Ocular and Cerebral Emboli From an Atrial Myxoma

Emboli from an atrial myxoma resulted in asymptomatic segmental retinal and choroidal arterial hyperfluorescent lesions on retinal angiography, mimicking an arteritis. The retinal lesions disappeared after removal of the atrial myxoma. Endothelial trauma by embolic material appears to be the mechanism of the angiographic findings.

Photo Essay Section Editor: Timothy J. McCulley, MD Ocular and Cerebral Emboli From an Atrial Myxoma Anne Kuonen, MD, François-Xavier Borruat, MD, PD, MER FIG. 1. Manifestations of emboli from atrial myxoma. A. Axial T2 MRI reveals ischemic lesions in the left caudate nucleus. B. The left fundus shows an area of paramacular nerve fiber layer whitening (arrows) and 2 cotton-wool spots (arrowheads). C. Fluorescein angiography of the left eye reveals areas of staining of the retinal arteriolar walls (arrows), which do not correlate with the fundus appearance in B. D. Indocyanine green angiography reveals several hyperfluorescent choroidal lesions (arrows). Abstract: Emboli from an atrial myxoma resulted in asymptomatic segmental retinal and choroidal arterial hyperfluorescent lesions on retinal angiography, mimicking an arteritis. The retinal lesions disappeared after removal of Neuro-Ophthalmology Unit, Hôpital Ophtalmique Jules Gonin, University of Lausanne, Lausanne, Switzerland. The authors report no conflicts of interest. Address correspondence to François-Xavier Borruat, MD, PD, MER, Hôpital Ophtalmique Jules-Gonin, Avenue de France 15, 1004 Lausanne, Switzerland; E-mail: [e-mail] Kuonen and Borruat: J Neuro-Ophthalmol 2017; 37: 309-310 the atrial myxoma. Endothelial trauma by embolic material appears to be the mechanism of the angiographic findings. Journal of Neuro-Ophthalmology 2017;37:309-310 doi: 10.1097/WNO.0000000000000453 © 2016 by North American Neuro-Ophthalmology Society A 30-year-old woman developed transient aphasia and right upper limb weakness. Brain MRI (Fig. 1A) and cerebral angiography showed diffuse lesions, suggesting 309 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay cerebral vasculitis. Although she had no visual complaints, an inferotemporal visual field defect was incidentally found in the left eye, as well as an area of left paramacular nerve fiber layer whitening without visible emboli (Fig. 1B). Fluorescein angiography disclosed numerous segmental hyperfluorescent lesions of retinal arterioles without leakage (Fig. 1C), and several choroidal hyperfluorescent areas were detected with indocyanine green angiography (Fig. 1D), all in the left eye. Extensive investigations revealed the presence of a left atrial myxoma by echocardiography. The patient underwent uneventful surgical resection of the atrial myxoma and, without any adjunctive therapy, complete regression of retinal lesions ensued. Retinal vasculitis is best characterized on retinal fluorescein angiography with early staining of the vessel walls followed by leakage of dye (1). In the context of neurological symptoms and neuroimaging suggestive of cerebral vasculitis, our patient exhibited numerous retinal arterial and choroidal hyperfluorescent lesions without late leakage in the left eye. As no retinal emboli were visible on fundus examination and neither regional arterial filling delay nor restricted areas of hypofluorescence were detected on fluorescein angiography, the patient was initially suspected of having atypical retinal vasculitis. Atrial myxoma was diagnosed and removed surgically, and the retinal lesions completely resolved. Atrial myxoma can cause retinal arterial lesions through 2 mechanisms. First, embolized tumoral fragments can induce direct traumatic endotheliopathy of retinal and choroidal arteries, similar to cholesterol emboli (2,3). Second, myxomatous cells have been demonstrated to secrete IL-6 and to stimulate antibody production leading to local inflammation (4). Subsequent deposition of immune complex or cross-linking reaction of antibody to tumor cell antigen with endothelial cell antigen can result in an ocular and cerebral leukocytoclastic vasculitis (5). In our patient, there was no dye leakage on retinal angiography, but only staining of the arterial walls. Therefore, we favored the hypothesis of direct endothelial trauma by multiple emboli from the atrial myxoma. 310 Our case initially was puzzling, as several vascular lesions were imaged on both the MRI and cerebral angiography, raising the possibility of a vasculitis. However, the angiographic presentation of the retinal arterial lesions was not typical of vasculitic process but was suggestive of an endotheliopathy. Neuro-ophthalmic manifestations of atrial myxomas are rare. In most cases, loss of vision results from an embolic event to the retinal circulation. Our report illustrates that multiple asymptomatic retinal emboli can also be caused by atrial myxomas. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: François-Xavier Borruat and Anne Kuonen; b. Acquisition of data: François-Xavier Borruat; c. Analysis and interpretation of data: François-Xavier Borruat and Anne Kuonen; Category 2: a. Drafting the manuscript: FrançoisXavier Borruat and Anne Kuonen; b. Revising it for intellectual content: François-Xavier Borruat and Anne Kuonen; Category 3: a. Final approval of the completed manuscript: François-Xavier Borruat and Anne Kuonen. REFERENCES 1. Leder HA, Campbell JP, Sepah YJ, Gan T, Dunn JP, Hatef E, Cho B, Ibrahim M, Bittencourt M, Channa R, Do DV, Nguyen QD. Ultra-wide-field retinal imaging in the management of noninfectious retinal vasculitis. J Ophthalmic Inflamm Infect. 2013;3:30. 2. Arruga J, Sanders MD. Ophthalmologic findings in 70 patients with evidence of retinal embolism. Ophthalmology. 1982;89:1336-1347. 3. Herbst M, Wattjes MP, Urbach H, Inhetvin-Hutter C, Becker D, Klockgether T, Hartmann A. Cerebral embolism from left atrial myxoma leading to cerebral and retinal aneurysms: a case report. AJNR Am J Neuroradiol. 2005;26:666-669. 4. Kishimto T, Hirano T, Kikutani H. Regulation of human B cell differentiation: molecular structure and immunological functions of human B cell differentiation factor (BSF2). Prog Immunol. 1986;6:357-367. 5. Podjasek JO, Wetter DA, Piettelkow MR, Wada DA. Cutaneous small-vessel vasculitis associated with solid organ malignancies: the Mayo Clinic experience, 1996 to 2009. J Am Acad Dermatol. 2012;66:e55-65. Kuonen and Borruat: J Neuro-Ophthalmol 2017; 37: 309-310 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.