvesse nenhuma causa secundária para o glaucoma. O fechamento palpebral forçado causa importante aumento da pressão intra-ocular e estes pacientes com blefaroespasmo, por apresentar contrações espasmódicas e repetitivas das pálpebras, poderiam estar sob risco aumentado de desenvolver glaucoma.

Avaliação da prevalência de gluacoma em pacientes com blefaroespasmo essencial / Prevalence of glaucoma in patients with essential blepharospasm

INTRODUÇÃO: O blefaroespasmo essencial é uma distonia focal caracterizada por contrações involuntárias, espasmódicas e bilaterais dos músculos protratores das pálpebras. O glaucoma é a principal causa de cegueira irreversível em adultos de países desenvolvidos e a elevação da pressão intra-ocular é o maior fator de risco para o desenvolvimento da doença. A contração voluntária forçada das pálpebras pode causar aumentos da pressão intra-ocular de até 90 mmHg. Uma vez que pacientes com blefaroespasmo essencial apresentam contrações palpebrais freqüentes e de forte intensidade, eles poderiam compor um grupo de risco para o desenvolvimento do glaucoma. MÉTODOS: Vinte e oito pacientes com blefaroespasmo essencial e 28 pacientes de grupo controle, formado por indivíduos com doenças palpebrais ou no seu pós-operatório, foram submetidos a um exame oftalmológico completo. No grupo de pacientes com blefaroespasmo, a avaliação foi realizada em 8 a 11 dias após o tratamento com toxina botulínica. Efetuou-se exame de refração e medida de melhor acuidade visual corrigida com tabela de Snellen, biomicroscopia em lâmpada de fenda, tonometria de aplanação, campo visual computadorizado, teste de sobrecarga hídrica e biomicroscopia de fundo para avaliação da papila óptica. RESULTADOS: 1) A prevalência de glaucoma nos pacientes com blefaroespasmo foi significativamente maior do que nos indivíduos do grupo controle, sendo diagnosticada em 14,3% e 3,6% dos casos, respectivamente (p=0,008). 2) A pressão intra-ocular foi significativamente maior nos pacientes com blefaroespasmo (15,80 ± 3,80 mmHg) do que nos indivíduos do grupo controle (13,90 ± 2,75 mmHg) (p= 0,004). 3) O pico da pressão intra-ocular na prova de sobrecarga hídrica foi significativamente maior nos pacientes com blefaroespasmo essencial (18,82 ± 4,47 mmHg) do que nos indivíduos do grupo controle (16,27 ± 2,69 mmHg) (p=0,0421). DISCUSSÃO: Diversos estudos descreveram a influência da compressão palpebral...

INTRODUCTION: Essential blepharospasm is a focal distonia characterised by involuntary, spasmodic, bilateral contractions of eyelid protractors. Glaucoma is the most important cause of irreversible blindness in adults in developed countries and high intraocular pressure is the major risk factor for development of the disease. Voluntary forced eyelid closure can produce an intraocular pressure increase of 90 mmHg. As patients with essential blepharospasm present frequent and strong eyelid contractions, they could be at risk for glaucoma development. METHODS: Twenty eight patients with essential blepharospasm and 28 patients of a control group, with eyelid diseases or in the post-operative period, were submitted to a complete ophthalmic examination. In the group of patiens with blepharospasm, the evaluation was done between 8 to 11 days after botulinum toxin treatment. We performed refractometry and best corrected visual acuity with Snellen chart, slitlamp biomicroscopy, applanation tonometry, automated perimetry, water drinking test and dilated funduscopy to evaluate optic discs. RESULTS: 1) Prevalence of glaucoma in patients with blepharospasm (14,3%) was higher than in the individuals from control group (3,6%) (p=0,008). 2) Intraocular pressure was higher in patients with blepharospasm (15,80 ± 3,80 mmHg) than in the individuals from control group (13,90 ± 2,75 mmHg) (p=0,004). 3) Intraocular pressure peaks in the water drinking test were higher in patients with essential blepharospasm (18,82 ± 4,47 mmHg) than in the individuals from control group (16,27 ± 2,69 mmHg) (p=0,0421). DISCUSSION: Several studies reported the influence of eyelid compression over intraocular pressure. The water drinking test has been considered as an indirect tool to measure outflow facility and it has good correlation with intraocular pressure peaks which are frequently missed in routine examinations. Patients with essential blepharospasm had higher mean intraocular pressures...

Retinal thickness assessed by optical coherence tomography (OCT) in pseudophakic macular edema.

PURPOSE: To evaluate the usefulness of the optical coherence tomography (OCT) retinal map in angiographic pseudophakic macular edema (ACME). METHODS: This is a prospective analysis of a group of 36 pseudophakic eyes at the fifth postoperative week, submitted to optical coherence tomography 2 macular map and fluorescein angiography on the same day and, a control group of twenty-two 20/20 vision eyes with a minimum 6-month postoperative period, submitted to optical coherence tomography 2 macular map only. Exclusion criteria were diabetes, any macular pathology and unreadable optical coherence tomography or angiogram. The angiograms were divided into three groups according to the edema intensity: Group I: level 0 (absence of angiographic pseudophakic macular edema); group II: levels 1 and 2 (incomplete and complete perifoveal angiographic pseudophakic macular edema respectively) and group III: levels 3 and 4 (up to one disc diameter and greater than one disc diameter angiographic pseudophakic macular edema area respectively). The nine zones of the optical coherence tomography macular map were compared between the control and the three subgroups of the study group and, between group I and groups II and III. RESULTS: Among the 36 eyes, 23 (64%) were classified as group I, 10 (28%) as group II and three (8%) as group III. The mean thickness of the fovea zone was 185+/-15 microm for the control group, 189+/-24 microm for group I, 213+/-33 microm for group II and 455+/-38 microm for group III. Significant differences: between group III and the control or group I for all zones (p<0.01) and, between group II and control or group I for the fovea and temporal inner zones (p<0.05). CONCLUSION: The optical coherence tomography retinal map is useful in angiographic pseudophakic macular edema diagnosis.

Retinal thickness assessed by optical coherence tomography (OCT) in pseudophakic macular edema / Espessura retiniana medida pela tomografia de coerência óptica (OCT) no edema macular do pseudofácico

PURPOSE: To evaluate the usefulness of the optical coherence tomography (OCT) retinal map in angiographic pseudophakic macular edema (ACME). METHODS: This is a prospective analysis of a group of 36 pseudophakic eyes at the fifth postoperative week, submitted to optical coherence tomography 2 macular map and fluorescein angiography on the same day and, a control group of twenty-two 20/20 vision eyes with a minimum 6-month postoperative period, submitted to optical coherence tomography 2 macular map only. Exclusion criteria were diabetes, any macular pathology and unreadable optical coherence tomography or angiogram. The angiograms were divided into three groups according to the edema intensity: Group I: level 0 (absence of angiographic pseudophakic macular edema); group II: levels 1 and 2 (incomplete and complete perifoveal angiographic pseudophakic macular edema respectively) and group III: levels 3 and 4 (up to one disc diameter and greater than one disc diameter angiographic pseudophakic macular edema area respectively). The nine zones of the optical coherence tomography macular map were compared between the control and the three subgroups of the study group and, between group I and groups II and III. RESULTS: Among the 36 eyes, 23 (64 percent) were classified as group I, 10 (28 percent) as group II and three (8 percent) as group III. The mean thickness of the fovea zone was 185±15 µm for the control group, 189±24 µm for group I, 213±33 µm for group II and 455±38 µm for group III. Significant differences: between group III and the control or group I for all zones (p<0.01) and, between group II and control or group I for the fovea and temporal inner zones (p<0.05). CONCLUSION: The optical coherence tomography retinal map is useful in angiographic pseudophakic macular edema diagnosis.

OBJETIVO: Medir a espessura retiniana pela tomografia de coerência óptica (OCT) no edema macular angiográfico do pseudofácico (ACME). MÉTODOS: Trabalho prospectivo composto de um grupo de estudo com 36 olhos pseudofácicos, entre a 4ª e a 6ª semana de pós-operatório, submetidos ao mapa macular com a tomografia de coerência óptica 2 e à angiofluoresceinografia no mesmo dia e, um grupo controle com 22 olhos pseudofácicos com visão igual a 20/20 e no mínimo 6 meses de pós-operatório, submetidos apenas ao mapa macular com o tomografia de coerência óptica 2. Os critérios de exclusão foram diabetes e maculopatias e exames de qualidade insuficiente para análise. Os angiogramas foram divididos em 3 grupos segundo a intensidade do edema: Grupo I: nível 0 (ausência de edema macular angiográfico do pseudofácico); grupo II: níveis 1 e 2 (edema macular angiográfico do pseudofácico perifoveal incompleto e completo) e grupo III: níveis 3 e 4 (edema macular angiográfico do pseudofácico até um e maior que um diâmetro papilar de área). As nove zonas do mapa macular da tomografia de coerência óptica foram comparadas entre o controle os três subgrupos do grupo de estudo e, entre o grupo I e os grupos II e III. RESULTADOS: Dos 36 olhos do grupo de estudo, 23 (64 por cento) foram classificados como grupo I, 10 (28 por cento) como grupo II e 3 (8 por cento) como grupo III. A espessura para a zona fóvea foi de 185±15 µm no grupo controle, de 189±24 µm no grupo I, de 213±33 µm no grupo II e de 455± 38 µm no grupo III. Diferenças significativas para esta região foram observadas entre o grupo controle ou grupo I e o grupo III para todas as zonas (p<0,01) e, entre o grupo I e o grupo II para a zona fóvea e a temporal interna (p<0,05). CONCLUSÃO: O programa de mapa macular da tomografia de coerência óptica 2 é útil para o diagnóstico de edema macular angiográfico do pseudofácico.

[Congenital anomalies of the optic disc associated with moyamoya disease: case report]. / Anomalias congênitas do disco optico associadas a doença de moyamoya; relato de caso.

Congenital anomalies of the optic disc can be associated with intracranial vascular abnormalities. We report a 9-year old boy with morning glory disc anomaly in one eye and optic disc and infrapapilar choroidal coloboma in the other. His past medical history was remarkable for a transient ischemic attack. A magnetic resonance angiogram of the brain was consistent with moyamoya disease. This case illustrates the important association between optic disc abnormalities and moyamoya disease. Funduscopic examination of suspicious cases can be helpful in establishing the clinical diagnosis. Early identification of the intracranial vascular anomalies may be of help in preventing severe neurological complications.

Anomalias congênitas do disco óptico associadas à doença de Moyamoya: relato de caso / Congenital anomalies of the optic disc associated with Moyamoya disease: case report

As anomalias congênitas do disco óptico podem estar associadas a anormalidades vasculares intracranianas. Relatamos o caso de um paciente de 9 anos com anomalia do disco óptico tipo morning glory em um olho e coloboma do disco óptico e coróide infrapapilar no outro. O paciente apresentava história pregressa de ataque isquêmico transitório e angioressonância cerebral consistente com doença de moyamoya. O presente relato ilustra a importante associação das anomalias congênitas do disco óptico com a doença de moyamoya. O exame fundoscópico de pacientes suspeitos pode trazer subsídios ao diagnóstico clínico, abrindo a possibilidade do reconhecimento precoce das anormalidades vasculares intracranianas encontradas no moyamoya, aumentando a perspectiva de eventual intervenção profilática frente às potenciais complicações.

Eyelid merkel cell carcinoma: report of three cases.

PURPOSE: To present three cases of Merkel cell carcinoma, a primary cutaneous neuroendocrine tumor that arises from Merkel cells, and to describe their clinical features, diagnosis, and management. METHODS: Case series. RESULTS: In only one case, the disease was clinically recognized. In the others, the diagnosis was confirmed by histology, and chalazion was initially suspected in one of them. Surgery was performed in all patients, and there was no evidence of metastatic disease. CONCLUSIONS: The rarity of eyelid Merkel cell carcinoma and the fact that it can simulate benign entities frequently lead to difficulties in diagnosis and a delay in the establishment of suitable therapy.

Ivermectina no tratamento de miíase orbitária: relato de caso / Ivermectine in the treatment of orbital myiasis: case report

OBJETIVO: Relatar um caso de miíase associada a carcinoma basocelular de órbita, tratada com ivermectina previamente ao ato cirúrgico. MÉTODOS: Apresentamos um caso e enfatizamos a eficácia da ivermectina no tratamento da miíase como terapêutica alternativa. RESULTADO: Após o tratamento com ivermectina via oral em dose única, observou-se a resoluçäo completa do quadro de miíase num período de 48 horas. CONCLUSÄO: A ivermectina pode ser utilizada com eficácia no tratamento da miíase orbitária, tornado desnecessária a remoçäo mecânica das larvas

Mediastinite após transplante cardíaco / Mediastinitis after cardiac transplantation

OBJECTIVE: Assessment of incidence and behavior of mediastinitis after cardiac transplantation. METHODS: From 1985 to 1999, 214 cardiac transplantations were performed, 12 (5.6 per cent) of the transplanted patients developed confirmed mediastinitis. Patient's ages ranged from 42 to 66 years (mean of 52.3 + or - 10.0 years) and 10 (83.3 per cent) patients were males. Seven (58.3 per cent) patients showed sternal stability on palpation, 4 (33.3 per cent) patients had pleural empyema, and 2 (16.7 per cent) patients did not show purulent secretion draining through the wound. RESULTS: Staphylococcus aureus was the infectious agent identified in the wound secretion or in the mediastinum, or both, in 8 (66.7 per cent) patients. Staphylococcus epidermidis was identified in 2 (16.7 per cent) patients, Enterococcus faecalis in 1 (8.3 per cent) patient, and the cause of mediastinitis could not be determined in 1 (8.3 per cent) patient. Surgical treatment was performed on an emergency basis, and the extension of the débridement varied with local conditions. In 2 (16.7 per cent) patients, we chose to leave the surgical wound open and performed daily dressings with granulated sugar. Total sternal resection was performed in only 1 (8.3 per cent) patient. Out of this series, 5 (41.7 per cent) patients died, and the causes of death were related to the infection. Autopsy revealed persistence of mediastinitis in 1 (8.3 per ent) patient. CONCLUSION: Promptness in diagnosing mediastinitis and precocious surgical drainage have changed the natural evolution of this disease. Nevertheless, observance of the basic precepts of prophylaxis of infection is still the best way to treat mediastinitis.

Experiência do servico de quimioterapia da Faculdade de Medicina da Fundaçäo ABC com pacientes portadoras de neoplasias mamárias / Preliminary experience of the medical oncology service of ABC foundation school of medicine with breast cancer patients

Durante o período de agosto de 1996 a maio de 1998, atendemos o total de 91 pacientes portadores de neoplasias mamárias referidas ao nosso serviço. A idade média foi de 53,9 anos, sendo que 57 destas mulheres tinham idade inferior a 55 anos. A distribuiçäo percentual dos estágios clínicos das pacientes foi:I-1,1, II-17,6, IIIa-14,3, IIIb-24,2 e IV=42,9. Aproximadamente 90 das pacientes foram tratadas com quimioterapia, 67,3 com cirurgia e 53,8 com radioterapia. Observou-se curiosamente, uma correlaçäo significativa entre o estágio clínico e a idade da paciente(p=0,0093). Pacientes mais idosas tinham estágios mais avançados da doença. Conclui-se que a populaçäo das pacientes por nós assistidas durante este período é jovem e apresenta casos avançados da doença. Portanto, medidas preventivas como auto-exame, consultas periódicas e mamografia devem ser enfática e urgentemente preconizadas para esta populaçäo

Quimioterapia em tumores de cabeça e pescoço / Chemotherapy for head and neck tumors

A cirurgia e a radioterapia säo modalidades terapêuticas eficazes no tratamento de carcinoma de cabeça e pescoço em estados precoces. Neste caso, pode se obter uma taxa de cura variando entre 50 a 100. Entretanto, em estados avançados o prognóstico é reservado e a quimioterapia surge como um alternativa que pode contribuir para o processo de cura da doença, mais especificamente quando combinada à radioterapia e/ou cirurgia(quimioterapia neoadjuvante), ou quando aplicada concomitantemente à radioterapia(quimioradioterapia). Nestes casos, deve-se avaliar qual das duas modalidades pode trazer melhor benefício, sem nos esquecermos da preservaçäo do órgäo. Em estágios avançados ou na doença recidivante, a quimioterapia é empregada somente como forma paliativa de tratemento, na tentativa de melhorar a disfagia e a dor ou de preservar o órgäo acometido. As drogas quimioterápicas que demonstram eficácia comprovada no tratamento dos tumores de cabeça e pescoço säo: Methotrexate, 5-fluorouracil, Cisplatina, Carboplatina e Bleomicina. Novas drogas estäo sendo estudadas, como a Vinorrelbina e os taxóides. A quimioterapia representa uma adiçäo importante aos algoritmos atuais de tratamento multidisciplinar dos tumores de cabeça e pescoço, seja pela possibilidade de cura ou pela melhora na qualidade de vida dos pacientes, já que pode potencialmente melhorar os resultados locais e até mesmo, em alguns casos, permitir a preservaçäo do órgäo acometido