Sunir J. Garg

BASICS

DESCRIPTION

• An acute, usually bilateral, patchy, posterior retinal whitening due to crush injuries of the body, or from systemic diseases such as pancreatitis

• Visual acuity is commonly affected but often improves somewhat over time.

EPIDEMIOLOGY

Incidence

Very rare eye disease (probably ∼1/1,000,000)

RISK FACTORS

• In 1910, Dr. Otmar Purtscher initially described bilateral patchy retinal whitening with hemorrhage in a patient who had sustained severe head trauma.

• Purtscher’s retinopathy also occurs following severe chest trauma and with long bone fractures.

• Similar findings have also been described in a variety of systemic diseases (see list under “Commonly Associated Conditions”), which have sometimes been referred to as Purtscher’s-like retinopathy.

PATHOPHYSIOLOGY

• Experimental data suggest that Purtscher’s retinopathy is likely a microembolic phenomenon.

• Possible activation of complement with secondary granulocytic reaction.

• The embolic particles may consist of fat, air, or fibrin. Disseminated intravascular coagulation may be another cause.

• Sparing of larger retinal vessels suggests involvement of precapillary arterioles.

ETIOLOGY

• Typically, Purtscher’s retinopathy occurs in association with severe head, chest, or long bone trauma.

• However, systemic medical conditions (see below under “Commonly Associated Conditions”) may also cause retinopathy identical to that caused by severe distant trauma; this has sometimes been referred to as “Purtscher-like retinopathy”

COMMONLY ASSOCIATED CONDITIONS

• Severe head, chest, or long bone trauma

• Acute pancreatitis

• Systemic lupus erythematosus

• Scleroderma

• Dermatomyositis

• Fat embolism syndrome

• Amniotic fluid embolism

• Chronic renal failure

• Childbirth

• Cryoglobulinemia

• Hemolytic uremic syndrome/thrombotic thrombocytopenic purpura

DIAGNOSIS

HISTORY

• Acute painless vision loss that occurs 1–2 days after trauma.

• Usually bilateral, although it may be asymmetric.

• Vision loss ranges from very mild to very severe.

• History of direct ocular trauma should be excluded.

PHYSICAL EXAM

• Typically, multiple areas of polygonal retinal whitening are seen between the retinal arterioles and venules (Purtscher Flecken).

• Cotton wool spots may also be seen in one or both eyes.

• The lesions are posterior to the equator.

• Minimal retinal hemorrhages are seen.

• No emboli are visible in retinal vessels.

• Late in the disease course, patients may develop disk pallor, attenuation of the retinal arterioles, alterations of the retinal pigment epithelium, and nerve fiber layer dropout.

DIAGNOSTIC TESTS & INTERPRETATION

Lab

• Typically, Purtscher’s retinopathy is associated with bodily trauma.

• However, the eye findings may be the first manifestation of underlying systemic illness, for which a systemic work-up may be warranted.

Imaging

• Fluorescein angiography (FA) typically shows multiple areas of small arteriolar occlusion and capillary dropout (nonperfusion), with late leakage in areas of retinal whitening.

• Optical coherence tomography (OCT) initially shows swelling of the nerve fiber layer, followed by thinning of the inner retina.

Pathological Findings

Findings on pathology are likely similar to those found in cotton wool spots, including retinal capillary obliteration and distension of the nerve fiber layer (cytoid bodies).

DIFFERENTIAL DIAGNOSIS

• Branch or central retinal artery occlusion

• Commotio retinae

• Valsalva retinopathy

• Shaken baby syndrome

• Terson’s syndrome

• Blood dyscrasias

• Fat embolism syndrome

• HIV retinopathy

• Interferon retinopathy

• Lupus retinopathy

TREATMENT

MEDICATION

• No treatments are recommended to treat Purtscher’s retinopathy.

• Any underlying systemic disease, however, should be treated to prevent further retinal damage.

ADDITIONAL TREATMENT

Issues for Referral

• Because of the array of systemic diseases that are associated with Purtscher’s retinopathy, referral is dependant on the systemic condition responsible for the ocular findings.

• Collaboration with a trauma specialist, internal medicine, gastroenterology, nephrology, or rheumatology may be warranted.

• If visual acuity loss is profound, eventual referral to a low vision specialist can be beneficial.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• Extent of visual recovery may be related to duration of retinal whitening.

• In patients with shorter duration of retinal whitening, visual acuity has been shown to improve.

• Patients with longer-standing retinal whitening have a more dismal visual prognosis.

Patient Monitoring

• If an active systemic illness is the cause of the retinopathy, patients should be examined at close intervals to assess if there is worsening of the retinopathy.

• Subsequently, patients may be monitored at longer intervals.

PATIENT EDUCATION

Patients should be aware that loss of visual acuity is often longstanding; however, vision loss typically does not worsen.

PROGNOSIS

Prognosis for visual recovery is guarded, although the disease is typically acute and nonprogressive.

COMPLICATIONS

Optic nerve damage may be present to varying degrees and manifests on fundus examination as optic atrophy later in the disease course.

ADDITIONAL READING

• Bhan K, Ashiq A, Aralikatti A, et al. The incidence of Purtscher retinopathy in acute pancreatitis. Br J Ophthalmol 2008;92(1):151–153.

• Agrawal A, McKibbin M. Purtscher’s retinopathy: epidemiology, clinical features and outcome. Br J Ophthalmol 2007;91(11):1456–1459.

• Agrawal A, McKibbin MA. Purtscher’s and Purtscher-like retinopathies: a review. Surv Ophthalmol 2006;51(2):129–136.

• Holak HM, Holak S. Prognostic factors for visual outcome in Purtscher retinopathy. Surv Ophthalmol 2007;52(1):117–118; author reply 118–119.

CODES

ICD9

• 360.43 Hemophthalmos, except current injury

• 362.83 Retinal edema

CLINICAL PEARLS

• Acute bilateral disease causing patchy whitening of the posterior inner retina

• Typically caused by distant and severe head, chest, or long bone trauma

• Identical findings have also been reported in association with a variety of other systemic illnesses, most commonly pancreatitis.

• Visual acuity typically affected, but the degree of loss is variable.

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