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Grand Rounds Purtscher’s Retinopathy Mark A. Ihnen, M.D. University of Louisville Department of Ophthalmology and Visual Sciences 4/4/2014

Grand Rounds Purtscher’s Retinopathy Mark A. Ihnen, M.D. University of Louisville Department of Ophthalmology and Visual Sciences 4/4/2014

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  • Grand Rounds Purtschers Retinopathy Mark A. Ihnen, M.D. University of Louisville Department of Ophthalmology and Visual Sciences 4/4/2014
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  • Presentation CC: I cant make out faces with my right eye. HPI: 40 WM c/o blurred central vision OD after being struck by a car while changing a flat tire on an interstate off-ramp. The patient also sustained multiple rib fractures/pneumothorax and a laceration to the left ear. Transported to UL Emergency Department.
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  • Presentation POH: None PMH: None Meds:None Meds: None Allergies: None
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  • Exam OD OS OD OS BCVA: 20/200 20/20 Pupils: 4 2 OU, no APD IOP: WNL OU EOM: Full OU Anterior Exam: Extensive subconjunctival hemorrhage OU, otherwise WNL
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  • Clinical Photos
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  • Dilated Fundus Exam at Bedside Fundus video OD demonstrating large peripapillary cotton-wool spots and superficial hemorrhages.
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  • Inpatient Clinical Course Patients left ear was surgically repaired Patients left ear was surgically repaired Thoracostomy tube was removed, stable for discharge. Thoracostomy tube was removed, stable for discharge. Arranged to follow-up on the day of discharge in our Retina Clinic. Arranged to follow-up on the day of discharge in our Retina Clinic.
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  • Dilated Fundus Exam: Clinic Photos Color fundus photo of the right eye demonstrating multiple, large, peripapillary, cotton-wool spots and superficial hemorrhages. Note the intervening clear zones between each CWS sparing vessels.
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  • Dilated Fundus Exam: Clinic Photos Color fundus photo of the left eye: Normal.
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  • HVF 24-2 OU HVF 24-2: Left eye: Full; Right Eye: Central scotoma. OSOD
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  • SD-OCT (OD) OCT image of right eye demonstrating elevation corresponding to large superficial cotton wool spot.
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  • SD-OCT (OS) OCT image of the left eye demonstrating normal foveal contour.
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  • FA of OD Mid phase FA of right eye demonstrating multiple areas of hypofluorescence corresponding to large CWS.
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  • FA of OD Late phase FA of right eye demonstrating multiple areas of hypofluorescence corresponding to large CWS with small amount of late leakage.
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  • FA of OS Mid phase FA of left eye within normal limits.
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  • Assessment and Plan 40 WM presenting with central scotoma OD and multiple peripapillary CWS following a thoracic compression injury. 40 WM presenting with central scotoma OD and multiple peripapillary CWS following a thoracic compression injury. DDX: DDX: Purtschers Retinopathy Purtschers Retinopathy Commotio Retinae Commotio Retinae Plan: Plan: Intravitreal Kenalog Injection Intravitreal Kenalog Injection
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  • Clinical Course Patient initially refused IVK injection and then reconsidered. Patient initially refused IVK injection and then reconsidered. Lost to follow-up. Lost to follow-up.
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  • Purtschers Retinopathy Introduction Introduction First described by Dr. Othmar Purtscher (18521927) in 1910. First described by Dr. Othmar Purtscher (18521927) in 1910. Originally observed in two severely traumatized patients with head injuries. Originally observed in two severely traumatized patients with head injuries. Fully described in a publication in 1912 by Dr. Purtscher. Fully described in a publication in 1912 by Dr. Purtscher. True Purtscher's retinopathy, as first described, is always associated with a traumatic injury. True Purtscher's retinopathy, as first described, is always associated with a traumatic injury. When there is a non-traumatic etiology the correct designation is Purtscher-like retinopathy. When there is a non-traumatic etiology the correct designation is Purtscher-like retinopathy. http://www.mrcophth.com/ophthalmologyhalloffame/purtscher.html
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  • Purtschers Retinopathy Epidemiology Epidemiology Incidence of 0.24 persons per million per year Incidence of 0.24 persons per million per year Clinical Presentation Clinical Presentation Patients present with decreased visual acuity, often sudden (usually within 48 hours) and severe (20/200 or worse) Patients present with decreased visual acuity, often sudden (usually within 48 hours) and severe (20/200 or worse) History of compression injury to chest, head or long bone fracture (fat embolism syndrome) History of compression injury to chest, head or long bone fracture (fat embolism syndrome) Fundoscopic signs include peripapillary cotton wool spots and/or superficial hemorrhages in over 92% of cases. Fundoscopic signs include peripapillary cotton wool spots and/or superficial hemorrhages in over 92% of cases. Purtscher flecken are considered pathognomic, but only occur in 50% of cases. Purtscher flecken are considered pathognomic, but only occur in 50% of cases. Typically bilateral but many times unilateral. Typically bilateral but many times unilateral.
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  • Purtscher-like Retinopathy Purtscher-like retinopathy: not associated with trauma. Purtscher-like retinopathy: not associated with trauma. Associations include: Associations include: Acute pancreatitis Acute pancreatitis Indication of multiorgan failure and is often associated with a fatal outcome Indication of multiorgan failure and is often associated with a fatal outcome Chronic renal failure Chronic renal failure Autoimmune Disease Autoimmune Disease SLE, scleroderma, dermatomyositis, Sjogren syndrome SLE, scleroderma, dermatomyositis, Sjogren syndrome Childbirth (amniotic fluid embolism) Childbirth (amniotic fluid embolism) Retrobulbar anesthesia Retrobulbar anesthesia Valsalva maneuver Valsalva maneuver
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  • Purtschers Retinopathy Diagnosis Diagnosis For trauma-related cases, the diagnosis is clinically apparent after fundus examination and no further workup is required. For trauma-related cases, the diagnosis is clinically apparent after fundus examination and no further workup is required. However, cases without trauma or causative medical condition require a comprehensive medical evaluation in conjunction with an internist. However, cases without trauma or causative medical condition require a comprehensive medical evaluation in conjunction with an internist.
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  • Purtschers Retinopathy Pathogenesis Pathogenesis Thought to be a result of injury-induced complement activation, which causes granulocyte aggregation and leukoembolization. Thought to be a result of injury-induced complement activation, which causes granulocyte aggregation and leukoembolization. This process in turn occludes small arterioles such as those found in the peripapillary retina. This process in turn occludes small arterioles such as those found in the peripapillary retina. Treatment Treatment No known effective treatment exists. No known effective treatment exists. Anecdotal reports of limited success with high dose systemic corticosteroids. Anecdotal reports of limited success with high dose systemic corticosteroids.
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  • Purtschers Retinopathy Prognosis Prognosis Although retinal whitening and hemorrhages slowly disappear over weeks to months, usually no significant recovery of vision occurs. Although retinal whitening and hemorrhages slowly disappear over weeks to months, usually no significant recovery of vision occurs.
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  • Systematic Review Systematic Review Mean visual acuity 20/200, range of 20/20 to LP. Mean visual acuity 20/200, range of 20/20 to LP. Trauma and acute pancreatitis were the most frequent etiologies. Trauma and acute pancreatitis were the most frequent etiologies. There was no statistically significant difference in VA improvement for patients treated with corticosteroids compared with observation. There was no statistically significant difference in VA improvement for patients treated with corticosteroids compared with observation. Trauma and pancreatitis were associated with higher probability of visual improvement. Trauma and pancreatitis were associated with higher probability of visual improvement.
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  • Case report : 24 WF with post partum Purtscher- like retinopathy treated with sub-tenon triamcinolone Case report : 24 WF with post partum Purtscher- like retinopathy treated with sub-tenon triamcinolone Presenting VA 20/200 OD 5 week follow-up: VA 20/60 Presenting VA 20/200 OD 5 week follow-up: VA 20/60
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  • Oral Indomethacin 25 mg/day for six weeks Oral Indomethacin 25 mg/day for six weeks 43 WM with Purtschers like retinopathy associated with valsalva maneuver: 43 WM with Purtschers like retinopathy associated with valsalva maneuver: Presenting VA CF OS 6 week followup VA 20/40 OS
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  • Thank You
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  • References 1. Atabay C, et al. Late visual recovery after intravenous methylprednisolone treatment of Purtscher's retinopathy. Ann Ophthalmol. 1993;25(9):330-333. 2.Behrens-Baumann W, Scheurer G, Schroer H. Pathogenesis of Purtscher's retinopathy. Graefes Arch Clin Exp Ophthalmol. 1992;230(3):286-291 3.Purtscher O. Ber Deutsche Ophth Ges 1910;36:294-301. 4.Jacob HS, Craddock PR, Hammerschmidt DE, Moldow CF. Complement-induced granulocyte aggregation: an unsuspected mechanism of disease. N Eng J Med. 1980;302:789-794. 5.Purtscher O. Angiopathia retinae traumatica. Lymphorrhagien des Augengrunes. Albrecht Von Graefes Arch Ophthalmol. 1912;82:347-371. 6.Scheurer G, Praetorius G, Damerau B, Behrens-Baumann W. Vascular occlusion of the retina: an experimental model. I. Leukocyte aggregates. Graefes Arch Clin Exp Ophthalmol. 1992; 230(3):275-280. 7.Maassen J, Oetting T. Purtscher's Retinopathy: 22-year-old male with vision loss after trauma. EyeRounds.org. May 18, 2005 8.BCSC: Retina and Vitreous: Purtschers Retinopathy: 105-106 9.Hsu J, Regillo CD. Distant Trauma with Posterior Segment Effects. Yanoff and Duker: Ophthalmolgoy 3 rd ed. Ch 6.43: 751-752.