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Purtscher Retinopathy as the Initial Sign of Thrombotic Thrombocytopenic Purpura/Hemolytic Uremic Syndrome

Purtscher Retinopathy as the Initial Sign of Thrombotic Thrombocytopenic Purpura/Hemolytic Uremic... A 52-YEAR-OLD African American woman had a 3-week history of progressive decline in bilateral vision. Ocular examination revealed bilateral diffuse retinal edema and whitening and confluent cotton-wool spots centered around the peripapillary region. Also noted were bilateral marked attenuation of the retinal arterioles, scattered retinal hemorrhages in both eyes, and a cherry-red spot in the right eye (Figure 1). Fluorescein angiography demonstrated severe capillary dropout of the macular and peripapillary retinal vasculature in both eyes in the arterial phase (Figure 2A, 2B) and perivascular leakage with severe capillary dropout in both eyes in the late arteriovenous phase (Figure 2C, 2D), accounting for a visual acuity of counting fingers at 2 feet in both eyes. The patient was initially diagnosed as having Purtscher retinopathy secondary to a questionable vasculitic/collagen vascular origin. In-hospital work-up revealed nonhemolytic anemia without thrombocytopenia, renal failure, neurological disease, or collagen vascular disease. Oral prednisone was given at the suggestion of a rheumatologic consultation to treat a presumed autoimmune origin. Follow-up ocular examination results remained unchanged. Nine days after the initial visit, the patient developed generalized seizures, acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia. Asystole followed, which was refractive to resuscitative efforts. An autopsy revealed evidence of platelet microthrombin in the eye, heart, lung, and kidney (Figure 3). Figure 1. View LargeDownload Fundus photograph of the right (A) and left (B) eyes demonstrating bilateral diffuse retinal edema, whitening, and cotton-wool spots centered around the peripapillary region. Also note bilateral marked attenuation of the retinal arterioles, scattered retinal hemorrhages in both eyes, and a cherry-red spot in the right eye. Figure 2. View LargeDownload Midphase flourescein angiography demonstrates severe capillary dropout of the macular and peripapillary retinal vasculature in right (A) and left (B) eyes. Late-phase flourescein angiography demonstrates perivascular leakage with severe capillary dropout in the right (C) and left (D) eyes. Figure 3. View LargeDownload Vascular lumen occluded by fibrin-platelet microthrombi (arrows) as seen in a retinal arteriole in A, the left eye (phosphotungstic acid–hematoxylin, original magnification ×400); B, a small arterial branch in the epicardial fat (hematoxylin-eosin, original magnification ×400); C, a glomerular capillary (hematoxylin-eosin, original magnification ×400); and D, a small arteriolar branch of the lung (hematoxylin-eosin, original magnification ×200). Comment Thrombotic thrombocytopenic purpura (TTP) is an uncommon disease characterized by diffuse platelet aggregation initially defined by the pentad of microangiopathic hemolytic anemia, thrombocytopenia, fever, central nervous system dysfunction, and renal disease. Proposed causes for platelet aggregation in TTP are intrinsically altered von Willebrand factor molecules or GPIIb/IIIa (a human platelet glycoprotein complex and autoantigen most commonly recognized by autoantibodies in autoimmune TTP). Without daily plasmapheresis, the mortality rate is 90%. The triad of clinical features—microangiopathic hemolytic anemia, thrombocytopenia, and uremia—constitutes hemolytic uremic syndrome (HUS). The distinction between TTP and HUS is not relevant for initial treatment; these patients are best described as having a single syndrome, TTP/HUS. Hemolytic anemia and thrombocytopenia have been documented to be the most frequent clinical indicators,1 and thus are sufficient to establish the diagnosis of TTP/HUS and to initiate plasmapheresis. Purtscher retinopathy was originally characterized as peripapillary retinal hemorrhages and multiple patches of superficial whitening seen in patients who experienced severe head trauma. Typically, fundus examination of Purtscher retinopathy reveals cotton-wool spots (which are usually flame-shaped and may cover retinal arterioles), retinal hemorrhages (flame-shaped or dot-and-blot), and Purtscher-flecken (which are typically polygonal and never cover neighboring arterioles). The theory that Purtscher retinopathy could occur secondary to platelet aggregation, as seen in TTP/HUS, is supported by fundus findings after platelet aggregate microembolization of the porcine ophthalmic vasculature.2 Purtscher and occlusive retinopathy have been frequently described in patients with TTP and HUS.3-6 These reported patients, however, had decreased platelets and renal failure or neurological disease along with ocular findings, thus making the diagnosis of TTP/HUS more obvious. Similar to those in our patient, ophthalmoscopic findings are often greatest within the peripapillary regions. This case is unique in that Purtscher retinopathy was the initial sign of TTP/HUS even prior to demonstrable thrombocytopenia, microangiopathic hemolytic anemia, renal failure, or neurological disease. Ophthalmologists should consider the rare possibility of TTP/HUS in patients with Purtscher retinopathy. Corresponding author: Dennis M. Marcus, MD, Department of Ophthalmology, Medical College of Georgia, 1120 15th St, Augusta, GA 30912-3400 (e-mail: dmarcus@mail.mcg.edu). References 1. Martinez FAPereira AOrdinas A Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome (TTP/HUS): description of a series of 35 patients. Med Clin (Barc). 1997;10949- 52Google Scholar 2. Schroer HScheurer GBehrens-Baumann W Vascular occlusion of the retina—an experimental model, II: platelet aggregates. Graefes Arch Clin Exp Ophthalmol. 1992;230281- 285Google ScholarCrossref 3. Power MRegillo CDCustis PH Thrombotic thrombocytopenic purpura associated with Purtscher retinopathy. Arch Ophthalmol. 1997;115128- 129Google ScholarCrossref 4. Lauer AKKlein MLKovarik WDPalmer EA Hemolytic uremic syndrome associated with Purtscher-like retinopathy. Arch Ophthalmol. 1998;1161119- 1120Google Scholar 5. Jellie HGGonder JRCanny CLArce FPKaufmann JC Ocular involvement in thrombotic thrombocytopenic purpura: the angiographic and histopathological features. Can J Ophthalmol. 1984;19279- 283Google Scholar 6. Benson DOFitzgibbons JFGoodnight SH The visual system in thrombotic thrombocytopenic purpura. Ann Ophthalmol. 1980;12413- 417Google Scholar http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

Purtscher Retinopathy as the Initial Sign of Thrombotic Thrombocytopenic Purpura/Hemolytic Uremic Syndrome

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Publisher
American Medical Association
Copyright
Copyright © 2001 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.119.9.1388
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Abstract

A 52-YEAR-OLD African American woman had a 3-week history of progressive decline in bilateral vision. Ocular examination revealed bilateral diffuse retinal edema and whitening and confluent cotton-wool spots centered around the peripapillary region. Also noted were bilateral marked attenuation of the retinal arterioles, scattered retinal hemorrhages in both eyes, and a cherry-red spot in the right eye (Figure 1). Fluorescein angiography demonstrated severe capillary dropout of the macular and peripapillary retinal vasculature in both eyes in the arterial phase (Figure 2A, 2B) and perivascular leakage with severe capillary dropout in both eyes in the late arteriovenous phase (Figure 2C, 2D), accounting for a visual acuity of counting fingers at 2 feet in both eyes. The patient was initially diagnosed as having Purtscher retinopathy secondary to a questionable vasculitic/collagen vascular origin. In-hospital work-up revealed nonhemolytic anemia without thrombocytopenia, renal failure, neurological disease, or collagen vascular disease. Oral prednisone was given at the suggestion of a rheumatologic consultation to treat a presumed autoimmune origin. Follow-up ocular examination results remained unchanged. Nine days after the initial visit, the patient developed generalized seizures, acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia. Asystole followed, which was refractive to resuscitative efforts. An autopsy revealed evidence of platelet microthrombin in the eye, heart, lung, and kidney (Figure 3). Figure 1. View LargeDownload Fundus photograph of the right (A) and left (B) eyes demonstrating bilateral diffuse retinal edema, whitening, and cotton-wool spots centered around the peripapillary region. Also note bilateral marked attenuation of the retinal arterioles, scattered retinal hemorrhages in both eyes, and a cherry-red spot in the right eye. Figure 2. View LargeDownload Midphase flourescein angiography demonstrates severe capillary dropout of the macular and peripapillary retinal vasculature in right (A) and left (B) eyes. Late-phase flourescein angiography demonstrates perivascular leakage with severe capillary dropout in the right (C) and left (D) eyes. Figure 3. View LargeDownload Vascular lumen occluded by fibrin-platelet microthrombi (arrows) as seen in a retinal arteriole in A, the left eye (phosphotungstic acid–hematoxylin, original magnification ×400); B, a small arterial branch in the epicardial fat (hematoxylin-eosin, original magnification ×400); C, a glomerular capillary (hematoxylin-eosin, original magnification ×400); and D, a small arteriolar branch of the lung (hematoxylin-eosin, original magnification ×200). Comment Thrombotic thrombocytopenic purpura (TTP) is an uncommon disease characterized by diffuse platelet aggregation initially defined by the pentad of microangiopathic hemolytic anemia, thrombocytopenia, fever, central nervous system dysfunction, and renal disease. Proposed causes for platelet aggregation in TTP are intrinsically altered von Willebrand factor molecules or GPIIb/IIIa (a human platelet glycoprotein complex and autoantigen most commonly recognized by autoantibodies in autoimmune TTP). Without daily plasmapheresis, the mortality rate is 90%. The triad of clinical features—microangiopathic hemolytic anemia, thrombocytopenia, and uremia—constitutes hemolytic uremic syndrome (HUS). The distinction between TTP and HUS is not relevant for initial treatment; these patients are best described as having a single syndrome, TTP/HUS. Hemolytic anemia and thrombocytopenia have been documented to be the most frequent clinical indicators,1 and thus are sufficient to establish the diagnosis of TTP/HUS and to initiate plasmapheresis. Purtscher retinopathy was originally characterized as peripapillary retinal hemorrhages and multiple patches of superficial whitening seen in patients who experienced severe head trauma. Typically, fundus examination of Purtscher retinopathy reveals cotton-wool spots (which are usually flame-shaped and may cover retinal arterioles), retinal hemorrhages (flame-shaped or dot-and-blot), and Purtscher-flecken (which are typically polygonal and never cover neighboring arterioles). The theory that Purtscher retinopathy could occur secondary to platelet aggregation, as seen in TTP/HUS, is supported by fundus findings after platelet aggregate microembolization of the porcine ophthalmic vasculature.2 Purtscher and occlusive retinopathy have been frequently described in patients with TTP and HUS.3-6 These reported patients, however, had decreased platelets and renal failure or neurological disease along with ocular findings, thus making the diagnosis of TTP/HUS more obvious. Similar to those in our patient, ophthalmoscopic findings are often greatest within the peripapillary regions. This case is unique in that Purtscher retinopathy was the initial sign of TTP/HUS even prior to demonstrable thrombocytopenia, microangiopathic hemolytic anemia, renal failure, or neurological disease. Ophthalmologists should consider the rare possibility of TTP/HUS in patients with Purtscher retinopathy. Corresponding author: Dennis M. Marcus, MD, Department of Ophthalmology, Medical College of Georgia, 1120 15th St, Augusta, GA 30912-3400 (e-mail: dmarcus@mail.mcg.edu). References 1. Martinez FAPereira AOrdinas A Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome (TTP/HUS): description of a series of 35 patients. Med Clin (Barc). 1997;10949- 52Google Scholar 2. Schroer HScheurer GBehrens-Baumann W Vascular occlusion of the retina—an experimental model, II: platelet aggregates. Graefes Arch Clin Exp Ophthalmol. 1992;230281- 285Google ScholarCrossref 3. Power MRegillo CDCustis PH Thrombotic thrombocytopenic purpura associated with Purtscher retinopathy. Arch Ophthalmol. 1997;115128- 129Google ScholarCrossref 4. Lauer AKKlein MLKovarik WDPalmer EA Hemolytic uremic syndrome associated with Purtscher-like retinopathy. Arch Ophthalmol. 1998;1161119- 1120Google Scholar 5. Jellie HGGonder JRCanny CLArce FPKaufmann JC Ocular involvement in thrombotic thrombocytopenic purpura: the angiographic and histopathological features. Can J Ophthalmol. 1984;19279- 283Google Scholar 6. Benson DOFitzgibbons JFGoodnight SH The visual system in thrombotic thrombocytopenic purpura. Ann Ophthalmol. 1980;12413- 417Google Scholar

Journal

Archives of Ophthalmology – American Medical Association

Published: Sep 1, 2001

Keywords: hemolytic-uremic syndrome,thrombotic thrombocytopenic purpura,purtscher's retinopathy

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