Within each disease category, specific diseases and their associated therapies have been addressed. Orbit pseudotumor an overview sciencedirect topics. Idiopathic inflammatory pseudotumor accounts for between 3% and 8. Nonspecific orbital inflammation nsoi, also known as orbital inflammatory pseudotumor, idiopathic orbital inflammation ioiand orbital inflammatory syndrome is the most common cause of painful orbital mass in adults. A 45yearold female patient presented as a walkin to our clinic with the chief complaint a swollen and painful right eye. Two search methods were used to identify subjects with a diagnosis of orbital cellulitis, ois, and orbital lymphoid lesions. When localized, inflammation can affect the extraocular muscles orbital myositis, lacrimal gland dacryoadenitis, sclera scleritis.
It is a chronic condition that acts much like a brain tumor. Idiopathic orbital inflammatory syndrome, also known as orbital pseudotumor, was. Orbital pseudotumor does not spread to other tissues or places in the body. Inflammatory orbital pseudotumor iop, also known as idiopathic orbital pseudotumor, is a benign, noninfective, inflammatory condition of the orbit without systematic or locally identifiable. Orbital pseudotumor, also known as orbital inflammatory pseudotumors oip, idiopathic orbital inflammation ioi, orbital inflammatory syndrome ois, idiopathic orbital inflammatory pseudotumor ioip, or nonspecific orbital inflammation nsoi is a benign, spaceoccupying, and noninfectious inflammatory condition of the orbit but may extend in the periorbital area. Pseudotumor orbital inflammatory syndrome idiopathic. Most orbital lymphomas are lowgrade neoplastic lesions, and the most frequently observed subtype is mucosaassociated lymphoid tissue malt lymphoma. Orbital pseudotumor is a common cause of unilateral proptosis in adults and accounts for approximately 10% of orbital mass lesions, 2 but is rarely seen in children. Orbital pseudotumor also known as idiopathic orbital inflammatory syndrome iois is a benign, noninfective inflammatory condition of the orbit without. Idiopathic orbital inflammatory syndrome, also known as orbital pseudotumor, first described by gleason in 1903 and by busse and hochhmein shortly after gleason 1903 characterized as a distinct entity in 1905 by birchhirschfeld. Inflammatory orbital pseudotumor can affect any or all structures within the orbit. The inflammation can be part of an underlying medical disorder eg, igg4related orbital disease or. Pdf idiopathic orbital inflammation with extraorbital.
Worlds best powerpoint templates crystalgraphics offers more powerpoint templates than anyone else in the world, with over 4 million to choose from. Dd include orbital cellulitis, wegener granulomatosis and lymphoma. Nonspecific orbital inflammation nsoi, also known as orbital inflammatory pseudotumor, idiopathic orbital inflammation ioiand orbital. Orbital inflammatory pseudotumor is a rare inflammatory condition of unknown cause that may extend intracranially, usually as a duralbased infiltrate. Intracranial extension of orbital inflammatory pseudotumor. Orbital inflammatory syndrome ois, commonly known as inflammatory orbital pseudotumor, is the most common cause of nonthyroidrelated noninfectious orbital disease. May not be a specific disease process, but due to various causes paranasal sinus tumors, rosaidorfman disease, inflammatory fibrosclerosis, dysthyroid ophthalmopathy, cholesterol or keratin granulomas, traumatic fat necrosis, prior hemorrhage or abscess. Idiopathic orbital inflammatory pseudotumor in chinese.
Some presentations of iois may mimic common conditions such as orbital cellulitis and optic neuritis. Many reports of inflammatory pseudotumor in different sites may be examples of undiagnosed igg4rd 6, 7. It is the most common painful orbital mass in the adult population, and is associated with proptosis, cranial nerve palsy tolosahunt syndrome, uveitis, and retinal detachment. Winner of the standing ovation award for best powerpoint templates from presentations magazine. She reported dizziness but denied diplopia, fever, and history of any trauma. The truth about mobile phone and wireless radiation dr devra davis duration. To study the clinical features of idiopathic orbital inflammatory pseudotumor ioip in chinese children. Idiopathic orbital inflammation ioi, formerly called orbital pseudotumor, is an unusual orbitalspecific disorder of unknown origin, characterized by nonspecific orbital inflammation. Using pacs, the keywords cellulitis, pseudotumor, and.
Orbital pseudotumor is the swelling of tissue behind the eye in an area called the orbit. The inflammatory response can be nonspecific, granulomatous, or vasculitic or due to reactive lymphoid hyperplasia. Orbital pseudotumor, also known as idiopathic orbital inflammatory syndrome iois, may have protean clinical manifestations. The first case reported was described by hamano et al several young patients with pancreatitis with no clear etiology and high serum levels of igg4 ended up developing pancreas sclerosis 1. It is a hypocellular lymphoid lesion, often incompletely replacing the orbital fat, lacrimal gland and extraocular muscles in which mature lymphocytes, plasma cells, histiocytes are seen.
Orbital pseudotumor, or idiopathic orbital inflammatory syndrome, is a nonspecific inflammatory process of unknown etiology that may mimic orbital lymphoma and comprises some 10% of all orbital mass lesions. Pdf orbital inflammatory disease oid represents a collection of inflammatory conditions affecting the orbit. The orbit protects the eyeball and the muscles and tissue that surround it. Orbital inflammatory pseudotumor symptoms and treatment upmc. Orbital lymphoma and idiopathic orbital inflammatory pseudotumor ioip represent the most common lymphoproliferative disorders affecting the orbit, accounting nearly 20% of all orbital mass lesions 1,2,3. Importance current practice to diagnose idiopathic orbital inflammation ioi is inconsistent, leading to frequent misdiagnosis of other orbital entities, including cancer. Wegeners granulomatosis presenting as orbital pseudotumor. Orbital inflammatory pseudotumor symptoms and treatment. Orbital pseudotumor is an idiopathic inflammatory condition that usually involves the extraocular muscles although, in some cases there is inflammatory change involving the uvea, sclera, lacrimal gland and retrobulbar soft tissues. Case report idiopathic orbital inflammatory pseudotumor. Idiopathic orbital inflammatory syndrome orbital pseudotumor. Idiopathic orbital inflammatory syndrome iois, also known as orbital pseudotumor, is one of the most common acute orbital processes. Orbital inflammatory pseudotumor, magnetic resonance imaging, mri, orbital mass, intracranial extension background inflammatory pseudotumor is a rare benign condition of unknown cause, characterized by unencapsulated masslike aggregates of myofibroblastic spindle cells and inflammatory cells, including lymphocytes 1. First described by birchhirschfield in 1905, idiopathic orbital inflammatory syndrome, also known as orbital pseudotumor, is a nonspecific, nonneoplastic inflammatory process of the orbit.
Diagnostic criteria of idiopathic orbital inflammation. The term pseudotumor was coined because these lesions mimic expansive, invasive, malignant tumors, both clinically and. Inflammatory pseudotumor ipt is a rarely occurring lesion with no identifiable local or systemic cause. Idiopathic orbital inflammation ioi, also known as orbital pseudotumor, is defined as a nonspecific, nonneoplastic inflammatory process of the orbit without identifiable local or. Unlike a tumor, however, the pseudotumor does not spread and does not invade nearby tissues. Extraorbital inflammatory pseudotumor of the head and neck. Less commonly there is inflammatory change involving the uvea, sclera, lacrimal gland, and retrobulbar soft tissue. Sclerosing inflammatory pseudotumor of the eye cornea. Orbital inflammatory pseudotumor and ischemic vasculitis in churgstrauss syndrome. Objective to define a set of criteria specific for the diagnosis of ioi design, setting, and participants a 3round modified delphi process with. The orbit is the hollow space in the skull where the eye sits. Orbital inflammatory pseudotumor is a rare inflammatory condition of unknown cause that may.
Pdf idiopathic orbital inflammatory pseudotumor is a chronic neoplasmlike inflammatory reaction, usually affecting the orbital tissues of both. There is no identifiable infectious, systemic, or neoplastic disorder. Inflammatory pseudotumor is a quasineoplastic lesion consisting of inflammatory cells and myofibroblastic spindle cells, 2, 3. Orbital inflammatory pseudotumor also known as idiopathic orbital inflammation syndrome, orbital pseudotumor, nonspecific orbital inflammation, and orbital inflammatory syndrome is defined as an idiopathic tumorlike inflammation. Idiopathic orbital inflammatory disease an overview. Surgical resection was the best treatment of choice, and orbital inflammatory pseudotumor also showed excellent response to steroid 14, but a high number of cases might recur or become. Nonspecific orbital inflammation idiopathic orbital. Orbital inflammation is typically an idiopathic process that occasionally may be identified with a specific local or systemic disease as the causative agent. Their clinical features were compared with those of adult cases of ioip. Idiopathic orbital inflammatory disease pseudotumor is a nonspecific inflammation involving the orbit.
Here we report the first case of orbital pseudotumor presenting with intraaxial magnetic resonance imaging mri changes. Differential diagnosis of oid ranges from idiopathic inflammatory disease to systemic or local inflammatory conditions to other associated. First described by birchhirschfield in 1905, idiopathic orbital inflammatory syndrome. It is also known as idiopathic orbital inflammatory syndrome iois. Idiopathic orbital inflammation american academy of. Mr imaging of orbital inflammatory syndrome, orbital. Orbital inflammatory syndrome orbital pseudotumor ento key. Theyll give your presentations a professional, memorable appearance the kind of sophisticated look that todays audiences expect. Radiologists should be familiar with this entity as a diagnostic consideration to avoid unnecessary surgery. Inflammatory orbital disease is a benign spaceoccupying inflammation involving orbital tissues. Inflammatory pseudotumor is a rare benign process mimicking malig nant processes and has been found in almost every organ system.
Idiopathic orbital inflammation radiology reference. An orbital inflammatory pseudotumor is an inflammatory reaction in the orbital tissues that surround the eyes. The nonsclerosing type is more common and has been reported to occur in almost every part of the body, including orbital tissues and the uveal tract. Improvements in diagnostic techniques as also better understanding of the pathology of orbital pseudotumor helped us to define orbital pseudotumor as a nonspecific idiopathic, benign inflammatory process characterized by polymorphous lymphoid infiltrate with varying degrees of fibrosis.
Pseudotumor orbit accounts for 10% of orbital tumors3. Inflammatory orbital disease msd manual professional edition. Nonspecific orbital inflammation idiopathic orbital inflammation. By specifying criteria, diagnosis of orbital inflammation will be improved. Orbital pseudotumor information mount sinai new york. Idiopathic orbital inflammation ioi, also known as orbital pseudotumor and nonspecific orbital inflammation, is an idiopathic inflammatory condition that most commonly involves the extraocular muscles. Idiopathic orbital inflammatory ioi disease, refers to a marginated masslike enhancing soft tissue involving any area of the orbit. Inflammatory pseudotumor most commonly involves the lung and the orbit, but it has been reported to occur in nearly every site in the body, from the central nervous system to the gastrointestinal tract, table 2.
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