O31 Giant cell arteritis - a rare ocular presentation

Case report - Introduction Giant cell arteritis (GCA) is a systemic vasculitis primarily affecting large- and medium-sized arteries. Classic symptoms include headache, scalp tenderness, jaw claudication and visual disturbances. Ophthalmic artery involvement commonly causes anterior ischaemic optic neuropathy. Uncommon ocular features include anterior segment ischaemia, hypotony, tonic pupil or rarely choroidal ischaemia. Heterogenicity of presentation can make diagnosis difficult leading irreversible visual loss. We report a case of bilateral macular choroidal ischaemia with atypical symptoms of GCA. It emphasises the need of complete evaluation in elderly patients with GCA and visual symptoms and the need to start aggressive treatment to prevent visual loss. Case report - Case description 73-year-old caucasian lady presented to the eye emergency department with diplopia. Medical history includes systemic hypertension, hypothyroidism and hyperlipidaemia, no past ocular history. Eye examination was normal except decompensated fourth nerve paresis. Thyroid function was normal. Diplopia resolved spontaneously. Patient re-presented with a floater in the right eye and left-sided atypical headache without jaw claudication. Investigations: normal FBC, CRP 126, ESR 100, PV 1.67. Following rheumatologist review she was commenced on oral prednisolone 60 mg with clinical suspicion of GCA. Temporal artery biopsy confirmed GCA. She had TIA subsequently. MRI revealed small area of acute infarct in left ganglio-capsular region. Clopidogrel was started for secondary prevention. In the ophthalmology clinic she saw a lacy pattern. Her Log MAR VA in right and left eye was 0.64 and 0.76, respectively. Fundoscopy revealed retinal pigment epithelial (RPE) mottling at the maculae, right more than left eye. Optical Coherence Tomography (OCT) macula revealed bilateral RPE elevations and serous pigment epithelial detachment bilaterally, patchy central RPE atrophy with external limiting membrane disruption, more pronounced in the right eye. Fundus fluorescein angiogram and indocyanine green angiography confirmed bilateral choroidal ischaemia (triangular shaped with the base at the equator) at the macula worse in right than left eye. Oral prednisolone was continued with gradual tapering. VA improved to Log MAR 0.5 and Log MAR 0.2 in right and left eye at six weeks. OCT showed signs of RPE re-modelling with resolution of sub retinal fluid (resolution of inflammation). At recent follow up Log MAR VA is 0.26 and 0.06 in right and left eye respectively. She is on oral prednisolone 20 mg once a day tapering 2.5 mg every 2 weeks. OCT shows further re-modelling of the ellipsoid zone in the left eye, but her right eye shows more RPE atrophy and thinning with RPE degeneration. Case report - Discussion We report an unusual case of GCA with atypical symptoms and bilateral choroidal ischaemia. Patients with GCA usually present with systemic symptoms and signs like headache, scalp tenderness, fever, and jaw claudication. Variable presentation can often lead to misdiagnosis and consequent irreversible loss of vision. Visual symptoms as the first and only sign of GCA was first reported in 1952. Posterior ciliary arteries in the eye can be affected leading to optic nerve infarction and subsequent anterior ischaemic optic neuropathy (AION). AION and visual field loss accounts for 80—90% of cases with ocular signs of GCA. Posterior ciliary artery occlusion can rarely cause patches of choroidal infarcts which appear as chorio-retinal degeneration in a couple of weeks. These patches are usually in the mid-peripheral fundus, usually triangular shaped with the base towards equator and apex toward posterior pole. In our case the presentation was very atypical in the sequence of symptoms. Her raised inflammatory markers raised the suspicion of GCA and prompt referral to rheumatology was done. Aggressive treatment with oral steroids was started with stomach and bone protection. Temporal artery biopsy confirmed the diagnosis. The bilateral triangular ischaemic areas found on FFA and ICG confirmed the macular choroidal ischemia. Her OCT also showed bilateral RPE mottling showing degenerative changes due to choroidal infarct from posterior ciliary artery occlusion. We managed to preserve the vision in our case by starting the timely aggressive steroid treatment. In summary, we report an unusual case of GCA with atypical symptoms and bilateral choroidal ischaemia where further visual loss was avoided due to timely intervention. GCA has variety of presentations; a combined team approach of ophthalmologists and rheumatologists can prevent irreversible visual loss in such cases. Case report - Key learning points GCA is a chronic idiopathic inflammation more commonly seen in the large- and medium-sized vessels. Posterior ciliary arteries in the eye can be affected in GCA leading to optic nerve infarction and subsequent anterior ischaemic optic neuropathy (AION). AION and visual field loss accounts for 80—90% of cases with ocular signs of GCA. Posterior ciliary artery occlusion can rarely cause patches of choroidal infarcts which appear as chorio-retinal degeneration in a couple of weeks. These patches are usually in the mid-peripheral fundus, usually triangular shaped with the base towards equator and apex toward posterior pole. Prompt diagnosis and aggressive treatment with corticosteroids can prevent visual loss in one or both eyes. Any patient over 50 years of age presenting with visual symptoms of amaurosis fugax, diplopia, or visual loss with ocular signs of anterior or posterior ischaemic optic neuropathy, central retinal artery occlusion or cilioretinal artery occlusion should create a high suspicion for GCA. This group of patients should have urgent ESR, CRP and PV evaluation. If suspected, high-dose corticosteroids must be started followed by temporal artery biopsy for confirmation. It is imperative to diagnose GCA early and start treatment urgently to prevent visual loss. A multidisciplinary team approach in patients with GCA can prevent sight loss and life too.

Evaluation of Haemodynamics and Morphology of Carotid Artery in Nonarteritic Anterior Ischaemic Optic Neuropathy

Purpose To compare haemodynamics and morphological changes of carotid artery in patients with unilateral non-arteritic anterior ischaemic optic neuropathy (NAION) with their contralateral side. Methods Twenty-six patients with unilateral NAION were included in this retrospective study. Colour Doppler imaging (CDI) was used to measure the haemodynamics and morphological changes of carotid artery, including blood flow velocities of common carotid artery (CCA) as well as the diameter of CCA, internal carotid artery (ICA), external carotid artery (ECA) and the thickness of carotid artery plaque. A paired-sample t test was performed to analyze those carotid artery hemodynamics and morphological changes between the affected eye and the unaffected contralateral eye. Subgroup analysis of the blood flow velocities of CCA was performed by dividing patients into two groups according to the presence/ absence of diabetes mellitus, hypertension or hyperlipidaemia. Results The average blood flow velocity of CCA in the affected eyes was 0.45 ± 0.13m/s, greater than that in the unaffected eyes (0.41 ± 0.15m/s) (p = 0.003; t=-3.321). Subgroup analysis found this significant bilateral difference was still existed in groups of diabetes mellitus, non-diabetes mellitus, and hypertension except for groups of non-hypertension, hyperlipidamemia, and non-hyperlipidaemia. The thickness of carotid artery plaque was 0.75 ± 1.33mm in the affected eyes, insignificantly differed from that in the unaffected eyes (0.55 ± 0.77mm) (p = 0.4). There were no difference in the diameters of the CCA, ICA, and ECA between the affected and unaffected eyes (p = 0.862, 0.758, and 0.72, respectively). Conclusions Increased CCA blood flow velocity might be associated with the development of NAION. Further study on larger scale are needed to confirm our findings.

MRI signs helpful in the differentiation of patients with anterior ischaemic optic neuropathy and optic neuritis

Background/AimsThe aim of this study was to identify specific MRI characteristics of anterior ischaemic optic neuropathy (AION) and optic neuritis (ON) that would aid in the differentiation between these two diagnoses.MethodsWe retrospectively analysed a consecutive case series including all patients with an MRI study of brain and orbit and the clinical diagnosis of either ON or AION. We examined the scans for restricted diffusion of the optic nerve, optic sheath diameter, enhancement and location of enhancement of the optic nerve and distribution of the white matter lesions.ResultsFifty patients met the inclusion criteria. We found an accuracy of 0.98 for the discrimination between AION and ON based solely on parameters extracted from MRI data. Dominance analysis to determine the most influential parameters showed that the enhancement pattern of the optic nerve and distribution of the white matter lesions had the biggest impact on the classification and led to a discrimination accuracy of 0.9 when used alone.ConclusionIn patients with an inconclusive clinical diagnosis, optic nerve enhancement pattern and distribution of white matter lesions can aid in the diagnosis and differentiation between AION and ON. Diffusion-weighted imaging did not add significant information to the diagnosis or help to differentiate between the two conditions.

Proning related bilateral anterior ischaemic optic neuropathy in a patient with COVID-19 related acute respiratory distress syndrome

Background Non-arteritic ischaemic optic neuropathy (NAION) is a rare but harmful complication of prone positioning. Prone mechanical ventilation is a therapeutic strategy which has been used extensively during the COVID-19 pandemic to treat acutely hypoxemic patients with COVID-19 related acute respiratory distress syndrome (ARDS). Though a small number of cases of unilateral NAION have been reported in patients testing positive for the SARS-CoV-2 virus, we describe what is to our knowledge, the first reported case of bilateral NAION occurring in a patient proned extensively for the treatment of COVID-19 related ARDS. We consider the potential aetiological factors leading to NAION after prone mechanical ventilation in patients with COVID-19 and suggest strategies to protect against its development. Case presentation : We report a case of severe, irreversible, visual impairment secondary to bilateral anterior ION in a fifty-five-year-old male who underwent eight episodes of prone mechanical ventilation to treat COVID-19 related ARDS. Once weaned from his sedation he reported bilateral painless vision loss, and bedside ophthalmological assessment identified a reduced visual acuity of 3/30 unaided in the left eye and counting fingers in the right. Dilated indirect ophthalmoscopy revealed inferotemporal optic disc oedema with splinter haemorrhages in the right eye and mild disc oedema, temporal pallor, and nerve fibre layer haemorrhages inferiorly in the left eye. Humphrey visual field 24 − 2 testing confirmed a severely constricted visual field with macular sparing on the right and depressed inferonasal vision with preserved peripheral vision on the left eye. OCT disc imaging shortly after diagnosis revealed bilateral disc swelling and flame haemorrhages in the right eye. Conclusions NAION is a devastating, but preventable complication of prone positioning, which may pose significant risk of vision loss in patients with COVID-19 related ARDS.

Vision loss in giant cell arteritis

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 in GCA, describing the relevant ophthalmic arterial anatomy and emphasising how ophthalmoscopy holds the key to a rapid diagnosis. The short posterior ciliary arteries supply the optic nerve head, while the central retinal artery and its branches supply the inner retina. GCA has a predilection to affect branches of posterior ciliary arteries. The most common mechanism of visual loss in GCA is anterior arteritic optic neuropathy due to vasculitic involvement of short posterior ciliary arteries. The second most common cause of visual loss in GCA is central retinal artery occlusion. When a patient aged over 50 years has both anterior ischaemic optic neuropathy and a central retinal artery occlusion, the diagnosis is GCA until proven otherwise, and they should start treatment without delay. The least common culprit is posterior ischaemic optic neuropathy, resulting from vasculitic involvement of the ophthalmic artery and its pial branches. Here, the ophthalmoscopy is normal acutely, but MR imaging of the orbits usually shows restricted diffusion in the optic nerve.

Non-arteritic anterior ischaemic optic neuropathy sequential to SARS-CoV-2 virus pneumonia: preventable by endothelial protection?

We present a case of non-arteritic anterior ischaemic optic neuropathy (NAION) with no ocular or systemic risk factors in a patient who recovered from a recent SARS-CoV-2 pneumonia. NAION is the most common acute optic neuropathy among individuals over 50 years of age. It results from a transient hypoperfusion of the optic nerve head circulation, especially in patients with low vascular compliance due to ocular or systemic risk factors. We attribute the ophthalmological condition to a SARS-CoV-2 virus-associated endotheliopathy that can be prevented with timely protection of endothelial function with vitamins D and K2.