Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Purtscher Retinopathy Clinical Presentation

  • Author: Edward Chaum, MD, PhD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Aug 04, 2015
 

History

Patients with traumatic Purtscher retinopathy present with a recent history of blunt chest trauma or head trauma. The severity of chest trauma is not correlated directly with the incidence or severity of retinopathy, which is observed in these patients.

Patients may present with unilateral or bilateral vision loss (possibly severe) generally within 2 days.

Macular cotton-wool spots and intraretinal hemorrhages in patients with this history of trauma are diagnostic of the condition. Larger capillary bed infarcts (Purtscher flecken) are also seen in the macula and region surrounding the optic nerve. Typically, there is sparing of the retina whitening immediately adjacent to the larger retinal vessels.

Patients also may present with a Purtscher-like retinopathy in the absence of trauma. Various systemic conditions have been associated with developing the following characteristic retinal findings:

  • Patients with acute pancreatitis are at risk for developing Purtscher-like retinopathy.
  • Patients with long bone fractures are at risk for developing fat embolization and Purtscher-like retinopathy.
  • Childbirth [13]
    • Purtscher-like retinopathy may be seen in the postpartum period.[14]
    • Purtscher-like retinopathy may be due to amniotic fluid embolization with a catastrophic presentation, including shock and disseminated intravascular coagulopathy (DIC).
    • Retinal arterial occlusions with microvascular infarcts and labor-associated hemorrhage have been reported in patients with preeclampsia and Purtscher-like retinopathy.
    • Protein C and protein S deficiency have been reported.
  • Purtscher-like retinopathy has been observed in patients with certain types of systemic vasculitides.
    • The most likely etiology is microvascular infarction due to complement C5a-induced leukocyte aggregation.
    • Purtscher-like retinopathy has been reported in diverse vasculitic and related diseases, including thrombocytopenia purpura, cryoglobulinemia, hemolytic uremic syndrome, juvenile dermatomyositis, and multiple myeloma.
    • Unexplained vision loss in patients with these conditions (eg, systemic lupus erythematosus, dermatomyositis, scleroderma) should raise the possibility of Purtscher-like retinopathy.
      Purtscher-like retinopathy in a patient with syste Purtscher-like retinopathy in a patient with systemic lupus erythematosus with microvascular encephalopathy and retinopathy. Multiple cotton-wool spots of varying sizes and ages surround the optic nerve. A branch arterial occlusion is present along the supertemporal arcade with retinal whitening between the major artery and vein. Multiple occlusions are seen in arterial and venous trees with disruption of the blood columns.
Next

Physical

The most common retinal findings in Purtscher retinopathy are cotton-wool spots, Purtscher flecken around the optic nerve, and intraretinal hemorrhages.

Less common reported findings include serous detachment of the macula, preretinal hemorrhages, dilated vessels, and optic disc edema.

Confluence of cotton-wool spots in the central macula may simulate the cherry-red spot that is seen in central retinal artery occlusion.

Retinal microinfarcts that are observed in patients with fat embolization are usually smaller in size and located in the peripheral, not central, retina.

Pigment migration and optic atrophy have been reported as late findings in the disease.

Previous
Next

Causes

Traumatic chest compression and blunt head trauma are common causes. Chest trauma that is associated with Purtscher retinopathy ranges from mild to severe; the degree is not necessarily indicative of the risk of developing retinopathy. Compressive chest injuries often are seen with unrestrained drivers in motor vehicle accidents (MVAs).

Mild Purtscher retinopathy in a patient after blun Mild Purtscher retinopathy in a patient after blunt chest compression as an unrestrained driver in a motor vehicle accident. This patient presented with visual loss in the left eye and had unilateral disease. The fundus photograph shows a large cotton-wool spot along the inferotemporal arcade and more subtle microvascular injury between the disc and fovea in the central macula. Whitening of the central fovea is present. Visual acuity was 20/200 but returned to 20/30 after 6 weeks.

Patients with known vasculitic disease (eg, systemic lupus erythematosus, scleroderma, dermatomyositis) are at risk for developing a Purtscher-like retinopathy with microvascular occlusion.

Childbirth and the uncommon complication of amniotic fluid embolism are known risk factors. There have been several case reports of Purtscher retinopathy in the setting of preeclampsia without other known risk factors.[12]

Acute pancreatitis is a known risk factor. The risk from acute pancreatitis may be due to fat embolization or complement activation with secondary leukocyte aggregation and occlusive vasculopathy.

Previous
 
 
Contributor Information and Disclosures
Author

Edward Chaum, MD, PhD Professor and Director, Vitreoretinal Service, Plough Foundation Professor of Retinal Diseases, Department of Ophthalmology, Professor of Pediatrics, Anatomy and Neurobiology, and Biomedical Engineering, University of Tennessee Health Science Center Hamilton Eye Institute

Edward Chaum, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, Association for Research in Vision and Ophthalmology, American Society of Retina Specialists, American Telemedicine Association, American Academy of Ophthalmology

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

Steve Charles, MD Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Macula Society, Retina Society, Club Jules Gonin

Disclosure: Received royalty and consulting fees for: Alcon Laboratories.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Russell P Jayne, MD Consulting Vitreoretinal Surgeon, The Retina Center at Las Vegas

Russell P Jayne, MD is a member of the following medical societies: American Medical Association, American Society of Cataract and Refractive Surgery, American Society of Retina Specialists

Disclosure: Nothing to disclose.

References
  1. Agrawal A, McKibbin MA. Purtscher's and Purtscher-like retinopathies: a review. Surv Ophthalmol. 2006 Mar-Apr. 51(2):129-36. [Medline].

  2. Purtscher O. Noch unbekannte Befunde nach Schadeltrauma. Berl Dtsch Ophthal Ges. 1910. 36:294-301.

  3. Agrawal A, McKibbin M. Purtscher's retinopathy: epidemiology, clinical features and outcome. Br J Ophthalmol. 2007 Nov. 91(11):1456-9. [Medline].

  4. Lai WW, Chen AC, Sharma MC, Lam DS, Pulido JS. Purtscher-like retinopathy associated with acute renal allograft rejection. Retina. 2005 Jan. 25(1):85-7. [Medline].

  5. Bhan K, Ashiq A, Aralikatti A, Menon KV, McKibbin M. The incidence of Purtscher retinopathy in acute pancreatitis. Br J Ophthalmol. 2008 Jan. 92(1):151-3. [Medline].

  6. Parc C. Purtscher-like retinopathy as an initial presentation of a thrombotic microangiopathy associated with antineoplastic therapy. Am J Hematol. 2007 Jun. 82(6):486-8. [Medline].

  7. Coban-Karatas M, Turunc T, Altan-Yaycioglu R. Purtscher-like retinopathy related to drug-induced hypersensitivity syndrome. Ocul Immunol Inflamm. 2012 Dec. 20 (6):475-7. [Medline].

  8. Sauer A, Nasica X, Zorn F, Petitjean P, Bader P, Speeg-Schatz C, et al. Cryoglobulinemia revealed by a Purtscher-like retinopathy. Clin Ophthalmol. 2007 Dec. 1 (4):555-7. [Medline].

  9. Okwuosa TM, Lee EW, Starosta M, Chohan S, Volkov S, Flicker M, et al. Purtscher-like retinopathy in a patient with adult-onset Still's disease and concurrent thrombotic thrombocytopenic purpura. Arthritis Rheum. 2007 Feb 15. 57 (1):182-5. [Medline].

  10. Dyrda A, Matheu Fabra A, Aronés Santivañez JR, Blanch Rubio J, Alarcón Valero I. Purtscher-like retinopathy as an initial presentation of iron-deficiency anaemia. Can J Ophthalmol. 2015 Feb. 50 (1):e1-2. [Medline].

  11. Harrison TJ, Abbasi CO, Khraishi TA. Purtscher retinopathy: an alternative etiology supported by computer fluid dynamic simulations. Invest Ophthalmol Vis Sci. 2011. 52(11):8102-7.

  12. Landes A, Jay WM. Purtscher-like retinopathy in a patient with preeclampsia. Seminars in Ophthalmology. 2009. 24:217-220.

  13. Blodi BA, Johnson MW, Gass JD, Fine SL, Joffe LM. Purtscher's-like retinopathy after childbirth. Ophthalmology. 1990 Dec. 97(12):1654-9. [Medline].

  14. Stewart MW, Brazis PW, Guier CP, Thota SH, Wilson SD. Purtscher-like retinopathy in a patient with HELLP syndrome. Am J Ophthalmol. 2007 May. 143(5):886-7. [Medline].

  15. Holak HM, Holak S. Prognostic factors for visual outcome in purtscher retinopathy. Surv Ophthalmol. 2007 Jan-Feb. 52(1):117-8; author reply 118-9. [Medline].

  16. Ayaki M, Yokoyama N, Furukawa Y. Postpartum central retinal artery occlusion simulating Purtscher's retinopathy. Ophthalmologica. 1995. 209(1):37-9. [Medline].

  17. Campo SM, Gasparri V, Catarinelli G, Sepe M. Acute pancreatitis with Purtscher's retinopathy: case report and review of the literature. Dig Liver Dis. 2000 Nov. 32(8):729-32. [Medline].

  18. Chandra P, Azad R, Pal N, Sharma Y, Chhabra MS. Valsalva and Purtscher's retinopathy with optic neuropathy in compressive thoracic injury. Eye. 2005 Aug. 19(8):914-5. [Medline].

  19. Chang M, Herbert WN. Retinal arteriolar occlusions following amniotic fluid embolism. Ophthalmology. 1984 Dec. 91(12):1634-7. [Medline].

  20. Cooper BA, Shah GK, Grand MG. Purtscher's-like retinopathy in a patient with systemic lupus erythematosus. Ophthalmic Surg Lasers Imaging. 2004 Sep-Oct. 35(5):438-9. [Medline].

  21. Fischbein F, Safir A. Monocular Purtscher's retinopathy. A fluorescein angiographic study. Arch Ophthalmol. 1971 Apr. 85(4):480-4. [Medline].

  22. Gass JD. Purtscher's-like retinopathy. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment. Mo: Mosby: St. Louis; 1997. 452-454.

  23. Jacob HS, Goldstein IM, Shapiro I, Craddock PR, Hammerschmidt DE, Weissmann G, et al. Sudden blindness in acute pancreatitis. Possible role of complement-induced retinal leukoembolization. Arch Intern Med. 1981 Jan. 141(1):134-6. [Medline].

  24. Kelley JS. Purtscher's retinopathy related to chest compression by safety belts. Fluorescein angiographic findings. Am J Ophthalmol. 1972 Aug. 74(2):278-83. [Medline].

  25. Marr WG, Marr EG. Some observations on Purtscher's disease: traumatic retinal angiopathy. Am J Ophthalmol. 1962. 54:693-705.

  26. Pratt MV, De Venecia G. Purtscher's retinopathy: a clinicohistopathological correlation. Surv Ophthalmol. 1970 Mar. 14(5):417-23. [Medline].

  27. Shah GK, Penne R, Grand MG. Purtscher's retinopathy secondary to airbag injury. Retina. 2001. 21(1):68-9. [Medline].

  28. Shaikh S, Ruby AJ, Piotrowski M. Preeclampsia-related chorioretinopathy with Purtscher's-like findings and macular ischemia. Retina. 2003 Apr. 23(2):247-50. [Medline].

  29. Umlas JW, Hedges TR 3rd, Zbyszewski K. Purtscher's retinopathy in a patient with acute pancreatitis. Ophthalmic Surg Lasers. 2001 May-Jun. 32(3):263-4. [Medline].

  30. Miguel AI, Henriques F, Azevedo LF, Loureiro AJ, Maberley DA. Systematic review of Purtscher's and Purtscher-like retinopathies. Eye (Lond). 2013 Jan. 27 (1):1-13. [Medline].

 
Previous
Next
 
Characteristic fundus findings of Purtscher retinopathy. Multiple cotton-wool spots surround the optic nerve after blunt thoracic trauma.
Mild Purtscher retinopathy in a patient after blunt chest compression as an unrestrained driver in a motor vehicle accident. This patient presented with visual loss in the left eye and had unilateral disease. The fundus photograph shows a large cotton-wool spot along the inferotemporal arcade and more subtle microvascular injury between the disc and fovea in the central macula. Whitening of the central fovea is present. Visual acuity was 20/200 but returned to 20/30 after 6 weeks.
Fluorescein angiogram of a patient who sustained blunt chest compression as an unrestrained driver in a motor vehicle accident shows focal microvascular occlusion in the area of the cotton-wool spot. Mild venous leakage and staining is seen in the perifoveal capillary bed just beneath the central fovea. A small amount of fluorescein leakage is also seen beneath the fovea.
Purtscher-like retinopathy in a patient with systemic lupus erythematosus with microvascular encephalopathy and retinopathy. Multiple cotton-wool spots of varying sizes and ages surround the optic nerve. A branch arterial occlusion is present along the supertemporal arcade with retinal whitening between the major artery and vein. Multiple occlusions are seen in arterial and venous trees with disruption of the blood columns.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.