Unusual presentation of retinitis pigmentosa with vasoproliferative tumour

A woman in early 70s with retinitis pigmentosa (RP) had sudden decrease in vision in her right eye. She previously had cataract surgery in left eye. Her best corrected visual acuity (BCVA) was 20/80 in the right eye and 20/200 in the left eye. Examination showed corneal decompensation, advanced pigmentary retinopathy and macular oedema in both eyes. Optical coherence tomography (OCT) confirmed bilateral cystoid macular oedema, initially thought to be from pigmentary retinopathy. However, fundus angiography found a vasoproliferative tumour (VPT), measuring 3.187 mm × 3.477 mm, in the temporal periphery of the right eye, identified as VPT. Multiple Intravitreal ranibizumab injections resolved the macular oedema over few years. During VPT treatment, corneal decompensation worsened in right eye, causing severe corneal oedema and Descemet’s folds, reducing vision to counting fingers. She underwent iris claw lens implantation and penetrating keratoplasty (PKP) in the right eye. After surgery, a VPT was clearly visible in the peripheral fundus.