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Tommy Bocca,* one of our glaucoma patients, needed cataract surgery in his right eye. The procedure went smoothly and resulted in 20/20 uncorrected visual acuity. At his first postoperative appointment, his eye was quiet, and he had no complaints. But that was no longer the case when the 75-year-old returned a month later, obviously upset and convinced that something had gone wrong with his surgery. This time around, he complained of decreased vision, pain and lid swelling in his right eye, along with headaches.
Mr. Bocca’s history was significant for long-standing bilateral glaucoma that was well controlled with latanoprost. He also had several other health problems, notably coronary artery disease, chronic obstructive pulmonary disease and arthritis. Eighteen years previously, he had been treated for kidney cancer with a radical right nephrectomy, but he had been tumor-free since. In addition, several members of Mr. Bocca’s family—his mother, sister and two half-brothers—had been treated for various cancers.
We Get a Look
According to Mr. Bocca, several days before this visit, the vision in his right eye became blurry. He also said that it felt like he had gotten “something in his eye.” He irrigated his eye, which provided some relief.
When we examined him, we noted that the vision in his right eye had decreased to 20/200. He had chemosis and 2+ conjunctival injection but no discharge. The central and inferior cornea were edematous, and there was 2+ flare in the anterior chamber without cells. His IOP was 22 mmHg. The IOL remained in good position, and the posterior segment appeared normal.
The cause of this new inflammation was unclear. Based on his description of events, we thought he might have been exposed to a toxic substance. He was started on prednisolone, moxifloxacin and atropine drops.
We saw Mr. Bocca several times over the course of the next two weeks. During that time, his corneal edema gradually worsened. His increasing pain did not respond to topical NSAIDs or oral over-the-counter pain medications. At that point, we referred him to one of our cornea specialists for further evaluation.
The specialist noted corneal striae, lid swelling and erythema, and felt that possible orbital pseudotumor warranted oral methylprednisolone. Mr. Bocca responded well to the medication. His vision improved to 20/40, his cornea became clearer, his lid swelling resolved, and the pain eased.
Regrettably, as our patient was tapered off the oral steroids, his symptoms returned with a vengeance. Mr. Bocca’s vision once again declined to 20/200, his eyelid swelling and chemosis worsened, and his pain became increasingly severe. He now complained of severe headaches that included brow and temporal tenderness. At this time, the differential diagnosis still included orbital inflammatory disease, but we also considered the possibilities of giant cell arteritis and/or pain due to orbital ischemia associated with his cardiovascular disease.
Blood work and imaging tests were ordered, and we consulted his primary care physician. The results of his complete blood count revealed anemia, with a hematocrit of 34, and a markedly elevated erythrocyte sedimentation rate (ESR) of 98 mm per hour. A temporal artery biopsy was normal. In the meantime, Mr. Bocca was again placed on systemic corticosteroids and began to feel much better.
What’s Your Diagnosis?
|When the patient’s problems returned, we ordered blood work and imaging tests. On this CT angiogram of his head and neck, we noted randomly scattered nodules in the upper lung, prompting us to order further imaging.
However, everything changed when we saw the results of the initial imaging tests. The CT angiogram of the head and neck discovered multiple small, apical pulmonary nodules. This prompted an additional CT scan of the chest, which revealed a smooth, pleural-based, soft-tissue mass in the right lateral midchest with associated destructive rib changes.
A fine-needle biopsy of the dominant pulmonary nodule found monomorphic plasma cells. Immunohistochemical stains were positive for lambda light chains. These findings were consistent with what had become a working diagnosis of plasmacytoma and multiple myeloma.
After further testing, IgG monoclonal light chains (Bence Jones proteins) were detected in Mr. Bocca’s urine, and markedly elevated IgG was found in his serum. Serum protein electrophoresis confirmed the diagnosis of multiple myeloma.
Multiple myeloma is a B-cell malignancy characterized by monoclonal proliferation of plasma cells and monoclonal protein in the blood and urine.
Ophthalmic findings. Although it is more typically associated with hypercalcemia, renal insufficiency, anemia and bone disease, multiple myeloma has been reported to present initially with ophthalmic symptoms and findings.1 These findings can include corneal deposits and crystals, spontaneous eyelid ecchymoses, orbital swelling and proptosis causing blurred vision and pain.
As is the case with more classic presentations of the disease, ophthalmic symptoms are caused by accumulation of aberrant protein and by the development of plasmacytomas. Deposition of immunoglobulin light chains or Bence Jones proteins can occur at any level in the cornea and appear as opacities or crystals. As in Mr. Bocca’s case, patients may have blurred vision and a foreign-body sensation.
In addition, amyloid deposition can occur in the lids and within the walls of blood vessels. The resultant vascular fragility may result in eyelid or conjunctival hemorrhages following minimal trauma.2
Orbital involvement has been reported to include soft-tissue plasmacytomas as well as bony lesions. These lesions can present with headache or pain, proptosis, and diplopia. Monomorphic plasma cells may be found via fine-needle aspiration or incisional biopsy. Subsequent flow cytometry is used to further characterize the protein type.3
Typical findings. The recommended workup for multiple myeloma includes a detailed history and laboratory tests. Patients often report recent weight loss, fatigue and bone pain. Anemia is present in 73 percent of patients at diagnosis, and there is evidence of renal impairment in 20 to 40 percent. As with our patient, the ESR may be elevated. Serum and urine protein electrophoresis and bone marrow biopsy are used to characterize the cell type and associated aberrant paraprotein.1
Treatment. Treatment depends on the patient’s age and extent of disease activity. Chemotherapy and stem cell transplantation are two possible options.
Multiple myeloma is a rare cause of corneal and orbital pathology. As such, it should be a part of the differential diagnosis for unusual presentations of eye and orbital pain.
We were extremely fortunate that imaging unintentionally revealed Mr. Bocca’s lung lesions. He did not have bony involvement in the orbit, and his orbital imaging did not show any masses.
Mr. Bocca was treated with localized radiation for the pulmonary plasmacytomas as well as systemic chemotherapy for the multiple myeloma. His vision normalized, and his ocular and orbital pain resolved.
Initially, Mr. Bocca’s multiple myeloma responded well to treatment. However, the disease recurred, and he is currently undergoing repeat chemotherapy.
*Patient’s name is fictitious.
1 Palumbo A, Anderson K. N Engl J Med. 2011;364(11):1046-1060.
2 Goldstein DA et al. Cornea. 2005;24(6):757-758.
3 Malik A et al. Indian J Ophthalmol. 2009;57(5):393-395.
Ms. Mitzimberg is a certified ophthalmic assistant at the Spokane Eye Clinic in Spokane, Wash. Dr. Smit is a glaucoma specialist at the Spokane Eye Clinic and a clinical instructor of ophthalmology at the University of Washington. The authors report no related financial interests.
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