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A 59-year-old white male presented to his general ophthalmologist complaining of pressure behind his left eye. He also reported declining visual acuity that was worse in the left eye than in the right. The ophthalmologist subsequently referred the patient to us for a retinal evaluation.
The patients medical and ocular history was unremarkable, and he reported no medication or tobacco use. Although we routinely question patients about tobacco use, the question is especially relevant in this case, as the patient could have cancer or metastatic disease.
Best-corrected visual acuity measured 20/20 in the right eye and 20/30 in the left. There was a 1.75D hyperopic shift in the left eye. Confrontation visual fields were constricted in the left eye but were full to careful finger counting in the right.
The pupil exam revealed a trace afferent pupillary defect in the left eye. Ocular motility testing was smooth and full in both eyes.
The anterior segment exam was unremarkable except for mild symmetric nuclear sclerosis in both eyes. The patients IOP measured 18mm Hg in the right eye and 24mm Hg in the left.
Dilated fundus exam of the left eye revealed orange-yellowish patches (figures 1 and 2). The fundus of the right eye was within normal limits. There was no vitritis in either eye.
The patients medical and ocular history was unremarkable, and he reported no medication or tobacco use. Although we routinely question patients about tobacco use, the question is especially relevant in this case, as the patient could have cancer or metastatic disease.
Best-corrected visual acuity measured 20/20 in the right eye and 20/30 in the left. There was a 1.75D hyperopic shift in the left eye. Confrontation visual fields were constricted in the left eye but were full to careful finger counting in the right.
The pupil exam revealed a trace afferent pupillary defect in the left eye. Ocular motility testing was smooth and full in both eyes.
The anterior segment exam was unremarkable except for mild symmetric nuclear sclerosis in both eyes. The patients IOP measured 18mm Hg in the right eye and 24mm Hg in the left.
Dilated fundus exam of the left eye revealed orange-yellowish patches (figures 1 and 2). The fundus of the right eye was within normal limits. There was no vitritis in either eye.
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| 1. Posterior pole view of the left eye showed these suspicious findings. 2. Fundus photo of the left eye shows pigmentary changes, namely orange-yellowish patches. |
Take the Retina Quiz
1. What do the orange-yellowish patches in the left fundus represent?
a. Fuchs spots, as seen in myopic degeneration.
b. Choroidal infiltrates.
c. Peau dorange, as seen in angioid streaks.
d. Areas of choroidal non-perfusion.
1. What do the orange-yellowish patches in the left fundus represent?
a. Fuchs spots, as seen in myopic degeneration.
b. Choroidal infiltrates.
c. Peau dorange, as seen in angioid streaks.
d. Areas of choroidal non-perfusion.
2. Which test would be least beneficial in diagnosing this patient?
a. B-scan echography.
b. Flow cytometry.
c. Magnetic resonance imaging.
d. Chest X-ray.
3. What is the correct diagnosis for this patient?
a. Acute multifocal posterior placoid pigment epitheliopathy.
b. Metastatic carcinoma.
c. Intraorbital lymphoma.
d. Myopic degeneration.
4. What is the best treatment option for this patient?
a. Systemic steroids.
b. External beam radiation.
c. Enucleation.
d. Photodynamic therapy.
5. What other body system most likely has concurrent involvement in this disease process?
a. Central nervous system.
b. Respiratory system.
c. Skeletal system.
d. Reproductive system.
For answers, see below.
Discussion
This patient has a choroidal infiltrative process in the left eye. B-scan echography demonstrated a solid lesion that involved most of the fundus and much of the intraconal orbit of the left eye (figure 3). A diagnostic A-scan revealed that the lesion was firm, regularly structured and vascular and that it had very low reflectivity.
The clinical appearance of the left fundus and echography findings led us to suspect an orbital lymphoma.
Magnetic resonance imaging (MRI) revealed a focal lobulated mass within the left orbit that encased the left globe and adjacent optic nerve. No mass was present within the right orbit, and no intracranial masses were present. Whole-body computed tomography (CT) and positron emission tomography (PET) scans showed no evidence of any masses or infiltrates.
Orbital biopsy of the left eyeincluding cytology, immunohistochemistry and flow cytometry testingconfirmed the presence of non-Hodgkins B cell lymphoma. This is the most common type of intraocular lymphoma.
Intraocular lymphoma has two distinct forms. One form, known as primary central nervous system lymphoma (PCNSL), originates in the central nervous system (CNS), including the retina. A second form, non-PCNSL, originates outside the CNS with intraocular metastasis. Primary intraocular lymphoma (PIOL), previously called reticulum cell sarcoma, is a subset of PCNSL. PIOL initially presents in the eye with or without CNS involvement.
PCNSL has a much higher prevalence than non-PCNSL.1 Even so, PCNSL is relatively rare, accounting for about 1% of all primary intracranial tumors.1 The incidence is rising, however, due to the increased number of patients who have immunodeficiency and immunosuppression. In immunocompetent patients, the median age of onset is the late 50s and 60s.1 The disease occurs more often in males than females (1.2 to 1.7 males for every female).1
PIOL often masquerades as chronic posterior uveitis. The most common ocular complaints are blurred vision and floaters. Occasionally, the eyes are red or painful. The disease is bilateral in 80% of cases.2
Typical findings on ophthalmic exam may include:
Keratic precipitates and mild anterior chamber cells and flare. But, we do not frequently observe these anterior segment findings.
Vitreous cells and haze. These are the most common ocular findings, although they were absent in this patient.
Multiple orange-yellowish subretinal infiltrates. These infiltrates, which result in overlying detachments of the retinal pigment epithelium (RPE), are pathognomonic for PIOL. These orange-yellowish changes are evident in the peripheral retina of our patient, as is an overlying exudative retinal detachment. Lymphoma infiltration under the RPE is recognized as leopard skin pigmentation overlying the mass. These infiltrates are readily apparent in the fundus photos of the patients left eye (figures 1 and 2).
Window defects on fluorescein angiography. These defects seem to correspond with tumor infiltrates at the level of the RPE. Early hypofluorescence of the retinal punctate lesions and infiltrates is typically followed by late hyperfluorescence.
Once PIOL is suspected, CNS involvement must be ruled out. So, promptly refer the patient for CT and/or MRI of the brain and for a lumbar puncture to evaluate the cerebrospinal fluid. CNS involvement eventually develops in about 85% of PIOL patients.3
A full systemic work-up also is necessary to rule out metastatic lymphoma. This work-up should include CT scans of the chest, abdomen and pelvis and a biopsy of the patients bone marrow.
Our patient underwent 17 cycles of external beam radiation therapy. If malignant lymphoid disease is confined to the orbit, patients typically receive 30Gy of photon irradiation. This dose has a success rate of about 97% in controlling the orbital involvement.4 Possible complications that may result from radiation treatment include cataract formation, radiation retinopathy and dry eye secondary to damage to the lacrimal and meibomian glands. Alternative therapies include chemotherapy and chemotherapy combined with radiotherapy.
In our patient, radiation therapy resulted in marked involution of the choroidal infiltrative mass in his left eye. His vision remains stable at 20/20 O.D. and 20/25 O.S., with a reduction in the previously noted hyperopic shift. We continue to comanage the patient with his oncologist. He presents every four months for serial photography and echography to monitor for any changes.
Answers: 1) b; 2) d; 3) c; 4) b; 5) a
Co-written by Lee McPherson, O.D., an optometric resident at Bascom Palmer Eye Institute in Miami.
This patient has a choroidal infiltrative process in the left eye. B-scan echography demonstrated a solid lesion that involved most of the fundus and much of the intraconal orbit of the left eye (figure 3). A diagnostic A-scan revealed that the lesion was firm, regularly structured and vascular and that it had very low reflectivity.
 |
| 3. B-scan echography demonstrated a solid lesion involving most of the fundus and much of the intraconal orbit O.S. |
The clinical appearance of the left fundus and echography findings led us to suspect an orbital lymphoma.
Magnetic resonance imaging (MRI) revealed a focal lobulated mass within the left orbit that encased the left globe and adjacent optic nerve. No mass was present within the right orbit, and no intracranial masses were present. Whole-body computed tomography (CT) and positron emission tomography (PET) scans showed no evidence of any masses or infiltrates.
Orbital biopsy of the left eyeincluding cytology, immunohistochemistry and flow cytometry testingconfirmed the presence of non-Hodgkins B cell lymphoma. This is the most common type of intraocular lymphoma.
Intraocular lymphoma has two distinct forms. One form, known as primary central nervous system lymphoma (PCNSL), originates in the central nervous system (CNS), including the retina. A second form, non-PCNSL, originates outside the CNS with intraocular metastasis. Primary intraocular lymphoma (PIOL), previously called reticulum cell sarcoma, is a subset of PCNSL. PIOL initially presents in the eye with or without CNS involvement.
PCNSL has a much higher prevalence than non-PCNSL.1 Even so, PCNSL is relatively rare, accounting for about 1% of all primary intracranial tumors.1 The incidence is rising, however, due to the increased number of patients who have immunodeficiency and immunosuppression. In immunocompetent patients, the median age of onset is the late 50s and 60s.1 The disease occurs more often in males than females (1.2 to 1.7 males for every female).1
PIOL often masquerades as chronic posterior uveitis. The most common ocular complaints are blurred vision and floaters. Occasionally, the eyes are red or painful. The disease is bilateral in 80% of cases.2
Typical findings on ophthalmic exam may include:
Keratic precipitates and mild anterior chamber cells and flare. But, we do not frequently observe these anterior segment findings.
Vitreous cells and haze. These are the most common ocular findings, although they were absent in this patient.
Multiple orange-yellowish subretinal infiltrates. These infiltrates, which result in overlying detachments of the retinal pigment epithelium (RPE), are pathognomonic for PIOL. These orange-yellowish changes are evident in the peripheral retina of our patient, as is an overlying exudative retinal detachment. Lymphoma infiltration under the RPE is recognized as leopard skin pigmentation overlying the mass. These infiltrates are readily apparent in the fundus photos of the patients left eye (figures 1 and 2).
Window defects on fluorescein angiography. These defects seem to correspond with tumor infiltrates at the level of the RPE. Early hypofluorescence of the retinal punctate lesions and infiltrates is typically followed by late hyperfluorescence.
Once PIOL is suspected, CNS involvement must be ruled out. So, promptly refer the patient for CT and/or MRI of the brain and for a lumbar puncture to evaluate the cerebrospinal fluid. CNS involvement eventually develops in about 85% of PIOL patients.3
A full systemic work-up also is necessary to rule out metastatic lymphoma. This work-up should include CT scans of the chest, abdomen and pelvis and a biopsy of the patients bone marrow.
Our patient underwent 17 cycles of external beam radiation therapy. If malignant lymphoid disease is confined to the orbit, patients typically receive 30Gy of photon irradiation. This dose has a success rate of about 97% in controlling the orbital involvement.4 Possible complications that may result from radiation treatment include cataract formation, radiation retinopathy and dry eye secondary to damage to the lacrimal and meibomian glands. Alternative therapies include chemotherapy and chemotherapy combined with radiotherapy.
In our patient, radiation therapy resulted in marked involution of the choroidal infiltrative mass in his left eye. His vision remains stable at 20/20 O.D. and 20/25 O.S., with a reduction in the previously noted hyperopic shift. We continue to comanage the patient with his oncologist. He presents every four months for serial photography and echography to monitor for any changes.
Answers: 1) b; 2) d; 3) c; 4) b; 5) a
Co-written by Lee McPherson, O.D., an optometric resident at Bascom Palmer Eye Institute in Miami.
1. Chan CC, Buggage RR, Nussenblatt RB. Intraocular lymphoma. Curr Opin Ophthalmol 2002 Dec;13(6):411-8.
2. Davis JL. Diagnosis of intraocular lymphoma. Ocul Immunol Inflamm 2004 Mar;12(1):7-16.
3. Char DH. Intraocular lymphoid lesions. In: Char DH. Clinical Ocular Oncology. 2nd ed. Philadelphia: Lippincott-Raven, 1997:192-200.
4. Lutz SC, Anderson SF, Wu CY, Townsend JC. Non-Hodgkins orbital lymphoma. Optom Vis Sci 2001 Sep;78(9):639-45.
Vol. No: 142:7Issue:
7/15/2005
