O31 Giant cell arteritis - a rare ocular presentation

Visual Loss Caused by Choroidal Ischemia Preceding Anterior Ischemic Optic Neuropathy in Giant Cell Arteritis

Biopsy-Proven Giant Cell Arteritis Associated Vision Loss in Chinese Subjects: A Case Series and Review of the Literature

Dear Editor, We read with interest the case reported by Patyal et al.[1] on Vogt-Koyanagi-Harada (VKH) disease associated with anterior ischemic optic neuropathy (AION) in a young woman presenting as acute angle-closure glaucoma. We are surprised by the authors' decision to attribute the disc damage to AION. Anterior ischemic optic neuropathy is typically associated with optic disc edema with neuroretinal rim pallor in majority.[2] It is also associated with “flu”-like symptoms, weight loss, fever of unknown origin, headaches, scalp tenderness, neck pain, jaw claudication and anemia, none of which were reported in this case. Investigational evidence such as delayed filling and late leakage at optic disc in fluorescein angiography (FA) and elevated erythrocyte sedimentation rate and C-reactive protein levels were also not reported. Nakao et al. in a series of 52 VKH patients reported 6 patients who developed non-reversible visual field loss attributed to AION.[3] All these eyes exhibited disc edema and AION features in FA, in addition to the systemic manifestations. In the interest of the readership, we would like to know from the authors how secondary angle-closure glaucoma (SACG) due to ciliary body swelling and suprachoroidal fluid accumulation in VKH was ruled out. We attribute the advanced glaucomatous disc damage seen at presentation to the recurrent SACG insult in the past as the authors themselves had suggested in the discussion. We also noted that the disc photo at 1-year follow-up did not display segmental pallor, especially in the inferior rim although the authors attribute the superior arcuate visual field defect to AION. There was no mention of the details of further glaucoma treatment in this young female with advanced disc damage. Thus, the diagnosis of this reported case is not agreeable to us. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Almost two-thirds of patients with giant cell arteritis (GCA) develop ocular symptoms and up to 30% suffer permanent visual loss. We review the three most common mechanisms for visual loss...

A 61-year-old female presented with a moderate decrease in vision in the left eye. The patient denied any other ocular or systemic symptoms related to giant cell arteritis. Visual acuity was 20/50 in the left eye with a 2+ relative afferent pupillary defect and markedly abnormal color vision. Dilated fundus examination and flourescein angiography revealed optic disc edema as well as a cilioretinal artery occlusion. Erythrocyte sedimentation rate was only slightly elevated. Subsequent biopsy of the superficial temporal artery confirmed the diagnosis of giant cell arteritis. Cilioretinal arteries are anatomical variants derived from the short posterior ciliary arteries. Arteritic anterior ischemic optic neuropathy typically results from thrombotic occlusion of the short posterior ciliary arteries. Consequently, arteritic occlusion of the short posterior ciliary arteries can result in concomitant occlusion of the cilioretinal artery. This case highlights the situation where clinical symptoms were not suspicious for giant cell arteritis but the presence of an anterior ischemic optic neuropathy and a cilioretinal artery occlusion was virtually pathognomonic for giant cell arteritis.

To report the unusual association between non-arteritic anterior ischaemic optic neuropathy (NAION) and non-ischaemic central retinal vein occlusion (CRVO) in two patients. Case reports are presented. Non-ischaemic CRVO was manifested by dilated, tortuous retinal veins with flame-shape retinal haemorrhages. Fluorescein angiography showed prolonged arteriovenous transit time and normal retinal capillary perfusion without macular oedema. The presence of colour vision abnormalities, relative afferent pupillary defects, pale disc swelling and visual field deficits indicated that the visual loss was attributable entirely to NAION. Laboratory investigations disclosed impaired fibrinolytic function in case 1 and the presence of antiphospholipid antibodies in case 2. Compression of the central retinal vein by the swollen optic nerve could have predisposed to CRVO. The presence of thrombophilic abnormalities may have contributed to the concomitant occlusion of posterior ciliary arteries and central retinal vein. Ischaemic optic neuropathy needs to be considered in patients with CRVO when the visual acuity is not consistent with the retinal pathology.

PurposeTo describe chorioretinal signs in a case series of Giant Cell Arteritis (GCA).MethodsThis is a multicenter retrospective observational case series with GCA that presented with a headache and an abrupt, unilateral loss in vision. Workup included temporal artery biopsies, intravenous fluorescein angiography, optical coherence tomography (OCT), optical coherence tomography angiography (OCTA), blood levels of erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP).ResultsThere are a total of 8 GCA instances presented. Average age was 74.5. (Range 68–83 years). The patients reported that one eye's visual loss had suddenly started, along with a fresh headache and other systemic symptoms. Eight patients exhibited choroidal ischemia, five paracentral acute middle maculopathy (PAMM) lesions, five cotton wool spots, four anterior ischemic optic neuropathy, and one central retinal arterial occlusion at the time of presentation. The average ESR at presentation was 68 mm/hr (range 4–110), and 4/6 individuals had a significant increase. The mean CRP level was 6.2 mg/dL (range 2.0–15.4), and the level was always over the normal range. All patients' temporal artery biopsies were positive.ConclusionAlongside PAMM lesions, cotton wool spots, anterior ischemic optic neuropathy, and central retinal artery occlusion, choroidal ischemia is a key angiographic indicator in the diagnosis of GCA. It may be crucial to recognize these typical ischemic chorioretinal signs while diagnosing GCA.

Dear Editor, Altitudinal visual field defect (VFD), which involves the loss of visual sensation in the horizontal half of the visual field, is caused mainly by anterior ischemic optic neuropathy (AION),1,2,3 or rarely by compressive neuropathy due to a tumor or aneurysm.4,5 However, acute-onset inferior altitudinal VFD is a hallmark of AION.1,2,3 We observed a patient with a rapidly developed inferior altitudinal VFD due to a small meningioma inside the optic canal, which was almost undetectable in standard imaging studies. A 65-year-old man with hypertension and hyperlipidemia complained of blurred vision persisting over several days. He denied having headache, diplopia, or ocular pain. He had a history of medullary infarction and was taking aspirin, antihypertensive medication, and a statin. With the exception of the old infarction, there were no abnormal findings on axial T2-weighted MRI scans obtained 10 months previously (Supplementary Fig. 1 in the online-only Data Supplement). An ophthalmologic examination revealed decreased visual acuity in the left eye (10/20) compared to the right (16/20). Humphrey perimetry revealed an inferior altitudinal VFD in the left eye, which was more severe on the nasal side than on the temporal side (Fig. 1A). There was no evidence of relative afferent pupillary defects, ptosis, proptosis, or disc swelling, or pallor on funduscopy. Ocular movement, intraocular pressures, and slit-lamp and optical coherence tomography findings were normal. The acute onset and painless monocular visual loss raised a suspicion of AION, leading to a focus on evaluating the patient's vascular etiology. However, there were no abnormal findings on MR angiography (Supplementary Fig. 1 in the online-only Data Supplement). A follow-up visual field test performed 3 weeks later revealed an aggravated altitudinal VFD, especially on the inferior temporal side (Fig. 1A). High-spatial-resolution (2-mm slice thickness) MRI with gadolinium enhancement of the orbit was performed to exclude compressive neuropathy mimicking AION. The results revealed a 1.2-cm meningioma on the posterior part of the left optic canal causing medial displacement and downward compression of the optic nerve (Fig. 1B and C). The patient underwent a frontotemporal craniotomy and resection of the meningioma. The tumor originated from the left side tuberculum sellar and extended into the optic canal. Although he reported a subjective improvement in vision immediately after surgery, the VFD was aggravated on the superior side just before surgery (Fig. 1A). Four months after resection of the tumor, his vision had partially recovered (Fig. 1A). Fig. 1 Serial Humphrey perimetries and high-resolution MRI of compressive optic neuropathy with a monocular inferior altitudinal visual field defect (VFD). A: Initial Humphrey perimetry shows an inferior altitudinal VFD in the left eye that is more severe on ... The main mechanism underlying the visual disturbance in compressive optic neuropathy is secondary disruption of the axoplasmic flow and demyelination of the optic nerve.6 Therefore, the onset of VFD usually progresses slowly. Since the optic nerve delivers visual information received from each of the retinal quadrants to the optic chiasm, the VFD can present in various patterns according to the initial direction of optic nerve compression. In this case the VFD started at the inferonasal area and extended to the inferotemporal area, which can be explained by a medial downward displacement of the optic nerve due to compression from the meningioma (Fig. 1D). The VFD was rapid and severe due to the meningioma being located on the optic canal and the diagnosis delay. Another possible mechanism is ischemia caused by vascular compression. Ischemic symptoms usually present as posterior ischemic optic neuropathy (PION) with compression of the collateral branches from the ophthalmic artery in the optic canal.7 However, ischemia is unlikely to be the main mechanism in this case for two reasons: 1) an altitudinal VFD is relatively rare in PION7 and 2) in this case the VFD worsened according to the direction of nerve compression and improved following surgical resection. Other features associated with compressive optic neuropathy were absent in this case, which may be due to the smallness of the tumor. Optic nerve meningioma arising within the optic canal is rare.8 These tumors are usually extremely small despite causing significant visual disturbance and can easily be overlooked on routine MRI. Furthermore, they may not have any orbital signs, and may present with a normal-appearing optic disc.8 Therefore, it is necessary to consider compressive optic neuropathy when a patient presents with an altitudinal VFD, even in the absence of other symptoms.

Editor,—Anterior ischaemic optic neuropathy (AION) is the most common cause of visual loss in giant cell arteritis (GCA). However, other presentations have been described including posterior ischaemic optic neuropathy, choroidal...

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AB0744 Ocular involvement in giant cell arteritis: A cross-sectional study

Nonarteritic Anterior Ischemic Optic Neuropathy: Time of Onset of Visual Loss

Abstract

Introduction:Here, we describe a patient who presented with anterior ischemic optic neuropathy (AION) andsubsequently developed uveitis.Case:A 69-year-old man was referred to our hospital and initially presented with best-corrected visualacuities (BCVA) of 20/40 (right eye) and 20/1000 (left eye) and relative afferent pupillary defect.Slit-lamp examination revealed no signs of ocular inflammation in either eye. Fundus examinationrevealed left-eye swelling and a pale superior optic disc, and Goldmann perimetry revealed left-eyeinferior hemianopia. The patient was diagnosed with nonarteritic AION in the left eye. One weeklater, the patient returned to the hospital because of vision loss. The BCVA of the left eye was sopoor that the patient could only count fingers. Slit-lamp examination revealed 1+ cells inthe anterior chamber and the anterior vitreous in both eyes. Funduscopic examination revealedvasculitis and exudates in both eyes. The patient was diagnosed with bilateral panuveitis, andtreatment with topical betamethasone was started. No other physical findings resulting from otherautoimmune or infectious diseases were found. No additional treatments were administered, and opticdisc edema in the left eye improved, and the retinal exudates disappeared in 3 months. Thepatient’s BCVA improved after cataract surgery was performed.Conclusion:Panuveitis most likely manifests after the development of AION.

There are only 2 published cases of anterior ischemic optic neuropathy (AION) occurring after neck dissection, to our knowledge. We report a case of bilateral AION following neck dissection, discuss the differential diagnoses, and compare the features of this case with those of previously described cases. There were none of the previously described risk factors for the development of AION after head and neck surgery (eg, hypotension, facial edema, or sudden massive hemorrhage) in this case, but there was prolonged diffuse postoperative bleeding. Also, the symptoms did not arise before the fifth postoperative day as they did in the other cases. Prolonged, mild postoperative bleeding is a risk factor for AION. Visual loss during the entire first postoperative week has to be regarded as a complication of surgery, requires the exclusion of several differential diagnoses, and must not be confounded with the patient's confusion or symptoms of withdrawal.