Title : An unusual presentation of retinitis pigmentosa with vasoproliferative tumor
Abstract:
Introduction: Retinal vasoproliferative tumors (VPTs) are rare benign lesions in the inferotemporal peripheral retina, appearing yellowish red on fundoscopy and containing vascular and glial components. While most VPTs occur without a known cause, a few are associated with conditions like retinitis pigmentosa (RP) which is characterized by progressive decline of both rod and cone photoreceptor cells resulting in night blindness and poor visual acuity.
While similar cases have been reported, this article offers a unique perspective by highlighting the use of anti-vascular endothelial growth factor (VEGF) therapy for managing cystoid macular edema secondary to RP with VPT.
Methods: Informed consent was obtained from the patient for the publication of this case report. The authors confirm that no data included in this submission can reveal the patient's identity.
Case Presentation: A 70-year-old female with RP presented with sudden decreased vision in her right eye. She was bilaterally pseudophakic, with a history of complicated cataract surgery in the right eye and recent cataract surgery in the left eye. Her best corrected visual acuity (BCVA) was 20/30 in the right eye and 20/80 in the left. Examination revealed decompensating cornea, advanced pigmentary retinopathy, and macular edema in both eyes.
Optical coherence tomography (OCT) confirmed bilateral macular edema. Initially, it was attributed to cataract surgery or pigmentary retinopathy. However, fundus angiography revealed a well demarcated hyperfluorescent area of 3.187 mm by 3.477m (2-disc diameter horizontally and 2-disc diameter vertically) in the periphery of temporal quadrant just at the end of the superior vascular arcade of the right eye, identified as a VPT. The patient received intravitreal anti-VEGF injection (ranibizumab), resolving the macular edema over one year.
During VPT treatment, her corneal decompensation worsened, leading to severe corneal edema and Descemet’s folds in the right eye, reducing vision to counting fingers. She underwent right eye artisan iris claw lens implantation and penetrating keratoplasty (PKP). Post-PKP, the capillary hemangioma was clearly visible at the peripheral fundus.
Conclusion: In conclusion, as of today, no one treatment has been declared as the gold standard management of VPTs. While several treatment options have been used for VPTs, including observation, laser photocoagulation, plaque brachytherapy and photodynamic therapy, this case report shows that anti-VEGF intra-vitreal injections could also potentially be an effective way to manage VPTs.
Presentation of such cases thus provides important data on deciding on the best treatment modality and act as steppingstones for further research studies and interventions.
