Purtscher Retinopathy and Purtscher-like Retinopathy 

Updated: Jul 19, 2019
Author: Edward Chaum, MD, PhD; Chief Editor: Andrew G Lee, MD 

Overview

Background

Purtscher retinopathy is a hemorrhagic and vasoocclusive vasculopathy, which, in 1912, was first described as a syndrome of sudden blindness associated with severe head trauma. These patients had findings of multiple white retinal patches and retinal hemorrhages that were associated with severe vision loss.[1, 2, 3]

Since its original description, Purtscher retinopathy has been associated with traumatic injury, primarily blunt thoracic trauma and head trauma, and numerous nontraumatic diseases.

Characteristic fundus findings of Purtscher retino Characteristic fundus findings of Purtscher retinopathy. Multiple cotton-wool spots surround the optic nerve after blunt thoracic trauma.

Purtscher-like retinopathy is seen in diverse conditions, including acute pancreatitis; fat embolization; amniotic fluid embolization; preeclampsia; hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome; and vasculitic diseases, such as lupus.

Pathophysiology

The original findings of white lesions in the retina associated with intraretinal and preretinal hemorrhages and papillitis were believed to be caused by lymphatic extravasation from trauma. These lesions are known as Purtscher flecken (larger infarcts of the retinal capillary bed) and cotton-wool spots (small retinal microinfarcts at the level of the nerve fiber layer). Fluorescein leakage in Purtscher retinopathy suggests that an acute endothelial cell injury is caused by trauma, possibly predisposing the retinal vessels to occlusion.[4, 5, 6]

The exact pathophysiology remains somewhat controversial, and different mechanisms have been proposed. The condition has been associated with various vasculopathies, including cryoglobulinemia, iron-deficiency anemia, thrombotic thrombocytopenic purpura, and drug-induced hypersensitivity, among others.[7, 8, 9, 10]

The most accepted mechanism is leukoembolization that causes arterial occlusion and infarction of the microvascular bed. Leukocyte aggregation, which is induced by complement C5a, is believed to be the most likely mechanism of embolization because of its known association with trauma, acute pancreatitis, and vasculitic diseases. Recent OCT findings in cases of Purtscher-like retinopathy also support the microembolization hypothesis.

Other possible sources of emboli include fat emboli in cases of long bone fractures and perhaps pancreatitis from enzymatic digestion of omental fat, amniotic fluid embolization during childbirth and postpartum, air emboli from traumatic chest compression, and granulocyte aggregation resulting from complement activation.

Other proposed mechanisms of vascular occlusion include angiospasm resulting from an acute rise in venous pressure from compressive chest injuries or possibly acute head injuries and endothelial cell damage resulting from acutely increased intraluminal pressure. A recent paper modeling shear stress in the retina suggests that Purtscher retinopathy may be a rheological event at a retinal posterior pole foci of vascular endothelial dysregulation, followed by downstream endothelin-induced vasculopathy.[11]

Epidemiology

Frequency

United States

Not reported

International

Not reported

Mortality/Morbidity

Bilateral manifestations are seen most commonly, but unilateral findings have been reported.

Decreased vision occurs in the affected eyes, generally in the range of 20/200 to counting fingers. Vision often improves over several months to a range of 20/30 to 20/200, depending on the severity of the retinal findings. Case reports of Purtscher retinopathy associated with preeclampsia have demonstrated poor visual outcomes in more than one half of these patients.[12]

Race

Not reported

Sex

Not reported

Age

Not reported

Prognosis

Visual prognosis is guarded, although initially decreased vision may improve over a period of months.[13]

The most important prognostic finding that is associated with a poor visual prognosis is central macular infarction.

Patient Education

Educate patients regarding the mandatory use of seat belts in cases of blunt trauma.

 

Presentation

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:

  • Patients with acute pancreatitis are at risk for developing Purtscher-like retinopathy.

  • Patients with long bone fractures are at risk for developing fat embolization and Purtscher-like retinopathy.

  • 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.

  • 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 syste 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 blun 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.

Patient perception of visual deficits in an eye wi Patient perception of visual deficits in an eye with Purtscher retinopathy
 

DDx

Diagnostic Considerations

In addition to its known and reported association with pancreatitis, pregnancy and trauma, Purtscher-like retinopathy has now been reported to be associated with many systemic conditions including hemolytic uremic syndrome,[16] myocardial infarction,[17] viral infections,[18, 19] and stem cell transplantation.[20] Other recently reported causes include gemcitabine therapy,[23] synthetic cannabinoids,[22] and injection of drugs and other compounds.[23, 24]

Differential Diagnoses

 

Workup

Laboratory Studies

Amylase level: Purtscher-like retinopathy is associated with acute pancreatitis; thus, an elevated amylase level may be diagnostic of this condition.

Complement C5a level: Activated complement C5a is associated with the development of Purtscher-like retinopathy in numerous conditions. This test may be diagnostic in presentations without an antecedent history of trauma.

A positive antinuclear antibody (ANA) test is helpful in establishing the diagnosis of certain collagen vascular diseases, such as lupus (>95% positive) and scleroderma (about 40% positive). Dermatomyositis rarely shows a positive ANA.

Similarly, anti–double-stranded DNA antibody is frequently positive in lupus (>75% positive) and scleroderma (about 15-50% positive).

Rheumatoid factor is positive in approximately 40% of patients with dermatomyositis.

Evidence of muscle disease and breakdown may be present in patients with dermatomyositis, to include the following:

  • Elevated serum transaminase

  • Elevated serum creatine phosphokinase

  • Elevated serum aldolase

  • Elevated serum myoglobin

  • Elevated urine myoglobin

In patients with lupus, antiphospholipid antibodies may be associated with an increased risk of retinal vascular thrombosis.

Imaging Studies

If the patient has a history of head trauma or thoracic trauma, obtain appropriate x-ray films or imaging studies.

Skull or rib fractures may be present.

Severe crushing injuries of the chest in people who are unrestrained during MVAs may be associated with severe deep vascular injury, cardiac or pulmonary contusion, and abdominal organ injury. These injuries may require more extensive investigation.

Other Tests

Electromyographic (EMG) studies may be helpful in establishing the diagnosis of dermatomyositis.

Procedures

Fluorescein angiography studies (early in the disease) demonstrate capillary leakage and staining of the retinal arteries. In severe disease, the following are often noted together:

  • Nonperfusion of the small arterioles that surround the central macula

  • Perivenous staining

  • Venous dilation and leakage

    Fluorescein angiogram of a patient who sustained b 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.

Histologic Findings

Histopathologic examination of an eye with prior Purtscher retinopathy shows evidence of inner retinal atrophy, which is consistent with inner retinal arterial occlusion. Late findings include nerve fiber layer dropout and evidence of optic atrophy.

 

Treatment

Medical Care

No proven treatment exists for Purtscher retinopathy that occurs after traumatic injury.

In patients with retinopathy due to systemic vasculitis, steroid therapy is theoretically beneficial.

Control of the underlying disease with other medications may be indicated.

Surgical Care

Provide surgical care as required for traumatic chest and head injuries that are associated with Purtscher retinopathy.

Consultations

Refer as indicated, based on the etiology of the retinopathy.

Activity

No restrictions are required, unless indicated by severe bilateral vision loss.

Prevention

Since blunt chest trauma is a common cause of Purtscher retinopathy, educate people to wear seat belts. This effort may reduce the risk of people being unrestrained in MVAs.

Further Outpatient Care

Further outpatient care is as indicated by the medical workup.

A retinal specialist or a general ophthalmologist should provide follow-up care to patients with Purtscher retinopathy until the condition is shown to be stable and does not require laser treatment for ischemic complications. Thereafter, the patient can be followed by a general eye care practitioner.

Further Inpatient Care

Further inpatient care is as indicated by the workup for trauma or systemic disease.

 

Medication

Medication Summary

No proven beneficial medical therapy for this condition is available. Treat the underlying conditions that have contributed to disease development.