Arteritic Orbital Ischemia Producing Afferent and Efferent Pupillary Defects

A 67-year-old woman presented with acute loss of vision to no light perception (NLP), a right afferent pupillary defect, and anisocoria with a nonreactive and dilated pupil in the right eye. Fundus examination showed pallid optic disc edema and a central retinal artery occlusion (CRAO) in the right eye. A temporal artery biopsy showed giant cell arteritis (GCA). Orbital involvement in GCA has been reported previously. However the combination of an afferent and efferent pupillary defect, NLP vision, pallid disc edema, and a CRAO in an elderly patient is likely a unique clinical combination that should strongly suggest GCA. Clinicians should be aware of the myriad presentations of GCA, including orbital ischemia.

Photo Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Arteritic Orbital Ischemia Producing Afferent and Efferent Pupillary Defects Mariam Hussain, BA, Ashwini Kini, MD, Bayan Al Othman, MD, Claudia Prospero Ponce, MD, Helen Li, MD, Andrew G. Lee, MD FIG. 1. Right eye fundus photograph demonstrating a cherry red spot. This is indicative of central retinal artery occlusion. There is also pallid disc edema, confirming the anterior ischemic arteritic process. Abstract: A 67-year-old woman presented with acute loss of vision to no light perception (NLP), a right afferent pupillary defect, and anisocoria with a nonreactive and dilated pupil in the right eye. Fundus examination showed pallid optic disc edema and a central retinal artery occlusion (CRAO) in the right eye. A temporal artery biopsy showed giant cell arteritis (GCA). Orbital involvement in GCA has been reported previously. However the combination of an afferent Texas A&M College of Medicine (MH, AGL), Bryan, Texas; Department of Ophthalmology (AK, BAO, CPP, HL, AGL), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; Department of Pathology and Genomic Medicine, Ocular Pathology (CPP), Houston Methodist Hospital, Houston, Texas; Community Retina Group (HL), Houston, Texas; The Houston Methodist Research Institute (AGL), Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery (AGL), Weill Cornell Medicine, New York, New York; Department of Ophthalmology (AGL), University of Texas Medical Branch, Galveston, Texas; University of Texas MD Anderson Cancer Center (AGL), Houston, Texas; Department of Ophthalmology (AGL), The University of Iowa Hospitals and Clinics, Iowa City, Iowa.; and Department of Ophthalmology (JMF), Portland, Oregon; Department of Ophthalmology (SLP), UCLA, Los Angeles, California; and Department of Ophthalmology and Visual Sciences (GPVS), Washington University in St. Louis, St. Louis, Missouri. The authors report no conflicts of interest. Address correspondence to Andrew G. Lee, MD, Blanton Eye Institute, Houston Methodist Hospital, 6560 Fannin Street Suite 450, Houston, TX 77030; E-mail: [e-mail] 530 and efferent pupillary defect, NLP vision, pallid disc edema, and a CRAO in an elderly patient is likely a unique clinical combination that should strongly suggest GCA. Clinicians should be aware of the myriad presentations of GCA, including orbital ischemia. Journal of Neuro-Ophthalmology 2020;40:530–532 doi: 10.1097/WNO.0000000000000838 © 2019 by North American Neuro-Ophthalmology Society A 67-year-old woman initially presented with acute onset loss of vision in her right eye. She had a moderate headache localized over her right temple. She had seen a dentist for symptoms of jaw pain that worsened after 8–10 bites of food. She was given a diagnosis of “gingivitis” and prescribed antibiotics and a short course of low-dose oral steroids. Four weeks later, the patient experienced acute vision loss. Her medical history, surgical history, family history, and social history were noncontributory. On examination, the visual acuity was no light perception (NLP) in the right eye and 20/40 in the left eye. The pupils were equal; the right pupil was amaurotic with a right afferent pupillary defect (APD). Extraocular Hussain et al: J Neuro-Ophthalmol 2020; 40: 530-532 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay FIG. 2. Right eye fluorescein angiogram. There is delayed retinal and choroidal filing, with the choroidal filling more delayed in the nasal than temporal posterior pole. There is also hypofluoresence of optic disc and macula from severe edema of optic disc and macula. FIG. 3. Pupil photograph. Right eye mild ptosis with anisocoria worse in bright light; the right eye pupil is larger than the left. motility and anterior segments were normal. Fundus examination of the right eye showed pallid disc edema and a cherry red spot consistent with anterior ischemic optic neuropathy (AION) and a central retinal artery occlusion (CRAO) (Fig. 1). The erythrocyte sedimentation rate, C-reactive protein, and platelet count were within normal range (but the patient had been treated previously with low dose of oral corticosteroids). Automated perimetry (Humphrey visual field 24-2) showed a superior and inferior arcuate defect in the left eye, but the fundus examination was normal on the left. She was empirically started on IV methylprednisolone (IVMP) 1,000 mg. Fluorescein angiogram showed delayed retinal and choroidal filing in the right eye (Fig. 2). On Day 2 of the hospital admission, the patient developed ptosis of the right eye and anisocoria that was worse in bright light (Fig. 3). She also had mild restriction of adduction, elevation, and depression of the right eye. A computed tomography angiogram was negative for aneurysm but failed to visualize the right ophthalmic artery. After 2 more days of intravenous steroids, the ptosis, anisocoria, and motility deficits for the right eye completely resolved; however, the vision remained NLP in the right eye. A temporal artery biopsy showed diffuse lymphocytic inflammation at the level of the intima, media, adventitia, and in the perivascular region surrounding the vasavasorum (Fig. 4). Although orbital presentations of giant cell arteritis (GCA) are well known, the combination of afferent and efferent pupillary defects, visual loss to NLP, AION, CRAO, and choroidal filling defects in an elderly patient is highly likely to be due to GCA. FIG. 4. Temporal artery biopsy, H&E stain. A. Low (·4) magnification showing a full-thickness lymphohistiocytic inflammation including the adventitia (arrow). There is irregular intimal hyperplasia (double head arrow) and significant narrowing of the lumen (asterisk). B. Low (·10) magnification revealing perivascular inflammation extended to the vasa-vasorum. C. Low (·10) magnification. Notice the preserved elastic lamina (multiarrows) and the multinucleated giant cells. Active inflammation at the level of the elastic lamina is demonstrated on higher (·20) magnification (D). Hussain et al: J Neuro-Ophthalmol 2020; 40: 530-532 531 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay GCA commonly involves the short posterior ciliary arteries; but, less commonly, the central retinal artery can be involved (1,2). A common optic nerve appearance in an AION is pallid disc edema, reflecting the extensive ischemic assault on the optic nerve head caused by occlusion of the vessel lumen secondary to the vessel wall thickening (3). In a study of 123 eyes with biopsy-confirmed GCA, 70.7% had AION, 6.5% had CRAO, and 4.9% had both AION and CRAO (4). Chen et al (5) conducted another study with 245 patients, 1 of whom had CRAO of the right eye and AION of the left eye. In addition, this patient had both afferent and efferent pathway involvement, manifesting as an APD and third nerve palsy. GCA infrequently presents as a third nerve palsy; when the third nerve is involved, the pupils are usually equal. However, cases of pupillary involvement with third nerve palsy in GCA have been reported (6–11). The involvement of both the short posterior ciliary arteries and central retinal artery combined with an efferent pupillary defect due to third nerve palsy is of clinical interest. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: M. Hussain; b. Acquisition of data: A. Kini, C. P. Ponce, and H. Li; c. Analysis and interpretation of data: A. Kini. Category 2: a. Drafting the manuscript: M. Hussain; b. Revising it for intellectual content: B. Al Othman and A. G. Lee. Category 3: a. Final approval of the completed manuscript: A. 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