Purtscher-like retinopathy is a rare eye condition that affects the retina, the light-sensitive tissue at the back of the eye. It involves damage to the small blood vessels within the retina, leading to a sudden decrease in vision. This condition is characterized by specific changes observed during an eye examination.
Understanding the Distinction
Purtscher-like retinopathy is distinct from classic Purtscher’s retinopathy, though they share similar retinal appearances. Purtscher’s retinopathy is traditionally linked to severe head or chest trauma, and was originally described with retinal hemorrhages and whitening.
In contrast, Purtscher-like retinopathy presents with similar retinal findings but occurs in the absence of trauma. It is associated with various non-traumatic systemic medical conditions. Both conditions are believed to stem from microvascular occlusion, where small blood vessels in the retina become blocked, leading to localized tissue damage.
A key distinguishing feature is the cause, but the retinal signs often overlap. Both can feature cotton-wool spots, retinal hemorrhages, and Purtscher flecken, which are polygonal areas of retinal whitening that typically spare the perivascular region. While Purtscher flecken are considered characteristic, cotton-wool spots are also common in both conditions.
Underlying Causes and Related Conditions
Purtscher-like retinopathy can stem from a diverse range of non-traumatic systemic conditions that lead to microvascular damage in the retina. Acute pancreatitis is a commonly associated condition, with Purtscher-like retinopathy potentially indicating multi-organ failure in these cases. Pancreatic adenocarcinoma, a type of pancreatic cancer, can also be a cause.
Renal failure, preeclampsia, and conditions related to childbirth, such as HELLP Syndrome, are additional causes. Autoimmune diseases, including systemic lupus erythematosus (SLE), are also linked to the development of Purtscher-like retinopathy. In these cases, the retinopathy may signify systemic disease activity.
Fat embolism syndrome can also lead to this condition. Other causes include certain blood disorders like hemolytic uremic syndrome and cryoglobulinemia, as well as lymphoproliferative disorders. The general mechanism involves the occlusion of precapillary arterioles in the retina due to various types of emboli or inflammatory processes, such as complement activation.
Recognizing Symptoms and Diagnosis
Individuals with Purtscher-like retinopathy experience a sudden, painless decrease in vision. This vision loss can range from partial to complete and may affect one or both eyes. It is bilateral in approximately 60% of cases and almost always bilateral when associated with acute pancreatitis. Patients may also notice visual field defects, such as central, paracentral, or arcuate scotomas, while peripheral vision usually remains unaffected.
Diagnosis primarily relies on a comprehensive eye examination, particularly ophthalmoscopy, which allows a doctor to view the back of the eye. Characteristic retinal changes include cotton-wool spots, which are whitish, slightly raised patches on the inner retina, and retinal hemorrhages, often flame-shaped. Purtscher flecken, polygonal areas of retinal whitening with clear borders, are also observed.
Imaging techniques further support the diagnosis. Optical coherence tomography (OCT) can reveal hyper-reflectivity in the inner retinal layers, corresponding to cotton-wool spots and Purtscher flecken, and may show macular edema or subretinal fluid in acute stages. Fluorescein angiography (FA) can identify blocked choroidal fluorescence, occluded retinal arterioles, and areas of capillary non-perfusion, providing detailed information about blood flow in the retina.
Treatment Approaches and Prognosis
There is no specific treatment directly targeting Purtscher-like retinopathy itself; management focuses on addressing the underlying systemic medical condition. Treating the root cause, such as acute pancreatitis or an autoimmune disease, is the primary approach. In many instances, vision may improve spontaneously over several weeks to months.
However, some individuals may experience permanent vision loss or residual visual field defects, such as optic atrophy or retinal thinning, following the resolution of acute retinal changes. Factors that can influence the visual outcome include the severity of the initial retinal damage and the promptness with which the underlying systemic condition is managed. While some reports suggest potential benefits from intravenous corticosteroids, a consistent improvement in visual outcome with steroid treatment has not been demonstrated in systematic reviews.