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Purpose of Review:Incontinentia pigmenti (IP) is a rare X-linked dominant phakomatosis that predominately presents with dermatologic manifestations but can also cause central nervous system and ocular abnormalities. Awareness of the ocular complications of IP is crucial to identify ocular abnormalities early and prevent permanent vision loss.Recent Findings:There have been significant recent advances in ocular diagnostic imaging in IP. Optical coherence tomography (OCT) has helped characterize outer plexiform layer abnormalities in the macula, which can help explain central vision loss in IP patients. OCT angiography (OCT-A) also identifies macular vascular changes that induce these foveal structural abnormalities and may supplement fluorescein angiography, the current standard of care to identify peripheral retinal ischemia and neovascularization for infants with IP. Additionally, recent studies have presented excellent anatomic outcomes years after laser photocoagulation to ischemic retina. Early data indicates that antivascular endothelial growth factor therapy can induce retinal revascularization, but runs the risk of late recurrent neovascularization and requires long-term monitoring.Summary:Ophthalmic imaging is evolving in the evaluation of IP and is increasingly guiding treatment modalities. A particular focus on the ocular manifestations of IP has been the ideal treatment for retinopathy in this disorder.

The consensus of lamellar macular hole repair generally entails vitrectomy with internal limiting membrane with/without epiretinal membrane peeling with gas tamponade, although the risk of a full thickness macular hole remains. In this case report, we investigate the role of the regenerative properties of autologous platelets in the repair of a lamellar macular hole with pars plana vitrectomy, an autologous platelet plug, and 12% C3F8 without prone posturing. All three patients in this case report experienced visual improvement along with anatomic closure of the lamellar macular hole. Further randomized studies with larger sample sizes will contribute to the existing data regarding this procedure and its outcomes.

Purpose: Dexamethasone intravitreal corticosteroid implants (OZURDEX) have been used to treat subretinal fluid associated with several retinal diseases. We describe the use of a dexamethasone intravitreal implant for symptomatic management in a case of X-linked (juvenile) retinoschisis (XLRS). Methods: A 19-year-old man diagnosed with XLRS was treated with a dexamethasone intravitreal implant for his newly symptomatic subretinal fluid. Conclusion: Dexamethasone intravitreal implant treatment in the setting of XLRS has not been previously described. To the authors' knowledge, this is the first description of the use of corticosteroid implants to treat symptomatic subretinal fluid in XLRS. Further studies are warranted to investigate the role of corticosteroid implant treatment in XLRS.