Retinal vasoproliferative tumour secondary to retinitis pigmentosa sine pigmento

Abstract

Retinitis pigmentosa can be associated with exudative vasculopathy in rare instances, which can manifest as retinal vasoproliferative tumours. We present the case of a 33-year-old woman previously diagnosed with retinitis pigmentosa sine pigmentosa in both eyes. She was asymptomatic and just came in for a routine follow-up eye examination. Thorough examination of the peripheral retina on the right eye revealed a dome-shaped retinal tumour with a feeder vessel and surrounding exudative changes at the superotemporal periphery, consistent with a secondary retinal vasoproliferative tumour from retinitis pigmentosa. She subsequently underwent focal laser photocoagulation of the tumour which resulted in tumour stabilisation. While exudative vasculopathy is very uncommon in retinitis pigmentosa, ophthalmologists need to be aware of its occurrence in such patients. Vision loss may occur from exudation, haemorrhage, retinal detachment and neovascularisation. A thorough examination of the peripheral retina is warranted in these cases.

Keywords: head and neck cancer; oncology; ophthalmology; retina.

Figure 1

Wide field fundus photograph of the right and left eyes showing mottled retinal pigment epithelium changes, attenuated retinal vessels and waxy optic disc pallor. The right eye has hazy media due to cataract. Note that the mass at the superotemporal periphery cannot be visualised even on wide field fundus photography. R, right; L, left.

Figure 2

Slit lamp photograph of the fundus of the right eye using contact lenses. View through a Mainster lens (A) shows the dome-shaped retinal mass at the superotemporal periphery. Higher magnification through a Goldmann lens (B) better visualises the mass (bordered by dotted lines) associated with a dilated feeder vessel (white arrowhead) and surrounding exudates (yellow arrowheads).

Figure 3

Full-field electroretinogram demonstrating reduction in both a-wave and b-wave amplitudes for both light-adapted and dark-adapted conditions.

Figure 4

Perimetry demonstrating complete ring scotoma for both eyes. OD, right eye; OS, left eye.

Figure 5

Fundus fluorescein angiography showing vascular leakage of the retinal vessels on the right eye. There was no leakage seen on the left eye. Note that both eyes exhibit background chorioretinal atrophic window defects. R, right; L, left.

Figure 6

Optical coherence tomography scans showing mild outer retinal atrophy but otherwise normal macular structure on both eyes. R, right; L, left.

Figure 7

Ocular ultrasonography on the right eye with transverse scanning at 11 o’clock reveals a 2.81×3.03×3.01 mm retinal mass (bordered by dotted lines) at the superotemporal periphery. The mass exhibits mixed low to moderate echogenicity on A-scan.

Figure 8

Slit lamp photograph of the fundus of the right eye using contact lens after treatment with focal laser photocoagulation showing laser marks (white arrowheads) completely bordering the tumour.