Ophthalmology – Purtscher’s Retinopathy

Basics

Description

Purtscher retinopathy is a rare acute occlusive microangiopathy characterized by sudden visual loss with posterior-pole retinal whitening, classically after severe trauma remote from the eye.

When an identical retinal phenotype occurs without trauma, it is termed:

Purtscher-like retinopathy

Typical fundus findings include:

• Purtscher flecken
• Cotton-wool spots
• Few retinal hemorrhages
• Posterior-pole predominance
• Variable optic disc edema

The disease is usually:

• Bilateral
• Asymmetric
• Acute
• Nonprogressive once the precipitating systemic disorder is controlled

Purtscher vs Purtscher-Like Retinopathy

Purtscher Retinopathy

Traditionally associated with:

• Severe head trauma
• Chest compression
• Long-bone fractures
• Major multisystem trauma

Purtscher-Like Retinopathy

Similar ocular findings associated with systemic disease such as:

• Acute pancreatitis
• Fat embolism syndrome
• Preeclampsia/HELLP
• Thrombotic thrombocytopenic purpura
• Hemolytic uremic syndrome
• Autoimmune disease
• Renal failure
• Amniotic fluid embolism

The retinal appearance is essentially the same.

Epidemiology

Purtscher retinopathy is:

Very rare

and true incidence is uncertain.

Because many patients are critically ill from their systemic disorder, milder ocular cases may go undiagnosed.

Pathophysiology

The leading mechanism is:

Occlusion of precapillary retinal arterioles and capillaries

causing focal inner retinal ischemia.

Several mechanisms may contribute.

Complement Activation

One important proposed mechanism is:

Complement activation → leukocyte aggregation → microvascular occlusion

This is particularly relevant in:

• Pancreatitis
• Severe inflammatory systemic disease

Microembolic Mechanisms

Potential embolic material includes:

• Fat
• Leukocyte aggregates
• Fibrin
• Platelet aggregates
• Air in selected traumatic settings

The exact mechanism may vary according to the underlying disorder.

Why Large Retinal Vessels Look Normal

The occlusion typically occurs at the level of:

• Precapillary arterioles
• Capillary beds

rather than major retinal arteries.

Therefore:

Visible emboli in large retinal vessels are usually absent.

Associated Conditions

Important associations include:

• Severe trauma
• Acute pancreatitis
• Fat embolism syndrome
• Preeclampsia
• HELLP syndrome
• TTP
• HUS
• Systemic lupus erythematosus
• Scleroderma
• Dermatomyositis
• Cryoglobulinemia
• Chronic renal failure
• Amniotic fluid embolism
• Severe systemic inflammatory states

Trauma Associations

Classic traumatic triggers include:

• Head trauma
• Chest compression
• Long-bone fracture
• Polytrauma

Direct ocular trauma should prompt consideration of:

• Commotio retinae
• Traumatic retinal vascular injury

rather than classic Purtscher retinopathy.

Acute Pancreatitis

Acute pancreatitis is one of the best-known causes of Purtscher-like retinopathy.

The proposed mechanism involves:

• Complement activation
• Leukocyte aggregation
• Retinal capillary occlusion

Ocular findings may occasionally precede recognition of severe systemic disease.

Fat Embolism Syndrome

Fat embolism after:

• Long-bone fracture
• Orthopedic trauma

may produce:

• Respiratory distress
• Neurologic changes
• Petechial rash
• Retinal ischemic changes

Purtscher-like retinal findings may overlap with:

Fat embolism retinopathy.

Autoimmune Disease

Purtscher-like changes have been described in:

• SLE
• Scleroderma
• Dermatomyositis

These disorders can independently produce retinal microangiopathy, so clinical context is important.

Clinical Presentation

Typical presentation is:

Acute painless reduction in vision

often developing:

• Within hours
• Within 1–2 days

after the precipitating event.

Laterality

Disease is usually:

Bilateral

but may be:

• Asymmetric
• Rarely unilateral

Visual Acuity

Vision may range from:

• Mildly reduced
• Moderate impairment
• Profound visual loss

Visual loss depends on involvement of:

• Foveal capillary circulation
• Optic nerve
• Extent of retinal ischemia

Visual Field

Possible field defects include:

• Central scotoma
• Paracentral scotoma
• Relative central depression

depending on macular involvement.

Fundus Findings

Classic findings are concentrated:

Posterior to the equator

especially around the:

• Optic disc
• Posterior pole
• Retinal vascular arcades

Purtscher Flecken

Purtscher flecken are the most characteristic finding.

They appear as:

• Polygonal
• White
• Superficial retinal patches

located between retinal arterioles and venules.

A helpful distinguishing feature is:

A narrow zone of retinal whitening that may spare the immediate vessel border

because the ischemia involves the precapillary bed.

Cotton-Wool Spots

Cotton-wool spots are common and represent:

Focal retinal nerve fiber layer ischemia

They may coexist with Purtscher flecken.

Retinal Hemorrhages

Hemorrhages are usually:

• Few
• Small
• Superficial or intraretinal

Extensive hemorrhage should prompt consideration of another diagnosis.

Optic Disc Findings

Possible findings include:

• Mild disc edema
• Peripapillary whitening
• Later optic disc pallor

Severe optic nerve involvement is associated with poorer visual prognosis.

Late Fundus Changes

As acute retinal whitening resolves, later findings may include:

• Optic atrophy
• Retinal arterial attenuation
• RPE mottling
• Inner retinal thinning
• RNFL loss

Diagnosis

Diagnosis is primarily:

Clinical

and depends on:

• Characteristic fundus appearance
• Compatible systemic or traumatic trigger
• Exclusion of other ischemic and inflammatory retinal disorders

Proposed Diagnostic Criteria

A commonly used framework supports the diagnosis when several of the following are present:

• Purtscher flecken
• Few retinal hemorrhages
• Cotton-wool spots confined largely to posterior pole
• Plausible precipitating systemic or traumatic event
• Compatible ancillary investigations

No single finding is completely pathognomonic.

Optical Coherence Tomography

OCT is very useful for documenting acute and chronic structural changes.

Acute Phase

May show:

• Inner retinal hyperreflectivity
• RNFL thickening
• Ganglion cell/inner plexiform edema
• Middle retinal ischemic changes

PAMM-Like Changes

Some eyes demonstrate changes resembling:

Paracentral acute middle maculopathy (PAMM)

with hyperreflectivity involving:

• Inner nuclear layer
• Middle retina

reflecting ischemia of the deep/intermediate retinal capillary plexuses.

Chronic OCT Changes

Later OCT may show:

• Inner retinal thinning
• RNFL loss
• Ganglion cell loss
• Foveal structural damage

These correlate with permanent visual deficit.

OCT Angiography

OCTA may demonstrate:

• Reduced capillary density
• Superficial plexus nonperfusion
• Deep plexus nonperfusion

It is useful for showing retinal microvascular ischemia without dye injection.

Fluorescein Angiography

FA may demonstrate:

• Capillary nonperfusion
• Delayed arteriolar filling
• Vascular leakage
• Late staining of ischemic areas

Findings vary according to disease severity.

Fundus Photography

Photography is useful for:

• Baseline documentation
• Following resolution of retinal whitening
• Comparing hemorrhage and cotton-wool spot burden

Fundus Autofluorescence

FAF is not essential but may show:

• Secondary RPE abnormalities

during later stages.

Laboratory Evaluation

There is no laboratory test that diagnoses Purtscher retinopathy itself.

Testing should instead be directed toward identifying the:

Underlying systemic cause

when not already known.

Systemic Workup

Depending on clinical context, consider:

• CBC
• Platelet count
• Renal function
• Liver function
• Coagulation profile
• Lipase/amylase
• Hemolysis studies
• Autoimmune testing

The exact workup should be driven by:

• Trauma history
• Systemic symptoms
• Pregnancy status
• Medical context

Differential Diagnosis

Important differentials include:

• Central retinal artery occlusion
• Branch retinal artery occlusion
• Commotio retinae
• Fat embolism retinopathy
• Hypertensive retinopathy
• Severe preeclampsia/HELLP retinopathy
• TTP/HUS retinopathy
• Lupus retinopathy
• HIV retinopathy
• Interferon retinopathy
• Retinal vein occlusion
• Valsalva retinopathy
• Terson syndrome
• Abusive head trauma in infants/children

Purtscher Retinopathy vs CRAO

Purtscher Retinopathy

• Usually bilateral
• Patchy polygonal retinal whitening
• Cotton-wool spots
• Few hemorrhages
• Systemic/traumatic trigger

CRAO

• Usually unilateral
• Diffuse retinal whitening
• Cherry-red spot
• Marked arterial attenuation
• Often abrupt profound vision loss

Purtscher Retinopathy vs Commotio Retinae

Purtscher

• Remote body trauma possible
• Usually bilateral
• Posterior microvascular ischemia
• Purtscher flecken

Commotio Retinae

• Direct ocular trauma
• Outer retinal photoreceptor injury
• Gray-white retinal opacity at site of impact
• Usually unilateral

Purtscher vs Valsalva Retinopathy

Valsalva retinopathy produces:

• Preretinal/subhyaloid hemorrhage
• Sudden painless central visual loss

without the characteristic:

• Purtscher flecken
• Cotton-wool spot pattern

Purtscher vs Terson Syndrome

Terson syndrome occurs with:

• Subarachnoid hemorrhage
• Intracranial hemorrhage
• Severe acute elevation of intracranial pressure

and typically causes:

• Vitreous hemorrhage
• Preretinal hemorrhage
• Intraretinal hemorrhage

rather than a primarily ischemic white-retina phenotype.

Treatment

Ocular Treatment

There is:

No proven sight-restoring ocular treatment

for Purtscher or Purtscher-like retinopathy.

Management is primarily:

• Observation
• Documentation
• Treatment of the systemic precipitating disorder

Systemic Treatment

Treat the underlying cause aggressively.

Examples include management of:

• Acute pancreatitis
• Major trauma
• TTP/HUS
• Preeclampsia/HELLP
• Autoimmune disease
• Renal failure
• Fat embolism syndrome

This may prevent further systemic and retinal injury.

Corticosteroids

High-dose corticosteroids have been reported in case series and case reports.

However:

There is no convincing evidence that corticosteroids improve final visual outcome in routine Purtscher retinopathy.

They should not be used solely for the retinal findings unless indicated for the:

Underlying systemic disease.

Anti-VEGF

Anti-VEGF therapy has:

No established role in uncomplicated Purtscher retinopathy.

It may be considered only if a separate VEGF-mediated complication develops.

Laser Treatment

Retinal laser has:

No routine role

because the disease is primarily an acute microvascular ischemic process.

Vitrectomy

Vitrectomy is not a treatment for the retinal ischemia itself.

It may be considered only for an unrelated or secondary surgical indication such as:

• Nonclearing vitreous hemorrhage

which is uncommon in classic disease.

Follow-Up

Initial follow-up depends on:

• Severity of visual loss
• Activity of systemic disease
• Extent of retinal ischemia

Repeat examination may include:

• Visual acuity
• Fundus photography
• OCT
• OCTA
• FA in selected cases

Resolution

Purtscher flecken and cotton-wool spots often fade over:

Weeks to months

but structural retinal damage may remain.

Prognosis

Visual prognosis is:

Variable

Spontaneous improvement is common, particularly when:

• Initial visual acuity is relatively preserved
• Foveal ischemia is limited
• Optic nerve involvement is absent
• Retinal whitening resolves quickly

Poor Prognostic Features

Features associated with worse visual outcome include:

• Severe initial visual loss
• Persistent retinal whitening
• Extensive capillary nonperfusion
• Foveal involvement
• Optic disc swelling followed by atrophy
• Significant inner retinal thinning

Long-Term Visual Outcome

Some patients recover:

• Several lines of visual acuity
• Near-normal vision

while others retain:

• Central scotoma
• Reduced acuity
• Contrast loss
• Permanent visual field defects

Complications

Potential sequelae include:

• Optic atrophy
• Inner retinal atrophy
• RNFL thinning
• Permanent scotoma
• Persistent central visual loss
• Retinal arterial attenuation
• RPE abnormalities

Patient Education

Patients should understand that:

• The retinal process is usually acute rather than continuously progressive
• Vision may improve spontaneously over weeks to months
• Final recovery depends on the degree of retinal and optic nerve ischemia
• Management of the associated systemic illness is essential

Ophthalmology Pearls

• Purtscher retinopathy is an acute occlusive retinal microangiopathy classically following severe trauma remote from the eye.
• The identical retinal phenotype associated with systemic disease is called Purtscher-like retinopathy.
• Major nontraumatic associations include acute pancreatitis, TTP/HUS, preeclampsia/HELLP, autoimmune disease, renal failure, and fat embolism syndrome.
• The characteristic lesion is the Purtscher flecken: polygonal patches of posterior retinal whitening related to precapillary arteriolar occlusion.
• Cotton-wool spots are common, while retinal hemorrhages are usually few rather than extensive.
• Disease is usually bilateral but asymmetric and causes acute painless visual loss.
• The leading mechanism is precapillary microvascular occlusion, potentially involving complement activation, leukocyte aggregation, and microemboli.
• OCT acutely shows inner/middle retinal hyperreflectivity and edema, with later inner retinal and RNFL thinning.
• OCTA and FA can demonstrate capillary nonperfusion.
• There is no proven ocular treatment that reliably restores vision; management focuses on treating the underlying systemic disorder.
• Systemic corticosteroids have been reported but have not demonstrated clear evidence of benefit for the retinal disease itself.
• Purtscher retinopathy should be distinguished from CRAO, commotio retinae, Terson syndrome, Valsalva retinopathy, and fat embolism retinopathy.
• Visual recovery is variable but spontaneous improvement is common; poor prognosis is associated with extensive foveal ischemia, persistent whitening, optic nerve involvement, and subsequent retinal atrophy.
• The disease is generally acute and self-limited rather than chronically progressive, although permanent ischemic damage may remain.