Mucopolysaccharidoses (MPS) are a rare group of lysosomal storage disorders characterized by the accumulation of incompletely degraded glycosaminoglycans (GAGs) in multiple organ systems including ...the eye. Visual loss occurs in MPS predominantly due to corneal clouding and retinopathy, but the sclera, trabecular meshwork and optic nerve may all be affected. Despite the success of therapies such as enzyme replacement therapy (ERT) and hematopoietic stem-cell transplantation (HSCT) in improving many of the systemic manifestations of MPS, their effect on corneal clouding is minimal. The only current definitive treatment for corneal clouding is corneal transplantation, usually in the form of a penetrating keratoplasty or a deep anterior lamellar keratoplasty. This article aims to provide an overview of corneal clouding, its current clinical and surgical management, and significant research progress.
Background
To evaluate the 6‐month efficacy and safety of the iStent microtrabecular bypass stent in patients with open‐angle glaucoma.
Design
A prospective, uncontrolled, interventional case series.
...Participants
Forty‐four eyes with open‐angle glaucoma were reported.
Methods
All subjects underwent ab interno implantation of a single iStent together with (n = 40) or without (n = 4) cataract surgery.
Main Outcome Measures
Patients were assessed at postoperative week 1, months 1 and 3, and quarterly, thereafter. Data collected included visual acuity, intraocular pressure measurement using Goldmann tonometry, number of glaucoma medications, and number and type of complications.
Results
The mean age of the sample was 76.8 years. The mean duration since glaucoma diagnosis was 5.3 years (standard deviation 2.9 years). The mean visual acuity was 0.53 logMAR at baseline that improved to 0.23 at 6 months postoperatively. The mean baseline intraocular pressure was 21.1 mmHg, and this decreased significantly to 16.7 mmHg at 6 months (P < 0.01). The mean number of drops prescribed preoperatively was 2.3, which decreased to 0.6 at 6 months (P < 0.01). Sixty‐six per cent of patients were drop‐free at 6 months. One patient developed an hyphaema following surgery; no other adverse events were recorded.
Conclusions
The iStent proved to be a safe and effective treatment for patients with open‐angle glaucoma over our 6‐month follow up period. Insertion resulted in a significant decrease in intraocular pressure as well as the number of topical antiglaucoma medications required for adequate intraocular pressure control.
Extreme corneal fragility and thinning, which have a high risk of catastrophic spontaneous rupture, are the cardinal features of brittle cornea syndrome (BCS), an autosomal-recessive generalized ...connective tissue disorder. Enucleation is frequently the only management option for this condition, resulting in blindness and psychosocial distress. Even when the cornea remains grossly intact, visual function could also be impaired by a high degree of myopia and keratoconus. Deafness is another common feature and results in combined sensory deprivation. Using autozygosity mapping, we identified mutations in PRDM5 in families with BCS. We demonstrate that regulation of expression of extracellular matrix components, particularly fibrillar collagens, by PRDM5 is a key molecular mechanism that underlies corneal fragility in BCS and controls normal corneal development and maintenance. ZNF469, encoding a zinc finger protein of hitherto undefined function, has been identified as a quantitative trait locus for central corneal thickness, and mutations in this gene have been demonstrated in Tunisian Jewish and Palestinian kindreds with BCS. We show that ZNF469 and PRDM5, two genes that when mutated cause BCS, participate in the same regulatory pathway.
Purpose
To examine outcomes through 36 months in phakic eyes with newly diagnosed primary open-angle glaucoma (POAG) naïve to therapy randomized to treatment with two trabecular micro-bypass stents ...or topical prostaglandin.
Methods
Subjects with POAG naïve to therapy, with intraocular pressure (IOP) ≥21 and ≤40 mmHg, were randomized to implantation of two stents or travoprost. Additional medication was to be prescribed post-treatment for elevated IOP or glaucomatous optic nerve findings. Of 101 randomized subjects, 100 subjects were followed for 24 months and 73 subjects were followed for 36 months. Follow-up on all subjects is ongoing.
Results
In this randomized cohort of 101 POAG subjects, 54 subjects underwent 2-stent surgery and 47 received topical travoprost. Mean pre-treatment IOP was 25.5 ± 2.5 mmHg in stent-treated eyes and 25.1 ± 4.6 mmHg in medication-treated eyes. By 3 years, mean IOP was 14.6 mmHg in stent eyes (with medication added in 6 eyes) and 15.3 mmHg in travoprost eyes (with a second medication added in 11 eyes). In the subset of eyes that did not require additional medical therapy, mean IOP was 14.5 mmHg and 15.7 mmHg in the respective groups. Ninety-one percent of stent eyes had 3-year IOP ≤18 mmHg without additional therapy (62% ≤ 15 mmHg) and 79% of travoprost eyes had 3-year IOP ≤18 mmHg (21% ≤ 15 mmHg). Safety was favorable in both groups.
Conclusions
In this prospective, randomized comparison of subjects with newly diagnosed POAG naïve to therapy, substantial IOP reduction with a favorable low complication rate was shown through 3 years after either 2 trabecular stents implanted as the sole procedure or topical travoprost therapy. These data suggest 2-stent implantation may be a viable initial treatment option comparable to topical prostaglandin in newly diagnosed POAG patients.
Trial registration: ClinicalTrials.gov identifier, NCT01443988.
Funding
Glaukos Corporation, Laguna Hills, CA.
Glaucoma Quality of Life Fea, Antonio Maria; Hengerer, Fritz; Lavia, Carlo ...
Journal of ophthalmology,
01/2017, Letnik:
2017
Journal Article
Recenzirano
Odprti dostop
Furthermore, knowing the degree of disability can potentially help to increase patient safety with appropriate guidelines, to recognize patients who can benefit from rehabilitation and evaluate the ...efficacy of those measures. Since the first studies on quality of life in glaucoma, several areas of interest have been identified and explored: (1) determining the symptoms that are more bothersome for the patients and correlating them with the stage of disease; (2) testing and building better methods to investigate the impact of glaucoma on the QoL; (3) assessing the impact of glaucoma on mental status; and (4) analyzing the impact of different therapies on the QoL of glaucoma patients. (1) Thanks to recent investigations, we are now aware that vision defects in glaucoma patients are not as simple as the traditional view of peripheral vision loss, but they affect several aspects related to a generally decreased image quality including glare, letters appearing faded when reading, and needing more light. (3) Glaucoma doctors always had the clinical impression that some psychological traits are typical of the glaucoma patients, but several papers demonstrated that glaucoma is a significant predictor of depression after adjustment for demographic factors and multiple comorbidities with a prevalence estimated around 10%.
ObjectivesTo determine the efficacy and safety of combined phacoemulsification and single first-generation iStent implantation over 84 months.Subjects/MethodsSingle-surgeon, single-centre, ...uncontrolled prospective interventional study in real-world settings. Forty-one patients with open-angle glaucoma on at least one antihypertensive drop underwent phaco-iStent surgery. This cohort was monitored over the subsequent 84 months. The primary outcome measure was intraocular pressure (IOP). Secondary outcome measures were number of glaucoma drops, visual acuity, cup-disc-ratio (CDR), mean deviation (MD) and visual field index (VFI). Thirty-one (76%) patients survived to 48 months and 19 (46%) patients to 84 months.ResultsAt 84 months, we demonstrate an absolute mean reduction of 4.87 mmHg (95% CI 1.62–7.64) for IOP and 0.59 (95% CI 0.03–1.16) for number of glaucoma drops. At the final clinic visit, LogMAR VA improved by 0.21 (95% CI 0.08–0.34), with no significant change in CDR, MD and VFI. Attrition was mainly due to death (27%) and further glaucoma surgery (12%). A single patient suffered from postoperative hyphaema, with no sequalae. Patients at high risk for progression to filtration surgery were defined as those on oral acetazolamide for IOP control, or those fulfilling all the following criteria: IOP ≥ 20 mmHg, CDR ≥ 0.7, MD ≤ −4.0 dB, number of drops ≥ 2. This group is to be considered for surgery as the next step in management according to NICE glaucoma guidelines. These patients (n = 14) demonstrated a more marked final IOP reduction of 6.85 mmHg (95% CI 3.97–9.75) vs 1.62 mmHg (95% CI 0.04–3.22) in their low-risk counterparts (n = 27). Reduction in glaucoma drops was 0.86 (95% CI 0.07–1.64) for the high-risk and 0.56 (95% CI 0.06–1.05) for the low-risk cohort. Further glaucoma surgery was required for 4 (29%) high-risk and 1 (0.5%) low-risk patients over the study period.ConclusionsOur results strongly suggest that the expected outcome of phaco-iStent surgery is a maintained reduction in intraocular pressure and number of glaucoma medications over 7 years. This is combined with a negligible rate of complications, a sustained improvement in central visual acuity, and a reliable maintenance of peripheral visual function.
Purpose
The Xen® Gel Stent (Allergan, Irvine, CA, USA) is a minimally invasive glaucoma surgery device implanted to reduce intra-ocular pressure (IOP) in glaucoma. The stents can fail to drain ...post-operatively due to scarring of the conjunctiva around the stent opening. Data on the success rates of revision surgery in patients with Xen stent failure are scarce. We present the first detailed report of the steps, outcomes and complications of Xen revision surgery with 12 months of follow-up data.
Methods
We share our experiences on the circumstances in which to perform Xen revision surgery, the steps of the surgery and the results of a retrospective interventional case series of all Xen revisions performed at a single centre from 2013 to 2018.
Results
A total of 151 Xen implants were inserted into eyes at our tertiary centre during the study period, of which 21 eyes (patients) underwent revision surgery. Mean pre-operative IOP was 26.1 (standard deviation 8.3) mmHg with the patient on two drops of anti-glaucoma medication. Four patients were excluded from the analysis due to incomplete data (medical records were unavailable for 2 patients; 1 patient died shortly after surgery; and 1 patient moved to another area). A non-functioning Xen implant was identified in another patient during revision surgery, and the procedure was converted to a trabeculectomy. The remaining 16 patients were included in the analysis, all of whom had a minimum follow-up of 12 months, with the longest follow-up being 4 years following revision surgery. Of these 16 patients, four required needling and 5-fluorouracil injection in the first 12 months following revision surgery, three required further glaucoma drainage surgery due to the failure of the revision surgery to control IOP in the first year and one suffered bleb-related endophthalmitis at the site of previous trabeculectomy surgery. Mean IOP at 12 months following revision surgery was 16.3 (standard deviation 3.7) mmHg on 0.7 drops of anti-glaucoma medication, which equates to a 37.5% reduction in IOP and a 65% reduction in the amount of IOP-lowering drops required.
Conclusion
Our study shares experience on when to perform Xen revision surgery and the steps required. The results of our small cohort are the first in the literature and show that revisions can achieve promising IOP and medication reductions. Some patients still require needling in the post-operative period to optimise outcomes.
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