Retrospective consecutive noncomparative case series.
PARTICIPANTS: Data on 68 patients with RCH were reviewed for the methods of treatment and visual outcome.
METHODS: Statistical analysis using cumulative multinomial logistic regression (generalized estimating equations) was performed to determine significant predictors of visual outcome.
RESULTS: Among 68 patients with RCH with
von Hippel-Lindau disease (n = 31) and without
von Hippel-Lindau disease (n = 37), we identified 174 RCH in 86 eyes. The overall median age at diagnosis of RCH was 30.8 years (range, 2.8-73.8 years). The RCH was bilateral in 18 cases (26%), and a family history of
von Hippel-Lindau disease was positive in 19 cases (28%). Twenty-nine of the
tumors (17%) touched the optic disc and were classified as juxtapapillary RCH, and the remaining 145 (83%) were extrapapillary in location. Ninety-nine (58%) RCH were 1.5 mm or smaller in size. The RCH were initially managed by observation (46%),
laser photocoagulation (25%), or
cryotherapy (23%). Small RCH (< or =1.5 mm in size; 63 of 99; 64%) and those touching the optic disc (14 of 29; 48%) were more likely to be initially observed. Sixty-three (82%) of the 77 RCH that were initially observed remained stable for a median follow-up of 84 months. The remaining 14 progressed and were successfully controlled with
laser photocoagulation or
cryotherapy. Either
laser photocoagulation or
cryotherapy was effective as the sole method of treatment in controlling 74% (26 of 35) and 72% (28 of 39) of extrapapillary
tumors, with a mean number of 1.2 and 1.1 sessions, respectively. In a multivariate model, the only variables that were significantly related to final vision of </= 20/400 were poor initial vision (P = 0.01, odds ratio [OR], 8.5; 95% confidence interval [CI], 1.7,42) and the presence of
retinal/
vitreous hemorrhage (P = 0.024, OR, 5.7; 95% CI, 1.3, 25.6).
CONCLUSIONS: