Here at Park Avenue Oculoplastic Surgeons, we have decided to start an Oculoplastics Case of the month to physicians in the Denver Metro area who have interest in seeing interesting clinical cases that we see at our practice every month that spark interest and clinical thoughtfulness.
September 2017 Case
A 64 year old male presented to a their doctor with the complaint of irritation, redness and swelling of their right eye for two weeks. At the time the vision was 20/20 without an afferent pupillary defect and good motility. Initially started on allergy drops, the patient after two weeks subsequently went and got a second opinion with an eye doctor because the patient started to develop ptosis. The second physician found that at this stage the eye motility was markedly decreased. The patient presented with ptosis of the right eye along with decreased motility in downgaze markedly but also in adduction and abduction. (The picture below reflects the patient in downgaze).
Additionally at that time, the patient was found to have 7 mm of proptosis in the right eye and a visual acuity of 20/60 with an afferent pupillary defect. The patient was sent for immediate imaging at the hospital.
Imaging revealed a mass in the medial portion of the right orbit that was infiltrating through the bone of the medial orbit and diffusely spreading along the inferior and medial rectus. There was no infiltration into the brain or sinuses.
The patient had received the imaging at a hospital emergency room and based on the clinical appearance the emergency room physician believed the patient to have orbital cellulitis. The patient was treated with IV antibiotics for 2 days with progression of symptoms until the oculoplastics team was consulted. A decision was made at that point to biopsy the mass to determine the cause. A biopsy was performed by Dr Thiagarajah using a transconjunctival approach of the inferior orbit. Several pieces were sent to pathology. Gross examination of the mass revealed a white rubbery mass.
Pathology revealed a a diffuse sheet of cells consist with lymphoma. Further testing revealed a MALT (Mucosal Associated Lymphoid Tissue) lymphoma subtype
Orbital lymphoma is a type of non-Hodgkin lymphoma that can be found in the conjunctiva, lacrimal gland, soft tissues of the eyelid, or extraocular muscles. MALT subtype is one of the most common tumors of the orbit. It accounts for 80% of all lymphoma found in the orbit.
Conjunctiva - A salmon patch appearance is common.
Orbit. - In the orbit MALT lymphoma is most commonly observed as a painless palpable mass that may also have proptosis, ptosis, diplopia, or abnormal ocular movement.
Lacrimal gland - Lacrimal gland involvement can present with bulging or prominence of the lacrimal glands but can progress to displacement of the globe in some cases.
Eyelids - Swelling or a palpable mass
Key portions of the examination include Hertel Examination or "worm's view of a patient" to determine proptosis.
Most often imaging is the next most important step . It can show enlarged extraocular muscles, mass or even enlargement of the lacrimal gland.
Lymphoid lesions of the orbit (benign reactive lymphoid hyperplasia, atypical lymphoid hyperplasia)
There are four main treatment options for orbital lymphoma: surgery, radiotherapy, chemotherapy, and immunotherapy. Often patients do need to have a systemic workup to make sure that there is no cancer in the rest of the body. Depending on whether the lymphoma is localized to the eye socket or spread to other parts determines treatment.
Stage I Confined to the orbit
Stage II Involvement of adjacent structures such as the sinuses, tonsil, and/or nose
Stage III Abdominal nodal disease below the diaphragm
Stage IV Disseminated involvement of one or more extranodal sites, such as liver or bone
Treatment considerations for the eye doctor
Surgery is often a last resort since radiation, chemotherapy and immunotherapy are the mainstay of treatment.
Radiation has high cure rates but also has the side effects that the eye doctor should be aware of:
Conjunctivitis and dry eye (most common)
Keratitis and corneal ulceration.
Immunotherapy and Chemotherapy have a secondary role for MALT lymphoma though most hematologist-oncologists manage these patients and determine what the best course of treatment is for patients.
Orbital lymphoma should be a consideration for physicians treating patients who present with a "pink eye" especially those that are not responsive to topical treatment. Motility, Exophthalmometry, and pupil examinations may prove to be critical in clinical decision making. MRI can be very helpful followed by orbital biopsy. Most patients with isolated orbital disease do well with treatment which tends to be radiation with adjunctive chemotherapy and/or immunotherapy. If you have a patient who is of clinical concern, we are happy to field a call or see the patient as a consult.