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Show Journal of Neuro- Ophthalmology 20( 3): J56- 158, 2000. © 2000 Lippincott Williams & Wilkins, Inc., Philadelphia Orbital Apex Lesion as the Presenting Manifestation of Sarcoidosis Ernesto I. Segal, MD, Rosa A. Tang, MD, Andrew G. Lee, MD, Dwayne L. Roberts, MD, and Gerald A. Campbell, MD A 32- year- old black woman presented with progressive pro-ptosis, diplopia, and optic disc edema of the right eye. A computed tomography scan of the orbit showed a right retroorbital mass. A gallium scan showed increased radiotracer activity in the right retroorbital region. Biopsy of the mass showed non-caseating granulomas that were compatible with sarcoidosis. The patient was treated with systemic steroids, and the propto-sis and diplopia resolved. Seven months later, the patient presented with contralateral optic neuropathy. Neuroimaging showed enlargement of the left optic nerve. The patient again responded to systemic steroid treatment and experienced complete restoration of vision. An orbital apex lesion may be the presenting manifestation of sarcoidosis. Key Words: Optic disc edema- Optic neuropathy- Orbital tumors- Sarcoidosis. Although a significant percentage of patients with sarcoidosis have ocular involvement ( 1), only 5% have neurological involvement ( including the optic nerve in 1- 5% ( 2- 4). The facial nerve is the most commonly involved cranial nerve, but the optic nerve is second in frequency ( 4,5). With the exception of lacrimal gland involvement, orbital lesions are uncommon, and an isolated orbital apex lesion without evidence of systemic disease is rarely the presenting sign ( 6). CASE REPORT A 32- year- old black woman presented with a 7- month history of diplopia and proptosis OD. She denied pain, decreased vision, and dry eyes. Her medical history was significant only for iron- deficiency anemia. Her family This work was supported in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York, New York. Manuscript received March 7, 1997; accepted May 8, 2000. From the Department of Ophthalmology and Visual Sciences ( EIS, RAT) and Pathology ( GAC), University of Texas Medical Branch, Galveston, Texas; and from the Departments of Ophthalmology, Neurology, and Neurosurgery ( AGL), The University of Iowa Hospitals and Clinics, Iowa City, Iowa. Address correspondence and reprint requests to R. A. Tang, MD, Department of Ophthalmology and Visual Sciences, University of Texas Medical Branch, Clinical Sciences Building, 301 University Boulevard, Galveston, TX 77555. history was unremarkable, and she was not taking any medication. On physical examination, she had a low-grade systolic murmur, her lungs were clear to auscultation, and no lymphadenopathy was detected. Ophthalmologic examination revealed an uncorrected visual acuity of 20/ 20 OU. Visual fields by confrontation were full, and there was no red desaturation. Unfortunately, no formal perimetry was performed. The pupils had brisk direct and consensual responses, and there was a relative afferent pupillary defect OD. She had a right moderate hypertropia and exotropia in primary position with marked inability to infraduct OD. Adduction, abduction, and supraduction OD were also relatively limited. Motility was normal OS. There was proptosis OD ( Fig. 1). Hertel exophthalmometry readings were 29 mm OD and 24 mm OS. Intraocular pressure measurements were normal OU. Slit- lamp examination showed conjunctival hyperemia and chemosis OD. There was no evidence of uveitis, iris or conjunctival nodules, or dry eye. Ophthalmoscopy OD showed optic disc edema and macular striae ( Fig. 2). The remainder of the fundus examinations were normal OD and OS. A computed tomography scan of the head and orbit showed a 2.5- cm enhancing mass in the inferior and posterior aspect of the right orbit ( Fig. 3) that displaced the globe forward and compressed the optic nerve. No other intracranial abnormalities were seen. Chest radiography was normal. Results of laboratory studies indicated a microcytic hypochromic anemia. Serum electrolytes, kidney function tests, calcium, and lysosomal levels were normal. Magnetic resonance imaging was not performed. The serum angiotensin converting enzyme level was 62 activity units ( a. u.) ( normal value, 14- 64). A gallium citrate scan showed increased radiotracer uptake in the right retroorbital region ( Fig. 4). A right orbitotomy was performed, and results of the biopsy showed noncaseat-ing granulomas ( Fig. 5) that are compatible with sarcoidosis. Results of cultures, stains for acid- fast organism, and tests for fungi were negative. At the time of surgery, the mass was noted to encase and infiltrate the inferior rectus muscle. The patient was treated with oral prednisone 80 mg per day. The steroid treatment was tapered 756 ORBITAL APEX LESION FIG. 1. External photograph shows proptosis, hypertropia, ex-otropia, and conjunctival hyperemia and chemosis OD. over 2 months. There was reduction in the size of the mass and resolution of the proptosis, ophthalmoplegia, and optic disc edema OD. Seven months later, the patient returned with a 1- month history of decreased vision OS and new headaches. Best- corrected visual acuity measured 20/ 20 OD and 20/ 200 OS. There was subjective red desaturation OS. Results of slit- lamp biomicroscopy were normal. A motility examination showed mild inferior restriction OD. The patient denied any pain associated with ocular movements. Ophthalmoscopic examination OS showed optic disc edema ( Fig. 6). The optic nerve OD was normal. Automated perimetry ( Humphrey 30- 2 visual field) revealed a dense central scotoma OS and a normal visual field OD. The erythrocyte sedimentation rate, syphilis serology, and antinuclear antibodies were normal. Serum angiotensin converting enzyme levels were elevated at 96 a. u. A computed tomography scan of the head and orbit ( Fig. 7) revealed enlargement of the left optic nerve and a small residual soft tissue mass in the right orbit. A diagnosis of sarcoid optic neuropathy was made, and the patient was treated with intravenous methyl-prednisolone 250 mg every 6 hours for 3 days, followed by a 2- month oral prednisone taper. There was resolu- FIG. 2. Fundus photographs show optic disc edema and macular striae OD. AND SARCOIDOSIS FIG. 3. Head and orbital computed tomography scans. A: Axial view shows an enhancing mass in the inferior and posterior aspect of the right orbit. B: Coronal view shows a mass involving the right inferior rectus muscle. tion of the optic disc edema and recovery of vision to 20/ 20 OS. DISCUSSION The differential diagnosis of an orbital apex mass includes neoplasms ( e. g., cavernous hemangioma, optic nerve glioma, meningioma, schwanoma, lymphoma, metastatic), inflammatory lesions ( orbital pseudotumor), and infection ( e. g., abscesses and granulomas). Sarcoidosis is a multisystem idiopathic inflammatory disease with a predilection for the chest. Histopathologically, sarcoid is characterized by noncaseating epithelioid cell granuloma. Patients with ocular disease may not have systemic findings ( 7,8), and ocular sarcoidosis may occur in the setting of known but presumed inactive systemic disease. The most common ocular manifestations FIG. 4. Gallium scan of the head and neck shows increased radiotracer activity in the right retro- orbital region ( arrow). The lacrimal glands are identified with normal radiotracer activity ( darker arrows). J Neuro- Ophthalmol, Vol. 20, No. 3, 2000 158 E. I. SEGAL ETAL. V? :<^ .- ; « f' - V - - v - ••>.'•-'':>.' H , ; ' ." ••'-;' •'" v.- oj; -• • *• • JL. * * * * * • / . - 1 . • * :^\ FIG. 5. Histopathology at low ( 12x) ( A) and high ( 1 OOx) ( B) power showing noncaseating granulomas with epithelioid cells, inflammatory giant cells, and lymphocytic infiltrates. FIG. 7. Head and orbit computed tomography scan. A: Axial view showing enlargement of the left optic nerve. B: Coronal view showing the left optic nerve enlargement ( arrow). 9 I of sarcoidosis are uveitis, conjunctival granulomas, and keratoconjunctivitis sicca. Although lacrimal gland infiltration is a relatively common finding in sarcoid, an isolated orbital apex lesion is an uncommon finding. Sarcoidosis can affect the optic nerve in numerous ways ( 2,4,10), including a focal granuloma of the optic nerve head, inflammatory or infiltrative retrobulbar neuropathy or papillitis, optic disc edema secondary to posterior segment inflammation, optic perineuritis, or papilledema ( secondary to increased intracranial pressure from intracranial sarcoid). The presentation of an acute optic neuropathy may mimic demyelinating optic neuritis. Intracranial involvement of the suprasellar region may cause chiasmal symptoms or diabetes insipidus ( 11). In our patient, inflammatory or infiltrative optic neuritis, compressive optic neuropathy, optic perineuritis, or a FIG. 6. Fundus photographs showing optic disc edema OS. combination of these mechanisms may have been responsible for the optic disc edema. The lack of visual acuity or visual field loss ( no formal perimetry was available), with an afferent pupillary defect, and the neuroim-aging findings suggest optic perineuritis. The clinical features and imaging were consistent with an orbital apex lesion that proved to be sarcoidosis. In addition, our patient later developed a contralateral optic neuropathy because of presumed sarcoid optic neuritis. Clinicians should consider sarcoidosis in the differential diagnosis of an orbital apex lesion, even in the absence of systemic or pulmonary radiographic evidence for the disease. REFERENCES 1. Myers M. Ocular sarcoidosis. Int Ophthalmol Clin 1990; 30: 254- 63. 2. Jordan DR, Anderson RL, Nerad JA, et al. Optic nerve involvement as the initial manifestation of sarcoidosis. Can J Ophthalmol 1988; 23: 232- 7. 3. Elgelken JD, Yuh WT, Carter KD, et al. Optic nerve sarcoidosis: MR findings. AJNR 1992; 13: 228- 30. 4. Galleta S, Schatz NJ, Glaser JS. Acute sarcoid optic neuropathy with spontaneous recovery. J Clin Neuroophthalmol 1989; 9: 27- 32. 5. Katz B. Disc edema, transient obscurations of vision, and a temporal fossa mass. Surv Ophthalmol 1991; 36: 133- 9. 6. Jakobiec FA, Jones JS. Orbital inflammation. In: Jasman W, Jaeger EA, eds. Duane's Clinical Ophthalmology. Vol 2. Philadelphia: JB Lippincott, 1993: 39^ 12. 7. O'Connor GR. Ocular sarcoidosis. Trans New Orleans Acad Ophthalmol 1983 ; 211- 22. 8. Simpson GV. Diagnosis and treatment of uveitis in association with sarcoidosis. Trans Am Ophthalmol Soc 1968; 66: 117- 41. 9. Brownstein S, Liszauer AD, Carey WD, et al. Sarcoidosis of the eyelid skin. Can J Ophthalmol 1990; 25: 256- 9. 10. Hart WM, Burde RM. Optic disk edema in sarcoidosis. Am J Ophthalmol 1979; 88: 769- 71. 11. Tang RA, Grotta JC, Lee KF, Lee YE. Chiasmal syndrome in sarcoidosis. Arch Ophthalmol 1983; 101: 1069- 73. J Neuro- Ophthalmol, Vol 20, No. 3, 2000 |
