History of present illness

A 26-year-old healthy male patient presented with a 1-week history of acute vision loss. Ocular history was noncontributory.

Ocular examination findings

Snellen visual acuity was 20/30 in the right eye (OD) and 20/70 in the left eye (OS). Intraocular pressures were normal, and external and anterior segment examination were unremarkable in both eyes (OU). Dilated retinal examination showed multiple cotton wool spots (CWS) in a peripapillary distribution OU and spot retinal hemorrhages in the inferior macula OS ( Fig. 45.1 ).

Color fundus photography shows multiple cotton wool spots in a peripapillary distribution. Note the associated large flame hemorrhage inferior to the fovea in the left eye (right image, white arrow).

Imaging

Optical coherence tomography (OCT) displayed central subretinal fluid (SRF) OU and band-like, hyperreflective infarcts of the inner (CWS) and middle (paracentral acute middle maculopathy [PAMM]) retina OU ( Figs. 45.2 and 45.3 ).

Optical coherence tomography (OCT) B scans of the right eye (left column) and left eye (right column) at baseline (A) show central subretinal fluid (SRF) in both eyes. Hyperreflective thickening in the inner retinal layer and hyperreflective bands in the inner nuclear layer correspond to cotton wool spots (CWS) and paracentral acute middle maculopathy, respectively. The OCT B scans of the right eye (left column) and left eye (right column) at follow-up (B) show resolved SRF in the right eye, improved SRF in the left eye, and improvement of the CWS in both eyes. There was slight improvement in the visual acuity of the left eye after 10 days with subjective improvement of vision.

Optical coherence tomography B scan of the left eye at baseline shows a cotton wool spot (solid arrow) in the nerve fiber layer and several paracentral acute middle maculopathy or paracentral acute middle maculopathy lesions (dotted arrows) in the middle retina.

Questions to ask

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  Did the patient endorse a history of diabetes? Diabetic retinopathy can present with CWS and retinal hemorrhage.

  
  
  
  
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    No. The patient’s hemoglobin A _1C was 5.1 at the time of the encounter.

  
  
  
  
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  What medications did the patient report taking? Medications such as interferon can cause CWS.

  
  
  
  
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    None

  
  
  
  
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  Did the patient report a history of trauma? Trauma has been associated with Purtscher retinopathy.

  
  
  
  
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    No

  
  
  
  
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  Did the patient admit to a history of autoimmune disorders? Systemic lupus erythematosus (SLE), scleroderma, and dermatomyositis can be complicated by a Purtscher-like retinopathy.

  
  
  
  
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    No. The patient denied any significant medical history.

  
  
  
  
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  Did the patient have any evidence of pancreatitis?

  
  
  
  
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    Yes. The patient presented to the emergency room 1 week earlier with severe vomiting and cramping and was found to have acute pancreatitis with a serum lipase level of 1721 (10–40 normal).

  
  

Assessment

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  This is a case of a 26-year-old healthy male patient with a recent admission for acute pancreatitis who presented with vision loss and peripapillary CWS OU.

Differential diagnosis

Differential Diagnoses	Etiologies
Purtscher retinopathy	• Trauma (head trauma, long bone fracture or crush injury, chest compression, barotrauma)
Purtscher-like retinopathy (morphologically identical to Purtscher retinopathy but with a nontrauma etiology)	• Pancreatitis (acute + chronic) • Malignancy (pancreatic adenocarcinoma, liquid tumors) • Connective tissue disorders (SLE, scleroderma, dermatomyositis) • Cryoglobulinemia • Hemolytic uremic syndrome/thrombotic thrombocytopenic purpura • Chronic renal failure • Pregnancy complications (preeclampsia, placenta abruptio, HELLP [hemolysis, elevated liver enzymes, and low platelets] syndrome) in women • Embolism (fat, amniotic fluid, air) • Orthopedic surgery • Orbital injections
Medications	Interferon
Local vascular	• Diabetic retinopathy • Incomplete central retinal artery occlusion • Retinal vasculitis

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