Clinical outcomes of treatment with idebenone in Leber's hereditary optic neuropathy in the Netherlands:A national cohort study

Purpose The purpose of the study was to present results from a national Dutch cohort of patients with Leber's Hereditary Optic Neuropathy (LHON) treated with idebenone. Methods The multicentre, open-label, retrospective evaluation of the long-term outcome of idebenone treatment of Dutch LHON patients on visual function and on thickness of the retinal ganglion cell layer. Patients included in the analysis had a confirmed mutation in their mitochondrial DNA encoding either of the seven subunits of complex I, had a reported loss of vision in at least one eye and had a follow-up of more than 6 months after their treatment was started. Control visits involved routine clinical examinations of visual function and retinal structure at (1) the start of treatment, (2) nadir (time of lowest visual acuity), (3) the time of recovery (if any), (4) the time of termination of treatment and (5) more than 6 months after termination of the treatment. Results Data from 72 patients were analysed. Treatment duration was 23.8 +/- 14.4 (mean +/- SD) months. A positive response, that is either a clinically relevant recovery (CRR) or a clinically relevant stabilization (CRS), occurred in 53% and 11% of the patients, respectively. The magnitude of CRR was 0.41 +/- 1.54 logMAR. CRR of visual acuity is associated with recovery of colour discrimination. The thickness of both the ganglion cell complex (GCC) and the retinal nerve fibre layer (RNFL) is irreversibly reduced. Conclusion Our results confirm that idebenone may help to restore or maintain visual function. Whether this effect will persist is still unknown. Thinning of retinal neural tissue appears to be permanent

Type, severity, management and outcome of ocular and adnexal firework-related injuries: the Rotterdam experience

PURPOSE To study the type, severity, management and outcome of firework-related adnexal and ocular injuries during New Year's Eve festivities. METHODS A retrospective analysis of 123 injured patients (143 eyes) treated at the Rotterdam Eye Hospital between 2009 and 2013. All ages were included and analysed according to age, gender, active participant or bystander, laterality, location, dimension and severity of injury. Outcome parameter was the final best-corrected visual acuity. RESULTS The mean age was 22 ± 13 years with 87% males and 53% bystanders. 52% were ≤18 years. There was a higher number of female than male bystanders (63% versus 51%, p = 0.30). 50% of the eyes sustained mild, 13% moderate and 37% severe trauma. Adults suffered more from severe injuries compared to children (42% versus 31%). The most frequent intervention was gunpowder removal (20%), followed by traumatic cataract surgery (12%) and amniotic membrane grafting (8%). 76% of patients were followed over 1 year. At the end of follow-up, 88 (61.5%) eyes had recovered fully, while 55 (38.5%) eyes suffered from persistent complications with reduced vision ≤0.8 in 30% of injured eyes. 15 patients (12%, 10 adults, five children) were considered legally blind (vision ≤0.1). Three (2%) eyes were subject to evisceration. CONCLUSION Every year, around New Year's Eve 30-45 victims were referred to the Rotterdam Eye Hospital; 50% sustained moderate-to-severe trauma. In severe firework injuries, patients required multiple treatments that may not prevent permanent blindness and/or functional/cosmetic disfigurement. The majority was bystander and younger than 18 years

Corneal transplantation for Fuchs´ endothelial dystrophy: A comparison of three surgical techniques concerning 10 year graft survival and visual function.

OBJECTIVE:Comparison of conventional Penetrating Keratoplasty (PKP), posterior mushroom PKP and Descemet's Stripping Automated Endothelial Keratoplasty (DSAEK) regarding overall graft survival of primary corneal transplants for Fuchs´ endothelial dystrophy (FED), best spectacle-corrected visual acuity (BSCVA) and astigmatism. METHODS:Single centre study using prospectively collected data from the national database for follow-up of corneal transplants. Main outcome parameters: 10 years graft survival, astigmatism at 24 months, pre- and post-operative BSCVA. RESULTS:In total, 721 cases were included: PKP, n = 171; posterior mushroom PKP, n = 91; and DSAEK, n = 459. There was no significant difference in graft survival between PKP, posterior mushroom PKP and the DSAEK technique (log-rank test, P = 0.12). The overall post-operative BSCVA improvement in all treatment groups was significant (paired t-test, P<0.001). Pre-operative BSCVA was better for the DSAEK group (0.68 ± 0.41 logMAR) as compared to the PKP (0.89 ± 0.53) and posterior mushroom PKP group (0.90 ± 0.58); ANOVA, P<0.001. After 24 months, BSCVA was significantly better for the DSAEK group (0.25 ± 0.26 logMAR) compared to the PKP (0.35 ± 0.29) and posterior mushroom PKP group (0.41 ± 0.42); ANOVA, P<0.001. A significant difference in astigmatism was found (median test, P<0.001) between the DSAEK (1.7 ± 1.1 D), PKP (4.6 ± 2.7 D) and posterior mushroom PKP group (4.5 ± 3.3 D). The significantly lower DSAEK-induced astigmatism was confirmed by vector analysis. CONCLUSION:There was no difference in graft survival and BSCVA improvement between conventional PKP, inverted mushroom PKP and DSAEK in this study. The significantly lower changes in astigmatism, wound stability and faster visual rehabilitation with DSAEK surgery are favourable aspects of this technique. The benefits of posterior lamellar keratoplasty warrant earlier intervention, which may contribute to preserve better vision for a prolonged period of remaining lifetime

A prospective multicentre randomized placebo-controlled superiority trial in patients with suspected bacterial endophthalmitis after cataract surgery on the adjuvant use of intravitreal dexamethasone to intravitreal antibiotics

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A pilot study on the use of prednisolone-encapsulated liposomes for the treatment of moderate-to-severe Graves’ orbitopathy with reduced systemic steroid exposure

Purpose: To demonstrate that long-circulating PEGylated liposomal prednisolone is a safe and effective therapy in patients with active moderate-to-severe Graves’ orbitopathy. Methods: Open-label, proof-of-concept, multicentre pilot study. Ten patients with moderate-to-severe Graves’s orbitopathy, who were euthyroid for at least three months. Long-circulating PEGylated liposomal prednisolone 150 mg was administered intravenously twice, with 2-week interval. Total follow-up was 12 months, with visits at baseline, week 2, 6, 13, 26 and 52. Physical, laboratory and ophthalmological examinations were performed. Response to treatment was defined as a reduction in Clinical Activity Score by ≥2 points; palpebral aperture by ≥3 mm; soft tissue signs by ≥2 grades; exophthalmos by ≥2 mm; and motility by >8 degrees or improvement in diplopia score. A response was sustained when equally observed at weeks 6 and 13. Results: One patient achieved a sustained response according to the predetermined definition. All patients showed a decrease in Clinical Activity Score after one infusion, with a mean decrease of two points. The Clinical Activity Score was ≤1 at week 52 for all patients. Improvement was also observed in the soft tissue signs. Most of the adverse events were mild and of a transient nature. Two patients required further treatment with intravenous methylprednisolone. Conclusion: This pilot study showed a positive effect of long-circulating PEGylated liposomal prednisolone on the Clinical Activity Score in patients with moderate-to-severe Graves’s orbitopathy, resulting in fewer hospital visits and possibly less glucocorticoid-related side-effects