Diagnosis, Workup and Treatment
This patient presented with a two-day history of sudden left eye proptosis and mild periorbital ecchymosis. The differential diagnosis in cases of acute onset proptosis and pain include orbital cellulitis or abscess, idiopathic orbital inflammatory syndrome, mucopyocele, orbital metastasis with secondary inflammation, lymphoproliferative disease and orbital hemorrhage. The patient reported that she had an acute onset of severe vomiting, which lasted for approximately three hours. She then went to bed and awoke about an hour later with severe pain and proptosis. In addition, because of her cardiovascular disease, the patient was on two antiplatelet drugs.
Because of the acute onset of her symptoms and inability to view previous imagery, the patient was re-imaged. A repeat CT scan revealed a biconvex mass of uniform density along the left orbital roof measuring 3 x 3 x 1.3 cm (See Figure 2). Compared to the report of the original scan, the size of the density was unchanged. The paranasal sinuses were normal and no foreign body was seen on the scan. The CT findings confirmed the clinical suspicion of acute orbital hemorrhage, in this case manifesting as a subperiosteal hematoma.
Given that the patient showed no signs of orbital compartment syndrome or optic neuropathy, had not progressed over 33 hours since the acute event, and reported that her symptoms had decreased, the patient was discharged home with detailed instructions to return immediately with any worsening of symptoms. Because of her significant cardiovascular disease history as well as the fact that antiplatelet therapy is not immediately reversible short of a platelet transfusion, she was continued on aspirin and clopidogrel.
To confirm the diagnosis of presumed periosteal hematoma, an MRI with and without gadolinium was obtained four days later, which illustrated enhancement of the mass before administration of gadolinium (See Figure 3). At her two week follow-up, the patient reported improvement in motility, pain and appearance (See Figure 1). Her clinical exam was normal except for bilateral cataracts.
Orbital hemorrhage is a potentially visually devastating clinical entity most commonly associated with trauma. The term spontaneous orbital hematoma by definition implies a lack of trauma. These cases most often have another identifiable cause, either within the orbit or secondary to systemic factors.
Orbital causes relate mostly to an anatomical aberration or defect. Overwhelmingly, the most common cause of all spontaneous orbital hemorrhages is an underlying orbital vascular anomaly. Specifically, venous anomalies such as varices are most often the cause; arterial and other subsets of vascular anomalies such as arteriovenous malformations have also been implicated.
Systemic conditions may predispose to spontaneous orbital hemorrhages. As the orbital venous system lacks valves, transient increases in pressure, most commonly by Valsalva maneuvers (e.g., childbirth, prolonged coughing or vomiting, etc.), can produce orbital hemorrhagic events. Vasculopathic risk factors, such as hypertension and hyperlipidemia, may be contributory. Blood disorders, such as uremia, sickle cell disease, leukemia and hemophilia have been reported as the underlying cause. Our patient had what is probably the most common “blood disorder” seen in patients over the age of 50 years: iatrogenic anticoagulation for underlying cardiovascular disease.
Management of spontaneous orbital hemorrhage follows the tenets for the treatment of an orbital compartment syndrome (OCS), regardless of cause. In most cases of orbital hemorrhage, the blood tracks within the fascial planes of the orbit and no discreet hematoma is present. In this case, a discreet subperiosteal was noted with an unclear etiology; however, because the patient had no evidence of optic neuropathy or a progressive OCS and was on antiplatelet therapy, an orbitotomy for exploration and biopsy was deemed unnecessary and potentially risky. Instead, an MRI was ordered several days later in the hopes of identifying the unique signal properties of blood breakdown products in the subacute phase, thereby confirming the clinical diagnosis while avoiding surgery. In this case, the high precontrast T-1 signal within the lesion was indeed noted. This transient finding, present approximately three to 14 days following the initial hemorrhage, is characteristic of hematomas: As deoxyhemoglobin degrades into the methemoglobin form, the lesion appears hyperintense on TI images before contrast dye administration.
The author would like to thank Jurij R. Bilyk, MD, of the Oculoplastic and Orbital Surgery Service at Wills Eye Institute, for his time and assistance.
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