First XEN implantation in Axenfeld- Rieger syndrome: A case report and literature review.

Purpose: To present the first report of a XEN45 gel stent implantation in a female with Axenfeld-Rieger syndrome (ARS), a rare congenital anomaly caused by abnormal neural crest migration during early embryogenesis. This shows promise as new minimally invasive therapeutic option in the treatment of secondary glaucoma in ARS. Observations: A 31-year-old female with known sporadic ARS was evaluated and treated at the Edith Wolfson Medical Center in Holon, Israel. The vision in her right eye was hand motion and 20/25 in the left eye. In the left eye the intraocular pressure (IOP) was up to 31 mmHg under maximal tolerated treatment. She refused Trabeculectomy or Glaucoma Drainage Device (GDD) surgery, but agreed to Minimally Invasive Glaucoma Surgery (MIGS). A Xen device was implanted in uneventful surgery. 15 months post operatively her IOP is 8 mmHg. Conclusions: XEN implantation, when technically feasible, is a suitable procedure in ARS. This shows promise as new minimally invasive therapeutic option in the treatment of secondary glaucoma in ARS. This has particular significance as these patients often require surgery at a young age.

Efficacy and safety of the ab interno gelatin stent in severe pseudoexfoliation glaucoma compared to non-pseudoexfoliation glaucoma at 6 months.

PURPOSE: To compare the efficacy and safety of the XEN45 Gel Stent surgery between patients with and without severe pseudoexfoliation glaucoma. METHODS: In this retrospective, single-center, comparative chart review, records of 24 eyes of 23 patients with pseudoexfoliation glaucoma and 24 eyes of 23 patients with non-pseudoexfoliation glaucoma with severe glaucoma, defined as uncontrolled intraocular pressure on maximally tolerated medical therapy, were reviewed. All patients were treated with XEN45 Gel Stent surgery, in either a standalone procedure (84.8%) or combined with phacoemulsification (15.2%). Mean intraocular pressure, mean number of intraocular pressure-lowering medications, change in best-corrected visual acuity and needling or rescue surgery rates were assessed. Success was defined as at least 20% decrease in baseline intraocular pressure among treatment-free patients. RESULTS: Patient characteristics were similar between the groups. At 6 months, intraocular pressure in the pseudoexfoliation glaucoma group decreased by 32% (24.3 ± 9 mmHg-14.8 ± 7 mmHg, p < 0.001), with comparable decrease in intraocular pressure in the non-pseudoexfoliation glaucoma group (22.6 ± 7 mmHg-16.7 ± 6 mmHg, p = 0.011). Similar rates of patients required topical anti-glaucoma therapy (29% vs 22%, p = 0.559), needling (54% vs 37%, p = 0.247), and rescue trabeculectomy (13% in both, p = 1.00) at the last follow-up. However, intraocular pressure decreased more in the pseudoexfoliation glaucoma group among treatment-free patients (-10.1 ± 8.0 mmHg vs -4.1 ± 8.1 mmHg, p = 0.043), and final intraocular pressure was lower in the pseudoexfoliation glaucoma group (12.2 ± 3.5 mmHg vs 15.8 ± 5.7 mmHg, p = 0.044). CONCLUSIONS: The XEN45 Gel Stent implant demonstrated similar efficacy and safety among severe pseudoexfoliation glaucoma and non-pseudoexfoliation glaucoma patients. Greater magnitude of decreased intraocular pressure occurred among treatment-free pseudoexfoliation glaucoma patients.

Experimental glaucoma and optic nerve transection induce simultaneous upregulation of proapoptotic and prosurvival genes.

PURPOSE: To investigate changes in gene expression induced by elevated intraocular pressure (IOP) and complete optic nerve transsection (ONT) over time. METHODS: A gene array of 18 signal transduction pathways was used to examine the changes in RNA profiles of retinas post-ONT in rats. Among the seven genes that were determined to be upregulated, four were confirmed to have higher expression by semiquantitative RT-PCR analysis: Ei24 and Gadd45a (both associated with apoptosis induced via the p53 pathway), IAP-1 (inhibitor of apoptosis protein 1), and Cdk2 (cell cycle regulation and apoptosis). Their mRNA levels were then studied by quantitative RT-PCR in experimental glaucoma and ONT over time. Levels of the corresponding proteins were evaluated by Western blot analysis and immunohistochemistry. RESULTS: Proapoptotic genes from the p-53 pathway (Ei24 and Gadd45a), Cdk2 and the prosurvival gene IAP-1 (a caspase inhibitor) were simultaneously and significantly upregulated early after ONT, returning to baseline at 2 weeks. In experimental glaucoma, Gadd45a was significantly upregulated 1 week after induction of increased IOP and stayed upregulated for 2 months and long after IOP returned to baseline. The prosurvival gene IAP-1 was simultaneously upregulated but returned to baseline earlier than the proapoptotic gene. Ei24 and Cdk2 were only slightly upregulated in glaucoma. Western blot analysis demonstrated upregulation of Gadd45a and IAP-1 proteins. Immunohistochemistry localized these changes to the retinal ganglion cell layer. CONCLUSIONS: Members of the p-53 signal transduction pathway are significantly involved in glaucoma and ONT. The endogenous caspase inhibitor IAP-1 is upregulated simultaneously, possibly as part of an intrinsic neuroprotective mechanism. Changes in glaucoma are gradual and last long after IOP returns to normal.

Optical coherence tomography in anterior segment imaging.

PURPOSE: To evaluate the ability of optical coherence tomography (OCT), designed primarily to image the posterior segment, to visualize the anterior chamber angle (ACA) in patients with different angle configurations. METHODS: In a prospective observational study, the anterior segments of 26 eyes of 26 patients were imaged using the Zeiss Stratus OCT, model 3000. Imaging of the anterior segment was achieved by adjusting the focusing control on the Stratus OCT. A total of 16 patients had abnormal angle configurations including narrow or closed angles and plateau irides, and 10 had normal angle configurations as determined by prior full ophthalmic examination, including slit-lamp biomicroscopy and gonioscopy. RESULTS: In all cases, OCT provided high-resolution information regarding iris configuration. The ACA itself was clearly visualized in patients with narrow or closed angles, but not in patients with open angles. CONCLUSIONS: Stratus OCT offers a non-contact, convenient and rapid method of assessing the configuration of the anterior chamber. Despite its limitations, it may be of help during the routine clinical assessment and treatment of patients with glaucoma, particularly when gonioscopy is not possible or difficult to interpret.