Sclerosing Orbital Pseudotumor

Idiopathic sclerosing orbital inflammation is a rare finding that is poorly delineated, immune mediated, and causes severe symptoms and disability. It has been described affecting the orbit in addition to other sites within the head and neck, but has rarely been described presenting as sinusitis. A case report and literature review were performed. A 14-year-old girl with right-sided face and eye pain and pressure for >1 month presented 3 days after endoscopic sinus surgery for presumed acute sinusitis. She subsequently developed ipsilateral vision loss and hypesthesia of the infraorbital nerve. MRI revealed a mildly enhancing soft tissue intensity lesion extending from the right maxillary sinus into the pterygopalatine fossa and orbital apex through the inferior orbital fissure. Biopsy specimens of the lesion were consistent with a sclerosing inflammatory lesion. High-dose steroids led to rapid improvement in vision and pain; however, the patient was unable to tolerate steroid weaning because of recurrence of eye pain and headache. Repeat imaging showed progression of the lesion. Rheumatology was consulted and the patient's steroid therapy was altered and her medications were expanded to include azathioprine. The patient's symptoms improved and subsequent imaging showed a reduction in the size and extent of the lesion. Idiopathic sclerosing inflammation is characterized by primary, chronic, and immunologically mediated fibrosis. Patients typically have a poor response to corticosteroid treatment or radiotherapy. Immunosuppressive therapy in addition to corticosteroids is the recommended treatment.

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“…3,10,11 The histological picture shows significant fibrosis associated with a sparse mixed chronic inflammatory cell infiltrate.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic Sclerosing Inflammation Presenting as Sinusitis

Barham

Dishop

Prager

2012

Allergy�Rhinol (Providence)

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“…This definition does not, however, summarize common clinical, therapeutical and anatomical characteristics. Clinical course (insidious, acute, relapsing), response to different therapeutic approaches (corticosteroids, immunosuppressive agents, radiation therapy, surgery) and intraorbital locations (any orbital structure or multifocal) vary from case to case in the literature 2,3,5,6…”

Section: Discussionmentioning

confidence: 99%

Atypical Presentation of Orbital Pseudotumor with Visual Loss as an Initial Manifestation

Türkoğlu

Balak²

2011

J Clin Neurol

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BackgroundAn orbital pseudotumor typically presents with periorbital pain, cranial nerve palsies and proptosis. Although visual deterioration is not unexpected in this pathology, its presentation solely with visual loss is unusual.Case ReportIn this short report, we summarize a case of orbital pseudotumor which presented solely with a decrease in visual acuity, and discuss the clinical and radiological findings.ConclusionsThis atypical presentation likely resulted from the orbital pseudotumor originating in the optic foramen, leaving the neurovascular structures of the superior orbital fissure untouched initially. In the early clinical period, an orbital pseudotumor may manifest itself solely by visual loss. It should therefore be included in the differential diagnosis of visual pathologies-even in the absence of orbital pain and symptoms related to ocular movements.

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“…Pediatric orbital pseudotumor accounts for between 6% to 16% of orbital pseudotumor. 1 The CT and MR imaging ®ndings, correlated with clinical response within days of administration of high dose steroid, facilitates a diagnosis in most cases, even without histopathologic con®rmation. 3 Clinical symptoms are primarily proptosis, diplopia, decreased ocular mobility and pain, and severe visual deterioration may occur in approximately 5% of cases.…”

Section: Discussionmentioning

confidence: 99%

Inflammatory orbital pseudotumor with infratemporal fossa extension mimicking temporomandibular joint dysfunction.

Lee¹,

Kim²,

Hwang³

et al. 2002

Dentomaxillofacial Radiology

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Inflammatory orbital pseudotumor is a benign space-occupying lesion of unknown origin that involves all or part of the fatty tissue within the orbit. Occasionally the disease may extend into the middle cranial, the pterygopalatine, and the infratemporal fossa through the various foramina of the orbit, although extension into the infratemporal fossa is very rare. We present a case which extends into the infratemporal fossa through the inferior orbital fissure, resulting in presenting symptoms mimicking temporomandibular joint dysfunction.

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Sclerosing Orbital Pseudotumor

Cited by 43 publications

References 0 publications

Idiopathic Sclerosing Inflammation Presenting as Sinusitis

Idiopathic Sclerosing Inflammation Presenting as Sinusitis

Atypical Presentation of Orbital Pseudotumor with Visual Loss as an Initial Manifestation

Inflammatory orbital pseudotumor with infratemporal fossa extension mimicking temporomandibular joint dysfunction.

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