CLINICAL CHALLENGES
It's Not Rare If It's In Your Chair
Modern imaging techniques can make the diagnosis easier -- even for one of the rare conditions that you "won't ever see."

We've all been to those lectures where the presenter lists a differential diagnosis and then shortens his list by saying, " Forget about these two, you'll never see them!" Well this month's case revolves around one of those rarities that you "won't ever see!"

Referred by a bump

When I met B.D. she was 74 years old and a self-described "tired, old lady." B.D.'s primary care provider referred her to my office because of a bump on her right upper eyelid. The referring doctor noted that this lesion had been present for about five months and had been recalci-trant to both topical and oral antibiotic therapy.

B.D. confirmed that the bump indeed had been there for about five months but that it would get smaller and then larger intermittently throughout that period. She reported that her right eye would feel irritated occasionally and would get red at times but that it would then clear up on its own. She denies ever having seen discharge in that eye.

According to B.D., the bump wasn't painful or sore to the touch and her left eye felt normal. She also said that her vision remained good except for those times when her right eye felt irritated. When this was the case, she said that the vision in that eye got "a little blurry."

Interestingly, she seemed more concerned with how she felt physically than with her eye. She mentioned numerous times that she felt "worn out," achy and tired, but dismissed these symptoms as inevitable signs of "old age." She did take a prescribed 81 mg aspirin daily and nadolol for hypertension, which she told me is under control.

Taking clinical notes

I measured B.D.'s visual acuity (VA) as 20/30 OU. The OS did improve slightly to 20/25 with a refraction, but the OD stayed the same. Her pupils were 5 mm in size and reactive to light OU. I saw no afferent pupil defect and extraocular muscle testing showed full range of motion in all directions of gaze OU.

My external viewing showed a mild ptosis of B.D.'s upper right lid. An elevated nodule was also visible (Fig. 1). I palpated the lesion and felt a solid mass underneath the lid in the superior temporal aspect of the OD. The area where I palpated the mass was coincident with the lacrimal gland. I asked B.D. to look down and I elevated her right upper lid. A large mass posterior to the lid and coincident with the lacrimal gland was readily visible (Fig. 2).

When I examined B.D.'s eyes with the slit lamp I was struck with how normal the remainder of her tissues were. The lid margins were conspicuously clear, there was no meibomian gland inspissation and no lid debris. The conjunctiva was white and quiet and the anterior chamber was free of cell and flare. She did have mild nuclear sclerotic cataracts, which are expected given her VA and age.

Other than the lesion OD, the other abnormal finding was on her cornea OD. At first it appeared normal, but when I instilled some sodium fluorescein (NaFl) dye I noticed a discrepancy from normal and a difference between the two eyes. The cornea OD didn't stain with NaFl; conversely, the dye didn't stay on the cornea at all in multiple areas. A mottled tear film was evident with some areas of negative staining OD. The tear film OS was abundant and not mottled. I calculated the tear break-up time (TBUT) at three seconds OD and eight seconds OS.

After I completed her retinal exam, which was normal OU, I performed a Schirmer's test. The Schirmer's strips wet 5 mm OD and 13 mm OS. The results of the Schirmer's and TBUT tests as well as the mottled pre-ocular tear film OD made it likely that B.D. had a dry eye, which was the most probable cause of her sporadic symptoms OD.

Fig. 1: Photo of patient's face. Notice the temporal lid droop and "lump" OD.	Fig. 2: Enlarged lacrimal gland is easily visualized when upper lid is everted.

Adding up the clues

B.D. had more severe dry eye in her right eye than her left and the lesion of her upper right lid was coincident with the lacrimal gland. The question I had was whether or not these two findings were related in any way and if B.D.'s systemic complaints of lethargy and achiness were related to her eye condition. Putting a definite diagnosis to the lesion was the most immediate and pressing chore I had. The lesion was long-standing and painless, thus allowing me to rule out acute infections such as hordeolum and dacryocystitis or dacryoadenitis.

Remember also that the lid margins and glands were free of any infection and that she hadn't been on any antibiotic for at least three weeks, thus making a chalazion an unlikely diagnosis. The position of the lesion also wasn't consistent with a chalazion. Because of its chronic nature, I felt that some type of tumor or other space-occupying lesion was the most likely etiology of the lesion.

I also felt that whatever the lesion was, it was compromising the function of the lacrimal gland because of its proximity to it. This criteria left me thinking that the lesion was either a lacrimal gland tumor, a lacrimal gland granuloma or a dermoid.

Also, whenever a lid lesion doesn't resolve with standard therapy or recurs you must be concerned about a sebaceous gland carcinoma.

Nearing a diagnosis

Modern imaging techniques make diagnosing lesions such as this one much easier and more accurate. Among the techniques available to us are B-Scan ultrasounds, CT scans, magnetic resonance imaging (MRI), orbital X-ray and biopsies of the lesion. Sometimes blood work such as a complete blood count (CBC), erythrocyte sedimentation rate (ESR) and other more specific tests are helpful in diagnosing some of the more occult types of presentations or cancers.

In this case, a B-scan ultrasound would provide a non-invasive and relatively inexpensive method of gaining more information about the lesion. The B-scan showed a definitely enlarged but solid lacrimal gland OD as compared to the lacrimal gland OS. It pinpointed the lesion as involving solely the lacrimal gland with no other tissue involvement. But other than indicating that the lesion was a solid, heterogeneous mass the B-scan gave us no other information as to whether it was cancerous, pre-cancerous or benign.

For soft tissue lesions such as this, MRI or X-rays are both excellent at defining tissue types. An X-ray of the area showed that the lacrimal gland was infiltrated by what the radiologist termed "granulomatous matter."

Putting a name to the lesion

A number of conditions cause granulomatous infiltration in the body, but few have a predilection for the lacrimal gland. Sarcoidosis is an auto-immune disorder that causes inflammatory changes in multiple tissues of the body. This inflammation results in granulomas being released and infiltrating certain organs.

The eye is a susceptible receptor site for these granulomas. To prove definitively that sarcoid was the reason for the mass I ordered an angiotensin converting enzyme (ACE) blood test and a chest X-ray. The ACE came back elevated, which is almost pathognomonic for sarcoid and the X-ray showed lung granulomas as well.

Taking care of the situation

After all this, I informed B.D. that she didn't have a tumor and that I felt that the "bump" was part of why she was feeling so poorly. I initiated artificial tear therapy q.i.d. and referred her to an internist for treatment of her sarcoid. When I last saw her about three months after initiation of her systemic therapy, B.D. was feeling much better both systemically and ocularly.

Contributing Editor Eric Schmidt, O.D., is director of the Bladen Eye Center in Elizabethtown, N.C. E-mail him at KENZIEKATE@aol