We present a case of an orbital varix masquerading as an orbital lymphoma. Our case underscores the importance of clinical correlation and thorough study of the ordered films by the ordering health care provider.
Case Presentation
An 84-year-old female veteran presented to the Bay Pines Veterans Affairs Healthcare System emergency department. She had a past ocular history of nonproliferative diabetic retinopathy in both eyes (OU) and senile cataracts OU. She had a complicated medical history most notable for congestive heart failure and Stage IV B cell follicular lymphoma, having received 6 rounds of chemotherapy, and has since been on rituximab maintenance therapy for the past few years.
The patient reported dyspnea on exertion, 30-pound weight gain, and ocular pain in her right eye (OD), more so than her left eye (OS) that was severe enough to wake her from sleep. She endorsed an associated headache but reported no visual loss or any other ocular symptoms other than conjunctival injection. On examination, the patient demonstrated jugular venous distension. X-ray imaging obtained in the emergency department demonstrated bilateral pleural effusions. Our patient was admitted subsequently for an exacerbation of congestive heart failure. She was monitored for euvolemia and discharged 4 days later.
During admission, imaging of the orbits was obtained. Computed tomography (CT) of the head without contrast demonstrated at least 4 intraorbital masses in the right orbit, measuring up to 22 mm in maximum diameter and at least 3 intraorbital masses in the left orbit, measuring up to 16 mm in diameter (Figure 1). Magnetic resonance imaging (MRI) with contrast of the brain and orbits was ordered, which demonstrated multiple bilateral uniformly enhancing, primarily extraconal masses present in both orbits, the largest of which occupied the superomedial aspect of the right orbit and measured 12 x 18 x 20 mm. Further, the ophthalmic veins were noted to be engorged. The cavernous did not demonstrate any thrombosis. No other ocular structures were compromised, although there was compression of the extraocular muscles in both orbits (Figures 2, 3, 4, 5, and 6). At that time, the reading radiologist suggested the most likely diagnosis was metastatic orbital lymphoma given the clinical history, which became the working diagnosis.
A few days after admission, the patient received an ophthalmic evaluation at the eye clinic. Visual acuity (VA) at this time was 20/200 that pinholed (PH) 20/70 OD and 20/30 without pinhole improvement OS. Refraction was -2.50 + 1.50 × 120 OD and -0.25 + 0.50 × 065 OS, which yielded visual acuities of 20/60 and 20/30, respectively. There was no afferent pupillary defect and pupils were symmetric. Goldmann tonometry demonstrated pressures of 11 mm of mercury OU at 1630. Slit-lamp and dilated fundus examinations were within normal limits except for 2+ nuclear sclerotic cataracts, large cups of 0.6 OD and 0.7 OS, and a mild epiretinal membrane OD. The decision was made to refer the patient to oculoplastic service for biopsy of the lesion to rule out a metastatic lymphoid solid tumor. At this juncture, the working diagnosis continued to be metastatic orbital lymphoma.
The patient underwent right anterior orbitotomy. Intraoperatively, after dissection to the lesion was accomplished, it was noted that the mass displayed a blue to purple hue consistent with a vascular malformation. It was decided to continue careful dissection instead of obtaining a biopsy. Continued dissection further corroborated a vascular lesion. Meticulous hemostasis was maintained during the dissection; however, dissection was halted after about 35-mm depth into the orbit, given concern for damaging the optic nerve. The feeding vessel to the lesion was tied off with two 5-0 vicryl sutures, and the specimen was cut distal to the ligation. During the procedure, pupillary function was continually checked. The rest of the surgery proceeded without any difficulty, and the specimen was sent off to pathology.
Pathology returned as an orbital varix with no thrombosis or malignant tissue. Surgery to remove lesions of the left orbit was deferred given radiologic findings consistent with vascular lesions, similar to the removed lesion from the right orbit. The patient is currently without residual periorbital pain after diuresis, and the patient’s oncological management continues to be maintenance rituximab. The remaining lesions will be monitored with yearly serial imaging.