ORIGINAL ARTICLES   

Minerva Oftalmologica 2007 September;49(3):73-6

Copyright © 2007 EDIZIONI MINERVA MEDICA

language: Italian

Clinical study on pericentral retinitis pigmentosa: correlation between inherited and sporadic forms

Maggio E., Maggio L., Solimeo A., D’Acunzo T., Gesualdo C., Testa F.

Dipartimento di Oftalmologia Seconda Università degli Studi di Napoli, Napoli

Aim. Pericentral retinitis pigmentosa is an atypical form of retinitis pigmentosa characterized by midperipheral retinal pigment epithelial atrophy or bone spicule pigmentation, a normal-appearing disk, macula and far periphery, minimal retinal arteriolar narrowing. The purpose of the study is to describe the clinical features of pericentral retinitis pigmentosa with reference to the transmission model.
Methods. Thirty-nine patients, 17 subjects from 7 families and 22 unhereditary cases, affected by pericentral pigmentary retinal degeneration were followed for a mean period of 10 years. All the patients underwent a complete ophthalmologic evaluation including ocular anamnesis, Ishihara color test, best-corrected visual acuity, biomicroscopy, fundus oculi examination, visual field evaluation, fundus photography, electroretinography (ERG) and fluorescein angiography (FAG). A genetic consult was made in order to identify the transmission model.
Results. Five patients demonstrated an autosomal dominant pattern of inheritance, while 12 exhibited an autosomal recessive pattern. In the remaining subject no hereditary transmission was detected. The majority of patients didn’t show a progression of the visual acuity defect over a mild period of ten years. Patients with a defined transmission model presented less severe clinical features compared to the remaining patients.
Conclusion. Similarly to previous studies, our examination showed that the progression of pericentral retinitis pigmentosa is slower than that of typical forms of RP. Moreover, we noticed that, among patients affected by pericentral retinitis pigmentosa, those with a defined transmission model showed a slower progression of clinical alterations compared to the remaining patients.