Contents
Occlusive microvasculopathy characterized by multiple retinal white areas around the optic nerve head and fovea with paravascular clearing which may be associated with intraretinal hemorrhages.
History:
Purtscher’s retinopathy or angiopathia retinae traumatica was first described in 1910, by Otmar Purtscher, an Austrian ophthalmologist, in a middle-aged man who complained about bilateral vision loss some hours after a severe head trauma. The ophthalmoscopic examination revealed multiple areas of retinal whitening (Purtscher flecken) with intraretinal hemorrhages confined to the posterior pole in both eyes. Despite the gravity of vision loss, the patient recovered his visual acuity without specific treatment.
After its original description, this typical fundus appearance was also observed in severe but nontraumatic conditions such as acute pancreatitis, amniotic fluid embolism, and collagen-vascular disorders and was designated as Purtscher-like retinopathy.
Some authors estimated an incidence of 0.24 cases per million per year (including Purtscher and Purtscher-like retinopathy), but it is possible that this number can be higher due to some asymptomatic cases. However, it is still considered a rare condition.
Aetiology
Traumatic aetiology:
Purtscher retinopathy
- Blunt thoracic trauma
- Blunt head trauma
Nontraumatic aetiology:
Purtscher-like retinopathy
- Acute pancreatitis
- Childbirth
- Fat embolism
- Connective tissue diseases:
- Systemic lupus erythematosus (SLE)
Pathophysiology
Embolisation of the peripapillary terminal arterioles supplying the superficial peripapillary capillary net.
Clinical features
Presents as loss of vision with a history of a possible precipitating event such as recent major trauma, pancreatitis, childbirth or renal failure.
- Sudden loss of visual acuity
Diagnosis
Fundus examination:
- Purtscher flecken (PATHOGNOMIC): Polygonal areas of whitening in the inner retina between the retinal arterioles and venules with characteristic clear zone between the affected retina and an adjacent arteri
- Cotton wool spots
- Minimal intraretinal hemorrhage
Fluorescein angiography:
Optical coherence tomography (OCT):
- Macular retinoschisis
- Separation in the inner retinal layers
- Macular oedema
- Capillary nonperfusion
- Retinal and/or disc edema
- Perivascular staining
- Late leakage from injured retinal vessels
Differential diagnosis:
- Branch/central retinal artery occlusion (BRAO/CRAO)
- Hypertensive retinopathy
- Diabetic retinopathy
- HIV retinopathy with cotton-wool spot
Management
Observation:
Acute lesions resolve spontaneously within 1–3 months after the onset
Treatment of underlying non-traumatic cause
IV high-dose steroids