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. In this report we describe four cases of orbital pseudotumor with extension beyond the orbit. Here we report the first case of orbital pseudotumor presenting with intraaxial magnetic resonance imaging mri changes. Orbital pseudotumor information mount sinai new york. 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. 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. Clini cally, the patient exhibits acute onset of propto sis, chemosis, pain, diplopia, and impaired ocular motility or, in some cases, a subacute or chronic onset of these symptoms and signs. It consists of hypocellular polymorphous infiltrate composed of mature lymphocytes, plasma cells, macrophages and. Idiopathic orbital inflammatory disease pseudotumor is a nonspecific inflammation involving the orbit. Two cases of orbital pseudotumor are described with computed tomographic ct studies showing extension of the pseudotumor beyond the confines of the bony orbit. Orbital pseudotumor which is also referred to as idiopathic orbital inflammatory disease or orbital inflammation or orbital swelling is one of the most common causes of a painful orbital mass in adults eye diseases. Hpi patient is a 63 year old female with a one year history of a right orbital mass that has mildly enlarged during this time period.
Ppt orbital tumors powerpoint presentation free to. Probability in medicine, levels of uncertainty, and the diagnosis of orbital disease with particular reference to orbital pseudotumor. A second patient had extension of orbital pseudotumor into the. This is a condition in which high pressure inside your head can cause problems with vision and headache. In one patient with orbital pseudotumor involving the medial rectus muscle, the pseudotumor extended through the medial wall of the orbit into the ethmoid sinus. Unilateral disease is the rule but bilateral presentations. 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. Idiopathic orbital inflammation ioi, also known as orbital pseudotumor, is defined as a nonspecific, nonneoplastic inflammatory process of the orbit without identifiable local or. Orbital pseudotumor international journal of emergency. Idiopathic orbital inflammation is a multifaceted disease with a wide spectrum of clinical, radiological, and histopathological presentations. A 45yearold female patient presented as a walkin to our clinic with the chief complaint a swollen and painful right eye. 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. The extraorbital extension of the orbital pseudotumor passed through the superior orbital fissure in all cases, through the inferior orbital fissure in two cases, and through the optic canal in one case. 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.
Less commonly there is inflammatory change involving the uvea, sclera, lacrimal gland, and retrobulbar soft tissue. Mr imaging of orbital inflammatory pseudotumors with. The types of orbital pseudotumors were a mass in the orbital apex n 3, diffuse form n 2, and myositis n 1. Current concepts in the diagnosis and management of idiopathic orbital inflammation. Orbital pseudotumor with cavernous sinus and sellar extension.
Differential diagnostic considerations included neoplastic processes such as orbital lymphoma with intracranial extension or sphenoid wing meningioma with intraorbital extension. Inflammatory orbital pseudotumor iop, also known as idiopathic orbital pseudotumor, is a benign, noninfective, inflammatory condition of the orbit without systematic or locally identifiable. The cellular infiltrate of orbital pseudotumor tends to be diffuse and multifocal in contrast to lymphoid neoplasm 1. The orbital pseudotumor is often presented in the medical field as the idiopathic orbital inflammatory syndrome abbreviated as iois. There may be a marginated enhancing soft tissue mass that involves any area of the orbit socket surrounding the eye. Inflammatory orbital pseudotumor is a benign, intraorbital inflammatory process of unknown etiology. Oct 05, 2017 the key findings with orbital inflammatory pseudotumor include pain, redness, diplopia, and decreased visual acuity, with proptosis, chemosis. Orbital inflammatory pseudotumor is a rare inflammatory condition of unknown cause that may extend intracranially, usually as a duralbased infiltrate. This paper describes a 4yearold girl who presented with sudden ptosis in. Nov 17, 2009 orbital pseudotumor an idiopathic orbital inflammation, a diagnosis of exclusion, is the third most common orbital disease, accounting for 56% of orbital disorders. To demonstrate a variety of mr imaging findings of orbital inflam matory pseudotumors with extraorbital extension. Idiopathic orbital inflammation orbital pseudotumor this is a challenging disease to diagnose with certainty, since it is a diagnosis of exclusion.
Idiopathic orbital inflammatory ioi disease, refers to a marginated masslike enhancing soft tissue involving any area of the orbit. Orbital pseudotumor can be a localized form of granulomatosis with polyangiitis as revealed by gene expression profiling james t. Orbital pseudotumor occurs more often in the third to sixth decades 5 with no strong sex predilection 4. Inflammatory orbital disease is a benign spaceoccupying inflammation involving orbital tissues.
The orbital pseudotumor accounts for 510% of all the orbital processes. Idiopathic orbital inflammatory pseudotumor is an inflammatory lesion of the orbit without any recognizable local or systemic causes 2. Idiopathic orbital inflammationorbital pseudotumor this is a challenging disease to diagnose with certainty, since it is a diagnosis of exclusion. Sclerosing orbital pseudotumor can rarely extend into the sinuses, brain, and other orbit. Pseudotumor of the orbit and limited wegeners granulomatosis. Inflammatory orbital pseudotumor can affect any or all structures within the orbit. Inflammatory orbital disease msd manual professional edition.
Iois is a diagnosis of exclusion, made only after other entities have been ruled out. 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. Orbital pseudotumor is typically characterized by the rapid development of pain, proptosis, and swelling around the eye and orbit in adults. First described by birchhirschfield in 1905, idiopathic orbital inflammatory syndrome, also known as orbital pseudotumor, is a nonspecific, nonneoplastic inflammatory process of the orbit. Orbital inflammatory syndrome orbital pseudotumor ento key. There is no sex predilection, with peak incidence between the fourth and sixth decades. A patient with bilateral orbital pseudotumor and bilateral cavitary pulmonary lesions is reported. Myositic type of idiopathic orbital pseudotumor in a 4. Intracranial extension of orbital inflammatory pseudotumor. It is most common in the 4th5th decade of life, unilateral or bilateral, and has no gender predilection.
In the days before ct and mri scans, doctors who noted swelling of the op tic disc the. Pdf idiopathic orbital inflammation with extraorbital. Orbital inflammatory pseudotumor orbital pseudotumor is the third most common primary tumor of the orbit and one of the most common causes of unilateral exophthalmos. Perhaps the most confusing differential diagnosis is that of pseudotumor and the condition reported by dunnington and berke,5 which they called chronic myositis of the orbit of unknown origin. Mr imaging of orbital inflammatory syndrome, orbital. Orbital pseudotumor symptoms, diagnosis, treatment. Six showed palpebral edema, in four we felt a tumor in the lacrimal gland in one of them it was bilateral, in four we observed hyperemic bulbar, three showed proptosis one was bilateral, three had. Five months later, she developed worsening headaches and recurrence of the temporal lobe lesion. Orbital pseudotumor is the swelling of tissue behind the eye in an area called the orbit. Invasive orbital pseudotumor ct demonstration of extension. Inflammatory orbital pseudotumor or nonspecific orbital inflammation is a benign, nonspecific, idiopathic inflammatory process characterized by a polymorphous lymphoid infiltrate with varying degrees of fibrosis. 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 does not spread to other tissues or places in the body. Involvement of tendinous insertions differentiates it.
T1 inflammatory orbital pseudotumor with extension beyond the orbit. They grow more slowly, cause less pain, and are characterized by scarring hardening of the tumor tissue. Idiopathic orbital inflammation radiology reference article. Orbital inflammatory syndrome ois, commonly known as inflammatory orbital pseudotumor, is the most common cause of nonthyroidrelated noninfectious orbital disease.
The orbit is the hollow space in the skull where the eye sits. N2 purpose to summarize the clinical profile and response to treatment of 4 biopsyproven cases of inflammatory orbital pseudotumor extending beyond the orbit. Nonspecific inflammation confined to the superior eyelid and superior extraocular muscles consistent with orbital pseudotumor. Inflammatory orbital pseudotumor with extension beyond the. The patient states a history of trauma to the given area 1. Foci of inflammation include the extraocular muscles, the lacrimal gland, the episcleral tissue, the orbital fat, the orbital apex. The relevance in reporting igg4rd cases, especially in unusual scenarios, such as the orbital pseudotumor, remains an important contribution to the many possible disease presentations, as this is still an underdiagnosed condition in which the treatment may lead to remission and prevent significant morbidity and mortality. A 57yearold white female, with a 3month history of headache and right palpebral edema, presented with marked right. A 57yearold white female, with a 3month history of headache and right palpebral edema, presented with marked. Intracranial extension of an idiopathic orbital inflammatory. Pseudotumor cerebri your doctor thinks you may have pseudotumor cerebri. Orbital pseudotumor, also known as idiopathic orbital inflammation ioi, is the third most common disease of the orbit and is characterized by nonspecific inflammation of the orbit with no identifiable cause. Bilyk basics description idiopathic orbital inflammatory syndrome iois is a nonspecific inflammation of orbital tissue with no identifiable local or systemic cause. Wegeners granulomatosis presenting as orbital pseudotumor.
The differential diagnosis of a specific pseudotumor and other orbital masses is both perplexing and interesting. The name means false brain tumor because its symptoms are similar. Orbital pseudotumor as the presenting symptom of crohns. 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. But, by local growth it can cause bulging of the eye proptosis, double vision diplopia and loss of vision. Due to unknown reasons, these tumor behave differently than other types of pseudotumor of the orbit. We report on the clinical features and the mr imaging findings of five cases of these unusual orbital inflammatory pseudotumors with extraorbital extension. Although inflammation is typically confined to the orbit, extraorbital involvement can occur.
Iois can involve any orbital tissue individually or in combination. Sclerosing orbital pseudotumor new york eye cancer center. It is the third most common orbital disease after thyroid orbitopathy and lymphoproliferative disorder. Orbital pseudotumor nonspecific orbital inflammatory disease introduction. Iois can involve virtually any orbital tissue individually, or in combination. 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 pseudotumor an idiopathic orbital inflammation, a diagnosis of exclusion, is the third most common orbital disease, accounting for 56% of orbital disorders. There may be a marginated enhancing soft tissue mass that involves any area of. The orbit protects the eyeball and the muscles and tissue that surround it. This is a pdf file of an unedited manuscript that has been accepted for publication. The inflammatory response can be nonspecific, granulomatous, or vasculitic or due to reactive lymphoid hyperplasia. Idiopathic orbital pseudotumor is extremely rare in pediatric age group and may cause real diagnostic problems. Wegeners granulomatosis presenting as orbital pseudotumor in.
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