History of present illness
A 74-year-old woman presented with a progressive decline in vision in both eyes over the past 2 weeks. She denies presence of or any changes in floaters, flashes of lights, curtain defect, eye pain, or any recent illnesses. She did not have any history of ocular diseases or surgeries.
Ocular examination findings
Visual acuities were 20/50 in the right eye and 20/30 in the left eye. Intraocular pressures were 13 mm Hg and 14 mm Hg in right and left eyes, respectively. Slit lamp examination showed moderate cataracts (2+ nuclear sclerosis, 3+ cortical) in both eyes. Fundus examination (limited by cataracts) revealed cotton wool spots (CWS) nasal to the optic disc and on the vascular arcades bilaterally. In the right eye, elevation of the macula, flame-shaped hemorrhages, and CWS were also documented ( Fig. 62.1 A, B).
Imaging
At presentation, wide-angle fundus photography showed CWS nasal to the optic disc and around the superior and inferior vascular arcades in both eyes. There were flame-shaped hemorrhages, as well as a preretinal hemorrhage in the superonasal periphery of the right eye. Optical coherence tomography (OCT) of the right eye showed serous retinal detachment (RD) in the macula, and the left eye demonstrated focal thickening of retinal nerve fiber layer temporal to the optic disc corresponding to a large CWS with no evidence of subretinal fluid ( Fig. 62.1 C, D). Fluorescein angiography (FA) showed hypo- and hyperfluorescent retinal pigment epithelium (RPE) changes (suggestive of leopard spot retinopathy) at the posterior pole, which is more prominent in the superior macula, along with diffuse, mild peripheral leakage from the capillaries in the right eye. However, there was no vascular leakage in the macula to explain subretinal fluid ( Fig. 62.2 ). A similar pattern of leakage was seen in the left eye. B-scan of the right eye was unremarkable except for a slightly thickened choroid.
Questions to ask
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Does the patient have any history of diabetes mellitus or hypertension? CWS and flame-shaped hemorrhages can be seen in diabetic retinopathy or hypertensive retinopathy.
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The patient had a history of hypertension that was well controlled with amlodipine, and most recent blood pressures were around 130/80 mm Hg.
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She also had prediabetes with a most recent hemoglobin A1C of 6.0%.
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Does the patient have any travel history or potential exposures to infectious agents? Is there any history of autoimmune diseases? Both infectious and inflammatory causes can be associated with serous RD.
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The patient denied any recent travel or exposure to pets or animals.
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She did have a self-reported history of rheumatoid arthritis for which she was not on any medication and was asymptomatic.
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What medications does the patient currently take? For instance, steroids are known to be a risk factor for central serous chorioretinopathy.
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The patient only takes amlodipine for hypertension and did not have any history of steroid use or other medications.
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The patient did state she was experiencing recent stressors as the sole breadwinner for her family.
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Assessment
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This is the case of a 74-year-old woman with well-controlled hypertension, prediabetes, and no ocular history who presented with unilateral serous RD in the right eye.
Differential diagnosis
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Vascular
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Exudative age-related macular degeneration
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Polypoidal choroidal vasculopathy
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Hypertensive retinopathy
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Diabetic retinopathy
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Inflammatory or infectious
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Vogt-Koyanagi-Harada disease
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Posterior scleritis
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Inflammatory diseases of the choroid (white dot syndromes)
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Ocular histoplasmosis
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Lupus choroidopathy
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Neoplastic
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Choroidal melanoma
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Choroidal metastases from leukemia or lymphoma
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Other causes
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Central serous chorioretinopathy
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Optic disc pit
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Working diagnosis
Because of high suspicion for systemic causes of the patient’s serous RD, blood work was done immediately, which included a complete blood cell count (CBC), comprehensive metabolic panel (CMP), inflammatory markers (erythrocyte sedimentation rate [ESR], C-reactive protein [CRP], rheumatoid factor, antinuclear antibodies (ANA), antineutrophil cytoplasmic antibodies, angiotensin-converting enzyme, and lysozyme), and infectious studies (QuantiFERON, treponemal studies, and Lyme assay). CBC showed 200 white blood cells/μL with large number of blasts. The patient was diagnosed with B-cell acute lymphoblastic leukemia (ALL) with central nervous system (CNS) involvement. Thus her serous RD was attributed to the initial presentation of acute leukemia, and CWS and retinal hemorrhages were the manifestations of leukemic retinopathy.
Multimodal testing and results
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Fundus photographs
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Retinopathy in leukemia can be a result of direct neoplastic infiltration or secondary causes such as anemia, thrombocytopenia, or hyperviscosity. Hemorrhages can occur at all levels of the retina and are usually located in the posterior pole. White-centered hemorrhages (known as Roth spots) are particularly suggestive of a systemic disorder. Other findings seen on fundus photography may include venous dilation and tortuosity, microaneurysms, or CWS.
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OCT
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OCT of the right eye showed serous RD in the macula. OCT can easily detect serous RD even if the subretinal fluid may not be apparent on a fundus examination or color fundus photography. OCT may also show thickening of the nerve fiber layer indicative of nerve fiber layer infarction and swelling.
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FA
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FA is useful to determine whether the serous RD is related to vascular leakage, which is the case in neovascular/exudative age-related macular degeneration, diabetic retinopathy, retinal vasculitis, and other retinal vascular pathologies. Signs of retinal vasculitis on FA should warrant an infectious workup but do not exclude the possibility of malignancy, particularly if an opportunistic infection is identified as a cause. In the present case, FA did not show any vascular leakage in the macula that could have explained the subretinal fluid, thus raising the suspicion for systemic causes.
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Laboratory testing
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Laboratory testing can be helpful in cases with serous RD to assess for systemic diseases. CBC and CMP are useful initial blood tests, and additional studies for infectious (tuberculosis, syphilis, and Lyme disease) and inflammatory (ESR/CRP) markers along with autoantibodies like rheumatoid factor and ANA might aid in the diagnosis.
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Cranioorbital imaging
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Once the patient was diagnosed with leukemia, orbital magnetic resonance imaging showed an enhancement of the right optic nerve likely due to leukemic infiltration.
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Management and follow-up care
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After laboratory test results, the patient was immediately notified and asked to go to the emergency department for direct hospital admission. She was hospitalized for a month, during which she was diagnosed as having B-cell ALL with CNS involvement. She was initiated on chemotherapy by the oncology team and initially followed by a retina specialist every 1 to 2 months. Her serous RD and retinal hemorrhages completely resolved 6 months after the initiation of chemotherapy ( Fig. 62.3 ).
