Características de orbitopatía asociada al tiroides y tasa de requerimiento de cirugía / Thyroid-associated orbitopathy characteristics and surgery requirement rate

RESUMO Objetivo: Conheça as características demográficas e clínicas da Órbita Associada da Tiroide (OAT), bem como a taxa de exigência da cirurgia orbital em pacientes do Centro Médico Nacional do Oeste. Métodos. Estudo observacional, transversal, descritivo e retrospetivo realizado analisando os registos de pacientes diagnosticados com OAT tratados num centro de cuidados de terceiro nível de janeiro de 2005 a julho de 2016. Os resultados. Um total de 236 órbitas de 118 pacientes foram avaliados, com uma idade média de 47,3 (13,2 anos, 74,6% eram do sexo feminino e 25,4% masculinos. 4,2% dos doentes foram tratados com hipotiroidismo, 94,1% com hipertireoidismo e 1,7% com goiter tóxico difuso. 44,9% dos doentes estudados com restrição de movimento ocular,10,2% com queratopatia de exposição e 51,7% com hipertensão intraocular. 34,7% dos doentes avaliados no serviço necessitaram de descompressão orbital, 16,1% de cirurgia palpebral e 8,5% de correção do hatrabisma. Na gestão conservadora destes doentes, 48,3% exigiam o uso de lubrificantes tópicos dos olhos, enquanto 52,5% dos pacientes necessitavam do uso de hipotensivos oculares em número variável. As conclusões. A OAT foi associada principalmente ao hipertiroidismo, sendo mais comum em pacientes do sexo feminino entre os 40 e os 59 anos; mais de 50% dos pacientes necessitaram do uso de hipotensivos oculares. Da mesma forma, a gestão cirúrgica foi realizada em mais de 50% dos pacientes, sendo a descompressão orbital a intervenção mais frequente.

ABSTRACT Objective. To know the demographic and clinical characteristics of Thyroid Associated Orbitopathy (TAO), as well as the requirement rate of orbital surgery in patients of the Orbit Service in the National Medical Center of the West, IMSS. Methods. Observational, cross-cutting, descriptive and retrospective study carried out analyzing the records of patients diagnosed with TAO and treated at a third-level care center from January 2005 to July 2016. Results. A total of 236 orbits of 118 patients were valued, with an average age of 47.3 ± 13.2 years, 74.6% were female and 25.4% male. 4.2% of patients were treated with hypothyroidism, 94.1% with hyperthyroidism and 1.7% with diffuse toxic goiter. 44.9% of patients studied had eye movement restriction,10.2% exposure keratopathy and 51.7% intraocular hypertension. 34.7% of patients valued in the service required orbital decompression, 16.1% palpebral surgery and 8.5% strabism correction. In the conservative management of these patients 48.3% required the use of topical eye lubricants, while 52.5% required the use of eye hypotensives in variable numbers. Conclusions. TAO was mainly associated with hyperthyroidism, being more common in female patients between the age of 40 and 59; more than 50% of patients required the use of eye hypotensives. Likewise, surgical management was performed in more than 50% of patients, with orbital decompression being the most frequent intervention.

Pérdida de agudeza visual progresiva en paciente con proptosis. Caso clínico y revisión de la literatura / Progressive visual impairment in a patient with proptosis. Case report and review of the literature.

Introducción: a raíz del siguiente reporte de caso clínico se pretende repensar el diagnóstico diferencial de los tumores orbitales y revisar la literatura existente al respecto. Caso: paciente de 54 años, fumadora, acude a nuestro centro por una pérdida de agudeza visual progresiva de dos años de evolución en el ojo derecho, que se acompañaba de proptosis. Las pruebas de imagen basadas en resonancia magnética y tomografía por emisión de positrones ­ tomografía computarizada (PET-TC) realizadas describían una lesión intraconal derecha de morfología indefinida, que rodeaba el nervio óptico. El estudio inmunohistoquímico y molecular anatomopatológico confirmó la sospecha de síndrome linfoproliferativo extranodal de bajo grado. Discusión: el manejo endoscópico de estas lesiones puede resultar en una menor comorbilidad en comparación con el abordaje externo tradicional. El papel de la cirugía radica en la obtención de una muestra de la lesión que permita un correcto diagnóstico. Conclusiones: el abordaje multidisciplinar con oftalmólogos, hematólogos y expertos en radioterapia permite obtener buenos resultados quirúrgicos y clínicos en la inmensa mayoría de casos.

Introduction: as result of the following clinical case report, we intend to review the differential diagnosis of orbital tumors and review the existing literature in this regard. Case report: a 54-year-old smoking patient, consulted to our department due to a progressive visual impairment over the last two years in her right eye. She presented proptosis in her clinical examination. Imaging studies based on MRI and PET-CT described a right intraconal lesion with an undefined morphology surrounding the optic nerve. Orbital tumors differential diagnosis is delicate. Nevertheless, Non-Hodgkin lymphomas followed by metastasis are the two most common found in this location. The immunohistochemistry and molecular studies, confirmed the suspected diagnosis of extranodal low-grade lymphoproliferative syndrome. Discussion: endoscopic management of these lesions may result in a lower comorbidity compared to traditional external approaches. Role of surgery lays in obtainment of a quality sample which allows a proper diagnosis. Conclusions: multidisciplinary approach with ophthalmologists, hematologists and radiotherapy experts enhance good surgical and clinical results in the vast majority of cases.

Técnica endonasal para cirurgia de descompressão orbitária em paciente com exoftalmopatia de Graves / Endonasal technique for orbital decompression surgery in a patient with Graves' exophthalmopathy

Resumo Paciente do sexo feminino, 29 anos, ex-tabagista, diagnosticada em setembro de 2012 com doença de Graves e apresentação rápida de exoftalmia bilateral. Na avaliação oftalmológica, apresentava motilidade preservada, proptose e bolsa de gordura superior em AO com retração de PPSS e PPII e exoftalmetria em OD de 26 mm,e em OE de 24 mm. Em maio de 2014,fez o mapeamento da retina que evidenciou cicatrizes de coriorretinite em ambos os olhos e campimetria computadorizada, apresentando degrau nasal em OD, contração superior, depressão centro-inferior. Em junho de 2016, realizou cirurgia de descompressão orbitária de paredes medial e inferior bilateral por via endoscópica com uso de endoscópio nasal Karl Storz, em 30 graus de óptica. A abordagem cirúrgica da oftalmopatia de Graves deve ser empregada na fase cicatricial exceto nos casos com risco de perda da visão. Antes realizada por acesso externo, atualmente a descompressão orbitária pode ser realizada via endoscópica, com mínima invasividade e permite a remoção da parede inferior e medial sem necessidade de incisões externas. É um procedimento seguro para o tratamento da orbitopatia distireoidiana associada a menor morbidade, no qual se evita lesões ao ducto nasolacrimal, nasofrontal ou ao infraorbital e se possibilita redução da proptose entre 3 a 4 mm. Os benefícios da descompressão estão relacionados com a melhora da acuidade visual, além do resultado estético. A continuidade do tratamento cirúrgico será realizada por meio de correção de retração palpebral seguida de blefaroplastia.

Abstract Female, 29, former smoker, diagnosed in September 2012 with Graves' disease and rapid presentation of bilateral exophthalmos. In the ophthalmologic evaluation, it presented preserved motility, proptosis and upper fat sac in OA with retraction of PPSS and PPII and exophthalmetry in OD of 26 mm, and in OE of 24 mm. In May 2014, he performed the mapping of the retina that showed scars of chorioretinitis in both eyes and computerized campimetry, presenting a nasal step in OD, superior contraction, central-inferior depression. In June 2016, he underwent orbital decompression surgery of the medial and inferior bilateral walls by endoscopic approach using the Karl Storz nasal endoscope at 30 degrees of optics. The surgical approach of Graves' ophthalmopathy should be used in the cicatricial phase except in cases with risk of loss of vision. Before performed by external access, orbital decompression can now be performed endoscopically, with minimal invasiveness and allows the removal of the inferior wall and Without external incisions. It is a safe procedure for the treatment of dysthyroidal orbitopathy, associated with lower morbidity, in which lesions are avoided in the nasolacrimal, nasofrontal, or infraorbital ducts and it is possible to reduce proptosis between 3 and 4 mm. The benefits of decompression are related to Improvement of visual acuity, besides the aesthetic result. The continuation of the surgical treatment will be performed by correction of palpebral retraction followed by blepharoplasty.

Optic Nerve Glioma with Unilateral Proptosis: An Interesting Case Report.

Optic nerve is most important cranial nerve responsible for visual functioning. Optic nerve involvement leading to blindness can be seen in various diseases, which cause swelling, inflammation, ischemia of the optic nerve. The optic nerve glioma (ONG) comprises 5% of all pediatric intracranial tumor and are a leading cause of unilateral proptosis. ONGs can be associated with neurofibromatosis and are more common than meningiomas. 20-30% of ONGs become symptomatic before the age of 10 years. Visual evoked potential testing is helpful in detecting asymptomatic gliomas. Early detection and prompt management can prevent blindness from gliomas. Treatment of ONG should be tailored to the individual patient. Our patient also showed the presence of unilateral ONG with proptosis. Magnetic resonance imaging was diagnostic. Partial optic atrophy was also present.

Langerhans Cell Histiocytosis Presenting with Hypopigmented Macules.

Langerhans cell histiocytosis (LCH) is a complex disease characterized by proliferation of the Langerhans cells. The clinical course is variable and ranges from a solitary lytic bone lesion or skin lesion with complete remission to a multisystem disorder with possible lethal outcome. The diagnosis is suspected by clinical signs and symptoms and radiological features commonly in craniofacial bones and skin lesions. Diagnosis is confirmed by biopsy and immunohistochemical studies. We present case of a 8 year old child presenting with proptosis, diabetes insipidus and hypopigmented macules on chest and back showing bilateral distribution which is a rare presentation. Skin biopsy revealed the diagnosis of Langerhans cell histiocytosis.

Clinical relevance of "bulging eyes" for the differential diagnosis of spinocerebellar ataxias / Relevância clínica do "bulging eyes" para o diagnóstico diferencial das ataxias espinocerebelares

Objective To investigate the relevance of the clinical finding of bulging eyes (BE) in a large Brazilian cohort of spinocerebellar ataxias (SCA), to assess its importance in clinical differential diagnosis among SCA. Methods Three hundred sixty-nine patients from 168 Brazilian families with SCA were assessed with neurological examination and molecular genetic testing. BE was characterized by the presence of eyelid retraction. Genetically ascertained SCA3 was detected in 167 patients, SCA10 in 68 patients, SCA2 in 20, SCA1 in 9, SCA7 in 6, and SCA6 in 3 patients. Results BE was detected in 123 patients with SCA (33.3%), namely 109 of the 167 SCA3 patients (65.3%) and in 5 of the others SCA patients (1 SCA10 patient, 2 SCA1 patients and 2 SCA2 patients). Conclusion BE was detected in the majority of patients with SCA3 (65.3%) and could be used with a clinical tool for the differential diagnosis of SCA. .

Objetivo Investigar a relevância do achado clínico de bulging eyes (BE) em uma grande amostra brasileira de pacientes com ataxias espinocerebelares (AEC), para avaliar sua importância no diagnóstico diferencial entre as AEC. Métodos Foram avaliados 369 pacientes de 168 famílias brasileiras com AEC através de exame neurológico e testes de genética molecular. BE foi caracterizado pela presença de retração palpebral. AEC3 foi determinada geneticamente em 167 pacientes, AEC10 em 68 pacientes, AEC2 em 20, AEC1 em 9, AEC7 em 6 e AEC6 foi encontrada em 3 pacientes. Resultados BE foi detectado em 123 pacientes com AEC (33,3%), correspondendo a 109 dos 167 pacientes com AEC3 (65,3%) e 5 pacientes com outras AEC (1 paciente com AEC10, 2 AEC1 e 2 pacientes com AEC2). Conclusão BE foi detectado na maioria dos pacientes com AEC3 (65,3%) e poderia ser usado com uma ferramenta clínica para o diagnóstico diferencial das AEC. .

Spontaneous globe luxation associated with chronic obstructive pulmonary disease.

Spontaneous globe luxation is a rarely reported condition which can lead to complications like optic neuropathy. Common causes are thyroid eye disease, shallow orbit and floppy eyelid syndrome. We report a case of spontaneous globe luxation with the onset and severity associated with chronic obstructive pulmonary disease (COPD). To our knowledge, this is the first case of spontaneous globe luxation associated with COPD.

Thyroid associated orbitopathy.

Thyroid associated orbitopathy, also known as Graves’ orbitopathy, is typically a self-limiting autoimmune process associated with dysthyroid states. The clinical presentation may vary from very mild disease to severe irreversible sight-threatening complications. Despite ongoing basic science and clinical research, the pathogenesis and highly effective therapeutic strategies remain elusive. The present article reviews the pathophysiology, clinical presentation, and management of this common, yet poorly understood disease, which remains a challenge to the ophthalmologist.

Peoría paradójica en fístulas carótido-cavernosas durales: cuando el cuerpo se enferma más para curarse / Paradoxical worsening in carotid-cavemous ductal fistulas: when the body get more sick in order to cure

Las fístulas carótido-cavernosas son comunicaciones arteriovenosas patológicas entre la arteria carótida interna intravenosa y el seno venoso-cavernoso que la rodea. Un sistema de flujo y presión elevados irrumpe sobre otro de bajo flujo y velocidad. Ello origina los síntomas y signos. La gran mayoría, son resultantes de traumatismos craneales siendo de alto flujo y alta velocidad-fístulas traumáticas o directas; menos comunes son las llamadas fístulas espontáneas o durales localizadas en la duramadre y alimentadas por ramas menígeas provenientes de las arterías carótidas externa, interna o ambas y de las arterias vertebrales. Estas últimas raras veces tienen implicaciones graves para la vida, pero la pérdida visual constituye el mayor riesgo; no obstante, con elevada frecuencia, ocurre la oclusión espontánea, por lo que se impone la observación hasta que la sintomatología justifique el tratamiento. En el curso evolutivo puede ocurrir la trombosis aguda de la fístula y más propiamente de la totalidad de la vena oftálmica superior la que produce un distintivo cuadro de notable agravamiento de los síntomas que en forma contradictoria ocurre antes de la mejoría y reversión de los síntomas, el síndrome de empeoramiento o peoría paradójica. Para ilustrar el síndrome se presentan los casos clínicos de los pacientes

Caroid-cavernous fistulas are pathological arteriovenous communications between the intracavernous internal carotid artery and the cavernous sinus. A system of high flow and pressure bursts over another system of low flow and slow velocity. This causes the symptoms and signs. The vast majority are related with head trauma being high flow and high speed fistulas: traumatic or direct. Less common are the so-called spontaneous or dural fistulas located in the duramater and fed by meningeal branches from the external, internal, or both carotic arteries and the vertebral arteries. These latest rarely have serious implications for life, but visual loss is the greatest risk; however, with high frequency spontaneously occlusion occurs, and then observation imposes until symptoms justified the treatment. During its evolution acute thrombosis of the fistula itself can occur along the entire length of the ophthalmic vein which produces a distinctive and paradoxical worserning and reversal of symptoms, which is called the syndrome of paradoxical worserning. Clinical cases of two patients are presented to illustrate the syndrome

Valores da exoftalmometria média na população adulta da região do Grande ABC, São Paulo/Brasil / Normal exophthalmometric values in adult population of ABC Region, Sao Paulo/Brazil

OBJETIVO: Estabelecer padrões de normalidade dos valores da exoftalmometria de ambos os olhos e da distância entre os rebordos orbitários laterais (base) em adultos jovens da população do Grande ABC São Paulo/Brasil. MÉTODOS: Realizou-se a exoftalmometria com o exoftalmômetro de Hertel, em 129 pacientes do sexo feminino e 75 do sexo masculino, na faixa etária de 25 a 55 anos. Os pacientes foram separados em três grupos raciais (brancos, negros e pardos). RESULTADOS: A variação da exoftalmometria encontrada na população em estudo foi de 10 a 23mm, média de 15,57 ±2,43mm. No sexo feminino a média foi de 15,42 ±2,40mm e no masculino, 15,83 ±2,47mm. Não se encontrou uma relação estatisticamente significativa entre os sexos feminino e masculino (p=0,25). Na raça branca a média foi de 15,57 ±2,46mm, na negra foi de 16,20 ±2,70mm e na parda foi de 15,02 ±2,01mm. A diferença foi estatisticamente significativa (p=0,008) entre as raças negra e parda. A medida da base variou de 104 a 125mm (média de 113,91 ±4,37mm). A diferença entre homens e mulheres foi estatisticamente significativa (p<0,001). Na raça branca a média da base encontrada foi de 112,18 ±4,02mm, na negra foi de 115,37 ±4,71mm e na parda, 115,18 ±3,66mm. A diferença da média da base foi estatisticamente significativa (p<0,001) entre as raças branca e negra e branca e parda. Nenhum paciente apresentou assimetria entre os dois olhos maior que 2mm. CONCLUSÃO: O padrão de normalidade da exoftalmometria encontrado para a população em estudo foi de 10 a 23mm e a base variou de 104 a 125mm.

PURPOSE: To determine the normal exophthalmometry of both eyes and of the distance between lateral orbital rims (the base measurement) in young adults from ABC Region, São Paulo/Brazil. METHODS: Exophthalmometry, by Hertel's exophthalmometer, was made in 129 female and 75 male subjects, age ranging from 25 to 55 years. The subjects were divided in three groups of races (white, black and mulatto). RESULTS: The values of exophthalmometry ranged from 10 to 23mm, mean 15,57 ±2,43mm. In female the mean was 15,42 ±2,40mm and in male, 15,83 ±2,47mm. There is no statistically significant difference between female or male (p=0,25). In whites subjects the mean was 15,57 ±2,46mm, in black was 16,20 ±2,70mm and in mulatto was 15,02 ±2,01mm. There was a statistically significant difference between blaks and mulattos (p=0,008). The base measurement ranged from 104 to 125mm (mean 113,91 ±4,37mm). There was a statistically significant difference between males and females (p<0,001). In whites the base was 112,18 ±4,02mm, in blacks 115,37 ±4,71mm and in mulattos, 115,18 ±3,66mm. There was a statistically significant difference for the base measurement between white subjects and black ones, and between white subjects and mulattos (p<0,001). No individual had more than 2mm of assimetry between eyes. CONCLUSION: The normal exophthalmometric value in the studied population was 10 to 23mm and the base measurement ranged from 104 to 125mm.

Role of new magnetic resonance imaging modalities in diagnosis of orbital masses: a clinicopathologic correlation

To evaluate the role of diffusion-weighted magnetic resonance imaging [MRI] and proton magnetic resonance spectroscopy [MRS] in the diagnosis of different orbital masses and their advantages over conventional MRI. The study included 20 patients presenting with proptosis. Every patient was subjected to thorough clinical examination, conventional MRI [Tl weighted, T2 weighted, and postcontrast Tl weighted if needed,] diffusion-weighted MRI, and proton MRS. Orbitotomy was performed, the orbital mass was excised, and histopathological examination was performed. Diffusion-weighted MRI could differentiate between benign lesions and malignant tumors in 70% of cases; however, overlap occurred in 30% of cases with benign tumors showing restricted diffusion whereas proton MRS could differentiate between benign and malignant tumors in 90% of cases. Diffusion-weighted MRI and proton MRS can potentially increase the accuracy of diagnosis of orbital masses through in vivo tissue characterization. Magnetic resonance spectroscopy seems to be the more accurate modality

Multiple myeloma presenting as bilateral orbital proptosis.

A 58-year-old-man presented with painful rapidly progressive bilateral proptosis with restricted ocular movements of 15 days duration. There was history of significant weight loss in the recent past. Computed tomography scan of the head and orbit revealed bilateral multiple, well-defined, round, soft tissue masses, isointense with muscles in intraconal and extraconal space. Fine needle aspiration cytology and incision biopsy from the lesion, urine for Bence-Jones proteins and immunofixation clinched the diagnosis of multiple myeloma. Skeletal survey did not reveal any bony involvement.The diagnosis of multiple myeloma should be kept in mind in cases of bilateral proptosis. Bony involvement is not universal in cases of orbital myeloma. Early diagnosis can be established with extensive biochemical and histopathological investigations and timely treatment is life saving for these patients.

Solitary fibrous tumor of the orbit presenting in pregnancy.

A 32-year-old woman, three months pregnant, reported with the complaint of protrusion of the right eye for six months. She gave history of rapid protrusion of eyeball for the last two months along with the history of double vision for the last one month. Computer tomography (CT) scan revealed a well-defined mass lesion in the intraconal space of the right orbit which was excised through a lateral orbitotomy approach. Histological examination and immunohistochemistry revealed a solitary fibrous tumor, which showed a rapid progression in pregnancy.

Proptosis de ojo izquierdo / Left eye proptosis

Se presenta el caso clínico de una paciente joven que acude al Servicio de Oftalmología del Hospital Nacional con leve disminución de la agudeza visual y proptosis del ojo izquierdo, sin antecedentes de traumatismo, cefalea, ni diplopía...(GG)

L'exophtalmie de l'enfant a l'Institut d'ophtalmologie tropicale de l'Afrique (IOTA)

Introduction. L'exophtalmie est un signe peu frequent. Determiner les aspects epidemiologiques; cliniques et les possibilites de prise en charge de l'exophtalmie de l'enfant au Centre hospitalier universitaire Institut d'Ophtalmologie Tropicale de l'Afrique (CHU-IOTA) etait l'objectif assigne a cette etude. Patients etmethode. Cette etude prospective et descriptive s'est deroulee de juillet 2006 a juin 2007 au CHU-IOTA; avec la collaboration de l'unite d'hematologie et d'oncologie pediatrique du CHU Gabriel Toure. Tous les patients ages de 0 a 15 ans qui presentaient une exophtalmie uni ou bilaterale; mesurable ou non etaient inclus.Un examen clinique complet etait realise et des examens complementaires appropries demandes. Toutes les informations etaient recueillies sur un questionnaire. Resultats. L'etude a retenu 46 yeux de 38 enfants : 15 OD; 15 OG et 8 bilateraux. L'age variait de 2 jours a 15 ans et sa moyenne etait de 9;6 ans. Le sex-ratio etait de 1;9 garcons pour 1 fille. La frequence de l'exophtalmie retrouvee etait de 0;3. L'exophtalmie constituait le motif de consultation dans 68;4des cas; soit 26 enfants. Notre classification des groupes etiologiques etait faite de 36;9de pathologies inflammatoires et/ou infectieuses (14 cas); de 31;6de pathologies tumorales (12 cas); de 13;1de traumatismes (5 cas); et de 18;4de causes indeterminees (7 cas). L'exophtalmie avait subi une regression millimetrique au Hertel dans 23;9des cas soit 11 yeux; pendant que 4 yeux malades avaient gagnes des lignes d'acuite visuelle; soit 8;7. Discussion. La frequence de 0;3de l'exophtalmie dans un centre de troisieme reference est suffisante pour alerter les autorites sanitaires quand on sait que cette pathologie est peu frequente. La classification est comparable a celle de Crawford evoquee par Desjardins. Les cellulites (50) et les retinoblastomes (33;3) etaient les deux etiologies les plus frequentes de l'exophtalmie. Conclusion. L'exophtalmie est un risque de perte de la fonction visuelle et meme vitale qu'il faut savoir prendre en charge a temps chez l'enfant

Carotid cavernous sinus fistula presenting with pulsating exophthalmos and secondary glaucoma.

Carotid cavernous sinus fistula is abnormal communications between the carotid arterial system and the venous cavernous sinus. Carotid cavernous sinus fistula can develop either because of trauma or spontaneous causes. Spontaneous carotid cavernous sinus fistula is often associated with a pre-existing aneurysm in the intracavernous portion of internal carotid artery. However, these fistulas may be congenital arteriovenous connections that open spontaneously in the settings of collagen vascular disease, atherosclerosis, hypertension, or may develop in females during peripartum period. A case of spontaneous carotid cavernous sinus fistula in a young adult male who presented with pulsating exophthalmos and secondary glaucoma is presented. Characteristic features of arteriovenous fistula--bruit, thrill, corkscrew episcleral vessels were present. Radiological investigations were done to confirm the diagnosis. Transvenous embolisation was done to close the fistula.