Purtscher Retinopathy - Risk Factors, Symptoms, Diagnosis, and Management

Introduction

Purtscher retinopathy is a rare and severe ocular condition. Otmar Purtscher first described it in 1910 in a man who suffered cranial trauma after falling from a tree. Purtscher retinopathy is associated with severe head trauma and can result in sudden blindness caused by the activation of compliments. Traumatic chest compression and blunt head trauma are related to this condition. Trauma can range from mild to severe. This condition is usually bilateral. It is seen in young or middle-aged men or women. Prognosis is variable and depends on the extent of retinal involvement and the diagnosis and treatment as early as possible.

What Is the Pathogenesis of Purtscher Retinopathy?

• Purtscher-like retinopathy is a rare and severe angiopathy that starts after some hours to some days after the initiation of the systemic disease.

• This condition is characterized by cotton-wool spots in the posterior pole, retinal hemorrhages, and Purtscher flecken (areas of retinal whitening) in the acute phase.

• Purtscher flecken are polygonal areas of whitening in the inner retina between the retinal arterioles and venules.

• These polygonal white areas have a clear zone between the affected retina and an adjacent arteriole and extend for an average of 50 μ on either side of retinal arteries and precapillary arterioles.

• These lesions are pathognomonic of this condition.

• These lesions must be differentiated from cotton wool spots on the superficial surface of vessels and have ill-defined borders.

• Optic disc swelling (swelling of the optic nerve), retinal edema, and pseudo-cherry spot can also be seen in this disease, significantly if the macula is affected. In the majority of cases, the condition is bilateral.

Who Is at Risk of Developing Purtscher Retinopathy?

• Patients with acute pancreatitis.

• Patients with long bone fractures.

• It is seen in the postpartum period.

• Shock and disseminated intravascular coagulopathy (a severe disorder in which proteins that control blood clotting become overactive) can also lead to Purtscher retinopathy.

• Patients with preeclampsia (a complication in pregnancy in which there is high blood pressure and an increased amount of protein in the urine) are predisposed to this condition.

• Protein C and protein S deficiency are associated with this condition.

• It can occur because of complement C5a-induced leukocyte aggregation.

• It is seen in conditions like thrombocytopenia purpura (an autoimmune disorder that starts affecting platelets), cryoglobulinemia (presence of abnormal proteins in the blood), hemolytic uremic syndrome (disorder in which blood vessels in the kidney become damaged and inflamed), juvenile dermatomyositis (seen in children affecting muscles and skin), and multiple myeloma (cancer of plasma cells).

• It is also associated with systemic lupus erythematosus (autoimmune system affecting the heart, kidney, joints, lungs, skin, and blood cells), dermatomyositis (disorder in which muscle weakness and skin rash can occur), and scleroderma (tightening and hardening of the skin).

• Some studies suggest this condition is associated with certain systemic diseases such as hemolytic uremic syndrome, myocardial infarction (which occurs because of lack of blood supply to the heart), dengue, other viral infections, and stem cell transplantation.

• Gemcitabine therapy, synthetic cannabinoids, periocular drug injections, and injection of polymethyl methacrylate are also associated with this condition.

What Are the Symptoms Associated With Purtscher Retinopathy?

• Hemorrhage in the retina.

• Whitening in the retina.

• Diminished visual acuity (decrease in sharpness of the vision).

• Visual field loss is also there.

• Sudden blindness.

What Are the Complications Associated With Purtscher Retinopathy?

• Vision loss can occur because of infarction of nerve fiber layers.

• It can cause damage to the ganglion cells of the retina and cause blindness in patients.

What Is the Differential Diagnosis of Purtscher Retinopathy?

1. Occlusion of Central Retinal Artery: Blockage occurs in the central retinal artery, leading to a cherry red spot.

2. Reaction Due to Drug: An abnormal immune system reaction to a medication.

3. Temporal Arteritis: Called giant cell arteritis and defined as inflammation of the arteries.

4. Increased Blood Pressure: An increase in blood pressure can also predispose to this condition.

5. Iatrogenic: Illness caused by medical examination or treatment.

6. Non-Ulcerative Inflammation: Interstitial keratitis in which there is non-ulcerative inflammation of corneal stroma.

7. HIV Infection Ocular Manifestations: Microangiopathy is the most common ocular manifestation.

8. Associated With Sjogren Syndrome: A disorder of the immune system that causes dry eyes and mouth.

9. Associated With Terson Syndrome: Intraocular hemorrhage associated with intracerebral bleeding.

What Is the Diagnosis of Purtscher Retinopathy?

• A complete ophthalmologic examination is performed to diagnose the condition.

• Optical coherence tomography is done to diagnose the condition. It shows hyperreflectivity in the inner retinal layers corresponding to cotton-wool spots and depicts varying degrees of macular edema.

• Fluorescein angiography is also done to show slight early masking of choroidal fluorescence in the affected area with subsequent arteriolar leakage. Choroidal hypofluorescence may persist up to five months after diagnosis.

• Electroretinography can also help in diagnosing the condition.

• The funduscopic examination also reveals bilaterally multiple retinal cotton wool spots and diagnoses edema in the macula.

• Purtscher-like retinopathy is also associated with acute pancreatitis, so there will be an increased level of amylase, and it helps in the diagnosis of the condition.

• There is activated complement 5a associated with Purtscher-like retinopathy in numerous conditions. The test is carried out when patients have a history of trauma.

• A positive antinuclear antibody test helps diagnose collagen vascular diseases like lupus and scleroderma. Dermatomyositis rarely shows positive antinuclear tests.

• Anti–double-stranded DNA antibodies are positive in patients with lupus and scleroderma.

• Rheumatoid factor is positive in patients suffering from dermatomyositis.

What Is the Treatment of Purtscher Retinopathy?

• High doses of intravenous steroids are used to treat Purtscher retinopathy.

• Corticosteroids are used to treat the condition as they stabilize damaged neuronal membranes and microvascular channels.

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

• Surgical care is also recommended for chest and head injuries.

• Papaverine hydrochloride and hyperbaric oxygen are used to treat the condition.

Conclusion

Purtscher retinopathy is an occlusive microvasculopathy that causes sudden vision loss and also blindness. It is a life-threatening situation, and it is essential to recognize it early. Some patients show spontaneous remission. The exact mechanism of etiology is unknown in this condition. The pathognomonic of this condition is the presence of cotton-wool spots, retinal hemorrhages, or Purtscher flecken. Corticosteroids have been considered a potential treatment. Nonsteroidal anti-inflammatory drugs and hyperbaric oxygen have also been proven to treat the condition well.