Painful bilateral ophthalmoparesis, marked proptosis, increased intraocular pressure, and blindness developed in a 29-year-old woman with protein C deficiency and catastrophic antiphospholipid syndrome. Magnetic resonance imaging of the orbits showed bilateral proptosis, globe tenting, and tethering of the optic nerves consistent with an orbital ischemic syndrome. Despite aggressive therapy for antiphospholipid syndrome, the patient died. The autopsy showed necrosis of orbital tissues. This is the first report of orbital ischemic syndrome from protein C deficiency and antiphospholipid syndrome.
A 48-year-old white woman complained of worsening blurry vision in both eyes lasting 1 year and pain in both eyes for the previous month, which worsened with eye movements. She had experienced frequent headaches and transient visual obscurations during the preceding month, but she denied nausea, vomiting, and diplopia. Her medical history was significant for hypertension, acid reflux, anemia, and retroperitoneal fibrosis requiring ureteral stenting in the past year. She had undergone hysterectomy 13 years earlier.Her family history was significant for leukemia in her father. Social history revealed that she was an 18 pack-year smoker, but had quit 16 years earlier. Her only medication was valsartan. She denied any use of methysergide.
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