Visually Disabling Non-Traumatic Orbital Hemorrhage in an Anticoagulated Patient with Factor VII Deficiency

Abstract

We recently evaluated a 41-year-old Hispanic woman who had developed acute vision loss in the right eye with bilateral orbital soft tissue swelling. The patient had been taking warfarin for deep vein thrombosis occurring for one week earlier and had an underlying Factor VII deficiency that was to be diagnosed later. The patient had been found to have an International Normalized Ratio (INR) of 10 at a routine follow-up visit and was treated with prothrombin complex concentrate (PCC) containing factors II, VII, IX, and X as an alternative to using fresh frozen plasma (FFP). The INR corrected to 5.9 but dropped to 9.7 within a few hours. During this time, she complained of severe headache and manifested bilateral proptosis. Within hours, she developed marked bilateral upper eyelid swelling and ecchymosis and became unable to open her right eye. Visual acuity was no light perception (NLP) OD and 20/50 OS with a right afferent pupil defect. Marked proptosis and hemorrhagic chemosis were present bilaterally, worse OD (Fig. 1). There was severe gaze restriction in all directions OU and intraocular pressures (IOPs) were 87 mm Hg OD and 71 mm Hg OS.FIG. 1: Bilateral hemorrhagic chemosis, proptosis, and periocular ecchymosis caused by warfarin anticoagulation superimposed on Factor VII deficiency. Arrows point to locations where canthotomy and cantholysis were performed.Bilateral canthotomy and cantholysis, performed at the bedside, led to drainage of blood from both orbits and improved IOP OU. The patient was then treated with topical IOP-lowering agents, intravenous acetazolamide 500 mg, and methylprednisolone 250 mg. After treatment with FFP, INR corrected to 3.3. Visual acuity quickly improved to 20/20 OS, but remained NLP OD. Ophthalmoscopy showed a central retinal artery occlusion OD. Head CT (Fig. 2) showed bilateral orbital soft tissue swelling and intraconal orbital blood on the right. The patient was to undergo right orbital decompression, but this was deferred when examination under anesthesia showed improvement in proptosis and a normal IOP. Laboratory evaluation revealed Factor VII deficiency.FIG. 2: Axial non-contrast CT shows bilateral orbital soft tissue swelling with heterogeneous density in the right intraconal space suggestive of blood (arrow).Orbital hemorrhages may be caused by trauma, intraocular surgery, vascular anomalies, or rarely by pre-existing coagulopathies (1-5). Krohel and Wright (1) reported 17 cases of spontaneous orbital hemorrhage, mostly from venous anomalies, but one patient had von Willebrand disease. Outcomes were noted to be excellent in patients aged under 70 years. Reports of hemorrhages extending into the orbit in patients with coagulation factor deficiencies include cases by Pomeranz (2) and Guirgis (3) in which evaluation revealed Factor VII deficiency and Factor IX deficiency, respectively. In both cases, the hemorrhage was believed to be precipitated by mild head trauma. Visual acuity improved from 20/200 to 20/20 after surgical orbital decompression. An early series by Rubenstein (4) of 123 cases with hemophilia found that orbital hemorrhagic complications occurred in 25 patients, but severe spontaneous orbital hemorrhage with permanent vision loss occurred in only one patient. Chang (5) reported acute orbital hemorrhage and visual loss in two patients anticoagulated with subcutaneous heparin. Neither had pre-existing coagulopathies. Visual acuity remained counting fingers and NLP despite treatment. This case involved Factor VII deficiency, which has been reported to cause spontaneous intracranial hemorrhage (6). However, ours may be the first reported case of non-traumatic orbital hemorrhage in the setting of warfarin anticoagulation and Factor VII deficiency. We report it to emphasize the need to consider a clotting disorder in cases of non-traumatic hemorrhage and to recognize that proptosis and eyelid swelling may be an early sign of devastating orbital hemorrhage in a patient with a clotting disorder or treatment with anticoagulants. Jared R. Younger, MD, MPH John G. McHenry, MD, MPH Department of Ophthalmology University of Texas Southwestern Dallas, Texas [email protected]