Early-onset Neovascular Inflammatory Vitreoretinopathy Due to Two de Novo CAPN5 Mutations in Chinese Patients: A Case Series.

PURPOSE: To explore the clinical and molecular characteristics, diagnosis, and treatment of early-onset autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV) in Chinese patients. METHODS: A retrospective, interventional case series was assembled from three ADNIV patients. RESULTS: The three ADNIV cases harbored de novo CAPN5 mutations (p.Arg289Trp and p.Leu73Val). The ages of onset ranged from 11 months to 2 years. All the cases presented with vitreous opacity and subretinal inflammatory exudations. During the postoperative follow-up, all the patients manifested with exaggerated postoperative inflammatory responses. An intravitreal Ozurdex injection could not effectively control ocular inflammation in ADNIV. Laser spots after panretinal photocoagulation were partly visible. CONCLUSIONS: Two de novo CAPN5 mutations (p.Leu73Val and p.Arg289Trp) could cause early-onset ADNIV. Panretinal photocoagulation during vitrectomy and an intravitreal Ozurdex injection could not significantly stop the progression of subretinal exudations and ocular inflammation in early-onset ADNIV patients.

Visual and morphological outcomes of vitreomacular traction syndrome in retinitis pigmentosa treated by vitrectomy.

Retrospective review of 13 eyes that had undergone pars plana vitrectomy (PPV) for vitreomacular traction (VMT) syndrome in retinitis pigmentosa (RP) and had a postoperative follow-up period ≥9mo in order to evaluate whether it is necessary to treat VMT in RP by vitrectomy. The 13 RP eyes suffering from VMT were evaluated by means of best corrected visual acuity (BCVA), anterior and posterior binocular examination, spectral-domain optical coherence tomography (SD-OCT) before and after operation. We detected that although vitrectomy could improve the macular morphology in some RP patients with VMT, visual outcomes might be limited and unpredicted most likely because of the long-standing retinal dysfunction, but the vitrectomy is necessary in order to remain or improve the central vision in some cases.