In 1910, Otmar Purtscher first reported it in a middle-aged patient with head trauma from a fall from a tree. It is a rare disease, a retinal vascular occlusive disease associated with trauma. It is classified as a remote traumatic retinopathy caused by trauma to areas other than the eye, such as head, neck, or chest contusions.
Similar conditions associated with systemic diseases, surgical procedures such as retrobulbar anesthesia, acute pancreatitis, thrombotic thrombocytopenic purpura, renal failure, or connective tissue disease are distinguished as Purtscher-like retinopathy. Fundus findings are common to both, and the pathophysiology is similar.
The estimated incidence is 0.24 per million per year, with possible underreporting4)6). About 60% are bilateral, and occasionally unilateral cases occur. When caused by acute pancreatitis, almost all cases are bilateral. Lesions are confined to the posterior pole (peripapillary and macular area) in 83–92% of cases. Trauma is the most frequent cause, followed by acute pancreatitis.
The causes of Purtscher-like retinopathy are diverse. In addition to acute pancreatitis, renal failure, collagen disease, pregnancy-induced hypertension/HELLP syndrome, fat embolism syndrome, Valsalva maneuver, hemolytic uremic syndrome, shaken baby syndrome, retrobulbar anesthesia, and steroid injections, cases have been reported in recent years due to COVID-19 infection1)9), hypertensive emergency2), filler injection3), ischemic colitis4), vaccination5), and C3 glomerulopathy8).
Cases caused by trauma such as head injury, chest compression, or long bone fracture are called Purtscher retinopathy. Cases caused by systemic diseases without trauma, such as acute pancreatitis or renal failure, are called Purtscher-like retinopathy. The fundus findings are common to both, and the treatment strategy is the same.
Visual impairment develops hours to days after the onset of trauma or related disease. The degree of visual impairment varies from very mild to hand motion vision. Visual field defects may also occur, presenting as central scotoma, paracentral scotoma, or arcuate scotoma.
Findings localized to the posterior pole (peripapillary and macular area) are characteristic.
Purtscher flecken
Shape: Polygonal, well-demarcated whitening.
Location: Inner retinal layers between arterioles and venules. Seen around retinal arteries.
Border: Clear border within 50 μm from the vessel. Perivascular sparing, where the retina around the vessel remains clear, is typical.
Frequency: Observed in about 63% of cases.
Pathophysiology: Inner retinal whitening due to precapillary arteriolar occlusion in the capillary bed.
Soft Exudate (Cotton-Wool Spot)
Shape: Fluffy white patch with indistinct borders.
Location: Focal infarction within the nerve fiber layer (NFL). Common around the optic disc.
Border: Indistinct and irregular.
Frequency: The most common finding, observed in 93% of cases.
Pathophysiology: Due to microinfarction within the NFL.
Lesions are classified into three zones (A, B, C) based on the extent of involvement. Two-thirds of cases involve only zone A, and zone C involvement is rare.
| Zone | Extent of lesion |
|---|---|
| A | Peripapillary area (within 4 disc diameters) |
| B | Posterior pole (up to the equator) |
| C | Extensive area including the periphery |
At the 2-month follow-up after onset, the following findings have been reported: normal fundus in 40%, optic atrophy in 64%, RPE mottling in 23%, retinal thinning in 14%, and retinal artery narrowing in 4%. Optic atrophy and retinal atrophy may persist.
Vision loss often occurs not immediately after trauma or systemic disease onset, but after a delay of several hours to several days. There is no eye pain. If vision gradually worsens after trauma, this condition should be considered.
Purtscher retinopathy (traumatic)
Head trauma: The oldest known cause. Traffic accidents, falls, blows, etc.
Chest compression: A sudden increase in intrathoracic pressure due to strong compressive trauma. Crush syndrome, chest crush by heavy objects.
Long bone fracture: Can develop due to fat embolism syndrome.
Purtscher-like retinopathy (non-traumatic)
Acute pancreatitis: The most frequent non-traumatic cause. Almost always bilateral.
Renal failure / C3 glomerulopathy: Activation of the alternative complement pathway is involved 6)8).
COVID-19 infection: Can develop even in mild cases due to complement activation and coagulation abnormalities 1)9).
Valsalva maneuver / defecation: Venous return disturbance due to increased intrathoracic pressure 7).
Other non-traumatic causes include collagen diseases / connective tissue diseases (SLE, dermatomyositis, scleroderma), pregnancy-induced hypertension / HELLP syndrome, fat embolism syndrome, hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, childbirth (amniotic fluid embolism), retrobulbar anesthesia, and shaken baby syndrome. In recent years, cases have also been reported after filler injection (including non-facial) 3), ischemic colitis 4), and after herpes zoster vaccine (Shingrix) administration 5).
It has been reported that even mild COVID-19 can cause retinal microvascular occlusion due to complement activation and coagulation abnormalities, leading to Purtscher-like retinopathy 1). A mechanism by which high concentrations of C5a due to cytokine storm promote thrombus formation has also been considered 9).
Two diagnostic criteria are used.
Agrawal et al. criteria require all of the following: ① presence of associated disease, ② unilateral/bilateral Purtscher flecken and/or superficial CWS, ③ limitation to the posterior pole, ④ no direct ocular trauma, ⑤ no emboli in retinal vessels, ⑥ minimal hemorrhage.
Miguel’s updated criteria (3 or more of 5 criteria) are shown below4).
| Criterion | Description |
|---|---|
| Criterion 1 | Presence of Purtscher flecken |
| Criterion 2 | Few to moderate retinal hemorrhages |
| Criterion 3 | Soft exudates (limited to posterior pole) |
| Criterion 4 | Presence of an explainable etiology |
| Criterion 5 | Complementary examination findings consistent with the diagnosis |
Diagnosis is made by clinical findings and fluorescein angiography.
Purtscher flecken are polygonal, occur in the capillary bed between arterioles and venules, and have clear borders within 50 μm of the vessel. Soft exudates are fluffy white spots with indistinct borders, caused by focal infarction in the NFL 2). Both may coexist in the same fundus.
There are no evidence-based treatment guidelines. Most cases are managed with observation, but steroid therapy may be used in some cases. Treatment of the underlying disease (e.g., acute pancreatitis, fractures) is the highest priority.
| Treatment | Evidence | Main Role |
|---|---|---|
| Observation | Recommended by systematic review | First-line |
| High-dose steroids | Not established in prospective trials | Adjunctive |
| Anti-VEGF agents | Case reports only | When macular edema is present |
Even without treatment, many cases resolve within a few months. Steroid therapy, antiplatelet therapy, and fibrinolytic therapy are sometimes used, but their effectiveness is unknown. A systematic review by Miguel et al. found no significant difference in visual improvement between the high-dose steroid group and the untreated group. A systematic review by Xia et al. (2017) also reported no difference in visual improvement with glucocorticoid therapy 4)5). Lesions tend to resolve spontaneously within 1 to 3 months 4).
High-dose intravenous steroids are the most commonly reported treatment, but evidence from prospective trials is not established. Mechanisms include stabilization of damaged neural membranes and microvascular channels, and suppression of granulocyte aggregation and complement activation. Case reports have used a tapering dose starting from prednisolone 60 mg 1)6).
Multiple systematic reviews have shown no significant difference in visual improvement between steroid-treated and untreated groups4)5). Treatment of the underlying disease is the highest priority, and lesions tend to resolve spontaneously within 1–3 months. The efficacy of steroid therapy, antiplatelet therapy, and fibrinolytic therapy is unknown, and evidence from prospective trials has not been established.
The main etiology is occlusion due to embolism of retinal arteriolar precapillaries. Damage to the vascular endothelium by multiple factors is involved in the pathogenesis.
The following mechanisms are hypothesized for Purtscher’s traumatic retinopathy:
The type of embolus varies depending on the causative disease. Fat (long bone fractures), pancreatic proteases (acute pancreatitis), leukocyte aggregation (leukoembolization), air, platelets, and fibrin have been reported.
Findings vary depending on embolus size. Large emboli cause confluent whitening similar to branch retinal artery occlusion, small ones cause soft exudates, and intermediate ones cause Purtscher flecken.
Activation of C5 and complement plays an important role with secondary lymphatic leakage. A pathway of complement activation → leukocyte aggregation (up to 50 μm) → precapillary occlusion has been suggested5)8).
Purtscher flecken are caused by occlusion of precapillary arterioles approximately 45 μm in diameter. A clear zone corresponding to the capillary-free area of 50 μm on both sides of retinal arteries and arterioles is formed.
Data are scarce. In a fatal case of acute pancreatitis reported by Kincaid et al., localized edema, cystic spaces, and disruption of normal structure were observed in the inner retina. Proteinaceous material (presumed recanalized thrombus) was found in the arteriolar lumen, with loss of photoreceptor outer segments, but the RPE and choroid were normal.
There is a case report showing the efficacy of eculizumab (C5 inhibitor) in Purtscher-like retinopathy associated with atypical hemolytic uremic syndrome 8). Based on the common pathophysiology of C3 glomerulopathy and complement dysregulation, its potential application in treating Purtscher-like retinopathy has been suggested.
Teru et al. (2025) first reported Purtscher-like retinopathy after acute ischemic colitis 4). A 72-year-old woman developed bilateral vision loss the day after hospitalization for abdominal pain and bloody stools. It improved spontaneously two weeks later with only treatment for colitis (metronidazole and ciprofloxacin).
Pee et al. (2023) reported the first case of Purtscher-like retinopathy and PAMM complicated by bilateral CF, alveolar hemorrhage, and cerebral infarction after injection of approximately 500 mL of breast hyaluronic acid filler 3). SD-OCT showed hyperreflective bands in the inner nuclear layer, and inner retinal disorganization persisted at 10 months.
Shroff et al. (2022) reported a case of unilateral Purtscher-like retinopathy after COVID-19 in which SS-OCTA revealed vascular dropout and flow deficits 9). It was shown that acute-phase flow deficits persist into the chronic phase, and OCTA is attracting attention as a tool for quantitatively assessing vessel density in the superficial and deep capillary plexuses.
Research is progressing on the correlation between acute-phase OCT findings (extent, severity, and depth of inner layer hyperreflectivity) and long-term visual function prognosis. It has been reported that the extent of inner layer atrophy and thinning of the macular ganglion cell layer may be indicators of poor prognosis, and the construction of an objective prognostic prediction model using OCT is expected.
