Sector retinitis pigmentosa [6 genes]

Sector retinitis pigmentosa is a rare and atypical form of hereditary retinal dystrophy in which involvement is limited to one or two sectors of the retina (with predominance of the nasal and/or lower sectors). As a common feature shared with classical RP, it shows the presence of pigment accumulations in a bone-spicule pattern, vascular thinning and possible papillary pallor, but its progression is usually slower and more localized, with affectation to the visual field according to the involved sector and relatively preserved function of the remaining retina.

RP (taking all forms into account) affects approximately 1 in 3,000-5,000 individuals. The sectoral form constitutes a minority subgroup within this spectrum, and its exact frequency is unknown.

Confirming the sector RP phenotype allows fine-tuning genetic counseling and prognosis about vision, distinguishing it from acquired pigmentations (inflammatory, traumatic, and/or vascular), planning targeted functional follow-up (visual field test, ERG, or autofluorescence/OCT) depending on the involved topography.

Service indicated upon suspicion of the disease.