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Ocular System

Purtscher-like retinopathy (PUR)

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.

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
Characteristic fundus findings of Purtscher retinopathy. Multiple cotton-wool spots surround the optic nerve after blunt thoracic trauma. | Lymphatic Extravasation From. (2018) Purtscher Retinopathy and Purtscher-like Retinopathy: Background, Pathophysiology, Epidemiology. Retrieved October 19, 2018, from https://emedicine.medscape.com/article/1225431-overview

Fluorescein angiography:

Fundus fluorescein angiography of the left eye (postoperative day 4) revealed blocked fluorescence in the sites corresponding to the retinal and preretinal hemorrhages, capillary dropout in the areas of Purtscher flecken and cotton-wool spots | Narendran, S., Saravanan, V. R., & Pereira, M. (2016). Purtscher-like retinopathy: A rare complication of peribulbar anesthesia. Indian journal of ophthalmology, 64(6), 464–466. https://doi.org/10.4103/0301-4738.187679

Optical coherence tomography (OCT):

  • Macular retinoschisis
  • Separation in the inner retinal layers
  • Macular oedema
Optical coherence tomography of the left eye (postoperative day 4) revealed edema of the inner retinal layers with hyperreflectivity of the nerve fiber layers corresponding to the areas of retinal whitening | Narendran, S., Saravanan, V. R., & Pereira, M. (2016). Purtscher-like retinopathy: A rare complication of peribulbar anesthesia. Indian journal of ophthalmology, 64(6), 464–466. https://doi.org/10.4103/0301-4738.187679
  • 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
Fundus photograph of the left eye. (a) Postoperative day 1. Numerous, peripapillary, soft exudates and superficial hemorrhages. (b) Follow-up at 2 weeks. (c) Follow-up at 4 weeks | Narendran, S., Saravanan, V. R., & Pereira, M. (2016). Purtscher-like retinopathy: A rare complication of peribulbar anesthesia. Indian journal of ophthalmology, 64(6), 464–466. https://doi.org/10.4103/0301-4738.187679

Treatment of underlying non-traumatic cause

IV high-dose steroids

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