RESUMO
Immunoglobulin G4-related disease (IgG4-RD) is characterized by a systemic fibroinflammatory infiltrate that often involves the orbit in addition to other tissues. Thus it has to be considered in the differential diagnosis of orbital tumors. We report the clinical case of a 64-year-old woman who presented with right mydriasis, progressive proptosis and paralysis of the third cranial nerve of 1 year of evolution. Cranial MRI identified an intraconal lesion of the right orbit, located between the external and inferior rectus muscles and the optic nerve, and she was scheduled for surgery by transcranial approach with lateral micro-orbitomy. A satisfactory macroscopic excision was achieved with no remarkable complications and a definitive deferred histological result of pseudotumor by IgG4-RD. Follow-up for 24 months showed no tumor recurrence, and the patient clinically improved from ophthalmoplegia. This case highlights the efficacy of lateral orbitotomy in the etiologic diagnosis and successful therapeutic outcome of complex orbital lesions associated with IgG4-RD pseudotumor.
Assuntos
Doença Relacionada a Imunoglobulina G4 , Órbita , Pseudotumor Orbitário , Humanos , Feminino , Pessoa de Meia-Idade , Pseudotumor Orbitário/cirurgia , Pseudotumor Orbitário/diagnóstico por imagem , Doença Relacionada a Imunoglobulina G4/cirurgia , Doença Relacionada a Imunoglobulina G4/complicações , Doença Relacionada a Imunoglobulina G4/diagnóstico por imagem , Órbita/cirurgia , Órbita/diagnóstico por imagem , Imageamento por Ressonância Magnética , Exoftalmia/etiologiaRESUMO
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.
Assuntos
Humanos , Masculino , Lactente , História do Século XXI , Órbita , Procedimentos Cirúrgicos Operatórios , Pseudotumor Orbitário , Neurofibromatoses , Neoplasias de Bainha Neural , Face , Neurofibroma , Órbita/anormalidades , Órbita/cirurgia , Procedimentos Cirúrgicos Operatórios/métodos , Pseudotumor Orbitário/cirurgia , Pseudotumor Orbitário/imunologia , Pseudotumor Orbitário/terapia , Neurofibromatoses/cirurgia , Neurofibromatoses/diagnóstico , Neoplasias de Bainha Neural/cirurgia , Neoplasias de Bainha Neural/terapia , Face/cirurgia , Neurofibroma/cirurgia , Neurofibroma/terapiaRESUMO
This is a retrospective case description of a single female patient found to have concomitant bilateral silent sinus syndrome, bilateral thyroid eye disease, unilateral dacryoadenitis, and idiopathic orbital inflammation that presented as progressive unilateral right-sided proptosis. The spectrum of inflammatory orbital diseases can make discerning between different entities challenging, but more unique in this case was the simultaneous presentation with bilateral silent sinus syndrome, a rare entity in its own right. Identifying each of these concurrent disease processes is important to establishing a multidisciplinary treatment approach to address all the patient's orbital and peri-orbital pathology. We hope to highlight the clinical and radiographic findings unique to each of these entities and share our approach to treatment in this complex case.
Assuntos
Dacriocistite/complicações , Oftalmopatia de Graves/complicações , Pseudotumor Orbitário/complicações , Doenças dos Seios Paranasais/complicações , Idoso , Dacriocistite/diagnóstico , Dacriocistite/cirurgia , Descompressão Cirúrgica , Endoscopia , Exoftalmia/etiologia , Feminino , Oftalmopatia de Graves/diagnóstico , Oftalmopatia de Graves/cirurgia , Humanos , Imageamento por Ressonância Magnética , Pseudotumor Orbitário/diagnóstico , Pseudotumor Orbitário/cirurgia , Doenças dos Seios Paranasais/diagnóstico , Doenças dos Seios Paranasais/cirurgia , Estudos Retrospectivos , Tomografia Computadorizada por Raios XAssuntos
Seio Frontal/diagnóstico por imagem , Doença Relacionada a Imunoglobulina G4/diagnóstico por imagem , Imageamento por Ressonância Magnética , Órbita/diagnóstico por imagem , Doenças dos Seios Paranasais/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Diagnóstico Diferencial , Seguimentos , Seio Frontal/patologia , Seio Frontal/cirurgia , Glucocorticoides/uso terapêutico , Humanos , Doença Relacionada a Imunoglobulina G4/patologia , Doença Relacionada a Imunoglobulina G4/cirurgia , Masculino , Órbita/patologia , Órbita/cirurgia , Pseudotumor Orbitário/diagnóstico por imagem , Pseudotumor Orbitário/patologia , Pseudotumor Orbitário/cirurgia , Doenças dos Seios Paranasais/patologia , Doenças dos Seios Paranasais/cirurgia , Complicações Pós-Operatórias/tratamento farmacológico , Rituximab/uso terapêutico , Adulto JovemAssuntos
Seio Etmoidal/anormalidades , Imunoglobulina G/análise , Seio Maxilar/anormalidades , Pseudotumor Orbitário/etiologia , Diplopia/etiologia , Seio Etmoidal/diagnóstico por imagem , Feminino , Humanos , Imageamento por Ressonância Magnética , Seio Maxilar/diagnóstico por imagem , Pessoa de Meia-Idade , Músculos Oculomotores/patologia , Músculos Oculomotores/cirurgia , Pseudotumor Orbitário/diagnóstico por imagem , Pseudotumor Orbitário/imunologia , Pseudotumor Orbitário/cirurgia , Tomografia Computadorizada por Raios XRESUMO
Orbital biopsy of nonspecific orbital inflammation, commonly referred to as "orbital pseudotumor," typically shows a combination of polyclonal lymphocytes, plasmocytes, leukocytes, macrophages, and variable degrees of collagen deposition. Herein, we report a patient with a positive history of mucocutaneous leishmaniasis who presented with an orbital mass with a histological profile of idiopathic orbital inflammation. Immunohistochemical and molecular analysis of the orbital specimens demonstrated that the orbital inflammation was associated with the presence of antigens of Leishmania braziliensis and DNA from the parasite.
Assuntos
Leishmania braziliensis/isolamento & purificação , Pseudotumor Orbitário/parasitologia , Idoso , Humanos , Masculino , Pseudotumor Orbitário/patologia , Pseudotumor Orbitário/cirurgiaAssuntos
Imunoglobulina G/imunologia , Pseudotumor Orbitário/diagnóstico , Paraproteinemias/diagnóstico , Adulto , Humanos , Imageamento por Ressonância Magnética , Masculino , Pseudotumor Orbitário/imunologia , Pseudotumor Orbitário/cirurgia , Paraproteinemias/imunologia , Paraproteinemias/cirurgia , Tomografia de Coerência ÓpticaRESUMO
OBJECTIVES: To describe the clinical features, treatment response, and follow-up of a large cohort of Italian patients with immunoglobulin (Ig)G4-related disease (IgG4-RD) referred to a single tertiary care centre. METHOD: Clinical, laboratory, histological, and imaging features were retrospectively reviewed. IgG4-RD was classified as 'definite' or 'possible' according to international consensus guidelines and comprehensive diagnostic criteria for IgG4-RD. Disease activity was assessed by means of the IgG4-RD Responder Index (IgG4-RD RI). RESULTS: Forty-one patients (15 females, 26 males) were included in this study: 26 with 'definite' IgG4-RD and 15 with 'possible' IgG4-RD. The median age at diagnosis was 62 years. The median follow-up was 36 months (IQR 24-51). A history of atopy was present in 30% of patients. The pancreas, retroperitoneum, and major salivary glands were the most frequently involved organs. Serum IgG4 levels were elevated in 68% of cases. Thirty-six patients were initially treated with glucocorticoids (GCs) to induce remission. IgG4-RD RI decreased from a median of 7.8 at baseline to 2.9 after 1 month of therapy. Relapse occurred in 19/41 patients (46%) and required additional immunosuppressive drugs to maintain long-term remission. Multiple flares occurred in a minority of patients. A single case of orbital pseudotumour did not respond to medical therapy and underwent surgical debulking. CONCLUSIONS: IgG4-RD is an elusive inflammatory disease to be considered in the differential diagnosis of isolated or multiple tumefactive lesions. Long-term disease control can be achieved with corticosteroids and immunosuppressive drugs in the majority of cases.
Assuntos
Doenças Autoimunes/imunologia , Glucocorticoides/uso terapêutico , Imunoglobulina G/imunologia , Imunossupressores/uso terapêutico , Pancreatite/imunologia , Sialadenite/imunologia , Idoso , Doenças Autoimunes/complicações , Doenças Autoimunes/tratamento farmacológico , Estudos de Coortes , Procedimentos Cirúrgicos de Citorredução , Feminino , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Pseudotumor Orbitário/etiologia , Pseudotumor Orbitário/imunologia , Pseudotumor Orbitário/cirurgia , Pancreatite/tratamento farmacológico , Pancreatite/etiologia , Recidiva , Indução de Remissão , Espaço Retroperitoneal , Estudos Retrospectivos , Sialadenite/tratamento farmacológico , Sialadenite/etiologiaRESUMO
INTRODUCTION: Pseudo-tumoral fongal rhinosinusitis is a rare and benign illness due to saprophyte germ, Aspergillus flavus. OBSERVATION: We reported two cases of invasive pseudo-tumoral fongal rhinosinusitis. CT scan helped with diagnosis and allowed for extension assessment. Complete surgical excision was done through external approach. Bacteriological examination evidenced the germ. DISCUSSION: Pseudo-tumoral invasive fongal rhinosinusitis remains a potentially serious pathology because of its local aggressiveness and its multiple extensions.
Assuntos
Aspergilose/complicações , Pseudotumor Orbitário/microbiologia , Rinite/microbiologia , Sinusite/microbiologia , Adolescente , Adulto , Aspergilose/diagnóstico por imagem , Aspergilose/cirurgia , Aspergillus/isolamento & purificação , Exoftalmia/diagnóstico por imagem , Exoftalmia/microbiologia , Exoftalmia/cirurgia , Feminino , Humanos , Pseudotumor Orbitário/diagnóstico por imagem , Pseudotumor Orbitário/cirurgia , Radiografia , Rinite/diagnóstico por imagem , Rinite/cirurgia , Sinusite/diagnóstico por imagem , Sinusite/cirurgiaRESUMO
OBJECTIVE: This case report presents our experience of endoscopic transnasal management of medial intra- and extraconal lesions. CASE REPORT: An endoscopic transnasal approach to intra-orbital lesions was used for nine patients. Four patients had intraconal lesions and five had lesions in the extraconal space. Post-operatively, seven patients reported an improvement in visual acuity and two reported stable vision. There were no complications of cerebrospinal leakage or diplopia. CONCLUSION: This case series demonstrated the safety and effectiveness of an endoscopic transnasal approach to managing intraconal and extraconal lesions. This minimally invasive technique should be considered a valid alternative for accessing orbital tumours, particularly those located in the medial compartment.
Assuntos
Endoscopia/métodos , Neoplasias Orbitárias/secundário , Neoplasias Orbitárias/cirurgia , Pseudotumor Orbitário/cirurgia , Adulto , Idoso , Criança , Estudos de Viabilidade , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neoplasias Orbitárias/diagnóstico , Pseudotumor Orbitário/diagnóstico , Cuidados Pós-Operatórios , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/terapia , Tomografia Computadorizada por Raios XAssuntos
Granuloma de Células Plasmáticas/diagnóstico , Laringoscopia , Doenças Nasais/diagnóstico , Pseudotumor Orbitário/diagnóstico , Idoso , Biópsia , Diagnóstico Diferencial , Gadolínio , Granuloma de Células Plasmáticas/cirurgia , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Procedimentos Cirúrgicos Nasais/métodos , Neoplasias Nasais/diagnóstico , Procedimentos Cirúrgicos Oftalmológicos/métodos , Pseudotumor Orbitário/cirurgia , Tomografia Computadorizada por Raios X/métodos , Resultado do TratamentoRESUMO
Inflammatory myofibroblastic tumor (IMT) is a neoplasm most commonly found in the abdominal-pelvic region, lung, and retroperitoneum. The tumor tends to affect soft tissues of children and young adults and can locally recur but rarely metastasizes. Histologically, the appearance is one of bland spindle cell proliferation with a prominent, chronic inflammatory infiltrate. This article describes 1 case of IMT found in the orbit that is presented with rapidly progressive painless proptosis. In the authors' review of the literature, they have only found 2 other case reports involving the orbit.
Assuntos
Miofibroblastos/patologia , Pseudotumor Orbitário/patologia , Biomarcadores/metabolismo , Crioterapia , Exoftalmia/diagnóstico , Humanos , Imageamento por Ressonância Magnética , Masculino , Miofibroblastos/metabolismo , Procedimentos Cirúrgicos Oftalmológicos , Pseudotumor Orbitário/metabolismo , Pseudotumor Orbitário/cirurgia , Adulto JovemRESUMO
The purpose of this report is to describe the clinical, radiographica, and histopathologic findings in two pediatric patients with orbital IgG4-related disease.
Assuntos
Imunoglobulina G/imunologia , Órbita/patologia , Pseudotumor Orbitário/patologia , Adolescente , Criança , Edema/diagnóstico , Eosinófilos/patologia , Doenças Palpebrais/diagnóstico , Feminino , Histiócitos/patologia , Humanos , Pseudotumor Orbitário/diagnóstico por imagem , Pseudotumor Orbitário/imunologia , Pseudotumor Orbitário/cirurgia , Plasmócitos/patologia , Esclerose/patologia , Tomografia Computadorizada por Raios XRESUMO
PURPOSE: To evaluate the outcomes of patients with idiopathic sclerosing orbital inflammation (ISOI) treated with radiotherapy with or without surgery. METHODS AND MATERIALS: We retrospectively reviewed 22 patients with histopathologically confirmed ISOI who had been refractory or intolerant to steroid therapy and treated with radiation with or without surgery. The radiation dose ranged from 20 to 40 Gy (median, 20 Gy) at 2 Gy per fraction. Presenting signs and treatment outcomes were assessed. RESULTS: Proptosis was the most common sign at presentation, seen in 19 (86.3%) patients, followed by restriction of extraocular movements in 10 (45.4%) patients. Response to radiotherapy was complete in 15 (68.1%) patients, partial in 3 (13.6%) patients, and none in 4 (18.2%) patients. At the median follow-up of 34 months, 14 (63.6%) patients had progression-free state of symptoms and signs, with the progression-free duration ranging from 3 to 75 months (median, 41.5 months), whereas 8 (36.4%) patients had recurrent or persistent disease although they had received radiotherapy. Of the 14 progression-free patients, 6 underwent a bimodality treatment of debulking surgery of ocular disease and radiotherapy. They had had no recurrent disease. Cataract was the most common late complications, and 2 patients experienced a Grade 3 cataract. CONCLUSION: Our study suggests that for patients with ISOI who are refractory or intolerant to steroid therapy, 20 Gy of radiotherapy appears to be effective for the control of disease with acceptable complications, especially when it is combined with surgery.
Assuntos
Pseudotumor Orbitário/radioterapia , Adulto , Idoso , Terapia Combinada/efeitos adversos , Terapia Combinada/métodos , Exoftalmia/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pseudotumor Orbitário/complicações , Pseudotumor Orbitário/tratamento farmacológico , Pseudotumor Orbitário/cirurgia , Dosagem Radioterapêutica , Indução de Remissão/métodos , Estudos Retrospectivos , Terapia de Salvação/efeitos adversos , Terapia de Salvação/métodos , Esteroides/uso terapêutico , Resultado do TratamentoRESUMO
Idiopathic orbital inflammatory pseudotumor is a chronic neoplasm-like inflammatory reaction, usually affecting the orbital tissues of both eyes and orbit. Isolated optic nerve involvement by the idiopathic inflammatory process is a rare finding and very few reports are available. Here a case of an isolated orbital nerve inflammatory pseudotumor presenting with gradually progressive unilateral loss of vision is described. It showed dramatic response to surgery and steroid treatment and its differential diagnoses are discussed.
Assuntos
Pseudotumor Orbitário/diagnóstico , Pseudotumor Orbitário/cirurgia , Descompressão Cirúrgica , Diagnóstico Diferencial , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Órbita/patologia , Órbita/cirurgia , Pseudotumor Orbitário/patologiaRESUMO
Herein we report a 52-year-old man with subacute right-sided proptosis and diffuse intraconal enhancing abnormality on MRI. Orbital biopsy revealed granulomatous inflammation consistent with idiopathic orbital inflammatory syndrome (IOIS), or orbital pseudotumor. However, further examination under polarizing light microscopy also revealed acetate fiber fragments within the orbit. Prominent speckles within the acetate fibers were identified as titanium by Energy Dispersive X-ray Analysis (EDXA). Acetate impregnated with titanium (as a delustrant) is a common synthetic fiber used in textile and clothing manufacture. The mechanism for entrance into the orbit in this case is not known. Granulomatous idiopathic orbital inflammatory syndrome without local or systemic cause is an uncommon clinical entity, with less than 50 cases reported in the literature. Predominance of lacrimal gland (and thus superficial) involvement in granulomatous IOIS suggests the possibility of occult foreign body in such cases.
Assuntos
Granuloma de Corpo Estranho/etiologia , Granuloma de Corpo Estranho/cirurgia , Pseudotumor Orbitário/diagnóstico , Pseudotumor Orbitário/cirurgia , Titânio/efeitos adversos , Biópsia por Agulha , Análise Química do Sangue , Meios de Contraste , Exoftalmia/diagnóstico , Exoftalmia/etiologia , Seguimentos , Granuloma de Corpo Estranho/diagnóstico , Humanos , Imuno-Histoquímica , Imageamento por Ressonância Magnética/métodos , Masculino , Manometria/métodos , Pessoa de Meia-Idade , Pseudotumor Orbitário/etiologia , Cuidados Pós-Operatórios/métodos , Prednisona/uso terapêutico , Resultado do TratamentoRESUMO
Orbital pseudotumor is a benign condition that accounts for approximately 10% of all orbital mass lesions. Any part of the orbit may be involved. The etiology is unknown. The presentation may be either acute or subacute. Patients may present with a palpable mass, a swollen eyelid, congestion, pain, diminished ocular motility, and/or decreased visual acuity. Approximately, 25% of patients present with bilateral disease. A modest proportion of patients experience resolution of their symptoms without treatment. Biopsy is indicated for those who do not respond to, or relapse after, first-line therapy. Oral corticosteroids are the initial treatment and approximately 80% of patients respond. Roughly half of those who respond to corticosteroids relapse. Second-line therapy consists of either low-dose radiotherapy (20-30 Gy at 2 Gy per fraction), cytotoxic chemotherapy, or immunosuppressive agents. Radiotherapy results in long-term local control rates of 50% or higher. Limited lesions may be successfully resected. A small subset of patients may experience inexorable progression to a fixed, painful, sightless eye and require orbital exenteration.
Assuntos
Pseudotumor Orbitário , Corticosteroides/uso terapêutico , Anti-Inflamatórios não Esteroides/uso terapêutico , Antineoplásicos/uso terapêutico , Biópsia , Diagnóstico Diferencial , Diagnóstico por Imagem/métodos , Humanos , Imunossupressores/uso terapêutico , Neoplasias Orbitárias/diagnóstico , Pseudotumor Orbitário/diagnóstico , Pseudotumor Orbitário/tratamento farmacológico , Pseudotumor Orbitário/patologia , Pseudotumor Orbitário/radioterapia , Pseudotumor Orbitário/cirurgia , Resultado do TratamentoRESUMO
AIMS: It was the aim of this study to perform a review of the clinical features, treatments and outcomes of patients with idiopathic sclerosing orbital inflammation (ISOI) in Taiwan. METHODS: All patients with histologically confirmed ISOI from 1995 to 2007 at the National Taiwan University Hospital were included. We reviewed the medical charts to see the clinical presentation, treatment modalities, treatment efficacy and final clinical status of these patients. RESULTS: Six male and 3 female patients ranging in age from 33 to 82 years were included. The most common symptoms were pain and blurred vision (both 77.8%). Reduced visual acuity and proptosis were the most common signs (both 89%). Five patients received oral steroid treatment with/without pulse therapy combined with tissue removal, the response to which was good in 1 patient and partially effective in the other 4 patients. One patient received oral steroids and surgery, followed by radiotherapy, with only partial response. Patients with oral prednisolone or surgery only all had poor responses. CONCLUSION: ISOI is rare and also difficult to diagnose and manage. Early intervention with steroids/immunosuppressants combined with surgical debulking procedures may result in control or regression of this recalcitrant disease.