Juxtapapillary capillary hemangiomas: Clinical features and visual acuity outcomes

Abstract

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.