SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY IMAGING OF PURTSCHER-LIKE RETINOPATHY Brandon J. Lujan, MD,*† Patrick A. Coady, MD,‡ H. Richard McDonald, MD*
Purpose: To report spectral domain optical coherence tomography findings in a patient with Purtscher-like retinopathy. Methods: Case report, image analysis, and literature review. Results: A 71-year-old woman presented with decreased vision, cotton-wool spots, and a deep whitish polygonal lesion in her left eye, which was hyperreflective on spectral domain optical coherence tomography. She was diagnosed with Purtscher-like retinopathy and later was found to have metastatic pancreatic cancer. Conclusion: Purtscher-like retinopathy can occur secondary to malignant pancreatic cancer. Purtscher-flecken can be characterized by cross-sectional and en face spectral domain optical coherence tomography and represent deep capillary ischemia. RETINAL CASES & BRIEF REPORTS 8:283–285, 2014
the inner plexiform, inner nuclear, and outer plexiform layers of the nasal retina. There was no intraretinal or subretinal fluid. The en face analysis through the inner nuclear and inner plexiform layers showed that hyperreflectivity corresponded to the area of polygonal whitening shown on the color fundus photograph. Two weeks after the examination, she was diagnosed with right lower extremity deep vein thrombosis. Laboratory workup showed mildly elevated erythrocyte sedimentation rate (35 mm/hour), significant anemia (hemoglobin, 7.5 g/dL), and a positive antinuclear antibody screen. Platelets were 219 and D-dimer was elevated at .20 mg/mL. Hepatitis serologies were negative. International normalized ratio was 1.2, and a normal hemoglobin phenotype was detected. The medical workup revealed gastric varices, and computed tomography imaging subsequently identified metastatic pancreatic cancer with metastases to the liver. After 1 month, she was admitted for altered mental status, was found to have multiple brain metastases, and had a severe cerebrovascular accident. Two and a half months after visual symptoms began, the patient died of complications of the stroke.
From the *West Coast Retina Medical Group, San Francisco, California; †Division of Vision Science, School of Optometry, University of California, Berkeley, California; and ‡Department of Ophthalmology, California Pacific Medical Center, San Francisco, California. Case Report A 71-year-old woman presented with a sudden spot in the vision of her left eye for 2 weeks. Her medical history was significant for recently diagnosed diabetes and hypercholesterolemia. Her visual acuity was 20/ 30 in the right and 20/40 in the left eye. Her blood pressure was 138/69 mmHg, and her intraocular pressure was 12 mmHg in both eyes. Anterior segment was significant for mild nuclear sclerotic cataract. Fundus examinations of the both eyes revealed cotton-wool spots scattered approximately 2 disk diameters from the optic nerve along the arcade. In the left macula, there was a deep whitishappearing, slightly thickened polygonal area approximately one disk diameter is size. There was a clear interval between the optic nerve and retinal whitening through which a subtle whitened vessel passed (Figure 1). Fluorescein angiography of the left eye showed early abnormal arterial filling in the area of retinal whitening. Spectral domain optical coherence tomography revealed a normal foveal contour with a discreet area of increased hyperreflectivity in
Discussion The inner retinal whitening appears to be consistent with clinical descriptions of “Purtscher-flecken.” On spectral domain optical coherence tomography, the areas of hyperreflectivity involve the inner plexiform, inner nuclear, and outer plexiform layers. Purtscher’s retinopathy has been shown to represent two ischemic retinal findings 1) cotton-wool spots and 2) “Purtscherflecken”.1 The pathogenesis of this disease is presumed to be from occlusion of small arterioles. This
Presented at the Pacific Retina Club Meeting, Los Angeles, CA, April 2013. None of the authors have any financial/conflicting interests to disclose. Reprint requests: Brandon J. Lujan, MD, West Coast Retina Medical Group, 1445 Bush Street, San Francisco, CA 94109; e-mail: [email protected]
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Fig. 1. A. Horizontal spectral domain optical coherence tomography frame-averaged cross-sectional scan through the fovea of the left eye. Nasal to the fovea there is markedly increased hyperreflectivity of the outer plexiform, inner nuclear layer, and inner plexiform layers. Henle’s fiber layer demonstrates increased reflectivity from what would be expected given the angle of the scan. There is a focal area of inner nuclear layer narrowing nasal to the hyperreflectivity. B. Color fundus photograph of the left eye demonstrating multiple cotton-wool spots, intraretinal peripapillary hemorrhage, and polygonal area of retinal whitening nasal to the fovea clinically consistent with “Purtscher-flecken,” location of A shown with horizontal line. C. En face optical coherence tomography overlaid on the color fundus photograph demonstrating the topographic distribution of retinal whitening derived from reflectivity data within the 43-mm ILM-based slab indicated in red. Fluorescein angiogram in the early phase (D) demonstrating abnormal arteriolar filling toward region of angiographically silent area of retinal whitening, and late phase (E) demonstrating increased hyperfluorescence in the distribution of this vessel.
hypothesis was tested by releasing fibrin clots 0.15 mm to 1.0 mm into the ophthalmic artery of a pig—this resulted in superficial and deep retinal infarcts.2 The spectral domain optical coherence tomography findings are consistent with relative ischemia in the deep capillary bed supplied by an occluded arteriole.3 “Purtscher-flecken” have been described “to have a polygonal margin and lie in the inner nuclear layer, between arterioles.”1 The use of en face optical coherence tomography analysis shows the close correlation between the volumetric optical coherence tomography findings and these clinical findings. Paracentral acute middle maculopathy was originally described by Sarraf et al,4 where five patients who were thought to have acute macular neuroretinopathy had spectral domain optical coherence tomography findings of hyperreflectivity in the outer
plexiform and inner nuclear layer junction (Type I lesions). Subsequently, this optical coherence tomography finding of deep capillary ischemia was demonstrated in a number of conditions in a variety of systemic conditions.5 Our patient demonstrates that this finding of deep capillary ischemia also represents the “Purtscher-flecken” found in Purtscher-like retinopathy. The expanding set of circumstances in which paracentral acute middle maculopathy and deep capillary ischemia are found suggests that this is likely not a single diagnostic entity, but rather a final common pathway of ischemic conditions. Key words: ischemia, retinal whitening, cottonwool spot, en face, Cirrus, Purtscher-flecken, pancreatic cancer, cancer, metastasis, paracentral acute middle maculopathy.
SD-OCT IMAGING OF PURTSCHER-LIKE RETINOPATHY
References 1. Schmidt D. The mystery of cotton-wool spots—a review of recent and historical descriptions. Eur J Med Res 2008;13:231–266. 2. Behrens-Baumann W, Scheurer G, Schroer H. Pathogenesis of Purtscher’s retinopathy. An experimental study. Graefes Arch Clin Exp Ophthalmol 1992;230:286–291. 3. Sarda V, Nakashima K, Wolff B, et al. Topography of patchy retinal whitening during acute perfused retinal vein occlusion by
285 optical coherence tomography and adaptive optics fundus imaging. Eur J Ophthalmol 2011;21:653–656. 4. Sarraf D, Rahimy E, Fawzi AA, et al. Paracentral acute middle maculopathy: a new variant of acute macular neuroretinopathy associated with retinal capillary ischemia. JAMA Ophthalmol 2013;131:1275–1287. 5. Yu S, Wang F, Pang CE, et al. Multimodal imaging findings in retinal deep capillary ischemia. Retina 2014;34:636–646.
Purpose: To report spectral domain optical coherence tomography findings in a patient with Purtscher-like retinopathy. Methods: Case report, image analysis, and literature review. Results: A 71-year-old woman presented with decreased vision, cotton-wool spots, and a deep whitish polygonal lesion in her left eye, which was hyperreflective on spectral domain optical coherence tomography. She was diagnosed with Purtscher-like retinopathy and later was found to have metastatic pancreatic cancer. Conclusion: Purtscher-like retinopathy can occur secondary to malignant pancreatic cancer. Purtscher-flecken can be characterized by cross-sectional and en face spectral domain optical coherence tomography and represent deep capillary ischemia. RETINAL CASES & BRIEF REPORTS 8:283–285, 2014
the inner plexiform, inner nuclear, and outer plexiform layers of the nasal retina. There was no intraretinal or subretinal fluid. The en face analysis through the inner nuclear and inner plexiform layers showed that hyperreflectivity corresponded to the area of polygonal whitening shown on the color fundus photograph. Two weeks after the examination, she was diagnosed with right lower extremity deep vein thrombosis. Laboratory workup showed mildly elevated erythrocyte sedimentation rate (35 mm/hour), significant anemia (hemoglobin, 7.5 g/dL), and a positive antinuclear antibody screen. Platelets were 219 and D-dimer was elevated at .20 mg/mL. Hepatitis serologies were negative. International normalized ratio was 1.2, and a normal hemoglobin phenotype was detected. The medical workup revealed gastric varices, and computed tomography imaging subsequently identified metastatic pancreatic cancer with metastases to the liver. After 1 month, she was admitted for altered mental status, was found to have multiple brain metastases, and had a severe cerebrovascular accident. Two and a half months after visual symptoms began, the patient died of complications of the stroke.
From the *West Coast Retina Medical Group, San Francisco, California; †Division of Vision Science, School of Optometry, University of California, Berkeley, California; and ‡Department of Ophthalmology, California Pacific Medical Center, San Francisco, California. Case Report A 71-year-old woman presented with a sudden spot in the vision of her left eye for 2 weeks. Her medical history was significant for recently diagnosed diabetes and hypercholesterolemia. Her visual acuity was 20/ 30 in the right and 20/40 in the left eye. Her blood pressure was 138/69 mmHg, and her intraocular pressure was 12 mmHg in both eyes. Anterior segment was significant for mild nuclear sclerotic cataract. Fundus examinations of the both eyes revealed cotton-wool spots scattered approximately 2 disk diameters from the optic nerve along the arcade. In the left macula, there was a deep whitishappearing, slightly thickened polygonal area approximately one disk diameter is size. There was a clear interval between the optic nerve and retinal whitening through which a subtle whitened vessel passed (Figure 1). Fluorescein angiography of the left eye showed early abnormal arterial filling in the area of retinal whitening. Spectral domain optical coherence tomography revealed a normal foveal contour with a discreet area of increased hyperreflectivity in
Discussion The inner retinal whitening appears to be consistent with clinical descriptions of “Purtscher-flecken.” On spectral domain optical coherence tomography, the areas of hyperreflectivity involve the inner plexiform, inner nuclear, and outer plexiform layers. Purtscher’s retinopathy has been shown to represent two ischemic retinal findings 1) cotton-wool spots and 2) “Purtscherflecken”.1 The pathogenesis of this disease is presumed to be from occlusion of small arterioles. This
Presented at the Pacific Retina Club Meeting, Los Angeles, CA, April 2013. None of the authors have any financial/conflicting interests to disclose. Reprint requests: Brandon J. Lujan, MD, West Coast Retina Medical Group, 1445 Bush Street, San Francisco, CA 94109; e-mail: [email protected]
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RETINAL CASES & BRIEF REPORTS´ 2014 VOLUME 8 NUMBER 4
Fig. 1. A. Horizontal spectral domain optical coherence tomography frame-averaged cross-sectional scan through the fovea of the left eye. Nasal to the fovea there is markedly increased hyperreflectivity of the outer plexiform, inner nuclear layer, and inner plexiform layers. Henle’s fiber layer demonstrates increased reflectivity from what would be expected given the angle of the scan. There is a focal area of inner nuclear layer narrowing nasal to the hyperreflectivity. B. Color fundus photograph of the left eye demonstrating multiple cotton-wool spots, intraretinal peripapillary hemorrhage, and polygonal area of retinal whitening nasal to the fovea clinically consistent with “Purtscher-flecken,” location of A shown with horizontal line. C. En face optical coherence tomography overlaid on the color fundus photograph demonstrating the topographic distribution of retinal whitening derived from reflectivity data within the 43-mm ILM-based slab indicated in red. Fluorescein angiogram in the early phase (D) demonstrating abnormal arteriolar filling toward region of angiographically silent area of retinal whitening, and late phase (E) demonstrating increased hyperfluorescence in the distribution of this vessel.
hypothesis was tested by releasing fibrin clots 0.15 mm to 1.0 mm into the ophthalmic artery of a pig—this resulted in superficial and deep retinal infarcts.2 The spectral domain optical coherence tomography findings are consistent with relative ischemia in the deep capillary bed supplied by an occluded arteriole.3 “Purtscher-flecken” have been described “to have a polygonal margin and lie in the inner nuclear layer, between arterioles.”1 The use of en face optical coherence tomography analysis shows the close correlation between the volumetric optical coherence tomography findings and these clinical findings. Paracentral acute middle maculopathy was originally described by Sarraf et al,4 where five patients who were thought to have acute macular neuroretinopathy had spectral domain optical coherence tomography findings of hyperreflectivity in the outer
plexiform and inner nuclear layer junction (Type I lesions). Subsequently, this optical coherence tomography finding of deep capillary ischemia was demonstrated in a number of conditions in a variety of systemic conditions.5 Our patient demonstrates that this finding of deep capillary ischemia also represents the “Purtscher-flecken” found in Purtscher-like retinopathy. The expanding set of circumstances in which paracentral acute middle maculopathy and deep capillary ischemia are found suggests that this is likely not a single diagnostic entity, but rather a final common pathway of ischemic conditions. Key words: ischemia, retinal whitening, cottonwool spot, en face, Cirrus, Purtscher-flecken, pancreatic cancer, cancer, metastasis, paracentral acute middle maculopathy.
SD-OCT IMAGING OF PURTSCHER-LIKE RETINOPATHY
References 1. Schmidt D. The mystery of cotton-wool spots—a review of recent and historical descriptions. Eur J Med Res 2008;13:231–266. 2. Behrens-Baumann W, Scheurer G, Schroer H. Pathogenesis of Purtscher’s retinopathy. An experimental study. Graefes Arch Clin Exp Ophthalmol 1992;230:286–291. 3. Sarda V, Nakashima K, Wolff B, et al. Topography of patchy retinal whitening during acute perfused retinal vein occlusion by
285 optical coherence tomography and adaptive optics fundus imaging. Eur J Ophthalmol 2011;21:653–656. 4. Sarraf D, Rahimy E, Fawzi AA, et al. Paracentral acute middle maculopathy: a new variant of acute macular neuroretinopathy associated with retinal capillary ischemia. JAMA Ophthalmol 2013;131:1275–1287. 5. Yu S, Wang F, Pang CE, et al. Multimodal imaging findings in retinal deep capillary ischemia. Retina 2014;34:636–646.
