Discussion
In a study of 242 patients, Bacorn and colleagues found that a clinician’s preoperative assessment correlated with histopathologic diagnosis in 75.7% of cases, whereas the radiology report was correct in only 52.4% of cases.1 Retrospective analysis identified clues that could have been used to more rapidly elucidate the true diagnosis for our patient.
In regard to symptomatology, orbital varices present with intermittent proptosis, vision loss, and rarely, periorbital pain unless thrombosed.2,3 The severity of periorbital pain experienced by our patient is atypical of an orbital varix especially in the absence of a phlebolith. A specific feature of orbital varix is enlargement with the Valsalva maneuver.3 Although the patient did not report the notedsymptoms, more pointed questioning may have helped elucidate our patient’s true diagnosis sooner.
Radiologically, the presence of a partial flow void (decreased signal on T2) is useful for confirming the vascular nature of a lesion as was present in our case. Specific to the radiologic evaluation of orbital varices, it is recommended to obtain imaging with and without the Valsalva maneuver.4 Ultrasound is a superb tool in our armamentarium to image orbital lesions. B-scan ultrasound with and without Valsalva should be able to demonstrate variation in size when standing (minimal distension) vs lying flat with Valsalva (maximal distension).4 Further, Doppler ultrasound would be able to demonstrate changes in flow within the lesion when comparing previously mentioned maneuvers.4 Orbital lymphoma would not demonstrate this variation.
The size change of an orbital varix lesion may be further demonstrated on head CT with contrast. On CT, an orbital varix will demonstrate isodensity to other venous structures, whereas orbital lymphomas will be hyperdense when compared to extraocular muscles.4,5 Further, a head CT without contrast may demonstrate phleboliths within an orbital varix.4 MRI should be performed with the Valsalva maneuver. On T1 and T2 studies, orbital varices demonstrate hypointensity when compared to extraocular muscles (EOMs).4 Lymphomas demonstrate a very specific radiologic pattern on MRI. On T1, they demonstrate isointensity to hypointensity when compared to EOMS, and on T2, they demonstrate iso- to hyperintensity when compared to EOMs.5 With respect to fluorodeoxyglucose (FDG) positron emission tomography (PET), our patient’s orbital lesion did not demonstrate FDG uptake. In patients where lymphoma previously demonstrated FDG PET uptake, the absence of such uptake strongly argues against malignant nature of the lesion (Figure 7).
Prominently enhancing lesions are more likely to represent varices, aneurysms, or other highly or completely vascular lesions. Any intraorbital intervention should be conducted as though a vascular lesion is within the differential, and appropriate care should be taken even if not specifically enunciated in the radiologic report.
Management of orbital varices is not standardized; however, these lesions tend to be observed if no significant proptosis, pain, thrombosis, diplopia, or compression of the optic nerve is present. In such cases, surgical intervention is performed; however, the lesions may recur. Our patient’s presentation coincided with her heart failure exacerbation most likely secondary to flow disruption and fluid overload in the venous system, thereby exacerbating her orbital varices. The resolution of our patient’s orbital pain in the left orbit was likely due to improved distension after achieving euvolemia after diuresis. In cases where varices are secondary to a correctable etiologies, treatment of these etiologies are in order. Chen and colleagues reported a case of pulsatile proptosis associated with fluid overload in a newly diagnosed case of heart failure secondary to mitral regurgitation.6 Thus, orbital pain due to worsened orbital varices may represent a symptom of fluid overload and the provider may look for etiologies of this disease process.
Conclusions
We present a case of an orbital varix masquerading as an orbital lymphoma. While the ruling out of a diagnosis that might portend a poor prognosis is always of paramount importance, proper use of investigative studies and a thorough history could have helped elucidate the true diagnosis sooner: In this case an orbital varix masquerading as an orbital lymphoma. Mainly, the use of the Valsalva maneuver during the physical examination (resulting in proptosis) and during radiologic studies might have obviated the need for formal biopsy. Furthermore, orbital pain may be a presenting symptom of fluid overload in patients with a history of orbital varices.