Further characterization of ARSK-related mucopolysaccharidosis type 10.

Mucopolysaccharidosis type 10 is caused by biallelic variants in ARSK, which encodes for a lysosomal sulfatase. To date, seven patients with a mild phenotype resembling spondyloepiphyseal dysplasia or multiple epiphyseal dysplasia have been described. In this report, we present two novel ARSK variants and report clinical and radiological findings of three patients. The patients' initial complaints were hip or knee pain and a waddling gait. All patients showed normal intelligence, normal hearing and eye examinations, and none had organomegaly. While typical dysostosis multiplex findings were not observed, mild platyspondyly with anterior beaking of some vertebral bodies, irregular vertebral endplates, wide ribs, inferior tapering of the ilea with a poorly developed acetabulum, irregularity of the central part of the femoral head, delayed ossification of the carpals were noted. Remarkably, all patients showed metaphyseal striation of the long bones, a crucial diagnostic clue to identify ARSK-related MPS type 10. Interestingly, vertebral involvement regressed during follow-up. On the other hand, hip dysplasia progressed in all patients. In conclusion, this study provides valuable long-term results on a recently discovered form of MPS.

Cataract Classification Systems: A Review.

Cataract is among the leading causes of visual impairment worldwide. Innovations in treatment have drastically improved patient outcomes, but to be properly implemented, it is necessary to have the right diagnostic tools. This review explores the cataract grading systems developed by researchers in recent decades and provides insight into both merits and limitations. To this day, the gold standard for cataract classification is the Lens Opacity Classification System III. Different cataract features are graded according to standard photographs during slit lamp examination. Although widely used in research, its clinical application is rare, and it is limited by its subjective nature. Meanwhile, recent advancements in imaging technology, notably Scheimpflug imaging and optical coherence tomography, have opened the possibility of objective assessment of lens structure. With the use of automatic lens anatomy detection software, researchers demonstrated a good correlation to functional and surgical metrics such as visual acuity, phacoemulsification energy, and surgical time. The development of deep learning networks has further increased the capability of these grading systems by improving interpretability and increasing robustness when applied to norm-deviating cases. These classification systems, which can be used for both screening and preoperative diagnostics, are of value for targeted prospective studies, but still require implementation and validation in everyday clinical practice.

Capsule Dynamics, Implantation, and Explantation of the Smaller Incision, New-Generation Implantable Miniature Telescope: a Miyake-Apple Study.

PURPOSE: The smaller incision, new-generation implantable miniature telescope is a novel implant to optimize vision in retinal patients with central vision loss. Using Miyake-Apple techniques we visualized the device implantation, repositioning, and explantation, while noting capsular bag dynamics. METHODS: Using the Miyake-Apple technique, we assessed capsular bag deformation after successful implantation of the device in human autopsy eyes. We assessed rescue strategies for converting a sulcus implantation to a capsular implantation and explantation strategies. We noted the occurrence of posterior capsule striae, zonular stress, and the haptics' arc of contact with the capsular bag after implantation. RESULTS: Acceptable zonular stress was observed during the successful implantation of the SING IMT. When it was implanted in the sulcus, one could reposition the haptics into the bag with two spatulas using counter-pressure in an effective strategy despite inducing tolerable, medium zonular stress. A similar technique, in reverse, allows safe explantation without damaging the rhexis or the bag, while inducing similar medium, tolerable zonular stress. In all eyes we examined, the implant considerably stretches the bag, inducing a capsular bag deformation and posterior capsule striae. CONCLUSION: The SING IMT can be safely implanted without significant zonular stress. In sulcus implantation and explantation, repositioning of the haptic is achievable without perturbing zonular stress using the presented approaches. It stretches average-sized capsular bags to support its weight. This is achieved by an increased arc of contact of the haptics with the capsular equator.

Reversible Multifocality Achieved Through Polypseudophakia. / Reversible Multifokalität durch Polypseudophakie.

Simultaneous implantation of a monofocal or monofocal toric intraocular lens (IOL) into the capsular bag and a multifocal IOL into the ciliary sulcus, referred to as duet procedure, allows us to create multifocality that is more easily reversible than the implantation of a capsular bag-fixated multifocal IOL. The optical quality and results after the duet procedure are equivalent to those of a capsular bag-fixated multifocal IOL. Patients who cannot tolerate the side effects of multifocal optics or who develop an ocular condition leading to loss of function such as age-related macular degeneration (AMD) or glaucoma in the course of their lives may benefit from the reversibility of the procedure.

Biomechanical Analysis of Tomographically Regular Keratoconus Fellow Eyes Using Corvis ST. / Biomechanische Analyse von tomografisch unauffälligen Keratokonus-Partneraugen mit dem Corvis ST.

BACKGROUND: Keratoconus is a bilateral, yet asymmetric disease. In rare cases, the second eye may show no signs of tomographic changes. The purpose of this study was to analyze the biomechanical characteristics in tomographically regular keratoconus fellow eyes. MATERIALS AND METHODS: This retrospective, consecutive case series analyzed 916 eyes of 458 patients who presented to our keratoconus clinic between November 2020 and October 2022. Primary outcome measures included best-corrected visual acuity (BCVA), tomographic Scheimpflug analysis using Pentacam AXL (Oculus, Wetzlar, Germany), and biomechanical assessment using Corvis ST (Oculus, Wetzlar, Germany). Tomographic changes were assessed via analysis of the anterior and posterior curvature, K-max, thinnest corneal thickness (TCT), the Belin/Ambrosio Deviation Display (BAD-D), and the ABCD-Grading. Biomechanical changes were analyzed using Corvis Biomechanical Index (CBI) and Tomographic Biomechanical Index (TBI). RESULTS: Of 916 eyes, 34 tomographically regular fellow eyes (7.4%) were identified and included in the analysis. Overall, the mean BCVA was - 0.02 ± 0.13 logMAR. Tomographic analysis showed mean K-max of 43.87 ± 1.21 D, mean TCT of 532 ± 23 µm, and mean BAD-D of 1.02 ± 0.43. Biomechanical analysis demonstrated mean CBI of 0.28 ± 0.26 and mean TBI of 0.34 ± 0.30. While normal CBI-values were observed in 16 (47%) of 34 eyes, only 13 eyes (38%) showed a regular TBI and only 7 eyes (21%) showed regular TBI and CBI. The sensitivity of CBI and TBI to detect a tomographically normal keratoconus fellow eye was 53% and 62%, respectively. CONCLUSION: A highly asymmetric corneal ectasia with regular tomographic finding in a fellow eye is rare among keratoconus patients. In such cases, a biomechanical analysis may be useful in detecting early signs of corneal ectasia. In our analysis, the TBI showed high sensitivity for detecting a biomechanical abnormality in tomographically regular fellow eyes.

Differential Diagnosis of Changes in Intraocular Lenses. / Differenzialdiagnostik von Veränderungen in Intraokularlinsen.

Differentiating between various intraocular lens (IOL) changes can be a challenge. In particular, certain IOL models carry the risk of late postoperative calcification. A major cause of IOL exchange surgery could be avoided if appropriate modifications were made during the IOL manufacturing process. The use of a hydrophilic acrylate carries the risk of IOL calcification, especially when a secondary procedure, such as a pars plana vitrectomy or other procedures using gas or air, is performed. In secondary IOL calcification, there is a wide range of opacification patterns, which are usually located in the centre on the anterior surface of the IOL or sometimes elsewhere. Often, granular deposits accumulate just below or on the surface of the IOL, leading to significant deterioration in visual quality and eventually requiring IOL exchange surgery. Therefore, in the case of eyes requiring secondary surgical intraocular intervention in the future, the use of hydrophilic IOLs should be critically evaluated. With regard to hydrophobic IOL materials, there are clear differences in the susceptibility to the formation of glistenings. Over time, there has been a significant decrease in glistening formation over the past 30 years due to optimisation of the material. With hydrophobic IOLs, special care should also be taken to avoid mechanical damage. In general, the only treatment option for functionally-impairing IOL opacification is surgical lens exchange, which carries potential risks of complications. In cases with a low degree of functional impairment, and especially in eyes with additional ocular diseases, it may be difficult to weigh the risk of additional surgery against the potential benefit. In some cases, it may be more appropriate not to perform an IOL exchange despite the IOL opacification. Recent visualisation methods that allow high-resolution analysis of the opacities in vivo and in vitro may be used in the future to estimate the functional effects of various IOL material changes on the optical quality.

The Genomics of Arthrogryposis, a Complex Trait: Candidate Genes and Further Evidence for Oligogenic Inheritance.

Arthrogryposis is a clinical finding that is present either as a feature of a neuromuscular condition or as part of a systemic disease in over 400 Mendelian conditions. The underlying molecular etiology remains largely unknown because of genetic and phenotypic heterogeneity. We applied exome sequencing (ES) in a cohort of 89 families with the clinical sign of arthrogryposis. Additional molecular techniques including array comparative genomic hybridization (aCGH) and Droplet Digital PCR (ddPCR) were performed on individuals who were found to have pathogenic copy number variants (CNVs) and mosaicism, respectively. A molecular diagnosis was established in 65.2% (58/89) of families. Eleven out of 58 families (19.0%) showed evidence for potential involvement of pathogenic variation at more than one locus, probably driven by absence of heterozygosity (AOH) burden due to identity-by-descent (IBD). RYR3, MYOM2, ERGIC1, SPTBN4, and ABCA7 represent genes, identified in two or more families, for which mutations are probably causative for arthrogryposis. We also provide evidence for the involvement of CNVs in the etiology of arthrogryposis and for the idea that both mono-allelic and bi-allelic variants in the same gene cause either similar or distinct syndromes. We were able to identify the molecular etiology in nine out of 20 families who underwent reanalysis. In summary, our data from family-based ES further delineate the molecular etiology of arthrogryposis, yielded several candidate disease-associated genes, and provide evidence for mutational burden in a biological pathway or network. Our study also highlights the importance of reanalysis of individuals with unsolved diagnoses in conjunction with sequencing extended family members.

A review of late intraocular lens opacifications.

PURPOSE OF REVIEW: Phacoemulsification cataract surgery is one of the most commonly performed surgical procedure worldwide. In the majority of cases, intraocular lenses (IOLs) are implanted. Due to the increasing life expectancy and the fact that cataract surgery is performed in earlier stages, the anticipated IOL duration in the eye has increased over the last decades. The aim of this study was to review the types and describe the characteristics of late intraocular lens opacifications. RECENT FINDINGS: Calcification was the most commonly reported type of opacification in hydrophilic IOLs; it usually negatively impacted the visual function and required IOL explantation. Glistening manifested in hydrophobic acrylic lenses and was frequent in some IOL models. In most cases glistening and subsurface nanoglistenigs do not lead to a decline in visual acuity or require IOL exchange. Current studies indicate that fluid-related phenomena may induce straylight, leading to a decrease of comfort and quality of vision. SUMMARY: Several reports on late IOL opacifications have been published in recent years. In some cases, particularly in glistening, the development of the opacifications might be related to IOL aging. The influence of the fluid-related microvacuoles on the quality of vision requires further research.

Laboratory analysis and ray visualization of diffractive optics with enhanced intermediate vision.

BACKGROUND: To assess the optical behavior of a new diffractive intraocular lens (IOL) and compare its performance to that of an established extended-depth-of-focus (EDOF) IOL. METHODS: This study assessed the Proming EDOF Multifocal AM2UX [Eyebright Medical Technology (Beijing) Co., Ltd., China] and the AT LARA 829MP [Carl Zeiss Meditec, Germany]. An experimental set-up with 0.01% fluorescein solution and monochromatic light (532 nm) was used to visualize the IOLs' ray propagation. In addition, the optical quality of the IOLs was assessed by measuring the modulation transfer function (MTF) values at 50lp/mm and 3.0 and 4.5 mm apertures on the optical bench OptiSpheric® IOL PRO II [Trioptics GmbH, Germany]. RESULTS: The ray propagation of the two IOLs showed two distinct foci. Light intensity assessment revealed that both IOLs allocate more energy to primary than secondary focus. At 3.0 mm pupil, the MTF values at 50lp/mm for the primary focus were 0.39 and 0.37, and for the secondary focus, 0.29 and 0.26 for the AT LARA and Proming IOLs, respectively. At 4.5 mm pupil, the single-frequency MTF for the primary focus was 0.51 and 0.24 and for the secondary focus 0.21 and 0.15 for the AT LARA and Proming IOLs, respectively. CONCLUSIONS: When tested with an aberration-free model cornea under monochromatic conditions, the Proming behaved as a low-add bifocal lens; however, its properties did not differ much from the well-established AT LARA EDOF IOL. The AT LARA outperformed the Proming at low defocus (up to 2D), while the latter demonstrated better image quality in the 2-3D range.

Reasons for explantation of phakic intraocular lenses and associated perioperative complications: cross-sectional explant registry analysis.

BACKGROUND: We discuss the safety, since their introduction, of phakic intraocular lenses (pIOLs) to correct refractive errors in healthy eyes. We investigated the reasons for pIOL explantation and the associated perioperative complications. METHODS: This retrospective, cross-sectional study included 69 pIOLs, explanted at a single tertiary center between July 2005 and March 2020: 34 angle-supported (G1), 28 iris-fixated (G2) and seven posterior chamber (G3) pIOLs. Case data including the reason for explantation was taken from the patient records. Intra- and postoperative complications were evaluated for an association with the pIOL. RESULTS: The mean duration in the eye was 10.4 (0.2-28) years. Cataractogenesis and subsequent surgery that required pIOL explantation was the reason in 42% of all cases. In 22%, cataract in combination with endothelial damage prompted explantation, with 26, 18 and 14% for G1, G2 and G3 respectively. The second most common reasons were corneal damage alone in the angle-supported group (26%), IOL subluxation in the iris-fixated group (18%), and photopic disturbance in the posterior chamber group (29%). In 68% of all explantations, the surgical course was unremarkable, while in the remaining cases perioperative complications were associated with the lens in 45.7%. CONCLUSION: Overall, the need for cataract surgery was the most common reason for pIOL explantation. Corneal complications were more frequent in the angle-supported pIOLs and their removal was associated with higher rates of complication compared to the other groups.

Evaluation of Dislocation Risk Factors With Total Hip Arthroplasty in Developmental Hip Dysplasia Patients: A Multivariate Analysis.

BACKGROUND: This study aimed to investigate the risk factors for dislocation in patients diagnosed with developmental dysplasia of the hip (DDH) who underwent total hip arthroplasty. METHODS: We retrospectively reviewed 40 patients who developed dislocation and compared them with 400 patients in the control group without hip instability. Patients-, surgery-, and implant-related factors were investigated. Risk factors were evaluated using multivariate logistic regression. RESULTS: The mean follow-up period was 32.3 months. The mean time to dislocation was 4.4 months. There were 7 men (17.5%) and 33 women (82.5%) in the dislocation group and 83 men (20.7%) and 317 women (79.3%) in the control group (P = .627). Diabetes mellitus (DM; P = .032) and history of previous hip surgery for DDH were associated with dislocation (P < .001). The subtrochanteric shortening osteotomy (P = .001), acetabular inclination (P = .037), acetabular anteversion (P < .001), femoral head size (P < .001), and postoperative infection (P = .003) were associated with dislocation. Major predictors of hip dislocation after total hip arthroplasty in patients with DDH were previous hip surgery (odds ratio [OR], 6.76; 95% confidence interval [CI], 1.86-24.6; P = .004), high hip center (OR, 2.90; 95% CI, 1.31-6.38; P = .008), DM (OR, 2.68; 95% CI, 1.06-6.80; P = .037), and acetabular inclination (OR, 2.62; 95% CI, 1.09-6.26; P = .03). CONCLUSION: Patients with DM and previous hip surgery should be informed about increased dislocation rates. Using a larger head diameter and restoration of the true hip rotation center are essential to prevent hip dislocation in these patients. Furthermore, accurate positioning of the acetabular inclination and anteversion are also important.

Glistening formation in a new hydrophobic acrylic intraocular lens.

BACKGROUND: The formation of fluid-filled microvacuoles, termed glistenings, is a common complication of intraocular lenses (IOLs) made from hydrophobic acrylate. Using our well-established in-vitro laboratory method, we evaluated a new IOL material's resistance to glistening formation. METHODS: An in-vitro stress test for glistening induction was performed on 20 samples of hydrophobic acrylic IOLs: ten of the new Eyecryl ASHFY600 (Biotech Vision Care, Ahmedabad, India) compared with ten samples of AcrySof IQ SN60WF (Alcon, Fort Worth, USA). The number of microvacuoles per square millimetre (MV/mm2) was evaluated in five sections of each IOL. The results for each model were compared and rated on a modified Miyata Scale for grading glistening severity. RESULTS: In all cases, glistening number was higher in the central section of the IOL optic than in the periphery. Mean number of MV/mm2 was highest in the central part of the AcrySof IQ SN60WF, with 41.84 (±27.67) MVs/mm2. The lowest number of glistenings was found in the five sections of the Eyecryl ASHFY600 with 0.52 (±0.24) MVs/mm2. Mean value of the Eyecryl ASHFY600 IOL, using the Miyata Scale, was Zero. CONCLUSION: In this in-vitro laboratory study, the new hydrophobic acrylic IOL showed a high resistance to microvacuole formation. Results from this in-vitro study suggest that glistening numbers will be low in clinical use in the Eyecryl ASHFY600.

Retinal nerve fibre and ganglion cell inner plexiform layer analysis by optical coherence tomography in asymptomatic empty sella patients.

Purpose: To investigate the clinical importance of the thicknesses of the retinal nerve fibre (RNFL) and ganglion cell and inner plexiform layer (GCL+) by spectral domain optic coherence tomography (SD-OCT) in asymptomatic empty sella (ES) patients.Materials and methods: In this cross-sectional, non-randomized prospective study, 44 ES patients and 74 age- and sex-matched healthy individuals were evaluated. All the patients and controls competed an automated 30-2 visual field (VF) test. The mean deviation (MD), pattern standard deviation (PSD), RNFL, and GCL + thickness values obtained with SD-OCT were compared statistically between the two groups.Results: No marked VF defects were found in either group, and there was no statistically significant between-group difference in MD or PSD values. In terms of RNFL thickness, the average and superior quadrant RNFL values of the ES patients were thinner than those of the controls, with statistical significance (p = 0.013 and p = 0.043, respectively). Although other measured RNFL quadrant thicknesses and foveal thickness (FT), macular volume (MV), and average macular thickness (AMT) values were reduced in the ES group, these differences were not statistically significant. The average GCL + value and GCL + values in six sectors in the patient group were significantly lower than those in the control group.Conclusions: Asymptomatic ES patients have a risk of primary ES syndrome and should be followed up using a multidisciplinary approach. Objective and quantitative RNFL and GCL + thickness measurements obtained with OCT can provide valuable data for monitoring these patients.

Arthroscopic versus open, medial approach, surgical reduction for developmental dysplasia of the hip in patients under 18 months of age.

Background and purpose - The value of arthroscopic surgical reduction in developmental hip dysplasia is poorly known. We compared the clinical and radiographic efficacy of arthroscopic and medial open surgical reduction in patients less than 18 months of age with developmental hip dysplasia. Patients and methods - 54 patients with a mean age of 11 months who were treated by Ludloff's medial open reduction technique (28 hips, Group L) or arthroscopic surgical reduction technique (26 hips, Group A) were evaluated in this case series. Data on age, sex, preoperative Tönnis grade, operative time, estimated blood loss, residual leg length discrepancy, range of motion (ROM), acetabular index (AI) angle, coverage ratio of the femoral head, continuity of Menard-Shenton line, re-dislocation rate, McKay classification, and Kalamchi-MacEwen avascular necrosis (AVN) classification were collected. Results - Preoperatively, the mean AI angle was 39° in Group L and 37° in Group A. At the latest follow-up, the mean AI was 26° in both groups. The mean femoral head coverage ratio was 79% in Group L and 80% in Group A. The Menard-Shenton line was intact in all patients. Residual leg length discrepancy or limited ROM was not detected in any patients. 4 patients in Group L and 2 in Group A were diagnosed with type 2 AVN. Interpretation - Arthroscopic surgical reduction in patients aged 6-18 months revealed promising clinical and radiographic outcomes similar to medial open reduction using Ludloff's technique.

[In Vitro Evaluation of the Optical Quality of Segmental Refractive Multifocal Intraocular Lenses]. / In-vitro-Untersuchung zur optischen Qualität segmental refraktiver multifokaler Intraokularlinsen.

OBJECTIVES: In customised patient care, it is important to know the optical quality of different intraocular lenses (IOL). In this study, the optical quality of three segmental intraocular lenses were compared. MATERIALS AND METHODS: The LENTIS Comfort LS-313 MF15, LENTIS Mplus X LS-313 MF30 and LENTIS High Add IOL LS-313 MF80 (Oculentis, Berlin, Germany) with a far power of + 21 D were analysed at the optical bench OptiSpheric IOL PRO (Trioptics GmbH, Wedel, Germany). The lenses have almost the same optical design but differ in the power of the near segment. The MF15 has a + 1.5 D addition to improve vision in intermediate distances, the MF30 has a near addition of + 3 D and the MF80 has a near addition of + 8 D. The modulation transfer function area (MTFa) and the Strehl ratio were examined for apertures of 3 mm (photopic) and 4.5 mm (mesopic). RESULTS: The MTFa values for the far focus are 33.34/30.80/51.53 (MF15/MF30/MF80) with an aperture of 3 mm and 25.38/22.52/43.15 for 4.5 mm. The MTFa values for the intermediate focus are 29.85/16.21/6.25 for a 3 mm aperture and 23.92/8.05/3.08 for 4.5 mm. The MTFa values for the near focus are 9.75/21.49/33.12 for an aperture of 3 mm and 4.95/22.70/31.68 for 4.5 mm. The Strehl ratio of the far focus is 0.34/0.30/0.52 for an aperture of 3 mm and 0.24/0.22/0.43 for 4.5 mm. For the intermediate focus, the Strehl ratio is 0.30/0.17/0.07 for an aperture of 3 mm and 0.24/0.08/0.03 for 4.5 mm. The Strehl ratio of the near focus is 0.10/0.22/0.33 for an aperture of 3 mm and 0.05/0.23/0.32 for 4.5 mm. CONCLUSION: We confirmed that the addition influences the optical quality of segmental bifocal intraocular lenses. For the far focus, the results of the MF15 and MF30 are similar. In intermediate distances, the MF15 achieves the best results. For near distances, the MF30 achieves better optical values than the MF15. The lens MF80, which has been designed for patients with maculopathies, achieves good results for far and near distances.

Ab interno gel implant in patients with primary open angle glaucoma and pseudoexfoliation glaucoma.

PURPOSE: To compare efficacy and safety results of an ab interno gel implant in patients with pseudoexfoliation glaucoma (PXG) and primary open angle glaucoma (POAG). METHODS: Retrospective analysis of the medical records of 110 consecutive eyes with open angle glaucoma who had received a XEN45 gel implant between March 2014 and June 2015. Intraocular pressure course, number of glaucoma medications, the need for additional intervention (including needling) and complications were evaluated until 12 months postoperatively. RESULTS: Data of 67 eyes with POAG and 43 eyes with PXG were analyzed. At 12 months postoperatively, the mean IOP had significantly decreased by 54.0% from preoperatively 31.85 ± 8.5 mmHg to 13.99 ± 2.6 mmHg in the POAG group, (p = 0.000; Wilcoxon test), and by 55.2% from 31.63 ± 9.0 mmHg to 13.28 ± 3.1 mmHg in the PXG group (p = 0.000; Wilcoxon test). The mean number of anti-glaucoma medications had significantly decreased from 3.25 ± 0.8 at baseline to 0.3 ± 0.7 medications at 12 months postoperatively in POAG eyes (p = 0.000; Wilcoxon test), and from 3.05 ± 1.0 to 0.3 ± 0.6 medications in PXG eyes (p = 0.000; Wilcoxon test). Hypotony (IOP ≤ 6 mmHg) was observed in 2 POAG eyes (3.0%) and in 5 PXG eyes (11.7%) at 1 month but normalized in all eyes at 12 months postoperatively. Severe complications were not observed. No statistically significant differences were found between PXG eyes and POAG eyes. CONCLUSION: Our data indicate that the XEN45 gel implant provides significant and comparable reduction in IOP and anti-glaucoma medication during the one-year follow-up period in POAG as well as PXG eyes. This suggests that it may be a noteworthy alternative to traditional filtering procedures in patients with POAG and PXG respectively.

Pure elbow dislocation in the paediatric age group.

PURPOSE: The main purpose of the present study was to evaluate the clinical results and prognosis of pure elbow dislocations in the paediatric age group following non-surgical treatment. METHODS: Acute traumatic pure elbow dislocations treated between January 2008 and January 2013 were evaluated. The median age was eight years. The mean follow-up time was 46 months. Active and passive range of motion (ROM), elbow stability, neurovascular status, functional status and any early or late complications were evaluated and recorded at the latest follow-up. RESULTS: The mean flexion-extension ROM was measured as 119.5 degrees. The mean pronation and supination were 67 and 79 degrees. Moderate instability was diagnosed in four cases. The mean Mayo Elbow Performance Score (MEPS) score was 91.6 points; the clinical outcome was excellent in nine patients, good in two and fair in one. CONCLUSIONS: Acute traumatic pure elbow dislocation in childhood is a very rarely seen emergency that can be treated safely with closed reduction combined long-arm plaster splinting and physical rehabilitation.

Failed treatment of long bone nonunions with low intensity pulsed ultrasound.

INTRODUCTION: The use of low intensity pulsed ultrasound (LIPUS) in the treatment of nonunions is still controversial. The present study is concerned with whether this procedure has a clinical use and which cofactors influence its therapeutic results. METHODS: In this prospective, single institution, observational study, data from October 2010 to October 2013 from 61 nonunions in 60 patients treated with EXOGEN(®) LIPUS therapy were analysed. The average age was 45.4 ± 9.81 (18-63) years. Treatment was primarily done on long bones of the lower extremity (75.4 %). All 61 nonunions were examined after treatment, and the rate of healing as well as functional and subjective results were evaluated. Based on clinical and radiological findings, patients were divided into two groups: G1-successful treatment; and G2-unsuccessful treatment. Groups were compared to one another to identify possible factors influencing treatment. RESULTS: Twenty (32.8 %) patients showed bone consolidation with an average time of healing of 5.3 (2-7) months. In patients without successful treatment, who underwent revision surgery instead, full weight bearing took on average 3.7 months longer, and they were able to return to work 6.8 months later. Most of the treated patients (70.5 %) reported no improvement in pain. In G2, 12 (29.3 %) patients suffered in their previous history from osteitis; in G1 there were only two patients (10 %) (p = 0.012). There were further significant differences in the age of the fracture, the type of osteosynthesis, the gap size, as well as the NUSS score. CONCLUSION: Despite patients being chosen strictly according to EXOGEN(®) indications, only a small number of patients with nonunions who underwent LIPUS therapy experienced successful treatment (32.8 %). Overall, its use resulted in a clear delay in the time of treatment, so that according to our results, the use of LIPUS should be seen critically in long bone nonunions and use should be made on a case-by-case basis.

Surgical Treatment of Hip Instability in Patients With Lower Lumbar Level Myelomeningocele: Is Muscle Transfer Required?

BACKGROUND: Treatment of hip instability in patients with lower lumbar level myelomeningocele is clinically challenging. Muscle transfer procedures, release of contractures, and intertrochanteric varus-rotation osteotomies have been described to restore weak or absent abductor strength as well as relocation of the hip. However, controlled trials evaluating hip instability in lower lumbar myelomeningocele are limited in the current literature. QUESTIONS/PURPOSES: The purposes of this study were to compare the (1) radiographic evidence for joint stability; (2) clinical outcomes (including abductor strength, ambulatory ability, and residual use of orthoses); and (3) complications between patients undergoing combined periarticular contracture releases and bony procedures with and without external oblique abdominal muscle transfers. METHODS: Between 2004 and 2013, 14 pediatric patients (16 hips) were treated for hip instability secondary to myelomeningocele using releases with or without muscle transfer. From those, 13 patients (15 hips) with a mean age of 6 years who had L3 to L5 level involvement were evaluated retrospectively. The patients were separated into two groups. Nine hips (in eight patients) were treated by performing a combination of external oblique abdominal muscle transfer to the greater trochanter, periarticular release of contractures, and bony procedures. These were compared with six hips (five patients) treated by performing a combination of periarticular release of contractures and bony procedures without external oblique abdominal muscle transfer. This study compared the results between two surgeons, one of whom always performed these muscle transfers in this setting and the other who never performed muscle transfer during the study period. The patients were clinically followed up at a mean of 41 months (range, 14-122 months); none of the patients was lost to followup. Radiographic evaluation criteria included Reimer's migration index, acetabular index, femoral neck-shaft angle, pelvic obliquity, and the presence of scoliosis. Clinical evaluation included muscle strength examination, periarticular contractures, necessity for using orthoses as walking aids, and Hoffer criteria of mobility. All complications were also noted from a chart review. RESULTS: There were no differences between the two groups regarding postoperative femoral head localization, reflecting the presence or absence of residual subluxation, according to Reimer's index (Reimer's index = 32%; range, 10%-40%, in the muscle transfer group compared with 27%; range, 15%-43%, in the no-transfer group at latest followup; p = 0.723). Postoperatively, abductor strength improved in the group treated with external oblique transfer compared with the group treated without muscle transfer (median improvement of 2 versus 0, p = 0.02), but this improvement neither resulted in a clinically important difference in Hoffer criteria of mobility (no change was detected in either group) nor decreased the need for use of an orthosis (no change was detected in either group). With the numbers available, there was no difference with respect to complications between the two groups (two complications versus one). CONCLUSIONS: External oblique abdominal muscle transfer did not provide a clinically important improvement in functional recovery when compared with patients with L3 to L5 myelomeningocele and hip instability who were treated without it. We therefore do not recommend a routine muscle transfer procedure during the operative management of hip instability in patients with lower lumbar level myelomeningocele. LEVEL OF EVIDENCE: Level III, therapeutic study.

Poloxamer-188 and citicoline provide neuronal membrane integrity and protect membrane stability in cortical spreading depression.

Under pathological conditions such as brain trauma, subarachnoid hemorrhage and stroke, cortical spreading depression (CSD) or peri-infarct depolarizations contribute to brain damage in animal models of neurological disorders as well as in human neurological diseases. CSD causes transient megachannel opening on the neuronal membrane, which may compromise neuronal survival under pathological conditions. Poloxamer-188 (P-188) and citicoline are neuroprotectants with membrane sealing properties. The aim of this study is to investigate the effect of P-188 and citicoline on the neuronal megachannel opening induced by CSD in the mouse brain. We have monitored megachannel opening with propidium iodide, a membrane impermeable fluorescent dye and, demonstrate that P-188 and citicoline strikingly decreased CSD-induced neuronal PI influx in cortex and hippocampal dentate gyrus. Therefore, these agents may be providing neuroprotection by blocking megachannel opening, which may be related to their membrane sealing action and warrant further investigation for treatment of traumatic brain injury and ischemic stroke.