Purtscher retinopathy is a chorioretinopathy that presents with characteristic retinal findings in the posterior pole associated with trauma or severe systemic disease. It was first described in 1910 by Austrian ophthalmologist Otmar Purtscher, who reported bilateral vision loss and retinal whitening after head trauma.
When triggered by trauma, it is called “Purtscher retinopathy”; when caused by systemic disease without trauma, it is termed “Purtscher-like retinopathy.” Both share similar clinical findings and pathophysiology.
A systematic review reports that approximately 57.7% of cases are bilateral [²].
Unilateral cases are associated with a slightly better visual prognosis [¹].
It can occur regardless of age or sex, but traumatic cases are more common in young males, and disease-related types follow the typical age of onset of the underlying disease. A recent systematic review reported a mean age of approximately 34.6 years [²].
QWhat is the difference between Purtscher retinopathy and Purtscher-like retinopathy?
A
Cases triggered by trauma (such as head injury or chest compression) are called Purtscher retinopathy, while those caused by systemic diseases such as acute pancreatitis, preeclampsia, renal failure, or SLE without trauma are called Purtscher-like retinopathy. The clinical findings and pathophysiology are common, and the treatment approach is the same.
Findings localized to the posterior pole (around the optic disc to the macula) are characteristic.
Three Main Findings
Soft exudates (cotton-wool spots): Most frequent, seen in 55–58% of cases in systematic reviews. They reflect nerve fiber layer infarction due to precapillary arteriole occlusion[¹][²].
Retinal hemorrhage: Observed scattered in the posterior pole in about half of cases [²].
Purtscher flecken: Seen in 51–53% of cases. These are well-defined white to pale yellow areas around the optic disc, corresponding to capillary-free zones, with a “clear zone” about 50 μm from retinal arterioles and venules [¹][²][⁴]. This finding is characteristic of the disease.
Other findings
Pseudo-cherry red spot: The fovea appears relatively red against a background of macular edema and retinal opacification. Differentiation from central retinal artery occlusion is necessary.
Optic disc edema: Seen in some cases.
Localized lesions: Limited to the posterior pole; the peripheral retina is usually not affected.
2 months after onset: Fundus findings normalize in about 40% of cases.
Two months after onset: approximately 64% of cases progress to optic atrophy.
Occlusion and atrophy of retinal vessels may remain as sequelae.
QWhen do symptoms appear? Immediately after trauma?
A
Visual loss often occurs not immediately after trauma or systemic disease onset, but after a delay of 24–48 hours. There is no eye pain, and if vision gradually worsens after trauma, this disease should be considered.
Acute pancreatitis: classically one of the most common disease-related causes. Pancreatic proteases are thought to cause embolism and vascular endothelial damage[¹][⁵].
Renal failure (chronic/acute): Also reported in dialysis patients.
Preeclampsia (pregnancy-induced hypertension): An important obstetric complication.
Systemic lupus erythematosus (SLE): Associated with autoimmune vasculitis. A recent systematic review of 168 cases found SLE to be the second most common cause after trauma (13.1%)[²].
Fat embolism syndrome: After long bone fractures or surgery.
Hemolytic uremic syndrome (HUS): Reported especially in children.
Others: Blood disorders, malignancies, after hematopoietic stem cell transplantation, etc.[¹][²].
As established diagnostic criteria, there are updated diagnostic criteria proposed by Miguel et al. [¹]. Diagnosis is made by meeting 3 or more of the following 5 items.
Presence of soft exudates (cotton-wool spots)
Presence of Purtscher spots
Retinal hemorrhage (limited to the posterior pole)
Presence of trauma or underlying disease (acute pancreatitis, renal failure, preeclampsia, etc.)
Lesions confined to the posterior pole without involving the periphery
OCT findings: In the acute phase, hyperreflectivity of the inner retinal layers (ischemic edema) corresponding to soft exudates and Purtscher flecken is observed, which changes to atrophy from the inner to outer layers in the chronic phase. It is noted as a biomarker for prognosis prediction[⁴][⁶].
Fluorescein angiography (FA): Useful for evaluating the extent of precapillary arteriolar occlusion and non-perfusion areas. Purtscher flecken are observed as non-fluorescent areas.
Electroretinography (ERG): Reduced amplitudes of a-wave and b-wave are observed. Used for objective assessment of retinal function.
Commotio retinae: Causes gray-white edema including the periphery after direct trauma. The lesion distribution differs.
QWhich test can confirm Purtscher spots?
A
Purtscher spots can be confirmed as well-defined white to pale yellow areas around the optic disc on slit-lamp fundus examination. On fluorescein angiography, they appear as non-fluorescent areas, and on OCT, hyperreflective inner layers are seen in the acute phase.
The most important treatment strategy is prompt treatment of the underlying systemic disease (e.g., acute pancreatitis, preeclampsia, renal failure). Stabilization of the systemic condition also affects the ocular prognosis.
High-dose intravenous steroids (e.g., methylprednisolone pulse therapy) are the most commonly reported treatment, but there is no established evidence from prospective comparative trials.
In a systematic review by Miguel et al., no significant difference in visual prognosis was found between the steroid treatment group and the untreated observation group [¹]. A prospective surveillance study in the UK and Ireland by Agrawal et al. also reported that approximately half of the eyes improved by two or more lines on the Snellen chart without treatment [³]. Therefore, some opinions recommend observation as the most preferred option.
When administering intravenous steroids: carefully consider after confirming that there are no contraindications for the underlying disease (acute phase of preeclampsia or pancreatitis).
When choosing observation: regularly follow up fundus findings, visual acuity, and OCT findings.
In either case, prioritize treatment of the underlying disease.
QWill vision recover with steroids?
A
The efficacy of intravenous steroids has been shown in individual reports, but prospective trials have not demonstrated a significant difference compared to the untreated group. Visual prognosis varies greatly by case, and a certain number of patients recover spontaneously. Prompt treatment of the underlying disease is the highest priority.
6. Pathophysiology and Detailed Mechanisms of Onset
The essence of Purtscher retinopathy is multiple occlusions of precapillary arterioles in the posterior pole of the retina. These occlusions are thought to occur through the following mechanisms.
Fat emboli: Associated with long bone fractures and fat embolism syndrome. Fat droplets from bone marrow enter the circulation.
Pancreatic protease-related: In acute pancreatitis, activated proteases damage vascular endothelium, leading to fibrin deposition and vessel wall injury.
Leukocyte aggregates: Activated neutrophils aggregate in response to trauma or inflammation, occluding precapillary arterioles.
Fibrin emboli: Formed in cases complicated by disseminated intravascular coagulation (DIC).
When C5 complement is activated by trauma or systemic disease, neutrophil aggregation and activation are promoted. Activated neutrophils are trapped in capillaries, causing vascular occlusion and inflammatory response.
Purtscher flecken are white lesions corresponding to areas without capillaries (capillary-free zones). Occlusion of precapillary arterioles approximately 50 μm in diameter leads to white edema in the inner retina that has lost perfusion. This is an independent finding with a different pathogenesis from soft exudates (nerve fiber layer infarction).
Due to the anatomical arrangement of the ophthalmic artery and posterior ciliary arteries, the precapillary arterioles in the posterior pole form a relatively isolated network. Microemboli preferentially accumulate in the posterior pole reflecting this anatomical vulnerability, while the peripheral retina is relatively protected.
7. Latest Research and Future Prospects (Research Stage Reports)
Currently, there are no prospective randomized controlled trials (RCTs) on the treatment of Purtscher retinopathy. The report by Miguel et al. showing no difference between the steroid group and the untreated group is based on a retrospective study with a low level of evidence [¹][²]. Large-scale prospective trials are urgently needed.
Research is progressing on the correlation between acute-phase OCT findings (extent, severity, and depth of inner layer hyperreflectivity) and long-term visual function outcomes. Alasil et al. reported an association between retinal nerve fiber layer swelling and long-term visual field defects, suggesting that the extent of inner layer atrophy and thinning of the macular ganglion cell layer may serve as indicators of poor prognosis [⁶]. The development of an objective prognostic prediction model using OCT is anticipated.
Pathophysiology Elucidation and Therapeutic Targets
Since activation of the C5 complement pathway plays a central role in the pathogenesis, complement inhibitors (such as anti-C5 antibodies) are being discussed as potential future treatments. However, their application in ophthalmology remains at the basic research stage.
QWill effective treatments be developed in the future?
A
Due to the lack of prospective comparative trials, it is currently not possible to determine the superiority or inferiority of treatments. Research on prognosis prediction using OCT findings and novel therapies targeting the complement pathway is advancing, and accumulation of evidence is expected in the future. Treatment selection currently relies on individual clinical judgment.
Miguel AIM, Henriques F, Azevedo LFR, Loureiro AJR, Maberley DAL. Systematic review of Purtscher’s and Purtscher-like retinopathies. Eye (Lond). 2013;27(1):1-13. PMID: 23174749
Abu Serhan H, Abuawwad MT, Taha MJJ, et al. Purtscher’s and Purtscher-like retinopathy etiology, features, management, and outcomes: A summative systematic review of 168 cases. PLoS One. 2024;19(9):e0306473. PMID: 39240905
Agrawal A, McKibbin M. Purtscher’s retinopathy: epidemiology, clinical features and outcome. Br J Ophthalmol. 2007;91(11):1456-1459. PMID: 17556428
Carrera CRL, Pierre LM, Medina FMC, Pierre-Filho PTP. Purtscher-like retinopathy associated with acute pancreatitis. Sao Paulo Med J. 2005;123(6):289-291. PMID: 16444390
Alasil T, Tokuhara K, Bowes LD, Fan J. Purtscher-like retinopathy: optical coherence tomography and visual field findings. Ophthalmic Surg Lasers Imaging. 2010;9:1-4. PMID: 20337320
Copy the article text and paste it into your preferred AI assistant.
Article copied to clipboard
Open an AI assistant below and paste the copied text into the chat box.
