Surgical treatment of myopic strabismus fixus: a graded approach

Background: Surgical treatment of myopic strabismus fixus is challenging. Options for its correction range from conventional combined recession-resection surgery to innovative surgical procedures aiming to correct the deviated muscle paths. In this report we review our experience and compare the results of various surgical options for treatment of strabismus fixus. Methods: We report the surgical outcomes of nine adults with acquired strabismus fixus due to myopia with a follow-up of 1year. Patients were enrolled between May 2003 and April 2007 in this retrospective study. The surgical procedure was determined depending on the angle of deviation and extent of motility impairment. A new transposition technique was performed in one patient who had an extreme variant of strabismus fixus. Results: Combined recession-resection surgery was performed in four patients with resulting small-angle esotropia. In patients with both esotropia and hypotropia due to muscle alignment, we performed an additional upward displacement of both horizontal recti muscles combined with a myopexy of the lateral rectus muscle. The results were satisfying; in particular in one patient who had a transposition procedure a significant improvement was achieved. Conclusions: For treatment of myopic strabismus fixus, a graded approach seems advisable. Combined recession-resection surgery yields good results for smaller deviations with mildly impaired motility, additional fixation techniques need to be applied once the horizontal muscle paths are deviated, and in extreme cases, a transposition procedure is require

Isolated oculomotor nerve palsy related to sinusitis?

The association of sinusitis with ocular motility disorders is a seductive theory due to their close anatomical vicinity. Typically, sinusitis can influence ocular motility by affecting single muscles or a combination of muscles and/or cranial nerves due to a local inflammatory tissue reaction. Although rare, sinusitis has been suggested at least as a cause for superior-branch oculomotor palsy. We report a case of progressive, isolated, third-cranial-nerve palsy caused by an aneurysm of the posterior-communicating artery that initially was thought to be related to pansinusiti

Inter-observer agreement for spectral- and time-domain optical coherence tomography image grading: a prospective study

The purpose of this study was to compare inter-observer agreement of Stratus™ OCT versus Spectralis™ OCT image grading in patients with neovascular age-related macular degeneration (AMD). Thirty eyes with neovascular AMD were examined with Stratus™ OCT and Spectralis™ OCT. Four different scan protocols were used for imaging. Three observers graded the images for the presence of various pathologies. Inter-observer agreement between OCT models was assessed by calculating intra-class correlation coefficients (ICC). In Stratus™ OCT highest interobserver agreement was found for subretinal fluid (ICC: 0.79), and in Spectralis™ OCT for intraretinal cysts (IRC) (ICC: 0.93). Spectralis™ OCT showed superior interobserver agreement for IRC and epiretinal membranes (ERM) (ICCStratus™: for IRC 0.61; for ERM 0.56; ICCSpectralis™: for IRC 0.93; for ERM 0.84). Increased image resolution of Spectralis™ OCT did improve the inter-observer agreement for grading intraretinal cysts and epiretinal membranes but not for other retinal change

Repeatability of nerve fiber layer thickness measurements in patients with glaucoma and without glaucoma using spectral-domain and time-domain OCT

Background: The aim of this work is to assess the repeatability of spectral-domain-OCT (SD-OCT) retinal nerve fiber layer thickness (RNFL) thickness measurements in a non-glaucoma group and patients with glaucoma and to compare these results to conventional time-domain-OCT (TD-OCT). Methods: In a prospective, comparative, observational case-control study, 50 eyes of 25 non-glaucoma and 22 eyes of 11 patients with primary open angle glaucoma (POAG) were included. SD-OCT and TD-OCT circle scans were centered on the optic disc. In each eye, OCT scans were performed three times by two independent observers. RNFL thickness was measured in four quadrants around the optic disc. In addition, the overall mean RNFL thickness was assessed. Intraclass correlation coefficients (ICC) and coefficients of variation (COV) were calculated. Inter-observer and inter-OCT repeatability was visualized by using Bland-Altman analysis. Results: Intra-observer repeatability for TD- OCT was good with an ICCmean RNFL thickness of 0.939 in non-glaucomas and 0.980 in glaucomatous eyes. For SD-OCT, intra-observer repeatability was higher with an ICC of 0.989 for non-glaucomas and 0.997 for glaucomatous eyes. COVs for TD-OCT ranged from 2.9-7.7% in non-glaucomas and from 6.0-13.3% in glaucoma patients. COVs for SD-OCT ranged from 0.3-1% in non-glaucomas and from 0.9-2.3% in glaucomatous eyes. COVs were influenced by various factors. In the glaucoma group, COVs were significantly higher (p < 0.001) compared to the non-glaucoma group. COVs increased by a mean of 5.1% when TD-OCT was used instead of SD-OCT (p < 0.001). Conclusions: SD-OCT RNFL thickness measurements in healthy volunteers and glaucoma patients showed good intra- and inter-observer repeatability. Especially in glaucomatous eyes, repeatability of SD-OCT was superior to TD-OC

Reproducibility of Nerve Fiber Layer Thickness Measurements Using 3D Fourier-Domain OCT

PURPOSE. Conventional time-domain optical coherence tomography (OCT) has been shown to provide reproducible retinal nerve fiber layer (RNFL) measurements. Recently, high-speed, high-resolution Fourier-domain 3D-OCT has been introduced to improve OCT quality. It can provide 6-mm 2 high-density scans to provide RNFL thickness measurements. The purpose of this study was to test the reproducibility of 3D-OCT RNFL thickness measurements in healthy volunteers. METHODS. Thirty-eight eyes were included in the study. Highdensity 6-mm 2 3D scans were registered by two independent operators. RNFL thickness was calculated for eight areas corresponding to the ETDRS areas and for two ring areas. The ETDRS grid was centered on the optic disc. Intraclass correlation coefficients (ICC) and coefficients of variation (COV) were calculated. Interobserver reproducibility was visualized by using Bland-Altman analysis. RESULTS. Intrasession reproducibility was good with a mean ICC of 0.90. The mean COV for operator 1 and 2 was 4.2% and 4%, respectively (range, 1.9%-6.7%). Highest reproducibility was found for the two ring areas and the superior and inferior quadrants. Mean differences in RNFL thickness measurements for ring 1 and 2 between operator 1 and 2 were 0.9 m (limits of agreement, Ϫ11.4 to ϩ9.6 m) and 0.1 m (limits of agreement Ϫ4.1 to ϩ3.9 m), respectively. CONCLUSIONS. 3D-OCT RNFL thickness measurements in healthy volunteers showed good intra-and interobserver reproducibility. 3D-OCT provides more RNFL thickness information compared to conventional time-domain OCT measurements and may be useful for the management of glaucoma and other optic neuropathies. (Invest Ophthalmol Vis Sci. 2008;49:5386 -5391) DOI:10.1167/iovs.07-1435 E valuation of the retinal nerve fiber layer (RNFL) is fundamental for diagnosing and managing glaucoma and other optic neuropathies. In the past, RNFL could be assessed subjectively only by slit lamp examination. This method requires clinical experience and offers only qualitative data. In addition, comparisons over time are almost impossible. Successively, other techniques such as color photographs of the optic disc or red-free photographs of the RNFL have become available and have facilitated comparisons over time. Scanning laser ophthalmoscopy and scanning laser polarimetry were the first instruments to allow objective and quantitative evaluation of the RNFL and the optic disc. Studies have been conducted to investigate the reproducibility of OCT RNFL thickness measurements, to assess the value of OCT as a clinical tool for distinguishing between healthy and glaucomatous eyes. Recently, improvements in OCT technology have been introduced. 10 -11 Fourier-domain (FD)-OCT provides increased resolution and scanning speed by recording the interferometric information with a Fourier-domain spectrometric method instead of adjusting the position of a reference mirror. Resolution is up to five times higher, and imaging speed is 60 times faster than in conventional time-domain OCT. 12-13 In addition to high image quality, it is important to have reliable and reproducible software programs to analyze the data acquired by FD-OCT. Previous versions of OCT (Stratus OCT3; Carl Zeiss Meditec, GmbH, Oberkochen, Germany) mostly used a 3.4-mm diameter circle scan centered on the optic disc to generate 512 A-scans. The RNFL thickness profile showed a characteristic curve with two peaks, one in the superior and one in the inferior quadrant. FD-OCT can perform a high-density raster-scan (512 ϫ 128 axial B-scans in a 6 mm 2 area). Recently, the peripapillary nerve fiber layer thickness profile was determined with FD-OCT by using high-density scanning. 14 These raster scans provide considerably more data for RNFL thickness analysis. The purpose of this study was to test the reproducibility of RNFL thickness measurements in healthy subjects by using FD-OCT high-density raster scans (3D OCT1000; Topcon, Tokyo, Japan). METHODS Thirty-eight eyes of 19 healthy subjects (10 women) with a mean age of 26 Ϯ 3 years were included in the study. Exclusion criteria were history of glaucoma, history of any other ocular disease, intraocular pressure greater than 21 mm Hg, or a refractive error of more than Ϫ5 or ϩ5 D. FD-OCT high-density scans were performed with the 3D OCT1000 system. The 3D OCT1000 is an FD-OCT device providing OCT images up to 50 times faster than time-domain OCTs with a sweep-scan technique. The device has a field angle of 45°with a color fundus camera included. The scanning range of the device is from 3 to 6 mm 2 . Horizontal resolution is Յ20 m and depth resolution is up to 5 m. As a light source, the system uses superluminescent diodes with a wavelength of 840 nm. Pupil diameter had to be at least 4 mm for scanning. High-density raster scans (512 ϫ 128 B-scans in a 6 mm 2 area) were centered on the From th

Characterization of two novel intronic OPA1 mutations resulting in aberrant pre-mRNA splicing.

BACKGROUND We report two novel splice region mutations in OPA1 in two unrelated families presenting with autosomal-dominant optic atrophy type 1 (ADOA1) (ADOA or Kjer type optic atrophy). Mutations in OPA1 encoding a mitochondrial inner membrane protein are a major cause of ADOA. METHODS We analyzed two unrelated families including four affected individuals clinically suspicious of ADOA. Standard ocular examinations were performed in affected individuals of both families. All coding exons, as well as exon-intron boundaries of the OPA1 gene were sequenced. In addition, multiplex ligation-dependent probe amplification (MLPA) was performed to uncover copy number variations in OPA1. mRNA processing was monitored using RT-PCR and subsequent cDNA analysis. RESULTS We report two novel splice region mutations in OPA1 in two unrelated individuals and their affected relatives, which were previously not described in the literature. In one family the heterozygous insertion and deletion c.[611-37_611-38insACTGGAGAATGTAAAGGGCTTT;611-6_611-16delCATATTTATCT] was found in all investigated family members leading to the activation of an intronic cryptic splice site. In the second family sequencing of OPA1 disclosed a de novo heterozygous deletion c.2012+4_2012+7delAGTA resulting in exon 18 and 19 skipping, which was not detected in healthy family members. CONCLUSION We identified two novel intronic mutations in OPA1 affecting the correct OPA1 pre-mRNA splicing, which was confirmed by OPA1 cDNA analysis. This study shows the importance of transcript analysis to determine the consequences of unclear intronic mutations in OPA1 in proximity to the intron-exon boundaries

Central Retinal Artery Occlusion: Current Practice, Awareness and Prehospital Delays in Switzerland

BACKGROUND AND PURPOSE: Central retinal artery occlusion (CRAO) often leads to permanent monocular blindness. Hence, early recognition and rapid re-perfusion is of paramount importance. This study aims to describe prehospital pathways in CRAO compared to stroke and study the knowledge about CRAO. METHODS: (1) Description of baseline characteristics, prehospital pathways/delays, and acute treatment (thrombolysis/thrombectomy vs. standard of care) of patients with CRAO and ischemic stroke registered in the Swiss Stroke Registry. (2) Online survey about CRAO knowledge amongst population, general practitioners (GPs) and ophthalmologists in Eastern Switzerland. RESULTS: Three hundred and ninety seven CRAO and 32,816 ischemic stroke cases were registered from 2014 until 2019 in 20 Stroke Centers/Units in Switzerland. In CRAO, 25.6% arrived at the hospital within 4 h of symptom onset and had a lower rate of emergency referrals. Hence, the symptom-to-door time was significantly longer in CRAO compared to stroke (852 min. vs. 300 min). The thrombolysis/thrombectomy rate was 13.2% in CRAO and 30.9% in stroke. 28.6% of the surveyed population recognized CRAO-symptoms, 55.4% of which would present directly to the emergency department in contrast to 90.0% with stroke symptoms. Almost 100% of the ophthalmologist and general practitioners recognized CRAO as a medical emergency and 1/3 of them considered IV thrombolysis a potentially beneficial therapy. CONCLUSIONS: CRAO awareness of the general population and physician awareness about the treatment options as well as the non-standardized prehospital organization, seems to be the main reason for the prehospital delays and impedes treating CRAO patients. Educational efforts should be undertaken to improve awareness about CRAO

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