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Hematologic Cancer

Optic Nerve Edema, Venous Stasis Retinopathy, and Peripheral Retinal Whitening in a Teenage Girl

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.

1 Credit CME

JAMA Ophthalmology

Published Online: August 6, 2020

JAMA Ophthalmology Clinical Challenge

Article Information and Disclosures

Suzanne M. Michalak, MD¹;

Landon C. Meekins, MD¹;

Mohsin H. Ali, MD²

Author Affiliations

¹Duke Eye Center, Department of Ophthalmology, Duke University, Durham, North Carolina
²The Retina Group of Washington, Sterling, Virginia

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A teenage girl newly diagnosed with idiopathic intracranial hypertension by her neurologist presented with 2 weeks of headaches, dizziness, and blurred vision. Her best-corrected visual acuity was 20/25 OD and 20/20 OS. There was no afferent pupillary defect, her Ishihara color plate findings were 10/10, and extraocular movements were intact in both eyes. An anterior slitlamp examination had unremarkable findings. A fundus examination showed hemorrhagic optic disc edema, peripapillary and subfoveal subretinal fluid, tortuous and dilated retinal vasculature, many intraretinal hemorrhages in all 4 quadrants, and far peripheral temporal inner retinal whitening in both eyes (Figure 1A). Optical coherence tomography demonstrated peripapillary and subfoveal subretinal fluid, intraretinal fluid, and swollen optic nerve heads in both eyes (Figure 1B). A fluorescein angiogram showed markedly delayed venous filling (1 minute) in all quadrants and far peripheral capillary nonperfusion in both eyes.

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Hyperviscosity syndrome associated with lymphoplasmacytic lymphoma with central nervous system involvement

C. Advise urgent plasmapheresis in consultation with oncology

Monoclonal and polyclonal paraproteinemias, such as lymphoplasmacytic lymphoma and multiple myeloma, are frequently associated with hyperviscosity syndrome (HVS).¹^- 4 This most commonly presents with mucosal bleeding, visual disturbances, and neurological symptoms.⁵ Up to 48% of patients with Waldenström macroglobulinemia, a type of lymphoplasmacytic lymphoma, show signs of HVS on fundus examination, including peripheral and central dot-blot hemorrhages, dilated retinal veins, tortuous retinal vasculature, and optic disc edema.² Hyperviscosity syndrome is an oncologic emergency, and prompt identification and treatment is essential.¹^,6^,7 Because patients with HVS often present with visual symptoms, ophthalmologists should keep HVS on the differential diagnosis for bilateral optic nerve edema with vascular congestion and tortuosity. If HVS is identified in time, plasmapheresis can reverse associated retinopathy and prevent other complications (eg, cerebral stroke).⁸

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Article Information

Corresponding Author: Suzanne M. Michalak, MD, Duke Eye Center, Department of Ophthalmology, Duke University, 2351 Erwin Rd, Durham, NC 27705 (suzanne.michalak@duke.edu).

Published Online: August 6, 2020. doi:10.1001/jamaophthalmol.2020.1398

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References

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Menke MN , Feke GT , McMeel JW , Branagan A , Hunter Z , Treon SP . Hyperviscosity-related retinopathy in Waldenstrom macroglobulinemia. Arch Ophthalmol. 2006;124(11):1601-1606. doi:10.1001/archopht.124.11.1601 PubMed Google Scholar Crossref

Feman SS , Stein RS . Waldenstrom’s macroglobulinemia, a hyperviscosity manifestation of venous stasis retinopathy. Int Ophthalmol. 1981;4(1-2):107-112. doi:10.1007/BF00139584 PubMed Google Scholar Crossref

Omoti AE , Omoti CE . Ophthalmic manifestations of multiple myeloma. West Afr J Med. 2007;26(4):265-268.PubMed Google Scholar

Fahey JL , Barth WF , Solomon A . Serum hyperviscosity syndrome. JAMA. 1965;192:464-467. doi:10.1001/jama.1965.03080190030008 PubMed Google Scholar Crossref

Castillo JJ , Treon SP . Initial evaluation of the patient with Waldenström macroglobulinemia. Hematol Oncol Clin North Am. 2018;32(5):811-820. doi:10.1016/j.hoc.2018.05.008 PubMed Google Scholar Crossref

Kwaan HC . Hyperviscosity in plasma cell dyscrasias. Clin Hemorheol Microcirc. 2013;55(1):75-83. doi:10.3233/CH-131691 PubMed Google Scholar Crossref

Menke MN , Feke GT , McMeel JW , Treon SP . Effect of plasmapheresis on hyperviscosity-related retinopathy and retinal hemodynamics in patients with Waldenstrom’s macroglobulinemia. Invest Ophthalmol Vis Sci. 2008;49(3):1157-1160. doi:10.1167/iovs.07-1254 PubMed Google Scholar Crossref

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.