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In the case of a nontraumatic injury, the terminology commonly used is “Purtscher-like retinopathy.” It is associated with various systemic diseases, including acute pancreatitis, pancreatic adenocarcinoma, thrombotic thrombocytopenic purpura, connective tissue disorders, and hemolytic uremic syndrome.
We report a case of Purtscher-like retinopathy associated with carotid artery dissection.
Report of a Case
A 40-year-old woman presented with sudden-onset vision loss in her right eye. Two days earlier she had an ischemic stroke of her right middle cerebral artery secondary to a right carotid artery dissection, which was confirmed by computed tomography angiography (Fig. 1A). Her medical history included uterine fibroids and iron-deficiency anemia. Her ocular history was unremarkable.
At presentation, her visual acuities measured 20/100 OD and 20/40 OS, improving to 20/60 OD and 20/20 OS with pinhole. Her intraocular pressures were within normal limits OU and she had a relative afferent pupillary defect OD. Her anterior segment examination was normal OU. Fundus examination revealed intraretinal hemorrhages, Roth spots, cotton-wool spots, Purtscher flecken OD (Fig. 1B) and a normal examination OS (Fig. 1C). Spectral domain optical coherence tomography revealed thickening and hyper-reflectivity of the inner retina, consistent with paracentral acute middle maculopathy (Fig. 2A). At 6-month follow-up her visual acuity improved to 20/25 OD with pinhole, and optical coherence tomography showed full resolution of the paracentral acute middle maculopathy lesions with mild parafoveal inner nuclear layer atrophy (Fig. 2B).
To our knowledge, this is the first reported case of Purtscher-like retinopathy secondary to carotid artery dissection. In young adults, carotid artery dissection is responsible for up to 20% of strokes.
Less common ophthalmologic features include transient monocular vision loss, nonarteritic ischemic optic neuropathy, posterior ischemic optic neuropathy, central retinal artery occlusion, ocular ischemic syndrome, and ocular motor nerve palsies.
The visual acuity loss in Purtscher-like retinopathy is variable, it can be bilateral or unilateral, and it may occur hours to days after the onset of injury or associated illness. Fundus findings are characterized by cotton-wool spots, retinal hemorrhages, optic disc swelling, and Purtscher flecken. Purtscher flecken are a pathognomonic finding and appear as polygonal areas of retinal whitening with well-defined margins, between arterioles and venules.
Management usually involves observation and treatment of the underlying etiology. Other treatments have been reported with variable success, including corticosteroids, papaverine hydrochloride, hyperbaric oxygen, pentoxifylline, and low-molecular-weight heparin. Notwithstanding these anecdotal reports, the benefit over observation has still not been clearly established.
The visual prognosis can be varied depending on etiology. Agrawal and McKibbin reported that optic disc swelling or leakage and capillary nonperfusion on fluorescein angiography, as well as involvement of the outer retina, are associated with a poor long-term outcome.
The pathogenesis of Purtscher and Purtscher-like retinopathy has been an area of considerable controversy given its lack of a precise etiology. The most accepted theory is from Agrawal and Mckibbin, who suggest that the pathogenesis involves embolic occlusion of the precapillary arterioles.
In our patient, it is postulated that microemboli from the carotid dissection were the source of occlusion. The embolic phenomenon observed in the eye is exceedingly rare after carotid artery dissection, as there is a reversal of flow within the ophthalmic artery to supply the brain during the dissection.