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Patient presents with swollen lid, unusual retinal findings
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A 48-year-old female presented to the clinic for evaluation of a swollen right upper lid. It had been swollen for approximately 6 months and had worsened. She noted an increased awareness and mild epiphora of the eye, but denied pain. She also denied any recent corresponding diplopia.
The patient had been seen by an optometrist 2 months previously who had prescribed tobramycin/dexamethasone drops, which she had used four times per day for 1 month, without improvement. She then sought care with a different optometrist, who referred her to our clinic for evaluation.
The patient was a native of the Philippines who had been living in the U.S. for approximately 15 years. She reported an unremarkable medical history and negative to questions about skin lesions, respiratory issues, urinary abnormalities and, more generally, any recent bouts of fever, malaise or weight loss. Likewise, her ocular history was negative for any previous events.
Her vision was good, with uncorrected acuity of 20/40 OD and 20/30 OS. Pinhole improved visual acuity to 20/25 OD and 20/20 OS. Pupillary functions were normal. Extraocular muscle function was slightly reduced on abduction in the right eye, but not enough for the patient to manifest diplopia in that field. Confrontation fields were full to finger counting.
External exam showed a moderately swollen, nonerythematous right upper lid temporally. Although the lesion was only moderate in size (estimated to be about 2 cm), the globe appeared displaced slightly inferiorly, without subjective diplopia. There was no lymphadenopathy of the preauricular or submandibular lymph nodes. Palpation of the lid yielded a moderate-sized, firm, nonmobile mass. Exophthalmometry with Hertel (Bernell) was nearly enophthalmic but not dramatically asymmetric, with measures of 14 mm OD and 12 mm OS and a base of 96 mm.
The right anterior segment structures were normal with the exception of mild temporal chemosis of the temporal conjunctiva and an asymmetrically more inferior lacrimal gland on lid eversion (although the microscopic appearance of the gland itself seemed typical). No irregular blood vessels were visible, and the growth itself was not visible. Slit lamp exam was normal for the left eye.
Undilated exam of the patient’s posterior segment showed irregular retinal findings involving the macula, so the patient was dilated with tropicamide and phenylephrine, which generated the lid retraction and pupil dilation seen in the first photo of the right eye. We did not dilate the left eye because the undilated exam showed normal structures both anteriorly and posteriorly.
Retinal evaluation showed a normal, well perfused optic nerve with a 0.4 cup (which was symmetric with the undilated view of the left eye). The macula had moderate granularity, and the retina showed an unusual latticework of yellow, presumably choroidal folds running from the superior temporal arcades through the fovea. The folds emanated from the far supratemporal periphery where a moderate retinal elevation was present. The zone of this elevation was approximately spherical 8 x 8 disc diameters disc areas, comprised of compact retinal tissue with no ruggae, demarcation line, breaks, holes, tears, feeder vasculature nor lattice within nor surrounding the elevation.
What’s your diagnosis?
See answer on the next page.
There are incidental findings in the ocular fundus in addition to the chief complaint of the swollen lid. How do you explain the posterior segment findings?
This presentation has multiple findings that must be attended to. This patient has a pain-free firm, nonmobile orbital mass of unclear composition in the zone of the lacrimal gland, and it is difficult to determine if the lacrimal gland is involved. The lack of mobility makes it clear this is not a lid growth, and the lack of pain makes it clear this is a chronic, slowly growing issue, but beyond that is difficult to say.
What about the retinal findings? They are certainly unusual. Is the retinal elevation a manifestation of the mass invading the globe? This patient has an uncommonly seen clinical entity, choroidal folds associated with her undifferentiated orbital mass.
Normal settings on spectral domain optical coherence tomography showed nothing of note. Perhaps raster would have, but it was not taken.
It was also noted that the folds did not change with gaze position, as assessed with binocular indirect ophthalmoscopy.
While choroidal folds have a number of sources, including papilledema, orbital inflammatory conditions and hypotony, they are probably seen most consistently as a result of retro-orbital tumors, where they are estimated to be present in more than 40% of cases (Singh et al.). Further, the location of the tumor will also influence both the appearance of the folds and their frequency. Those tumors that fall within the muscle cone will display more linear folds, which emanate from the nerve, while those falling outside the muscle cone typically cause curved folds (as seen in our patient), which have their convex side toward the disc. The likelihood of encountering choroidal folds increases the further anterior in the orbit the lesion is.
The underlying mechanism likely has to do with the compressive stress the orbital tumor places directly on the globe leading to indentation of the globe (as with our patient) and subsequent scleral edema and disruption of the choriocapillaris or, when in the muscle cone, from nerve compression and disc edema (as with those seen in papilledema). Folds may resolve upon removal of their causative masses.
The fact that the patient was almost enophthalmic on exophthalmometry was surprising, as we generally associate orbital masses, especially those that cause choroidal folds, with the opposite phenomenon (Sharma et al.). However, after carefully considering the lesion, we could have predicted its effect on the position of the globe. The location of the lesion was the driving feature in this regard – anterior, in the zone of the lacrimal gland. It was so far anterior and firmly adherent to the bony structure of the supratemporal orbit that the vector of force it placed on the globe was inferiorly, medially and internally directed, resulting in a somewhat enophthalmic and medially/hypodeviated globe.
As far as identifying the mass, the cause could be cancerous, inflammatory, infectious or granulomatous, and the patient should be worked up appropriately. Reasonable special testing may include blood work to identify any underlying granulomatous process, sarcoidosis or granulomatosis with polyangiitis, specifically (newer preferred terminology for Wegener’s granulomatosis). In addition, imaging should be ordered to help fully determine the size of the lesion as well as provide further diagnostic clues.
To rule these conditions out we ordered CBC with differential, serum ACE, serum ANCA and ANA, all of which were normal. In addition to the bloodwork described, an MRI of the orbits was ordered. In hindsight, a CT of the orbit with thin sections would have been preferable for differentiation. The ability of imaging to differentiate tissue masses is incomplete, so referral for removal and biopsy is important in any undifferentiated mass that is compressing the globe.
To this end, our patient was referred to oculoplastics, and 10 days later the mass was removed. Pathology identified a pleomorphic adenoma, an unusual benign tumor of the lacrimal gland that classically presents as a slowly developing, pain-free proptosis. Although the list of tumors that led to choroidal folds is long (the development of folds has more to do with tumor size than precise make-up), pleomorphic adenomas are among the most frequently associated with folds (Sharma et al.). The patient is currently recovering uneventfully.
This case is useful as an illustration of an infrequently seen but highly associated finding of orbital tumors: choroidal folds. It further serves as a reminder of the diagnostic importance of funduscopy, even in cases where you may feel it is unlikely that the retina may be involved. Certainly, without retinal evaluation and subsequently missing the retinal findings, one’s level of concern for the lesion may be reduced, and appropriate care may be delayed.
- References:
- Sharma V, et al. Retina. 2006;26(4):415-421;doi:10.1097/01.iae.0000238544.67003.a4.
- Singh D, et al. Eye. 2012;26(3):448-453. doi: 10.1038/eye.2011.308.
- Taban M, et al. Eye. 2007;21(1):147-150. doi:10.1038/sj.eye.6702479.
- Vaidhyanath R, et al. Orbit. 2008;27:410-418. doi.org/10.1080/01676830802333667.
- For more information:
- Aaron Bronner, OD, is a staff optometrist at Pacific Cataract and Laser Institute in Kennewick, Wash. He can be reached at abronner@gmail.com.
- Edited by Leo P. Semes, OD, FAAO, a professor of optometry, University of Alabama at Birmingham and a member of the Primary Care Optometry News Editorial Board. He may be reached at lsemes@uab.edu.
Disclosures: Bronner reports no relevant financial disclosures. Semes is an advisor or on the speakers bureau for Alcon, Allergan, Bausch + Lomb, Genentech, Maculogix, OptoVue, Shire and ZeaVision. He is a stockholder with HPO.