Grand Rounds Purtscher’s Retinopathy Mark A. Ihnen, M.D. University of Louisville Department of Ophthalmology and Visual Sciences 4/4/2014 Presentation CC: “I can’t 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. Presentation POH: None PMH: None Meds: None Allergies: None Exam BCVA: OD 20/200 OS 20/20 Pupils: 4 2 OU, no APD IOP: WNL OU EOM: Full OU Anterior Exam: Extensive subconjunctival hemorrhage OU, otherwise WNL Clinical Photos Dilated Fundus Exam at Bedside Fundus video OD demonstrating large peripapillary cotton-wool spots and superficial hemorrhages. Inpatient Clinical Course Patient’s left ear was surgically repaired Thoracostomy tube was removed, stable for discharge. Arranged to follow-up on the day of discharge in our Retina Clinic. 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. Dilated Fundus Exam: Clinic Photos Color fundus photo of the left eye: Normal. HVF 24-2 OU OS HVF 24-2: Left eye: Full; Right Eye: Central scotoma. OD SD-OCT (OD) OCT image of right eye demonstrating elevation corresponding to large superficial cotton wool spot. SD-OCT (OS) OCT image of the left eye demonstrating normal foveal contour. FA of OD Mid phase FA of right eye demonstrating multiple areas of hypofluorescence corresponding to large CWS. FA of OD Late phase FA of right eye demonstrating multiple areas of hypofluorescence corresponding to large CWS with small amount of late leakage. FA of OS Mid phase FA of left eye within normal limits. Assessment and Plan 40 WM presenting with central scotoma OD and multiple peripapillary CWS following a thoracic compression injury. DDX: Purtscher’s Retinopathy Commotio Retinae Plan: Intravitreal Kenalog Injection Clinical Course Patient initially refused IVK injection and then reconsidered. Lost to follow-up. Purtscher’s Retinopathy Introduction First described by Dr. Othmar Purtscher (1852–1927) in 1910. Originally observed in two severely traumatized patients with head injuries. Fully described in a publication in 1912 by Dr. Purtscher. 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. http://www.mrcophth.com/ophthalmologyhalloffame/purtscher.html Purtscher’s Retinopathy Epidemiology Incidence of 0.24 persons per million per year Clinical Presentation 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) 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. Typically bilateral but many times unilateral. Purtscher-like Retinopathy Purtscher-like retinopathy: not associated with trauma. Associations include: Acute pancreatitis Indication of multiorgan failure and is often associated with a fatal outcome Chronic renal failure Autoimmune Disease SLE, scleroderma, dermatomyositis, Sjogren syndrome Childbirth (amniotic fluid embolism) Retrobulbar anesthesia Valsalva maneuver Purtscher’s Retinopathy Diagnosis 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. Purtscher’s Retinopathy Pathogenesis 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. Treatment No known effective treatment exists. Anecdotal reports of limited success with high dose systemic corticosteroids. Purtscher’s Retinopathy Prognosis Although retinal whitening and hemorrhages slowly disappear over weeks to months, usually no significant recovery of vision occurs. Systematic Review Mean visual acuity 20/200, range of 20/20 to LP. 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. Trauma and pancreatitis were associated with higher probability of visual improvement. 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 Oral Indomethacin 25 mg/day for six weeks 43 WM with Purtscher’s like retinopathy associated with valsalva maneuver: Presenting VA CF OS 6 week follow–up VA 20/40 OS Thank You 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: Purtscher’s Retinopathy: 105-106 9. Hsu J, Regillo CD. Distant Trauma with Posterior Segment Effects. 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