Orbitopatía tiroidea. Puntos clave para el diagnóstico y tratamiento / Thyroid orbitopathy. Tricks for diagnosis and treatment

La orbitopatía tiroidea (OT) es una patología autoinmune de etiología desconocida. Se trata de una enfermedad edematosa e inflamatoria crónica y a veces subaguda o aguda, cuyas características principales son edema palpebral, úlceras corneales, hipertensión ocular, exoftalmos generalmente bilateral, estrabismo restrictivo, diplopía y neuropatía óptica. El objetivo principal es realizar una revisión y actualización acerca del enfoque diagnóstico y terapéutico de la OT. Realizar una revisión y actualización acerca del enfoque diagnóstico, terapéuticos de la OT. Métodos: Revisión de la literatura publicada referente a la OT y manera actual de enfocar esta patología. Resultados: La OT es una enfermedad inflamatoria orbitaria con un posible origen autoinmune y que suele asociarse a trastornos metabólicos de la glándula tiroidea. Fisiopatológicamente su mecanismo no está claro. Afecta mayormente a mujeres y está negativamente influenciada por factores como el tabaquismo, la edad, el sexo y la raza. Es imprescindible clasificarla en cuanto a su severidad y su actividad para un correcto manejo. Conclusiones: El conocimiento clínico de OT es esencial para el diagnóstico precoz de la enfermedad. El tratamiento médico, en caso de OT activa, debe ser precoz, agresivo y acorde a la fase en que se presente el paciente con el fin de evitar las graves consecuencias de la OT. El tratamiento quirúrgico deberá ser lógico y ordenado, pero a su vez rápido, con el fin de devolver al paciente al entorno laboral, social y familiar. (AU)

Purpose: Thyroid orbitopathy (TO) is an autoimmune disease of unknown etiology. It is a chronic and sometimes subacute or acute edematous and inflammatory disease, the main characteristics of which are eyelid edema, corneal ulcers, ocular hypertension, generally bilateral exophthalmos, restrictive strabismus, diplopia and optic neuropathy. The main purpose of this work is to review and update the current diagnostic and therapeutic approaches. To propose a practical, basic and precocious approach based in the knowledge of the TO. Methods: Literature review and exposition of our experience in the management of TO. Results: TO is an inflammatory orbital disease that probably has an autoimmune origin and most of the time is related to systemic disorders of the thyroid gland. Pathogenesis of the disease is not yet fully understood. Women are more likely to develop TO, and the disease is clearly affected by several factors such as smoking, age, sex and race. It is crucial to determine TO severity and activity for a correct management. Conclusions: Clinical knowledge is essential for the early diagnosis of this disorder, and it is the most important factor for the proper management of the disease. The medical treatment must be initiated promptly and should be aggressive and based in the current phase of the disease, in order to avoid the severe damage that follows TO. The surgical approach must be logical and sequential, but, on the other hand, must be rapid and aggressive in order to return the patient to his/her labour, social and familiar environment. (AU)

Técnicas de imagen en la orbitopatía asociada al tiroides / Imaging Techniques in Thyroid-associated Orbitopathy

La orbitopatía asociada al tiroides es la primera causa de afección inflamatoria orbitaria y de exoftalmo en adultos. Su progresión puede generar complicaciones, las cuales, ante un manejo inoportuno u omisión diagnóstica, pueden conllevar a disfunción visual irreversible. De aquí la importancia de garantizar un diagnóstico y tratamiento adecuados, para lo cual es fundamental un correcto manejo imagenológico. Con el propósito de actualizar y sistematizar el conocimiento sobre el diagnóstico por imágenes de la orbitopatía asociada al tiroides, se realizó una revisión de las publicaciones relacionadas con el tema de las últimas dos décadas. La búsqueda y la localización de la información se apoyaron en la elección de palabras clave/descriptores que configuraron su perfil. Además, se analizaron las ecografías, tomografías computarizadas y resonancias magnéticas de los pacientes diagnosticados con esta enfermedad en el Instituto Cubano de Oftalmología Ramón Pando Ferrer, durante los últimos 10 años. Todo lo investigado confirma que las técnicas de imagen son claves para establecer el diagnóstico de la oftalmopatía asociada al tiroides, pero jamás sustituirán a la historia clínica detallada y el examen físico minucioso. El médico se equivoca gravemente cuando pretende obviar la importancia del juicio clínico. La omisión de este principio, en el mejor de los casos, solo conducirá a diagnósticos casuales(AU)

Thyroid-associated orbitopathy is the leading cause of orbital inflammatory disease and exophthalmos in adults. Its progression can generate complications, which, in case of inopportune management or diagnostic omission, can lead to irreversible visual dysfunction. Hence the importance of guaranteeing an adequate diagnosis and treatment, for which a correct imaging management is essential. With the purpose of updating and systematizing the knowledge on the imaging diagnosis of thyroid associated orbitopathy, a review of the publications related to the subject of the last two decades was carried out. The search and localization of the information was supported by the choice of keywords/descriptors that configured its profile. In addition, ultrasound scans, CT scans and MRI scans of patients diagnosed with this disease at the Cuban Institute of Ophthalmology Ramón Pando Ferrer, during the last 10 years, were analyzed. All that has been investigated confirms that imaging techniques are key to establish the diagnosis of thyroid-associated ophthalmopathy, but they will never replace a detailed clinical history and a thorough physical examination. The physician is seriously mistaken when he pretends to ignore the importance of clinical judgment. The omission of this principle will, at best, only lead to casual diagnoses(AU)

Orbital inflammatory disease.

Orbital inflammatory disease (OID), commonly known as orbital pseudotumour, is an inflammatory disease of unknown cause. It has different forms of presentation and different degrees of severity. Its variable nature is the main cause for this disease to be misdiagnosed and misclassified. The prognosis of OID depends on the tissues affected and the histology. OID usually responds favourably to systemic steroid treatment. However, empiric steroids may mask other underlying diseases that respond well to this treatment as well, namely, IgG4-related disease or lymphoproliferative disorders. This fact has led to controversy among various authors as some recommend performing a biopsy in most of the cases, whereas others defend that this procedure should only be performed if the patient has not responded to empiric steroid treatment. Although steroids have been the mainstream treatment of OID, the side effects, relapse rates and lack of response in some cases have resulted in them being replaced by immunosuppressive and immunomodulator therapies that currently stand as a key steroid-sparing treatment option, in addition to radiotherapy and surgery. The aim of this review is to update the evidence on the diagnosis and treatment of OID.

Enfermedad inflamatoria orbitaria idiopática / Orbital inflammatory disease

La enfermedad inflamatoria orbitaria idiopática (EIOI), comúnmente conocida como pseudotumor orbitario, es una enfermedad inflamatoria de etiología desconocida. Sus síntomas pueden ser muy variables tanto en intensidad, gravedad, formas de presentación o gravedad. Esta heterogeneidad ha condicionado que sea una entidad difícil de definir y clasificar. El pronóstico de la EIOI depende de su localización, presentación e histología. La EIOI suele responder favorablemente a los corticoides sistémicos, sin embargo, este hecho puede hacer que la entidad sea confundida con otras enfermedades que también tienen buena respuesta a corticoides, como la enfermedad relacionada con la IgG4 y las enfermedades linfoproliferativas. Esta controversia ha alzado una polémica entre autores que defienden la realización de biopsia previa al tratamiento en la mayoría de los casos, frente a otros que afirman que la biopsia debe indicarse en lesiones que no responden adecuadamente al tratamiento médico empírico. Si bien los corticoides se sitúan como los protagonistas de la EIOI, los efectos secundarios, las tasas de recidivas y la falta de respuesta de algunos subtipos han permitido el paso a agentes inmunosupresores e inmunomoduladores que ocupan un escalón fundamental en la terapia combinada o ahorradora de corticoides, junto con la radioterapia y la cirugía. El objetivo de esta revisión es actualizar la evidencia sobre el diagnóstico y tratamiento de la EIOI.

Orbital inflammatory disease (OID), commonly known as orbital pseudotumour, is an inflammatory disease of unknown cause. It has different forms of presentation and different degrees of severity. Its variable nature is the main cause for this disease to be misdiagnosed and misclassified. The prognosis of OID depends on the tissues affected and the histology. OID usually responds favourably to systemic steroid treatment. However, empiric steroids may mask other underlying diseases that respond well to this treatment as well, namely, IgG4-related disease or lymphoproliferative disorders. This fact has led to controversy among various authors as some recommend performing a biopsy in most of the cases, whereas others defend that this procedure should only be performed if the patient has not responded to empiric steroid treatment. Although steroids have been the mainstream treatment of OID, the side effects, relapse rates and lack of response in some cases have resulted in them being replaced by immunosuppressive and immunomodulator therapies that currently stand as a key steroid-sparing treatment option, in addition to radiotherapy and surgery. The aim of this review is to update the evidence on the diagnosis and treatment of OID.

Pacientes con compromiso orbitario y renal: Diagnósticos diferenciales poco frecuentes / Patients with orbitary and kidney commitment: Rare differential diagnoses

Se comunica una serie de tres casos clínicos que consultaron al servicio de Reumatología por compromiso orbitario y renal. Uno de ellos presentó pseudotumor orbitario con proteinuria en rango nefrótico; se realizó biopsia y se encontró infiltrado linfoplasmocitario denso y fibrosis estoriforme con inmunohistoquímica: 15 células IgG4+ por campo de alto poder y relación IgG/IgG4 ≤40%, concluyendo diagnóstico de enfermedad relacionada por IgG4. El segundo y tercer caso presentaron compromiso ocular con "ojos de mapache" y lesiones amarillentas en párpados, ambos con proteinuria >500 mg/24 h, con biopsia de piel rojo Congo positiva y birrefringencia verde manzana con luz polarizada. Se discuten distintos diagnósticos diferenciales poco frecuentes a tener en cuenta en estos pacientes.

A series of three cases that consulted the rheumatology service due to orbital and renal involvement is reported. One of them presented orbital pseudotumor with proteinuria in the nephrotic range, a biopsy was performed, finding dense lymphoplasmacytic infiltrate and storiform fibrosis with immunohistochemistry: 15 IgG4 positive cells per HPF and IgG/IgG4 ratio ≤40%, concluding diagnosis of IgG4 related disease. The second and third cases presented ocular involvement with raccoon eyes and yellowish lesions on the eyelids, both with proteinuria greater than 500 mg/24 h, with apple-green birefringence of amyloid on congo red staining. Different rare differential diagnoses to take into account in these patients are discussed.

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.

Tratamiento de la oftalmopatía de Graves / Treatment of Graves’ ophthalmopathy

La oftalmopatía de Graves constituye una enfermedad inflamatoria con afectación fundamental de los músculos extraoculares y de la órbita, y representa la principal manifestación extratiroidea en los pacientes con enfermedad de Graves-Basedow. La etiopatogenia subyacente es inmunomediada mediante un mimetismo molecular con el receptor de la tirotropina en los fibroblastos oculares. El principal tratamiento en los casos moderados-graves se basa en la inmunosupresión sistémica, y son de elección los glucocorticoides a dosis altas. Sin embargo, algunos pacientes presentan intolerancia al tratamiento o mala respuesta a los glucocorticoides. En los últimos años se han descrito novedades terapéuticas en cuanto a la posología de los tratamientos inmunosupresores empleados, así como la irrupción de la terapia biológica en este campo. El objetivo de esta revisión es actualizar el tratamiento de la oftalmopatía inflamatoria, así como presentar opciones terapéuticas alternativas en aquellos casos corticorresistentes o con intolerancia a glucocorticoides. (AU)

Graves’ ophthalmopathy is an inflammatory disease with primary involvement of the extraocular muscles and the orbit. It encompasses the most common extra-thyroid manifestation in patients with Graves-Basedow disease. The underlying cause is molecular mimicry with the TSH receptor in ocular fibroblasts, leading to an immuno-mediated pathogenesis. Glucocorticoids at high doses are the cornerstone in moderate-severe cases. However, some patients are corticorresistant or intolerant. In recent years, therapeutic novelties have been described in terms of the dosage of the immunosuppressive treatments used, as well as the emergence of biological therapy in this field. The objective of this review is to update the treatment of Graves’ ophthalmopathy, as well as to present alternative options in patients resistant or intolerant to glucocorticoids. (AU)

Treatment of Graves' ophthalmopathy. / Tratamiento de la oftalmopatía de Graves.

Graves' ophthalmopathy is an inflammatory disease with primary involvement of the extraocular muscles and the orbit. It encompasses the most common extra-thyroid manifestation in patients with Graves-Basedow disease. The underlying cause is molecular mimicry with the TSH receptor in ocular fibroblasts, leading to an immuno-mediated pathogenesis. Glucocorticoids at high doses are the cornerstone in moderate-severe cases. However, some patients are corticorresistant or intolerant. In recent years, therapeutic novelties have been described in terms of the dosage of the immunosuppressive treatments used, as well as the emergence of biological therapy in this field. The objective of this review is to update the treatment of Graves' ophthalmopathy, as well as to present alternative options in patients resistant or intolerant to glucocorticoids.

El ojo: Un rompecabezas diagnóstico: Estudio observacional descriptivo y revisión / The Eye: A Diagnostic Puzzle: Descriptive observational study and review

Se comunica una serie de casos, multicéntricos de la cual participaron cinco instituciones. La muestra fue de 17 pacientes, de los cuales 11 pertenecían al Hospital Dr. J.M. Cullen. Todos consultaron por compromiso orbitario y/o periorbitario. El compromiso en hombres fue de 23.4% y un 76.6% en mujeres. La edad media en años fue de 45.4 (17-69 años). Dentro de los diagnósticos encontrados, cinco casos fueron Enfermedad Relacionada con IgG4 (ER-IgG4), dos casos de Enfermedad de Erdheim Chester (EEC), dos Xantogranuloma, dos xantelasmas, un caso de metástasis de cáncer de mama, un caso de orbitopatía tiroidea, un caso de Amiloidosis con mieloma múltiple, y tres sin diagnóstico. Se revisan los diagnósticos diferenciales encontrados.

A series of multicentric cases is reported, of which five institutions participated. The sample was of 17 patients, of which 11 belonged to our Hospital, the Dr. J.M. Cullen Hospital. All consulted for orbital and/or periorbital commitment. The commitment in men was 23.4% and 76.6% in women. The average age in years was 45.4 (17-69 years). Among the diagnoses found, five cases were IgG4-Related Disease, two cases of Erdheim Chester Disease, two Xantogranuloma, two xanthelasmas, a case of breast cancer metastases, a case of thyroid orbitopathy, a case of Amyloidosis with multiple myeloma, and three without diagnosis. Differential diagnoses found are reviewed.

Thyroid orbitopathy masked a carotid-cavernous fistula. Case report. / Orbitopatía tiroidea enmascara una fístula carótido-cavernosa. Presentación de un caso.

The case concerns a 73 year-old female with a diagnosis of hyperthyroidism of 8 months onset. Since then, she has suffered hyperaemia and ocular proptosis. Bilateral exophthalmos was observed in the physical examination, along with mild conjunctival hyperaemia in the right eye associated with dilated episcleral vessels. Computed tomography showed enlarged inferior rectus with no signs of ocular nerve or ophthalmic superior vein compression. In the orbital Doppler ultrasound scan, there was an arterialised flow over the superior ophthalmic vein, giving a possible diagnosis of cavernous carotid. Thyroid orbitopathy with a cavernous carotid fistula is an unusual combination in which the clinical signs of both pathologies can mask the complete diagnosis; thus it is important to be acquainted with both pathologies for their correct management and multidisciplinary treatment.

Effectiveness of oral counselling for smoke cessation in Graves orbitopathy patients. / Efectividad del consejo médico para dejar de fumar en pacientes con orbitopatía de Graves.

OBJECTIVE: Smoking is an important risk factor for Graves orbitopathy (GO) and it is modifiable. The advice to stop smoking has been included in all the clinical practice guidelines of GO. However, the effectiveness of this practice remains unknown. The purpose of this study is to assess the change in the smoking habit in patients affected with GO after an oral counselling for smoking cessation. MATERIAL AND METHODS: A retrospective cohort of GO patients was studied. The patients received a significant oral counsel during the first consultation with the ophthalmologist. 33 GO patients were explored in the ophthalmology clinic during 2013 and 2014 and the study was done throughout a telephone questionnaire in 2015. The main outcome was the number of cigarettes smoked daily before and after consultation with the endocrinologist and the ophthalmologist. Other medical and socioeconomic factors were recorded. RESULTS: The mean number of cigarettes that were smoked was 13.6 (SD 9.66) and 6.3 (SD 7.73) before and after the consultation done at the ophthalmology office (T-test paired, P=0.0006). 42.42% achieved smoking cessation and 30.3% decreased their smoking habit. Patients who stopped smoking suffered usually from active and severe GO, had more stable jobs and received greater support from their relatives and friends. CONCLUSION: A firm and strong oral counsel held for smoke cessation was effective in GO patients. This disease deeply affects patients' quality of life, making them more prone to change their habits.

Descompresión orbitaria endoscópica transnasal en orbitopatía tiroidea severa / Transnasal endoscopic orbital decompression in severe thyroid orbitopathy

RESUMEN La orbitopatía tiroidea es una enfermedad autoinmune, en la que una reacción inflamatoria genera aumento de la presión orbitaria con protrusión de su contenido. A menudo es autolimitada y sus síntomas más frecuentes son retracción palpebral, exoftalmo y diplopía. Existen casos severos con compromiso de la agudeza visual por compresión del nervio óptico. El diagnóstico es clínico, pero debe complementarse con una tomografía computarizada. Su tratamiento depende de la gravedad y actividad de la enfermedad, siendo los procedimientos quirúrgicos, como la descompresión orbitaria, de elección en exoftalmo y neuropatía óptica compresiva. El pilar de tratamiento en la orbitopatía tiroidea severa es la cirugía descompresiva. Se han descrito múltiples técnicas, pero con limitaciones. La descompresión endoscópica transnasal, es considerada actualmente el procedimiento de elección, ya que permite una buena visualización de la pared medial, con resultados comparables y menores complicaciones, respecto a métodos tradicionales. Describimos un caso de oftalmopatía tiroidea severa, con exoftalmo, diplopía y disminución de la agudeza visual, en la que se realizó una descompresión endoscópica con muy buenos resultados.

ABSTRACT Thyroid orbitopathy is an autoimmune disease in which an inflammatory reaction generates increased orbital pressure with protrusion of its contents. It is often self-limiting and its most frequent symptoms are eyelid retraction, exophthalmos and diplopia. There are severe cases with compromised visual acuity due to compression of the optic nerve. The diagnosis is clinical, but must be complemented with a computed tomography scan. Its treatment depends on the severity and activity of the disease and the surgicals procedures such as orbital decompression is the best choice in exophthalmos and compressive optic neuropathy. The treatment in severe thyroid orbitopathy is decompressive surgery. Multiple techniques have been described, but with limitations. The transnasal endoscopic decompression is currently considered the gold standard, since it allows a good visualization of the medial wall with comparable results and less complications, compared to traditional methods. We present a case of severe thyroid ophthalmopathy, with exophthalmos, diplopia and decreased visual acuity, in which a transnasal endoscopic decompression was performed with very good outcomes.

Response to tocilizumab treatment in Graves' ophthalmopathy by measuring rectus muscle thickness and chemosis using optical coherence tomography. / Evaluación del grosor de los músculos extraoculares y la quemosis tras tocilizumab en oftalmopatía de Graves mediante tomografía de coherencia óptica.

PURPOSE: To assess the extraocular muscle thickness and chemosis after treatment with tocilizumab in patients with active Graves' ophthalmopathy by optical coherence tomography. METHODS: Case series of five patients with active Graves' ophthalmopathy (clinical activity score ≥4/10) treated with 4 doses of tocilizumab. These patients had been previously treated with corticosteroids with no response. Spectral-domain optical coherence tomography was employed to determine lateral and medial rectus muscle thickness and chemosis before and after 4 doses of tocilizumab given monthly. Scanning was performed at 3 and 9 o'clock (nasal and temporal). RESULTS: The study included four women and one man with a median age of 52 years (range: 38-73). Median Graves' ophthalmopathy activity duration was 17 months (12-18). Median medial rectus and determine lateral thicknesses pre-treatment were 249µm (174-366) and 337µm (142-443), respectively. Median chemosis was 409µm (290-610). After tocilizumab treatment, median muscle thicknesses reduced to 157µm (88-187) and 197µm (99-290), respectively (P=.043; Wilcoxon) and chemosis to 59µm (0-78). Median clinical activity score decreased from 5 (4-8) to 1 (0-3). CONCLUSIONS: A reduction in extraocular muscle thickness and chemosis was observed after treatment with tocilizumab in Graves' ophthalmopathy patients using an optical coherence tomography, so this technique could be a useful complementary technique to assess the therapeutic responses.

Descompresión orbitaria por vía endoscópica nasal en la orbitopatía tiroidea: nuestra experiencia / Endoscopic orbital decompression for the management of thyroid eye disease: our experience / Decompressão orbitaria por via nasal endoscópica na orbitopatia tírida: nossa experiência

INTRODUCCIÓN: La orbitopatía tiroidea es una de las manifestaciones extratiroideas más frecuentes de la enfermedad de graves. El diagnóstico es clínico y con estudios de imágenes. El tratamiento depende de la etapa en que se encuentre la enfermedad, pudiendo ser conservador o quirúrgico, siendo la descompresión orbitaria el pilar del tratamiento. OBJETIVOS: Describir la técnica quirúrgica y las complicaciones más frecuentes. Comprobar los beneficios en la reducción del exoftalmos, la mejoría de la agudeza visual y la descompresión del nervio óptico...

INTRODUCTION: Thyroid orbitopathy is one of the most frequent extra thyroid manifestations of Graves' disease. The diagnosis is clinical and with imaging studies. The treatment depends on the stage in which the disease is found; can be conservative or surgical, the orbital decompression is the pillar of the treatment. OBJECTIVES: Describe the surgical technique and the most frequent complications checking the benefits in the reduction of exophthalmos, the improvement of visual acuity and decompression of the optic nerve…

INTRODUÇÃO: A orbitopatia tireoidiana é uma das manifestações extra tireóides mais freqüentes da doença de graves. O diagnóstico é clínico e com estudos de imagem. O tratamento depende da fase em que a doença é encontrada; podendo ser conservador ou cirúrgico, sendo a descompressão orbital o pilar do tratamento. OBJETIVOS: Descreva a técnica cirúrgica e as complicações mais frequentes. Verificar os benefícios na redução do exoftalmos, a melhora da acuidade visual e descompressão do nervo óptico...

Orbitopatía asociada al tiroides en edades pediátricas / Thyroid-associated orbinopathy at pediatric ages

La enfermedad de Graves es rara en los niños y adolescentes, y aunque cursa con una clínica bastante florida, no siempre se piensa en este diagnóstico, y en no pocas ocasiones llegan a la consulta de Endocrinología remitidos de servicios de Psicología y/o Psiquiatría por un predominio de alteraciones conductuales (hiperquinesia, irritabilidad). La afección ocular puede aparecer en relación o no con los estados de hipertiroidismo, y se presenta en muchos casos con el paciente eutiroideo, por lo que se nombra actualmente orbitopatía asociada al tiroides. Es una enfermedad autoinmune y autolimitada que afecta el tejido conectivo, y su evolución es menos grave que en el adulto. El tratamiento de esta entidad es muy particularizado en las edades pediátricas, debido a que las modalidades terapéuticas por sí mismas no cumplen todos los criterios de efectividad y seguridad. Teniendo en cuenta el grado y la severidad de la orbitopatía se establecen los protocolos de atención. Es válido señalar que en estas edades -por lo general- se presentan formas leves o moderadas. Existen tres líneas de tratamiento: el medicamentoso, con antitiroideos de síntesis; el iodo radiactivo solo, o combinado con terapia esteroidea; o la extirpación quirúrgica de la glándula tiroides. En niños es muy raro que se llegue a la cirugía orbitaria. Con el objetivo de reagrupar información actualizada sobre este tema se presenta esta revisión(AU)

Graves´ disease is rare in children and adolescents, and although it develops with a fairly abundant clinic, this diagnosis is not always taken into account and in many occasions, the patients are referred to the endocrinology service from the psychology and /or psychiatry service because of predominant behavioral alterations (hyperkinesia, irritability). This eye disease may appear, either associated or not, to the conditions of hyperthyroidism, and it occurs many times in the euthyroid patient, so it is presently called thyroid-associated orbinopathy. It is an autoimmune, autolimited disease affecting the connective tissue and its progression is less severe than in the adult. The treatment is very particularized at pediatric ages because the therapeutic modalities as such do not meet all the required effectiveness and safety criteria. Taking into account the degree and the severity of orbinopathy, the respective care protocols are set. Generally speaking, it is worthwhile to point out that at these ages; there are moderate or slight forms of disease. There are also three lines of treatment: medication with synthesis anti-thyroid; the radioiodine therapy alone or in combination with steroidal therapy; and the surgical excision of the thyroid gland. It is very uncommon that a child undergoes orbital surgery. The objective of this review was to reorganize updated information about this topic(AU)

Toxina botulínica A en el músculo recto superior para la corrección de la retracción palpebral / Botulinum toxin A in the upper rectus muscle for the correction of eyelid retraction

Objetivo: evaluar los resultados terapéuticos obtenidos con la inyección de toxina botulínica en el músculo recto superior en pacientes con retracción palpebral moderada y grave en el curso de la orbitopatía tiroidea; determinar la influencia de algunos factores relacionados con estos e identificar las complicaciones y las reacciones adversas asociadas a su uso terapéutico. Métodos: se realizó un estudio descriptivo prospectivo en el Servicio de Oftalmología del Hospital Hermanos Ameijeiras en una serie de 21 casos con retracción palpebral moderada y grave en el curso de la orbitopatía tiroidea. La inyección de toxina botulínica en el músculo recto superior para la corrección de la retracción de la orbitopatía tiroidea se realiza por primera vez en el país. Resultados: fueron satisfactorios en el 66,7 por ciento de los casos. La edad y la gravedad clínica de la retracción fueron los factores que influyeron sobre la respuesta terapéutica (p= 0,013 y p= 0,015 respectivamente). Conclusiones: la inyección de toxina botulínica en el músculo recto superior es efectiva en la mayoría de los casos tratados, sobre todo en adultos jóvenes y con menor gravedad clínica de la retracción. La hipercorrección es la complicación más temida de este procedimiento(AU)

Objective: to evaluate the therapeutic results of the botulinum toxin injection in the upper rectus muscle in patients with moderate and severe eyelid retraction in the course of thyroid orbitopathy, and to determine the influence of some factors related to these patients and to identify the complications and adverse reactions associated to its therapeutic use. Methods: prospective and descriptive study was carried out at the ophthalmological service of Hermanos Ameijeiras hospital in a 21 case series study with moderate and severe eyelid retraction in the course of thyroid orbitopathy. The injection of botulinum toxin into the upper rectus muscle for the correction of retraction in thyroid orbitopathy was performed for the first time in the country. Results: in this group, 66.7 percent of patients had satisfactory results. Age and clinical severity of retraction were the factors having influence on the therapeutic response (p= 0.013 and p= 0.015 respectively). Conclusions: the botulinum toxin injection into the upper rectus muscle is effective in most of treated cases, mainly in young adults with less clinical retraction severity. Hypercorrection is the most fearful complication in this procedure(AU)

Toxina botulínica A en el músculo recto superior para la corrección de la retracción palpebral / Botulinum toxin A in the upper rectus muscle for the correction of eyelid retraction

Objetivo: evaluar los resultados terapéuticos obtenidos con la inyección de toxina botulínica en el músculo recto superior en pacientes con retracción palpebral moderada y grave en el curso de la orbitopatía tiroidea; determinar la influencia de algunos factores relacionados con estos e identificar las complicaciones y las reacciones adversas asociadas a su uso terapéutico. Métodos: se realizó un estudio descriptivo prospectivo en el Servicio de Oftalmología del Hospital Hermanos Ameijeiras en una serie de 21 casos con retracción palpebral moderada y grave en el curso de la orbitopatía tiroidea. La inyección de toxina botulínica en el músculo recto superior para la corrección de la retracción de la orbitopatía tiroidea se realiza por primera vez en el país. Resultados: fueron satisfactorios en el 66,7 por ciento de los casos. La edad y la gravedad clínica de la retracción fueron los factores que influyeron sobre la respuesta terapéutica (p= 0,013 y p= 0,015 respectivamente). Conclusiones: la inyección de toxina botulínica en el músculo recto superior es efectiva en la mayoría de los casos tratados, sobre todo en adultos jóvenes y con menor gravedad clínica de la retracción. La hipercorrección es la complicación más temida de este procedimiento(AU)

Objective: to evaluate the therapeutic results of the botulinum toxin injection in the upper rectus muscle in patients with moderate and severe eyelid retraction in the course of thyroid orbitopathy, and to determine the influence of some factors related to these patients and to identify the complications and adverse reactions associated to its therapeutic use. Methods: prospective and descriptive study was carried out at the ophthalmological service of Hermanos Ameijeiras hospital in a 21 case series study with moderate and severe eyelid retraction in the course of thyroid orbitopathy. The injection of botulinum toxin into the upper rectus muscle for the correction of retraction in thyroid orbitopathy was performed for the first time in the country. Results: in this group, 66.7 percent of patients had satisfactory results. Age and clinical severity of retraction were the factors having influence on the therapeutic response (p= 0.013 and p= 0.015 respectively). Conclusions: the botulinum toxin injection into the upper rectus muscle is effective in most of treated cases, mainly in young adults with less clinical retraction severity. Hypercorrection is the most fearful complication in this procedure(AU)

Relación Clínico-ecográfica de la Orbitopatía asociada a tiroides / Clinical and sonographic relationship associated with thyroid orbitopathy

Se busca cualificar y cuantificar los cambios clínicos no detectables en los estadíos iniciales de la enfermedad tiroidea, a través de la ecografía orbitaria y la ecografía Doppler-dúplex color; adicionalmente medir los valores hemodinámicos de la arteria y vena oftálmicas, con el fin de detectar modificaciones precoces observables en el nervio óptico. Estudio descriptivo, transversal, y correlacionalde 80 pacientes; 40 pacientes con diagnóstico de enfermedad tiroidea y 40 pacientes grupo control, a los que se les realizó ecografía orbitaria y Doppler color desde Julio a Diciembre. Del grupo con enfermedad tiroidea, 90% eran femeninos y 10% masculinos; 75% tenían entre 25-59 años, 45% hipertiroideos, 35% hipotiroideos y 10% eutiroideos; 35% presentaron hábitos tabáquicos positivos y 47,5% hábitos cafeínicos. El 87,3% tenían exoftalmometrías<23mm. Hubo reflectividad moderada en 136 músculos (43%) predominando en el complejo recto superior-elevador del párpado superior. El índice de pulsatibilidad, índice de resistencia y la velocidad pico sistólico de la arteria oftálmica se encontró elevados en los hipertiroideos. Los índices de la vena oftálmica como V1 y V2 estuvieron elevados en los tres grupos (hiper, hipo y eutiroideos). Concluyendo que el eco orbitario evalúa alteraciones cuantitativas y cualitativas tales como grosor muscular, alteraciones en la reflectividad interna de los mismos, edema asociados a las glándulas lagrimales, edema en la cabeza de los nervios ópticos, cambios en la grasa orbitaria, etc., los cuales son significativos en el diagnóstico y seguimiento de estos pacientes. El eco Doppler reveló alteraciones hemodinámicas importantes en parámetros índices de pulsatibilidad y velocidad pico sistólico en los pacientes hipertiroideos.

This paper seeks to qualify and quantify non detectable clinical changes in the initial stages of thyroid disease, through orbit ultrasound and Doppler color duplex ultrasonography; as well as to measure the hemodynamic values of the ophthalmic artery and vein for early detection of observable changes in the optic nerve. Descriptive, cross-sectional and correlational field study of 80 patients, 40 patients diagnosed with thyroid disease and 40 control group patients, who underwent orbital color Doppler ultrasound from July 2006 to December 2007. From the thyroid disease group, 90% were female and 10% male; 75% were between 25-59 years, 45% with hyperthyroidism, 35% with hypothyroidism and 10% euthyroid; 35% had positive smoking habits and 47.5% caffeine habits. 87.3% with <23mm of Exophtalmometry. Moderate reflectivity was present in 136 muscles (43%) with predominance in the upper-right lifting complex of the upper eyelid. Pulsatility index, resistance index and systolic peak velocity of the ophthalmic artery were elevated in hyperthyroidism subjects. Indices of the ophthalmic vein as V1 and V2 were elevated in all groups (hyper, hypo and euthyroid). It is concluded that the orbital eco evaluates quantitative and qualitative alterations such as edema and fibrosis, which are significant in the diagnosis and monitoring of these patients. The Doppler echo revealed significant hemodynamic changes in pulsatility index parameters and systolic peak velocity in hyperthyroidism patients.

Resultados del tratamiento de la orbitopatía tiroidea y factores que influyen sobre la respuesta terapéutica / Results of the treatment of thyroid orbinopathy and factors affecting the therapeutic response

Objetivo: evaluar los resultados del tratamiento de la orbitopatía tiroidea y determinar cuáles son los factores que influyen sobre ellos. Métodos: se realizó un estudio observacional en una cohorte de 82 pacientes con orbitopatía tiroidea, durante el período 1997-2009. Resultados: en los casos con orbitopatía tiroidea activa, la corticoterapia se inició a los 19 meses de evolución y no fue satisfactoria en la mayoría de ellos, por lo que para preservar la visión se realizaron varias intervenciones quirúrgicas. Los factores que influyeron sobre la respuesta terapéutica fueron el tabaquismo y la actividad de la orbitopatía tiroidea (OR= 30,479 y OR= 14,557, respectivamente). Se obtuvieron buenos resultados con el uso de la toxina botulínica A y con la cirugía en la corrección del estrabismo y la retracción. Conclusiones: el diagnóstico y el tratamiento precoz es la conducta terapéutica ideal para los pacientes con orbitopatía tiroidea. El tabaquismo y la actividad de la orbitopatía tiroideainterfirieren en la respuesta terapéutica. La toxina botulínica A y la cirugía son efectivas en la corrección del estrabismo y la retracción palpebral de la orbitopatía tiroidea(AU)

Objective: to evaluate the results of the treatment of thyroid orbinopathy and to determine the factors affecting them. Methods: observational study of a cohort of 82 patients with thyroid orbinopathy during the 1997-2009 period. Results: corticosteroid-bases therapy began nineteen months after the onset of disease and was unsatisfactory in most of cases with active thyroid orbitopathy, so it was necessary to perform several surgeries to preserve the vision. The influential factors on the therapeutic response were smoking and thyroid orbinopathy activity (OR= 30,479 and OR= 14,557, respectively). Good results were attained with botilinum toxin A and strabismus correction surgery and retraction. Conclusions: early diagnosis and treatment is the best therapy for patients with thyroid orbinopathy. Smoking and thyroid orbinopathy negatively affect the therapeutic response. Botulinum toxin A and surgery are effective options in the strabismus correction and palpebral retractions of thyroid orbinopathy(AU)

Oftalmopatía de Graves y tratamiento con I131 / Graves´ Ophthalmopathy and Radioiodine Treatment

La oftalmopatía de Graves (OG) es un desorden autoinmune que representa la manifestación extratiroidea más frecuente de la Enfermedad de Graves. Se describen múltiples factores que pueden influir en el desarrollo/progresión de la enfermedad, entre ellos el tratamiento con I131. El objetivo de esta monografía es analizar la bibliografía existente sobre la relación entre OG y tratamiento con I131, teniendo en cuenta su fisiopatología así como los factores de riesgo asociados y su profilaxis.

Graves´ ophthalmopathy (GO) is an autoimmune disorder representing the most common extrathyroidal manifestation of Graves' disease. Multiple factors can influence the development / progression of the disease, including treatment with 131I. The aim of this paper is to analyze the existing literature on the relationship between OG and treatment with I131, considering the pathophysiology and associated risk factors and prophylaxis.