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. Author manuscript; available in PMC: 2026 Aug 13.
Published in final edited form as: Ophthalmic Surg Lasers Imaging Retina. 2018 Oct 1;49(10):e157–e160. doi: 10.3928/23258160-20181002-21

Swept-Source Optical Coherence Tomography Angiography of an Amalric Choroidal Infarction in a Rare Presentation of Giant Cell Arteritis With Bilateral Corneal Edema

Ann Q Tran 1, Nicolas A Yannuzzi 1, Elie H Motulsky 1, Xiao Yi Zhou 1, Anat Galor 1, Sander R Dubovy 1, Philip J Rosenfeld 1, Byron L Lam 1
PMCID: PMC13461306  NIHMSID: NIHMS2199300  PMID: 30395679

Abstract

A 73-year-old woman with 2 weeks of progressive painless vision loss was found to have bilateral corneal edema, jaw claudication, and temporal headache. Multimodal imaging revealed an Amalric choroidal infarct in the left eye visualized by widefield indocyanine green angiography and swept-source optical coherence tomography angiography (SS-OCTA). Prompt intravenous corticosteroid treatment resulted in 20/20 vision, and giant cell arteritis (GCA) was confirmed by a temporal artery biopsy. The case underscores the use of widefield SS-OCTA as a non-invasive test to aid in the diagnosis of GCA, as well as bilateral cornea edema as a rare presentation of GCA.

INTRODUCTION

Giant cell arteritis (GCA) is the most common systemic granulomatous vasculitis in patients older than 50 years of age.1 Vision loss results from anterior ischemic optic neuropathy (88.0% to 92.9%), cilioretinal artery (10.0% to 21.8%) or central retinal artery occlusion (4.1% to 14.1%), posterior ischemic optic neuropathy (7.1%), and rarely anterior segment ischemia.2,3

Optical coherence tomography angiography (OCTA) allows for noninvasive three-dimensional reconstruction of the chorioretinal vascular architecture, an alternative to traditional dye-based angiography. Acute choroidal ischemia with an unusual triangular area of whitening was first described by Amalric in 1971 after a posterior ciliary artery occlusion.4 Spectral-domain OCTA (SD-OCTA) has been previously reported to identify Amalric choroidal infarcts.5 Swept-source OCTA (SS-OCTA) utilizes a longer central wavelength, improving visualization of the deep retinal and choroidal vasculature.6

To our knowledge, this is the first report of the use of widefield SS-OCTA in identifying an Amalric choroidal infarct in an uncommon presentation of GCA with bilateral corneal edema, anterior segment ischemia and choroidal infarction.

CASE REPORT

A 73-year-old woman presented to her primary care doctor with worsening jaw pain, initially diagnosed with temporomandibular joint syndrome. Two months later, she developed painless, bilateral vision loss associated with photophobia. Medical history included hypertension and hyperlipidemia. Best-corrected visual acuity was hand motion and 20/60 and intraocular pressures were 12 mm Hg and 6 mm Hg in the right and left eyes, respectively. Review of systems was notable for weight loss, fatigue, neck pain, and scalp tenderness. Slit-lamp examination disclosed bilateral corneal edema, striate keratopathy, and anterior chamber flare (Figures 1A-1D). Temporal artery pulses were diminished on palpation.

Figure 1.

Figure 1.

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Bilateral corneal edema in a 73-year-old woman with giant cell arteritis. (A) Slit-lamp photograph of the right eye and (B) left eye discloses mild corneal edema. (C) Cross-section of the right eye discloses prominent corneal edema with (D) striate keratopathy.

Posterior segment examination of the right eye was limited from corneal edema. The left optic nerve was cupless with minimal edema. Widefield color fundus imaging showed a triangular shape temporal retinal whitening (Figure 2A). Fluorescein angiography and indocyanine green angiography showed early hypofluorescence corresponding to an area of decreased choroidal perfusion with late hyperfluorescence around the borders (Figures 2B and 2C). En face 12 mm × 12 mm SS-OCTA (Plex-Elite 9000; Carl Zeiss Meditec, Dublin, CA) montaged images revealed preservation of retinal flow using a total retina slab (Figure 2D) and an Amalric choroidal infarction with decreased choroidal flow using a choroidal slab with projection artifact removal (Figure 2E).

Figure 2.

Figure 2.

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Conventional dye-based and optical coherence tomography angiography reveal a wedge-shaped Amalric choroidal infarct. (A) Widefield color fundus photograph of the left eye shows temporal chorioretinal whitening. (B) Early phase fluorescein angiogram shows delayed choroidal filling. (C) Indocyanine green angiogram shows a triangular area of hypocyanescence. (D) Swept-source optical coherence tomography angiogram 12 mm × 12 mm en face montaged image using a total retina slab shows a preserved perfusion of the retina. The total retina slab consists of an upper boundary that follows the internal limiting membrane and a lower boundary that follows the retinal pigment epithelium. At the level of the choroid (E) the 12 mm × 12mm en face montaged image shows a triangular temporal flow deficit in the choroid that includes the choriocapillaris and corresponds to the Amalric choroidal infarct region depicted by dye-based angiograms (B, C). The choroidal slab consists of an upper and a lower boundary that both follow the Bruch’s membrane with a 100 μm thickness slab.

Laboratory testing revealed a normal erythrocyte sedimentation rate for adjusted age and gender (32 mm/hr) and a mildly elevated C-reactive protein (1.6 mg/dL). The patient received 3 days of intravenous 1 gm methylprednisolone daily followed by 80 mg of oral prednisone daily. Temporal artery biopsy done 4 days after starting the steroids confirmed the diagnosis of GCA with a narrowed lumen, multinucleated giant cells, transmural granulomatous inflammation (Figure 3A), and fragmented internal elastic lamina (Figure 3B) on histopathology. After 6 months, the patient — who was now on 20 mg prednisone daily — had light perception in the right eye and was 20/20 in the left eye.

Figure 3.

Figure 3.

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Histopathological examination of the temporal artery biopsy at 200x magnification. (A) Hematoxylin and eosin stain reveals a narrowed atretic lumen, a transmural granulomatous inflammation, and multinucleated giant cells (*) consistent with giant cell arteritis. (B) Verhoeff’s elastic stain discloses a discontinuous and fragmented internal elastic lamina (arrow).

DISCUSSION

Our case highlights the use of widefield SS-OCTA as a noninvasive test to aid in the diagnosis of GCA, as well as a rare presentation of bilateral corneal edema in GCA. Anterior segment ischemia likely resulted from decreased perfusion of the anterior ciliary artery or long posterior ciliary artery, both branches of the ophthalmic artery, supplying two-thirds and one-third of the blood stream of the anterior segment, respectively. Corneal edema from GCA is hypothesized to occur due to decreased oxygen levels in the tear film, lid vasculature and aqueous humor, leading to increased production of lactic acid from anaerobic respiration, which serves as an osmotic solute within the cornea.7 Our patient had no evidence of viral corneal endotheliitis, acute hydrops keratoconus, contact lens intolerance or Fuchs dystrophies. Fortunately, with prompt diagnosis and treatment of GCA, our patient was able to recuperate 20/20 vision in the eye with the Amalric choroidal infarction, whereas other cases of bilateral ocular ischemia have resulted in severe visual loss up to no light perception despite the use of intravenous corticosteroids.8 This patient also had decreased IOP in the left eye likely from ciliary body ischemia, leading to underappreciated hypotony.

Multimodal imaging allowed us to detect a triangular Amalric choroidal infarction, which is a rarely reported manifestation in GCA.9 Amalric choroidal infarcts occur in a variety of other systemic or isolated ocular diseases outside of GCA including carotid dissection, retrobulbar hemorrhage, malignant hypertension, lupus nephropathy, Raynaud’s disease, polyarteritis nodosa, sickle cell disease, and complications of panretinal photocoagulation.5 Amalric choroidal infarcts may also be accompanied by other signs of ocular ischemia and outer retinal ischemia with an ellipsoid disruption.10 Patients older than 50 years of age presenting with choroidal ischemia should be investigated for GCA, and fluorescein angiography has been thought to be a diagnostic adjunct in identifying posterior segment ischemia in patients with GCA.5,11 Although SD-OCTA has described Amalric choroidal infarct in GCA showing reduced flow through the choriocapillaris at the triangular zone of ischemia,4 here we present the first case using widefield SS-OCTA montaged images to illustrate an Amalric choroidal infarction.

In conclusion, widefield SS-OCTA imaging in GCA demonstrated an Amalric choroidal infarction. The findings on SS-OCTA and widefield dye-based angiography were comparable, except that SS-OCTA images were obtained noninvasively. Given GCA can have a variety of presentations and requires prompt diagnosis and management to prevent irreversible vision loss, the ability to noninvasively assess vascular-occlusive changes can aid the clinician to better manage the patient.

Acknowledgments

Research support was provided by the NIH Center Core Grant P30EY014801 and Carl Zeiss Meditec.

Dr. Galor receives financial support from the Department of Veterans Affairs, Veterans Health Administration, Office of Research and Development, Clinical Sciences Research EPID-006-15S, R01EY026174, NIH Center Core Grant P30EY014801, and Research to Prevent Blindness Unrestricted Grant. The remaining authors report no relevant financial disclosures.

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