Actualización en anomalías vasculares de la órbita en pediatría: diagnóstico por imágenes y tratamiento / Update on orbital vascular anomalies in pediatrics: imaging studies and management

Las anomalías vasculares de la órbita (AVO) son un grupo heterogéneo de patologías que pueden presentarse con frecuencia en el cono orbitario, la región periorbitaria o dentro de la órbita misma. Las AVO se dividen en tumores y malformaciones. Su presentación clínica más frecuente es el exoftalmos, asociado o no a alteración del eje visual. Además, pueden presentar complicaciones agudas, como hemorragia intralesional o celulitis entre las más frecuentes, y complicaciones crónicas, como ambliopía y afectación de la agudeza visual a largo plazo. La evolución de las técnicas de imágenes, el uso de nuevos fármacos y la utilización de innovadores procedimientos en radiología intervencionista han posibilitado obtener una mejora significativa en los procesos diagnósticos y terapéuticos de estos pacientes, permitiendo un diagnóstico y tratamiento preciso.

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment. The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management.

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.

Bilateral cavernous sinus and left dural sigmoid sinus thrombosis associated with extreme exertion: a case report / Trombose bilateral do seio cavernoso e do seio sigmoide esquerdo associada a exercício extremo: relato de caso

ABSTRACT Septic cavernous sinus thrombosis is a rare but often debilitating and potentially fatal disease. We describe a case of bilateral orbital cellulitis with rapidly progressing cavernous sinus thrombosis and left sigmoidal sinus thrombosis in an immunocompetent 20-year-old military man who had undergone intensive physical training. The patient presented with rapid painful swollen left eye for 2 days. The examination results were gross proptosis with total ophthalmoplegia. He was treated with intravenous antibiotics and corticosteroid. At 1 week, visual acuity improved to 20/20 OU, with a normal intraocular pressure. There was a significant improvement in proptosis. The ocular motility of the right eye was fully restored, with slight residual ophthalmoplegia in the left eye. There was no residual illness or recurrence of illness at 3 months' follow-up.

RESUMO A trombose séptica do seio cavernoso é uma condição rara, mas frequentemente debilitante e potencialmente fatal. Descrevemos um caso de celulite orbital bilateral com progressão rápida para trombose do seio cavernoso e trombose do seio sigmoide esquerdo, em um militar imunocompetente de 20 anos de idade que havia sido submetido a treinamento físico intenso. O paciente apresentou um inchaço rápido e doloroso no olho esquerdo por 2 dias. Os resultados do exame foram proptose macroscópica com oftalmoplegia total. Ele foi tratado com antibióticos intravenosos e costicosteróide. Em 1 semana, a acuidade visual melhorou para 20/20, com pressão intraocular normal. Houve uma melhora significativa na proptose. A motilidade ocular do olho direito foi totalmente restaurada, com leve oftalmoplegia residual no olho esquerdo. Não houve doença residual ou recorrência da doença após três meses de acompanhamento.

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.

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

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

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

Mucormicosis rino-órbito-cerebral crónica: una patología poco frecuente / Chronic rhino-orbital-cerebral mucormycosis: an uncommon pathology

Resumen La mucormicosis rino-órbito-cerebral (ROC) crónica es una patología poco frecuente, con un número reducido de casos publicados en la literatura, cuyas manifestaciones son muy diversas e inespecíficas. El tratamiento se basa en la experiencia de casos y series de casos. Las herramientas terapéuticas incluyen el uso de antifúngicos endovenosos y orales por tiempo prolongado, asociado o no a debridamiento quirúrgico amplio, pudiendo requerir incluso exenteración orbitaria. Presentamos a continuación un caso de mucormicosis ROC crónica, junto con las dificultades para su diagnóstico y manejo, en el que destaca el enfrentamiento multidisciplinario. Dada la poca frecuencia de esta enfermedad, nos parece relevante difundirlo.

Abstract Chronic rhino-orbital-cerebral mucormycosis is a rare condition with a small number of cases that have been published, whose manifestations are very diverse and nonspecific. The treatment is based on case series experiences. Therapeutic options include the use of long-term intravenous and oral antifungals, associated or not with extensive surgical debridement, and may even require orbital exenteration. We present below a case of chronic rhino-orbital-cerebral mucormycosis with the challenge of diagnosis and management in which multidisciplinary work is fundamental. Since it is an uncommon pathology, it seems relevant to share the information.

Schwannoma orbitario en un niño con proptosis aguda / Orbital schwannoma in a child with acute proptosis

El schwannoma es un tumor primario, habitualmente, benigno, procedente de las células de Schwann, productoras de la vaina de mielina que recubre los nervios periféricos. Constituye menos del 10 % de los tumores intracraneales y es infrecuente en la edad pediátrica.Se presenta a un paciente de 6 años y 11 meses de edad, previamente sano, con antecedente de cefalea holocraneana intermitente asociado a proptosis y disminución de la agudeza visual del ojo izquierdo, epífora y estrabismo, con evidencia tomográfica de una masa retroocular. Se realizó la exéresis macroscópicamente completa, con diagnóstico anatomopatológico de schwannoma orbitario

Schwannoma is a usually benign primary tumor. It develops from the Schwann cells, which produce the myelin sheath that surrounds the peripheral nerves. It represents less than 10 % of the intracranial tumors, and it is infrequent in the pediatric age.We hereby present a 6-year-and-11-month-old previously healthy patient, with a history of intermittent generalized cephalea associated with proptosis and a diminished visual acuity of the left eye, epiphora and strabismus, with radiological evidence of retro-ocular mass. A macroscopically complete exeresis was performed, with an anatomopathological diagnosis of orbital schwannoma

Proptosis ocular como una manifestación de la granulomatosis con poliangeítis (Wegener): Reporte de un caso y revisión de la literatura / Ocular proptosis as a manifestation of Wegener's granulomatosis: A case report

RESUMEN La granulomatosis con poliangeítis (Wegener) es considerada como una enfermedad granulomatosa sistémica, no infecciosa, caracterizada histológicamente por una vasculitis necrosante de pequeño vaso. El tracto respiratorio superior e inferior son los más frecuentemente afectados, en asociación a manifestaciones renales. Sin embargo, también se describe el compromiso aislado de un solo órgano, como es el caso del globo ocular y la órbita. Presentamos el caso de una mujer cuya principal manifestación consistió en un proceso inflamatorio de tejido periorbitario y proptosis del globo ocular izquierdo. La paciente fue valorada en manejo conjunto con el servicio de plástica ocular, otorrinolaringología y finalmente se derivó a reumatología para inicio de terapia inmunosupresora. Se realizó una revisión de la literatura, en búsqueda de reportes de caso y series de caso que permitieran comparar las presentaciones clínicas y desenlaces más frecuentes.

A B S T R A C T Granulomatosis with polyangiitis (Wegener) is considered as a systemic, non-infectious granulomatous disease, histologically noted for its small vessel necrotising vasculitis. The upper and lower respiratory tracts are the most frequently affected, in association with renal manifestations. However, the isolated involvement of a single organ, such as the eyeball and the orbit is also described. The case is presented of a woman whose main manifestation was an inflammatory process of periorbital tissue and proptosis of the left eyeball. The patient was evaluated jointly with eye plastic surgery, and the ear, nose and throat, and rheumatology departments. A literature review was carried out, looking for case reports and case series that allowed comparisons to made between the clinical manifestations and the most frequent outcomes.

Zigomicosis y síndrome hemofagocítico en un paciente inmunocompetente: informe de caso / Zygomycosis and hemophagocytic lymphohistiocytosis in an immunocompetent patient: a case report

Introducción: la zigomicosis es una infección fúngica poco frecuente, con alta tasa de mortalidad y de mal pronóstico. Afecta principalmente a pacientes inmunocomprometidos. La asociación con el síndrome hemofagocítico es extremadamente inusual, más aún en pacientes inmunocompetentes, con pocos ejemplos registrados en la literatura. Caso clínico: se presenta el caso de un paciente masculino inmunocompetente de 40 años con diagnóstico de mucormicosis y síndrome hemofagocítico que evoluciona desfavorablemente, con fallo multiorgánico, a pesar de los esfuerzos médicos. Conclusión: la asociación de mucormicosis con síndrome hemofagocítico en un paciente inmunocompetente es extremadamente rara; existen pocos casos informados en Latinoamérica. Debemos tener presente esta asociación, ya que requiere un tratamiento agresivo y soporte vital avanzado. (AU)

Introduction: zygomycosis is a rare fungal infection that carries with high mortality rates. This poor prognosis, rapidly progressive infection mainly affects immunocompromised patients. The association with hemophagocytic lymphohistiocytosis is extremely unusual, even more in immunocompetent patients, with few cases reported. Case: we present the case of an immunocompetent male patient who was diagnosed with zygomycosis and hemophagocytic lymphohistiocytosis. Despite medical efforts he developed multiorganic failure. Conclusion: the association of mucormycosis with hemophagocytic lymphohistiocytosis in an immunocompetent patient is exceptional with few cases reported in Latin America. We must always suspect this association considering they require aggressive treatment and advanced life support. (AU)

Bilateral metastases to the extraocular muscles from small cell lung carcinoma / Metástases bilaterais aos músculos extraoculares de um carcinoma de pequenas células do pulmão

ABSTRACT Bilateral orbital metastases restricted to the extraocular muscles (EOMs) are exceedingly rare. We report a case of bilateral extraocular muscle metastases from a small cell lung carcinoma and provide a review of the relevant literature. A 56-year-old smoker presented with proptosis, motility changes, and a relative afferent pupillary defect of the left eye, with a previous history of a small cell lung carcinoma. An orbital computerized tomography scan revealed a mass restricted to the left medial rectus. An incisional biopsy confirmed metastasis. Visual acuity of the left eye decreased rapidly, and right globe proptosis became evident. Orbital magnetic resonance imaging at two months follow-up showed marked left orbital mass enlargement and a new right lateral rectus mass. The patient was maintained on palliative care and died from metastatic disease-related complications.

RESUMO As metástases orbitárias bilaterais restritas aos mús­culos extraoculares são extremamente raras. Os autores apresentam um caso de metástases bilaterais, localizadas aos musculares extraoculares com base num carcinoma de pequenas células do pulmão e revisão da literatura relevante. Um homem, fumador, de 56 anos recorreu ao serviço de urgência por proptose, alterações de motilidade ocular extrínseca e um defeito pupilar aferente relativo do olho esquerdo, com história pessoal de carcinoma de pequenas células do pulmão. A tomografia computadorizada orbitária revelou uma massa restrita ao reto medial esquerdo. Uma biópsia incisional confirmou o diagnóstico de metástase. A acuidade visual do olho esquerdo diminuiu rapidamente e surgiu uma proptose do globo ocular direito. A ressonância magnética orbitária aos dois meses de seguimento revelou um aumento da massa orbitária esquerda e uma nova massa no reto lateral direito. O paciente foi mantido em cuidados paliativos e faleceu devido a complicações relacionadas com doença metastática.

Paquimeningitis y glomerulonefritis crescéntica en vasculitis p-ANCA / Pachymeningitis and crescentic glomerulonephritis in p-ANCA vasculitis

Resumen Las vasculitis asociadas a anticuerpos contra el citoplasma del neutrófilo configuran patologías multisistémicas caracterizadas por afectar pequeños vasos. Se ha descrito compromiso de sistema nervioso central en 2-8% de los casos, rara vez al inicio del cuadro clínico. Este corresponde al caso de un hombre de 67 años de edad con vasculitis p-ANCA, quien debutó con proptosis, paquimeningitis y disfagia severa. Posteriormente deterioro renal, necesidad de hemodiálisis, ciclofosfamida y recambio plasmático terapéutico con lo cual alcanza criterios de remisión parcial y egresa de diálisis. Las vasculitis ANCA se relacionan con una amplia gama de signos y síntomas inespecíficos. La edad de aparición, creatinina inicial y requerimiento de diálisis, son factores pronósticos. El inicio oportuno del tratamiento es fundamental, requiriendo por tanto un alto índice de sospecha. (Acta Med Colomb 2018; 43: 107-110).

Abstract Vasculitis associated with antibodies against the neutrophil cytoplasm they form multisystemic pathologies characterized by affecting small vessels. Central nervous system involvement has been described in 2 - 8% of cases, rarely at the beginning of the clinical picture. This corresponds to the case of a 67-year-old man with p-ANCA vasculitis, who debuted with proptosis, pachymeningitis and severe dysphagia. Subsequently, renal deterioration, the need for hemodialysis, cyclophosphamide and therapeutic plasma exchange, with which it reaches criteria of partial remission and dialysis discharge. ANCA vasculitis is associated with a wide range of nonspecific signs and symptoms. The age of onset, initial creatinine and dialysis requirement are prognostic factors. The timely initiation of treatment is fundamental, requiring therefore a high index of suspicion. (Acta Med Colomb 2018; 43: 107-110).

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.

Endovascular Treatment of a Giant Dissecting Aneurysm in an Infant

Introduction The incidence of intracranial aneurysms in the pediatric population is low, and endovascular treatment is becoming a safe and minimally invasive treatment option. In the present study, the occurrence of special features of cerebral aneurysm in children, in comparison to adults, is also described. Case Report A 3-month-old female infant presented with progressive proptosis and divergent strabismus at the right eye, in addition to inconsolable crying. Cerebral resonance, angiotomography and angiography exams demonstrated angiodysplasia in the right internal carotid artery with two large paraclinoid dissecting aneurysms with wide neck. The right internal carotid artery was occluded with coils by endovascular approach, without detriment to the perfusion of the ipsilateral hemisphere and without neurological deficits. The patient achieved good recovery, and a late control angiotomography confirmed the exclusion of the aneurysms. Conclusion Parent artery sacrifice via endovascular approach is an effective therapeutic option, but a long-termfollow-up is necessary to avoid recurrence and bleeding.

Paciente com proptose ocular associado a mucocele etmoidal: apresentação clínica e tratamento / Patient with ocular properties associated with ethmoidal mucocele: clinical presentation and treatment

Resumo Relato de um caso clínico de proptose ocular relacionado com mucocele etmoidal, o caso é raro e seu acometimento ocular não é comumente relacionado na literatura. Ressaltamos ainda sua apresentação clínica e o tratamento instituído. W.L.S, Paciente do sexo masculino, 27 anos procedente de Fortaleza-CE. Comparece ao ambulatório HUWC com queixa de baixa da acuidade visual percebida há 10 dias em olho direito (OD). Ao exame oftalmológico melhor acuidade visual corrigida de 20/200 OD e 20/20 olho esquerdo (OE), ausência de abdução e elevação do OD com ptose leve (distância margem reflexo 1-2 mm) e diplopia ao olhar para direita. Proptose OD de aspecto axial de grau moderado (24mm). Biomicroscopia lâmpada de fenda sem alterações. Pressão intraocular: 18mmHg OD 12 mmHg OE. Fundo de olho: OD aspecto palidez discreta de disco, sem outras alterações e OE dentro da normalidade. Solicitada tomografia de crânio (TC) que demonstrou presença de lesão arredondada em área do seio etmoidal penetrando parede óssea da órbita do OD sugestiva de mucocele etmoidal. Encaminhado para serviço de otorrinolaringologia do HUWC, sendo submetido a procedimento cirúrgico endoscópico etmoidectomia. Um mês após o procedimento cirúrgico a acuidade visual OD 20/25, movimentos oculares presentes e simétricos, ausência de diplopia e persistência de leve palidez em OD. Expansão da mucocele pode ocorrer ao longo de muitos anos ou rapidamente. Acometimento ocular com baixa da acuidade visual é incomum como primeiro sintoma, quando ocorre merece tratamento imediato. O tratamento cirúrgico é geralmente indicado no caso e deve ser instituído assim que possível para adequada recuperação visual.

Abstract Report of a clinical case of ocular proptosis related to ethmoidal mucocele, the case is rare and ocular involvement is not commonly related in the literature. We also emphasize its clinical presentation and the treatment instituted. W.L.S., male, 27 years old from Fortaleza-CE. He presents to the HUWC outpatient clinic complaining of low visual acuity perceived for 10 days in the right eye (OD). The ophthalmologic exam showed a best corrected visual acuity of 20/200 OD and 20/20 left eye (OS), absence of abduction and elevation of OD with light ptosis (distance margin 1 - 2 mm) and diplopia when looking to the right. Proptose OD of moderate aspect axial aspect (24mm). Biomicroscopy slit lamp without changes. Intraocular pressure: 18mmHg OD 12 mmHg OS. Background of eye: OD discrete pallor disk appearance, no other changes and OS within normality. A CT scan was performed that showed presence of a rounded lesion in the ethmoidal sinus area penetrating the bone wall of the OD orbit suggestive of ethmoidal mucocele. Forwarded to the otorhinolaryngology service of the HUWC, being submitted to endoscopic surgical procedure etmoidectomy. One month after the surgical procedure visual acuity of OD 20/25, present and symmetrical ocular movements, absence of diplopia and persistence of mild pallor in OD. Expansion of mucocele may occur over many years or rapidly. Ocular involvement with low visual acuity is uncommon as the first symptom, when it occurs it deserves immediate treatment. Surgical treatment is generally indicated in the case and should be instituted as soon as possible for adequate visual recovery.

Caso Clínico: Uso de Biomodelos Tridimensionales para la Planificación Preoperatoria de Tumores Craneales Fronto - orbitarios / Case Report: Use of Tridimensional Biomodels for Surgical Planning in Fronto-orbital Bone Tumors

INTRODUCCIÓN: La impresión tridimensional de biomodelos ha demostrado en los últimos años ser de gran utilidad para el diagnóstico, tratamiento y planificación preoperatoria en prácticamente todas las especialidades quirúrgicas. En este reporte se presenta la experiencia inicial con el empleo de biomodelos tridimensionales, para la planificación pre quirúrgica de un paciente con displasia fibrosa fronto-orbitaria operado en Cuenca, Ecuador. CASO CLÍNICO: Paciente de sexo masculino de 11 años de edad que desde hace 4 años presentó una¿ masa frontal derecha dura, inmóvil, no dolorosa, de crecimiento progresivo, que produjo deformidad orbitaria con exoftalmia e hipotropia. La tomografía craneal demostró una lesión ósea de núcleo hipodenso, de 5 cm de diámetro mayor, con compromiso del techo de la órbita, porción lateral del seno paranasal frontal y extensión intracraneal extradural. EVOLUCIÓN: Para planificar la cirugía se elaboró un modelo óseo tridimensional que se usó para explicar al paciente y sus padres el objetivo del procedimiento y como se realizaría. El día de la intervención, se dibujaron las osteotomías y craneotomía en el modelo anatómico, plan que se aplicó exactamente en el paciente. El postoperatorio transcurrió sin novedades, la tomografía computarizada de control evidenció una resección completa de la lesión y una adecuada reconstrucción orbitaria. El paciente y sus familiares se mostraron muy satisfechos con las explicaciones dadas. CONCLUSIONES: La impresión 3D es una herramienta que cada vez gana más espacio en la docencia y también en la planificación quirúrgica pues permite disponer de modelos anatómicos muy precisos y simular el procedimiento operatorio, antes de realizar el procedimiento en el paciente real. (AU)

BACKGROUND: 3Dprinting of biomodels has shown in recent years to be very useful for diagnosis,treatmentandpreoperativeplanninginpracticallyall surgical specialties. Inthis reportispresentedthe initial experiencewiththeuseoftridimensionalbiomodels,forpre-surgicalplanninginapatientwithfronto-orbital fibrous dysplasia operated in Cuenca, Ecuador. CASE REPORT: An 11 years old boy presented with a 4-year history of a slow-growing, hard, non-mobile, painless rightfrontalmass which caused orbital deformity, proptosis, and hypotropia. Cranial Computer Tomography showed a 5 cm bone tumor with hypodense center compromising the orbital roof and the lateral aspect ofthe frontal paranasal sinus with intracranial extradural expansion. EVOLUTION: To design the surgery, a tridimensional bone model was elaborated and used to explain the patient and his parents the aim of the procedure and how it will be performed. The day of the intervention, the osteotomies and craniotomy were drawn on the anatomical model, plan that was exactly applied to the patient. The postoperative period was uneventful, control CT scan showed a complete resection of the lesion and an adequate orbital reconstruction. The patient and his relatives were very satisfied with the explanations given. CONCLUSIONS: 3D printing is a very useful surgical tool with wide applications in planning and education that allows simulate in very accurate biomodels an operative procedure before it was done in the actual patient(AU)

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)