[Online First] Dominant cystoid macular dystrophy associated with mutations in the RP1L1 gene

AIM: To describe the long-term observation of vitrectomy without subretinal hemorrhage (SRH) management for massive vitreous hemorrhage (VH) secondary to polypoidal choroidal vasculopathy (PCV). METHODS: This is a retrospective, consecutive case series. A total of 86 eyes of 86 patients with >14d of massive VH associated with PCV were included. All patients underwent vitrectomy without SRH management, followed by intravitreal ranibizumab injections and/or photodynamic therapy (PDT) as needed. The main outcome measures were best-corrected visual acuity (BCVA), postoperative adverse events and the recurrence of VH. RESULTS: The average follow-up period was 25.5+/-9.2mo (range 12-35mo). Mean BCVA at baseline (2.16+/-0.39 logMAR) had improved significantly, both 3mo after surgery (1.42+/-0.66 logMAR, P<0.001) and by the last visit (1.23+/-0.74 logMAR, P<0.001). The common postoperative complications included macular subretinal fibrosis in 14 eyes (16.3%) and ciliary body detachment in 4 eyes (4.7%). Nineteen eyes (22.1%) received following treatment with ranibizumab injections without/with PDT, and 15 (17.4%) were resolved. Four eyes (4.7%) had recurrent hemorrhage during the follow-up period. In multiple regression analysis, thicker SRH (beta=0.33, P=0.025) in the preoperative B-scan and the presence of foveal subretinal fibrosis (beta=0.28, P=0.018) in the follow up were associated with poor postoperative BCVA. CONCLUSION: Vitrectomy without SRH management for massive VH secondary to PCV improved/stabilized visual function in the long-term observation. Eyes presenting with thicker SRH preoperatively and forming foveal subretinal fibrosis in the follow-up period tended to have worse BCVA.