Man complains of foggy vision, photosensitivity

Examination showed bilateral scattered cotton wool spots and diffuse retinal hemorrhages, several of which had white centers.

Issue: March 25, 2013

ByGregory R. Blaha, MD, PhD

ByKirstin L. Tawse, MD

A 68-year-old man presented to our office complaining of intermittent foggy vision in both eyes, periodic spots in his vision and increased photosensitivity for the past 7 months. He first reported these symptoms to his primary care physician approximately 4 months before presentation and had a supposedly unremarkable ophthalmic exam at that time. He subsequently was referred to neurology for his visual symptoms and underwent CT and MRI of the brain, both of which were unremarkable.

The patient had no ocular history, and his medical history was significant for coronary artery disease, diabetes mellitus and hypertension. His medications included metformin, glipizide, lisinopril and metoprolol. He said that his diabetes was well-controlled and he never had evidence of diabetic retinopathy, although he did not know his recent HgA1c. He otherwise felt well and denied any recent illness or fatigue.

Examination

In our office, the patient’s vision was 20/20 in the right eye and 20/25 in the left. IOP was 18 mm Hg in the right eye and 26 mm Hg in the left. Anterior segment exam was notable for neovascularization of the iris at the pupillary margin in both eyes, left greater than right, and gonioscopy revealed neovascularization of the angle in both eyes with peripheral anterior synechiae in the left eye. Dilated fundus exam revealed scattered cotton wool spots in both eyes and diffuse retinal hemorrhages, several of which had white centers (Figure 1).

Fluorescein angiography revealed scattered microaneurysms and deep retinal hemorrhages bilaterally (Figure 2). Perfusion was relatively intact on peripheral sweeps, and no neovascularization was visualized.

Figure 1a. Diffuse bilateral retinal hemorrhages most abundant in the mid-periphery and scattered cotton wool spots.

Images: Tawse KL, Blaha GR

Figure 1b. Prominent white centers were noted in many of the hemorrhages.

Figure 2. Fluorescein angiography showed scattered microaneurysms and deep retinal hemorrhages without evidence of capillary nonperfusion or neovascularization.

The patient received intravitreal Avastin (bevacizumab, Genentech) on the day of presentation in the left eye and 4 days later in the right eye. He was also started on Cosopt drops (dorzolamide and timolol, Merck) in the left eye.

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What is your diagnosis?

White-centered retinal hemorrhages

Although the differential diagnosis for white-centered retinal hemorrhages includes diabetic retinopathy, a wide variety of alternative pathologies should be considered in this patient given his atypical history and exam findings.

A basic workup was ordered at his initial presentation, including normal carotid ultrasounds and a transthoracic echo. However, a complete blood count revealed a white blood cell count of 205,000. Upon receipt of the lab work, the patient was urgently referred to hematology/oncology where he underwent a bone marrow biopsy, confirming the diagnosis of chronic myelogenous leukemia (CML) in the chronic phase and the presence of the Philadelphia chromosome.

Discussion

Visual disturbances with white-centered retinal hemorrhages (Roth spots) on exam have been described previously as the initial presentation of CML. The retinal findings are thought to result from prolonged leukocytosis causing whole blood hyperviscosity and subsequent ischemic damage. Microaneurysm formation and retinal neovascularization have also been described in such cases. Although classically associated with septic chorioretinitis (bacterial endocarditis), the differential for white-centered retinal hemorrhages is extensive and includes diabetic retinopathy, severe anemia, multiple myeloma and leukemia. Histopathology of the white centers of the lesions has revealed fibrin and platelet aggregations thought to result from capillary rupture, extrusion of whole blood and subsequent initiation of the coagulation cascade. The presence of such findings on exam should prompt the clinician to maintain a high level of suspicion and rule out potentially life-threatening etiologies.

In our patient, the presence of pre-existing diabetes and possible underlying endothelial dysfunction may have acted synergistically with the effects of hyperviscosity to produce the retinal findings described. These processes may together have created a degree of ischemia sufficient to produce neovascularization of the iris and angle. However, the absence of concurrent capillary nonperfusion and retinal neovascularization is unusual.

Clinical course

The patient returned 2 weeks after his initial visit with subjectively improved vision, resolution of rubeosis and normalization of his eye pressure. He is scheduled to begin chemotherapy with dasatinib and will continue to be followed by ophthalmology throughout the course of his treatment.

References:

Duane TD, et al. Ophthalmology. 1980;87(1):66-69.
Kapadia R, et al. CMAJ. 2011;doi:10.1503/cmaj.100561.
Ling R, et al. Postgrad Med J. 1998;doi:10.1136/pgmj.74.876.581.
Mandić BD, et al. Coll Antropol. 2005;29 Suppl 1:141-143.
Rosenthal AR. Ophthalmology. 1983;90(8):899-905.
Tong P, et al. The Lancet. 2003;doi:10.1016/S0140-6736(03)12568-8.
Yannuzzi LA. The Retinal Atlas. 1st ed. Saunders; 2010:Chapter 8.

For more information:

Kirstin L. Tawse, MD, and Gregory R. Blaha, MD, PhD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.
Edited by Kavita Bhavsar, MD, and Michelle C. Liang, MD. They can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.