FUNCTIONAL AND MORPHOLOGICAL EVALUATION OF PURTSCHER RETINOPATHY Kisaburo Yamada, MD, PhD,* Celso S. Matsumoto, MD, PhD,* Kenichi Kimoto, MD, PhD,* Naoyuki Tanimoto, MD, PhD,† Kei Shinoda, MD, PhD,* Kazuo Nakatsuka, MD, PhD*
Purpose: To determine the extent of retinal damage in Purtscher retinopathy (PR). Methods: A longitudinal analysis of the retinal morphology and function was made by optical coherence tomography and electroretinography, respectively, on a patient with unilateral PR. Results: Optical coherence tomography showed a marked thickening of the inner retinal layers in the acute phase and a thinning of the inner retinal layers and alteration of the outer layers in the chronic phase. The site of these changes corresponded with the cotton-wool patches. The results of electroretinography indicated reduced inner layer function in the acute phase and partial recovery in the chronic phase. Conclusion: The inner and outer retinal layers are altered morphologically and functionally in eyes with PR. The dynamic evaluation on retinal morphology and function may be useful in understanding the pathophysiology of PR. RETINAL CASES & BRIEF REPORTS 4:55–58, 2010
both.1,2 This prompted us to conduct a longitudinal analysis of the morphologic and functional changes by optical coherence tomography (OCT) and electroretinography (ERG), respectively, in a patient with unilateral PR.
From the *Department of Ophthalmology, Oita University Faculty of Medicine, Hasama-machi, Yufu-shi, Oita, Japan; and †Centre for Ophthalmology, Institute for Ophthalmic Research, University of Tuebingen, Tuebingen, Germany.
P
urtscher retinopathy (PR) is characterized by a sudden painless loss of vision in one or both eyes associated with patches of retinal whitening and hemorrhages in the posterior pole and pallor of the optic disk. These changes usually follow head trauma, long bone fracture, or other trauma.1 The damage is thought to arise from the occlusion of arterioles by intravascular microparticles and is localized to only the inner retinal layers. However, it has not been determined whether the damage is focal or diffuse or whether it is confined to the inner or outer retina or
Case Report A 76-year-old woman noticed a sudden painless decrease in the vision in her right eye 10 days after a fracture of the sternum and right ribs in an automobile accident. She came to our clinic 16 days later when her vision did not improve. Her corrected visual acuity was 0.3 in the right eye and 0.9 in the left eye. Ophthalmoscopy showed multiple cotton-wool spots and retinal hemorrhages, and fluorescein angiography showed dye leakage from the arterioles around the optic disk in the right eye (Figure 1). Optical coherence tomography showed a thickening or irregularity of the inner retinal layers corresponding to the sites of the cotton-wool spots and retinal edema. A retinal detachment was observed at the fovea of the right eye (Figure 1). Goldmann perimetry detected a central scotoma and an enlargement of the physiologic blind spot (Figure 1). The left eye was completely normal. The ERGs recorded according to the International Society for Clinical Electrophysiology of Vision standards (http://www. iscev.org/standards/index.html) are shown in Figure 2. Because a statistical evaluation could not be made with one case, we compared the ERG responses of the affected eye with that of the fellow eye, which showed no abnormality of the fundus ophthalmoscop-
Supported by Researches on Sensory and Communicative Disorders from the Ministry of Health, Labor, and Welfare, Japan. None of the authors has a proprietary interest in any material or method mentioned. Reprint requests: Kei Shinoda, MD, PhD, Department of Ophthalmology, Oita University Faculty of Medicine, Hasama-machi, Yufushi, Oita 879-5593, Japan; e-mail: [email protected]
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Fig. 1. Clinical findings in the acute and chronic phases of a patient with PR. A, Fundus photographs at the initial examination (acute phase) showing multiple cotton-wool spots and retinal hemorrhages in the right eye. B, Fluorescein angiogram at the initial examination showing dye leakage from the small arterioles around the optic disk in the right eye. C, Fundus photograph of the right eye taken 8 months after the initial examination showing a disappearance of the cotton-wool spots and retinal hemorrhages. The dotted lines in A and C indicate the scanning lines of the OCT scans shown in Parts D–G. D and F, OCT images taken at the initial examination (acute phase) showing a thickening and irregularity of the inner retinal layers at the sites corresponding to the cotton-wool spots and retinal edema (indicated by *), and sensory retinal detachment at the fovea (indicated by arrow) in the right eye. E and G, OCT images taken at the same period with C (chronic phase) showing a segmental thinning of the inner layer and disappearances of the outer segments (indicated by arrowheads) corresponding to the cotton-wool spot or retinal edema (E and G, respectively). H, Goldmann visual fields in the acute phase showing a central scotoma and enlargement of physiologic blind spot in the right eye. I, Goldmann visual fields performed 8 months after the onset (chronic phase) showing a central scotoma and enlargement of physiologic blind spot in the right eye.
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FUNCTION AND MORPHOLOGY OF PURTSCHER RETINOPATHY
Fig. 2. Electroretinograms recorded according to the International Society for Clinical Electrophysiology of Vision protocol in the acute (upper row) and chronic (lower row) phases. The values of the amplitudes and implicit times of each wave are shown in Table 1. In the acute phase, the amplitudes of the rod b-wave and photopic negative response (PhNR) were reduced, and the implicit times were prolonged. In the chronic phase, the rod b-wave has recovered. The PhNR became larger but was still smaller than that of the fellow eye.
ically and by fluorescein angiography. In the acute phase, the amplitudes of the scotopic b-wave and photopic negative response (PhNR) were smaller (Figure 2; Table 1), and the implicit times were prolonged. A diagnosis of unilateral PR was made, and a hemostatic agent (carbazochrome sodium sulfonate), peripheral vasodilator (kallidinogenase), and antiphlogistic enzyme (pronase) were prescribed. After 8 months, her visual acuity improved to 0.5 in the right eye, and a marked improvement was found in the appearance of the fundus and visual fields (Figure 1). However, OCT demonstrated segmental thinning of the inner retinal layers and a disappearance of the photoreceptor outer segments in the areas corresponding to the cotton-wool spot and retinal edema (Figure 1). The rod b-wave recovered, but the a- and b-waves and oscillatory potentials of the mixed rod-cone ERGs were reduced and their implicit times were prolonged (Figure 2, Table 1). The PhNR became larger but was still smaller than that of the fellow eye. The recovery of the ERGs coincided with the morphologic recovery of the retinal OCT images.
Discussion Although the etiology of PR is unknown, it is generally associated with head or body trauma leading to embolic damage to the peripapillary retina.1 Earlier studies on patients with PR have shown damage to only the inner retinal layers that are manifested as Purtscher-flecken and cotton-wool spots. However, an involvement of the choriocapillaris was reported in a clinicopathologic study.3 At present, little information is available on the electrophysiological features of PR.4 Our case had a reduction of ERG components stemming from the inner layer in the acute phase, and the reduction was most prominent in the PhNR. In the chronic phase, the PhNR improved but still there remained some inner
Table 1. Electroretinographic Analysis of the Patient With Unilateral PR Acute Phase
Rod Mixed rod and cone Photopic flash 30-Hz flicker
Chronic Phase
Normal Range*
Component
Amplitude
Implicit Time
Amplitude
Implicit Time
Amplitude
Implicit Time
b-wave a-wave b-wave Op† a-wave b-wave PhNR
0.83 1.05 1.05 1.06 1.01 0.98 0.55 1.06
1.11 1.01 1.03 1.02 1.02 1.02 1.08 1.11
1.02 0.94 0.89 0.88 1.14 0.89 0.88 0.87
1.05 1.02 1.06 1.06 1.01 1.00 1.08 1.02
0.87–1.08 0.97–1.08 0.95–1.12 0.93–1.09 0.91–1.22 0.89–1.16 0.91–1.19 0.92–1.15
0.97–1.02 0.99–1.02 0.99–1.03 0.99–1.01 0.99–1.06 0.99–1.06 0.97–1.04 0.99–1.07
Data are shown as the ratio of each parameter in the affected eye to that in the healthy fellow eye (a/f ratio). The a/f ratio outside of the 5–95 percentile range in normal subjects is marked in bold. *Normal range is calculated from R/L ratio of normal subjects in our clinic and shown as (5 percentile–95 percentile). †Op analysis was made on the sum of each Op component. Op, oscillatory potentials.
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layer functional damage even after the ophthalmoscopic resolution of the retinal pathology. These findings indicate that the retinal impairment in our patient existed diffusely. Our case had a marked thickening of the inner retinal layers in the acute phase and a thinning in the chronic phase. In addition, OCT showed damage to the outer retina in the acute phase and disappearance of the transition between the photoreceptor outer segments and retinal pigment epithelium in the chronic phase. These morphologic findings are in good agreement with those reported by Soliman et al,5 whose patient with PR showed a disorganization of the retina between the retinal photoreceptor outer segments and the retinal pigment epithelium in addition to a thinning of the outer nuclear layer. The ERG changes in our patient can be partly accounted for by these morphologic alterations. Because PR is considered to be caused by damage in the peripapillary region of the retina, two changes observed in the full-field ERGs were unexpected. First in the acute phase, the rod b-wave was reduced but the photopic b-wave was preserved, and in the chronic phase, the rod b-wave was normal, whereas the photopic b-wave and flicker ERG were relatively reduced. In rabbits, cones are more resistant than rods to transient ischemia, but after the ischemia is resolved, cones do not recover as well as rods.6 Second, in the acute phase, the b-wave and the PhNR were reduced, but in the choronic phase, the a-wave was also subtly reduced. These findings suggest that acute choroidal circulatory disturbances might have led to outer layer damages. These findings are supported by a study that showed choriocapillaris alterations in a clinicopathologic study of a patient with PR.3 The ERG reductions were moderate, and a statistical evaluation could not be made with this one case.
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Additional studies are needed to determine whether the diffuse retinal damage suggested by the ERG findings is present in PR patients and whether the ERG changes are pathognomonic for PR. Our observations were made on a single case and cannot be expanded to other patients with PR. However, our findings highlight the value of the layer-bylayer functional analysis by ERG and the simultaneous morphologic analysis by OCT.5,7,8 Key words: electroretinogram, optical coherence tomography, photopic negative response, Purtscher retinopathy.
References 1. 2.
3.
4.
5.
6.
7.
8.
Agrawal A, McKibbin MA. Purtscher’s and Purtscher-like retinopathies: a review. Surv Ophthalmol 2006;51:129 –136. Beckingsale AB, Rosenthal AR. Early fundus fluorescein angiographic findings and sequelae in traumatic retinopathy: case report. Br J Ophthalmol 1983;67:119 –123. Kincaid MC, Green WR, Knox DL, Mohler C. A clinicopathological case report of retinopathy of pancreatitis. Br J Ophthalmol 1982;66:219 –226. Haq F, Vajaranant TS, Szlyk JP, Pulido JS. Sequential multifocal electroretinogram findings in a case of Purtscher-like retinopathy. Am J Ophthalmol 2002;134:125–128. Soliman W, Zibrandtsen N, Jørgensen T, Sander B, Alsbirk PH, Larsen M. Sequels of Purtscher’s retinopathy imaged by enhanced optical coherence tomography. Acta Ophthalmol Scand 2007;85:450 – 453. Brunette JR, Olivier P, Zaharia M, Blondeau P, Lafond G. Rod-cone differences in response to retinal ischemia in rabbit. Doc Ophthalmol 1986;63:359 –365. Holak HM, Holak NH, Schenk C, Olinger A, Holak SA. Correlation of retinal thickness with the extent of Purtscher’s retinopathy (in German). Ophthalmologe 2006;103:798 – 805. Meyer CH, Callizo J, Schmidt JC, Mennel S. Functional and anatomical findings in acute Purtscher’s retinopathy. Ophthalmologica 2006;220:343–346.
Purpose: To determine the extent of retinal damage in Purtscher retinopathy (PR). Methods: A longitudinal analysis of the retinal morphology and function was made by optical coherence tomography and electroretinography, respectively, on a patient with unilateral PR. Results: Optical coherence tomography showed a marked thickening of the inner retinal layers in the acute phase and a thinning of the inner retinal layers and alteration of the outer layers in the chronic phase. The site of these changes corresponded with the cotton-wool patches. The results of electroretinography indicated reduced inner layer function in the acute phase and partial recovery in the chronic phase. Conclusion: The inner and outer retinal layers are altered morphologically and functionally in eyes with PR. The dynamic evaluation on retinal morphology and function may be useful in understanding the pathophysiology of PR. RETINAL CASES & BRIEF REPORTS 4:55–58, 2010
both.1,2 This prompted us to conduct a longitudinal analysis of the morphologic and functional changes by optical coherence tomography (OCT) and electroretinography (ERG), respectively, in a patient with unilateral PR.
From the *Department of Ophthalmology, Oita University Faculty of Medicine, Hasama-machi, Yufu-shi, Oita, Japan; and †Centre for Ophthalmology, Institute for Ophthalmic Research, University of Tuebingen, Tuebingen, Germany.
P
urtscher retinopathy (PR) is characterized by a sudden painless loss of vision in one or both eyes associated with patches of retinal whitening and hemorrhages in the posterior pole and pallor of the optic disk. These changes usually follow head trauma, long bone fracture, or other trauma.1 The damage is thought to arise from the occlusion of arterioles by intravascular microparticles and is localized to only the inner retinal layers. However, it has not been determined whether the damage is focal or diffuse or whether it is confined to the inner or outer retina or
Case Report A 76-year-old woman noticed a sudden painless decrease in the vision in her right eye 10 days after a fracture of the sternum and right ribs in an automobile accident. She came to our clinic 16 days later when her vision did not improve. Her corrected visual acuity was 0.3 in the right eye and 0.9 in the left eye. Ophthalmoscopy showed multiple cotton-wool spots and retinal hemorrhages, and fluorescein angiography showed dye leakage from the arterioles around the optic disk in the right eye (Figure 1). Optical coherence tomography showed a thickening or irregularity of the inner retinal layers corresponding to the sites of the cotton-wool spots and retinal edema. A retinal detachment was observed at the fovea of the right eye (Figure 1). Goldmann perimetry detected a central scotoma and an enlargement of the physiologic blind spot (Figure 1). The left eye was completely normal. The ERGs recorded according to the International Society for Clinical Electrophysiology of Vision standards (http://www. iscev.org/standards/index.html) are shown in Figure 2. Because a statistical evaluation could not be made with one case, we compared the ERG responses of the affected eye with that of the fellow eye, which showed no abnormality of the fundus ophthalmoscop-
Supported by Researches on Sensory and Communicative Disorders from the Ministry of Health, Labor, and Welfare, Japan. None of the authors has a proprietary interest in any material or method mentioned. Reprint requests: Kei Shinoda, MD, PhD, Department of Ophthalmology, Oita University Faculty of Medicine, Hasama-machi, Yufushi, Oita 879-5593, Japan; e-mail: [email protected]
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2010
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VOLUME 4
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NUMBER 1
Fig. 1. Clinical findings in the acute and chronic phases of a patient with PR. A, Fundus photographs at the initial examination (acute phase) showing multiple cotton-wool spots and retinal hemorrhages in the right eye. B, Fluorescein angiogram at the initial examination showing dye leakage from the small arterioles around the optic disk in the right eye. C, Fundus photograph of the right eye taken 8 months after the initial examination showing a disappearance of the cotton-wool spots and retinal hemorrhages. The dotted lines in A and C indicate the scanning lines of the OCT scans shown in Parts D–G. D and F, OCT images taken at the initial examination (acute phase) showing a thickening and irregularity of the inner retinal layers at the sites corresponding to the cotton-wool spots and retinal edema (indicated by *), and sensory retinal detachment at the fovea (indicated by arrow) in the right eye. E and G, OCT images taken at the same period with C (chronic phase) showing a segmental thinning of the inner layer and disappearances of the outer segments (indicated by arrowheads) corresponding to the cotton-wool spot or retinal edema (E and G, respectively). H, Goldmann visual fields in the acute phase showing a central scotoma and enlargement of physiologic blind spot in the right eye. I, Goldmann visual fields performed 8 months after the onset (chronic phase) showing a central scotoma and enlargement of physiologic blind spot in the right eye.
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FUNCTION AND MORPHOLOGY OF PURTSCHER RETINOPATHY
Fig. 2. Electroretinograms recorded according to the International Society for Clinical Electrophysiology of Vision protocol in the acute (upper row) and chronic (lower row) phases. The values of the amplitudes and implicit times of each wave are shown in Table 1. In the acute phase, the amplitudes of the rod b-wave and photopic negative response (PhNR) were reduced, and the implicit times were prolonged. In the chronic phase, the rod b-wave has recovered. The PhNR became larger but was still smaller than that of the fellow eye.
ically and by fluorescein angiography. In the acute phase, the amplitudes of the scotopic b-wave and photopic negative response (PhNR) were smaller (Figure 2; Table 1), and the implicit times were prolonged. A diagnosis of unilateral PR was made, and a hemostatic agent (carbazochrome sodium sulfonate), peripheral vasodilator (kallidinogenase), and antiphlogistic enzyme (pronase) were prescribed. After 8 months, her visual acuity improved to 0.5 in the right eye, and a marked improvement was found in the appearance of the fundus and visual fields (Figure 1). However, OCT demonstrated segmental thinning of the inner retinal layers and a disappearance of the photoreceptor outer segments in the areas corresponding to the cotton-wool spot and retinal edema (Figure 1). The rod b-wave recovered, but the a- and b-waves and oscillatory potentials of the mixed rod-cone ERGs were reduced and their implicit times were prolonged (Figure 2, Table 1). The PhNR became larger but was still smaller than that of the fellow eye. The recovery of the ERGs coincided with the morphologic recovery of the retinal OCT images.
Discussion Although the etiology of PR is unknown, it is generally associated with head or body trauma leading to embolic damage to the peripapillary retina.1 Earlier studies on patients with PR have shown damage to only the inner retinal layers that are manifested as Purtscher-flecken and cotton-wool spots. However, an involvement of the choriocapillaris was reported in a clinicopathologic study.3 At present, little information is available on the electrophysiological features of PR.4 Our case had a reduction of ERG components stemming from the inner layer in the acute phase, and the reduction was most prominent in the PhNR. In the chronic phase, the PhNR improved but still there remained some inner
Table 1. Electroretinographic Analysis of the Patient With Unilateral PR Acute Phase
Rod Mixed rod and cone Photopic flash 30-Hz flicker
Chronic Phase
Normal Range*
Component
Amplitude
Implicit Time
Amplitude
Implicit Time
Amplitude
Implicit Time
b-wave a-wave b-wave Op† a-wave b-wave PhNR
0.83 1.05 1.05 1.06 1.01 0.98 0.55 1.06
1.11 1.01 1.03 1.02 1.02 1.02 1.08 1.11
1.02 0.94 0.89 0.88 1.14 0.89 0.88 0.87
1.05 1.02 1.06 1.06 1.01 1.00 1.08 1.02
0.87–1.08 0.97–1.08 0.95–1.12 0.93–1.09 0.91–1.22 0.89–1.16 0.91–1.19 0.92–1.15
0.97–1.02 0.99–1.02 0.99–1.03 0.99–1.01 0.99–1.06 0.99–1.06 0.97–1.04 0.99–1.07
Data are shown as the ratio of each parameter in the affected eye to that in the healthy fellow eye (a/f ratio). The a/f ratio outside of the 5–95 percentile range in normal subjects is marked in bold. *Normal range is calculated from R/L ratio of normal subjects in our clinic and shown as (5 percentile–95 percentile). †Op analysis was made on the sum of each Op component. Op, oscillatory potentials.
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layer functional damage even after the ophthalmoscopic resolution of the retinal pathology. These findings indicate that the retinal impairment in our patient existed diffusely. Our case had a marked thickening of the inner retinal layers in the acute phase and a thinning in the chronic phase. In addition, OCT showed damage to the outer retina in the acute phase and disappearance of the transition between the photoreceptor outer segments and retinal pigment epithelium in the chronic phase. These morphologic findings are in good agreement with those reported by Soliman et al,5 whose patient with PR showed a disorganization of the retina between the retinal photoreceptor outer segments and the retinal pigment epithelium in addition to a thinning of the outer nuclear layer. The ERG changes in our patient can be partly accounted for by these morphologic alterations. Because PR is considered to be caused by damage in the peripapillary region of the retina, two changes observed in the full-field ERGs were unexpected. First in the acute phase, the rod b-wave was reduced but the photopic b-wave was preserved, and in the chronic phase, the rod b-wave was normal, whereas the photopic b-wave and flicker ERG were relatively reduced. In rabbits, cones are more resistant than rods to transient ischemia, but after the ischemia is resolved, cones do not recover as well as rods.6 Second, in the acute phase, the b-wave and the PhNR were reduced, but in the choronic phase, the a-wave was also subtly reduced. These findings suggest that acute choroidal circulatory disturbances might have led to outer layer damages. These findings are supported by a study that showed choriocapillaris alterations in a clinicopathologic study of a patient with PR.3 The ERG reductions were moderate, and a statistical evaluation could not be made with this one case.
●
2010
●
VOLUME 4
●
NUMBER 1
Additional studies are needed to determine whether the diffuse retinal damage suggested by the ERG findings is present in PR patients and whether the ERG changes are pathognomonic for PR. Our observations were made on a single case and cannot be expanded to other patients with PR. However, our findings highlight the value of the layer-bylayer functional analysis by ERG and the simultaneous morphologic analysis by OCT.5,7,8 Key words: electroretinogram, optical coherence tomography, photopic negative response, Purtscher retinopathy.
References 1. 2.
3.
4.
5.
6.
7.
8.
Agrawal A, McKibbin MA. Purtscher’s and Purtscher-like retinopathies: a review. Surv Ophthalmol 2006;51:129 –136. Beckingsale AB, Rosenthal AR. Early fundus fluorescein angiographic findings and sequelae in traumatic retinopathy: case report. Br J Ophthalmol 1983;67:119 –123. Kincaid MC, Green WR, Knox DL, Mohler C. A clinicopathological case report of retinopathy of pancreatitis. Br J Ophthalmol 1982;66:219 –226. Haq F, Vajaranant TS, Szlyk JP, Pulido JS. Sequential multifocal electroretinogram findings in a case of Purtscher-like retinopathy. Am J Ophthalmol 2002;134:125–128. Soliman W, Zibrandtsen N, Jørgensen T, Sander B, Alsbirk PH, Larsen M. Sequels of Purtscher’s retinopathy imaged by enhanced optical coherence tomography. Acta Ophthalmol Scand 2007;85:450 – 453. Brunette JR, Olivier P, Zaharia M, Blondeau P, Lafond G. Rod-cone differences in response to retinal ischemia in rabbit. Doc Ophthalmol 1986;63:359 –365. Holak HM, Holak NH, Schenk C, Olinger A, Holak SA. Correlation of retinal thickness with the extent of Purtscher’s retinopathy (in German). Ophthalmologe 2006;103:798 – 805. Meyer CH, Callizo J, Schmidt JC, Mennel S. Functional and anatomical findings in acute Purtscher’s retinopathy. Ophthalmologica 2006;220:343–346.
