Bilateral Serous Retinal Detachment in Thrombotic Thrombocytopenic Purpura 23-year-old woman diagnosed as having
-^-thrombotic thrombocytopenic (TTP)
purpura
gest compromise of the barrier func¬ tion of the retinal pigment epithelial cells between the retinal and choroidal
treated with oral steroids, plas¬ ma, and spun packed red blood cells. Two days later, she underwent plasmapheresis and immediately developed blurred vision in both eyes: best corrected visual acuity was 20/80 OD and 20/400 OS. Intraocular pressures were normal. Fundus examina¬ tion showed diffuse serous retinal detach¬ ments (SRDs) bilaterally (Fig 1), and the right eye had paravenous retinal hemor¬ rhages nasally, along both temporal ar¬ cades, and a preretinal hemorrhage superi¬ or to the disc. Fluorescein dye studies showed patchy choroidal perfusion and ex¬ tensive hyperfluorescenc with leakage into the subretinal space bilaterally (Fig 2). After 3 days of daily plasmapheresis and oral prednisone therapy, her visual acuity returned to 20/30 OU, and the retinas were reattached. Her blood pressure remained normal throughout this admission. Two months later, the right eye hemor¬ rhages were resolving, but the peripapil¬ lary choroidal filling defects and a diffuse disturbance of the retinal pigment epitheli¬ um persisted bilaterally (Fig 3). We believe this is the only reported case of SRD associ¬ ated with TTP in which paravenous and large preretinal hemorrhages have been noted.
subretinal space. It is known that the serum fibrinogen level, as the single major determinant of serum viscosity, is elevated in patients with TTP. It is also true that since plasmapheresis de¬ creases the serum fibrinogen level, it also decreases serum viscosity. The precise role these factors play obvious¬ ly remains speculative, but the sudden onset of SRD in our patient after plas¬ mapheresis and a subsequent reduc¬ tion in serum viscosity, as well as the rapid resolution of the subretinal fluid, cannot be coincidental.5 M. MADISON SLUSHER, MD Holly P. Pugh, MD Richard P. Hackel Winston-Salem, NC
COMMENT
References
was
Several of the eight previously re¬ ported cases of SRD complicating TTP
preterminal events1"4; currently, patients with TTP live longer, and
were
SRDs and blurred vision may be the initial signs and symptoms of the dis¬ ease. Thus, their recognition may be
lifesaving.4
It has been suggested that microthrombotic events in the segmental choroidal vasculature cause SRD; the hyperfluorescence and "leakage" shown here at the margins of poorly perfused choroidal areas (Fig 2) sug-
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circulations,3 reflecting incompetence pigment epithelium and allowing large subretinal accumula¬ tions of serous fluid. Another possible contributing mechanism is the poten¬ tial effect of altered serum viscosity on fluid movement across compromised retinal pigment epithelial cells into the of the retinal
1. Percival SPB. Ocular findings in thrombotic thrombocytopenic purpura (Moschcowitz's disease). Br J Ophthalmol. 1970;54:73-78. 2. Cogan DG. Ocular involvement in disseminated intravascular coagulopathy. Arch Ophthalmol.
1975;93:1-8.
3. Lambert SR, High KA, Cotlier E, Benz EJ Jr. Serous retinal detachments in thrombotic thrombocytopenic purpura. Arch Ophthalmol.
1985;103:1172-1174.
4. Hemeter W. Presumed thrombotic thrombo-
cytopenic purpura associated with bilateral serous retinal detachments. Am J Ophthalmol. 1988;105: 421-422. 5. Tsuboi S, Pederson JE. Effect of plasma osmolality and intraocular pressure on fluid movement across the blood-retinal barrier. Invest Ophthalmol Vis Sci. 1988;29:1747-1749.
Fig 1. Left, Composite fundus photograph of the right eye showing a large serous detachment of the posterior retina. Note that the prere¬ tinal hemorrhages are present along major veins; a "boat-shaped" preretinal hemorrhage superior to the disc obscures the retinal vascu-
lature. Right, Composite photograph of the left eye showing a welldefined serous retinal detachment and no retinal hemorrhages. Its pronounced bullous segment is located along the inferior vascular arcade (arrows).
Fig 2. —Fluorescein angiographie studies performed initially showed both eyes to have patchy filling of the choroidal vessels around both discs. These filling defects persisted into early recirculatory phases. Left, The right eye predictably demonstrates blockage of fluorescein by the large preretinal hemorrhages (white arrows), profuse leakage of dye nasal to the disc, and diffuse areas of deep hyperfluorescence
along the superior arcade vessels, presumed to be sites of leakage of subsensory retinal fluid (black arrows). Right, The left eye, at a slightly earlier transit time, displays these same areas of hyperfluorescence as more discrete points of leakage and a pronounced area of bullous retinal detachment inferiorly.
—
Fig 3. —Fluorescein angiographie frames obtained approximately 2 months after rapid onset and resolution of serous retinal detachment. No plasmapheresis had been performed since the patient's original hospitalization. Left, The right eye shows persistence of preretinal hemorrhage, peripapillary choroidal filling defects, and nonspecific, diffuse hyperfluorescence along both arcades, and displays punctate
areas of hypofluorescence where retinal pigment epithelium has clumped (the equivalent of Elschnig's spots) to block dye transmis¬ sion; this is especially obvious nasal to the disc (arrows). Right, The angiogram of the left eye also suggests there is nonperfused choroid along superior and inferior arcade vessels.
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-^-thrombotic thrombocytopenic (TTP)
purpura
gest compromise of the barrier func¬ tion of the retinal pigment epithelial cells between the retinal and choroidal
treated with oral steroids, plas¬ ma, and spun packed red blood cells. Two days later, she underwent plasmapheresis and immediately developed blurred vision in both eyes: best corrected visual acuity was 20/80 OD and 20/400 OS. Intraocular pressures were normal. Fundus examina¬ tion showed diffuse serous retinal detach¬ ments (SRDs) bilaterally (Fig 1), and the right eye had paravenous retinal hemor¬ rhages nasally, along both temporal ar¬ cades, and a preretinal hemorrhage superi¬ or to the disc. Fluorescein dye studies showed patchy choroidal perfusion and ex¬ tensive hyperfluorescenc with leakage into the subretinal space bilaterally (Fig 2). After 3 days of daily plasmapheresis and oral prednisone therapy, her visual acuity returned to 20/30 OU, and the retinas were reattached. Her blood pressure remained normal throughout this admission. Two months later, the right eye hemor¬ rhages were resolving, but the peripapil¬ lary choroidal filling defects and a diffuse disturbance of the retinal pigment epitheli¬ um persisted bilaterally (Fig 3). We believe this is the only reported case of SRD associ¬ ated with TTP in which paravenous and large preretinal hemorrhages have been noted.
subretinal space. It is known that the serum fibrinogen level, as the single major determinant of serum viscosity, is elevated in patients with TTP. It is also true that since plasmapheresis de¬ creases the serum fibrinogen level, it also decreases serum viscosity. The precise role these factors play obvious¬ ly remains speculative, but the sudden onset of SRD in our patient after plas¬ mapheresis and a subsequent reduc¬ tion in serum viscosity, as well as the rapid resolution of the subretinal fluid, cannot be coincidental.5 M. MADISON SLUSHER, MD Holly P. Pugh, MD Richard P. Hackel Winston-Salem, NC
COMMENT
References
was
Several of the eight previously re¬ ported cases of SRD complicating TTP
preterminal events1"4; currently, patients with TTP live longer, and
were
SRDs and blurred vision may be the initial signs and symptoms of the dis¬ ease. Thus, their recognition may be
lifesaving.4
It has been suggested that microthrombotic events in the segmental choroidal vasculature cause SRD; the hyperfluorescence and "leakage" shown here at the margins of poorly perfused choroidal areas (Fig 2) sug-
Downloaded From: http://archopht.jamanetwork.com/ by a New York University User on 06/15/2015
circulations,3 reflecting incompetence pigment epithelium and allowing large subretinal accumula¬ tions of serous fluid. Another possible contributing mechanism is the poten¬ tial effect of altered serum viscosity on fluid movement across compromised retinal pigment epithelial cells into the of the retinal
1. Percival SPB. Ocular findings in thrombotic thrombocytopenic purpura (Moschcowitz's disease). Br J Ophthalmol. 1970;54:73-78. 2. Cogan DG. Ocular involvement in disseminated intravascular coagulopathy. Arch Ophthalmol.
1975;93:1-8.
3. Lambert SR, High KA, Cotlier E, Benz EJ Jr. Serous retinal detachments in thrombotic thrombocytopenic purpura. Arch Ophthalmol.
1985;103:1172-1174.
4. Hemeter W. Presumed thrombotic thrombo-
cytopenic purpura associated with bilateral serous retinal detachments. Am J Ophthalmol. 1988;105: 421-422. 5. Tsuboi S, Pederson JE. Effect of plasma osmolality and intraocular pressure on fluid movement across the blood-retinal barrier. Invest Ophthalmol Vis Sci. 1988;29:1747-1749.
Fig 1. Left, Composite fundus photograph of the right eye showing a large serous detachment of the posterior retina. Note that the prere¬ tinal hemorrhages are present along major veins; a "boat-shaped" preretinal hemorrhage superior to the disc obscures the retinal vascu-
lature. Right, Composite photograph of the left eye showing a welldefined serous retinal detachment and no retinal hemorrhages. Its pronounced bullous segment is located along the inferior vascular arcade (arrows).
Fig 2. —Fluorescein angiographie studies performed initially showed both eyes to have patchy filling of the choroidal vessels around both discs. These filling defects persisted into early recirculatory phases. Left, The right eye predictably demonstrates blockage of fluorescein by the large preretinal hemorrhages (white arrows), profuse leakage of dye nasal to the disc, and diffuse areas of deep hyperfluorescence
along the superior arcade vessels, presumed to be sites of leakage of subsensory retinal fluid (black arrows). Right, The left eye, at a slightly earlier transit time, displays these same areas of hyperfluorescence as more discrete points of leakage and a pronounced area of bullous retinal detachment inferiorly.
—
Fig 3. —Fluorescein angiographie frames obtained approximately 2 months after rapid onset and resolution of serous retinal detachment. No plasmapheresis had been performed since the patient's original hospitalization. Left, The right eye shows persistence of preretinal hemorrhage, peripapillary choroidal filling defects, and nonspecific, diffuse hyperfluorescence along both arcades, and displays punctate
areas of hypofluorescence where retinal pigment epithelium has clumped (the equivalent of Elschnig's spots) to block dye transmis¬ sion; this is especially obvious nasal to the disc (arrows). Right, The angiogram of the left eye also suggests there is nonperfused choroid along superior and inferior arcade vessels.
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