Purtscher-like Retinopathy as a Rare Presentation of Cryoglobulinemia.

Purtscher's retinopathy is a rare condition that is noted in cases related to various types of trauma. The characteristic finding in the fundus is the presence of multiple Purtscher flecken. Purtscher-like retinopathy has a similar presentation in the fundus but is not associated with trauma. We report the case of a 55-year-old male who presented a severe visual loss during course of a hepatitis C infection. Ophthalmic examination indicated peripapillary cotton wool spots and superficial retinal whitening in the macula. Cryoglobulinemia complicating hepatitis C was discovered in this patient. Purtscher-like retinopathy as a rare presentation of cryoglobulinemia should be considered in patients with a history of hepatitis infection.

INTRODUCTION

Purtscher's retinopathy is a rare condition described in 1910 by Otmar Purtscher in a middle-aged male who fell from a tree onto his head and suffered a brief loss of consciousness.1 The fundus characteristics are Purtscher flecken, which are multiple cotton wool spots of varying sizes. The term “Purtscher-like retinopathy” is sometimes used to describe retinopathy in conditions other than trauma.

Cryoglobulinemia is defined by presence of serum immunoglobulins (Igs) that precipitate with cold temperature and resolubilize when warm. They are part of systemic vasculitis associated with liver disease and often manifest from underlying chronic active or persistent hepatitis most commonly, hepatitis C infection.2 We report a rare case of Purtscher-like retinopathy in a male with chronic hepatitis C-associated cryoglobulinemia.

CASE REPORT

A 55-year-old male presented to our hospital with sudden-onset blurring of the vision in his right eye when he woke. Visual acuity was 1/400 in the right eye and 20/20 in the left eye. Fundus examination of the right eye indicated multiple cotton wool patches of various sizes surrounding the optic disc and retinal whitening in the macula. The left eye had superficial retinal hemorrhages and small cotton wool spots. There were no lesions in the peripheral fundus of either eye [Figure 1]. Fluorescein angiography demonstrated that the arm-to-retina circulation time was 33 s (normally ≤15 s in our clinic); the areas of retinal vascular nonperfusion in the right macula corresponded to the areas where the cotton wool patches were found [Figure 2]. There were no plaques in the arteries or apparent spasm of the retinal vessels. There was no staining of the retinal vessels in the late phase of fluorescein angiography.

Figure 1

Fundus examination of the right eye showing multiple cotton wool spots of various sizes surrounding the optic disc and retinal whitening in the macula. The left eye had superficial retinal hemorrhages and small cotton wool spots

Figure 2

Fluorescein angiography indicated that the arm-to-retina circulation time was 33 s, the areas of retinal vascular nonperfusion in the right macula corresponded to the cotton wool spots

The patient's medical history revealed active chronic hepatitis C and hypertension. Systemic evaluation indicated hypertension (160/90 mmHg). Laboratory workup indicated acute renal insufficiency (creatinine 2.1 mg/dl) and normal pancreatic enzymes. A renal biopsy indicated Type I membranoproliferative glomerulonephritis and intravascular deposition of Igs - IgG and IgM - and complement consistent with Type II mixed cryoglobulinemia. Serum antinuclear antibody was negative. Fibrinogen and complement (C3, C4, and C5) levels were low. The C4 fraction had collapsed in the serum. The signs and laboratory workup indicated the patient had cryoglobulinemia.

The patient was treated with pegylated interferon and ribavirin and an antihypertensive treatment. Complement levels returned to normal; cryoglobulins became undetectable, and the patient's visual acuity improved to 20/200 in the right eye over 2 months. At 4 months, the superficial retinal whitening, cotton wool spots, and retinal hemorrhages all resolved, and the patient's visual acuity remained 20/200 in the right eye with optic atrophy and narrowing of the arteries in the right eye [Figure 3].

Figure 3

The right eye 4 months after treatment shows that the superficial retinal whitening, cotton wool spots, and retinal hemorrhages all resolved; however, optic atrophy with arterial narrowing was evident

DISCUSSION

Purtscher-like retinopathy does occur in patients with acute pancreatitis, systemic lupus erythematous, HELLP syndrome, and renal failure, adenocarcinoma of the pancreas with no relation to trauma.34

The lesions are characterized by Purtscher flecken that consist of many discrete areas of retinal whitening and cotton wool patches that are retinal microinfarctions at nerve fiber layer with or without retinal hemorrhages.

Purtscher's retinopathy is diagnosed based on patient history, clinical presentation, and laboratory workup. In the majority of cases, the ophthalmic manifestations are asymmetric bilaterally, and the symptoms can be unilateral. Fluorescein angiography is performed to look for arterial occlusion and areas of capillary leakage.5

Cryoglobulinemia is characterized by the presence of one or more Igs in the serum that precipitate at temperatures below 37°C and redissolve on rewarming.6 A hepatitis C virus (VHC) infection is the cause in the majority of mixed cryoglobulinemia cases (60-90%).6

The pathogenesis of Purtscher's retinopathy remains uncertain and reflects the various etiologies (blood pressure, embolism, and vasculitis).7 When cryoglobulins solidify or become gel-like, they can cause inflammation and block blood vessels. Purtscher associated retinopathy can be due to leukocytes intravascular agglomeration resulting in microthrombi, in response to an abnormal activation of complement.78 VHC infects the lymphoid clots and causes monoclonal or polyclonal B lymphocyte proliferation with production of antibodies (including cryoglobulins) resulting in deposits of immune complex and circulating complement in small caliber vessels.9 Clinicians should consider cryoglobulinemia in the differential diagnosis of a patient with Purtscher-like retinopathy and a history of hepatitis C. Collaboration between ophthalmologists and internists is required for similar cases.

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Conflicts of interest

There are no conflicts of interest.