Pseudotumor orbit accounts for 10% of orbital tumors3. The orbital pseudotumor is often presented in the medical field as the idiopathic orbital inflammatory syndrome abbreviated as iois. Pseudotumor cerebri symptoms and causes mayo clinic. The orbital pseudotumor accounts for 510% of all the orbital processes. Inflammatory orbital disease msd manual professional edition. Idiopathic orbital inflammation ioi, also known as orbital pseudotumor. Foci of inflammation include the extraocular muscles, the lacrimal gland, the episcleral tissue, the. Iois can involve virtually any orbital tissue individually, or in combination. Orbital inflammatory disease oid represents a spectrum of. Richards abskalka hwroberts fjflint a pseudotumor of the orbit and retroperitoneal fibrosis. It is among the 3 rd most common orbital diseases along with thyroid orbitopathy and lymphoproliferative disorder and accounts for 510% of orbital processes. It may be acute, subacute, chronic, or is rare cases recurrent. Unilateral disease is the rule but bilateral presentations.
In the days before ct and mri scans, doctors who noted swelling of the op tic disc the beginning of the optic nerve in the back of the eye were. Pseudotumor medical definition merriamwebster medical. The orbit protects the eyeball and the muscles and tissue that surround it. Orbital pseudotumor idiopathic orbital inflammatory disease is a nongranulomatous, acute or subacute inflammatory disease that usually occurs in the anterior orbit or midorbit and often involves the lacrimal gland. Probability in medicine, levels of uncertainty, and the diagnosis of orbital disease with particular reference to orbital pseudotumor. Supervoltage radiotherapy was used in 21 orbits of 19 patients with idiopathic inflammatory orbital pseudotumor. Occlusive and constrictive changes of the proximal segments of the. It is considered among the most common diseases of the orbit, after the thyroid orbitopathy and the lymphoproliferative disorder. Sep 11, 2019 pseudotumor cerebri soodoetoomur seruhbry occurs when the pressure inside your skull intracranial pressure increases for no obvious reason. 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 inflammation or pseudotumor represents a nongranulomatous inflammatory process in the orbit or eye with no known local or systemic causes.
Noninfectious etiologies are abundant, but often grouped under the heading of orbital pseudotumor or idiopathic orbital inflammation until a specific etiology is elucidated. Surfacecoil mr of orbital pseudotumor 141 fifteen patients with clinical presentations compatible with idiopathic inflammatory orbital pseudotumor were examined by ct and mr imaging to determine if mr could add specificity to the ct appearance of this entity. Iois is a diagnosis of exclusion, made only after other entities have been ruled out. Idiopathic orbital pseudotumor is extremely rare in pediatric age group and may cause real diagnostic problems. Idiopathic sclerosing orbital inflammation clinical pharmacy and. Scribd is the worlds largest social reading and publishing site. The orbit is the hollow space in the skull where the eye sits. First described by birchhirschfield in 1905, idiopathic orbital inflammatory syndrome, also known as orbital pseudotumor, is a nonspecific, nonneoplastic inflammatory process of the orbit. Myositic type of idiopathic orbital pseudotumor in a 4year.
Orbital pseudotumor nonspecific orbital inflammatory disease introduction. Idiopathic orbital inflammatory syndrome, also known as orbital. But, by local growth it can cause bulging of the eye proptosis, double vision diplopia and loss of vision. Orbital pseudotumor uf health, university of florida health. Inflammatory orbital disease is a benign spaceoccupying inflammation involving orbital tissues. 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. Idiopathic orbital inflammatory disease pseudotumor is a nonspecific inflammation involving the orbit. Sclerosing orbital pseudotumor can rarely extend into the sinuses, brain, and other orbit. 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. Biopsy is usually not performed at presentation, as the risk of producing damage to vital structures within the orbit outweighs the benefits. Association of orbital vein deformities and myositis 305 twenty adult patients with orbital pseudotumor were studied with orbital venography and magnification arteriography. This is a condition in which high pressure inside your head can cause problems with vision and headache. 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. It is a hypocellular lymphoid lesion, often incompletely replacing the orbital fat, lacrimal gland and extraocular muscles in which mature.
Orbital pseudotumor is the swelling of tissue behind the eye in an area called the orbit. Clinically, orbital pseudotumor has been categorized as myositis, dacryoadenitis, anterior, apical and diffuse process. Orbital pseudotumor is a benign intraorbital process confined to the orbit but extra orbital involvement can occur. Anterior orbital pseudotumor posterior scleritis orbital pseudotumor is a benign idiopathic inflammatory process involving the structures in the orbit which may present in several different patterns. In our study, idiopathic orbital inflammation occurred more frequently in middleaged persons, affected more women than men, and typically manifested with unilateral periorbital pain and edema. 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 does not spread to other tissues or places in the body. Infectious etiologies include bacteria and fungi, most often related to sinus disease.
There may be a marginated enhancing soft tissue mass that involves any area of the orbit socket surrounding the eye. Inflammatory orbital pseudotumor iop, also known as idiopathic orbital pseudotumor, is a benign, noninfective, inflammatory condition of the orbit without systematic or. Orbital pseudotumor definition of orbital pseudotumor by. Clinically, orbital pseudotumor has been categorized as myositis, dacryoadenitis, anterior. Nonspecific orbital inflammation nsoi, also known as orbital inflammatory pseudotumor, idiopathic orbital inflammation ioiand orbital. Orbital pseudotumor igg4rd is a major cause of orbital inflammatory disease and appears to account for 25 to 50 percent of orbital pseudotumors. Right orbital pseudotumor with preorbital cellulitis orbital pseudotumor is an idiopathic inflammatory lesion of the orbit most commonly involving the extraocular muscles most commonly the lateral rectus muscle, but can involve any of the orbital contents including the retroorbital fat and the lacrimal gland. Idiopathic orbital inflammatory syndrome, also known as orbital pseudotumor, is a syndrome of nonspecific inflammation of orbital tissues with no identifiable local or systemic cause. Orbital inflammatory syndrome orbital pseudotumor ento key. Pseudotumor definition of pseudotumor by medical dictionary.
Pdf the purpose of this paper is to provide an overview of the diagnosis and management of orbital pseudo tumor. Its also called idiopathic intracranial hypertension. There may be a marginated enhancing soft tissue mass that involves any area of. Myositic type of idiopathic orbital pseudotumor in a 4.
It is also known as idiopathic orbital inflammatory syndrome iois. Computed tomography ct scans were obtained in 14 patients. The cellular infiltrate of orbital pseudotumor tends to be diffuse and multifocal in contrast to lymphoid neoplasm 1. To provide an overview of the spectrum of diseases known as idiopathic orbital inflammatory syndrome also known as orbital pseudotumor, with emphasis on. Idiopathic orbital inflammatory disease an overview. It consists of hypocellular polymorphous infiltrate composed of mature lymphocytes, plasma cells, macrophages and. Ultrasound and computed tomographic ct scanning typically shows a diffuse infiltration of the orbit, an inflammation of the eye wall sclera, andor tsign with the optic nerve. Pseudotumor of the orbit and limited wegeners granulomatosis. Orbital pseudotumor igg4rd is a major cause of orbital inflammatory disease and appears to account for 25 to 50 percent of orbital pseudotumors, including those originally orbital cellulitis view in chinese. Orbital pseudotumor is typically characterized by the rapid development of pain, proptosis, and swelling around the eye and orbit in adults. Intracranial extension of orbital inflammatory pseudotumor. This patient was considered to have the limited form of wegeners granulomatosis that has been described recently, and his 15year survival with this disease when last seen is unequaled in the literature. Nonspecific orbital inflammation idiopathic orbital inflammation. Orbital magnetic resonance imaging is the single most important diagnostic test, but serologic studies are necessary to exclude a systemic cause.
Orbital pseudotumor is a benign, noninfective, protean clinical syndrome of unknown etiology. This paper describes a 4yearold girl who presented with sudden ptosis in. She reported dizziness but denied diplopia, fever, and history of any trauma. Seventeen orbits 15 patients were initially treated with systemic corticosteroids, but recurrence of orbital inflammation during dosage tapering was the most frequent indication for radiotherapy. A patient with bilateral orbital pseudotumor and bilateral cavitary pulmonary lesions is reported. Pdf to provide an overview of the spectrum of diseases known as idiopathic orbital. It is the third most common orbital disease after thyroid orbitopathy and lymphoproliferative disorder.
Bilyk basics description idiopathic orbital inflammatory syndrome iois is a nonspecific inflammation of orbital tissue with no identifiable local or systemic cause. Definitionorbital pseudotumor is the swelling of tissue behind the eye in an area called the orbit. Free abstract a 42yearold immunecompetent woman with orbital pseudotumor and presumed intracranial extension into the temporal lobe responded well clinically and radiologically to highdoses of corticosteroids. Idiopathic orbital pseudotumor is a benign, noninfectious, and nonneoplastic disease with unknown cause. Are systemic corticosteroids useful in the management of orbital pseudotumors. Pseudotumor cerebri is a condition in which the pressure around your brain increases, causing headaches and vision problems. A 45yearold female patient presented as a walkin to our clinic with the chief complaint a swollen and painful right eye. The inflammatory response can be nonspecific, granulomatous, or vasculitic or due to reactive lymphoid hyperplasia.
Pseudotumor orbital inflammatory syndrome idiopathic. The classical form of orbital pseudotumor is the cellular variety which presents acutely and mimics lymphoid tumors. Fineneedle aspiration biopsy is useful in evaluating patients with presumed orbital pseudotumor. Right orbital pseudotumor with pre orbital cellulitis orbital pseudotumor is an idiopathic inflammatory lesion of the orbit most commonly involving the extraocular muscles most commonly the lateral rectus muscle, but can involve any of the orbital contents including the retro orbital fat and the lacrimal gland. Inflammatory orbital pseudotumor can affect any or all structures within the orbit. Idiopathic orbital pseudotumor iop is a benign inflammatory condition of the orbit without identifiable local or systemic causes. Pseudotumor cerebri is a brain condition that causes the same symptoms as a brain tumor. Idiopathic orbital inflammation is a multifaceted disease with a wide spectrum of clinical, radiological, and histopathological presentations.
Less commonly there is inflammatory change involving the uvea, sclera, lacrimal gland, and retrobulbar soft tissue. Orbital pseudotumor symptoms, diagnosis, treatment. A 45yearold female patient presented as a walkin to our clinic with. Orbital pseudotumor occurs more often in the third to sixth decades 5 with no strong sex predilection 4. Clinically, orbital pseudotumor has been categorized as myositis, dacryoadenitis, anterior, apical and diffuse. Orbital pseudotumor information mount sinai new york. Iois can involve any orbital tissue individually or in combination. 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. Radiotherapy for idiopathic inflammatory orbital pseudotumor. The increased intracranial pressure can cause swelling of the optic nerve and result in vision loss.
Idiopathic orbital inflammation radiology reference article. Pseudotumor cerebri your doctor thinks you may have pseudotumor cerebri. Orbital inflammation is generally categorized as infectious or noninfectious. Idiopathic orbital inflammatory ioi disease, refers to a marginated masslike enhancing soft tissue involving any area of the orbit.