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:
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Patients with acute pancreatitis are at risk for developing Purtscher-like retinopathy.
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Patients with long bone fractures are at risk for developing fat embolization and Purtscher-like retinopathy.
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Childbirth [14]
Purtscher-like retinopathy may be seen in the postpartum period. [15]
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.
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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 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.
Physical
The most common retinal findings in Purtscher retinopathy are cotton-wool spots, Purtscher flecken around the optic nerve, and intraretinal hemorrhages.
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 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.
Case reports of Purtscher-like retinopathy have now described associations with many systemic conditions, including hemolytic uremic syndrome, [16] myocardial infarction, [17] dengue and other viral infections, [18, 19] and stem cell transplantation. [20] Other recently reported causes include gemcitabine therapy, [21] synthetic cannabinoids, [22] periocular drug injections, [23] , and injection of polymethyl methacrylate, termed "Brazilian booty retinopathy," in association with paracentral acute middle maculopathy. [24]
Physical Examination
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.
Complications
Vision loss due to Purtscher and Purtscher-like retinopathy results from infarction of nerve fiber layers and damage to the retinal ganglion cells, which communicate visual inputs from the retina to the brain. The location and size of these infarcts relative to the central macula directly affect the visual deficits caused by the disease. The image below was created by a patient (photographer) with unilateral Purtscher retinopathy following a motor vehicle accident. It demonstrates his visual deficit after resolution of the acute injury.
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Characteristic fundus findings of Purtscher retinopathy. Multiple cotton-wool spots surround the optic nerve after blunt thoracic trauma.
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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.
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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.
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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.
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Patient perception of visual deficits in an eye with Purtscher retinopathy
