Management of Purtscher Retinopathy
There is no standardized treatment protocol for Purtscher retinopathy, but high-dose systemic corticosteroid therapy may improve visual outcomes when administered early after symptom onset.
Definition and Pathophysiology
Purtscher retinopathy is a rare occlusive microvasculopathy characterized by:
- Sudden visual loss following trauma (classic Purtscher) or associated with systemic conditions (Purtscher-like)
- Multiple areas of superficial retinal whitening (Purtscher flecken) primarily in the posterior pole
- Cotton wool spots, retinal hemorrhages, and potential optic disc edema
- Pathogenesis likely involves embolic occlusion of pre-capillary arterioles
Clinical Presentation and Diagnosis
The diagnosis is primarily clinical, based on:
- Acute painless vision loss
- Characteristic fundoscopic findings including:
- Multiple whitish patches in the macular and peripapillary areas
- Cotton wool spots
- Retinal hemorrhages
- Purtscher flecken (polygonal areas of retinal whitening between arterioles and venules)
Diagnostic workup should include:
- Comprehensive ophthalmologic examination
- Fluorescein angiography (showing multifocal retinal arteriolar occlusion)
- Optical coherence tomography (OCT)
- Systemic evaluation to identify underlying causes
Treatment Approach
First-line Treatment
- High-dose systemic corticosteroids are the most commonly used treatment, though evidence is limited 1
- Methylprednisolone 500-1000 mg/day for 3-5 days, followed by oral prednisone taper
- Should be initiated as early as possible after symptom onset
Additional Treatment Options
- Observation alone (many cases show spontaneous improvement)
- Anti-VEGF therapy may be considered for cases with macular edema 2
- Management of underlying systemic conditions when present
Monitoring and Follow-up
- Regular ophthalmologic examinations to monitor visual acuity and retinal changes
- Follow-up intervals based on severity:
- Initially every 2-4 weeks
- Then every 3-6 months until stabilization
Prognosis
Visual outcomes are variable:
- Approximately 50% of patients show some visual recovery without treatment
- Early treatment with high-dose corticosteroids may improve outcomes 1
- Poor prognostic factors include:
- Extensive retinal whitening
- Optic disc involvement
- Delayed treatment
- Associated macular edema
Special Considerations
Associated Conditions
Purtscher-like retinopathy has been reported with:
- Acute pancreatitis
- Systemic lupus erythematosus
- Antiphospholipid syndrome
- Adult-onset Still disease
- Following cardiac surgery
- Renal failure
These underlying conditions should be identified and treated appropriately 2, 3, 4.
Complications
- Permanent visual impairment
- Optic atrophy
- Retinal pigmentary changes
Pitfalls and Caveats
- Delayed diagnosis: The condition may be overlooked in trauma patients with multiple injuries
- Limited evidence: No randomized controlled trials exist to guide treatment
- Variable response: Not all patients respond to corticosteroid therapy
- Systemic contraindications: High-dose steroids may be contraindicated in some patients with comorbidities
Visual rehabilitation services should be offered to patients with permanent visual impairment to maximize residual vision and promote independence.