Pachychoroid neovasculopathy in extramacular choroidal neovascularization.

PURPOSE: To review a series of extramacular choroidal neovascular membranes (CNVMs) in the context of their choroidal features, as determined by optical coherence tomography (OCT). METHODS: Patients with extramacular CNVMs were identified from a tertiary care center through a review of records. The charts and cases were reviewed using multimodal imaging including fundus photography, OCT, fluorescein angiography (FA), and indocyanine angio-graphy (ICG). RESULTS: Of six patients with extramacular CNVMs evaluated in this series, four patients (66.7%) exhibited pachychoroidopathy on OCT imaging under or adjacent to the extramacular CNVM. All four of these patients also exhibited pachychoroidopathy in the macular OCT distant from the CNVM. CONCLUSION: Pachychoroidopathy is implicated in some cases of extramacular CNVMs. This represents the first report, to our knowledge, of pachychoroidopathy in extramacular CNVM.

Retinal vascular nonperfusion in siblings with Dandy-Walker variant.

We report the case of a 2-month-old girl with Dandy-Walker variant who presented with strabismus, pathologic myopia measuring -16.00 D in each eye, diffuse chorioretinal atrophy and pigment mottling in the macula of both eyes, and areas of retinal capillary nonperfusion in both eyes. The patient's brother also has Dandy-Walker variant and was found to have bilateral severe myopia, myopic fundi, tilted optic disks with peripapillary atrophy, extensive areas of white without pressure, areas of lattice degeneration, and several chronic-appearing atrophic retinal holes surrounded by pigmentation. We hypothesize that children with Dandy-Walker variant may present with refractive errors such as pathologic myopia and with diverse retinal findings, including retinal ischemia. A lower threshold for ophthalmologic examination may be considered in this population.

Retinal Anatomy and Pathology.

Normal retina contains neuroretina and retinal pigment epithelium. The neuroretina consists of outer and inner segments of photoreceptors (rods and cones), external limiting membrane, outer nuclear layer, outer plexiform layer, inner nuclear layer, inner plexiform layer, ganglion cell layer, nerve fiber layer and internal limiting membrane. There is a broad spectrum of retinal pathology including congenital abnormalities, dystrophies, degenerations (notably age-related macular degeneration), retinal vascular diseases, toxicities, inflammatory diseases, neoplasms, retinal detachment, trauma and retinal involvement of systemic diseases. This chapter presents a few major pathological processes in retinal diseases, especially processes that are amenable to pharmacotherapeutics.

Practice Patterns in Retinopathy of Prematurity Treatment for Disease Milder Than Recommended by Guidelines.

PURPOSE: To characterize the frequency of and clinical indications for which experts treat retinopathy of prematurity (ROP) milder than type 1 disease, the recommended threshold for treatment from established consensus guidelines. DESIGN: Descriptive analysis. METHODS: setting: Multicenter. STUDY POPULATION: A database of 1444 eyes generated prospectively from all babies screened for ROP at 1 of 6 major ROP centers whose parents provided informed consent. INTERVENTION: Retrospective review of the database and charts to identify all patients treated for ROP milder than type 1. MAIN OUTCOME MEASURE: Indication(s) for treatment. RESULTS: A total of 137 eyes of 70 infants were treated for ROP. Of these 137 eyes, 13 (9.5%) were treated despite a clinical diagnosis milder than type 1 ROP. Indications for treatment included active ROP with the fellow eye being treated for type 1 ROP (2 eyes, 15.4%); concerning structural changes (9 eyes, 69.2%), including tangential traction with temporal vessel straightening concerning for macular dragging (8 eyes, 61.5%) and thick stage 3 membranes with anteroposterior traction concerning for progression to stage 4 ROP (3 eyes, 23.1%); persistent ROP at an advanced postmenstrual age (4 eyes, 30.8%); and/or vitreous hemorrhage (3 eyes, 23.1%). CONCLUSIONS: Experts in this study occasionally recommended treatment in eyes with disease less than type 1 ROP. This study has important clinical implications and highlights the role of individual clinical judgment in situations not covered by evidence-based treatment guidelines.

Resolution of foveal schisis in X-linked retinoschisis in the setting of retinal detachment.

Patients who have undergone vitrectomy for complications of X-linkedretinoschisis (XLRS) have demonstrated an improvement in foveal schisis after pars plana vitrectomy. We report the case of a 10-year-old boy with XLRS and bilateral foveal schisis in whom macula-involving retinal detachment in one eye was associated with a resolution of foveal schisis in that eye. This case provides additional support for a role of vitreoretinal traction in the pathogenesis of foveal schisis in XLRS.

Treatment of cytomegalovirus retinitis with cytomegalovirus-specific T-lymphocyte infusion.

Cytomegalovirus (CMV) retinitis is a potentially blinding infection that affects immunocompromised patients who are unable to generate a T-cell response against the organism. Infusion of CMV-specific leukocytes has been shown to be effective in patients with systemic CMV infection, especially those resistant to standard therapies. The authors report a case of a patient with CMV viremia with progressive retinitis in whom infusion of third-party donor-derived CMV pp65-specific T cells alone prompted resolution of CMV retinitis. This case suggests a potential role for CMV-specific leukocyte infusion in the treatment of CMV retinitis, especially in cases resistant or refractory to antiviral therapies.