RESUMO
BACKGROUND: Thyroid eye disease (TED) is a condition caused by inflammatory damage to the periocular tissue that often leads to double vision. Teprotumumab is an insulin-like growth factor 1 receptor antibody that was FDA approved for the management of TED in 2020, although much is yet to be elucidated regarding its effects on diplopia outcomes among patients with TED. Diplopia is a significant and life-altering effect of TED. Previous studies have reported the effect of teprotumumab on double vision subjectively using the Gorman diplopia score. However, there is a gap in the literature addressing the effect of teprotumumab treatment on objective ocular alignment measures. The purpose of our study was to address this gap. METHODS: We performed a retrospective review of patients who were diagnosed with TED, presented with diplopia, and treated with teprotumumab in a single-center academic ophthalmology practice. The primary outcome was change in ocular alignment in primary gaze position at 6 months (completion of teprotumumab treatment). Secondary outcomes included change in ocular alignment in other gaze positions, proptosis, eyelid position, and clinical activity score (CAS) at 6 months compared with baseline. To determine what factors may predict ocular alignment response to teprotumumab, we analyzed baseline characteristics among 3 groups, divided based on whether ocular alignment was worsened, stable, or improved at 6 months. RESULTS: Seventeen patients met inclusion criteria, 3 (18%) worsened, 10 (59%) were stable, and 4 (24%) improved. CAS ( P = 0.02) was significantly different among the groups and was higher in those who worsened and those who improved compared with those who remained stable. Right gaze horizontal prism deviation ( P = 0.01) and left gaze horizontal prism deviation ( P = 0.03) were significantly different among the groups, with a greater degree of left gaze horizontal prism deviation in the worse group than the stable group ( P = 0.04). CONCLUSIONS: Our study demonstrated that most patients remained stable after teprotumumab treatment regarding ocular alignment in primary gaze and the number of patients who improved was slightly higher than the number of patients who worsened after teprotumumab treatment. There are some baseline measures, such as CAS and right and left gaze horizontal prism deviation that can help better predict how a patient will respond to teprotumumab treatment. Our results can better inform physicians of how to counsel patients with TED when considering teprotumumab therapy.
Assuntos
Exoftalmia , Oftalmopatia de Graves , Humanos , Oftalmopatia de Graves/complicações , Oftalmopatia de Graves/tratamento farmacológico , Diplopia/diagnóstico , Diplopia/tratamento farmacológico , Diplopia/etiologia , Anticorpos Monoclonais Humanizados/uso terapêutico , Exoftalmia/complicaçõesRESUMO
Paciente varón de 50 años quien tras 3 meses de un traumatismo encefalocraneano presenta proptosis, quemosis y exoftalmos en el ojo izquierdo. Posteriormente desarrolla dismetría en las extremidades izquierdas y hemiparesia derecha. Se establece el diagnóstico de fístula carótido-cavernosa (FCC) asociado a hiperintensidad de señal en FLAIR y captación difusa de contraste al nivel de la protuberancia y pedúnculo cerebeloso, de aspecto pseudotumoral. Dicho hallazgo fue compatible con congestión venosa. Sus síntomas fueron fluctuantes; comenzó con síntomas orbitarios y luego de territorio posterior con mejoría de los síntomas orbitarios. La embolización con microcoils de la FCC produjo la resolución de los síntomas oculares seguido por mejoría de los síntomas de tronco encefálico. Los hallazgos en resonancia magnética revirtieron considerablemente al año de seguimiento. Describimos un caso de FCC directa con congestión venosa en el tronco encefálico y síntomas fluctuantes con una considerable mejoría clínica e imagenológica después del tratamiento
A 50-year-old male patient who, after 3 months of cranial brain trauma, presented proptosis, chemosis and exophthalmos in the left eye. Subsequently, dysmetria develops in the left extremities and right hemiparesis. The diagnosis of carotid-cavernous fistula (FCC) associated with hyperintensity of signal in FLAIR and diffuse contrast uptake at the level of the pseudo tumoral protuberance and cerebellar peduncle was established. This finding was compatible with venous congestion. His symptoms were fluctuating, he started with orbital symptoms and then from the posterior fossa with improvement of the orbital symptoms. FCC microcoil embolization produced resolution of ocular symptoms followed by improvement of brainstem symptoms. Magnetic resonance findings significantly reversed one year of follow-up. We describe a case of direct FCC with venous congestion in the brainstem and fluctuating symptoms with a considerable clinical and imaging improvement after treatment
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Fístula Carótido-Cavernosa/complicações , Fístula Carótido-Cavernosa/cirurgia , Infarto Cerebral/etiologia , Tronco Encefálico/cirurgia , Tronco Encefálico/diagnóstico por imagem , Tronco Encefálico/patologia , Traumatismos Cranianos Penetrantes/complicações , Exoftalmia/complicações , Espectroscopia de Ressonância Magnética , Crânio/diagnóstico por imagem , Embolização TerapêuticaRESUMO
No disponible
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Hemangioma Cavernoso/diagnóstico por imagem , Hemangioma Cavernoso/cirurgia , Neoplasias Orbitárias/diagnóstico por imagem , Neoplasias Orbitárias/cirurgia , Complicações Pós-Operatórias , Exoftalmia/complicações , Exoftalmia/patologia , Diplopia/complicações , Blefaroptose/complicações , Exotropia/diagnóstico por imagem , Exotropia/patologiaRESUMO
CASO CLÍNICO: Presentamos el caso de una mujer de 64 años con tumefacción palpebral, dacrioadenitis bilateral y exoftalmos, e historia de rinitis crónica y asma bronquial. Se evidenció aumento de IgG4 sérica y se realizó biopsia incisional de glándulas lagrimales que demostró fibrosis e infiltrado linfoplasmocitario con células productoras de IgG4. Discusión: El compromiso orbitario en enfermedad relacionada con IgG4 es frecuente. La dacrioadenitis bilateral es la manifestación más común. La histopatología es esencial para el diagnóstico de la enfermedad y excluir malignidad
CASE REPORT: The case is presented of a 64-year-old woman with bilateral palpebral swelling and dacryoadenitis, exophthalmos, and a history of chronic rhinitis and asthma. An increase in serum IgG4 was observed, and an incisional biopsy of lacrimal glands was performed, which showed fibrosis and a lymphoplasmacytic infiltrate with IgG4 producing cells. DISCUSSION: Orbital involvement in IgG4-related disease is frequent. Bilateral dacryoadenitis is the most common manifestation. Histopathology is essential for the diagnosis and to exclude malignancy
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Doenças Orbitárias/diagnóstico , Doenças Orbitárias/tratamento farmacológico , Imunoglobulina G/imunologia , Xeroftalmia/diagnóstico , Aparelho Lacrimal/cirurgia , Biópsia , Dacriocistite/complicações , Exoftalmia/complicações , Anemia/complicações , Hipergamaglobulinemia/complicações , Imuno-Histoquímica , Diagnóstico DiferencialRESUMO
La enfermedad relacionada con IgG4 (ER-IgG4) es una condición clínica recientemente reconocida, con múltiples aspectos aún no dilucidados. Se caracteriza por el compromiso fibroinflamatorio de múltiples órganos; con hallazgos clínicos, serológicos e histopatológicos que representa un importante reto para el clínico. Clásicamente descrita como una lesión tumoral expansiva con fibrosis estoriforme, infiltración linfoplasmocítica (IgG4 positiva) e IgG4 sérica elevada. Las características clínicas son variables, se describe tanto compromiso pancreático como extrapancreático, es de predominio en varones asiáticos mayores de 50 años, y rara vez es descrita en personas de raza negra. Presentamos el caso de una mujer, adolescente, afro-colombiana, que presenta protrusión ocular unilateral inexplicable, con hallazgos histopatológicos que revelan infiltración de células linfocíticas y plasmáticas en la glándula lacrimal, con positividad para IgG4, descartándose otras condiciones, lo que confirma una dacrioadenitis por enfermedad relacionada con IgG4
IgG4-related disease (IgG4-RD) is a recently recognized clinical condition with multiple aspects not yet elucidated. It is characterized by a fibrous inflammatory process that involves multiple organs and clinical, serological and histopathological findings, which represent a major challenge for the clinician. Classically described as an expansive tumor lesion with storiform fibrosis, lymphoplasmacytic infiltration (IgG4-positive) and elevated serum IgG4. Clinical features are variable, and pancreatic as well as extrapancreatic involvement has been reported, more frequently in Asian men over 50 years and rarely described in black people. We report the case of an Afro-Colombian teenage woman, who had a unilateral ocular protrusion of unknown cause, with histopathologic findings that revealed infiltration of lymphocyte and plasma cells into the lacrimal gland. It was positive for IgG4, ruling out other conditions, and confirming IgG4-related dacryoadenitis
Assuntos
Humanos , Feminino , Adolescente , Dacriocistite/diagnóstico , Dacriocistite/imunologia , Exoftalmia/complicações , Aparelho Lacrimal/citologia , Biópsia , Doenças Autoimunes/diagnóstico , Imunoglobulina G/imunologia , Exoftalmia/imunologia , Órbita/diagnóstico por imagem , Biomarcadores/metabolismo , Medula Óssea/diagnóstico por imagem , Medula Óssea/imunologia , Ensaio de Imunoadsorção Enzimática , Dacriocistite/tratamento farmacológico , Azatioprina/uso terapêutico , Imuno-Histoquímica/métodosRESUMO
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.
Assuntos
Humanos , Feminino , Lactente , Artéria Carótida Interna/cirurgia , Aneurisma Intracraniano/cirurgia , Procedimentos Endovasculares/métodos , Dissecção Aórtica/cirurgia , Exoftalmia/complicações , Exotropia/complicações , Angiografia por Tomografia ComputadorizadaRESUMO
No disponible
Assuntos
Humanos , Feminino , Criança , Adenoma/patologia , Adenoma , Neoplasias Maxilares/cirurgia , Neoplasias Maxilares , Exoftalmia/complicações , Biópsia/métodos , Neoplasias Maxilares/patologia , Artéria Carótida Externa/patologiaRESUMO
No disponible
Assuntos
Humanos , Feminino , Adulto , Hematoma/cirurgia , Hematoma , Hiperêmese Gravídica/complicações , Transtornos da Visão/complicações , Hematoma/etiologia , Exoftalmia/complicações , Midríase/complicações , Diagnóstico DiferencialRESUMO
No disponible
Assuntos
Humanos , Masculino , Lactente , Hemangioma/complicações , Hemangioma/tratamento farmacológico , Exoftalmia/complicações , Exoftalmia , Midazolam/uso terapêutico , Anestesia , Hemangioma , Sons Respiratórios/fisiopatologia , Fadiga/complicações , Espectroscopia de Ressonância Magnética/métodos , Capnografia/métodos , Hemangioma/fisiopatologiaRESUMO
CASO CLÍNICO: Se presenta el caso de un varón de 34 años que consultó por dolor en canto interno de ojo izquierdo, con diplopía y proptosis progresiva. Con exoftalmos y masa palpable en canto interno, la tomografía computarizada reveló una lesión que la biopsia confirmó como fibroma osificante. DISCUSIÓN: El fibroma osificante es una lesión fibro-ósea benigna que afecta en su mayoría a la mandíbula. La presentación clínica y sus complicaciones varían según su localización. La clínica, tomografía computarizada e histopatología son fundamentales para el diagnóstico definitivo. El tratamiento quirúrgico es multidisciplinario y el seguimiento es a largo plazo
CLINICAL CASE: The case concerns a 34 year-old man, who presented with pain in the medial canthus in his left eye, with proptosis and diplopia. The examination showed exophthalmus and a palpable mass at the inner canthus. The computed tomography revealed a lesion, which was confirmed by biopsy to be a ossifying fibroma. DISCUSSION: Ossifying fibroma is a benign fibro-osseous lesion that mostly affects the jaw. Clinical presentation and complications vary according to its location. Clinical examination, imaging, and histopathology are essential for definitive diagnosis. Surgical treatment is multidisciplinary and long-term follow up is needed
Assuntos
Humanos , Masculino , Adulto , Fibroma Ossificante/cirurgia , Fibroma Ossificante , Diplopia/complicações , Diplopia/cirurgia , Diplopia , Exoftalmia/complicações , Exoftalmia , Tomografia/métodos , Acuidade Visual/efeitos da radiaçãoRESUMO
CASO CLÍNICO: Presentamos el caso de un osteoma frontoetmoidal derecho con invasión intraorbitaria en un varón de 43 años que comenzó como un cuadro de dolor retroocular con proptosis, inflamación peripalpebral y diplopía binocular por limitación del recorrido muscular. Se realizó cirugía abierta mediante abordaje subcraneal anterior, con extirpación de varios fragmentos de osteoma, cuyo análisis histológico confirmó el diagnóstico. DISCUSIÓN: Los osteomas son tumores benignos formadores de hueso de crecimiento lento. Habitualmente son asintomáticos, hasta que producen complicaciones por efecto masa en el cerebro o en la órbita, o a nivel local, hasta que obstruyen el drenaje del seno donde asientan, generando mucoceles
CASE REPORT: A case is presented of a right frontoethmoidal osteoma with intraorbital invasion in a 43-year-old male, who was seen in the clinic with a proptosis, retro-orbital pain, peri-palpebral inflammation, and binocular diplopia due to muscular route limitation. Open surgery was performed using a subcranial approach, with removal of several fragments of osteoma. Histological analysis confirmed the diagnosis. DISCUSSION: Osteomas are benign bone-forming tumours with slow growth. They are usually asymptomatic until mass effect complications occur in the brain or in the orbit, or locally, generating mucoceles due to sinus drainage obstruction
Assuntos
Humanos , Masculino , Adulto , Osteoma/complicações , Osteoma/tratamento farmacológico , Osteoma , Exoftalmia/complicações , Diplopia/complicações , Diplopia/tratamento farmacológico , Toxinas Botulínicas Tipo A/uso terapêutico , Seio Etmoidal/patologia , Seio Etmoidal , Combinação Amoxicilina e Clavulanato de Potássio/uso terapêutico , Prednisona/uso terapêutico , Omeprazol/uso terapêutico , Dipirona/uso terapêuticoRESUMO
CASO CLÍNICO: Mujer de 38 años, que durante una inmersión a 7 m presenta dolor ocular, hiperemia y exoftalmos. Fue diagnosticada de barotrauma con diplopia transitoria. Acude a urgencias 10 días después sin diplopia, pero leve exoftalmos izquierdo. En la tomografía computarizada se observa imagen de ocupación extraconal en techo orbitario izquierdo. Posteriormente se realiza una resonancia magnética y se confirma la presencia de hematoma intraorbitario. Discusión: El barotrauma ocular es normalmente una condición benigna producida en su mayoría por inexperiencia del buceador. Sin embargo, es importante una exploración minuciosa para descartar lesiones potencialmente dañinas para la visión o para la vida
CASE REPORT: A 38-year-old woman who, during a scuba dive at 7 metres, suffered from eye pain, hyperaemia, and exophthalmos. She was diagnosed with ocular barotrauma with transient diplopia. She was seen in the emergency room 10 days later, with no diplopia, but mild left proptosis. In the computed tomography an image of extraconal occupation is observed in the left orbital roof. The magnetic resonance confirmed an intraorbital haematoma. Discussion: Ocular barotrauma is usually a benign condition mostly occurring in an inexperienced diver. However, a detailed examination is important to rule out potential vision and life threatening conditions
Assuntos
Humanos , Feminino , Adulto , Diplopia/complicações , Diplopia , Barotrauma/complicações , Barotrauma , Exoftalmia/complicações , Exoftalmia , Hematoma/complicações , Dor Ocular/complicações , Dor Ocular/etiologia , Dor Ocular , Hiperemia/complicações , Hiperemia , Tomografia Computadorizada de Emissão/métodos , Tomografia Computadorizada de Emissão , Imageamento por Ressonância Magnética/métodosRESUMO
CASO CLÍNICO: Hombre de 17 años, con un tumor intraocular izquierdo de 2 años de evolución y pérdida visual progresiva. Presentó rotura escleral durante la enucleación. Microscópicamente, las tinciones de H-E, PAS e inmunohistoquímica (NSE, GAFP, SYN, y CD99) demostraron un tumor maligno de células pequeñas, redondas y azules, con necrosis, apoptosis e invasión al nervio óptico, cuerpo ciliar, coroides, cámara anterior y esclerótica. La SYN resultó positiva y el CD99 negativo en células neoplásicas, confirmándose un retinoblastoma pobremente diferenciado. Discusión: El retinoblastoma es el tumor intraocular maligno primario más frecuente en niños, aunque ocasionalmente afecta a otros grupos de edad. La inmunohistoquímica es obligada en los retinoblastomas pobremente diferenciados
CASE REPORT: A 17-year- old male with 2 years history of an intraocular mass and progressive visual loss of the left eye. Spontaneous sclera rupture occurred during enucleation. Microscopic evaluation with H-E, PAS and immunohistochemistry (NSE, GAFP, SYN, CD99) revealed a small blue round cell malignant neoplasm with extensive necrosis and apoptosis. The optic nerve, ciliary body, choroid, anterior chamber, and sclera were infiltrated. SYN was positive and CD99 was negative in neoplastic cells, consistent with a poorly differentiated retinoblastoma. DISCUSSION: Retinoblastoma is the most frequent primary intraocular malignant tumour in childhood, but occasionally older patients can be affected. Immunohistochemistry is mandatory in poorly differentiated retinoblastomas
Assuntos
Adolescente , Humanos , Retinoblastoma/complicações , Retinoblastoma/fisiopatologia , Retinoblastoma , Exoftalmia/complicações , Exoftalmia , Enucleação Ocular/métodos , Enucleação Ocular , Neoplasias Oculares/complicações , Neoplasias Oculares , Retinoblastoma/microbiologia , Diagnóstico Diferencial , Transtornos da Visão/complicações , Transtornos da Visão , Fotomicrografia/instrumentação , Fotomicrografia/métodosRESUMO
No disponible
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Fístula Carótido-Cavernosa/cirurgia , Fístula Carótido-Cavernosa , Artéria Carótida Interna/cirurgia , Artéria Carótida Interna , Exoftalmia/complicações , Exoftalmia/cirurgia , Exoftalmia , Imageamento por Ressonância Magnética/instrumentação , Angiografia Cerebral/métodos , Exoftalmia/fisiopatologia , Seio Carotídeo/patologia , Imageamento por Ressonância Magnética/métodos , Angiografia Cerebral/instrumentação , Seio Carotídeo/cirurgia , Seio CarotídeoRESUMO
CASO CLÍNICO: Paciente mujer de 78 años remitida para estudio de pseudotumor orbitario izquierdo de 17 días de evolución. Observamos proptosis, dolor sin limitación de los movimientos oculares, edema periorbitario y ptosis palpebral, clínica compatible con pseudotumor orbitario. La resonancia magnética orbitaria evidencia una masa adyacente al globo ocular ocupando la vertiente superoexterna y aumento de partes blandas a nivel de la glándula lagrimal. El estudio anatomopatológico de una lesión palpebral demuestra la presencia de granulomas no caseificantes. DISCUSIÓN: Se realiza el diagnóstico de sarcoidosis orbitaria definitiva. El tratamiento oral con corticoides y metotrexato logra el control de la enfermedad
CASE REPORT: 78 year-old female patient being investigated for a left orbital pseudotumour of 17 days onset. She had proptosis, pain, with no limitation of eye movements, periorbital oedema, and upper eyelid ptosis, compatible with an orbital pseudotumour. The MRI showed an upper outer left orbital mass adjacent to the side of eyeball and an increase in soft tissue at the level of lacrimal gland. The histopathology study of an eyelid lesion demonstrated the presence of non-caseating granulomas. DISCUSSION: A definitive diagnosis of orbital sarcoidosis was made. Oral treatment with corticosteroids and methotrexate achieved gradual control of the disease
Assuntos
Idoso , Feminino , Humanos , Pseudotumor Orbitário/complicações , Pseudotumor Orbitário , Sarcoidose , Sarcoidose/tratamento farmacológico , Corticosteroides/uso terapêutico , Metotrexato/uso terapêutico , Imageamento por Ressonância Magnética/métodos , Angiografia por Ressonância Magnética , Sarcoidose/complicações , Sarcoidose/etiologia , Exoftalmia/complicações , Exoftalmia/tratamento farmacológico , Prednisona/uso terapêuticoRESUMO
CASO CLÍNICO: Describimos el caso de un paciente varón de 51 años de edad, con antecedente de trauma contuso en la región frontal y desde hace 1 año con disminución de la agudeza visual, proptosis y paresia del músculo recto lateral del ojo derecho. Se le diagnosticó de un mucocele etmoidal derecho y esfenoidal bilateral, drenándose por abordaje externo y endoscópico, con mejoría de los síntomas y sin recurrencias a los 10 meses. DISCUSIÓN: Los mucoceles esfenoidales representan el 1% de todos los mucoceles y pueden presentarse con diferentes manifestaciones clínicas. La escisión quirúrgica es el tratamiento de elección y una intervención temprana está indicada para prevenir complicaciones
CASE REPORT: The case is presented of a 51-year old male patient with a history of blunt trauma in the frontal region and a one-year history of vision loss, proptosis and paresis of the lateral rectus muscle of the right eye. A right ethmoid and bilateral sphenoid mucocele was diagnosed. Drainage was performed using an external and endoscopic approach with improvement in symptoms without recurrence at 10 months. DISCUSSION: Sphenoid mucoceles represent 1% of all mucoceles and may present with different clinical manifestation. Surgical excision is the treatment of choice, and early intervention is indicated to prevent complications
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Doenças do Nervo Óptico/complicações , Doenças do Nervo Óptico/cirurgia , Doenças do Nervo Óptico , Endoscopia/métodos , Exoftalmia/complicações , Exoftalmia , Seio Etmoidal/patologia , Seio Etmoidal , Acuidade Visual , Tomografia Computadorizada de Emissão , Mucocele/patologia , MucoceleRESUMO
OBJETIVOS: Reportar los resultados del manejo multidisciplinario de pacientes con retinoblastoma, incluyendo supervivencia global, tasa de enucleación y éxito de la quimiorreducción sistémica. MÉTODOS: Estudio retrospectivo 27 pacientes (37 ojos) con diagnóstico de retinoblastoma, tratados por un equipo multidisciplinario en el Hospital San Juan de Dios. Se incluyeron información demográfica, características clínicas, supervivencia, tratamiento local y sistémico. Se realizó además un subanálisis de tratamiento con quimioterapia intraarterial (QIA). RESULTADOS: Catorce pacientes (52%) fueron de sexo masculino. La mediana de edad al ingreso fue de 8 meses (0,16-90). La mediana ± desviación estándar de seguimiento fue de 33 ± 21 meses. Diez casos (37%) se diagnosticaron después de los 15 meses de edad, con una mediana de 35 meses (24-90). En 17 (63%) pacientes fueron retinoblastomas unilaterales, mientras 10 (37%) tuvieron retinoblastoma bilateral. La leucocoria, aislada o asociada a otros signos, fue el motivo de consulta más frecuente (63%). La tasa global de enucleación fue del 57% (n = 21), siendo el tratamiento primario en 15 (55%) pacientes. La tasa de enucleación en retinoblastoma unilateral fue del 76,5% y en retinoblastoma bilateral del 60% de un ojo y el 10% de ambos. Diecisiete (63%) pacientes recibieron quimioterapia sistémica (media de ciclos: 5,3 ± 2,1). El éxito global de la quimiorreducción sistémica y la terapia focal fue del 68%. Tres pacientes fueron tratados con QIA como terapia de rescate, logrando controlar el tumor en 2 pacientes a 6 meses de seguimiento, siendo los primeros casos en Chile. La supervivencia fue del 100%. CONCLUSIÓN: El manejo multidisciplinario del retinoblastoma permite una supervivencia y una morbilidad comparable con la literatura internacional
OBJECTIVE: To report the results of the multidisciplinary management of patients with retinoblastoma, including survival, enucleation rate, and systemic chemoreduction success. METHODS: A retrospective study was conducted on 27 patients (37 eyes) diagnosed with retinoblastoma, and treated by a multidisciplinary team in San Juan de Dios Hospital. Demographic information, clinical characterization, survival, local and systemic treatments were included in the analysis. Patients treated with intra-arterial chemotherapy (IAC) were also reviewed. RESULTS: The study included14 male patients (52%). The median of age at presentation was 8 months (0.16-90). The median follow-up time was 33 ± 21 months. The diagnosis was made in 10 (37%) cases after 15 months old, with a median of 35 months (24-90). 17 (63%) patients had unilateral retinoblastoma, and 10 (37%) bilateral retinoblastoma. Leukocoria, isolated or associated with other signs, was the most frequent reason for referral (63%). Global enucleation rate was 57% (n = 21), being the primary treatment in 15 (55%) patients. Enucleation rate in unilateral retinoblastoma was 76.5%, and for bilateral retinoblastoma, it was 60% for one eye and 10% for both. Systemic chemotherapy was prescribed in 17 (63%) patients, with a mean number of cycles of 5.3 ± 2.1. The overall success of chemoreduction and focal therapy in order to avoid external radiotherapy and/or enucleation was 68%. Three patients were treated with IAC as a salvage therapy, controlling the tumor in 2 patients at 6 months of follow-up. These are the first cases reported in Chile. Survival rate was 100%. CONCLUSION: Multidisciplinary management of retinoblastoma led to a survival rate and morbidity comparable with international reports
Assuntos
Humanos , Masculino , Feminino , Retinoblastoma/induzido quimicamente , Retinoblastoma/metabolismo , Enucleação Ocular/instrumentação , Enucleação Ocular/métodos , Estrabismo/congênito , Estrabismo/genética , Exoftalmia/diagnóstico , Retinoblastoma/irrigação sanguínea , Retinoblastoma/cirurgia , Enucleação Ocular/enfermagem , Enucleação Ocular/reabilitação , Estrabismo/diagnóstico , Estrabismo/metabolismo , Exoftalmia/complicações , Estudos RetrospectivosRESUMO
CASO CLÍNICO: Varón de 21 años con historia de exoftalmos izquierdo y diplopía de 2 semanas de evolución. La resonancia magnética mostró una lesión muy vascularizada etmoido-orbitaria con invasión de base del cráneo anterior y extensión orbitaria. La biopsia etmoidal confirmó un tejido fibrovascular compatible con angiofibroma. DISCUSIÓN: El angiofibroma nasofaríngeo juvenil (ANJ) es un tumor benigno con características locales de malignidad debido a su capacidad de invadir áreas adyacentes. En nuestro caso, el comienzo se presenta con manifestaciones de extensión orbitaria. Consideramos necesario un conocimiento amplio y un abordaje multidisciplinario con el fin de mejorar el pronóstico
CLINICAL CASE: The case is presented of a 21 year-old male with a history of left proptosis and diplopia of two weeks of onset. The MRI showed an ethmoid-orbital vascular lesion with anterior skull base invasion and orbital extension. Biopsy of the ethmoid confirmed fibrovascular tissue, which supported the diagnosis of angiofibroma. DISCUSSION: It is a benign neoplasm with local characteristics of malignancy due to its ability to invade adjacent areas. In this case, the debut presented with manifestations of orbital extension. A broad and multidisciplinary approach is needed in order to improve prognosis
Assuntos
Humanos , Masculino , Adulto Jovem , Angiofibroma/induzido quimicamente , Angiofibroma/patologia , Neoplasias Nasofaríngeas/induzido quimicamente , Neoplasias Nasofaríngeas/patologia , Neoplasias Oculares/tratamento farmacológico , Neoplasias Oculares/radioterapia , Exoftalmia/congênito , Exoftalmia/metabolismo , Neoplasias de Cabeça e Pescoço/diagnóstico , Angiofibroma/diagnóstico , Angiofibroma/prevenção & controle , Neoplasias Nasofaríngeas/diagnóstico , Neoplasias Nasofaríngeas/metabolismo , Neoplasias Oculares/complicações , Neoplasias Oculares/cirurgia , Exoftalmia/complicações , Exoftalmia/cirurgia , Neoplasias de Cabeça e Pescoço/tratamento farmacológicoAssuntos
Humanos , Feminino , Idoso de 80 Anos ou mais , Conjuntivite/complicações , Conjuntivite/etiologia , Conjuntivite , Prurido/complicações , Exoftalmia/complicações , Exoftalmia/terapia , Exoftalmia , Conjuntivite/fisiopatologia , Atenção Primária à Saúde/métodos , Atenção Primária à Saúde/tendências , Zigoma/patologia , Zigoma , Exoftalmia/etiologia , Imageamento por Ressonância Magnética , Imageamento por Ressonância Magnética/métodos , Tomografia , Tomografia Computadorizada de EmissãoRESUMO
CASO CLÍNICO: Mujer de 27 años con proptosis del ojo derecho. Las pruebas de imagen mostraron una lesión extracónica en el tercio anterior medial de la órbita que afectaba al músculo recto interno. Se realizó exéresis del tumor con radioterapia adyuvante. El diagnóstico fue de sarcoma pleomorfo indiferenciado de alto grado. Tras 6 años de seguimiento no se ha observado recidiva. DISCUSIÓN: El sarcoma pleomorfo indiferenciado es una neoplasia maligna muy infrecuente en la región orbitaria. Tiene un rápido crecimiento local y es potencialmente metastatizante, por lo que debe hacerse estudio de extensión. El tratamiento de elección es la cirugía (AU)
CASE REPORT: A 27-year-old female presented to us with a complaint of proptosis of her right eye. Imaging tests showed an extraconal mass in the medial orbital wall, which affected the medial rectus. The tumor was removed and adjuvant radiotherapy was required. The histological examination showed an undifferentiated high-grade pleomorphic sarcoma of the orbit. There has not been recurrence after 6 years of follow-up. DISCUSSIÓN: Undifferentiated high-grade pleomorphic sarcoma is an uncommon cause of malignant orbital mass. It grows fast and can metastasize, so extension studies are required. Surgical excision is the treatment of choice (AU)