From the Department of Ophthalmology (TA, KT, JF), Loma Linda University, Loma Linda; and Riverside County Regional Medical Center (LDB), Moreno Valley, California.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Tarek Alasil, MD, Department of Ophthalmology, Loma Linda University, 11234 Anderson Street, Loma Linda, CA 92354.
Purtscher’s retinopathy is a vaso-occlusive vasculopathy originally described in patients after severe head trauma.1 Purtscher-like retinopathy (PLR) is seen in some patients with acute pancreatitis, long bone fractures, chest compression injuries, and connective tissue diseases.2 In PLR, there may be loss of visual acuity that may be accompanied by field loss in the form of central, paracentral, or arcuate scotomas.2
Biomicroscopic features of PLR may include cotton-wool spots and flame hemorrhages.1 The optical coherence tomography (OCT) findings in PLR have been described in previous case reports.1,3,4
We present a case of PLR where Humphrey visual fields and Cirrus HD-OCT (Spectral Domain Technology, Zeiss) were compared at presentation and at 6 month follow-up.
A 30-year-old male with recent hospitalization for acute pancreatitis presented with decreased vision in the right eye. His visual acuity was 20/100 right eye (OD) and 20/20 left eye (OS). A right relative afferent papillary defect was observed. Scattered cotton-wool spots in the posterior retina were seen bilaterally (Figs. 1A and 1B).
Figure 1. (A) Dilated Fundus Examination of the Right Eye Revealed Sharp Optic Nerve Margins, Scattered Cotton-Wool Spots in the Posterior Pole, and Significant Macular Edema. (B) Dilated Fundus Examination of the Left Eye Revealed Sharp Optic Nerve Margins, Scattered Cotton-Wool Spots in the Peripapillary Area, Flame-Shaped Hemorrhages, and an Anomalous Inferior Arcade Extending Superiorly into the Macula. (C) Mid-Phase Flourescein Angiogram of the Right Eye is showing Patchy Hypoflourescent Areas of Nonperfusion. (D) Mid-Phase Fluorescein Angiogram of the Left Eye with Blockage from the Intraretinal Hemorrhages. There is Subtle Leakage near the Clinical Locations of Cotton-Wool Spots.
Flourescein angiogram of the right eye showed areas of nonperfusion and late-frame leakage in the posterior retina which clinically corresponded to the cotton-wool spots (Fig. 1C). Fluorescein angiogram of the left eye demonstrated similar, but less dramatic changes (Fig. 1D). The patient was diagnosed with bilateral PLR secondary to acute pancreatitis.
Cirrus HD-OCT (Spectral Domain Technology, Zeiss) and Humphrey visual field 24–2 (VF) were obtained. OCT of the right eye demonstrated focal swelling of the retinal nerve fiber layer (RNFL) and central subretinal edema (Figs. 2A and 2B). OCT of the left eye has retinal thickening supranasally but no central edema (Fig. 2C). VF of the right eye showed a partial inferior altitudinal defect extending to the optic nerve with a supranasal deficit. The left eye showed an inferior nasal step (Fig. 3).
Figure 2. (A) Cirrus HD-OCT of the Right Eye Demonstrated Focal Swelling of the Retinal Nerve Fiber Layer RNFL, which Corresponded to the Cotton-Wool Spots Seen Clinically. Subretinal Fluid was Present Centrally. Central Subfield Thickness = 461 μm, Volume = 11.3 mm3, Average Thickness= 313 μm). (B) Cirrus HD-OCT Overlay of the Right Eye is Showing Significant Retinal Thickening Corresponding to Cotton-Wool Spots Seen Clinically with Extension Centrally to Involve the Fovea. The Peripapillary Macular Region has Marked Edema, which did not Fit on the Standard Micrometer Scale. (C) Cirrus HD-OCT Overlay of the Left Eye is Showing Macular Thickening Supranasally Corresponding to the Cotton-Wool Spots Seen Clinically. The Edema does not Extend into the Foveal Region.
Figure 3. (Right) Humphrey Visual Field (central 24–2) of the Right Eye is Showing Inferior Central, and Paracentral Changes that Extend to the Optic Nerve. There is also a Supranasal Deficit (MD = −16.00 DB, PSD = 11.93 DB). (Left) Humphrey Visual Field (central 24–2) of the Left Eye is Showing a Small Inferior Nasal Step (MD = −3.78, PSD = 4.53 DB).
Six months later, his visual acuity improved to 20/30 right eye (OD). The cotton wool spots, flame-shaped hemorrhages, and macular edema resolved (Fig. 4). Repeat VF showed persistent visual field defects that were slightly worse bilaterally (Fig. 5).
Figure 4. Cirrus HD-OCT of the Right Eye Demonstrated Resolution of the Subretinal Fluid. RPE Was Noted to be Intact (Central Subfield Thickness = 256 μm, Volume = 8.1 mm3, Average Thickness = 225 μm).
Figure 5. (Right) Humphrey Visual Field (central 24–2) of the Right Eye is Showing Inferior Arcuate Defects Extending to the Optic Nerve that are More Dense than Initial Presentation. Superior Nasal Step is Still Present. A New Supratemporal Paracentral Scotoma is also Present (MD = −16.52 DB, PSD = 12.21 DB). (Left) Humphrey Visual Field (central 24–2) of the Left Eye Showing Enlargement of the Inferior Nasal Defect (MD = −6.48, PSD = 7.01 DB).
The mechanism of retinal injury in acute pancreatitis is not entirely known as several different mechanisms may all play a role. There is an activation of complement, particularly complement C5a.6 This induces the formation of leukocyte and platelet aggregates as large as 50 μm in diameter which are sufficient in size to occlude the retinal capillaries.7 Microparticles have been shown to damage the inner retina in experimental studies.8,9 Additionally, lipase may cause endothelial damage and activation of the clotting cascade.10–15
These events can cause ischemia of the RNFL, disrupt the cellular axoplasmic transport, and cause swelling of the axons that appear clinically as cotton-wool spots. Experimental studies of Purtscher’s retinopathy and PLR have demonstrated damage to the inner retina through the occlusion of small arterioles by intravascular microparticles. The outer retina may also be involved in PLR, as seen in multifocal electroretinogram findings, possibly through secondary choriocapillaris infarctions.16
The clinical and OCT findings typically improve parallel to visual acuity recovery in PLR.3 In our case, the OCT thickening improved over time as did the visual acuity and biomicroscopic findings. Meyer et al. reported a case of Purtscher’s retinopathy, where excellent correlation was found between the OCT lesion sizes and corresponding microperimetry scotoma.1 There was no correlation between the OCT findings and the VF defects in our patient.
Because the VF defects were more peripheral and similar to ischemic optic neuropathy field deficits, it is possible that there was more wide-spread micro-infarctions caused by the pancreatitis. Both the RNFL and the optic nerve may have suffered an ischemic event secondary to the pancreatitis. Although there was never any clinical evidence of optic nerve pallor, an afferent pupil defect was noted on presentation.
The VF defects seen on presentation slightly progressed on follow-up 6 months later. Reasons for this may be that the initial micro-infarctions continued to enlarge due to release of local ischemic chemical factors, the micro-infarctions were still evolving and not fully mature when the initial VF was obtained, or that new micro-infarctions occurred subsequent to initial testing. Further studies should be done to evaluate the effectiveness of intervention early in the disease course of PLR and whether any intervention can prevent or halt further ischemic damage. There have been only a few reports with the use of systemic steroids.1,2 Other modalities, such as local anti-inflammatory medications, have not been published to date.
This demonstrates an interesting case of PLR secondary to acute pancreatitis, where OCT and VF findings were utilized throughout the follow-up. Whereas OCT may be useful as a clinical tool to monitor improvement in function over time, the initial VF testing may be a stronger indicator of long-term permanent damage. The potential permanence of visual field deficits is an important aspect of the disease to discuss with PLR patients.
Further studies with such ancillary testing may better define the pathogenesis and natural course of the disease.
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- Jacob HS, Craddock PR, Hammerschmidt DE, et al. Complement-induced granulocyte aggregation: an unsuspected mechanism of disease. N Engl J Med. 1980;302:789–794.
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