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Roman Shinder, Qasiem Nasser, Justin Gutman, Shelly Brejt, Michelle Williams, Bita Esmaeli; Inflammatory Orbital Pseudotumor with involvement of Contiguous Periorbital Structures. Invest. Ophthalmol. Vis. Sci. 2011;52(14):1065.
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Inflammatory orbital pseudotumor (IOP) involving contiguous structures is an uncommon presentation with only a few prior reports. We detail the presentation, radiographic & histologic findings, & treatment outcomes in 3 patients with this condition.
3 consecutive patients who were evaluated for IOP with involvement of contiguous structures at The University of Texas M. D. Anderson Cancer Center between November 2003 and October 2010 were reviewed.
2 females & 1 male had a median age of 18 years (range 5-35). Presenting signs & symptoms included pain, periorbital edema, proptosis, globe dystopia, restricted ocular movement with diplopia, & decreased vision. 2 patients had concurrent disease in the orbit & maxillary sinus (Fig 1), while 1 patient had extension from the lateral orbit into the temporal fossa (Fig 2). In 2 patients there was bony orbital wall erosion, but in the third patient the orbital walls were intact. The diagnosis of IOP in all patients was suspected based on exam & CT findings and confirmed via orbital biopsy. In 1 patient (Fig 1) a biopsy of the maxillary sinus was followed by a second biopsy of the orbit to rule out malignancy, given the atypical large mass and significant bony erosion. 2 of the cases were confirmed as sclerosing pseudotumor based on histology. Treatment consisted of high dose oral steroids with a slow taper in all patients; in 1 patient (Fig 1) methotrexate was added to allow successful steroid taper. At last follow up (median 14 months, range 5-26), all patients had improvement of clinical & radiographic findings without residual orbital pain.
IOP is typically confined to the orbit, but in some cases there may be involvement of the contiguous structures such as paranasal sinuses and/or temporal fossa with or without bony erosion. Bony erosion of the orbital walls is worrisome for a malignant process or Langerhans cell histiocytosis, and in such cases, the diagnosis of IOP requires confirmatory biopsy.
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