Unilateral pigmentary degeneration of the retina associated with heterochromia iridis.
BACKGROUND
For the past 5 years, a 56-year-old patient has been displaying monocular progressive pigmentary changes in the left eye. Heterochromy of the left eye has been known since childhood. The other eye is clinically and functionally normal. The patient was adopted and he has no children. Therefore, we have no family history.


METHODS
The patient was examined clinically and by means of electroretinography, electrooculography, perimetry, computer tomography, pulsatile ocular blood flow (POBF) measurement, serology and Doppler sonography.


RESULTS
Electrophysiology displayed a considerable reduction of scotopic and photopic ERGs, a reduced dark-through, and a reduced light-rise in the left eye, whereas the fellow eye was normal. The visual field was limited to 5 deg around the fixation point, and a peripheral crescent-shaped arch encircled the temporal-inferior quadrant concomitant to the pigmentary changes. By computer tomography and Doppler sonography a vascular affection was excluded. The left eye displayed lower POBF values. All serological tests were found negative.


CONCLUSIONS
The clinical picture and negative exclusion criteria indicate a unilateral retinitis pigmentosa. However, with regard to the literature an unequivocal diagnosis can only be made upon hereditary evidence.
Grisanti
S
S
Department for Vitreoretinal Surgery, University Eye Clinic Cologne, Germany.
Diestelhorst
M
M
Lebek
J
J
Walter
P
P
Heimann
K
K
D001783:Blood Flow Velocity; D003623:Dark Adaptation; D004585:Electrooculography; D004596:Electroretinography; D005500:Follow-Up Studies; D006801:Humans; D015863:Iridocyclitis / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D007498:Iris / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011673:Pulsatile Flow; D012174:Retinitis Pigmentosa / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D014057:Tomography, X-Ray Computed; D018608:Ultrasonography, Doppler; D014792:Visual Acuity; D058609:Visual Field Tests; D014794:Visual Fields