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Bilateral areas of retinal whitening and cotton wool spots seen in young man
The patient presented with abdominal pain, nausea and vomiting, and an acute onset of a scotoma.
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A 24-year-old man was admitted to the hospital with abdominal pain, nausea and vomiting. On admission, his blood pressure was 185/102, his heart rate was 160, and he was mildly febrile with a temperature of 100.9°F. One day later, he complained of the acute onset of a scotoma in his left eye just temporal to his central vision. He denied pain or other associated symptoms.
History
The patient’s ocular history was significant only for mild myopia, and his medical history was significant only for attention deficit hyperactivity disorder. Family history was notable for a brother who is a glaucoma suspect. On questioning, the patient reported an alcohol intake of five to six drinks per day.
Examination
On examination, the patient’s best corrected visual acuity was 20/25 in the right eye and 20/100 eccentrically in the left eye. His pupils were equal, round and reactive without an afferent pupillary defect. Confrontational visual fields were full. IOP and anterior segment examination were normal. Dilated fundus exam was remarkable for multiple cotton wool spots and retinal whitening bilaterally, left worse than right, and a pseudo-cherry red spot in the left macula (Figure 1). Optical coherence tomography showed hyperreflectivity of the outer retina in both eyes, as well as inner retinal thickening and hyperreflectivity in the left eye (Figures 2a and 2b). Fundus fluorescein angiography revealed decreased fluorescence in the region of retinal whitening in the left eye, with otherwise normal choroidal and retinal filling (Figures 3a and 3b).
Figure 1. Color fundus photos revealing bilateral cotton wool spots and areas of retinal whitening, left eye greater than right, as well as a pseudo-cherry red spot in the left macula.
Images: Muakkassa N, Witkin AJ
Figure 2. OCT of the right eye (a) revealing outer retinal hyperreflectivity. OCT of the left eye (b) revealing hyperreflectivity of both the inner and outer retina and inner retinal thickening.
Figure 3a. Fundus fluorescein angiogram of the right eye with normal choroidal and retinal filling.
Figure 3b. Fundus fluorescein angiogram of the left eye revealing decreased fluorescence due to blockage or capillary dropout in the region of retinal whitening, with normal retinal and choroidal filling.
What is your diagnosis?
Acute onset of scotoma
Purtscher’s retinopathy occurs secondary to head or chest trauma and presents with cotton wool spots, retinal whitening and/or retinal hemorrhages. This can be eliminated from our differential given the absence of trauma. Several systemic conditions have been known to result in a similar fundus appearance, which is often referred to as Purtscher-like retinopathy. These include pancreatitis, systemic lupus erythematosus, other collagen vascular diseases, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, fat or amniotic fluid embolus, and chronic renal failure. Systemic hypertension, HIV retinopathy, cytomegalovirus (CMV) retinitis and leukemia are other diseases that may sometimes present with similar fundus findings.
Although our patient was hypertensive during his admission, the lack of disc edema or retinal hemorrhages makes hypertensive retinopathy less likely. Possible infectious causes include HIV retinopathy or CMV retinitis. HIV retinopathy and early CMV retinitis may present with cotton wool spots. Later findings of CMV retinitis include retinal hemorrhages, necrosis and vasculitis. These infectious causes are less likely in our patient given he does not have a history of HIV nor is he immunocompromised. Leukemic retinal infiltrates may present similarly, with flat patches of retinal whitening, and leukemia should be ruled out with a peripheral blood smear. A CBC can also reveal platelet or other blood cell abnormalities, which might indicate platelet or blood cell abnormalities from other systemic diseases on the differential diagnosis. The lack of any signs or symptoms of collagen vascular disease, such as skin rash or joint disease, makes collagen vascular disease unlikely; however, an autoimmune work-up might be indicated if other testing is normal.
Given the patient’s symptoms of abdominal pain, nausea and vomiting, as well as his admittedly high alcohol intake, pancreatitis resulting in Purtscher-like retinopathy is highest on the differential for this patient.
Diagnosis and management
The patient’s medical work-up revealed an elevated lipase level, and abdominal CT imaging confirmed a diagnosis of acute alcoholic pancreatitis. The fundus findings of cotton wool spots in a peripapillary distribution and areas of retinal whitening (Purtscher flecken) are typical for Purtscher-like retinopathy secondary to pancreatitis. Over his hospital stay, the patient improved with intravenous hydration and bowel rest.
He was discharged home and returned to the ophthalmology clinic 5 days later. Visual acuity was finger counting in the left eye but improved to 20/25 through the pinhole. Fundus examination revealed improving cotton wool spots and areas of retinal whitening (Figure 4). Three weeks later, visual acuity remained finger counting with improvement to 20/25 through the pinhole. The fundus findings continued to improve (Figure 5), but inner retinal thinning developed, as seen on OCT (Figure 6).
Figure 4. Color fundus photos 5 days after presentation showing resolving cotton wool spots and retinal whitening.
Figure 5. Color fundus photos 1 month after presentation showing almost complete resolution of retinal findings.
Figure 6. OCT at 1 month showing inner retinal thinning of the left eye.
Discussion
Purtscher’s retinopathy was first described in 1910 as peripapillary retinal infarctions associated with head trauma. Since then, a similar fundus appearance has been described in association with many systemic conditions, including pancreatitis, collagen vascular disease, air or fat emboli, and chronic renal failure. In these non-traumatic cases, the fundus appearance is often referred to as Purtscher-like retinopathy. Most cases are bilateral, although findings may be unilateral or asymmetric. Fundus findings are usually restricted to the posterior pole and may include cotton wool spots, Purtscher flecken (polygonal areas of retinal whitening), retinal hemorrhages or optic disc edema. Fluorescein angiography may show leakage and/or areas of hypofluorescence, which may be due to choroidal hypoperfusion, retinal capillary nonperfusion, or blockage secondary to retinal hemorrhages or retinal edema.
There are several proposed mechanisms to explain these fundus findings. In pancreatitis and collagen vascular diseases, the proposed theory is the activation of complement factors, resulting in leukocyte, fibrin and platelet aggregation within retinal vessels. Histopathologic studies have revealed occlusion of retinal arterioles with inner retinal edema and disruption of the photoreceptor layer. Occluded choroidal vessels have been noted as well, suggesting choroidal ischemia may also be present in this condition and might contribute to the outer retinal abnormalities seen on OCT in our patient.
Visual acuity and visual field loss varies and depends on the area of ischemic retina. There is no proven treatment for this condition, although there are case reports of improvement in visual acuity after treatment with systemic steroids. The underlying medical condition should be treated with the goal of preventing further ischemia. Retinal findings usually resolve within 1 to 3 months. Late findings include inner retinal thinning in areas of ischemia, retinal pigment epithelium mottling, attenuation of retinal vessels and optic atrophy. Recovery of vision is variable, and visual acuity may be limited due to retinal or optic nerve ischemia.