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Show Journal of Neuro- Ophthalmology 20( 4): 268- 272, 2000. © 2000 Lippincott Williams & Wilkins, Inc., Philadelphia Bilateral Orbital Pseudotumor With Suprasellar and Pulmonary Involvement: Report of a Case • Yu- Hung Lai, MD, Hwei- Zu Wang, MD, Rong- Kung Tsai, MD, William F. Hoyt, MD, and Bi- Fang Lee, MD A 39- year- old man had bilateral proptosis and blurred vision for 1 week. Computed tomography and magnetic resonance imaging showed signs of bilateral orbital pseudotumor, a suprasellar mass, and pulmonary infiltration. Biopsies from retrobulbar and bronchial sites showed similar inflammatory tissue. His disease resolved with pulsed corticosteroid therapy. Key Words: Orbital pseudotumor- Systemic inflammatory pseudotumor. The term orbital pseudotumor has been used to describe idiopathic orbital inflammation simulating orbital neoplasm ( 1- 5). The condition is characterized by acute onset of exophthalmos, lid and conjunctival edema, decreased eye movement, and pain. It is more commonly unilaterally than bilaterally ( 1- 6). Bilateral orbital pseudotumor has an increased incidence of systemic disease ( 2). We report a rare case of bilateral orbital pseudotumor with cerebral and pulmonary involvement. CASE REPORT A 39- year- old man visited our outpatient department of ophthalmology with a 1 - week history of progressive blurring of vision, pain, and proptosis OU. He had had flu- like symptoms several weeks earlier. His general health had been good except for ' sinusitis' operated on at a local hospital 2 months earlier. The family history was unremarkable. His best corrected visual acuity was 20/ 200 OD and 20/ 25 OS. He had a 10- prism- diopter ( 10A) right esotropia. He had limited abduction, adduction, and depression OD and limited abduction OS ( Fig. 1A). Hertel exoph-thalmometer readings were 21 mm OD and 20 mm OS. Manuscript received November 16,1999; accepted June 27, 2000. From the Department of Ophthalmology, Kaohsiung Medical University, Kaohsiung, Taiwan ( YHL, HZW, RKT); the Department of Ophthalmology, University of California, San Francisco, San Francisco, California ( WFH); and the Department of Nuclear Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan ( BFL). Address correspondence and reprint request to Rong- Kung Tsai, MD, Department of Ophthalmology, Kaohsiung Medical University, 100 Shih- Chuan 1st Road, Kaohsiung, 807, Taiwan. An afferent pupillary defect was present OD. The conjunctivae were chemotic. Results of other ophthalmologic examinations such as intraocular pressure and fundus examination were normal. Blood examinations were normal except for an elevated percentage of eosinophils ( 13%). Erythrocyte sedimentation rate was elevated ( 63 mm). Thyroglobulin antibody ( 78.2 IU/ ml; normal range, < 25 IU/ ml) and microsomal antibody ( 90 IU/ ml; normal range, < 25 IU7 ml) were mildly elevated. Results of thyroid function tests indicated hypothyroidism ( thyroid- stimulating hormone [ TSH], 2.4 jjiU/ ml; normal range, 0.94- 2.84 u, U/ ml; T3, 80.5 ng/ dl; normal range, 98.4- 144.2 ng/ dl; T3U, 25.9%; normal range, 29.37- 37.65%; T4, 2.9 | xg/ dl; normal range, 6.5- 9.5 / jug/ dl; and free T4 index [ FTI], 0.75; normal range, 2.19- 3.13). Elevated EB- VCA ( Epstein- Barr- viral capsid antigen) immunoglobulin G ( IgG) ( 1: 160; significant if > 1: 40) and positive HLA- DR were detected. Results of tests for cytoplasmic pattern of anti-neutrophil cytoplasmic antibody ( c- ANCA), antinuclear antibodies ( ANA), rheumatoid factor, Venereal Disease Research Laboratory ( VDRL) test, EB- VCA IgM, blood sugar, blood urea nitrogen ( BUN), creatinine, electrolytes, and routine analysis of urine were normal. The angiotensin- converting enzyme level was not measured because of unavailability of testing facilities. Visual- evoked responses ( VER) showed poor responses OU ( worse OD) with increased latency and decreased amplitude to patterned reversal stimuli. Humphrey automated perimetry indicated visual field defect OU ( worse OD). Roentgenograms of the skull were normal. A chest radiograph showed a mass around the carina and increased interstitial pattern in bilateral lower lung fields. On computed tomographic ( CT) scans of the orbit, there were poorly demarcated soft tissue masses in the lateral aspect of the right orbit along the lateral rectus muscle, in the apex of the right orbit, and in the superolateral aspect of the left orbit ( Fig. 2A). Right ethmoid sinusitis and bilateral maxillary opacities also were noted. In addition, an irregular, well- enhanced soft tissue mass was found in the suprasellar region, near the right parahippocampal gyrus and the cavernous sinuses bilaterally ( Fig. 3A). Magnetic resonance imaging ( MRI) 268 BILATERAL ORBITAL PSEUDOTUMOR WITH SUPRASELLAR AND PULMONARY INVOLVEMENT 269 FIG. 1. A: Bilateral proptosis and limited abduction ( OD) were noted before the corticosteriod treatment. B: Only residual limitation on abduction of his right eye was noted after the treatment. showed similar findings ( Fig. 3B). CT scan of the chest showed interstitial thickening in both lungs, a mildly enhanced soft tissue mass in the middle and lower third of the esophagus, and enlarged lymph nodes at pretracheal and prevascular areas ( Fig. 4). Bronchoscopy showed, in addition to a bulging mass around the carina, multiple segments of the bronchi with hyperemia and concentric narrowing. Biopsy of lesions in the trachea and bronchi showed chronic nonspecific round- cell inflammation. No malignant cells were found in the specimens ( Fig. 5A, B). In pathologic examinations of all the specimens from the excisional biopsy of the retrobulbar tissue ( 1 x 0.5 x 0.2 cm3), there were no noncaseating granulomatous nodules or giant cells, which are characteristic of sarcoidosis. The specimens only showed chronic inflammation compatible with a diagnosis of orbital pseudotumor. No malignant cells or signs of vasculitis could be found ( Fig. 5C, D). Results of a nasopharyngeal examination were normal. Galium- 67 scan indicated an increased activity in lacrimal glands, paranasal sinuses, and lung fields bilaterally. The patient received 500 mg methylprednisolone intravenously twice per day for 3 days, and then he received 60 mg prednisolone orally once per day on successive days. Three weeks after onset of his disease, visual acuity was 20/ 20 OU, he had 15- prism diopter ( 15A) of esotropia. There was no residual limitation of eye movement except abduction OD ( Fig. IB), and there was no afferent pupillary defect. Hertel exophthalmometer readings were ( OD 13 mm; OS 14.5 mm), and Humphrey automated perimetry was normal OU. Prednisolone was gradually tapered over the next 5 months. CT scans showed residual enhancing soft tissue masses in the superolateral aspect of each orbit ( Fig. 2B). No mass could be found in the chest or intracranially. He has been maintained on low doses of oral prednisolone and azathioprine. FIG. 2. CT scan of the orbit. A: Before the Corticosteroid treatment, axial views showed inflammatory soft tissue masses in the lateral aspect of the right orbit along the lateral rectus muscle and its tendon, in the apex of the right orbit, and in the superolateral aspect of the left orbit. B: Residual well- enhanced soft tissue masses were noted in the superolateral aspect of the bilateral orbits after the treatment. J Neuro- Ophthalmol, Vol. 20, No. 4, 2000 270 Y.- H. LAIETAL. FIG. 3. A: An irregular, well- enhanced soft tissue mass was found in the suprasellar region, around the right parahippocampal gyrus and bilateral caverneous sinuses before the corticosteroid treatment. B: Same well- enhanced soft tissue masses in T1- weighted MR imaging. DISCUSSION Orbital inflammation may result from Graves orbitopa-thy, sarcoidosis, vasculitis, lymphoma, Kimura disease, or orbital pseudotumor ( 1,2,7). Although our patient had hypothyroidism, the clinical features ( acute onset; ocular pain; elevated erythrocyte sedimentation rate [ ESR]; and infiltrative mass with extraocular muscle enlargement, including tendonous insertion) were not typical of Graves orbitopa-thy. In addition, his dramatic response to corticosteroid therapy also made Graves orbitopathy unlikely. The hypothyroidism may have been related to the inflammatory involvement of his pituitary gland or stalk. Although angiotensin- converting enzyme test is not available in our hospital, there was no sign ( clinical or laboratory) of sarcoidosis involving lung, skin, eyes, nervous system, heart, kidneys, or the musculoskeletal system. Serum angiotensin- converting enzyme ( ACE) levels were increased in approximately 63.5% of the biopsy-proven sarcoid ( 8). Pathologically, there were no characteristic findings of sarcoidosis in a biopsy specimen from the orbit ( as large as 1 x 0.5 x 0.2 cm3 in size), which makes a diagnosis of sarcoidosis unlikely. A diagnosis of vasculitis such as Wegener granulomatosis is not likely, for the following reasons: First, no signs of Wegener granulomatosis were found in the respiratory tract, kidneys, musculoskeletal system, eye, and skin. Second, there was no pathologic evidence of necrotizing granulomatous vasculitis in the tissue samples from lungs or orbit. Moreover, there was no support for this diagnosis from laboratory studies: the negative c- ANCA, negative rheumatoid factor, normal urinalysis, and normal blood studies. Kimura disease or angiolymphoid hyperplasia with eosinophilia preferentially affect skin of the head and neck ( 7). In lids and orbits, the lesions are usually single. Multiple subcutaneous nodules, sometimes associated with eosinophilia ( of peripheral blood), have been reported, particularly in Asians. Although there was eosinophilia of the peripheral blood of our patient, no other signs supported a diagnosis of Kimura disease. Because of the dramatic response to the steroid therapy, the lack of malignant cells in biopsy tissue, and the lack of other evidence of lymphoma, a diagnosis of lymphoma was improbable. Biopsy of orbital lesions and lung lesions indicated a similar process of chronic inflammation compatible with the diagnosis of inflammatory pseudotumor. FIG. 4. CT scan of the chest. A: A mild- enhance soft tissue mass in the middle to lower third portion of the esophagus ( arrow); B: enlarged lymph nodes at pretracheal and prevascular areas were found ( arrow). J Neuro- Ophlhalmol, Vol. 20, No. 4, 2000 BILATERAL ORBITAL PSEUDOTUMOR WITH SUPRASELLAR AND PULMONARY INVOLVEMENT 271 FIG. 5. A: Biopsy specimens of the lesion in the trachea and bronchi showed chronic nonspecific inflammation ( original magnification x100, HE). B: Eosinophils ( arrowhead), lymphocytes, PMNs, and mast cells were noted in high- power images of the pulmonary specimens ( original magnification x400, HE). C: Biopsy specimens of retrobulbar tissue showed nonspecific chronic inflammation compatible with a diagnosis of orbital pseudotumor ( original magnificaiton x100, HE). D: Lymphocytes, PMNs, plasma cells ( arrowhead), and eosinophils ( arrow) were noted in high- power images of the orbital specimens ( original magnification x400, HE). Orbital pseudotumors are usually unilateral ( 1- 6). Bilateral orbital pseudotumor in adults usually suggests a systemic disorder, as was true in our patient. The pathologic findings in orbital pseudotumors vary, without obvious differences between unilateral and bilateral cases ( 5). Orbital pseudotumor with systemic involvement has been reported as a manifestation of multifocal fibroscle-rosis. This is a disease of unknown cause, characterized by fibrous lesions occurring at a variety of sites such as mediastinum, retroperitoneal loci, thyroid, bile ducts, and orbits ( 3,9). The histologic diagnosis of multifocal fibrosclerosis depends on finding extensive deposition of hyalinized fibrous tissue, commonly arranged in more or less concentric whorls around extinct or attenuated blood vessels. A diagnosis of multifocal fibrosclerosis is not likely in our patient. There have been cases with solitary nonorbital inflammatory pseudotumors ( 3) involving lungs ( 10,11), brain ( 12), gallbladder ( 13), and esophagus ( 14). Histologically, nonorbital pseudotumors are composed of fibrous tissue with an inflammatory cell infiltrate. Unlike in their orbital counterparts, the dominant cell population consists of spindle or plasma cells ( 3,10- 14). To our knowledge, these pseudotumors do not occur in association with orbital pseudotumors. Only a few cases of orbital pseudotumor have been reported with intracranial extension ( 15,16) or nasal sinus involvement ( 17). The case presented here had bilateral orbital pseudotumor with pulmonary and intracranial involvement. After systemic corticosteroid therapy, the lesions in lungs and brain disappeared, along with the shrinkage of the orbital pseudotumor. This therapeutic response provides evidence that the inflammatory processes in the orbit, lungs, and brain were identical. REFERENCES 1. Kennerdell JS, Dresner SC. The nonspecific orbital inflammatory syndromes. Surv Ophthalmol 1984; 29: 93- 103. 2. Snebold NG. Noninfectious orbital inflammations and vasculitis. In: Albert DM and Jakobiec FA, eds. Principles and practice of ophthalmology: clinical practice. Philadelphia: WB Saunders, 1994: 1923- 39. 3. Mombearts I, Goldschmeding R, Schlingemann RO, et al. What is orbital pseudotumor? Surv Ophthalmol 1996; 41: 66- 78. 4. Chavis RM, Garner A, Wright JE. Inflammatory orbital pseudotumor: a clinicopathologic study. Arch Ophthalmol 1978; 96: 1817- 22. 5. Blodi FC, Gass JDM. Inflammatory pseudotumour of the orbit. Br J Ophthalmol 1968; 52: 79- 93. 6. Nicole S, Palma L, Giuffre R. Orbital pseudotumor: pathologic and therapeutic considerations. J Neurosurg Sci 1982; 26: 57- 60. J Neuro- Ophthalmol, Vol. 20, No. 4, 2000 272 Y.- H. LAI ET AL. 1. Jakibiec FA, Font RL. Orbit: Noninfectious orbital inflammations. In: Spencer WH, ed. Ophthalmic Pathology: an Atlas and Textbook. Philadelphia: WB Saunders, 1986: 2803- 7. 8. Shorr AF, Torrington KG, Parker JM. Serum angiotensin converting enzyme does not correlate with radiographic stage at initial diagnosis of sarcoidosis. Respir Med 1997; 91: 399- 401. 9. Comings DE, Skubi KB, Eyes JV, et al. Familial multifocal fibro-sclerosis. Findings suggesting that reproperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Riedel's thyroiditis, and pseudotumor of the orbit may be different manifestations of a single disease. Ann Intern Med 1967; 66: 884- 92. 10. Berardi RS, Lee SS, Chen HP, et al. Inflammatory pseudotumors of the lung. Surg Gynecol Obstet 1958; 46: 207- 18. 11. Matsubara O, Tan- Liu NS, Kenney RM, et al. Inflammatory pseudotumor of the lung: Progression from organizing pneumonia to fibrous histiocytoma or to plasma cell granuloma in 32 cases. Hum Pathol 1988; 19: 807- 14. 12. Le Marc'hadour F, Fransen P, Labat- Moleur F, et al. Intracranial plasma cell granuloma: a report of four cases. Surg Neurol 1994; 42: 481- 8. 13. Ikeda H, Oka T, Imafuku I, et al. A case of inflammatory pseudotumor of the gallbladder and bile duct. Am J Gastroenterol 1990; 85: 203- 6. 14. Pettinato G, Manivel JC, De Rosa N, et al. Inflammatory myofi-broblastic tumor ( plasma cell granuloma): clinicopathologic study of 20 cases with imrnunohistochernical and ultrastructural observation. Am J Clin Pathol 1990; 94: 538- 46. 15. Frohman LP, Kupersmith MJ, Lang J, et al. Intracranial extension and bone destruction in orbital pseudotumor. Arch Ophthalmol 1986; 190: 230- 42. 16. Noble SC, Chandler WE, Lloyd RV. Intracranial extension of orbital pseudotumor: A case report. Neurosurg 1986; 18: 798- 801. 17. Eshaghian J, Anderson RL. Sinus involvement in inflammatory orbital pseudotumor. Arch Ophthalmol 1981; 99: 627- 30. / Neuro- Ophthalmol, Vol. 20, No. 4, 2000 |
