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Purtscher's Retinopathy

Purtscher Retinopathy

Purtscher's retinopathy is a medical condition characterized by sudden and possibly severe visual impairment, in the setting of prior extraocular trauma or other systemic, non-traumatic pathologies.


Presentation

Sudden visual impairment is a key characteristic of Purtscher's retinopathy. All reported cases involve individuals who experienced symptoms, like reduced visual acuity and visual field or a combination of both with the majority of the patients reporting combined defects of visual acuity and visual field [1]. Extremely profound ocular dysfunction, such as sudden blindness, may also be induced, as can annular, central or paracentral scotomas and extensive defects occupying various segments of the visual field [2].

Ocular symptoms usually arise 1-2 days after a traumatic event or other acute, Purtscher's-retinopathy-related conditions [3] [4] [5]. The aforementioned defects associated with vision loss can be unilateral or bilateral.

Other features of Purtscher's retinopathy are due to the fact that it is strongly associated with extraocular trauma. So patients may present with evidence of head or thoracic injuries. Individuals who have developed the retinopathy without prior trauma may present with symptoms of a variety of other pathologies that have been associated with the condition. These include:

Vomiting
  • The case is described of a 32-year-old man suffering from alcoholism who came to the Emergency Unit with vomiting, fever and sharp epigastric pain irradiating to the chest and upper abdomen.[ncbi.nlm.nih.gov]
Epigastric Pain
  • The case is described of a 32-year-old man suffering from alcoholism who came to the Emergency Unit with vomiting, fever and sharp epigastric pain irradiating to the chest and upper abdomen.[ncbi.nlm.nih.gov]
Abdominal Pain
  • Six months later, he experienced intractable abdominal pain. Serum amylase and lipase levels were elevated markedly.[ncbi.nlm.nih.gov]

Workup

Fundoscopy is the first step towards achieving an accurate diagnosis of Purtscher's retinopathy. Findings consistent with this diagnosis include [4]:

  • Cotton-wool spots. These constitute restricted regions of retinal ischemia, which are observed as white areas of axonal swelling obstructing the axoplasmal flow and are detected in up to 90% of the cases [8] [9].
  • Retinal hemorrhages may appear on the superficial nerve fiber, in which case they display a flame-like shape, or on the inner retinal layers; in the latter case, they assume a "dot and blot" pattern. They are also observed in up to 90% of the affected patients.
  • "Purtscher flecken" are deemed pathognomonic for Purtscher's retinopathy but are detected in only half of the patients [9]. Purtscher flecken are polygonal regions of whitening of the retina; it is believed that they occur due to regional capillary bed infarction.
  • Normal optic disc, which tends to assume an edematous appearance later in the course of the disorder.

Another diagnostic modality that can be employed is fluorescein angiography. It is expected to illustrate regional ischemia of the retina and capillary bed, possibly accompanied by venous dilation and perivenous staining [10].

Given the fact that Purtscher's retinopathy is primarily associated with extraocular trauma, especially head or thoracic trauma, workup may include a radiograph or a computerized tomography scan (CT scan) of the affected region. In case the retinopathy is not induced by trauma, it may be linked to other diseases, such as acute pancreatitis, activated complement C5a, systemic lupus erythematosus, scleroderma, dermatomyositis and other pathologies. Therefore, laboratory tests that need to be performed to assess these possibilities encompass:

  • Serum amylase, transaminase, myoglobin, aldolase, creatine phosphokinase, urine myoglobin
  • Serum rheumatoid factor, ANA, anti-double-stranded DNA antibody
  • Complement C5a levels
Schistocytosis
  • The presence of schistocytosis and the secondary occurrence of hemolytic anemia were consistent with a diagnosis of thrombotic thrombocytopenic purpura (TTP).[pediatrics.aappublications.org]

Treatment

  • CONCLUSIONS: Both treatment and no treatment improve vision in Purtscher's retinopathy patients, but the difference between no treatment and glucocorticoid therapy had no statistical significance.[ncbi.nlm.nih.gov]
  • We review previous reports on the retinal pathophysiology of Purtscher retinopathy and discuss the potential benefit of treatment with high-dose intravenous corticosteroids.[ncbi.nlm.nih.gov]
  • However, in general, there are no treatments for Purtscher's retinopathy. If it is caused by a systemic disease or emboli, then those conditions should be treated.[en.wikipedia.org]
  • The case is presented with a discussion of the pathophysiology, diagnosis, prognosis, and treatment options for this entity.[ncbi.nlm.nih.gov]
  • There is at present no recognised treatment for the condition.[ncbi.nlm.nih.gov]

Prognosis

  • Reports on visual prognosis in the literature are not uniform.[ncbi.nlm.nih.gov]
  • However, vision recovery does occur in some cases, and reports have varied on the long-term prognosis.[en.wikipedia.org]
  • The case is presented with a discussion of the pathophysiology, diagnosis, prognosis, and treatment options for this entity.[ncbi.nlm.nih.gov]
  • The prognosis for patients with decreased vision is unpredictable.[ncbi.nlm.nih.gov]
  • Prognosis Purtscher's retinopathy can lead to loss of vision, [7] and recovery of vision may occur very little. [2] However, vision recovery does occur in some cases, and reports have varied on the long-term prognosis. [8] References Purtscher O (1912[ipfs.io]

Etiology

  • The etiology of the retinopathy is best explained by retinal vascular occlusion due either to fat embolism or thrombosis.[ncbi.nlm.nih.gov]
  • This rare phenomenon will be discussed, its literature will be reviewed, and its possible etiology will be suggested.[ncbi.nlm.nih.gov]
  • The etiology of this phenomenon has been linked to fat necrosis and involvement of complement and the immune system.[ncbi.nlm.nih.gov]
  • We review the literature on posttraumatic retinopathies; and outline the clinical characteristics of interest for injury assessment, as well as the differential diagnosis with other etiologies(AU) Responsible library: ES1.1 Localization: BNCS[pesquisa.bvsalud.org]
  • Although the patient presented with an atypical underlying etiology, he exhibited classic retinal findings for Purtscher's retinopathy.[ncbi.nlm.nih.gov]

Epidemiology

  • Scientific report Clinical science - Scientific reports Purtscher’s retinopathy: epidemiology, clinical features and outcome Ashish Agrawal 1, Martin McKibbin 2 1 External Eye Diseases & Refractive Surgery, Manchester Royal Eye Hospital, Manchester, UK[doi.org]
  • Purtscher’s retinopathy: epidemiology, clinical features and outcome. Br J Ophthalmol. 2007;91(11):1456–9. CrossRef PubMed PubMedCentral Google Scholar 2. Behrens-Baumann W, Scheurer G, Schroer H. Pathogenesis of Purtscher’s retinopathy.[link.springer.com]
  • "Purtscher's retinopathy: epidemiology, clinical features, and outcome". British Journal of Ophthalmology. 91 (11): 1456–9. doi : 10.1136/bjo.2007.117408. PMC 2095457. PMID 17556428. Schmidt, D; Otto, T (June 2004).[wikidoc.org]
  • Purtscher’s retinopathy: epidemiology, clinical features and outcome. Br J Ophthalmol 2007 ; 91 : 1456 –9.[cmaj.ca]
Sex distribution
Age distribution

Pathophysiology

  • The case is presented with a discussion of the pathophysiology, diagnosis, prognosis, and treatment options for this entity.[ncbi.nlm.nih.gov]
  • We review previous reports on the retinal pathophysiology of Purtscher retinopathy and discuss the potential benefit of treatment with high-dose intravenous corticosteroids.[ncbi.nlm.nih.gov]
  • Purtscher's retinopathy is a traumatic angiopathy with an uncertain pathophysiology. A case report and review are presented, and the role of optometry in its management is discussed.[ncbi.nlm.nih.gov]
  • […] bone trauma Acute pancreatitis Amniotic fluid embolism Chronic renal failure Dermatomyositis Fat embolism syndrome Scleroderma Systemic lupus erythematosus (SLE) Thrombotic thrombocytopenic purpura (TTP) Purtscher's retinopathy likely involves complex pathophysiology[en.wikipedia.org]
  • Pathophysiology Purtscher's retinopathy likely involves complex pathophysiology, with several contributing factors, including complement -mediated aggregates, fat, air, fibrin clots and platelet clumps. [5] The diseases leads to the formation of cotton[wikidoc.org]

Prevention

  • Treatment to prevent further occurrences may involve treating heart arrhythmias, hypertension, carotid stenosis, and high cholesterol.[authorstream.com]

References

Article

  1. Agrawal A, McKibbin M. Purtscher's retinopathy: epidemiology, clinical features and outcome. Br J Ophthalmol. 2007 Nov; 91(11): 1456–1459.
  2. Blodi BA, Johnson MW, Gass JD, Fine SL, Joffe LM. Purtscher's-like retinopathy after childbirth. Ophthalmology. 1990 Dec;97(12):1654-9.
  3. 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.
  4. Buckley SA, James B. Purtscher's retinopathy. Postgrad Med J 1996; 72: 409 -412.
  5. Purtscher O. Noch unbekannte Befunde nach Schaedeltrauma. Ber Ophthalmol Ges 1910; 36: 294-310.
  6. Marr WG, Marr EG. Some observation on Purtscher's disease: traumatic retinal angiopathy. AmJ Ophthalmol 1962; 54: 69Q-7t)5.
  7. Roden D, Fitzpatrick G, O' Donoghue H, Phelan D. Purtscher's retinopathy and fat embolism. British Journal of Ophthalmology. 1989; 73: 677-679.
  8. Proença Pina J, Ssi-Yan-Kai K, de Monchy I, Charpentier B, Offret H, Labetoulle M. Purtscher-like retinopathy: case report and review of the literature. J Fr Ophtalmol. 2008;31 (6:609).
  9. Miguel AIM, Henriques F, Azevedo LFR, Loureiro AJR, Maberley DAL. Systematic review of Purtscher's and Purtscher-like retinopathies. Eye (Lond). 2013 Jan; 27(1): 1–1.
  10. Williams DF, Mieler WF, Williams GA. Posterior segment manifestations of ocular trauma. Retina 1990; 1O(suppl 1): 35-44.

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Last updated: 2019-07-11 20:52