History of present illness
A 41-year-old man with a history of sickle cell disease presents with a grayish spot in the center of the visual field of his left eye. The onset of symptoms was sudden, and the patient notes no previous episodes. Although the patient notes mild improvement, the symptoms have been persistent for 5 days before the initial presentation. The patient did not have associated ocular pain, flashes, or floaters, and the fellow right eye was unaffected.
Ocular examination findings
Visual acuities with correction were 20/20 in the right eye and 20/25 in the left eye. Intraocular pressures were 15 mm Hg bilaterally. Pupils were round and reactive without a relative afferent pupillary defect in each eye. External and anterior segment examination were unremarkable. On dilated fundus examination, both optic nerves appeared normal without edema or pallor. The right macula was flat without edema. The left macula had patchy grayish-white perifoveal lesions with a blunted foveal reflex. The vessels of both eyes were slightly attenuated and had a sclerotic appearance peripherally. The peripheral examination of the right eye demonstrated 360 degrees of peripheral laser scars with fibrosis and patches of regressed neovascularization ( Fig. 49.1 ). The peripheral examination of the left eye similarly had peripheral laser scars and signs of regressed neovascularization ( Fig. 49.2 ). There were no salmon patch hemorrhages, sunburst lesions, iridescent spots, retinal or preretinal hemorrhages, or cotton wool spots in either eye.
Optical coherence tomography
Imaging
Optical coherence tomography (OCT) of the macula of the right eye showed intact retinal layers without edema ( Fig. 49.3 ). The left eye demonstrated patchy perifoveal hyperreflective thickened bands at the level of the inner plexiform layer, inner nuclear layer, and outer plexiform layer, with sparing of the outer nuclear and deeper retinal layers ( Fig. 49.4 ).
Questions to ask
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Does the patient have any symptoms of giant cell arteritis (GCA)? Although the patient is not of the classic age or gender, GCA may cause retinal ischemia and is a life-threatening condition that must be ruled out.
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The patient did not endorse any headache, scalp tenderness, jaw pain, unexplained weight loss, fever, or generalized malaise when asked about these symptoms.
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Does the patient have any major systemic or ocular conditions that could predispose to localized retinal ischemia, such as diabetic retinopathy, hypertension, retinal vein occlusion, or artery occlusion?
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The patient has a history of sickle cell disease, which may be associated with retinal vascular occlusions. This patient had previous laser retinopexy performed for associated stage 3 proliferative sickle cell retinopathy.
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Does the patient have any environmental risk factors? It is thought that vasopressors, such as caffeine or medications (e.g., oral contraceptives), or dehydration may precipitate a vasoocclusive episode.
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None present
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What is the gender of the patient and age of the patient? Acute macular neuroretinopathy (AMN) is a rare entity that presents similarly and typically affects young, healthy women in their teens to thirties.
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The patient is a man and is in his early 40s.
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Assessment
A 41-year-old man with a history of sickle cell disease presents with paracentral scotoma of the left eye with corresponding hyperreflective lesions of the inner nuclear layer on OCT.
Differential diagnosis
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Paracentral acute middle maculopathy (PAMM)
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AMN
Working diagnosis
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PAMM
Multimodal testing and results
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Fluorescein angiography (FA)
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FA demonstrates areas of peripheral capillary dropout in both eyes with areas of regressed seafans surrounded by laser scars. Chorioretinal scars are visualized corresponding to earlier laser retinopexy. Some leakage is seen in both eyes in areas of regressed seafans. In addition, areas of arteriovenous anastomoses are seen in both eyes. No obvious filling defects or gross abnormalities are noted in the macular region of both eyes ( Figs. 49.5 and 49.6 ).
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