Objective: To describe the clinical features, association with von Hippel-Lindau (VHL) disease and visual acuity outcomes of patients with a juxtapapillary capillary hemangioma. Design: Retrospective observational case series. Participants: Seventy-two eyes of 68 patients identified with a juxtapapillary capillary hemangioma. Follow-up data of at least 6 months duration were available for 60 eyes. Methods: A retrospective chart review of patients diagnosed with a juxtapapillary capillary hemangioma examined at four medical centers. Main Outcome Measures: Age at diagnosis, visual acuity (VA) at first examination and at last follow-up, tumor growth pattern and location, associated clinical features, type of treatment, association with VHL, and presence of peripheral hemangiomas were recorded for each patient. Results: On initial examination, VA was ≥20/40 in 43 of 70 eyes (61%) and was ≥20/200 in 60 eyes (86%). At an average follow-up of 5.4 years (range, 0.5-19 years), VA of ≥20/40 was achieved in 21 eyes (35%) and ≥20/200 in 33 eyes (55%). Patients with VHL had poorer initial VA (48% vs. 70% with VA ≥20/40, and 74% vs. 93% with VA ≥20/200) and final VA (26% vs. 41% with VA ≥20/40, and 39% vs. 65% with VA ≥20/200) compared with patients without VHL. Patients with VHL more commonly were seen at an earlier age (average, 20 vs. 44 years, P < 0.001), with bilateral (17% vs. 0%), and/or peripheral (39% vs. 0%) (P < 0.001) tumors that more often had an endophytic growth pattern (63% vs. 22%, P = 0.001) compared with patients without VHL. Patients selected for laser treatment generally had poorer initial (52% vs. 74% with VA ≥20/40, 79% vs. 96% with VA ≥20/200) and final VAs (18% vs. 56% with VA ≥20/40, 45% vs. 67% with VA ≥20/200) compared with patients not treated with laser. Conclusions: On long-term follow-up of patients with a juxtapapillary capillary hemangioma, the VA generally worsens. Patients with VHL and a juxtapapillary hemangioma more often present at a younger age, have tumors with an endophytic growth pattern, and have bilateral, multiple tumors. Treatment with laser photocoagulation results in variable VA outcomes. (C) 2000 by the American Academy of Ophthalmology.
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