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Show ORIGINAL CONTRIBUTION Radiosensitive Orbital Inflammation Associated with Temporal Arteritis Kimberly P. Cockerham, MD, Glenn C. Cockerham, MD, Henry G Brown, MD, and Ahmed A. Hidayat, MD Abstract: A 75- year- old woman developed acute loss of vision in the OD, ipsilateral periocular pain, an afferent pupillary defect, sectoral optic disc edema, and later ipsilateral proptosis and an intraconal mass. She denied any symptoms of temporal arteritis, and a sedimentation rate was normal. Orbital biopsy demonstrated chronic granulomatous inflammation with perivasculitis. A temporal artery biopsy disclosed findings consistent with temporal arteritis. Following 2000 cGy of external beam radiation, her visual function and orbitopathy completely resolved. This unusual presentation of orbital inflammation in association with temporal arteritis demonstrates that pathologic findings of temporal arteritis may be clinically nonspecific and that external beam radiation may be an effective therapy in this setting. ( JNeuro- Ophthalmol 2003; 23: 117- 121) Orbital inflammation may be isolated ( nonspecific orbital inflammation), or associated with systemic inflammation such as Wegener granulomatosis, polyarteritis nodosa, sarcoidosis, or, rarely, temporal arteritis ( 1- 22). Nonspecific orbital inflammation is classically acute in onset but may be recurrent. On pathologic inspection, a mixed inflammatory infiltrate without epithelioid cells is typically present. Orbital inflammation associated with systemic disease is commonly subacute and bilateral. Diagnosis is often difficult, as laboratory evaluation findings may be negative for a specific disorder and systemic involvement can occur decades later. Department of Ophthalmology ( KPC, GCC) and Department of Pathology ( GCC, HGB), Allegheny General Hospital, Pittsburgh, Pennsylvania; and Department of Ophthalmic Pathology ( AAH), Armed Forces Institute of Pathology, Washington, DC. Address correspondence to Kimberly P. Cockerham, MD, Allegheny Ophthalmic and Orbital Associates, 420 East North Avenue, Suite 116, Pittsburgh, PA 15212, USA; E- mail: kpcorb@ aol. com Presented at the Vancouver Orbital Symposium on Graves' Orbitopathy and Orbital Disease, Vancouver, British Columbia, Canada, March 2002. Temporal arteritis classically appears in patients of at least 65 years of age with new- onset headache, jaw claudication, anterior ischemic optic neuropathy, myalgias, fatigue, and weight loss. Temporal artery tenderness and elevated sedimentation rate are characteristic. Neuro-ophthalmic manifestations also include posterior ischemic optic neuropathy, central retinal or cilioretinal artery occlusion, and cranial neuropathies. Orbital manifestations include orbital ischemic syndrome and, very rarely, orbital inflammation ( 1- 9). The orbital inflammation is often cor-ticosteroid- responsive, but visual loss may occur despite immunosuppressive therapy ( 5). Radiation therapy has not been previously considered. CASE REPORT A 75- year- old woman noticed acute visual loss and periocular pain in the OD. She denied weight loss, anorexia, muscle aches, jaw claudication, or temporal tenderness. She had had elevated cholesterol controlled with gemfibrozil; there was no history of hypertension or diabetes and she was a nonsmoker. Visual acuity was 20/ 70 OD and 20/ 20 OS. A right afferent pupillary defect, sectoral optic disc edema, and an inferotemporal visual field defect were noted ( Fig. 1). Westergren erythrocyte sedimentation rate was 27 mm/ h. A diagnosis of nonarteritic ischemic optic neuropathy was made, and the patient was placed on daily aspirin. The patient returned 2 months later with improvement in her visual acuity to 20/ 25 OD and resolution of her periocular pain. One month later, she reported worsening vision and periocular pain. She still denied other symptoms of temporal arteritis. Visual acuity had fallen to finger counting OD and 20/ 20 OS. She had an afferent pupillary defect OD. The optic disc edema had improved but the right visual field had worsened ( Fig. 2). A repeat erythrocyte sedimentation rate was 29 ( C- reactive protein < 0.1). The patient was treated with prednisone 80 mg/ d. A temporal artery biopsy, performed 10 days later, was reported to be negative for temporal arteritis, so her prednisone was tapered over 2 weeks. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. J Neuro- Ophthalmol, Vol. 23, No. 2, 2003 117 JNeuro- Ophthalmol, Vol. 23, No. 2, 2003 Cockerham et al. FIG. 1. Humphrey 30- 2 visual field at presentation demonstrates defects with a nerve fiber bundle configuration and temporal hemianopic features, consistent with apical optic nerve involvement ( mean deviation - 8.45 dB). When the patient returned 1 month later, having been completely weaned off corticosteroid medication for 2 weeks, visual acuity had improved to 20/ 60 OD and remained 20/ 20 OS. But she now demonstrated 3 mm of right axial proptosis and inferior scleral show ( Fig. 3). Her right periocular ache had returned following the corticosteroid FIG. 2. Humphrey 30- 2 visual field 3 months later shows deterioration ( mean deviation - 16.53 dB). FIG. 3. Four months after presentation, right proptosis with inferior scleral show has developed. FIG. 4. Axial computed tomography scan shows a homogeneous apical orbital mass that extends through the superior orbital fissure. FIG. 5. Orbital biopsy shows chronic inflammatory cells surrounding an artery { arrow) within the orbital tissue. Most of the inflammatory cells are mature lymphocytes ( hema-toxylin- eosin, magnification X320.) Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. 118 © 2003 Lippincott Williams & Wilkins RADIOSENSITIVE ORBITAL INFLAMMATION JNeuro- Ophthalmol, Vol. 23, No. 2, 2003 ".*.,,.. ' - if V ^ '• • ' ' • ' ' * ' • " • . > - ^ ^ FIG. 6. Temporal artery biopsy. A: Granulomatous inflammation is present within the adventitia and outer media. Numerous epithelioid cells are seen ( arrow); no multinucleated giant cells were present. The intima was not inflamed ( hema-toxylin- eosin, magnification X 320). B: Higher power view demonstrates fibrous scar in the area of disruption of the internal elastic lamina. Minimal inflammation is present ( Masson trichrome stain). C: Special staining for elastin demonstrates disruption of the internal elastic lamina associated with scarring of the inner position of the vessel wall ( Verhoeff- van Giessen stain). taper. She denied any symptoms compatible with Wegener granulomatosis, polyarteritis nodosa, or sarcoidosis. Orbital computed tomography demonstrated an apical homogeneous mass extending through the superior ophthalmic fissure that was well circumscribed and distinct from the optic nerve and extraocular muscles ( Fig. 4). A fine needle biopsy was nondiagnostic. A lateral orbitotomy with incisional biopsy revealed an infiltrative, friable, and well- vascularized mass. Histopathologic review disclosed chronic inflammation consisting of lymphocytes, plasma cells, and occasional epithelioid cells ( Fig. 5). Minimal atypia and no mitotic figures were seen. Immunohisto-chemical stains were negative for cytokeratins, S- 100, and HMB- 45. Lymphoid markers demonstrated a mixture of B-cells and T- cells without light chain restriction. Review of the original temporal artery biopsy specimen demonstrated findings consistent with vasculitis, including a focus of chronic inflammation with rare epithelioid cells in the adventitia and media ( Fig. 6A), as well as thickening and myxoid change of the intima ( Fig. 6B) and FIG. 7. Four weeks after radiation treatment, proptosis and inferior scleral show are no longer evident. discontinuity of the internal elastic lamina ( Fig. 6C). There were no giant cells. Tests for C- Anca, p- Anca, and angio-tensin- converting enzyme were negative. Complete systemic evaluation by a rheumatologist, including appropriate laboratory testing and imaging, failed to demonstrate any systemic clinical abnormalities. The patient was intolerant to the side effects of continued oral corticosteroid treatment, so external beam radiation was performed ( 2000 cGy over 2 weeks). One month after irradiation, orbitopathic signs had disappeared ( Fig. 7) and visual function had returned to normal ( Fig. 8). DISCUSSION We have described a case of granulomatous orbital inflammation resulting in compressive optic neuropathy. FIG. 8. Four months after radiation treatment, the visual field defects have resolved. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. 119 JNeuro- Ophthalmol, Vol. 23, No. 2, 2003 Cockerham et al. TABLE 1. American College of Rheumatology 1990 criteria for the diagnosis of temporal arteritis* Age at disease onset > 50 years New onset or type of localized head pain Temporal artery tenderness or decreased pulsation ESR > 50 mm/ hour ( Westergren) Temporal artery vasculitis characterized by any of the following: Mononuclear cell infiltration Granulomatous inflammation Multinucleated giant cells * At least three criteria must be present for diagnosis. ESR, erythrocyte sedimentation rate. are rare. Reported sites of orbital involvement include the extraocular muscles, optic nerve sheath, and intraconal fat ( Table 2). Most patients, especially those with isolated extraocular muscle involvement, have demonstrated resolution of their orbital symptoms and signs with oral corticosteroid treatment. In the one case with an intraconal lesion similar to our case, permanent visual loss occurred despite corticosteroid and cyclophosphamide therapy ( 1- 10). This case highlights the lack of clinical specificity of the pathologic findings of temporal arteritis and the potential utility of temporal artery biopsy in the face of an inaccessible orbital mass. Moreover, we believe that it is the first report of granulomatous orbital inflammation associated with temporal arteritis in which radiation therapy produced full recovery. The patient also demonstrated pathologic evidence of temporal arteritis but had only some of the typical clinical manifestations of that condition ( Table 1). Several vasculitides have been implicated in orbital inflammation. Wegener granulomatosis and polyarteritis nodosa are candidates, as both entities have also been noted to cause a granulomatous vasculitis of the temporal artery with and without giant cells. Fibrinoid necrosis, if present, is characteristic of these entities, but the pathologic features affecting the temporal artery can be indistinguishable from those of temporal arteritis ( 1- 5). Could our patient have had Wegener granulomatosis? We consider it unlikely because the c- ANCA was normal and there was a lack of sinus or pulmonary findings ( 14,15). However, cANCA- negative orbital inflammation is a diagnostic possibility. Polyarteritis nodosa would seem an unlikely diagnosis in the absence of p- ANCA positivity and without renal, neurologic, or skeletal muscle involvement ( 16- 22). If our patient had temporal arteritis, she had a very atypical variant. Visual recovery and orbital involvement 10. 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