Unilateral Purtscher's retinopathy

Daniel Fuentes-Perez; Marlon Garcia-Roa; Ariadna Silva-Lepe; Miguel Vazquez-Membrillo

doi:10.4103/pajo.pajo_79_21

CASE REPORT

Year : 2021 | Volume : 3 | Issue : 1 | Page : 12

Unilateral Purtscher's retinopathy

Daniel Fuentes-Perez¹, Marlon Garcia-Roa¹, Ariadna Silva-Lepe², Miguel Vazquez-Membrillo¹
¹ Department of Retina and Vitreous, Instituto Mexicano de Oftalmologia, Queretaro, Mexico
² Department of Neurophthalmology and Electrophysiology, Instituto Mexicano de Oftalmologia, Queretaro, Mexico

Date of Submission	25-Jan-2021
Date of Acceptance	10-Feb-2021
Date of Web Publication	07-Apr-2021

Correspondence Address:
Dr. Miguel Vazquez-Membrillo
Department of Retina and Vitreous, Instituto Mexicano de Oftalmologia, Queretaro
Mexico

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pajo.pajo_79_21

Abstract

We report herein a case of a 17-year-old male who presented status postmotor vehicle accident resulting in severe polytrauma and reduced visual acuity in his right eye (OD). Best-corrected visual acuity (BCVA) was 20/400 OD and 20/20 in his left eye (OS). Slit-lamp examination confirmed normal anterior segment anatomy in both eyes (OU). Indirect ophthalmoscopy and subsequent fluorescein angiography confirmed the presence of multiple cotton-wool spots, intraretinal hemorrhages, a well-circumscribed area of macular whitening, and vascular tortuosity throughout the posterior pole and peripapillary region, all suggestive of an arterio-occlusive event OD, with a normal fundus examination OS. Imaging of the macula with optical coherence tomography demonstrated hyperreflectivity of the inner retina, along with subfoveal irregularities of the outer retinal segments OD. Systemic steroid therapy was initiated, and after 1 month, BCVA improved to 20/70 OD.

Keywords: Optical coherence tomography, polytrauma, Purtscher's retinopathy, retina

How to cite this article:
Fuentes-Perez D, Garcia-Roa M, Silva-Lepe A, Vazquez-Membrillo M. Unilateral Purtscher's retinopathy. Pan Am J Ophthalmol 2021;3:12

How to cite this URL:
Fuentes-Perez D, Garcia-Roa M, Silva-Lepe A, Vazquez-Membrillo M. Unilateral Purtscher's retinopathy. Pan Am J Ophthalmol [serial online] 2021 [cited 2023 Mar 27];3:12. Available from: https://www.thepajo.org/text.asp?2021/3/1/12/313168

Introduction

Purtscher's retinopathy (PR) was first described in 1910 by Otmar Purtscher, who reported retinal changes associated with bilateral visual loss in a middle-aged man, after a severe head injury.^[1] Despite the severity of the visual loss, the patient was able to spontaneously recover without intervention.^[2],[3] Since the original description of PR, systemic vasculopathies that result in a similar constellation of findings, but not associated with trauma, have been termed “Purtscher-like retinopathy” .^[2],[4] The incidence of PR has been estimated at 0.24 people per million per year,^[1] however, it is believed that this may be an underestimation given the often asymptomatic nature of the pathology.^[1],[4] PR is characterized by acute, often bilateral (~60%) traumatic vasculopathy, with notable findings including retinal hemorrhages, cotton-wool spots, and areas of retinal whitening between arterioles and venules known as Purtscher flecken.^[2] Recent advancements in microvascular retinal imaging with optical coherence tomography angiography (OCT-A) provide a noninvasive modality to resolve and dynamically measure blood flow in retinal vascular plexuses.^[5] We present here a case of PR after severe head trauma.

Case Report

A 17-year-old male status post rollover motor vehicle accident presented for ophthalmological evaluation with a chief complaint of severe vision loss OD in the setting of polytrauma and acute head injury. His best-corrected visual acuity (BCVA) was measured to be 20/400 OD, and 20/20 OS, with full versions and no evidence of gaze restriction or cranial nerve palsy. His pupillary examination was positive for a brisk relative afferent pupillary defect OD. Slit-lamp examination of the anterior segment was within normal limits OU, however, indirect ophthalmoscopy OD of the posterior pole and parapapillary region demonstrated diffuse cotton-wool spots, intraretinal hemorrhages, vascular tortuosity, and engorgement, along with macular pigmentary changes and FFA findings [Figure 1]a, [Figure 1]b, [Figure 1]c. Retinal imaging studies with macular OCT (Revo NX, Optopol Tech., Zawiercie, Poland) demonstrated focal thinning and irregularities of the outer limiting membrane and ellipsoid zone at the level of the fovea [Figure 1]d. OCT-A (Revo NX, Optopol Tech., Zawiercie, Poland) demonstrated microvascular nonperfusion in superficial, deep, and choriocapillaris plexuses. Ophthalmic examination and imaging OS was noted to be within normal limits. The patient received oral prednisone treatment (0.6 mg/kg/day) with taper over the course of 1 month. At his 2-week follow-up visit, his vision improved slightly to 20/200 OD. Fundoscopic examination revealed resolution of intraretinal hemorrhages and improvement of the retinal lesions [Figure 2]a, with a macular OCT demonstrating restitution of the layers of the outer retina [Figure 2]b and [Figure 2]c. His BCVA at 1 month was found to be 20/70 OD, and fundoscopic evaluation showed further improvement of the retinal lesions [Figure 3]a. Further analysis with fluorescein angiography (FA) demonstrated persistence of peripapillary hypofluorescence phenomena and OCT B improvement OD [Figure 3]b and [Figure 3]c. OCT B-scan progression analysis is shown in [Figure 4] documenting the dynamic structural changes noted over this time period.

Figure 1: (a) Cotton.wool spots, intraretinal hemorrhages, and vascular tortuosity. (b) Arteriolar occlusion, capillary nonperfusion in early phase of fluorescein angiography. (c) Stanning and leakage at late phase. (d) OLM and ellipsoid area irregularity at subfoveal level and hyperreflectivity of the inner layers

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Figure 2: (a) Week 2 retinography, cotton.wool spots and intraretinal hemorrhage reduction. (b and c) Optical coherence tomography (white line corresponds to B and blue line corresponds to C) showing improvement of the external retina lesions and persistence of the internal retina hyperreflectivity

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Figure 3: (a) Week 4 retinography, reduction in retinal lesions. (b) Fluorescein angiography showing less persistence of peripapillary hypofluorescent lesions. (c) Optical coherence tomography showing resolution of the external retinal lesions with slight persistence of the lesions in the nerve fiber layer

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Figure 4: Multiple macular optical coherence tomography images showing the evolution over time of the changes in the ellipsoid area and at the inner layers. (a) At presentation. (b) 15 days. (c) 30 days

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Discussion

Purtscher and Purtscher-like retinopathy are clinical diagnoses made when a constellation of findings are seen associated with visual loss of variable severity. Fundoscopic findings commonly include cotton-wool spots (93%), intraretinal hemorrhages (65%), and pathognomonic lesions representing occlusion of precapillary arterioles known as Purtscher flecken or Purtscher spots (50%-“63%).^[3] These ~ 50 μm lesions are seen as polygonal areas of retinal whitening near the junction of retinal arteries and precapillary arterioles.^[1],[6] Purtscher flecken are furthermore usually multiple, discrete, variable in size, and predominantly occur in areas of vaso-occlusive susceptibility such as the peripapillary and macular regions.^[7] This is in contrast to cotton-wool spots that are characterized as being more superficial with more poorly defined borders. Inflammation of the optic nerve, as well as retinal edema and the appearance of perifoveal retina whitening (pseudo-cherry-red spot), can be seen in the initial phase but are less common.^[7] Unilateral presentation, as in our case, has been described in approximately 40% of cases,^[1] and has been seen in association with facial trauma, periorbital corticosteroid injection, retrobulbar anesthesia, and Valsalva maneuver.^[8]

The pathogenesis of PR is yet to be formally elucidated. Occlusion and infarction of the precapillary arteriolar bed could be secondary to complement activation with subsequent leukocyte aggregation and microembolism of the retinal arterioles.^[2] The most accepted theory is a microembolization and occlusive event secondary to fat, platelet, or leukocyte aggregation.^[2],[3]

In a previously reported case series, within 2 months of the injury, 40% of patients with PR had complete resolution of retinal findings with a normal fundoscopic examination. However, 64% had clinical signs of optic atrophy, 23% had alterations of the retinal pigment epithelium, 14% had retinal thinning, and 4% had narrowing of the retinal arteries.^[1] Poor prognostic signs included optic disc edema, and FA findings such as choroidal hypoperfusion, external retina involvement, lack of retinal capillary perfusion, and prolonged leakage of retinal lesions. The absence of macular edema or a pseudo-cherry-red spot is associated with better visual outcomes at 6 months.^[7]

Treatment of PR with systemic corticosteroids remains controversial. Several reports conclude that corticosteroid treatment is not associated with significant visual improvement. They suggest the preferred course to be simple observation, or treatment of the underlying etiology, thus obviating the risk of adverse systemic side effect.^[1] Yet, other reports conclude that systemic corticosteroid therapy can hasten visual recovery by stabilizing the damaged neuronal membrane and microvascular channels, in addition to inhibiting granulocyte aggregation related to complement activation.^[9] Given the significant variability in the mechanism and severity of PR, it is intuitive that uniform consensus on a preferred treatment does not exist. It is likely that PR associated with thrombotic microangiopathy will be less responsive to steroid when compared to inflammatory etiologies such as complement activation.

In the literature, there are two case reports of unilateral PR successfully treated with combined IV and oral steroids, with improvements in BCVA to 20/40 from a baseline of finger counting.^[2],[4] In our case, oral prednisone alone was used, and recovery of three Snellen lines was seen within a month of the initial injury. Of note, Purtscher flecken were not seen at presentation, and the cotton-wool spots and preretinal hemorrhages had not resolved completely 1 month after the initial injury. OCT-A demonstrated microvascular nonperfusion of superficial, deep, and choriocapillaris plexuses, which partially resolved but persisted in location even after the resolution of the cotton-wool spots [Figure 5]. As much more is learned about the natural history and pathophysiology of PR from these newer imaging modalities, improvements in treatment algorithms will become possible.

Figure 5: (a) Superficial, (b) Deep retinal, and (c) Choriocapillaris plexuses on optical coherence tomography angiography at presentation, multiple irregular areas of capillary nonperfusion superotemporal and inferotemporal to the disc. (d-f) Week 4, with a partial reduction of nonperfusion areas

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgment

We thank Yolanda Villalpando-Gómez, MD, Veronica Romero-Morales, MD, and Paulina Ramirez-Neria, MD, from the Retina and Vitreous Department, Instituto Mexicano de Oftalmología (IMO), Querétaro, México. This work received technical support from Rafael González-Flores, IMO, and from Luis Aguilar, Alejandro De León, Carlos Flores, and Jair García of the Laboratorio Nacional de Visualización Científica Avanzada, UNAM. We thank Fernando Pérez-Pérez, MD head of the Ophthalmopediatric Department, IMO. This work received support from Nu Nu Kyin, MSIV, and from Anurag Shrivastava, MD, from the Department of Ophthalmology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY, USA.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Agrawal A, McKibbin M. Purtscher's retinopathy: Epidemiology, clinical features and outcome. Br J Ophthalmol 2007;91:1456-9.
2.	Agrawal A, McKibbin MA. Purtscher's and Purtscher-like retinopathies: A review. Survey Ophthalmol 2006;51:129-36.
3.	Burton TC. Unilateral Purtscher's retinopathy. Ophthalmology 1980;87:1096-105.
4.	Wang AG, Yen MY, Liu JH. Pathogenesis and neuroprotective treatment in Purtscher's retinopathy. Jpn J Ophthalmol 1998;42:318-22.
5.	Gil P, Raimundo M, Marques JP, Póvoa J, Silva R. Optical coherence tomography angiography characterization of acute and late stage Purtscher retinopathy. Eur J Ophthalmol 2018;28:NP1-6.
6.	Holló G. Frequency of Purtscher's retinopathy. Br J Ophthalmol 2008;92:1159.
7.	Holak HM, Holak S. Prognostic factors for visual outcome in Purtscher retinopathy. Surv Ophthalmol 2007;52:117-8.
8.	Yusuf IH, Watson SL. Purtscher retinopathies: Are we aiming at the wrong target? Eye 2013;27:783-5.
9.	Hammerschmidt DE, White JG, Craddock PR, Jacob HS. Corticosteroids inhibit complement-induced granulocyte aggregation. A possible mechanism for their efficacy in shock states. J Clin Invest 1979;63:798-803.