Pseudotumor inflamatorioocular asociado a IgG4. Reporte de un caso / IgG4-Associated Ocular Inflammatory Pseudotumor. A Case Report

Resumen Introducción: El pseudotumor inflamatorio ocular asociado a IgG4 es un proceso inflamatorio no neoplásico y poco frecuente con una incidencia de 0.28-1.08 por cada 100,000 personas. El diagnóstico de esta patología es de exclusión debiendo cumplir criterios histopatológicos, agrandamiento de un órgano en específico y concentraciones serias de IgG4. El tratamiento tiene como objetivo evitar las complicaciones secundarias. Caso clínico: Mujer de 54 años de edad sin patologías previas referidas, acude refiriendo que 6 meses previos presenta de forma súbita y sin causa aparente prurito a nivel de globo ocular y párpado izquierdo, además de disminución de la agudeza visual y dolor retroocular, motivo por el que acude a valoración. Al examen físico presentó movimientos oculares normales, proptosis izquierda, dolor a la palpación, hiperemia subconjuntival, opacidad corneal, ausencia de reflejo rojo y más renitente a la presión el ojo izquierdo en relación con el contralateral. Tomografía cráneo-toraco-abdominal reporta a nivel ocular izquierdo imágenes sugestivas de melanoma coroideo sin hallazgos de actividad metastásica. Se realiza enucleación de ojo izquierdo. Se envía pieza a patología la cual reporta infiltrado difuso linfoplasmocítico positivas para IgG4. Conclusión: La enfermedad por IgG4 ocular es una patología muy infrecuente de clínica inespecífica y diagnóstico complejo. Sin embargo, un abordaje rápido y correcto es fundamental para evitar complicaciones.

Abstract Introduction: IgG4-associated ocular inflammatory pseudotumor is a rare, non-neoplastic inflammatory process with an incidence of 0.28-1.08 per 100,000 people. The diagnosis of this pathology is one of exclusion, having to meet histopathological criteria, enlargement of a specific organ, and serious concentrations of IgG4. Treatment aims to avoid secondary complications. Clinical case: A 54-year-old woman with no previous reported pathologies, reports that for the past 6 months she has itching at the level of the eyeball and the left eyelid, suddenly and without apparent cause, in addition to decreased visual acuity and retro-ocular pain; this is why she asked for the evaluation. The physical examination revealed normal eye movements, left proptosis, pain on palpation, subconjunctival hyperemia, corneal opacity, absence of red reflex, and the left eye being more resistant to pressure in relation to the contralateral eye. The cranio-thoraco-abdominal tomography reported images at the left ocular level that were suggestive of choroidal melanoma without findings of metastatic activity. Enucleation of the left eye was performed. The specimen was sent to pathology which reported diffuse lymphoplasmacytic infiltrate positive for IgG4. Conclusion: Ocular IgG4 disease is a very rare pathology with non-specific symptoms and complex diagnosis. However, a quick and correct approach is essential to avoid complications.

Proptosis como presentación inicial de un fibroma osificante etmoidal en edad pediátrica

El fibroma osificante es un tumor óseo benigno poco común, cuya localización más frecuente son los huesos maxilofaciales, pero su origen en el seno etmoidal es raro. La mayor incidencia se describe entre la segunda y la cuarta décadas de la vida. Se efectuó una revisión en la literatura nacional sobre fibromas osificantes etmoidales con extensión orbitaria, y no se encontraron reportes publicados. Se presenta el caso de una paciente de 10 años de edad que fue remitida a consulta de Oculoplastia por notar protrusión ocular izquierda desde hacía tres meses. Se le realizó ultrasonido, tomografía computarizada y resonancia magnética en los que se evidenció una lesión tumoral de seno etmoidal con compromiso de órbita izquierda que ocasionaba proptosis, con características imagenológicas sugestivas de un probable fibroma osificante. La biopsia endoscópica nasal confirmó el diagnóstico.

Ossifying fibroma is an uncommon benign bone tumor, whose most frequent location is the maxillofacial bones, but its origin in the ethmoid sinus is rare. The highest incidence is described between the second and fourth decades of life. A review of the national literature on ethmoid ossifying fibromas with orbital extension was carried out, and no published reports were found. We present the case of a 10-year old female patient who was referred to Oculoplasty consultation for noticing left ocular protrusion for three months. Ultrasound, computed tomography and magnetic resonance imaging showed a tumor lesion of the ethmoidal sinus with involvement of the left orbit causing proptosis, with imaging characteristics suggestive of a probable ossifying fibroma. Nasal endoscopic biopsy confirmed the diagnosis.

Small cell neuroendocrine carcinoma with primary orbital involvement – The unseen, and a review of literature

Neuroendocrine neoplasms are derived from the epithelial lineages mainly of respiratory tract, with predominant neuroendocrine differentiation. There are only a handful of documented cases of paranasal small cell neuroendocrine carcinomas (SNEC) with primary orbital involvement. Here, the authors describe a 33-year-old male patient with rapidly progressive swelling of the right lower lid with proptosis since 4 weeks. On contrast-MRI orbit, an ill-defined multilobulated mass measuring 3.6 × 3.1 cm with intense homogenous enhancement was seen in the right retrobulbar space involving the right ethmoid sinus. On incisional biopsy, a poorly differentiated mass containing numerous small round blue cells and scanty intervening stroma with prominent necrosis and apoptosis was seen. Immunohistochemistry was strongly positive for synaptophysin. He was diagnosed as a case of SNEC and received chemotherapy, with good response till date of 9 months of follow up. The authors present a literature review and describe challenges in management of a primary orbital SNEC.

‘A down and out eye’ - A case report of unilateral eccentric proptosis secondary to frontoethmoidal mucocele

Proptosis, the forward protrusion of the eyeball, is a common manifestation of a wide variety of diseases inside the orbit and its spaces. The causes of unilateral proptosis are innumerable and its evaluation requires a multidisciplinary approach. Paranasal sinus mucoceles are epithelium-lined cystic masses usually resulting from obstruction of sinus ostia. The close proximity of paranasal sinus mucoceles to the orbit and skull base predispose patients to significant morbidity. Primary ethmoid mucocele is an uncommon entity, especially in the absence of prior ear, nose and throat complaints, and therefore should remain an important differential when a patient presents with a unilateral swelling causing proptosis. Herein, we report an unusual case of a primary frontoethmoid mucocele in a 35 years old woman who presented with a painless swelling with an obvious displacement of the left eye since 1 year, associated with restriction of ocular movements, thus prompting radio imaging of orbits. The results showed a large well-defined expansile lesion suggestive of Left Eye frontoethmoidal mucocele, prompting an immediate surgical referral.

Determination of exophthalmometry values in the North Indian population

Purpose: To determine exophthalmometry values (EV) in the north Indian population and to find its correlation with the age, gender, height, weight, body mass index (BMI), locality, and base value of the population. Methods: A total of 1000 eligible participants with 504 females and 496 males with age >5 years were included in this observational cross-sectional study. Age, gender, and locality of the subjects were noted. Hertel’s exophthalmometry was performed on all subjects. The exophthalmometry values and base values were recorded. Height and weight were measured for all participants. BMI was calculated using the parameters. Results: The overall mean exophthalmometry value (mm) was 14.94 ± 2.43 mm with a range between 8 and 22 mm. There was no significant difference in EV between the two eyes. Males recorded a significantly higher mean EV of 15.4 ± 2.51 mm as compared to females with a value of 14.49 ± 2.27 mm. Base value of Hertel’s exophthalmometer had a mean value 100.78 ± 5.63 mm and a range of 84–120 mm. Age, height, weight, BMI, and locality of the subjects were found to have a significant effect on the exophthalmometry as well as the base value of the population. A significant correlation was also seen between exophthalmometry values and base values of the population. Conclusion: Our study provided the normal exophthalmometric range for the north Indian population and also demonstrated the relationship of age, gender, height, weight, BMI, locality, and base value with the exophthalmometric values

Linfoma ocular primario vs celulitis orbitaria en tiempos de COVID-19 / Primary ocular lymphoma vs orbital cellulitis in times of COVID-19

Los tumores sólidos neoplásicos de tejido linfoide se caracterizan por proliferación acelerada de la porción linforreticular del sistema retículo endotelial, su incidencia mundial es de 4/100,000 prevalente en féminas entre 50-70 años, de etiología desconocida, asociado a helicobacter pylori, epstein barr y VIH. Clínicamente están presentes los síntomas B: fiebre, sudoraciones nocturnas, pérdida de peso, prurito y astenia. ausentes en el caso que nos ocupa de una paciente femenina de 46 años de edad que acudió a consulta de oftalmología por aumento de volumen a nivel de anejos de ojo izquierdo, proptosis indolora y disminución de la visión, con diagnóstico inicial de celulitis orbitaria tratada ambulatoriamente sin resolución. Se administró antibioticoterapia intravenosa sin mejoría clínica, se realizaron estudios complementarios de imagen radiológica, ultrasonido, resonancia magnética con evidencia de imagen tumoral de crecimiento antero lateral, desplazamiento de globo ocular, diámetro mayor de 57,3 mm, de características isointensas heterogéneas, secuencia T1 y flair, erosión de pared interna de orbita y la biopsia excisional informó linfoma primario de anexo ocular orbitario tipo no-Hodgkin. El abordaje acucioso con estudios complementarios para descartar neoplasias orbitarias en pacientes con celulitis orbitaria o dacriocistitis de evolución tórpida es necesario en todo momento, independiente de las condiciones atípicas de pandemia por COVID-19 que dificultan su manejo(AU)

Solid neoplastic tumors of lymphoid tissue are characterized by accelerated proliferation of the lymphoreticular portion of the reticulum endothelial system, their worldwide incidence is 4/100,000 prevalent in females between 50-70 years of age, of unknown etiology, associated with helicobacter pylori, epstein barr and HIV. Clinically, symptoms B are present: fever, night sweats, weight loss, itching and asthenia, absent in the present case of a 46-year-old female patient who came to the ophthalmology office due to increased volume at the level of the appendages. of the left eye, painless proptosis and decreased vision, with an initial diagnosis of orbital cellulitis treated outpatiently without resolution. Intravenous antibiotic therapy was administered without clinical improvement, complementary radiological imaging studies, ultrasound, magnetic resonance imaging were performed with evidence of an anterolateral growth tumor image, ocular globe displacement, diameter greater than 57.3 mm, heterogeneous isointense characteristics, T1 sequence and flair, erosion of the internal wall of the orbit and the excisional biopsy reported primary non-Hodgkin type orbital annex lymphoma. A careful approach with complementary studies to rule out orbital neoplasms in patients with orbital cellulitis or dacryocystitis of torpid evolution is necessary at all times, regardless of the atypical conditions of a COVID-19 pandemic that make its management difficult(AU)

Proptosis is associated with thiol-disulfide in patients with Graves' ophthalmopathy

ABSTRACT Objective: Graves' ophthalmopathy (GO) is a vision-threatening finding observed in approximately half of Graves' disease patients. The pathophysiology of GO is unclear, and one of the suspected factors is oxidative stress. In our study, we compared the relationship between proptosis and SH-SS in patients diagnosed with GO. Materials and methods: In this prospective study, 40 recently diagnosed Graves' disease patients with proptosis, 40 recently diagnosed Graves' disease patients without GO and 30 healthy individuals with similar demographic characteristics were included. Serum thiol-disulfide (SH-SS) measurements were performed. Eye examinations were performed by a single ophthalmologist to check for the presence of GO, and proptosis values were recorded with a Hertel exophthalmometer. Results: Total SH values were lower in the group with proptosis than in the other groups (p < 0.05). Total and native SH values were lower in patients without proptosis than in the control group (p < 0.05). Total SH, native SH and SS levels were independently associated with proptosis (p < 0.05). According to this analysis, it was found that increasing SS and decreasing total and native SH levels increased the probability of proptosis by 24.4%, 32.7% and 32.4%, respectively. Conclusion: A decrease in SH, which is a natural antioxidant that protects the body against oxidative stress, and an increase in SS are important signs of oxidative damage. Proptosis and SH-SS are closely related in GO. This may help us detect GO and proptosis in Graves' patients. It can also assist in developing new options for preventing and treating GO.

Orbital varix presenting with enophthalmos and intermittent proptosis: a case report

We report the case of a 22-year-old male, presenting with right enophthalmos and intermittent exophthalmos observed after valsalva maneuver. Orbital imaging revealed a retrobulbar vascular lesion with multiple phleboliths suggestive of orbital varices. On magnetic resonance angiography, no obvious feeder vessel could be delineated. We decided to manage the patient conservatively, in the absence of complications such as proptosis with corneal exposure, optic nerve compression, orbital hemorrhage. Orbital varix is a low flow vascular anomaly which generally presents with an intermittent exophthalmos. Development of enophthalmos associated with orbital varices as in our case, is very rare.

Proptosis in Otorhinolaryngology: An Overview

Abstract Introduction Ear, nose and throat (ENT)pathologies can present with proptosis as the early manifestation. The majority of ENT pathologies present with ocular manifestations, due to close anatomical relationship. Objective To study proptosis in ENT disorders in terms of etiology, pathogenesis, and management. Methods The present article is a prospective study conducted at a tertiary care center, in Telangana, India. Patients presenting with proptosis secondary to ENT disorders were thoroughly evaluated and subjected to surgical exploration after providing a signed informed consent. Results Sixty cases of proptosis secondary to ENT disorders have been reported. The most common cause was sinonasal squamous cell carcinoma followed by juvenile nasopharyngeal angiofibroma and allergic fungal rhinosinusitis. Conclusion Many ENT disorders can present with proptosis. Proptosis needs to be evaluated completely for proper management and to limit the morbidity associated with it.

Kimura Disease as a Rare Cause of Proptosis: A Case Report

ABSTRACT@#Kimura disease (KD) is a rare chronic inflammatory disorder of unknown aetiology that primarily affects the head and neck region with lymph node involvement. Young to middle-aged adult Asian males are predominantly affected. The most common presentation is painless subcutaneous swelling in the head and neck region, while proptosis or orbital involvement is very rarely reported. KD shares some features with other inflammatory and neoplastic disorders, including lymphoma; thus, investigations to confirm the diagnosis should not be delayed. Systemic corticosteroids are commonly used to treat KD and show an excellent response; however, the optimal treatment is still uncertain, and KD has a high recurrence rate. We describe the case of a patient with KD who presented with proptosis and post-auricular swelling, which responded well to oral prednisolone treatment.

Spontaneous Subperiosteal Orbital Haemorrhage (SSOH): An Unusual Complication of Acute Coronary Syndrome Treatment

@#Acute coronary syndrome is a medical emergency with a high mortality and morbidity. Reperfusion therapy is widely used in its management. Ocular complications following reperfusion therapy is rare. A 51-year-old man treated with streptokinase, antithrombotic and dual antiplatelet therapy, developed mild proptosis following treatment. After four days, there was sudden worsening of the proptosis associated with orbital compartmental syndrome (OCS). The CT scan of the orbit showed a large spontaneous subperiosteal orbital hemorrhage (SSOH) occupying half of the left orbit. Despite performing an urgent lateral canthotomy and inferior cantholysis, the patient developed irreversible vision loss due to compressive optic neuropathy. While a few cases of mild SSOH have been reported in the medical literature, this is the first documented case of secondary bleed in SSOH. Early recognition and intervention by the treating physician, is crucial in preventing blindness.

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.

Orbital cellulitis: early intervention saves vision

Orbital cellulitis describes an infection involving the soft tissues posterior to the orbital septum including the fat and muscle within the bony orbit. This condition is associated with severe sight and life-threatening complications. Distinguishing it from preseptal cellulitis is difficult, but important. Acute sinusitis is the commonest predisposing factor. Clinical findings alone are not specific enough to distinguish between preseptal and post septal orbital cellulitis. Early diagnosis using CT orbit is important to rule out complications such as orbital cellulitis, subperiosteal abscess. The most common location of subperiosteal abscess is the medial wall of the orbit. Transnasal endoscopic drainage of the abscess is a functional and minimally invasive technique and is the treatment of choice at present. Early diagnosis and intervention are mandatory to prevent the visual loss and life-threatening complication.Here, the authors describe a 2 months old infant with orbital cellulitis and medial subperiosteal abscess and treated with transnasal endoscopic drainage of the subperiosteal abscess.

Proptosis por Celulitis Orbitaria en paciente Pediátrico: reporte de un caso / Proptosis for Orbital Cellulitis in a Pediatric Patient: case report

Orbital cellulitis is an infectious disease that occurs most frequently in the pediatric age. The most common underlying factor for its development is ethmoidal sinusitis. The microorganisms associated with orbital infection are S. pneumoniae, S. aureus, H. influenzae and M. catarrhalis, whose therapeutic failure brings serious complications that include loss of vision, meningitis and intracranial infection. In the following case we want to capture the doctor's actions in this infectious pathology, the diagnoses we should rule out and antibiotic treatment. We report the case of a previously healthy 2-year-old male patient who consulted for an increase left bipalpebral volume, associated to impossibility of spontaneous opening of left eye and febrile peak of 39.4 ° C, with TAC of paranasal sinuses and orbit, where shows left medial intraorbital abscess, with preseptal compromise that determines left proptosis, deciding to start associated antibiotic therapy for orbital cellulitis with compromise preseptal. In view of the slow evolution, a study was started to rule out the associated tumor process, which is discarded, maintaining an antibiotic for 21 days with clinical improvement after these. Orbital cellulitis in the pediatric age should be diagnosed quickly and in a timely manner, since it is a medical emergency, it is a condition that requires hospitalization and management with systemic antibiotics.

A report of orbital metastasis from a urinary bladder adenocarcinoma

A 60-year-old man presented with sudden-onset proptosis of the left eye and intermittent diplopia of 2 months duration. Ophthalmic examination revealed bilateral eyelid retraction, left eye proptosis and a firm, non-tender mass (2 cm × 1.5 cm) in left supero-medial orbit with restricted extraocular movements. Contrast-enhanced computed tomography showed a well-defined, enhancing antero-medial orbital mass which was removed via anterior orbitotomy approach. The histopathology/immunohistochemistry showed adenocarcinoma; metastasis of urothelial origin. The oncology consultation and metastatic workup revealed a urinary bladder carcinoma with distant metastasis. Our patient expired within 6 months of diagnosis. The ophthalmic symptoms due to orbital metastasis may be the first presentation of some cancer patients.

Histiocitosis de células de Langerhans en su presentación focalizada en órbita. Revisión del tema a propósito de un caso / Langerhans cell histiocytosis in their orbitfocused presentation. Review of the topic regarding a case

Resumen Objetivo: Describir un caso de histocitosis de células de Langerhans en un paciente pediátrico. Método: Paciente de 4 años con proptosis, diplopía, fiebre y pérdida de agudeza visual, con imagen en tomografía computarizada evidente de masa orbitaria con erosión ósea. Se realizó biopsia excisional por craneotomía coronal, el resultado histopatológico fue histiocitosis de células de Langerhans. Resultados: El estudio anatomopatológico transoperatorio mostró una neoplasia de células pequeñas redondas y azules, compatible con rabdomiosarcoma. Las pruebas de inmunohistoquímica concluyeron el diagnóstico de histiocitosis de células de Langerhans. Conclusión: La histiocitosis de células de Langerhans es una enfermedad poco frecuente que requiere de una sospecha clínica y diagnóstico oportuno, adecuar el tratamiento y mejorar la sobrevida de los pacientes.

Abstract Objective: To describe a case of Langerhans cell histiocytosis in a pediatric patient. Method: A 4-year-old patient with proptosis, diplopia, fever and loss of visual acuity, with evident image in a computed tomography of orbital mass with bone erosion. An excisional biopsy was performed by coronal craniotomy. The histopathological result was Langerhans cell histiocytosis. Results: The anatomopathological study showed a neoplasm of small round and blue cells, compatible with Rhabdomyosarcoma. Immunohistochemical tests concluded the diagnosis of Langerhans cell histiocytosis. Conclusion: Langerhans cell histiocytosis is a rare disease that requires clinical suspicion and a timely diagnosis to adjust the treatment and improve the survival of patients.

Disseminated cysticercosis presenting with bilateral proptosis: A case report

Rationale: Disseminated cysticercosis is characterized by presence of cysts in multiple body organs, like brain, skin, eyes, muscles and rarely heart and lungs. Patient concerns: A 22-year-old man presented with bilateral proptosis of 1-year duration. He also had two episodes of cysticercosis encephalitis. In the second episode of encephalopathy, the patient died. Diagnosis: Disseminated cysticercosis. Interventions: Corticosteroids (Initially intravenous dexamethasone 0.4 mg/kg/day for 2 weeks, followed by oral prednisolone 1.0 mg/kg/day). Outcomes: The patient died of cysticercosis encephalitis approximately 2 months later. Lessons: Disseminated cysticercosis in our case presented only with proptosis as he had very heavy infestation of the brain and eyes. Heavy larval infestation in a patient with disseminated cysticercosis can be life-threatening.

Disseminated cysticercosis presenting with bilateral proptosis: A case report

Rationale: Disseminated cysticercosis is characterized by presence of cysts in multiple body organs, like brain, skin, eyes, muscles and rarely heart and lungs. Patient concerns: A 22-year-old man presented with bilateral proptosis of 1-year duration. He also had two episodes of cysticercosis encephalitis. In the second episode of encephalopathy, the patient died. Diagnosis: Disseminated cysticercosis. Interventions: Corticosteroids (Initially intravenous dexamethasone 0.4 mg/kg/day for 2 weeks, followed by oral prednisolone 1.0 mg/kg/day). Outcomes: The patient died of cysticercosis encephalitis approximately 2 months later. Lessons: Disseminated cysticercosis in our case presented only with proptosis as he had very heavy infestation of the brain and eyes. Heavy larval infestation in a patient with disseminated cysticercosis can be life-threatening.