Patterns of visual field progression in patients with retinitis pigmentosa

Ophthalmology. 1998 Jun;105(6):1069-75. doi: 10.1016/S0161-6420(98)96009-2.


Objective: The purpose of the study was to determine whether distinct patterns of visual field progression are present in patients with retinitis pigmentosa (RP) and to evaluate the correlation between these patterns, if present, and different genetic subtypes of RP.

Design: A retrospective analysis of patterns of visual field progression in RP was performed.

Participants: Visual fields of 162 patients with RP, including 55 with type 2 Usher syndrome, who had at least 3 Goldmann visual field examinations during a period of at least 3 years were reviewed.

Main outcome measures: Goldmann visual fields.

Results: Visual fields of 86 patients could be classified into one of three specific patterns of visual field progression. Pattern I included those patients with a progressive concentric loss of visual fields; pattern II included those with visual field loss that began superiorly and subsequently developed an arcuate scotoma that progressed either from the nasal (IIA) or the temporal (IIB and IIC) side; and pattern III included patients whose visual field loss was characterized initially by a complete or incomplete midperipheral "ring scotoma" that broke through into the periphery. The end stage of all these patterns was a residual central visual field, sometimes also associated with a small peripheral island. In 53 of the 162 patients, the pattern of visual field loss could not be categorized because of an advanced stage of field loss at the time of the initial examination.

Conclusions: Distinctive patterns of visual field progression can be observed in patients with retinitis pigmentosa and type 2 Usher syndrome. There were no intrafamilial variations in the pattern of visual field loss in our data on 24 patients from 11 families. Within certain genetic subtypes, there was a predilection for a preponderance of a specific pattern of visual field progression. Future studies may be able to correlate these patterns of visual field loss with different genetic mutations. A greater understanding as to why certain patterns of field loss exist could potentially provide greater insight into the various pathogenetic mechanism(s) by which photoreceptor cells degenerate in this group of patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Deafness / congenital
  • Deafness / physiopathology
  • Disease Progression
  • Genetic Linkage
  • Humans
  • Retinal Degeneration / congenital
  • Retinal Degeneration / physiopathology
  • Retinitis Pigmentosa / genetics
  • Retinitis Pigmentosa / physiopathology*
  • Retrospective Studies
  • Vision Disorders / physiopathology*
  • Visual Field Tests
  • Visual Fields*
  • X Chromosome