17

QUESTION

HOW DO I WORK UP AND MANAGE A PATIENT WITH A WHITE-CENTERED RETINAL HEMORRHAGE?

Seema Garg, MD, PhD

Although originally thought to be pathognomonic for subacute bacterial endocarditis (SBE), a white-centered retinal hemorrhage (WCRH), often called a Roth spot,¹ is seen in a variety of systemic and ocular conditions.

While the pathologic basis of the white centers may be focal ischemia, inflammatory infiltrate, a colony of infectious organisms, or accumulation of neoplastic cells, most often it is a fibrin-platelet plug.² In the latter case, the plug forms in response to acute retinal capillary rupture. While the myriad causes of WCRH may seem unrelated, most of them show a common predisposition to capillary extravasation. The WCRH is actually a manifestation of the capillary bleeding and subsequent repair process.

One way to think about the differential of WCRH is to determine the mechanism of capillary rupture.^3,4 For instance, thrombocytopenia (resulting from low-grade disseminated intravascular coagulopathy from SBE or leukemia) can cause capillary bleeding. Furthermore, ischemia from other causes (eg, anemia, anoxia, carbon monoxide poisoning, prolonged intubation) can cause damage to the retinal capillary endothelium, and resultant bleeding. Increased capillary fragility (seen in diseases such as hypertension, preeclampsia, and diabetes) can also cause capillary rupture. Elevated venous pressure (seen in intracranial hemorrhage, birth trauma, and nonaccidental injury in infants) can also be the cause of capillary rupture. Thus, keeping in mind the mechanism of capillary rupture helps to understand the diverse causes of the WCRH.

I have divided diseases associated with WCRH into disorders of the hematopoietic system, immune-mediated collagen vascular disease, trauma, disseminated fungal or bacterial infection, ischemia (including ocular ischemic syndrome [OIS] and preeclampsia), and vascular malformations. Less often, WCRH can be seen in patients with diabetes mellitus but usually only if there is an acute change in blood glucose.

If a patient has a disorder of the hematopoietic system, such as anemia,5 leukemia,⁶ or multiple myeloma,⁷ a thorough history may reveal that the patient has a preexisting acute or chronic hematologic disorder. If the patient is anemic, he or she may have symptoms, such as fatigue or shortness of breath on exertion. The patient may have signs of thrombocytopenia, such as petechiae. In addition to WCRH, the ocular exam may reveal a hypopyon, disc edema, retinal vascular sheathing, and cotton-wool spots in cases of leukemia. A laboratory work-up should consist of a complete blood count, which would be abnormal in anemia and leukemia (leukocytosis and thrombocytopenia). At this point, it would be important to consult with a hematologist/oncologist, who may note immature abnormal white blood cells in the peripheral smear as well as on a bone marrow biopsy. Figure 17-1 shows fundus photos of a completely asymptomatic 47-year-old healthy male in which WCRH hemorrhages were noted incidentally. After a thorough history revealed no preexisting conditions and a negative review of systems, a complete blood count showed leukocytosis (white blood cell count = 108,000/mcL, normal range 3500 to 10,000/mcL). The patient was immediately referred to the hematologist/oncologist who performed a bone marrow biopsy and ultimately diagnosed chronic myelogenous leukemia (CML). The importance of the role of ophthalmologist in the diagnosis of asymptomatic individuals cannot be underestimated. In this case, the presence of WCRH and a high index of suspicion by the ophthalmologist led to the appropriate work-up and diagnosis of leukemia.

If a patient has an immune-mediated collagen vascular disease, such as polyarteritis nodosa, a history may reveal muscle and joint pain, excessive fatigue, and abdominal pain. In addition to WCRH, the ocular exam may show uveitis, scleritis, and retinal vascular sheathing. Laboratory work-up for immune-mediated collagen vascular diseases would include an erythrocyte sedimentation rate, C-reactive protein, antinuclear antibodies, rheumatoid factor, antineutrophil cytoplasmic antibodies, and complement factors, and, if abnormal, consultation with a rheumatologist for further work up and management.

Disseminated fungal, viral, or bacterial infections can cause WCRH, as in cases of fungemia, syphilis, HIV, or ocular toxoplasmosis.⁸ The exam may show cell or flare in the anterior chamber, posterior uveitis, or even panuveitis. The work-up should include an aqueous or vitreous tap, a serum rapid plasma reagin/fluorescent treponemal antibody-absorption to rule out syphilis and a serum HIV test. With a history of a recent fever and new heart murmur, consider serial blood cultures as well as a transesophageal echocardiogram to evaluate for valve vegetations in cases of suspected SBE.

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