Bilateral vasoproliferative retinal tumors in a patient with autosomal dominant aniridia.
OBJECTIVE
To document retinal vasoproliferative tumors in a patient with aniridia.


METHODS
A 32-year-old woman with known autosomal dominant aniridia and lifelong visual acuity of approximately 20/80 noted further visual acuity loss.


RESULTS
Examination revealed horizontal nystagmus and visual acuity of 20/100 in both eyes. Both eyes displayed a narrow rim of rudimentary iris with visualization of the entire lens and zonule. The cornea was clear and minimal posterior subcapsular cataract was noted. Both eyes showed diffuse retinal pigment epithelial alterations. The fovea was flat and without depression on optical coherence tomography. Inferiorly in both eyes was exudative retinal detachment from ill-defined vascular retinal tumors of approximately 15 mm diameter and 3.5 mm thickness. Both eyes were treated with plaque radiotherapy.


CONCLUSIONS
Although aniridia typically affects the anterior segment of the eye, vision-threatening retinal detachment from vasoproliferative tumor can occur.
From the *Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania; the †Department of Ophthalmology, University of Washington, Seattle, Washington; and ‡Vitreoretinal Associates, Seattle, Washington.
Angela K
AK
Au
Carol L
CL
Shields
Robert E
RE
Kalina
Craig G
CG
Wells
Jerry A
JA
Shields