Case of the Month Trial Answer

This patient was found to have asymptomatic bilateral cotton-wool spots (CWS) after an admission for ulcerative colitis. As the attached images demonstrate, the cotton-wool spots were fading one month later, and the fluorescein angiogram did not show leakage or severe occlusive disease.

There are many diagnostic considerations. Both diabetes and hypertension can present with cotton-wool spots. However, if there were many CWS in a patient with diabetes or hypertension, there would typically also be hemorrhages and/or microaneurysms, which were not present here. Some causes of CWS, including altitude retinopathy and radiation retinopathy, can be ruled out by history. The absence of hemorrhages and other distinctive clinical features argues strongly against venous occlusive disease or carotid occlusive disease. Conditions that result in hyperviscosity or hypercoagulability are unlikely in the absence of hemorrhages but should be considered. Similarly, leukemia can present with cotton-wool spots, but having white-centered hemorrhages is more typical. Infectious etiologies should be considered, particular HIV. Immune and collagen vascular diseases can also feature CWS, particularly systemic lupus erythematosus. Another important consideration is Purtscher retinopathy (typically caused by long-bone fractures or blunt chest trauma), or Purtscher-like retinopathy, which most commonly occurs in the setting of pancreatitis.

On examination, the blood pressure was normal. Our laboratory testing revealed a normal white count, but there was anemia with a hemoglobin of 8.8 and a hematocrit of 28.0. Platelets were elevated at 545. The sed rate was also elevated at 38 and the C-reactive protein was 3.01 (normal less than or equal to 0.30). The PT and PTT were normal. The chem 7 was unremarkable with a normal glucose. Testing for HIV was negative. The ANA and antiphospholipid antibodies were also negative. The angiotensin converting enzyme was normal at 53. The amylase was elevated during her recent hospitalization at 345 (normal less than 160) but was 53 when we saw her one month later.

Elevation of platelets, sed rate, and CRP are non-specific markers of inflammation. The elevated amylase indicates pancreatitis. This patient most likely had Purtscher-like retinopathy due to pancreatitis. Autoimmune pancreatitis can be associated with inflammatory bow disease.(1) Our patient’s clinical improvement one month after hospitalization in conjunction with normalization of the amylase level portends a good prognosis, and she is being following.

1. Ramos LR, Sachar DB, DiMaio CJ, et al. Inflammatory bowel disease and pancreatitis: A review. Journal of Crohn’s and Colitis 2016; 10: 95-104.