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      Purtscher-Like Retinopathy

      case-report

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          Abstract

          Purtscher-like retinopathy is associated with retinal hemorrhages and ischaemia probably due to the complement-mediated leukoembolization. It is a rare and severe angiopathy found in conditions such as acute pancreatitis. Case. We present a case of a 53-year-old man who presented with a Purtscher-like retinopathy associated with the development of acute pancreatitis in the context of a Klatskin tumour (a hilar cholangiocarcinoma). The ophthalmologic evaluation revealed the best corrected visual acuity (BCVA) of 20/32 in the right eye (RE) and of 20/40 in the left eye (LE); biomicroscopy of anterior segment showed scleral icterus and fundoscopy revealed peripapillary cotton-wool spots, optic disc edema, and RPE hypo- and hyperpigmentation in the middle peripheral retina in both eyes with an intraretinal hemorrhage in the LE. 15 months after the initial presentation, without ophthalmological treatment, there was an improvement of BCVA to 20/20 in both eyes and optical coherence tomography (OCT) revealed areas of reduction of retinal nerve fiber layer thickness corresponding to the previous cotton-wool spots. Conclusion. Purtscher-like retinopathy should not be neglected in complex clinical contexts. Its unclear pathophysiology determines an uncertain treatment strategy, but a meticulous follow-up is compulsory in order to avoid its severe complications.

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          Most cited references16

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          Purtscher's and Purtscher-like retinopathies: a review.

          Purtscher's retinopathy is a rare condition, seen in patients with a history of trauma or other systemic disease. The clinical appearance would suggest embolic occlusion of the pre-capillary arterioles as the most likely pathogenesis, although other factors leading to blood-vessel damage may also contribute. The diagnosis is made on clinical grounds and supported by intravenous fluorescein angiography. Without treatment, the majority of patients recover some of their visual function. Treatment with systemic steroids may improve visual outcome in some patients but at present there is little evidence to support such treatment routinely.
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            Purtscher's retinopathy: epidemiology, clinical features and outcome.

            To study the incidence, systemic associations, presenting features and natural history of Purtscher's retinopathy in the UK and Ireland. Cases were collected prospectively by active surveillance through the British Ophthalmological Surveillance Unit. Clinical details were obtained using an incident questionnaire, with follow-up at 1 and 6 months. Clinical details were obtained for 15 cases over 12 months. These were associated with road traffic accidents in 6 cases, chest compression in 6 cases and acute pancreatitis in 3 cases. All cases were symptomatic and presented with loss of visual acuity, visual field or a combination. Bilateral involvement was noted in 9 cases. The acute retinal signs of cotton wool spots, retinal haemorrhage and Purtscher flecken cleared within 1 month in 26% of eyes and within 6 months in all eyes. The most common chronic signs were optic disc pallor and atrophy of the retinal pigment epithelium. Without treatment, 50% of eyes improved by at least 2 Snellen lines at final follow-up and 23% improved by at least 4 Snellen lines. Only 1 of the 24 eyes had a final acuity worse than that recorded at presentation. Purtscher's retinopathy is a rare but sight-threatening eye condition, most commonly seen in young or middle-aged men and after trauma. Spontaneous visual recovery of at least 2 Snellen lines is seen in half of the cases.
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              Corticosteroids inhibit complement-induced granulocyte aggregation. A possible mechanism for their efficacy in shock states.

              Granulocyte (PMN) aggregation and embolization may underlie complement (C)-mediated organ dysfunction in such syndromes as hemodialysis neutropenia and Purtscher's ischem;c retinopathy. Because of clinical and pathologic parallels, we have further suggested a role for this phenomenon in the genesis of the adult respiratory distress syndrome (ARDS). Because corticosteroids are commonly used in immune diseases, and have particularly been claimed efficacious in shock and ARDS, we tested the capability of methylprednisolone (MP), hydrocortisone (HC), and dexamethasone (DEX) to inhibit PMN aggregation. Aggregation engendered in vitro by zymosan-activated plasma (ZAP) was inhibited by MP and HC at concentrations approximating plasma levels achieved with the large bolus (30 mg/kg i.v) therapy advocated in shock states; DEX was almost without effect. 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. Steroid inhibition of aggregation seemed not to involve disruption of receptor function, because aggregation induced by alternative agents, n-formyl-Met-Leu-Phe and the ionophore A23187, was also inhibited by MP. Moreover, corticosteroid inhibition of PMN prostaglandin synthesis is also an unlikely explanation for our results, since aspirin and ibuprofen failed to block aggregation and arachidonic acid neither effected aggregation itself nor ameliorated the steroid effect. Our studies provide a plausible rationale for the empiric observation that high-dose corticosteroids may benefit patients with syndromes associated with microvascular leukostasis.
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                Author and article information

                Journal
                Case Rep Ophthalmol Med
                Case Rep Ophthalmol Med
                CRIOPM
                Case Reports in Ophthalmological Medicine
                Hindawi Publishing Corporation
                2090-6722
                2090-6730
                2015
                6 January 2015
                : 2015
                : 421329
                Affiliations
                Department of Ophthalmology, Hospital de Santo António, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, Portugal
                Author notes

                Academic Editor: Maurizio Battaglia Parodi

                Author information
                http://orcid.org/0000-0001-9148-2786
                Article
                10.1155/2015/421329
                4302349
                25632360
                509e5694-150c-4d42-9ded-76a1be53f007
                Copyright © 2015 Rita Massa et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 19 October 2014
                : 18 December 2014
                Categories
                Case Report

                Ophthalmology & Optometry
                Ophthalmology & Optometry

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