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:
Entire Body System
- HELLP Syndrome
View Article PubMed Google Scholar Stewart MW, Brazis PW, Guier CP, Thota SH, Wilson SD: Purtscher-like retinopathy in a patient with HELLP syndrome. Am J Ophthalmol. 2007, 143: 886-887. 10.1016/j.ajo.2006.12.005. [jmedicalcasereports.biomedcentral.com]
Purtscher-like retinopathy in a patient with HELLP syndrome. Am J Ophthalmol 2007;143:886-7. 5. Agrawal A, McKibbin MA. Purtscher′s and Purtscher-like retinopathies: A review. Surv Ophthalmol 2006;51:129-36. 6. [meajo.org]
Tung CF, Peng YC, Chen GH, Chow WK, Yang DY, Hu WH (2001) Hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome with acute cortical blindness. Zhonghua Yi Xue Za Zhi (Taipei). 64 (8):482–485 Google Scholar 6. [doi.org]
Purtscher-like retinopathy in a patient with HELLP syndrome. Am J Ophthalmol. 2007 May. 143(5):886-7. [Medline]. Holak HM, Holak S. Prognostic factors for visual outcome in purtscher retinopathy. [emedicine.medscape.com]
- Fever
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]
A 23-year-old man with a history of heavy alcohol intake for past 1 year reported with complaints of vomiting, fever, and sharp epigastric pain radiating to the back and flanks. The symptoms started 3 h back and were nonremitting. [ojoonline.org]
Six weeks prior to ophthalmic evaluation, the patient presented to the emergency department with fever, cough, and progressive dyspnea. [hindawi.com]
Dengue Fever Presenting as Purtscher-like Retinopathy. Ocul Immunol Inflamm. 2018. 26 (5):660-665. [Medline]. Durrani AK, Mehta VJ, Kim SJ. Postinfluenza Purtscher-like Retinopathy with Acute Macular Neuroretinopathy. [emedicine.medscape.com]
Skin
- Purpura
purpura Accepted October 27, 2011. [pediatrics.aappublications.org]
Zurück zum Zitat Power MH, Regillo MC, Custis PH (1997) Thrombotic thrombocytopenic purpura associated with Purtscher retinopathy. [springermedizin.de]
[…] several non-traumatic systemic diseases. [2] Purtscher's retinopathy may also be associated with acute pancreatitis, vasculitis, embolization of such materials as fat and amniotic fluid, [3] systemic lupus erythematosus, thrombotic thrombocytopenic purpura [wikidoc.org]
Musculoskeletal
- Arthritis
Arthritis Rheum. 2007 Feb 15. 57 (1):182-5. [Medline]. 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. [emedicine.medscape.com]
Arthritis Rheum 2007; 57 (1): 182–185. 18. Holak HM, Holak S. Prognostic factors for visual outcome in Purtscher retinopathy. Surv Ophthalmol 2007; 52 (1): 117–118 with author reply 118–9. 19. Parc C. [nature.com]
Eyes
- Cotton Wool Spots
The patient was diagnosed with Purtscher's retinopathy in the right eye due to cotton-wool spots observed during fundus examination, an air embolism discovered on chest X-ray, and a history of head/chest trauma. [ncbi.nlm.nih.gov]
McLeod D: Why cotton wool spots should not be regarded as retinal nerve fibre layer infarcts. Br J Ophthalmol 2005;89:229–237. [karger.com]
wool spots and/or superficial hemorrhages in over 92% of cases. [slideplayer.com]
- Retinal Lesion
The retinal lesions gradually settle down over weeks and then the retina may appear normal. In severe cases, pigmentary changes and optic atrophy may ensue. [emj.bmj.com]
"Retinal lesions following long bone fractures". Ophthalmology. 92 (3): 370–4. doi : 10.1016/s0161-6420(85)34023-x. PMID 3991127. ↑ Buckley, Sally A.; James, B (July 1996). "Purtscher's retinopathy". [wikidoc.org]
"Retinal lesions following long bone fractures.". Ophthalmology. 92 (3): 370–4. doi : 10.1016/s0161-6420(85)34023-x. PMID 3991127. ↑ Buckley, Sally A.; James, B (July 1996). "Purtscher's retinopathy.". [ipfs.io]
"Retinal lesions following long bone fractures". Ophthalmology. 92 (3): 370–4. doi:10.1016/s0161-6420(85)34023-x. PMID 3991127. External links[edit] [en.wikipedia.org]
Zurück zum Zitat Chuang EL, Miller FS, Kalina RE (1985) Retinal lesions following long bone fractures. Ophthalmology 92: 370–374 PubMed Chuang EL, Miller FS, Kalina RE (1985) Retinal lesions following long bone fractures. [springermedizin.de]
- Visual Impairment
Sudden visual impairment is a key characteristic of Purtscher's retinopathy. [symptoma.com]
Abstract This case study describes a patient with multiple trauma associated with acute visual impairment. Funduscopic examination revealed scattered and confluent cotton wool exudates bilaterally. [ncbi.nlm.nih.gov]
Abstract Purtscher retinopathy is an occlusive microangiopathy associated with traumatic causes and usually causes visual impairment. For non-traumatic origins, it is called Purtscher-like retinopathy (PLR). [pubmed.ncbi.nlm.nih.gov]
American Journal of Ophthalmology 134(1): 125-128, July, 2002 Purtscher's retinopathy: a case of visual impairment associated with multiple trauma. [eurekamag.com]
Patients brought to the emergency department with multiple trauma should have a complete eye examination including dilated fundus examination, as patients with a mild degree of visual impairment may not be detected[ 3 ]. [djo.org.in]
Psychiatrical
- Suggestibility
This case suggests that HBO therapy may be a treatment option for Purtscher's retinopathy. [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
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]
Pathogenesis and neuroprotective treatment in Purtscher's retinopathy. Jpn J Ophthalmol 1998;42:318–322. 9. Atabay C, Kansu T, Nurlu G. Late visual recovery after intravenous methylprednisolone treatment of Purtscher's retinopathy. [synapse.koreamed.org]
The appearance of PLR may be associated with the implantation of an endovascular stent for the treatment of intracranial aneurysms. [pubmed.ncbi.nlm.nih.gov]
Prognosis
Reports on visual prognosis in the literature are not uniform. [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 [wikidoc.org]
Etiology
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]
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. [slideplayer.com]
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. Br J Ophthalmol 2007 ; 91 : 1456 –9. [cmaj.ca]
"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]
Pathophysiology
The case is presented with a discussion of the pathophysiology, diagnosis, prognosis, and treatment options for this entity. [ncbi.nlm.nih.gov]
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
Using intravital fluorescence microscopy, we observed PMN aggregation and embolization in the mesenteric vessels of rats given intra-arterial infusions of ZAP; this was also prevented by pretreatment with 30 mg/kg MP. [jci.org]
Treatment to prevent further occurrences may involve treating heart arrhythmias, hypertension, carotid stenosis, and high cholesterol. [authorstream.com]
"Force majeure" term will be regarded as outside of the concerned party's reasonable control and any situation that "Turkiye Klinikleri" cannot prevent even though it shows due diligence. [turkiyeklinikleri.com]
Few studies reported strategies to prevent selection bias, information bias, or other bias. Risk of bias in each study for each of those variables can be requested to the corresponding author. Figure 2 Risk of bias graph. [nature.com]
References
- Agrawal A, McKibbin M. Purtscher's retinopathy: epidemiology, clinical features and outcome. Br J Ophthalmol. 2007 Nov; 91(11): 1456–1459.
- Blodi BA, Johnson MW, Gass JD, Fine SL, Joffe LM. Purtscher's-like retinopathy after childbirth. Ophthalmology. 1990 Dec;97(12):1654-9.
- 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.
- Buckley SA, James B. Purtscher's retinopathy. Postgrad Med J 1996; 72: 409 -412.
- Purtscher O. Noch unbekannte Befunde nach Schaedeltrauma. Ber Ophthalmol Ges 1910; 36: 294-310.
- Marr WG, Marr EG. Some observation on Purtscher's disease: traumatic retinal angiopathy. AmJ Ophthalmol 1962; 54: 69Q-7t)5.
- Roden D, Fitzpatrick G, O' Donoghue H, Phelan D. Purtscher's retinopathy and fat embolism. British Journal of Ophthalmology. 1989; 73: 677-679.
- 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).
- 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.
- Williams DF, Mieler WF, Williams GA. Posterior segment manifestations of ocular trauma. Retina 1990; 1O(suppl 1): 35-44.