Sclerosing orbital pseudotumor

We report two patients who underwent orbital exploration yielding the diagnosis of sclerosing orbital pseudotumor. The presenting symptoms were exophthalmos, visual loss, abnormal ocular mobility, and ocular pain. Computed tomographic (CT) scans showed masses in the orbital apex. Steroids were ineffective. Orbital pseudotumor is a heterogeneous diagnostic category of lymphoid infiltrations of the orbit with a wide spectrum of pathological conditions and intraorbital locations. The clinical presentation typically includes the sudden onset of pain, diplopia, lid edema, and exophthalmos. Visual loss is uncommon. Most cases resolve spontaneously or respond to steroid treatment. Although fibrosis may be a prominent histological finding, the literature contains little information concerning its significance. We discuss the evidence for considering the sclerosing pseudotumors to be a significant variant with unique clinical behavior. Although features suggestive of pseudotumor were present in our case, the presence of visual loss and an apical mass shown on the CT scan led to the presumptive diagnosis of tumor and exploratory operation. Neurosurgeons should be aware of this entity as a cause of visual loss and orbital mass. Proper suspicion may in some cases permit transorbital biopsy and avoid craniotomy, inasmuch as operation is of no therapeutic benefit in this disease.