Penetrating keratoplasty in brittle Cornea syndrome: Case series and review of the literature.

It concerns three siblings (two 28 year old twin boys and a 25 year old woman) who presented a previous history of rupture of eyeball in one eye and very poor vision in the other. At the first ophthalmoscopic and instrumental evaluation, three patients presented with bluish sclera and keratoglobus in the intact eye. A genetic analysis with whole exome sequencing was then performed on the three siblings, identifying a biallelic variant of the PRDM5 gene that led to the diagnosis of Brittle Cornea Syndrome (BCS), a rare autosomal recessive disorder characterized by corneal thinning and blue sclera. To preserve the only intact eye from possible breakage, the three siblings were trained in using protective measures (polycarbonate goggles etc.) to carry out close monitoring of symptoms and were asked to continue with follow-up visits for ocular and systemic diseases associated with BCS. Given the poor best corrected visual acuity achievable with glasses and contact lenses, penetrating keratoplasty was performed, achieving good visual acuity maintained in the 2-year follow-up in two of the three patients. Knowledge of this pathology and its clinical manifestations is essential for early diagnosis and correct management of this rare but very debilitating pathology. To our knowledge, this is the first case series of BCS reported in an Albanian population.

Argon laser trabeculoplasty for congenital ectropion uveae: Case report and review of the literature.

A 18-year-old woman with unilateral glaucoma secondary to congenital ectropion uveae (CEU) in the right eye, without other systemic and ocular associations. Intraocular pressure (IOP) was uncontrolled under topical therapy. The visual field (VF) was within normal limits; retinal nerve fiber layer (RNFL) thickness, as evaluated by Optical Coherence Tomography (OCT), was reduced. The patient refused surgical intervention. After evaluation of the iridocorneal angle, ALT was performed as follows: 95 spots were applied for the 270 ° of visible trabecular meshwork using a laser beam diameter of 50 µm, an exposure time of 0.1 s and a power of 750 mw). Mean IOP in the right eye, as evaluated by diurnal tonometric curve, was 10 mmHg, 16 mmHg and 20 mmHg respectively one month, four months and eight months after ALT, with no additional topical therapy, and 18 mmHg twelve months after ALT with topical therapy. CEU is a rare, non-progressive anomaly characterized by the proliferation of iris pigment epithelium on anterior surface of iris; glaucoma, in this disorder, is often poorly controlled with medical therapy alone. In a phase of initial glaucomatous damage and with a not particularly high IOP, as in this case, ALT could be an alternative therapeutic option at least in the short-term, when surgery is not feasible immediately. In fact, given its limited effectiveness over time, ALT can be used as a temporary control measure of IOP, pending surgery to become feasible.

Live surgery outcomes in cataract surgery.

PURPOSE: To evaluate and compare the outcomes of live surgery (LS) and no-live surgery (NLS) on cataract surgery with implantation of different types of intraocular lenses (IOLs). METHODS: Retrospective, contralateral eye, case series of patients that underwent cataract surgery in live or non-live view during two consecutive editions of national meetings. Both eyes of the same patients were implanted with the same IOL, one in LS and the other in NLS. RESULTS: 108 eyes of 54 patients, aged between 50 and 82 (72 ± 8.2) and implanted with different types of IOLs, were reviewed. Both eyes in each patient were well matched at baseline in terms of intraocular biometric characteristics, corneal curvature and endothelial cell density (ECD) (p > 0.05). There are no statistically significant differences between the biometric and topographic parameters, aberrometric data and the loss of ECD in the post-operative outcomes (p > 0.05). However, comparing the different types of IOLs, there is a significant loss of ECD in eyes implanted with a toric IOL during LS (p = 0.0014 and p = 0.04, in 2017 and 2018 edition respectively). CONCLUSIONS: In this series of live cataract surgery, eyes operated in LS or NLS have comparable outcomes, underlying the importance and the benefits of live view in terms of medical education and the low-risk of complications. Nevertheless, we have found a significant increase in ECD loss in patients implanted with toric IOLs during LS.

Evaluation of Demarcation Line after Epithelium-Off Iontophoresis Corneal Collagen Cross-Linking for Progressive Keratoconus.

The aim of the study was to visualize and evaluate the characteristics and depth of the demarcation line with anterior segment optical coherence tomography (AS-OCT) after epithelium-off iontophoresis corneal collagen cross-linking (epi-off I-CXL). In this prospective, consecutive, single center study 18 eyes of 18 patients with keratoconus were involved. One month after epi-off I-CXL, all the patients underwent an AS-OCT scan to search for a demarcation line and its characteristics. The corneal stromal demarcation line was identified in all the eyes. Mean depth of the corneal stromal demarcation line was 261.8 ± 46.7 µm (range: 184 to 362 µm), at 56.7 ± 12% corneal depth. In conclusion, epi-off I-CXL determines a demarcation line that can be visualized with AS-OCT, which seems clearly distinguishable and similar to that created in standard CXL.