Differential Diagnoses of Diseases Involving the Extrinsic Ocular Musculature ­ A Pictorial Essay

Introduction There are some inflammatory, infectious, and neoplastic diseases affecting the extrinsic orbital musculature (EOM) that present with pain, decreased visual acuity, and proptosis. Imaging is fundamental to the differential diagnoses of these diseases with similar clinical presentations. The present case series report has as main objective to illustrate and discuss the main pathologies that affect the orbit. Material and Methods The present series of cases discusses the main pathologies that can affect the extraocular musculature that can be characterized by computed tomography (CT) or magnetic resonance imaging (MRI) using cases from our institution. Results and Discussion The present study compiled several cases of ophthalmopathy from our institution to illustrate and address some of these pathologies, such as orbital lymphoma, Grave disease, metastases, periorbital cellulitis, and idiopathic orbital inflammatory syndrome. The diseases are discussed according to the presentation of clinical cases with emphasis on the main imaging findings of each pathology. Conclusion Computed tomography and MRI can help in the diagnosis and follow-up of the diseases that affect the EOM. We must be conversant with the main characteristics of the pathologies presented in the present case series report, since such findings together with clinical data can confirm the diagnosis of these diseases or at least help to narrow the differential diagnoses.

Enfermedad inflamatoria orbitaria idiopática. Presentación de un caso / Idiopathic orbital inflammatory disease. Case presentation

RESUMEN El pseudotumor orbitario es la tercera causa de oftalmoplejía dolorosa; constituye un desafío diagnóstico pues obliga a descartar enfermedades de etiología muy diversa que lo provocan. Se presenta el caso de una paciente femenina, de color de piel blanca, de procedencia urbana, ama de casa, fumadora, que no practica ejercicios ni lleva dieta, con antecedentes de hipertensión arterial y artritis reumatoidea, que acudió al Servicio de Oftalmología por presentar síntomas inflamatorios anexiales: edema palpebral, quemosis conjuntival, vasos conjuntivales dilatados, que se acompañaban de proptosis, oftalmoplejía dolorosa con diplopía y de una masa tumoral palpable a nivel de la porción supero-externa de la órbita. Se ingresó y se le realizaron estudios imagenológicos e histológicos, que solo aportaron signos inflamatorios y una pansinusitis. Se concluyó como un pseudotumor orbitario en su forma aguda de aparición, asociada a la artritis reumatoidea, cuyo diagnóstico se realizó por exclusión sobre la base de los resultados negativos de los estudios imagenológicos y de la biopsia. Llevó tratamiento con antibióticos parenterales y altas dosis de esteroides orales con regresión del cuadro y mejoría clínica. Se decidió la presentación del caso porque en la provincia no existen reportes sobre esta entidad, y por el énfasis en cómo llegar al diagnóstico y su correcto manejo, evitando con ello secuelas en el órgano visual.

ABSTRACT Orbital pseudotumor is the third cause of painful ophthalmoplegia; it constitutes a diagnostic challenge as it forces us to rule out diseases of very diverse etiology that cause it. A female patient, of white skin color, of urban origin, housewife, smoker, who does not practice exercises or is not on a diet, with a history of high blood pressure and rheumatoid arthritis, who attended the Ophthalmology service for presenting Adnexal inflammatory symptoms: eyelid edema, conjunctival chemosis, dilated conjunctival vessels, which were accompanied by proptosis, painful ophthalmoplegia with diplopia and a palpable tumor mass at the level of the superior-external portion of the orbit was presented. She was admitted and imaging and histological studies were performed, which only revealed inflammatory signs and pansinusitis. It was concluded as an orbital pseudotumor in its acute onset form, associated with rheumatoid arthritis, the diagnosis of which was made by exclusion on the basis of the negative results of imaging studies and biopsy. She was treated with parenteral antibiotics and high doses of oral steroids with regression of the symptoms and clinical improvement. The presentation of the case was decided because in the province there are no reports on this entity, and because of the emphasis on how to reach the diagnosis and its correct management, thereby avoiding effects in the visual organ.

Uso terapéutico de la hialuronidasa y la triamcinolona en el pseudotumor orbitario / Therapeutic use of hyaluronidase and triamcinolone in orbital pseudotumor

RESUMEN El origen del pseudotumor orbitario no es del todo conocido. Se admite su naturaleza inflamatoria granulomatosa e inespecífica en diferentes localizaciones. El pseudotumor orbitario se define como una respuesta inflamatoria celular pleomórfica, que está usualmente confinado a estructuras de la órbita y tiene una evolución limitada. En este trabajo se presenta una paciente femenina de 16 años, con diagnóstico de pseudotumor orbitario corroborado por biopsia y tomografía axial computarizada, refractaria al tratamiento con esteroides sistémicos, por lo que se decide iniciar con la aplicación de hialuronidasa y triamcinolona en el espacio peribulbar. Los casos agudos casi siempre responden rápidamente al tratamiento con cortocoesteroides, como prednisona, pero debemos tener en cuenta que existen pacientes que son refractarios al tratamiento, por lo que es necesario buscar procedimientos alternativos. Una opción es el uso de hialuronidasa para destruir las uniones extracelulares, y difundir un esteroide de manera local, como la triamcinolona, más efectiva dentro del tejido inflamatorio para provocar un efecto localizado de este. A los tres meses del tratamiento hubo una regresión total del cuadro en esta paciente(AU)

ABSTRACT The exact etiology of orbital pseudotumor is unknown, but its granulomatous unspecific inflammatory nature at various locations has been recognized. Orbital pseudotumor is defined as a cellular pleomorphic inflammatory response of limited evolution often confined to orbital structures. A case is presented of a female 16-year-old patient diagnosed with orbital pseudotumor confirmed by biopsy and computerized axial tomography, refractory to treatment with systemic steroids, due to which it is decided to start treatment with hyaluronidase and triamcinolone in the peribulbar space. Acute cases often respond fast to treatment with corticosteroids such as prednisone. It should be borne in mind that there are patients who are refractory to treatment for whom alternative treatments should be sought. An option is the use of hyaluronidase to destroy extracellular junctions and locally spread a steroid such as triamcinolone, most effectively within the inflammatory tissue to ensure its localized effect. Total regression of the patient's status was observed at three months of treatment(AU)

Neurofibromatose tipo 1 com acometimento do nervo infraorbital: relato de caso / Neurofibromatosis type 1 with infraorbital nerve involvement: a case report

A neurofibromatose tipo 1 é uma doença autossômica dominante rara, com manifestações clínicas diversas. Sua apresentação mais marcante é a presença de neurofibromas (tumores da bainha neural) cutâneos ou internos, que também podem ocorrer de forma esporádica, associados a outras manifestações sistêmicas, como manchas café com leite e lesões oculares. Por serem tumores da bainha de mielina, os neurofibromas podem acometer diversos nervos periféricos, incluindo nervos da face. Apresentamos o caso de um paciente de 1 ano, portador de neurofibromatose tipo 1, com neurofibroma em nervo infraorbital direito, com o acesso proposto para tratamento cirúrgico que fornecesse ampla visualização e acesso a lesão, sem comprometimento estético importante, permitindo preservação de partes moles e adequado crescimento facial.

Neurofibromatosis type 1 (NF1) is a rare autosomal dominant disease with multiple clinical manifestations. Its most significant presentation is cutaneous or subcutaneous neurofibromas (myelin sheath tumors), which may be associated with other systemic manifestations such as caféau- lait spots and eye involvement. Neurofibromas can affect several peripheral nerves, including the facial nerves. This report presents a case of a 1-year-old patient with NF1 with right infraorbital nerve neurofibroma in which the proposed access for surgical treatment allowed adequate visualization of the tumor with good aesthetic results, preservation of the soft tissues, and normal facial growth.

Miositis orbitaria en adolescente / Adolescents´ orbital myositis

Introducción: La miositis orbitaria aguda es una afección inflamatoria de la musculatura extrínseca ocular muchas veces de causa desconocida. Forma parte de un grupo de afecciones inflamatorias orbitarias inespecíficas englobadas bajo el término de pseudo tumor orbitario. Afecta principalmente a las mujeres jóvenes. Objetivo: Describir el caso de una adolescente con miositis orbitaria aguda, afección inflamatoria poco frecuente en la edad pediátrica. Presentación del caso: Se trata de una adolescente de 14 años, mestiza, femenina, quien sufrió dengue por tres ocasiones poco tiempo antes de presentarse con dolor ocular del ojo izquierdo, fiebre elevada intermitente, protrusión ocular intermitente, vómitos, cefalea, decaimiento, pérdida de peso y de apetito. El ojo izquierdo presentaba edema del parpado superior y limitación a la abducción, musculatura ocular extrínseca: diplopía horizontal a mirada derecha a izquierda. El ultrasonido ocular informa presencia de imagen quística que no impresiona ser de contenido hemático. El resultado de la tomografía axial computarizada de órbita y cráneo simple fue compatible con diagnóstico de miositis orbitaria. Se indicó tratamiento inmediato con esteroides orales y se remitió al Instituto de Oftalmología Ramon Pando Ferrer para seguimiento. Conclusiones: La miositis orbitaria exige un alto índice de sospecha para un diagnóstico y tratamiento correcto. El dengue pudiera estar implicado en la etiología de la enfermedad(AU)

Introduction: Acute orbitary myositis is an inflammatory condition of the extrinsic ocular musculature, often of unknown cause. It is part of a group of non-specific orbital inflammatory conditions encompassed by the term of orbital pseudotumors. It mainly affects young women. Objective: To describe the case of a teenager with acute orbital myositis which an inflammatory condition not frequent in the pediatric ages. Case presentation: This is a 14-year-old, mixed-race, female patient, who suffered dengue three times before presenting ocular pain in the left eye, intermittent high fever, intermittent ocular protrusion, vomiting, headache, weakness, loss of weight and appetite. The left eye presents edema of the upper eyelid and limitation in abduction. The extrinsic ocular musculature presented: horizontal diplopia in right to left gaze. The ocular ultrasound informs: cystic image that does not impress to be of hematic content. A computerized axial tomography of the orbit and simple skull was performed, and the result was compatible with the diagnosis of orbital myositis. Immediate treatment with oral steroids was indicated and the patient was sent to the Ramon Pando Ferrer Ophthalmology Institute for follow-up. Conclusion: Orbital myositis requires a high index of suspicion for a correct diagnosis and treatment. Dengue seems to be involved in the etiology of the disease(AU)

Clinical outcomes and pathological characteristics of immunoglobulin G4-related ophthalmic disease versus orbital inflammatory pseudotumor

BACKGROUND/AIMS: This study investigated the clinical and pathological features of immunoglobulin G4 (IgG4)-related ophthalmic disease. To clarify the features, we compared IgG4-related ophthalmic disease and orbital inflammatory pseudotumor. METHODS: We retrospectively reviewed the medical records of 103 patients who were initially diagnosed with orbital inflammatory pseudotumor, and identified 16 cases in which the diagnosis was based on surgical biopsy and for which data in medical records were sufficient for analysis. Immunohistochemical staining of pathological specimens for IgG and IgG4 was performed. Finally, six of IgG4-related ophthalmic disease patient and 10 of orbital inf lammatory pseudotumor patient were analyzed. RESULTS: The IgG4-related ophthalmic disease group had more IgG4-positive plasma cells and a higher IgG4/IgG plasma cell ratio than the orbital inflammatory pseudotumor group. Collagenous fibrosis and lacrimal gland involvement were significantly more frequent in the IgG4-related ophthalmic disease group. Dense lymphocyte infiltration, obliterative phlebitis, and bilateral lesions were more frequent in IgG4-related ophthalmic disease, but the differences were not significant. The recurrence-free period was shorter in the IgG4-related ophthalmic disease group (p = 0.035). CONCLUSIONS: The location of the lesion (lacrimal gland), count and ratio of IgG4-positive plasma cells, and collagenous fibrosis aid the diagnosis of IgG4-related ophthalmic disease in patients with idiopathic orbital mass-like lesions. In addition, maintenance therapy should be considered in patients with IgG4-related ophthalmic disease to prevent recurrence.

Pseudotumor orbital bilateral recorrente / Recorrent bilateral orbits pseudo-tumor

Resumo O pseudotumor orbitário é uma doença inflamatória idiopática benigna. Os autores apresentam um caso manifestado em adolescente de 12 anos, diagnosticado por meio do exame clínico, laboratorial e radiológico. Houve boa resposta ao tratamento proposto com corticosteroides. O relato é seguido de breve retomada literária acerca do tema.

Abstract The orbital pseudotumor is a benign idiopathic inflammatory disease. The authors present a case manifested in 12 years old boy, diagnosed by clinical, laboratory and radiological examination. There was a good response to treatment with corticosteroids proposed. The report is followed by brief literary resume on the subject.

Pseudotumor esclerosante de órbita / Sclerosing orbital pseudotumor

RESUMO O pseudotumor esclerosante de órbita é um subtipo raro de pseudotumor inflamatório idiopático de órbita. É mais comum em adultos e apresenta diagnóstico de exclusão. A primeira linha de tratamento são os esteroides. O subtipo esclerosante apresenta resposta moderada aos esteroides devido à predominância de fibrose e colágeno na histologia. Relatamos o caso de um paciente com diagnóstico histológico de pseudotumor esclerosante de órbita que teve boa resposta ao tratamento com corticoide associado à azatioprina.

ABSTRACT Sclerosing orbital pseudotumor is a rare subtype of idiopathic orbital inflammatory pseudotumor. It's more common in adults and presents diagnosis of exclusion. Steroids represent the first option of treatment. The sclerosing orbital pseudotumor subtype shows moderate response to steroids due to the predominance of fibrosis and collagen in its histology. We report on a case of a patient with histologic diagnosis of sclerosing orbital pseudotumor successfully treated with corticosteroid associated with azathioprine.

Orbital compartment syndrome in idiopathic orbital inflammatory disease: A case report

Orbital compartment syndrome (OCS) is a visual threatening ocular emergency. We report a 50-year-old male with acute presentation of OCS, a rare manifestation of idiopathic orbital inflammatory disease. At presentation, high intraocular pressure was reduced by prompt lateral canthotomy and cantholysis. The disease responded to systemic steroids and treatment resulted in good visual outcome. Detail evaluation and early detection and treatment are mandatory to prevent permanent vision loss.

Orbital pseudotumour as a presentation of paediatric ulcerative colitis

A 2-year-old girl presented with a one-day history of acute-onset bilateral painful, swollen eyes and a two‑month history of loose stools. Physical examination revealed a right eyelid swelling with proptosis. Magnetic resonance imaging revealed a right orbital pseudotumour. The patient responded well to treatment with intravenous antibiotics and nonsteroidal anti-inflammatory drugs. However, three weeks later, she was readmitted with a vasculitic lesion over her left upper chest, with mucous-bloody diarrhoea. Histopathology confirmed the diagnosis of ulcerative colitis. The patient was treated with intravenous pulse methylprednisolone and sulphasalazine. Two weeks after discharge, she was readmitted for cutaneous vasculitis and worsening diarrhoea. The patient's bowel and extraintestinal diseases resolved upon addition of infliximab to her treatment regimen. Her inflammatory markers also normalised. Azathioprine was subsequently added. Infliximab was discontinued after four doses and prednisolone was tapered off. The patient remained well without any flare-up after 24 months of follow-up.

A Case of MALT Lymphoma with Left Inferior Rectus Muscle Invasion

PURPOSE: To report an unusual case of mucosa-associated lymphoid tissue (MALT) lymphoma localized to the left inferior rectus muscle. CASE SUMMARY: A 52-year-old male presented with double vision 6 months in duration, which was exacerbated in the down-gaze. On initial examination, 6 and 30 prism diopters (PDs) of left hypertropia were observed in primary gaze and down gaze, respectively. Prominently limited infraduction was also observed in his left eye. Computed tomography (CT) revealed contrast enhancing mass on the left inferior rectus muscle. He was diagnosed with suspicious orbital pseudotumor and treated with systemic steroid for 2 months. Double vision and limited infraduction was improved and the mass size was decreased on CT. After 21 months, the patient revisited the clinic with the same symptoms. In the down-gaze, 35 PDs of hypertropia were observed in his left eye along with limited infraduction. CT revealed an enlarged mass and left superior rectus muscle recession along with an incisional biopsy of the left inferior rectus muscle were performed. Infiltration by small lymphoid cells was detected with hematoxylin-eosin and immunohistochemical stainings. Subsequently, the patient was diagnosed with MALT lymphoma localized to the left inferior rectus muscle. CONCLUSIONS: This is the first description of MALT lymphoma with inferior rectus muscle invasion in a Korean patient. In a patient with strabismus and limited duction, the possibility of extraocular muscle tumor including lymphoma should be considered. Homogenously contrast-enhanced mass on orbit CT can help in to make a diagnosing extraocular muscle lymphoma.

Quiste dermoide orbitario: reporte de un caso / Orbital dermoid cyst: case report

Los quistes dermoides son lesiones benignas que se producen a partir de tejido ectodérmico ectópico que queda atrapado en la fusión de los procesos embrionarios, alrededor de la quinta semana de vida intrauterina. Su conformación histológica es definida por una pared revestida de epitelio, con un número variable de anexos cutáneos y una cavidad central que contiene queratina descamada, grasa y pelos. Constituyen la tumoración orbitaria no neoplásica más frecuente en la infancia; su crecimiento es lento, y a nivel orbitario se asocian a las suturas frontocigomática y frontoetmoidal. Se presenta un caso clínico que demuestra la historia natural de esta condición a nivel de la órbita izquierda, junto con los detalles de su diagnóstico y tratamiento…

Dermoid cysts are benign lesions that develop from ectopic ectoderm tissue that stay capture in the embrionary process around the fifth intrauterine week of life. Its histology is compound by a wall of epithelium with a variety of cutaneous annexes and a great central cavity that contains desquamated keratin, fat and hair. Dermoid cysts are the more frequently orbitary tumors in childhood. It growth slowly and its orbital locations are in the zygomaticofrontal suture and the frontoethmoidal suture. We present the natural behavior of a left orbital dermoid cyst, diagnosed and surgical treatment…

Superior Rectus-Levator Palpebrae Complex Myositis Presenting as Isolated Painless Ptosis

No abstract available.

Clinical Features and Results of Steroid Therapy for Orbital Inflammatory Pseudotumor

PURPOSE: To evaluate the clinical features and treatment outcomes of steroid therapy for orbital inflammatory pseudotumor. METHODS: Sixty-four patients diagnosed with orbital inflammatory pseudotumor were reviewed retrospectively. Patients with a follow-up period of less than 6 months were excluded from the study. The pseudotumor was classified into myositic, lacrimal, anterior, diffuse, or apical type according to orbital computed tomography findings. All patients were initially treated with systemic corticosteroids and evaluated for response to the treatment. Treatment outcome was considered a "success" if the patient had complete relief of symptoms with no recurrence, and a "failure" if the patient had no or only partial relief of symptoms or showed relapse. Factors affecting the treatment outcome were analyzed. RESULTS: The most frequent lesion subtype was myositis. Periorbital edema was the most common symptom and was evident in 53.1% of the patients. Thirty-eight patients (59.4%) showed treatment success. Age, sex, bilaterality, and mean follow-up length did not correlate with the treatment outcome. A short interval from symptom onset to treatment time and apical subtype were significantly associated with good steroid response (p < 0.05). CONCLUSIONS: In orbital inflammatory pseudotumor, myositis was the most common subtype. A short interval from symptom onset to treatment time and apical subtype were associated with good steroid response.

Clinical Characteristics of Pediatric Orbital Pseudotumors

PURPOSE: To describe the clinical manifestations, radiologic findings, and treatment outcomes of pediatric pseudotumors. METHODS: A retrospective chart review of patients diagnosed with pediatric pseudotumor (age under 20 years old) from August 2008 to February 2012 was performed. RESULTS: Thirteen patients (16 eyes) were included in this study. The mean age of the subjects was 14.2 years (5-20 years). Swollen eyelid (56.3%), ptosis (43.8%), conjunctival injection (18.8%), localized mass (18.8%), limitation of ocular movement (12.5%), proptosis (6.3%), and decreased visual acuity (6.3%) were found initially. Dacryoadenitis (62.5%), myositis (18.8%), anterior orbital inflammation (18.8%), and diffuse type (6.3%) were observed on orbital computed tomography (CT). Among the 13 patients (16 eyes), 8 patients (10 eyes) were administered oral systemic corticosteroids, 2 patients (2 eyes) received IV systemic corticosteroid, 1 patient (1 eye) received systemic corticosteroids combined with NSAID, and 2 patients (3 eyes) were prescribed NSAIDs only. Symptoms improved 4.1 days after initiation of treatment. CT scans revealed that one patient experienced diffuse-type disease recurrence twice. CONCLUSIONS: Orbital pseudotumors, which are rare in younger people, are likely to respond very well to corticosteroid treatment, and the recurrence of orbital inflammation is rare.

Surgical Outcome of Levator Recession for Correction of Upper Eyelid Retraction

PURPOSE: To assess the efficacy of levator recession under local anesthesia to treat upper eyelid retraction. METHODS: Records of 12 patients (12 lids) were reviewed retrospectively. Postoperative cosmetic results were assessed as good, fair, or poor based on the upper lid height and symmetry. Preoperative and postoperative marginal reflex distance (MRD1, mm), upper eyelid asymmetry, lid lag, lagophthalmos, and ocular exposure symptoms were compared. The reoperation rate was also evaluated. RESULTS: Causes accounting for upper lid retraction were Graves ophthalmopathy (9 lids, 75.0%), orbital pseudotumor (2 lids, 16.7%), and hypercorrection from previous ptosis operation (1 lid, 8.3%). At a mean +/- standard deviation of 27.7 +/- 24.0 months follow-up (range, 5-60 months), 11 patients (91.7%) showed significantly better cosmetic results. MRD1 decreased an average of 3.1 +/- 1.3 mm from 6.3 +/- 1.5 mm preoperatively to 3.2 +/- 0.9 mm postoperatively (p < 0.001). Upper lid asymmetry, lagophthalmos, and lid lag were also reduced significantly (p < 0.001). Overcorrection occurred in 3 lids (25%) and required levator advancement. Eleven patients (91.7%) experienced complete resolution of dry eye symptoms following levator recession. CONCLUSIONS: Levator recession showed good cosmetic results up to 2 years after surgery for upper eyelid retraction.

Hemangioma cavernoso orbitario: presentación de un caso clínico y revisión del tema / Orbital cavernous hemangioma: report of a case and review of the topic

El hemangioma cavernoso orbitario (HCO) constituye una tumoración vascular benigna no infiltrante, que crece lentamente y en función de la clínica, se decidirá la actitud terapéutica más conveniente, la observación o la extirpación quirúrgica. El diagnóstico definitivo se establece tras la extirpación del tumor y posterior estudio histopatológico del mismo. Presentamos el caso de una niña de 6 años de edad, con proptosis ocular derecha progresiva, con diagnóstico imagenológico sugestivo de lesión vascular, el cual fue confirmado en el estudio anatomo-patológico tras la intervención quirúrgica y la paciente evolucionó satisfactoriamente.