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| CASE REPORT |
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| Year : 2020 | Volume
: 9
| Issue : 12 | Page : 6273-6275 |
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Horner syndrome with transient visual impairment
Frank A Orlando, Maria Elisa Lupi
Department of Community Health and Family Medicine, University of Florida, Gainesville, FL, United States
| Date of Submission | 14-Jul-2020 |
| Date of Decision | 17-Sep-2020 |
| Date of Acceptance | 02-Oct-2020 |
| Date of Web Publication | 31-Dec-2020 |
Correspondence Address:
Dr. Frank A Orlando
U.F. Health Family Medicine – Springhill II, 4197 NW 86th Terrace, Gainesville, FL - 32606
United States
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jfmpc.jfmpc_1444_20
A 57-year-old female presented with headache, miosis, and ptosis diagnosed as Horner syndrome (HS). After delaying the recommended diagnostic imaging, she experienced transient, unilateral visual impairment in bright light. The patient was subsequently determined to have a spontaneous internal carotid artery dissection (ICAD) and secondary retinal ischemia with minimal cardiovascular risk factors and no history of preceding trauma. She wore dark glasses, received gabapentin for pain control, and was anticoagulated for a total of 4 months at which time the ICAD resolved despite a residual blepharoptosis and anisocoria.
Keywords: Amaurosis fugax, anisocoria, blepharoptosis, blindness, facial pain, headache, Horner's syndrome, Horner, Horners, internal carotid artery dissection, miosis, neck pain, ptosis, transient monocular vision loss
How to cite this article:
Orlando FA, Lupi ME. Horner syndrome with transient visual impairment. J Family Med Prim Care 2020;9:6273-5 |
| Introduction | |  |
HS is divided into pre-ganglionic (1st and 2nd order) and post-ganglionic (3rd order) based on lesion location, and primary care physicians must understand the various presentations to narrow the differential diagnosis and guide study selection. 1st and 2nd order lesions have the classic triad: unilateral miosis, ptosis, and anhidrosis. 3rd order lesions, presenting without anhidrosis, require expedited neuroimaging given the severe underlying etiologies: ICAD and cavernous sinus thrombosis. Other 3rd order lesions include neck/nasopharyngeal masses and benign causes like otitis media and cluster headaches. We present a case of acute HS with neck pain and visual changes requiring ICAD workup.
| Case Report | | |
A 57-year-old, non-smoking female with hyperlipidemia presented to clinic with complaints of a gradually worsening, intense right-sided head pressure and eye swelling for 1 week associated with malaise, sore throat, nasal congestion, and nausea. On exam, right-sided findings included blepharoptosis [Figure 1], conjunctival injection, nasal congestion, neck tenderness without lymphadenopathy or bruit, and a miotic pupil that failed to dilate under dim lighting [Figure 2]. The neurologic and cardiopulmonary exams were otherwise normal. | Figure 2: We reverse lit the eye in a totally dark room before an infrared light source to capture the patient's miosis. The reflection on her nose is a camera artifact that could not be eliminated
Click here to view |
She delayed STAT imaging insisting on a sinus infection and presented to the ER four days later dizzy, with tongue numbness, and seeing a “blue light” from her right eye. MRI/MRA and CTA showed right ICAD with high grade stenosis (at least 90%) entering the petrous bone [Figure 3]. She was admitted on enoxaparin and warfarin for anticoagulation, atorvastatin for stroke prevention, and gabapentin for pain. Exposure to bright light continued to cause a transient visual impairment, and she needed sunglasses indoors. Enoxaparin was stopped post-discharge after two therapeutic INRs, and warfarin was continued for 4 months when follow-up CTA showed ICAD resolution. She was then transitioned to ASA. Now 6 years later, the blepharoptosis and anisocoria have remained chronic. | Figure 3: Axial, T2-weighted, TIRM, fat-suppressed, dark-fluid MRI shows the right internal carotid artery dissection
Click here to view |
| Discussion | | |
HS is a rare neurologic condition, and this case highlights the importance of primary care physicians making a timely clinical diagnosis so expeditious neuroimaging and antithrombotic therapy can prevent stroke and death.[1],[2] When ipsilateral cranial or cervical pain accompany an acute HS, ICAD needs to be ruled out urgently.[3] In addition to causing HS by injuring the adjacent sympathetic plexus, an ICAD can compress local cranial nerves via pseudo-aneurysm 8-16% of the time, particularly IX-XII[4] and infrequently V and VII.[5] Cavernous sinus thrombosis also causes a 3rd order HS but with ophthalmoparesis, particularly cranial nerve VI with no other brainstem signs. An MRI cavernous sinus should be performed in these patients.
Even though patients with carotid artery dissection may have a subtle history of antecedent neck trauma, it can also occur spontaneously, especially in those with hypertension, smoking, on oral contraception, or with a connective tissue disorder.[6] Furthermore, the majority of carotid artery dissections are idiopathic.[6] Therefore, when acute HS presents with symptoms of ICAD but no recent trauma and a lower than expected cardiovascular risk, it is still critical for primary care physicians to follow through with STAT imaging, even if the patient is hesitant, hoping for a benign cause. In this case, MRI/MRA with fat suppression is the best initial screening test and is non-invasive, although digital subtraction angiography is still considered the gold standard.[3] Axial, T1-weighted, pre-contrast, fat-suppressed MRI is necessary to diagnose ICAD when the vessel lumen is not narrowed, making the dissection undetectable on CTA and MRA.[7] Importantly, pharmacological testing by an ophthalmologist is generally only useful for isolated anisocoria, otherwise it could delay diagnosis and treatment. Stroke is a major risk in ICAD patients for two weeks following HS onset.[8] Patients with high-grade ICA stenosis or ICA occlusion rarely present with an episodic vision impairment-related exclusively to light exposure, a symptom related to transient retinal ischemia (retinal claudication).[9],[10]
The differential diagnosis of headache and transient visual impairment also includes amaurosis fugax, a transient monocular vision loss (TMVL) or binocular blindness lasting seconds or minutes. TMVL has a diverse etiology, but some use amaurosis fugax to exclusively describe its most common vascular causes, retinal ischemia from ICA stenosis or embolism,[11] which have a 2-3% per year stroke risk.[11],[12] In such cases, the TMVL is classically described as descending over the field of vision “like a curtain or shade,” or less commonly ascending from below, but can also be shaded, black, or blurred vision.[10]
Retinal artery stroke is among a group of ocular arterial occlusive disorders that cause a permanent monocular blindness rather than HS. Ocular arterial occlusive disorders include central or branch retinal artery occlusion and ocular ischemic syndrome, each of which has a cerebral stroke risk that is significantly increased independent of internal carotid stenosis.[12] Some feel the terms “ocular migraine” and “retinal migraine” are oxymorons that should not be used in the medical vernacular because migraines causing binocular vision loss are a cortical process while vasospasm causing TMVL is a retinal process.[13],[14] Moreover, migraines classically produce positive visual phenomena such as scintillations rather than visual loss without scintillations, although TIA can also produce positive visual phenomena.[14] While migraine alone will not produce HS, cluster headaches can cause HS and have a similar presentation to ICA.[15]
We were granted institutional permission to do clinical research and obtained informed consent to take and publish this patient's photographs. The patient's miosis was difficult to reproduce in a photograph until we reverse lit the eye in a totally dark room before an infrared light source; then the anisocoria showed up dramatically [Figure 2]. Because of this process, this photo must be black and white, and there is a reflection on her nose from a camera artifact. This artifact could not be eliminated without significant cropping, even when we consulted an expert professional photographer.
Early recognition of HS remains challenging for primary care physicians in the outpatient setting. A clinical diagnosis of HS obligates quick neuroimaging to confirm the etiology followed by prompt treatment to prevent chronic stroke sequelae and death.
Acknowledgement
We would like to thank photographer Harry Rosa, Ariel Pomputius, Emma Djabali, Marie Fucci, Sandy Campbell, and Drs. Sonal Tuli, Eric Grieser, and Carlos Díez-Freire for their support.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]
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