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.

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.

Apresentação atípica da oftalmopatia de Graves / Atypical presentation of Graves’ ophthalmopathy

A oftalmopatia de Graves é a doença orbitária mais comum e acomete 25 a 50% dos pacientes portadores da Doença de Graves e é mais frequente no sexo feminino, entre a segunda e quinta décadas de vida. A doença apresenta uma fase aguda e uma crônica, evoluindo lenta e progressivamente até estabilizar, sendo raros os casos de resolução espontânea. O tratamento dependerá da fase em que a doença se encontra e está baseado principalmente na corticoterapia via oral e endovenosa e/ou radioterapia, sendo a colchicina empregada em casos isolados. No seguinte relato de caso, abordaremos uma forma atípica de manifestação clínica da Oftalmopatia de Graves em paciente eutireoideia com anticorpos negativos na sua apresentação inicial.

Graves ‘ophthalmopathyis themost commonorbital diseaseand affects25-50% of the patients withGraves’ disease. It`s morecommon in females, between the second andfifth life`s decade. The disease hasanacute andachronic stage, slowly progressing until it stabilizes, with rarecasesof spontaneous resolution.The treatment depends on thestage andthe disease ismainly treated withoralor intravenous corticosteroids with or without radiotherapy;colchicine is usedin individual cases. In the followingcase report, we discuss an atypicalmanifestation ofGraves’ ophthalmopathyin an euthyroid patientwith negative antibodiesin the inicial presentation.

Orbitopatía de Graves en pediatría / Graves’ orbitopathy in pediatrics

Graves’ orbitopathy (GO) is rare in pediatric patients, however is the most common extrathyroid manifestation of Graves’ disease (GD), being present in 30-67% of patients. GO is an autoimmune inflammatory disorder involving orbital connective and fatty tissues as well as the extraocular muscles. In children, GO is less common and less severe than in adults. The most common symptoms are upper eyelid retraction, conjunctival injection, and proptosis and periorbital edema. Severe complications include dysthyroid optic neuropathy, corneal ulceration and eyeball subluxation. The diagnosis is established by clinical, laboratory and imaging findings. There are no management guidelines for GO in children but adult recommendations include the assessment of clinical activity and its severity, to implement the best treatment. Supportive therapies are intended to relieve symptoms and prevent corneal damage in mild cases. Tobacco exposure should also be avoided. The first line of treatment is systemic administration of corticosteroids in active and severe cases. Other options as somatostatin use and retrobulbar radiation have not been used in children, therefore their use is not recommended.

Development of Thyroid-Associated Ophthalmopathy in Patients Who Underwent Total Thyroidectomy

PURPOSE: To report clinical characteristics of thyroid-associated ophthalmopathy (TAO) in patients who previously underwent total thyroidectomy for thyroid cancer or a benign mass of the thyroid. MATERIALS AND METHODS: Of the patients who were diagnosed with TAO from March 2008 to March 2012, we performed a retrospective chart review on those who had undergone total thyroidectomy for thyroid cancer or a benign mass of the thyroid before the occurrence of ophthalmopathy. RESULTS: Of the 206 patients diagnosed with TAO, seven (3.4%) met the inclusion criteria. The mean age of the subjects was 47.4 years, and all were female. Six patients were diagnosed with papillary thyroid cancer, and one was diagnosed with a benign mass. The duration between total thyroidectomy and onset of TAO ranged from 3-120 months (median 48 months). Ophthalmic manifestations varied among cases. Except for the patient who was diagnosed with a benign mass, all patients showed hyperthyroid status and were under Synthroid hormone treatment at the time of TAO development. Five of these six patients had positive levels of thyroid-stimulating hormone (TSH) receptor autoantibodies. CONCLUSION: TAO rarely develops after total thyroidectomy, and the mechanism of TAO occurrence is unclear. However, most patients showed abnormalities in thyroid function and TSH receptor autoantibodies.

Graded Decompression of Orbital Fat and Wall in Patients with Graves' Orbitopathy

PURPOSE: To investigate the results of graded decompression of orbital fat and walls in Graves' orbitopathy (GO) considering the degree of proptosis reduction at surgery and preoperative computed tomography (CT) findings. METHODS: This is a retrospective interventional case series. Graded orbital fat and wall decompression was performed in 90 orbits of 55 patients. In patients with enlarged extraocular muscles and minimal orbital fat proliferation in preoperative CT scans, one- or two-wall decompression of posterior orbit was performed with minimal fat excision. In other cases, the maximal amount of fat tissue was removed from the post-septal area to the apex. If the proptosis was not satisfactorily symmetrically reduced at surgery, one- or two-wall decompression was performed successively. Symmetric reduction of proptosis was consistently confirmed intraoperatively to assure that a desired amount of exophthalmos reduction was achieved. RESULTS: Four types of decompression were performed: fat only (group 1), fat and one-wall (group 2), fat and two-wall (group 3), and two-wall and minimal fat decompression (group 4). The mean preoperative Hertel value (20.6 +/- 2.8 mm) was reduced significantly at six months postoperatively (16.1 +/- 2.3 mm). Proptosis significantly decreased with a mean of 4.3 +/- 1.7 mm, and the reduction was greatest (5.1 +/- 2.1 mm) in group 3. In group 1, a significant correlation between Hertel change and the volume of resected orbital fat was found (r = 0.479). Diplopia was newly developed or aggravated postoperatively in eight patients, and six of these patients were in group 3. With the exception of one patient, visual acuity improved to nearly normal postoperatively in all patients with optic neuropathy. CONCLUSIONS: Graded orbital decompression of orbital fat and bony walls, as assessed by the degree of proptosis reduction during surgery, was effective and predictable with minimal complications in GO patients with vision-threatening or cosmetically disfiguring proptosis.

Graded Decompression of Orbital Fat and Wall in Patients with Graves' Orbitopathy

PURPOSE: To investigate the results of graded decompression of orbital fat and walls in Graves' orbitopathy (GO) considering the degree of proptosis reduction at surgery and preoperative computed tomography (CT) findings. METHODS: This is a retrospective interventional case series. Graded orbital fat and wall decompression was performed in 90 orbits of 55 patients. In patients with enlarged extraocular muscles and minimal orbital fat proliferation in preoperative CT scans, one- or two-wall decompression of posterior orbit was performed with minimal fat excision. In other cases, the maximal amount of fat tissue was removed from the post-septal area to the apex. If the proptosis was not satisfactorily symmetrically reduced at surgery, one- or two-wall decompression was performed successively. Symmetric reduction of proptosis was consistently confirmed intraoperatively to assure that a desired amount of exophthalmos reduction was achieved. RESULTS: Four types of decompression were performed: fat only (group 1), fat and one-wall (group 2), fat and two-wall (group 3), and two-wall and minimal fat decompression (group 4). The mean preoperative Hertel value (20.6 +/- 2.8 mm) was reduced significantly at six months postoperatively (16.1 +/- 2.3 mm). Proptosis significantly decreased with a mean of 4.3 +/- 1.7 mm, and the reduction was greatest (5.1 +/- 2.1 mm) in group 3. In group 1, a significant correlation between Hertel change and the volume of resected orbital fat was found (r = 0.479). Diplopia was newly developed or aggravated postoperatively in eight patients, and six of these patients were in group 3. With the exception of one patient, visual acuity improved to nearly normal postoperatively in all patients with optic neuropathy. CONCLUSIONS: Graded orbital decompression of orbital fat and bony walls, as assessed by the degree of proptosis reduction during surgery, was effective and predictable with minimal complications in GO patients with vision-threatening or cosmetically disfiguring proptosis.

Eficácia das terapias não convencionais (Pentoxifilina e Nicotinamida/Alopurinol) comparadas a metilprednisolona intravenosa no tratamento da oftalmopatia de Graves / Effectiveness of unconventional therapies ( Pentoxifylline and Nicotinamide / Allopurinol ) compared to intravenous methylprednisolone in the treatment of Graves' ophthalmopathy

A oftalmopatia de Graves (OG) se constitui em uma das manifestações clínicas mais marcantes da Doença de Graves. Mais freqüente em mulheres, pode atingir qualquer faixa etária e produzir alterações negativas na qualidade de vida dos pacientes acometidos. Trata-se de doença auto-imune cuja fisiopatologia centra-se em atividade inflamatória desencadeada por diversos antígenos presentes nos tecidos extra-ocular, com infiltrado linfocitário importante, adipogênese e produção de glicosaminoglicanos levando ao aspecto típico de inflamação e proptose. Os casos mais leves podem ser tratados com medidas locais, enquanto que casos mais graves, frequentemente, recebem terapia com glicocorticoides, particularmente pulsoterapia com metilprednisolona. O objetivo deste estudo foi comparar o uso de dois esquemas terapêuticos (associação de nicotinamida/alopurinol ou pentoxifilina por via oral) com a terapia padrão (corticóide em pulsoterapia). Foram recrutados pacientes portadores de OG classificados como moderada ou moderadamente grave e em atividade (definidos pelo NO SPECS e CAS) , distribuídos em três grupos de tratamento, a saber : G1- Pentoxifilina , G2- Nicotinamida/Alopurinol e G3- Metiprednisolona em pulsoterapia, distribuídos por conveniência. Para investigar a resposta ao tratamento clínico instituído, foram usados o CAS, a medida da proptose ocular por meio de exoftalmômetro, a dosagem de interleucinas séricas(TNF-α e IL-6), registros fotográficos dos olhos dos pacientes avaliados e a TC de órbitas...

Graves ’ophthalmopathy (GO) constitutes one of the most striking manifestations of Graves’ disease. More common in women, ocurr at any age group and produce negative changes in the quality of life of affected patients. It is an autoimmune disease whose physiopathology focuses on various inflammatory activity triggered by antigens present in extraocular tissues with significant lymphocyte infiltration, production of glycosaminoglycansadipogenesis leading to the typical appearance of inflammation and proptosis. The mild cases can be treated with local measures, while more serious cases often receive glucocorticoid therapy, particularly methylprednisolone pulse therapy. The aim of this study was to compare the use of two regimens (combination of nicotinamide / allopurinol or pentoxifylline orally) with standard therapy (intravenous steroid pulse therapy). We recruited patients with OG classified as moderate or moderately severe and active (defined by NO SPECS and CAS), divided into three treatment groups, namely: Pentoxifylline-G1, G2-Nicotinamide /Allopurinol-and G3 Metiprednisolona in pulse, distributed for convenience. To investigate the response to clinical treatment, the CAS were used to measure the ocular proptosis through exophtalmomether, the dosage of serum interleukins (TNF-α and IL-6), photographic records of the eyes of patients evaluated CT orbits...

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.

Bilateral versus unilateral thyroid eye disease.

Aims: The aim of this study was to compare demographics, clinical manifestations, associated systemic and ocular factors, severity and activity of patients with unilateral thyroid eye disease (U-TED) versus bilateral thyroid eye disease (B-TED). Materials and Methods: In a cross-sectional study, all patients with Graves’ hyperthyroidism and primary hypothyroidism seen in an endocrinology clinic were included from September 2003 to July 2006. Demographics, complete eye examination, severity score (NOSPECS, total eye score), and clinical activity score were recorded and compared in the B-TED and U-TED groups of patients. Results: From 851 patients with thyroid disorders, 303 (35.6%) had TED. Thirty-two patients (32/ 303, 10.56%) were found to have U-TED. Patients with U-TED (mean age 31.6 ± 11.6 years) were significantly younger than patients with B-TED (mean age 37.7 ± 14.7 years). Monovariate analysis (Chi-square and independent sample t-test) showed a significantly higher severity score in B-TED (U-TED 4.09±4.05, B-TED: 6.7±6.3; P= 0.002) and more activity score in B-TED (U-TED= 1.03±0.96, B-TED: 1.74±1.6, P= 0.001). However, multivariate analysis did not show any significant difference between the two groups in terms of age, gender, type of thyroid disease, duration of thyroid disease and TED, severity and activity of TED, smoking habit, and presentation of TED before or after the presentation of thyroid disease (0.1<P<1). Conclusion: This study did not find any significant difference between U-TED and B-TED in relation to the demographics, type of thyroid disease, associated findings, and severity and activity of TED.

Ultrasonography compared to magnetic resonance imaging in thyroid-associated Graves' ophthalmopathy / Comparação de ultrassonografia à ressonância magnética nuclear na oftalmopatia de Graves associada à tiroide

OBJECTIVE: To compare ultrasonography (US) to magnetic resonance imaging (MRI) and the clinical activity score (CAS) in Graves' ophthalmopathy. SUBJECTS AND METHODS: Nineteen patients underwent extraocular muscle thickness measurements by US and MRI, reflectivity by US and signal-intensity ratio by MRI. There were also twelve US control subjects. RESULTS: US median thicknesses were greater than in controls. Correlation was found between US and MRI in the median thickness of the left eye rectus medial muscle as well as between signal-intensity ratio (SIR) and thickness by US. An inverse correlation was found between reflectivity and SIR in the inferior and lateral rectus. On associating the tests for detecting activity the best results were obtained with CAS plus MRI (sensitivity 75 percent), and US and MRI (positive predictive value 77 percent and specificity 80 percent). CONCLUSION: CAS and US results showed poor correlation with MRI results suggesting that they cannot replace each other but when combined these methods can improve the evaluation of thyroid-associated ophthalmopathy.

OBJETIVO: Comparar a ultrassonografia (US) à ressonância magnética nuclear (RMN) e o índice de atividade clínica (IAC) na oftalmopatia de Graves. SUJEITOS E MÉTODOS: Dezenove pacientes submetidos à medida da espessura dos músculos extraoculares por US e RMN, refletividade ao US e razão da intensidade de sinal (RIS) à RMN. Grupo controle para US de 12 indivíduos. RESULTADOS: Espessura mediana ao US foi maior que dos controles. Houve correlação entre US e RMN na espessura mediana dos músculos retos mediais dos olhos esquerdos e entre a RIS e a espessura ao US e correlação inversa entre refletividade e SIR nos retos inferior e lateral. Detectando atividade, os melhores resultados foram associando IAC e RMN (sensitividade de 75 por cento) e US e RMN (valor preditivo positivo de 77 por cento e especificidade de 80 por cento). CONCLUSÃO: Resultados do IAC e US mostraram pouca correlação com a RMN, sugerindo que não podem ser substituídos, mas, quando combinados, esses métodos podem melhorar a avaliação da oftalmopatia associada à tiroide.

Orbital pseudotumor: a differential diagnosis of Graves' ophthalmopathy / Pseudotumor de órbita: um diagnóstico diferencial de oftalmopatia de Graves

The objective of this study is to report and discuss a rare and inflammatory cause of exophthalmos. This report describes a patient with exophthalmos, who was initially diagnosed with euthyroid Graves' with good response to therapy. After 8 years of follow-up, she had recurrence of symptoms and a new evaluation revealed the final diagnosis of orbital pseudotumor. Orbital pseudotumor is an uncommon disorder that both radiologically and clinically mimics a malignant process or other inflammatory disease, such as Graves' ophthalmopathy.

O objetivo deste estudo é relatar e discutir uma causa de exoftalmia rara e inflamatória. Este artigo relata uma paciente com exoftalmia que recebeu diagnóstico inicial de oftalmopatia de Graves eutireoideana com boa resposta à terapia. Após oito anos de seguimento, houve recorrência dos sintomas e uma nova avaliação revelou o diagnóstico final de pseudotumor orbitário. Pseudotumor orbitário é uma condição incomum que mimetiza clínica e radiologicamente uma doença maligna ou inflamatória, como a oftalmopatia de Graves.

Tratamento cirúrgico da proptose na doença de graves / Surgical treatment in graves disease proptosis

Introdução: a proptose é uma protusão anormal, para diante, de um ou ambos os olhos. É a manifestação extratireoidiana mais comum da doença de Graves. Os problemas estéticos e funcionais apresentam muitostranstornos aos pacientes. Objetivo: demonstrar a melhora estética e funcional dos pacientes operados. Métodos: foram operados 3 pacientes eutireoideos com proptose bilateral há mais de 6 meses, removendo 3 paredesda órbita. Resultados: todos os pacientes apresentaram melhora estética e funcional. Conclusões: aremoção óssea na proptose é método eficaz e de baixo risco cirúrgico.

Background: Proptosis is anabnormal protrusion, anteriorly, either unilateral orbilateral.It is the commonestextra-thyroidien manifestation of Graves’ disease. Patientsexperiment aesthetic and functional problems. Objective: Show the aesthetic and functional improvement ofoperated patients.Methods: Three euthyroidien patients were operated to correct a 6 months older bilateralproptosis where it was removed three wall of the orbit. Results: All the patients presented aesthetic and functiona limprovement.Conclusions: Orbit bonesresection in proptosis is an efficient and low risk surgical method.

Estudio de las células natural killer como marcador inmunológico en pacientes con oftalmopatía distiroidea / Study of natural killer cells as an immune marker in patients with dysthyroid ophthalmopathy

Objetivo: analizar la correlación e influencia inmunológica de las células "Natural killer" con el desarrollo de la oftalmología distiroidea. Método: se incluyen 30 pacientes, estudiándose con tomografía computada de órbita y resonancia magnética, estudio hormonal e inmunológico. Resultados: 83.33 por ciento sexo femenino y 16.6 por ciento masculino. Edad promedio 39.97 años, 57.7 por ciento con enfermedad tiroidea controlada, complemento C3 11.16 y C4 26.54, natural killer basal 437.93 (rango 350-715) y posterior a tratamiento 308.1 (rango 188-405). Se demostró por imagen compromiso a músculos en el 77 por ciento sin diagnóstico. Conclusiones: la lesión muscular se correlacionó con el estado inmunológico del paciente incluso sin exoftalmos. La mejoría de las células NK con el tratamiento inmusupresor fue evidente y se sugiere una estrecha relación con el desarrollo de anomalías musculares y estrábica. Es importante distoroideo integrar un diagnóstico inmunológico y estrabológico temprano.

Objective: to analyze the immunological activity of natural killer cells in graves disease. Method: we studied 30 patients without strabismus. Orbital tomography, magnetic resonance hormonal and immunological test were incluided. Results: 83.33 per cent female, 16.6 per cent male. Age: 39.97 years old. 57.7 per cent of patients had normal hormonal levels. natural killer 437.93 pre-treatment, and 308.1 post treatment. Muscle damage was demostrated in 77 per cent of cases. Conclusion: we demonstrated a correlation between muscle damage and immunological disorders without an important exoftalmos. Improvement with corticoid therapy of natural killer alls levels was evident and a close relationship with de development of muscular anomalies and strabismus is suggested. In all graves patients it is important to integrate an early immunological and strabismological diagnosis.