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   Table of Contents      
CASE REPORT
Year : 2016  |  Volume : 2  |  Issue : 2  |  Page : 99-101

Isolated unilateral traumatic purtscher's retinopathy: An unusual presentation


Department of Ophthalmology, Government Medical College, Kozhikode, Kerala, India

Date of Web Publication19-May-2016

Correspondence Address:
Padma B Prabhu
Department of Ophthalmology, Government Medical College, Kozhikode - 673 008, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2394-7438.182729

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  Abstract 

This report describes a 55-year-old healthy male, who presented with defective vision, 2 days after a fall from height. Ophthalmoscopy showed multiple superficial retinal hemorrhages with cotton wool spots near the optic disc of the right eye. The left eye was normal. There was no evidence of focal neurological deficits, long bone fractures, or chest compression. With the history of trauma and characteristic retinal findings, a diagnosis of Purtscher's retinopathy was made. The patient was treated with tapering dose of systemic steroid. Visual acuity failed to regain with development of consecutive optic atrophy.

Keywords: Cotton wool spots, optic atrophy, Purtscher flecken, Purtscher's retinopathy, retinal hemorrhages


How to cite this article:
Prabhu PB, Mufallel MA, Solomon CB, Unnikrishnan S, Raju KV. Isolated unilateral traumatic purtscher's retinopathy: An unusual presentation. MAMC J Med Sci 2016;2:99-101

How to cite this URL:
Prabhu PB, Mufallel MA, Solomon CB, Unnikrishnan S, Raju KV. Isolated unilateral traumatic purtscher's retinopathy: An unusual presentation. MAMC J Med Sci [serial online] 2016 [cited 2020 Feb 23];2:99-101. Available from: http://www.mamcjms.in/text.asp?2016/2/2/99/182729


  Introduction Top


Purtscher's retinopathy is a rare entity. It presents with severe defective vision (visual field loss) following head injury, chest trauma, or crush injury.[1] It may also occur in other illnesses such as pancreatitis, systemic lupus erythematosus (SLE), childbirth, and disseminated intravascular coagulation (DIC) following preeclampsia. The resultant retinal vascular occlusion manifests as hemorrhages, cotton wool spots (CWSs), and whitening of retina. Optic disc is not usually affected.


  Case Report Top


A 55-year-old male came with gross defective vision in the right eye, 2 days after a fall from a height of 4 m. It did not improve or deteriorate with time. He had no other neurological deficits or nervous system complaints. He was a perfectly healthy man until the fall. General examination findings and systems were within normal limits. There were no signs of major soft tissue or bony injury externally. Computed tomography (CT) head was normal. There was a healing abrasion of 2 cm × 2 cm below and lateral to the left lower eyelid upon which he impacted on the ground at the time of fall. Ocular examination showed orthophoric eyes. Extraocular movements were normal. Anterior segments of both eyes were normal. Grade II relative afferent pupillary defect (RAPD) was noted in the right eye (opposite to the side of impact). Visual acuity in the right eye was half a meter counting fingers and that in the left eye was 6/9 (20/30). Intraocular pressure was within normal limit bilaterally. On fundus examination, multiple superficial retinal hemorrhages and CWSs were seen extending three disc diameters around optic disc in the right eye. In between the CWSs, multiple areas of polygonal retinal whitening suggestive of Purtscher flecken were observed [Figure 1] and [Figure 2]. A diagnosis of Purtscher's retinopathy was made. Fundus fluorescein angiography (FFA) revealed hypofluorescent capillary nonperfusion areas around the optic disc. He was empirically started on systemic steroids (intravenous [IV] dexamethasone) which was continued for 10 days. Then, it was changed to oral prednisolone and tapered over 2 weeks. IV methylprednisolone was deferred due to the late presentation (48 h after trauma). At the end of 4 weeks follow-up, the visual acuity remained the same, and ophthalmoscopic examination revealed resolving hemorrhages and early consecutive optic nerve atrophy [Figure 3]. Visual evoked potential showed increased latency and decreased amplitude of responses in the affected eye. Spectral domain optical coherence tomography (OCT) showed retinal atrophy [Figure 4].
Figure 1: Fundus picture Right eye showing Purtscher's retinopathy

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Figure 2: Fundus picture Left eye showing normal retina

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Figure 3: Consecutive optic atrophy with resolving Purtscher's retinopathy

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Figure 4: Optical coherence tomography showing retinal nerve fiber atrophy

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  Discussion Top


Purtscher's retinopathy, first described by the Austrian ophthalmologist, Othmar Purtscher, is a syndrome of sudden blindness following head injury.[1] The cases reported in the literature often presented with loss of visual acuity, visual field, or both. The field loss (central, paracentral, or arcuate scotoma) occurs immediately after head trauma or within 24–48 h of the event. It is bilateral in 60% of cases. Purtscher's retinopathy is also described along with pancreatitis, vasculitis as in SLE, embolic episodes - as amniotic fluid embolism and fat embolism, DIC, thrombotic thrombocytopenic purpura, dermatomyositis, and chronic renal failure. This group is termed as Purtscher-like retinopathy.[2],[3]

Multiple areas of polygonal retinal whitening between the retinal arterioles and venules (Purtscher flecken) and/or superficial CWSs in one or both eyes, typically restricted to the posterior pole defines Purtscher's retinopathy.[4] It may be accompanied by retinal hemorrhages. Usually, evidence of direct ocular trauma is lacking (as in this case). Symptomatic Purtscher's retinopathy is rare.[1],[2],[4] It is seen most often in young men. Visual field loss is not noticed by the individual unless it is extensive, central, inferior, or bilateral, especially in the critical stage of the disease. More often, Purtscher's retinopathy is incidentally detected on cross consultations or as and when the subject recovers from his antecedent illness. Underreporting of cases of asymptomatic Purtscher's retinopathy affects the proper understanding of the incidence, risk factors, clinical pattern, and prognosis of this entity.[5] Purtscher flecken, though pathognomonic, is reported in about 23–62% of symptomatic cases. In contrast to Purtscher flecken, cotton-wool spots occur in >90% cases. They have ill-defined edges and are located superficially over vessels. Macular edema, optic disc swelling, and pseudo-cherry red spot has been reported.[6] Those with massive Purtscher flecken and optic disc edema had a poor initial vision and visual recovery (as in our case). The consecutive atrophy of retinal pigment epithelium and optic nerve is also common in cases presenting with Purtscher flecken.[5],[6],[7] This indicates the possible involvement of the choroidal vasculature in the acute phase in such cases. Cross-sectional and en-face spectral domain OCT is helpful in demonstrating deep capillary ischemia.[8]

The exact etiopathogenesis of the disease is not clear. Complement-mediated aggregates of fat, air, fibrin clot, or platelet clump causes microvascular or macrovascular occlusion involving the retinal vessels.[6],[7],[8] This explains Purtscher-like retinopathy in nontraumatic cases. High intracranial pressure generated at the time of impact or chest compression may be considered as another reason for retinal hemorrhages.[9] It is known that the major retinal vessels and their larger side branches pass obliquely through the retinal nerve fiber layer (RNFL) from their superficial location on the optic disc to assume their usual position beneath the RNFL elsewhere in the fundus. At these neurovascular crossings, splaying of axonal bundles occurs. Transient hypertension within the thin-walled retinal veins which occurs during impact and the accompanying passive hyperdistention, in the peripapillary RNFL may result in compression damage to the axon bundles. CWSs develop from obstruction of axoplasmic transport on either side of the relevant venous segment, usually within 24–48 h after the inciting incident.[10]

Characteristic retinal findings in a setting of trauma or thrombotic/embolic events usually suffice for diagnosis.[6] The role of FFA in demonstrating the features of arteriolar blockage is only corroborative. The final visual prognosis in severe cases depends on the extent and site of involvement and is often not encouraging despite treatment of identified etiological factors.[6],[7] The effect of systemic corticosteroids in enhancing vision is controversial. Isolated case reports suggest that treatment of Purtscher's retinopathy with high-dose, IV steroids may be beneficial.[11] Earlier resolution of the acute changes was associated with a better initial and final visual outcome. Spontaneous visual recovery of at least 2 Snellen lines is reported in 50% of cases.[6]

Differentials of acute gross visual deficits following trauma include traumatic optic neuropathy (TON) and optic nerve head avulsion. In TON, retina and optic nerve appears normal in the context of marked defective vision and RAPD. High-resolution CT orbit is essential to rule out fracture at the optic canal. Optic nerve avulsion is associated with vitreous hemorrhage and preretinal hemorrhage in front of the optic nerve head with distortion of the peripappillary area. B-scan ultrasonography and magnetic resonance imaging orbit are diagnostic modalities of choice in such a case.

Our case was unique in its unilateral presentation, opposite to the site of impact, without evidence of any internal contusions, bony injuries, or organ damage. It is also unusual that the disease manifested after a period of 48 hours post-trauma.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Myron Y, Duker JS. Ophthalmology. 3rd ed. Edinburgh: Mosby. 2008. p. 750-4.  Back to cited text no. 1
    
2.
Miguel AI, Henriques F, Azevedo LF, Loureiro AJ, Maberley DA. Systematic review of Purtscher's and Purtscher-like retinopathies. Eye (Lond) 2013;27:1-13.  Back to cited text no. 2
    
3.
Yan Y, Shen X. Purtscher-like retinopathy associated with dermatomyositis. BMC Ophthalmol 2013;13:36.  Back to cited text no. 3
    
4.
Agrawal A, McKibbin MA. Purtscher's and Purtscher-like retinopathies: A review. Surv Ophthalmol 2006;51:129-36.  Back to cited text no. 4
    
5.
Holló G. Frequency of Purtscher's retinopathy. Br J Ophthalmol 2008;92:1159.  Back to cited text no. 5
    
6.
Agrawal A, McKibbin M. Purtscher's retinopathy: Epidemiology, clinical features and outcome. Br J Ophthalmol 2007;91:1456-9.  Back to cited text no. 6
    
7.
Holak HM, Holak S. Prognostic factors for visual outcome in Purtscher retinopathy. Surv Ophthalmol 2007;52:117-8.  Back to cited text no. 7
    
8.
Alasil T, Tokuhara K, Bowes LD, Fan J. Purtscher-like retinopathy: Optical coherence tomography and visual field findings. Ophthalmic Surg Lasers Imaging 2010;Mar 9;1-4.  Back to cited text no. 8
    
9.
Gohari M, Soleimani AR, Manaviat MR, Shojaei A. Single cotton wool spot as a late manifestation of head trauma. J Ophthalmic Vis Res 2014;9:272-5.  Back to cited text no. 9
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10.
McLeod D. Why cotton wool spots should not be regarded as retinal nerve fibre layer infarcts. Br J Ophthalmol 2005;89:229-37.  Back to cited text no. 10
    
11.
Atabay C, Kansu T, Nurlu G. Late visual recovery after intravenous methylprednisolone treatment of Purtscher's retinopathy. Ann Ophthalmol 1993;25:330-3.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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