Case

A 24-year old young male presented to clinic with acute vision loss following a recent motorcycle accident. The patient was wearing a helmet but did experience chest trauma. He was diagnosed with a left rib fracture and the CT pan-scan was otherwise unremarkable. Ophthalmic examination revealed BCVA 20/800 OD and 20/20 OS. A mild red desaturation OD was noted following dilation. Slit lamp exam was within normal limits OU. Dilated fundus examination was within normal limits OS. However, the right eye revealed mild blurring of the optic disc margin as well as extensive cotton-wool spots (CWS) throughout the posterior pole, most concentrated in the peripapillary area. A subhyaloid vitreous hemorrhage was also denoted. The patient was diagnosed with Purtscher retinopathy and initial observation was recommended but patient was lost to follow up.

Introduction

Purtscher retinopathy is an occlusive microvascular disease commonly associated with head trauma and thoracic compression. Non-traumatic causes may include those associated with increased risk of emboli formation, such as long bone fractures, thrombotic diseases, pancreatitis, and renal failure. These non-traumatic presentations are referred to as “Purtscher-like retinopathy.” 

Diagnosis of Purtscher retinopathy is clinical, with 60% of cases being bilateral and confined to the posterior pole. The most common finding is numerous CWS confined to the posterior pole. The pathognomonic sign is described as large areas of paravascular whitening (Purtscher flecken) adjacent to clear retinal zone. Unlike CWS, purstcher flecken are localized to the intraretinal space on OCT and associated with pre-capillary occlusion. The extensive posterior pole whitening appearance may give the appearance of a “pseudo” cherry red spot in the macula. 

Other findings include retinal hemorrhages (which tend to be less abundant than the CWS) and both optic nerve and macular edema.

Recent criteria in the diagnosis of the disease includes 3 of the following:

Purtscher flecken

Retinal hemorrhages 

Cotton-wool spots (confined to the posterior pole)

Known plausible etiology 

Diagnostic modality results that complement the diagnosis (e.g. FA, OCT, VF, etc)

Differentials include:

Retinal vasculopathies such as DR and HTR

Posterior uveitis: infectious and/or inflammatory 

Lupus retinopathy

Neoplastic retinopathies 

Management

The most common management of Purtscher retinopathy is close observation along with treatment/management of any underlying etiology. IV steroids have been used but this treatment option is controversial. The use of anti-VEGF injection is reserved for concomitate macular edema or severe ischemia.

 

Credits: Ramsey Yusuf MD*, Diana Shechtman OD**, Michael J. Venincasa MD,** Caroline Borie MD,*

*Bascom Palmer Eye institute

**Loh Ophthalmology & Retina Consultants of Miami

 

References

Giani A, Deiro AP, Sabella P. Spectral Domain-Optical Coherence Tomography and Fundus Autofluorescence Findings in A Case of Purtscher-Like Retinopathy. Retina Cases Brief Rep. 2010;5(2):167–170.

Carrera CRL, Pierre LM, Medina FMC, Pierre-Filho PDTP. Purtscher-like retinopathy associated with acute pancreatitis. Sao Paulo Med J. 2005;123(6):289–91. doi:/S1516-31802005000600008.

Miguel MA, Henriques F, Azevedo LFR, et al. Systmeic review of Putscher’s and Putscher’s like retinopathies. Eye 2013; 27: 1-23. 

Purtscher O . Noch unbekannte befunde nach schadeltrauma. Ber Dtsch Ophthalmol Ges1910; 36: 294–301.