Purtscher Retinopathy

What is Purtscher Retinopathy?

Purtscher retinopathy is a condition where the small blood vessels in the eye become blocked. It’s identified by multiple white spots near the optic nerve head (the point where the optic nerve enters the back of the eye) and the fovea (the center of the retina). These white spots surround the small blood vessels in the eye, which may also be linked with blood leaking inside the retina.

The main features of Purtscher retinopathy include Purtscher flecken (white patches on the retina), cotton wool spots (small, fluffy white spots on the retina), and slight bleeding within the retina. The treatment depends on the cause of the condition. The effectiveness of systemic steroids (medication to reduce inflammation) in treating this condition needs further investigation.

Typically, Purtscher retinopathy often shows up after a severe decrease in vision following a head injury. This was first described by Otmar Purtscher, hence the name. In his initial description, he noticed it was linked with multiple white patches on the surface of the retina, bleeding in the retina, and swelling of the optic disc (the area where the optic nerve enters the eye).

If someone shows signs of Purtscher retinopathy but doesn’t have a history of clear trauma, it’s called Purtscher-like retinopathy.

What Causes Purtscher Retinopathy?

Purtscher retinopathy is damage to the retina of the eyes, which can be linked with trauma or surgery. Some incidents that could lead to this include serious head injuries, pressure on the chest, fractures of long bones like the thigh bone, dislocation of the shoulder or fractures in the upper arm. Other factors could be barotrauma, which is injury caused by changes in pressure, and harm to infants through physical abuse.

Similar eye damage can occur, known as Purtscher-like retinopathy, due to other health problems such as acute or chronic pancreatitis (inflammation of the pancreas), pancreatic adenocarcinoma (a type of pancreas cancer), and embolism (obstruction of a blood vessel by air, amniotic fluid, or fat). Conditions like chronic kidney failure, severe eye failure associated with squamous cell carcinoma of the cervix (a form of cervical cancer), rejections after kidney transplants, and nephrotic syndrome (a kidney disorder causing your body to leak large amounts of protein into the urine) can also be causes.

This particular type of retinopathy can also be associated with connective tissue disorders like systemic lupus erythematosus (an autoimmune disease), scleroderma (a group of diseases affecting the connective tissues), dermatomyositis (a muscle disease), and thrombotic thrombocytopenic purpura (a rare blood disorder). It can also be related to other conditions such as cryoglobulinemia in hepatitis C (an abnormality of proteins in the blood), hemolytic uremic syndrome (a condition that affects blood and blood vessels), preeclampsia (high blood pressure and signs of damage to another organ system) and HELLP syndrome (a syndrome that affects pregnant women).

An array of activities and events like weight lifting, childbirth, the Valsalva maneuver (blowing forcefully with the nose and mouth closed), and severe allergic reactions could also be associated with these conditions. Additionally, it may be linked to certain drugs and treatments like Hodgkin lymphoma after a bone marrow transplant and antineoplastic therapy (cancer treatments), hypersensitivity to drugs, Gemcitabine therapy, and after coil embolization (a procedure to treat aneurysms).

Conditions like adult-onset Still disease (form of arthritis accompanied by fever and rash), pemphigus vulgaris (a rare skin condition), and adult-onset Still disease can also be causes. Even some surgeries like prostatectomy (removal of the prostate), endonasal dacryocystorhinostomy (a procedure to treat a blocked tear duct), and vascular surgery can be associated with it. It’s also noticed in some cases after getting a cosmetic “Brazilian booty” procedure or from COVID-19. Once more, even orthopedic surgeries could be a cause for Purtscher or Purtscher-like retinopathy.

Risk Factors and Frequency for Purtscher Retinopathy

Purtscher retinopathy, a condition affecting the retina of the eye, affects roughly 0.24 out of every million people each year. However, because many cases do not show symptoms, the actual number of people with Purtscher retinopathy could be higher.

Signs and Symptoms of Purtscher Retinopathy

Purtscher retinopathy typically begins with a sudden decrease in vision, which is not painful, in both eyes within two days following a traumatic event. The severity of eye involvement doesn’t necessarily match the severity of the chest trauma.

In some cases, even without trauma, a condition resembling Purtscher retinopathy may appear. It’s important to note that these instances can be related to various disorders. Therefore, a thorough medical history is crucial, especially in ruling out pancreatitis and systemic lupus erythematosus. In about 60% of cases, both eyes are affected. If only one eye seems affected, subtle changes may be present in the other eye. There is generally no direct eye trauma and no visible blockages in the eye’s arteries.

A set of diagnostic criteria has been suggested for Purtscher retinopathy:

• Purtscher flecken – polygonal patches of whitening at the back of the eye
• A small to moderate number of retinal hemorrhages (1 to 10)
• Cotton-wool spots, typically in the back of the eye
• A probable or plausible explanatory cause
• Additional tests that support the diagnosis

The affected eye area can be divided into zones:

• Zone A – extends horizontally for four disc diameters on either side of the fovea
• Zone B – extends to the outer edge of the eye
• Zone C – extends to the very edge of the retina

A broad definition of Purtscher retinopathy includes a range of combinations including related health problems like acute pancreatitis, chest injury, and areas of polygonal retinal whitening. They also have cotton wool spots in one or both eyes with minimal associated retinal hemorrhage, and no obvious blockages in the large retinal vessels or any direct eye trauma.

Purtscher flecken are characterized by multiple patches of whitening around the optic disc or the back of the eye. Cotton wool spots are whitish, slightly raised patches on the inner surface of the retina positioned along the retinal nerve fiber layer. These lesions have blurry edges and sit superficial to retinal vessels.

Additional features of Purtscher retinopathy might include disc swelling, fluid accumulation in the macula, arterial blockage, or the perception of a bright red spot in the center of the visual field. Additionally, serious fluid detachment of the macula is usually a feature in cases related to pregnancy-induced high blood pressure or other causes of severe high blood pressure. A preretinal hemorrhage is a rare feature.

Visual acuity can range from 20/20 (normal) to only being able to perceive light. The patient might complain about an area in their field of vision where they can’t see (central or paracentral scotoma).

Testing for Purtscher Retinopathy

If your doctor suspects you have a condition called Purtscher retinopathy, which happens mostly due to severe trauma, they might suggest several eye tests. These tests could help them understand the condition of your eyes better. For instance, they may use a fundus fluorescein angiogram (FFA), a special procedure that uses a fluorescent dye to illuminate the blood vessels in the back of your eye. This can help them spot any blockages or leaks in the blood vessels, as well as areas where blood flow may be poor or nonexistent.

Another test is the indocyanine green angiogram. This test uses a special green dye to examine your choroid, a layer of blood vessels and connective tissue in the back of your eye. If an area appears less green (or hypocyanescent), it indicates a lack of blood flow which may contribute to poor vision.

Optical coherence tomography (OCT) is yet another test used. It’s an imaging technique that captures high-resolution pictures of your eye’s tissues using light waves. In the early stages of the condition, OCT might reveal unusually bright areas in your retina (the light-sensitive layer at the back of your eye), along with fluid buildup and swelling. Later on, it might show thinning of the retina and loss of light-sensing cells.

The OCT angiography test can be useful to show areas in your eye with poor blood flow. It can also help track if and when these areas start getting better, which would lead to improvement in your vision.

Your doctor might also recommend a multifocal electroretinogram to assess how well the different layers of your retina are functioning. Lastly, a visual field test can indicate any blind spots you might have and how much of your peripheral vision remains.

If there’s no history of severe trauma, your doctor might want to rule out a similar condition, Purtscher-like retinopathy. They may check levels of certain enzymes to rule out inflammation of the pancreas and particular autoimmune disorders. They might also recommend imaging tests to check for pancreas-related problems or cancer, and tests for certain proteins associated with inflammation and clotting.

Remember, early detection of eye problems can lead to more effective treatment. So, if you have any concerns about your vision following trauma, it’s essential to reach out to your healthcare provider for an evaluation.

Treatment Options for Purtscher Retinopathy

Managing Purtscher retinopathy, an illness that affects the retina leading to sudden vision loss, primarily involves treating the underlying cause and providing additional treatments to support healing. From analysis and reviews of different treatment methods, it has been found that using steroids doesn’t show any significant difference in improving vision when compared to just monitoring the condition.

However, some studies suggest that a high dose of methylprednisolone (a type of steroid), administered intravenously and subsequently taken orally, may lead to improvements in vision and the structure of the affected area. This may work by stabilizing the small blood vessels in the eye, helping neuronal membranes and preventing the clumping together of a type of white blood cell known as granulocytes.

For cases where there is swelling in the central part of the retina, known as macular edema, medicines used to inhibit unwanted growth of blood vessels, such as bevacizumab, may prove useful.

If Purtscher-like retinopathy appears in individuals with lupus, an autoimmune condition where the body’s immune system attacks its own tissues, it often signifies that the lupus is more active. These cases need systemic steroids (taken orally and possibly given intravenously) and treatments to suppress the immune system.

In an unusual case where Purtscher-like retinopathy occurred alongside a blood disorder called atypical hemolytic uremic syndrome, treatment with eculizumab, a drug that prevents a part of your immune system known as the complement system from harming the body’s own cells, has been successful.

Other potential treatments for this retinopathy include oral nonsteroidal anti-inflammatory drugs such as indomethacin, and a drug to widen the blood vessel called papaverine hydrochloride. Future research to manage thrombotic microangiopathy, which is a crucial part of many cases, is crucial. Bortezomib, a drug used to treat certain types of cancer, can decrease an antibody known as ADAMTS-13, which plays a key role in thrombotic microangiopathy, and might serve as a potential treatment option in these cases.

What else can Purtscher Retinopathy be?

When trying to diagnose Purtscher-like retinopathy, which is a disease that affects the back part of the eye, there are several other conditions that doctors may need to rule out since they can have similar symptoms. These include:

• Hypertensive retinopathy, which may also show multiple ‘cotton wool spots’ on the eye, but lacks a specific kind of spots known as Purtscher flecken. Other signs: changes at the points where arteries and veins cross, swelling of the optic disc, flame-shaped bleeding, swelling of the disc, hardened deposits in the nasal part of the fovea, and hypertensive choroidopathy.
• Lupus retinopathy, a condition associated with systemic lupus erythematosus, a disease that affects multiple systems in the body.
• Partial central retinal arterial occlusion.
• Endogenous endophthalmitis, which is an inflammation inside the eye.
• Retinitis, an inflammation of the retina.
• Giant cell arteritis, a disease that causes inflammation in the arteries.
• HIV retinopathy, eye problems caused by HIV infection.
• Ischemic central retinal venous occlusion, a blockage of the vein that drains blood from the eye.
• Post-febrile retinitis, inflammation of the retina following a fever.
• Commotio retinae, damage to the eyes from a blunt force trauma.

What to expect with Purtscher Retinopathy

The outcome for your vision can vary greatly, and in severe cases, the chances of recovery are usually low. Certain signs might suggest a poorer prognosis, these include:

– damage to the macula (the part of the eye responsible for sharp, central vision),
– extended period of acute changes in the retina (the light-sensitive area at the back of the eye),
– swelling of the optic disk (the area where the optic nerve enters the eye),
– reduced blood flow in the choroid (the layer of blood vessels and connective tissue between the white of your eye and the retina),
– severe lack of blood flow in the retinal capillaries (tiny blood vessels in the retina),
– having had an episode of Purtscher retinopathy (a rare condition involving sudden decrease of vision) in the same eye before, and
– damage in the outer retina (the outer layer of the retina).

In a study reporting on 15 cases of people with eye damage caused by road accidents with or without a broken long bone, chest compression, and acute pancreatitis, only one person’s vision ended up worse than when they first presented for treatment. With no treatment but only observation and time, nearly one quarter of the eyes improved by at least 4 lines on an eye chart, and half improved by at least 2 lines.

Factors connected with a higher risk of death when suffering from this type of retinopathy include TTP (a blood clotting disorder), cancer, cryoglobulinemia (a condition involving abnormal proteins in the blood), and systemic lupus erythematosus (an autoimmune disease affecting many systems in the body).

Possible Complications When Diagnosed with Purtscher Retinopathy

The lasting effects of Purtscher retinopathy or Purtscher-like retinopathy include:

• Deterioration of the optic nerve (optic atrophy/optic disc pallor)
• Damage to the pigment cells in the retina (retinal pigment epithelial atrophy)
• Thinning of the layer of nerve fibers in the retina (thinning of the retinal nerve fiber layer)
• Thinning of the fovea, a small depression in the retina where visual perception is highest (foveal thinning)
• Narrowing or covering of retinal vessels (attenuation or sheathing of retinal vessels)

Preventing Purtscher Retinopathy

If you have pancreatitis, it’s important to avoid alcohol. This can help prevent further damage to your pancreas. Additionally, using seat belts can help you prevent injuries when you’re traveling by car. These injuries could potentially lead to a condition called Purtscher retinopathy, which affects the eyes. So, seat belts not only keep us safe during accidents, but may also protect us from certain eye conditions.