A 54-year-old white man reported transient left monocular visual loss lasting 2 minutes on standing. Five days later, while walking, suddenly his left eye “just saw gray with some sparing to the left side” for 3-5 minutes. He had a 3-decade history of pars planitis in the left eye requiring pars plana vitrectomy and cataract surgery with insertion of an intraocular lens. The patient denied systemic symptoms. He experienced 2 additional similar events, 1 with bending down.
Initial ophthalmologic examination was normal except for chronic retinal changes in the left eye from pars planitis. Laboratory testing revealed normal complete blood count (CBC) including platelet count of 339,000 per cubic millimeter and erythrocyte sedimentation rate (ESR) of 20 mm/h. Fluorescein angiography showed normal choroidal filling, delayed retinal filling at 28 seconds, enlarged foveal avascular zone with microaneurysms, and peripheral vascular staining with areas of peripheral nonperfusion in the left eye. MRI and MRA of the brain and neck were normal.
The patient continued to experience transient monocular visual loss (TMVL) in the left eye and underwent neuro-ophthalmic evaluation. He denied headache, jaw claudication, scalp or temporal tenderness, loss of appetite, weight loss, and fever or symptoms of polymyalgia rheumatica. Physical examination, including carotid auscultation and blood pressure measurement, was normal. Visual acuity was 20/15, right eye, and 20/25, left eye. There was a left relative afferent pupillary defect (RAPD) of 0.6 log units. Biomicroscopic examination was unremarkable and intraocular pressures were 14 mm Hg in both eyes with open angles on gonioscopy. Automated visual field testing was normal on the right, and there was an inferonasal defect on the left. Indirect ophthalmoscopy of the right eye showed peripheral vascular sheathing. In the left eye, the optic disc was normal, there was pigment mottling in the fovea, multiple cotton wool spots in the posterior pole, and peripheral vascular sheathing.
Laboratory testing revealed a number of normal studies including CBC, platelet count, metabolic panel, hypercoagulable studies, fluorescent treponemal antibody absorption, Lyme and Bartonella antibodies, hepatitis panel, HIV, angiotensin-converting enzyme, antinuclear antibody, antineutrophil cytoplasmic antibody, and purified protein derivative skin testing. ESR was 6 mm/h, C-reactive protein (CRP) was 2.57 mg/L (normal, <5 mg/L), and haptoglobin was 245 mg/dL (normal, <200 mg/dL). A rheumatology consultant suggested temporal artery biopsy because the arteries were unusually prominent, although nontender and pulsatile. The biopsy revealed granulomatous inflammation with multiple giant cells, fragmentation, and duplication of the internal elastic lamina consistent with temporal arteritis (TA). Chest radiograph (CXR) showed a dilated and tortuous aorta, and thoracic MRI revealed dilation of the ascending aorta without aortic valve compromise.
The patient was treated with high-dose oral corticosteroids and had no further episodes of TMVL, and the cotton wool spots disappeared. The left RAPD and the visual field defect remained unchanged. The left optic disc became mildly pale. The temporal arteries became significantly smaller in size. The CBC, ESR, and CRP have remained normal, and the haptoglobin normalized after nearly 12 months of steroid therapy. To date, repeat imaging of the aorta has not been performed.
The most common identifiable cause of TMVL is atheromatous disease affecting the internal carotid arterial system (1). In patients with TA, transient visual symptoms may precede permanent blindness in 50%-64% by an average of 8.5 days. Visual loss results from optic nerve, retinal, or choroidal ischemia and may be exacerbated by postural changes (2).
Elevated ESR and CRP are well-established aids in the diagnosis of TA. When combined, the 2 markers provide a sensitivity of up to 99.2% (3). Although normal ESR, CRP, or both are well documented in biopsy-proven TA, the exact prevalence is uncertain. Salvarani and Hunder (4) reported ESR less than 40 mm/h in 5.4% and less than 50 mm/h in 10.8% of 167 patients with active TA. Thrombocytosis over 400,000 per microliter may be another useful marker for positive temporal artery biopsy and make the diagnosis of TA 6 times more likely (5). Other markers may include fibrinogen, which is often elevated in TA but low in other conditions that raise the ESR; interleukin (IL)-6, which may be more sensitive than the ESR and comparable to the CRP; and other, less, well-studied reactants such as haptoglobin, IL-2, interferon-γ, IL-1β, platelet-derived growth factor, and vascular endothelial growth factor (5).
Thoracic and abdominal aortic structural damage is a well-documented complication of giant cell arteritis and occurs in a significant proportion of patients with TA, sometimes necessitating surgical repair. In a prospective study, Garcia-Martinez et al reported the development of aortic aneurysm or dilation in 22% of patients with biopsy-proven TA after a mean follow-up of 5.4 years. Interestingly, this finding was significantly more frequent among patients with weak inflammatory markers (6). MRI or positron emission tomography may be used in the detection of aortic involvement, to follow disease activity and response to therapy. Although we do not routinely screen patients with TA for the presence of thoracic aneurysm, the CXR finding led to further investigation in this case. While it cannot be said with complete certainty, it may be that the aortic dilation seen in this patient is the result of TA. Awareness of the possible presence of aortic aneurysm in TA may aid prompt recognition and therapy to avoid life-threatening complications.
As in our patient, occult TA (OTA) presents with local ischemic symptoms without the symptoms or signs of systemic inflammation (7). In a prospective study, Hayreh et al (8) reported 21.2% incidence of OTA in patients presenting with ocular involvement from TA. Amaurosis fugax was highly suggestive of TA, occurring in 33% of patients with occult disease. Interestingly, the ESR and CRP values were relatively lower in the group without systemic symptoms. Other studies (9,10) have found that lower ESR values and the absence of constitutional symptoms may be associated with an increased risk of irreversible cranial ischemic complications and visual loss.
It is important to consider the diagnosis of TA in patients presenting with TMVL, retrobulbar optic neuropathy, cotton wool spots, or dilated nontender temporal arteries in an otherwise asymptomatic relatively young patient even with normal inflammatory markers. Patient follow-up is mandatory to monitor their potential ocular and systemic morbidity.
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