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Medline ® Abstract for Reference 22

of 'Clinical features, diagnosis, and management of von Hippel-Lindau disease'

Treatment of retinal capillary hemangioma.
Singh AD, Nouri M, Shields CL, Shields JA, Perez N
Ophthalmology. 2002;109(10):1799.
OBJECTIVE: To report on the methods of treatment and the visual outcome of eyes with retinal capillary hemangioma (RCH).
DESIGN: 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: RCH can be safely observed initially in selected cases. Laser photocoagulation and cryotherapy are the mainstays of treatment in most cases. Early detection of RCH and treatment before the onset of severe visual loss is recommended.
Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.