Apical bleeding: a rare cause of blindness after orbital decompression / Sangramento apical: uma causa rara de cegueira pós descompressão orbitária

ABSTRACT Orbital decompression is widely performed for the management of proptosis for cosmetic and functional cases of Graves orbitopathy. The main side effects include dry eye, diplopia, and numbness. Blindness after orbital decompression is extremely rare. The mechanisms of vision loss after decompression are not well described in the literature. Considering the devastating effect and rarity of this complication, this study presented two cases of blindness after orbital decompression. In both cases, vision loss was provoked by slight bleeding in the orbital apex.

RESUMO A descompressão orbitária é uma cirurgia amplamente empregada para correção da proptose em casos cosméticos e funcionais da orbitopatia de Graves. Os principais efeitos colaterais induzidos pela descompressão são olho seco, diplopia e parestesias. Amaurose pós descompressão é uma complicação extremamente rara e cujos mecanismos são pouco discutidos na literatura. Considerando o efeito devastador representado pela perda visual e a escassez de relatos dessa complicação, os autores apresentam dois relatos de amaurose após descompressão orbitária. Nos dois casos a perda visual ocorreu devido a sangramento de pequena monta no ápice orbitário.

Total recovery of visual acuity in a pediatric patient with compressive optic neuropathy secondary to sphenoid sinus mucocele / Recuperação total da acuidade visual na neuropatia óptica compressiva secundária à mucocele do seio esfenoidal em paciente pediátrico

ABSTRACT We present an unusual case of a 13-year-old male pediatric patient with a diagnosis of sphenoid sinus mucocele. The patient suffered a progressive loss of visual acuity over three months followed by a total recovery of his visual acuity after surgery. The patient presented at the emergency room complaining of progressive loss of visual acuity in his left eye which decreased to hand motion over the preceding months. Imaging studies revealed a cystic mass, suggestive of sphenoid sinus mucocele, which was causing compressive optic neuropathy and proptosis. The patient was scheduled for a sphenoidectomy and resection of the mass. Three days after surgery, the patient's visual acuity in the left eye was 20/20, indicating complete recovery from his symptoms. We suggest that the excellent outcome in this patient may be attributable to his age. His ongoing physical development might have been the decisive factor in the recovery of his visual acuity following compressive optic neuropathy secondary to sphenoid sinus mucocele. Further research is needed to verify this proposed explanation.

RESUMO Apresentamos um caso incomum de paciente pediátrico com diagnóstico de mucocele de seio esfenoidal, que apresentou perda progressiva da acuidade visual ao longo de três meses, resultando em recuperação total da acuidade visual após a cirurgia. Paciente do sexo masculino, 13 anos, procurou o pronto-socorro, queixando-se de perda progressiva da acuidade visual do olho esquerdo nos últimos três meses. Exames de imagem revelaram uma massa cística sugestiva de mucocele de seio esfenoidal, causando neuropatia óptica compressiva e proptose. O paciente foi agendado para esfenoidectomia e ressecção da massa. Três dias após a cirurgia, a acuidade visual do paciente no olho esquerdo era de 20/20, apresentando recuperação completa dos sintomas. Diante dos resultados de nosso paciente, sugerimos que a idade do paciente pode ser decisiva na recuperação da acuidade visual de uma neuropatia óptica compressiva secundária à mucocele de seio esfenoidal. Mais pesquisas são necessárias para verificação desses dados.

Erdheim-Chester disease with chorioretinal and orbital involvement: a case report / Doença de Erdheim-Chester com envolvimento coriorretiniano e orbitário: relato de caso

ABSTRACT A 42-year-old woman presented with bilateral proptosis, chemosis, leg pain, and vision loss. Orbital, chorioretinal, and multi-organ involvement of Erdheim-Chester disease, a rare non-Langerhans histiocytosis, with a negative BRAF mutation was diagnosed based on clinical, radiological, and pathological findings. Interferon-alpha-2a (IFNα-2a) was started, and her clinical condition improved. However, 4 months later, she had vision loss with a history of IFNα-2a cessation. The same therapy was administered, and her clinical condition improved. The Erdheim-Chester disease is a rare chronic histiocytic proliferative disease that requires a multidisciplinary approach and can be fatal if left untreated because of multisystemic involvements.

RESUMO Uma mulher de 42 anos apresentou proptose bi-lateral, quemose, dor nas pernas e perda de visão. Com base em achados clínicos, radiológicos e patológicos, foi diag-nosticada doença de Erdheim-Chester com acometimento orbitário, coriorretiniano e multiorgânico. Trata-se de uma rara histiocitose não Langerhans negativa para a mutação BRAF. Foi iniciado tratamento com interferon alfa-2a (IFNα-2a) e o quadro clínico melhorou. No entanto, quatro meses depois, a paciente apresentou perda visual após a cessação do IFNα-2a. A mesma terapia foi administrada novamente e sua condição clínica melhorou novamente. A doença de Erdheim-Chester é uma doença proliferativa histiocítica crônica rara que necessita de uma abordagem multidisciplinar e pode ser fatal se não tratada, devido a envolvimentos multissistêmicos.

Compromiso oftálmico en enfermedad relacionada con IgG4 / Ophthalmic involvement in IgG4-related disease

Resumen Introducción: la enfermedad oftálmica relacionada con IgG4 (EOR-IgG4) presenta una frecuencia del 11-59%. Pocos estudios describen las disparidades con los pacientes con ER-IgG4 extraoftálmica (NO EOR-IgG4). Objetivos: describir las características clínicas, imagenológicas, anatomopatológicas, resultados de laboratorio y tratamiento de la EOR-IgG4, y compararlas con las de los pacientes NO EOR-IgG4. Materiales y métodos: se realizó un estudio descriptivo sobre una cohorte de 54 pacientes con ER-IgG4. Se reclutaron 16 pacientes con EOR-IgG4 y 38 con NO EOR-IgG4. Se compararon ambos grupos. Resultados: la EOR-IgG4 predominó en mujeres. El 75% presentó afectación oftálmica bilateral. El antecedente de asma se asoció al grupo NO EOR-IgG4 (p=0,018). Los pacientes con EOR-IgG4 presentaron niveles séricos menores de IgE e IgG total, y la glándula lagrimal fue la estructura más afectada. Predominó el infiltrado linfoplasmocitario y eosinofílico, siendo la fibrosis estoriforme más frecuente que la no estoriforme en el grupo EOR-IgG4. Conclusiones: si bien los resultados fueron similares a lo reportado previamente, en discordancia con otras series, encontramos asociación negativa entre el asma y los niveles de IgG total sérica en los pacientes EOR-IgG4.

Abstract Introduction: IgG4-related ophthalmic disease (IgG4-ROD) presents a frequency of 11-58.8%. Few studies describe the disparities with patients with extraophthalmic IgG4-related disease (NOT IgG4-ROD). Objectives: describe the clinical, imaging, pathological characteristics, laboratory results, and treatment of IgG4-ROD characteristics; and compare them with those of the NOT IgG4-ROD patients. Materials and methods: a descriptive study was carried out on a cohort of 54 patients with ER-IgG4. 16 patients with IgG4-ROD and 38 with NOT IgG4-ROD were recruited. The data was analyzed with the SPSS Statistics 19 software. Results: IgG4-ROD predominated in women. 75% presented bilateral ophthalmic involvement. A history of asthma was associated with the NOT IgG4-ROD group (p=0,018). Patients with IgG4-ROD presented lower serum levels of IgE and total IgG, and the lacrimal gland was the most affected structure. Lymphoplasmacytic and eosinophilic infiltrates predominated, with storiform fibrosis being more frequent than non-storiform in the IgG4-ROD group. Conclusions: although the results were similar to those previously reported, in disagreement with other series, we found a negative association between asthma and serum total IgG levels with EOR-IgG4 patients.

Cavernous sinus thrombosis with bilateral orbital vein involvement and diffuse ischemic retinopathy / Trombose do seio cavernoso com envolvimento bilateral das veias orbitais e retinopatia isquêmica difusa

ABSTRACT A 53-year-old man with a 3-day history of periorbital swelling and vision loss in the left eye was found to have septic cavernous sinus thrombosis with bilateral orbital vein involvement causing congestive orbitopathy. He was treated with an emergent canthotomy and cantholysis, intraocular pressure-lowering drops, antibiotics, anticoagulation, and serial examinations. Optical coherence tomography ultimately revealed diffuse ischemic destruction of both layers of the retina, which suggested occlusion of the ophthalmic artery or the short posterior ciliary arteries and central retinal artery without intracavernous internal carotid artery involvement. The patient remained without light perception in the left eye after treatment.

RESUMO Um homem de 53 anos, com história de 3 dias de edema periorbital e perda de visão no olho esquerdo, apresentou trombose séptica do seio cavernoso com envolvimento bilateral das veias orbitais, causando uma orbitopatia congestiva. O paciente foi tratado com uma cantotomia e cantólise de emergência, colírios para redução da pressão intraocular, antibióticos, anticoagulantes e exames seriados. A tomografia de coerência óptica finalmente demonstrou destruição isquêmica difusa de ambas as camadas da retina, sugerindo uma oclusão da artéria oftálmica ou das artérias ciliares posteriores curtas e da artéria retiniana central, com ausência de envolvimento do segmento intracavernoso da artéria carótida interna. O paciente permaneceu sem percepção luminosa no olho esquerdo.

Síndrome do ápice orbitário, apresentação aguda e implicações tardias: uma série de casos / Orbital apex syndrome, acute presentation and late implications: a series of cases

RESUMO O ápice orbitário é uma região na qual estão contidas estruturas ósseas, vasculares e neurais. Patologias que acometem essa região podem desencadear um conjunto de sinais e sintomas característicos, dando origem a síndrome do ápice orbitário. É uma entidade rara, que consiste em sinais de envolvimento das estruturas nervosas, que atravessam o forame óptico e a fissura orbitária superior, comprometendo os nervos oculomotor, troclear, abducente; a divisão oftálmica do nervo trigêmeo e o nervo óptico. Suas causas incluem afecções neoplásicas, vasculares, traumáticas, infecciosas, inflamatórias e idiopáticas. Muitas vezes, nós nos deparamos com patologias sem tratamento curativo, portanto deve-se atentar para o controle da sintomatologia e a prevenção das possíveis implicações tardias. O objetivo desta série de casos é relatar algumas das causas da síndrome do ápice orbitário e sua apresentação clínica aguda, além de alertar sobre as possíveis implicações crônicas.

ABSTRACT Orbital apex is a region involving bone, vascular and neural structures. Pathologies involving this region may lead to several symptoms and signals and to orbital apex syndrome. It is a rare syndrome that is characterized by signals involving nervous ocular motor nerves through the optic foramen and the superior orbital fissure. This can affect the oculomotor, the third canal and abducens nerves; the ophthalmic division of the fifth cranial nerve and the optic nerve. Its causes include neoplastic, vascular, traumatic, infectious, inflammatory, and idiopathic conditions. We often deal with conditions with no treatment, so it is necessary to control the symptoms and prevent late implications. The purpose of this case series is to report on the causes of orbital apex disease and its potential chronic implications.

Periorbital ecchymosis and subconjunctival hemorrhage due to leech therapy for headache / Equimose periorbital e hemorragia subconjuntival devido à terapia com sanguessugas para dor de cabeça

ABSTRACT A 62-year-old woman was admitted to our clinic with the complaints of periorbital ecchymosis and subconjunctival hemorrhage that are visible, especially on the right eye. We noted that her complaints began the day after she underwent leech therapy on the glabella area for headache. On the glabella, 2 leech bites were observed close to the right side. Examination revealed ecchymosis on the bilateral eyelids and subconjunctival hemorrhage on the inferolateral and medial limbus on the right eye. No treatment was initiated, rather control measures were recommended. The follow-up after 1 month revealed that the patient's complaints had disappeared.(AU)

RESUMO Uma paciente de 62 anos procurou nosso ambulatório com queixas de equimose periorbital e hemorragia subconjuntival, visíveis principalmente no olho direito. Descobrimos que suas queixas começaram no dia seguinte a um tratamento para dor de cabeça com sanguessugas na área da glabela. Na glabela, 2 mordidas de sanguessuga foram encontradas próximas ao lado direito. Durante os exames da paciente, foram detectadas equimoses nas pálpebras bilaterais e hemorragia subconjuntival no limbo ínfero lateral e medial do olho direito. Nenhum tratamento foi iniciado, sendo recomendado apenas controle. No acompanhamento, observou-se que as queixas da paciente desapareceram em cerca de um mês.(AU)

Hematoma subperiosteal orbitário secundário a barotrauma em aviação civil / Orbital subperiosteal hematoma secondary to barotrauma in civil aviation

RESUMO Os diagnósticos diferenciais que compõem as proptoses agudas são, muitas vezes, desafiadores. A anamnese e o exame clínico exigem do oftalmologista atenção especial aos detalhes que permitem diferenciar quadros relativamente benignos e autolimitados de quadros que evoluirão com incapacidades permanentes. Relatamos o caso de uma paciente de 49 anos que, durante viagem de avião, apresentou dor ocular, hematoma periorbitário e proptose do olho esquerdo súbitos. Referia diplopia aguda incapacitante. Exames de tomografia e angiorressonância magnética confirmaram diagnóstico de sinusopatia do seio etmoidal esquerdo e hematoma subperiosteal da órbita esquerda, associado ao barotrauma. Apesar de raro, o diagnóstico de hematoma subperiosteal não traumático deve ser considerado diferencial em relação a proptoses agudas, sendo a anamnese fundamental para essa elucidação diagnóstica.

ABSTRACT Differential diagnoses of acute proptosis are often challenging. History and clinical examination require from ophthalmologists special attention to details, which make it possible to differentiate relatively benign and self-limited conditions from those that will progress to permanent disabilities. We report a 49-year-old female patient who had sudden eye pain, periorbital hematoma and proptosis of the left eye during a commercial flight. She also complained of disabling acute diplopia. Computed tomography and magnetic resonance angiography imaging confirmed the diagnosis of subperiosteal hematoma of the left orbit, associated with left ethmoid sinus disease. Although rare, non-traumatic subperiosteal hematoma should be considered in differential diagnoses of acute proptosis, and history taking is fundamental to elucidate the picture.

A three-dimensional printed photopolymer resin implant for orbital rehabilitation for evisceration / Implante de resina fotocurável para evisceração produzido por impressora tridimensional

ABSTRACT Purpose: To evaluate the biocompatibility of three-dimensional (3D) printed orbital spheres for evisceration. Materials: A total of 10 consecutive patients (eight females and two males; mean age, 46.8 ± 14.2 years) underwent evisceration of blind painful eyes. 3D spherical implants produced by a rapid prototype machine were used to restore orbital volume. The implants were produced from a commercially available photocurable resin (Fullcure®). Systemic toxicity was evaluated by comparing serum biochemical measurements (creatine phosphokinase, aspartate aminotransferase, alanine aminotransferase, albumin, creatinine, urea, alkaline phosphatase, and C-reactive protein) before and at 12 months after surgery. Local toxicity was assessed by the evaluation of signs of socket inflammation at the first postoperative month. Changes in implant size were determined by computed tomography scans at 2 and 12 months after surgery. Results: The postoperative evaluations were uneventful. The biochemical evaluation showed no significant changes after surgery. None of the patients presented signs of orbital implant inflammation, infection, exposure, or extrusion. Computed tomography scan evaluations revealed no changes in implant size. Conclusion: To the best of our knowledge, this is the first phase-1 clinical study to certify the biocompatibility of the Fullcure resin for orbital implants in humans. The 3D printing technology permits fast and accurate production of implants for this purpose.

RESUMO Objetivos: Avaliar a biocompatibilidade das esferas produzidas por impressora tridimensional em evisceração. Pacientes e métodos: Evisceração por olho cego doloroso foi realizada em 10 pacientes consecutivos (8 mulheres, idade média: 46.8 ± 14.2 anos). Os implantes esféricos foram produzidos pelo sistema de prototipagem rápida utilizando dados tridimensionais computadorizados. O material utilizado para produção dos implantes foi a resina fotocurável Fullcure®. A avaliação da toxicidade sistêmica do material foi realizada por meio da dosagem de marcadores bioquímicos (creatina fosfoquinase, aspartato aminotransferase, alanina aminotransferase, albumina, creatinina, ureia, fosfatase alcalina, e proteína C-reactiva) antes da cirurgia e aos 12 meses de pós-operatorio. A avaliação da toxicidade local foi realizada por meio do registro qualitativo dos sinais inflamatórios no lado operado durante o primeiro mês de pós-operatório. O tamanho dos implantes foi medido em tomografias computadorizadas (CT) aos 2 e 12 meses de pós-operatório. Resultados: A avaliação bioquímica mostrou que os marcadores estudados não sofreram alterações significativas após a cirurgia. Nenhum paciente apresentou sinais de inflamação atípica, infecção, exposição ou extrusão. A avaliação tomográfica não demonstrou mudanças nos tamanhos dos implantes. Conclusão: O presente trabalho é o primeiro estudo clínico realizado para atestar a biocompatibilidade dos implantes orbitais de resina fotocurável Fullcure. A produção dos implantes pela técnica de impressão tridimensional, utilizando essa resina, permite a disponibilização rápida e acurada do produto final

CT diagnosis of primary orbital lipid prolapse / 中国医师进修杂志

Objective To investigate the CT findings of primary orbital lipid prolapse. Methods The CT data of 13 patients with primary orbital lipid prolapse in Jiaxing Traditional Chinese Medicine Hospital Affiliated to Zhejiang Chinese Medical University from January 2012 to June 2018 were retrospectively analyzed. Results In all of the 13 cases of primary orbital lipid prolapse, there were 12 cases of bilateral orbital lipid prolapse and 1 case of unilateral orbital lipid prolapse. The CT images showed that the fat density masses in the posterior orbital septum were herniated from the outer quadrant of the orbit. The tumor was crescent shaped and connected with the orbital adipose tissue. The mass and the orbital adipose tissue were natural and homogeneous. Conclusions CT has distinctive imaging findings in the diagnosis of primary orbital lipid prolapse, and it is helpful for the diagnosis and differential diagnosis of this disease.

Bilateral periorbital necrotizing fasciitis: case report / Fasciite necrosante periorbital bilateral: relato de caso

ABSTRACT Necrotizing fasciitis is a severe infection of the subcutaneous tissue characterized by necrosis of the superficial fascia and overlying skin and is usually associated with previous trauma and comorbidities. Periorbital necrotizing fasciitis is rare and commonly causes visual loss and soft tissue defects. A better prognosis relies critically on early diagnosis, prompt medical treatment, and timely surgical intervention. We describe a rare case of periorbital necrotizing fasciitis in the absence of an inciting event. A 55-year-old female patient presented with acute painful swelling and redness of the right upper eyelid that spread to both eyelids bilaterally within 24 h. We swiftly started the patient on intravenous antibiotic therapy, and we surgically debrided the necrotic tissue the following day. We performed two further procedures to improve eyelid closure and appearance. Despite the severe presentation, timely antibiotic therapy and proper surgical interventions led to a successful outcome in this case.

RESUMO Fasciite necrosante é uma infecção grave do tecido subcutâneo, caracterizada pela necrose da fáscia superficial e da pele sobrejacente. Traumas prévios e cormobidades geralmente estão associados à fasciite necrosante. Fasciite necrosante periorbital é rara. Perda visual e defeitos em tecidos moles são as morbidades mais comuns. Diagnóstico precoce, tratamento clínico rápido e intervenção cirúrgica oportuna levam a um melhor prognóstico. Reportamos um caso incomum de fasciite necrosante periorbital bilateral sem eventos desencadeantes. Uma paciente de 50 anos apresentou edema e eritema na pálpebra superior direita, que progrediu em 24 horas para ambas pálpebras bilateralmente. Ela era previamente hígida. A paciente foi submetida a debridamento cirúrgico do tecido necrótico, no mesmo dia. A paciente foi submetida a outras duas cirurgias, o que melhorou o fechamento palpebral e a aparência. Apesar da gravidade da doença, antibioticoterapia e cirurgias oportunas foram cruciais para o desfecho bem sucedido deste caso.

Pseudotumor orbital bilateral recorrente / Recorrent bilateral orbits pseudo-tumor

Resumo O pseudotumor orbitário é uma doença inflamatória idiopática benigna. Os autores apresentam um caso manifestado em adolescente de 12 anos, diagnosticado por meio do exame clínico, laboratorial e radiológico. Houve boa resposta ao tratamento proposto com corticosteroides. O relato é seguido de breve retomada literária acerca do tema.

Abstract The orbital pseudotumor is a benign idiopathic inflammatory disease. The authors present a case manifested in 12 years old boy, diagnosed by clinical, laboratory and radiological examination. There was a good response to treatment with corticosteroids proposed. The report is followed by brief literary resume on the subject.

Clinical analysis of orbital complications due to rhinosinusitis in 28 cases / 中华耳鼻咽喉头颈外科杂志

Objective@#To investigate the clinical characteristics of orbital complications due to rhinosinusitis and to provide the basis for the diagnosis and treatment of this disease.@*Methods@#Retrospectively analyzed the clinical data of 28 patients with orbital complications due to rhinosinusitis who were admitted into the Affiliated Hospital of Qingdao University, Department of Otorhinolaryngology from August 2009 to April 2017. In all 28 cases, there were 1 case with preseptal cellulites, 9 cases with orbital cellulites, 13 cases with subperiosteal orbital abscess and 5 cases with orbital abscess. Among all the patients, 9 were younger than 14. Two patients were in ages from 14 to 17 and 7 patients were at or above 18 years. Absolute medicine therapy was performed on 8 patients and combined therapy of operation and pharmacotherapy on 20 patients.@*Results@#The patients were followed up for 2 to 19 months. Both the general symptoms and ocular symptoms disappeared and nasal ventilation function recovered well.@*Conclusions@#The treatment for orbital complications of rhinosinusitis includes absolute medicine therapy and combined therapy of operation and pharmacotherapy. And the selection of treatment depends on disease stage. Timely diagnosis and reasonable treatment would be beneficial in the recovery of patients.

Detection of IgG4 and autoantibodies in patients with orbital disease of unknown origin / 中华检验医学杂志

Objective To detect the serum IgG4 and autoantibodies levels in patients with orbital disease of unknown reasons,and to investigate their values in patients with orbital disease.Methods A total of 366 patients with orbital disease of unknown reasons recruited in the Department of Ophthalmology,Beijing Tongren Hospital Affiliated to Capital Medical University from October 2013 to October 2016 were retrospectively enrolled as orbital disease group,and 266 patients with autoimmune disease in the same period from the Department of Rheumatology of the hospital were selected as controls.The serum IgG4 was detected by rate scattering method,antinuclear antibody(ANA),anti-double-stranded DNA(dsDNA)antibody as well as anti-extractable nuclear antigen(ENA)antibody were measured by indirect immunofluorescence assay,and anti-neutrophil cytoplasmic antibody(ANCA)was detected by enzyme linked immunosorbent assay,all of which were compared between the orbital disease patients and the controls using chi-square test.Results The positive rate of the serum IgG4 in the patients with orbital disease was 36.1％(132/366),obviously higher than that in the controls(27.1％,72/266),the difference being statistically significant(x2 =5.705,P=0.017).And the positive rate of serum IgG4≥1 350 mg/L(29.0％,106/366)in the patients with orbital disease was higher than that in the controls(21.8％,58/266; x2 =4.107,P=0.043).The positive rate of ANA in the patients with orbital disease was 17.8％(65/366),obviously lower than that in the controls(28.6％,76/266),the difference also being statistically significant(x2 =10.389,P=0.001).The positive rate of anti-ENA antibody in the patients with orbital disease was 4.6％(17/366),also obviously lower that that in the controls(9.0％,24/266),with statistically significant difference as well(x2 =4.866,P=0.027).No anti-dsDNA antibody was detected in the patients with orbital disease.Only three patients with orbital disease(0.8％,3/366)were found ANCA positive,and no statistically significant difference was found in comparison with the controls(3.0％,8/266; x2 =3.127,P=0.077).Conclusions Elevated IgG4 level was commonly seen in the patients with orbital disease,where as autoantibodies were negative in the most of the patients,indicating that IgG4 might correlate with orbital disease,and part of orbital disease may belong to the IgG4-related orbital disease.

Diseases treated in oculoplasty

Most people think that ophthalmology only treat diseases of the eyeball, but there are also many diseases of the accessory organs around the eyeball. Oculoplasty is a field of ophthalmology that deals with diseases of the eyelids, lacrimal system, and orbit. These accessory organs play important roles in protecting the eyes and supporting their function. Sometimes, diseases of these organs cause cosmetic problems, as well as functional problems. In the past, this field was considered rather indifferently and was not well recognized, so the treatment of these diseases was not specialized. However, concomitantly with recent improvements in quality of life and the increased desire of patients for these diseases to be treated, the importance of this field is increasing and many oculoplastic specialists are being trained. In the oculoplastic field, wide range of diseases are treated. In this report, the author provides a brief overview of the most important and common oculoplastic diseases.

Fibroma de Tarso / Tarsal Fibroma

RESUMO O objetivo dos autores é relatar um caso de fibroma de tarso, uma tumoração na reborda orbitária súpero-temporal direita com crescimento lento ao longo de anos. A análise da peça obtida da região tarsal mostrou uma lesão bem delimitada, constituída por deposição de fibras colágenas densas, com proliferação de células fusiformes, sem atipias, com revestimento de células conjuntivais, caracterizando fibroma da placa tarsal.

ABSTRACT The objective of the authors is to report the case of a tarsal fibroma, a tumoration on the superotemporal side of the right orbit with slow growth over the years. Analysis of the anatomical specimen obtained from the tarsal region showed a well-defined lesion, consisting of deposition of dense collagen fibers, with fusiform cells without atypia, with coating conjunctival cells, featuring fibroma of the tarsal plate.

Apresentação incomum e agressiva de melanoma intraorbitário / Unusual and aggressive presentation of intraorbital melanoma

RESUMO O objetivo dos autores é relatar um caso de melanoma intraorbitário de apresentação atípica e agressiva, formando grande massa dolorosa de aspecto eritematoso e inflamatório projetando-se da órbita esquerda com o globo ocular danificado em seu ápice. A análise da peça identificou melanoma maligno com componentes celulares epitelióide, fusocelular e anaplásico.

ABSTRACT The purpose of this study is to report a intraorbital melanoma case with atypical and aggressive presentation, forming a large painful mass with erythematosus and inflammatory aspect protruding from the left orbit with eyeball damaged at its peak. Piece analysis identified malignant melanoma compound of epithelioid, spindle and anaplastic cells.

Isolated primary amyloidosis of the inferior rectus muscle mimicking Graves’ orbitopathy / Amiloidose primária isolada do músculo reto inferior simulando orbitopatia de Graves

ABSTRACT The diagnosis of Graves’ orbitopathy is usually straightforward. However, orbital diseases that mimick some clinical signs of Graves’ orbitopathy may cause diagnostic confusion, particularly when associated to some form of thyroid dysfunction. This report describes the rare occurrence of localized inferior rectus muscle amyloidosis in a patient with autoimmune hypothyroidism, who was misdiagnosed as Graves’ orbitopathy. A 48-year-old man complained of painless progressive proptosis on the left side and intermittent vertical diplopia for 6 months. The diagnosis of Graves’ orbitopathy was entertained after magnetic resonance imaging revealing a markedly enlarged, tendon-sparing inferior rectus enlargement on the left side, and an autoimmune hypothyroidism was disclosed on systemic medical workup. After no clinical improvement with treatment, the patient was referred to an ophthalmologist and further investigation was performed. The presence of calcification in the inferior rectus muscle on computed tomography, associated with the clinical findings led to a diagnostic biopsy, which revealed amyloid deposition. This report emphasizes that a careful evaluation of atypical forms of Graves’ orbitopathy may be crucial and should include, yet with rare occurrence, amyloidosis in its differential diagnosis.

RESUMO O diagnóstico de orbitopatia de Graves usualmente é fácil de ser estabelecido. No entanto, doenças da órbita que simulam alguns sinais clínicos da orbitopatia de Graves podem levar à confusão diagnóstica, particularmente quando associada à alguma forma de disfunção tireoidiana. Relatamos a ocorrência rara de amiloidose localizada no músculo reto inferior em paciente com hipotireoidismo autoimune, que recebeu inicialmente o diagnóstico errôneo de orbitopatia de Graves. Paciente masculino, 48 anos, com queixa de proptose progressiva e indolor do lado esquerdo e diplopia vertical intermitente há 6 meses. O diagnóstico de orbitopatia de Graves foi considerado após a realização de ressonância magnética, que revelou aumento importante do músculo reto inferior esquerdo, sem acometimento do tendão, e uma propedêutica sistêmica detectou hipotireoidismo autoimune. Como não houve melhora com o tratamento clínico, o paciente foi encaminhado a um oftalmologista, que realizou nova investigação. A presença de calcificação no músculo reto inferior na tomografia computadorizada, associada aos achados clínicos, levou a uma biópsia da lesão, que demonstrou a deposição de material amiloide. Este relato enfatiza como uma avaliação minuciosa das formas atípicas de orbitopatia de Graves é essencial e deve incluir a ocorrência, embora rara, de amiloidose no diagnóstico diferencial da orbitopatia de Graves.

Pseudotumor esclerosante de órbita / Sclerosing orbital pseudotumor

RESUMO O pseudotumor esclerosante de órbita é um subtipo raro de pseudotumor inflamatório idiopático de órbita. É mais comum em adultos e apresenta diagnóstico de exclusão. A primeira linha de tratamento são os esteroides. O subtipo esclerosante apresenta resposta moderada aos esteroides devido à predominância de fibrose e colágeno na histologia. Relatamos o caso de um paciente com diagnóstico histológico de pseudotumor esclerosante de órbita que teve boa resposta ao tratamento com corticoide associado à azatioprina.

ABSTRACT Sclerosing orbital pseudotumor is a rare subtype of idiopathic orbital inflammatory pseudotumor. It's more common in adults and presents diagnosis of exclusion. Steroids represent the first option of treatment. The sclerosing orbital pseudotumor subtype shows moderate response to steroids due to the predominance of fibrosis and collagen in its histology. We report on a case of a patient with histologic diagnosis of sclerosing orbital pseudotumor successfully treated with corticosteroid associated with azathioprine.

Progress in the development of common experimental animal models of the eye neoplasm and the orbital diseases / 中华实验眼科杂志

The eye neoplasm and the orbital diseases are a class of serious illness which will cause visual function damage and even life threatening.At present,the etiology and pathogenesis of the most eye neoplasm and orbital diseases are still not clear,which has an adverse effect on the treatment.The medical experimental animal models are the ideal model to simulate human diseases.At the same time,the establishment of related animal models with the eye neoplasm and the orbital diseases is a helpful aid in getting to know the etiology and pathogenesis,providing a basis for studying the etiology and pathogenesis of these diseases.Some progress has been achieved some progress in recent years,however,compared to other diseases,the related animal models of these are still few.The progress in the common experimental animal models of the eye neoplasm and the orbital diseases,the animal models of the thyroid-associated ophthalmopathy (TAO),the orbital adenoid cystic carcinoma,the retinoblastoma (RB),the uveal melanoma,and the idiopathic orbital inflammatory pseudotumor were reviewed in this article.