An atypical form of retinitis pigmentosa: A case report

This case contributes to the new knowledge of unilateral pericentral RP. Multimodal imaging provides valuable information to better understand the disease. The development is significantly slow and defines a better clinical course than typical RP. Pericentral RP presents with unremarkable night blindness, which can be easily misdiagnosed by ophthalmologists.

Unilateral pericentral retinitis pigmentosa (RP) is a rare, atypical, mild form of RP that affects the pericentral retina close to the vascular arcades and tends to spare the far-peripheral retina in one eye, while the fellow eye remains completely unaffected. A 71-year-old Chinese woman presented with blurred vision in her right eye for several decades, however, her left eye was unaffected. Fundus photography revealed bone-spicule pigmentation located at the pericentral retina of her right eye. Spectral-domain optical coherence tomography revealed significant atrophy of the outer retina and retinal pigment epithelium, and an evident absence of the ellipsoid zone in the right eye. The visual field showed a wide-range paracentral scotoma in the right eye. The full-field electroretinogram demonstrated subnormal amplitudes of dark and light adaption in the right eye. No significant changes were observed during a further 5-year follow-up. Multimodal imaging provides valuable information to better understand unilateral pericentral RP. The development of unilateral pericentral RP is significantly slow and defines a better clinical course than the typical RP. Pericentral RP presents with slight visual reduction and unremarkable night blindness, which can be easily misdiagnosis by ophthalmologists. Reasonable prevention measures, screening methods, and timely diagnosis are beneficial for RP patients.