A rare complication of cutaneous melanoma is the development of melanoma-associated
retinopathy (MAR), a condition characterized by the acute onset of night blindness,
persistent pulsating photopsias, and constriction of the visual field.1 The pathogenetic mechanisms of the syndrome have
been clarified by the detection of immunoglobulins directed against bipolar
cells of the retina.2 The symptoms generally
spread to the other eye within an interval of 4 days to 2 months.3,4 We report a case of MAR that
remained unilateral during an observation period of 18 months.
A cutaneous malignant melanoma was removed from the left leg of a 45-year-old
woman in 1992. Because a systematic search revealed no metastasis, no further
therapy was administered.
One evening in January 2000, she suddenly experienced difficulty seeing
in dark conditions through her right eye, which greatly interfered with her
stereovision. She also reported shimmering lights that fluctuated in brightness.
Because an ophthalmological examination demonstrated good visual acuity
and no fundus alterations, the patient's complaints were judged to be of psychic
origin. Four months later, a metastatic cutaneous melanoma was removed from
her left leg. She underwent chemotherapy and was referred to our electrophysiological
laboratory because of her persisting visual complaints.
She still had a best-corrected visual acuity of 20/20 OU. Although an
ophthalmoscopy consistently showed no alterations in the right eye, a previously
unknown uveal nevus was detected in the macular area of the left eye. Computerized
perimetry (Octopus) revealed a marked visual field constriction and a general
depression of light sensitivity in the right eye. The left eye exhibited no
perimetric alterations (Figure 1).
A, Visual field of the right eye
(left) and left eye (right) estimated using Octopus equipment. The x- and
y-axes represent dimensions of the visual field in degrees. B, Cumulative
defect curves (Bebie curve) from the 59 central points in rank order cumulative
Full-field electroretinograms (ERGs) were recorded with gold-foil electrodes
in both eyes after maximal pupil dilatation and a 30-minute period of dark
adaptation. A stroboscopic lamp placed behind the patient provided flashing
lights on a Ganzfeld screen. Scotopic, maximal, and photopic responses were
recorded according to the International Society for Clinical Electrophysiology
of Vision recommendations. The single-flash, rod-dominant ERG showed a markedly
decreased scotopic response in the right eye, whereas maximal-intensity stimulation
elicited a typical ERG that showed no abnormalities, a selective b-wave depression
with a preserved a wave (Figure 2).
Both the scotopic rod-isolated response and the maximal response in the left
eye were in the normal range. Fifty individual ERG and oscillatory potential
responses to blue light flashes were averaged. The averaged ERG and oscillatory
potentials were greatly impaired in the right eye but normal in the left eye.
from the right eye (left) and left eye (right) of the patient. A, Scotopic,
rod-isolated response. B, Maximal, dark-adapted response. C, Electroretinographic
(ERG) response to red flash. D, Averaged ERG response to 50 blue flashes.
E, Averaged oscillatory potentials to 50 blue flashes. Calibrations: 50 µV;
In the immunocytochemistry test, the patient's serum displayed strong
binding to retinal bipolar cells, similar to that of a known patient with
Five control electrophysiological examinations together with perimetry
and ophthalmoscopy demonstrated no changes during the following 15-month observation
period. Unfortunately, the patient developed agranulocytosis in July 2001
and died of pulmonary metastases and respiratory failure 1 month later. The
family did not allow an autopsy.
In our case, the acute night blindness, sensations of shimmering lights,
normal ERG results, and constricted visual field, together with the normal
appearance of the retina and the preserved visual acuity, were diagnostic
of MAR. The positive immunological reaction of the serum substantiated the
diagnosis. The case is peculiar because the condition failed to spread to
the other eye during an 18-month observation period. Until recently, all reported
MAR cases were bilateral with some interocular latency difference in the appearance
of symptoms.3,4 Although
cases with unilateral normal-appearing ERGs have been reported, no evidence
of melanoma-related immune activity was found.5
In our case, some damage to the other eye might have remained undetected by
the electrophysiological tests, or the tragic outcome may have prevented a
late manifestation of the disease in that eye. Nonetheless, the relatively
long observation period raises the possibility that MAR manifests unilaterally
in some patients.
This work was supported by ETT/Hungary grants 56411 and 57404.
We thank Ann Milam, PhD (University of Pennsylvania, Philadelphia),
for performing the immunochemistry test.
Corresponding author and reprints: György Benedek, Department
of Physiology, Faculty of Medicine, University of Szeged, H-6720 Szeged, POB
427, Hungary (e-mail: email@example.com).
Janáky M, Pálffy A, Kolozsvári L, Benedek G. Unilateral Manifestation of Melanoma-Associated Retinopathy. Arch Ophthalmol. 2002;120(6):866-869. doi: