LENS OPACITIES IN NEUROFIBROMATOSIS-2 - FURTHER SIGNIFICANT CORRELATIONS

This prospective study of 96 individuals from 29 families with neurofi bromatosis 2, 49 of whom were affected, confirms in an extended series the previously reported association between posterior subcapsular/cap sular cataract and neurofibromatosis 2. Posterior subcapsular/capsular cataracts were found in 36 (80%) of the 45 affected individuals (four individuals were excluded from statistical analyses). In addition, th e association of peripheral cortical lens opacities with neurofibromat osis 2 was found to be statistically significant. Seventeen of the pat ients with neurofibromatosis 2 (37.8%) had peripheral cortical catarac ts in comparison with none of the unaffected family members (p<0.0001) . In three patients peripheral cortical opacities were present despite the absence of posterior subcapsular/capsular cataracts. These findin gs support the inclusion of cortical cataracts of early onset, in addi tion to posterior subcapsular/capsular cataracts, in the diagnostic cr iteria of neurofibromatosis 2.