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Show Journal of Clinical Neuro-ophthalmology 13(2): 119-123, 1993. © 1993 Raven Press, Ltd., New York Bilateral Isolated Retrobulbar Optic Neuropathy in Limited Wegener's Granulomatosis Clifford J. Belden, M.D., Latif M. Hamed, M.D., and Anthony A. Mancuso, M.D. Wegener's granulomatosis causes a variety of ophthalmologic disorders, some of which occasionally constitute the initial presentation of the disease. We describe a patient who presented with bilateral, isolated, consecutive, posterior optic neuropathy with light perception and no light perception vision. The patient had no other symptoms or signs of orbital disease. Investigations revealed cavitary lung lesions, positive antineutrophilic cytoplasmic antibody (ANCA) titers, subtle focal enhancement of the intracanalicular optic nerves on magnetic resonance imaging, and a confirmatory bronchial biopsy, This exceedingly rare ocular presentation of Wegener's granulomatosis may pose a diagnostic quandary. Key Words: Optic neuropathy-Wegener's granulomatosis-- ANCA. From the Department of Ophthalmology (CJ.B., L.M.H.) and Radiology (A.A.M.), University of Florida College of Medicine, Gainesville, Florida, U.S.A. This work was supported by an unrestricted grant from Research to Prevent Blindness, Inc., New York, New York. Address correspondence and reprint requests to Dr. Latif M. Hamed, Department of Ophthalmology, University of Flonda, Box 100284, JHMHSC, Gainesville, FL 32610-0284, U.S.A. 119 Wegener's granulomatosis is an uncommon systemic necrotizing granulomatous vasculitis that, in the classic form, involves the lung, nasopharynx, and kidney (1). Ocular involvement occurs in 25 to 40% of cases (2-5), and may constitute the presenting signs and symptoms of the disease, particularly in the limited form (4,6,7). Optic neuropathy generally occurs either secondary to inflammation of vessels supplying the optic nerve or compression from contiguous sinus or orbital disease (2,5, 8,9). We describe a patient who developed isolated, bilateral consecutive, retrobulbar optic neuropathy as the presenting manifestations of Wegener's granulomatosis. CASE REPORT A 55-year-old woman was referred to our institution with the diagnosis of bilateral consecutive optic neuropathy. The patient had been well until approximately 1 year previously when she began having sinus congestion and pressure. At 9 months prior to admission she had an episode of "colitis" associated with a purpuric rash on her lower extremities with nontender nodules over her ankles, knees, and elbows. The colitis required hospitalization and multiple transfusions. A colonic biopsy revealed "vasculitis." She was treated with prednisone with resolution of her symptoms. The prednisone was slowly tapered over the next 6 months, and a repeat colonoscopy showed no evidence of colitis. At 6 weeks prior to admission the patient noted a central scotoma in her left visual field with reduced vision. This reportedly cleared spontaneously over the next week, but then recurred, at which time she sought an ophthalmologic evaluation. Vision was decreased in the left eye to hand 120 c. J. BELDEN ET AL. motions with an afferent pupillary defect. Westergren sedimentation rate was 32 mmHg. She was begun on oral prednisone, 10 mg daily. A spinal tap revealed 1 WBC, 111 RBC, glucose of 65 mg%, and protein of 52 mg%. Myelin basic protein and oligoclonal bands were absent. Cerebral spinal fluid IgG synthesis was mildly elevated at 4.25 mg/24 hours. Cerebral spinal fluid cultures, cryptococcal antigen, toxoplasmosis IgG antibody, and VORL were negative. The patient lost vision in the right eye 14 days later, to the level of counting fingers. She was admitted to the hospital with a diagnosis of bilateral optic neuritis and Solu-Medrol, 500 mg intravenously, was administered daily for 5 days. Her vision improved slightly, and she was discharged on no corticosteroids. Her vision remained stable for several days, then slowly decreased over the next 3 to 4 days. She was referred to our institution 9 days after discharge, when she awoke and could not see light with either eye. The patient complained of continuing sinus pressure and mild left periorbital discomfort. There was no pain on ocular movement. No known toxic exposures had occurred, and she denied tobacco and alcohol use. Past medical history was significant for B12 injections, Ouricef, and iron. Family history was noncontributory. On examination, vision was light perception in the right eye and no light perception in the left. Pupils were 6 mm each, with a trace reaction to light bilaterally, without a relative afferent defect. There was no proptosis or any other sign of orbital involvement. Applanation tensions were normal. Results of slit lamp and dilated funduscopic exam-inations were normal. Specifically, the optic discs showed no edema, hemorrhages, or pallor. The patient was admitted for intravenous SoluMedrol, 250 mg every 6 hours. Further workup included a chest radiograph, which revealed bilateral apical infiltrates, right greater than left, with a differential diagnosis, including tuberculosis and Wegener's granulomatosis (Fig. 1). A PPO (purified protein derivative) skin test was placed along with controls, and the results revealed the patient to be anergic. Because tuberculosis could not be ruled out, INH (isoniazid) and rifampin were begun orally. Magnetic resonance imaging of the orbits with gadolinium and fat suppression revealed bilateral subtle enhancement of the intracanalicular optic nerve sheaths with contiguous enhancement of the dura overlying the cavernous sinus (Fig. 2A). The intraorbital and intracranial portions of the optic nerves, as well as the optic chiasm and tracts appeared normal. Laboratory evaluation was significant for a white blood cell count (WBC) of 9,700/mm3 , and microcytic anemia with a hematocrit of 30.2%. Electrolytes, liver function tests, urinalysis, PT, and PIT were all normal. Westergren sedimentation rate was elevated to 72 mm/h, and C3 and C4 complement levels were mildly elevated. HATTS, VORL, rheumatoid factor, antinuclear antibodies, and Lyme titers were negative. An otolaryngologic examination revealed ulceration of the nasal septum, and a biopsy revealed squamous mucosa with acute and chronic inflammation and scale crust formation. An initial transbronchial biopsy revealed no significant vasculitis, granulomas, or inflammation. FIG. 1. Admission chest radiograph demonstrati ng biapical i nfi Itrates, with the right greater than the left. No cavitation is evident. These infiltrates were more extensive than those on a comparison film from 8 months previously. OPTIC NEUROPATHY IN WEGENER'S GRANULOMATOSIS 121 (A.) FIG. 2. (A) Magnetic resonance image obtained on the patient's first admission with subtle enhancement of the optic nerve sheaths (arrow) and dura overlying the cavernous sinus (arrowheads); all findings are more prominent on the right. (B) On the patient's second admission, a repeat magnetic resonance imaging study again revealed enhancement of the intracanalicular optic nerve sheaths (arrow) as well as enhancement of the dura just anterior to the cavernous sinus on the right (arrowheads) and planum sphenoidale (open arrow). The patient was continued on high-dose intravenous corticosteroids for 10 days with slow improvement in her vision to count fingers in both eyes. She was discharged on Medrol, 48 mg per day orally, as well as antituberculosis medications. Two weeks later the patient awoke with her vision decreased to light perception in the right eye and hand motion in the left. She was again admitted and placed on Solu-Medrol 250 mg every 6 hours. The antineutrophilic cytoplasmic antibody (ANCA) sent to the laboratory on her previous admission returned positive at 52 units «22 units is negative; Specialty Laboratories Inc., Santa Monica, CA). The following day Cytoxan therapy was begun intravenously. Her vision improved to count fingers bilaterally over the next 2 days. A repeat magnetic resonance imaging study of the orbits again revealed enhancement of the intracanalicular optic nerve sheaths bilaterally and enhancement of the planum sphenoidale and meninges overlying the cavernous sinus on the right (Fig. 2B). The patient was discharged, and Medrol, orally 16 mg three times a day, was prescribed. Vision continued to improve slowly. On follow-up she was noted to have increase in cough, sputum, and some hemoptysis. A chest radiograph revealed increased cavitation in the right upper lobe. The patient was admitted for antibiotics intravenously. A repeat bronchoscopy was performed and a 0.5-mm lesion was seen intrabronchially and biopsied. Pathologic examination revealed necrotizing granulomatous vasculitis consistent with Wegener's granulomatosis . Over the next 2 months the patient was maintained on oral Medrol and Cytoxan with maintenance of her vision at the level of count fingers bilaterally. A repeat antineutrophilic cytoplasmic antibody titer (ANCA), taken 3 months after the original, had decreased to 30 units. DISCUSSION Wegener's granulomatosis is a systemic necrotizing granulomatous vasculitis (1) with frequent ophthalmologic (2,3,7,8) and neurologic manifestations (10,11). It occurs in a classic form with involvement of lungs, sinuses, and kidneys, and in a Jelm Nellro·ophthalmol. Vol. 13. No.2, 1993 122 c. J. BELDEN ET AL. limited form that spares the kidneys and has an overall better prognosis (4,12,13). Ocular involvement may be the presenting complaint in Wegener's granulomatosis, particularly in the limited form of the disease (6,7). Ocular involvement generally is of two varieties-focal and contiguous. Focal disease occurs independent of respiratory tract involvement and frequently involves sclera, episclera, cornea uvea, and retina. Contiguous disease results from direct spread of inflammation from surrounding sinuses and is manifest by orbital inflammation (2-4,7,8). Our patient had some atypical features of Wegener's granulomatosis that made the diagnosis more difficult. Initially, the presence of ulcerative lesions in the nasal cavity and tracheobronchial tree was suggestive of Wegener's granulomatosis, but the location of the cavitary lung lesions in the upper lobes was somewhat unusual, suggesting the possibility of tuberculosis. The case for Wegener's was strengthened when the antineutrophilic cytoplasmic antibody (ANCA) was positive. The history of colitis and purpura occurring 9 months prior to the visual loss strengthened the case for a vasculitic disorder. It is tempting to speculate that had a diagnosis been made at that time and appropriate therapy instituted, the visual loss might have been averted. Our clinical suspicion for Wegener's granulomatosis remained despite the initial nondiagnostic biopsies, partially due to the positive ANCA, leading to the second, confirmatory biopsy. Recent studies have documented the usefulness of ANCA for the workup of suspected vasculitic disorders of the eye (14). It should be emphasized that the initial monocular visual loss in our patient should have inspired a complete workup for giant cell arteritis, even though the patient is at the younger end of the spectrum for the condition. This is of obvious importance so that appropriate therapy may be initiated within the narrow window of opportunity between unilateral and consecutive visual loss. The optic nerve is the most commonly affected cranial nerve in Wegener's granulomatosis (15), and may become involved either as a result of orbital disease with resultant compression, or because of inflammation of vessels supplying the optic nerve (4,5,7-9). In a 1957 review of ocular complications of Wegener's, Straatsma (3) describes one patient who developed decreased vision and proptosis who on autopsy had encasement of the nerve with necrotic granuloma, but with preservation of the nerve substance. In a review of 140 patients with Wegener's gran- TOm Neuro-ophlhalmol. Vol. 13. No.2, 1993 ulomatosis, Bullen et aL (2) found 9 patients with optic nerve involvement; 6 of these were due to contiguous orbital disease, but 3 had no evidence of orbital disease. Unlike our patient, all three had optic disc swelling (2). Spalton (7) described eight patients with the limited form of Wegener's granulomatosis, all of whom had ocular involvement. Four of these patients had orbital disease, and one developed bilateral optic neuropathy from compression of the optic nerves by a parasellar mass (7). In the review of Haynes et aL (8), of 29 patients with Wegener's granulomatosis, 4 developed vasculitic involvement of the optic nerve. AIl 4 patients showed proptosis; 2 of these had vision of no light perception at presentation, and 1 recovered 20/20 vision after treatment with corticosteroids and Cytoxan (8). Anderson et aL (15) described a patient who developed bilateral consecutive loss of vision with normal findings on fundoscopy. At craniotomy a layer of abnormal tissue over the anterior fossa and encasing the optic nerves was found. On histology there was chronic inflammation and multinucleate giant cells, but no evidence of vasculitis (15). The radiographic finding in our patient of enhancement of the meninges in the anterior cranial fossa and optic nerve sheaths may represent a similar process. The pattern of dural enhancement contiguous with the optic nerve sheath and sparing of the nerve both anterior and posterior to this area suggest primary involvement of the meninges with Wegener's granulomatosis and secondary involvement of the optic nerves. Acute isolated consecutive retrobulbar optic neuropathy has been rarely described either as a presenting sign or accompanying Wegener's granulomatosis at any stage. We could find only one brief citation, provided by Miller (16), of a case apparently similar to ours, but as the details of the case are lacking, the status of orbital involvement is unknown (17). Our patient demonstrates that optic neuropathy associated with Wegener's disease may pose a diagnostic quandary. Absence of signs of orbital inflammation and absence of optic disc swelling in the setting of acute consecutive bilateral optic neuropathy has been rarely reported in Wegener's disease and initially suggested posterior ischemic optic neuropathy due to giant cell arteritis. REFERENCES 1. Godman GC, Churg J. Wegener's granulomatosispathology and review of the literature. Arch PathoI1954;58: 533--53. OPTIC NEUROPATHY IN WEGENER'S GRANULOMATOSIS 123 2. Bullen CL, Liesegang TJ, McDonald TJ, et al. Ocular complications of Wegener's granulomatosis. Ophthalmology 1983;90:279-90. 3. Straatsma BR. Ocular manifestations of Wegener's granulomatosis. Am J OphthalmoI1957;44:789-99. 4. Robin JB, Schanzlin OJ, Meisler OM, et al. Ocular involvement in the respiratory vasculitides. Sun> Ophthalmol1985; 30:127--40. 5. Cutler WB, Blatt 1M. The ocular manifestations of lethal midline granuloma (Wegener's granulomatosis). Am J Ophthalmol 1956;42:21-35. 6. Coutu RE, Klein M, Simmons L, et al. 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Diagnostic value of antineutrophil cytoplasmiC antibodies in scleritis associated with Wegener's granulomatosis. Ophthalmology 1992; 99:125-32. 15. Anderson JM, Jamieson DG, Jefferson JM. Non-healing granuloma and the nervous system. Q J Med 1975;44:30917. 16. Miller NR. Walsh alld Hoyt's clillicallleuro-ophthalmology. 4th ed. Vol. 4. Baltimore: Williams & Wilkins, 1992:2641. 17. Arruga J, et al. Bilateral retrobulbar optic neuritis in Wegener's granulomatosis. Presented at the VIIth International Neuro-ophthalmology Symposium. Winchester, England, June 23--29, 1990. I Clill Ncuro-ophtiullmol, Vol. 13, No.2, 1993 |
