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Objectives To evaluate the outcomes and complications of Descemet-stripping automated endothelial keratoplasty (DSAEK) in children with congenital hereditary endothelial dystrophy (CHED). Methods Eighteen eyes from 10 children with CHED who underwent standard phakic DSAEK were enrolled in this retrospective interventional case series. Medical records including slit-lamp biomicroscopy, cycloplegic refraction, visual acuity, and measurements of central corneal thickness, corneal topography, and endothelial cell density were evaluated. All complications were recorded. Results The mean age of cases was 8.1±4.2 (range: 3-16) years at the time of surgery. Descemet-stripping automated endothelial keratoplasty was performed successfully in all eyes, which survived in 16/18 (88.9%) of cases during a median follow-up period of 38 (range 19-64) months. In patients with clear graft, best-corrected visual acuity was improved to a range of 20/100 to 20/40 and improved to fix and follow in one younger child. Endothelial cell density decreased by an average of 42.1%, comparing baseline and 6-month follow-up and was relatively stable thereafter. A marked flattening of the anterior corneal curvature (mean change of anterior K reading: +2.56±3.14 diopters) was observed after DSAEK. The only major complication in patients with clear graft was intraocular pressure elevation in 61.1% of the eyes, which was easily controlled with one antiglaucoma medication. None of the eyes developed cataract. Conclusion Descemet-stripping automated endothelial keratoplasty is a safe and successful procedure in children with an acceptable graft survival in a mid-term follow-up period. A marked hyperopic shift could occur after DSAEK in children with CHED.

Purpose Lichen planopilaris (LPP) is a chronic lymphocytic- mediated disorder that selectively involves hair follicles, leading to cicatricial alopecia. The eyelash follicle and meibomian gland (MG) might also be involved in the disease process leading to ocular surface problems. Methods In this Cross-sectional case -control study, twenty-three patients with histologically confirmed LPP and 23 healthy controls were recruited in this controlled study. To quantify the signs and symptoms of LPP, we used the Lichen Planopilaris Activity Index. The ophthalmologic examination was performed by the same ophthalmologist at Farabi Eye Hospital. Results Patients with LPP had a statistically significant lower tear breakup time (worse test outcome) compared with the control (7.1 6 4.1 vs. 9.6 6 4.4; respectively, P = 0.011). Both MG quality score and expressibility were higher in patients with LPP than in control subjects (14.1 6 4.3 vs. 9.3 6 4.4; P = 0.001; 1.5 6 1.0 vs. 0.4 6 0.8; P = 0.001; respectively). The Schirmer test score was not different between groups. A significant but weak correlation was found between the Lichen Planopilaris Activity Index and tear breakup time. Other ophthalmologic examinations including conjunctiva, lid margin shape, intraocular pressure, and funduscopy were normal in patients with LPP. Conclusions Patients with LPP, scored worse in ocular surface tests. This finding warrants special scrutiny for dry eye and MG dysfunction in these patients.

Purpose To describe a case of bilateral choroidal neovascularization in a patient with beta-thalassemia major and an interventional therapeutic attempt. Method Retrospective case report. Result A known case of beta-thalassemia major presented with gradual vision loss. Fundoscopy revealed bilateral macular edema accompanied by intraretinal hemorrhages. Optical coherence tomography and fluorescein angiography were compatible with the diagnosis of choroidal neovascularization. No signs of angioid streaks or other related abnormalities were noticed in examination and imaging. The patient was treated with intravitreal bevacizumab, and visual acuity improved within 6 months of follow-up. Conclusion Bilateral choroidal neovascularization can be a presenting pathology in patients with beta-thalassemia major. Anti-vascular endothelial growth factor is a promising treatment option.

Purpose To evaluate the best piggyback intraocular lens (IOL) implantation method and the outcomes in nanophthalmos eyes and to define the postoperative structural changes. Setting Farabi Eye Hospital, Tehran, Iran. Design Prospective case series. Methods Candidates for refractive surgery who were nanophthalmic were recruited. Patients had refractive lens exchange followed by implantation of both IOLs in the bag or 1 IOL in the bag and 1 IOL in the sulcus. The baseline and follow-up visual acuity, refractive status, and structural Scheimpflug imaging were evaluated. Ultrasound biomicroscopy (UBM) was performed 6 months postoperatively. Results The study comprised 9 nanophthalmic patients (18 eyes) with a mean preoperative uncorrected distance visual acuity (UDVA) of 1.53 logarithm of the minimum angle of resolution (logMAR) ± 0.3 (SD), mean corrected distance visual acuity (CDVA) of 0.34 ± 0.2 logMAR, and mean spherical equivalent (SE) of +13.55 ± 4.0 diopters (D). The mean postoperative UDVA improved from baseline. There was no significant difference in the mean UDVA and CDVA between the 2 IOL groups. Postoperatively, both groups had a significant improvement in SE, a significant rise in anterior chamber depth and angle, and similar UBM measurements. Conclusions Piggyback IOL implantation was an effective refractive procedure in nanophthalmic eyes. Both implantation methods resulted in similar outcomes; however, the small number of patients in each group made it less likely that possible differences would be found. The increase in angle values might help prevent the development of closed-angle glaucoma.

The purpose of this study was to evaluate the vascular density (VD) and the flow area on optic nerve head (ONH) and peripapillary area, and the impact of age and sex using optical coherence tomography angiography (OCTA) in healthy human subjects.Both eyes of each volunteer were scanned by an RTVue XR Avanti; Optovue with OCTA using the split-spectrum amplitude-decorrelation angiography algorithm technique. Masked graders evaluated enface angiodisc OCTA data. The flow area of ONH and the VD were automatically calculated.A total of 79 eyes of patients with a mean age of 37.03±11.27 were examined. The total ONH (papillary and peripapillary) area VD was 56.03%±4.55%. The flow area of the ONH was 1.74±0.10 mm/1.34 mm. The temporal and inferotemporal peripapillary VD was different between male and female patients. Increasing age causes some changes in the flow area of the ONH and the papillary VD from the third to the fourth decade (analysis of variance test; P0.05).A normal quantitative database of the flow area and VD of the papillary and peripapillary area, obtained by RTVue XR with OCT angiography technique, is presented here.