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Objective: To describe onset clinical features predicting time to first relapse and time to long-term visual, motor and cognitive disabilities in paediatric-onset aquaporin-4 antibody (AQP4-IgG) neuromyelitis optica spectrum disorders (NMOSDs).Methods: In this retrospective UK multicentre cohort study, we recorded clinical data of paediatric-onset AQP4-IgG NMOSD. Univariate and exploratory multivariable Cox proportional hazard models were used to identify long-term predictors of permanent visual disability, Expanded Disability Status Scale (EDSS) score of 4 and cognitive impairment.Results: We included 49 paediatric-onset AQP4-IgG patients (38.8% white, 34.7% black, 20.4% Asians and 6.1% mixed), mean onset age of 12±4.1 years, and 87.7% were female. Multifocal onset presentation occurred in 26.5% of patients, and optic nerve (47%), area postrema/brainstem (48.9%) and encephalon (28.6%) were the most involved areas. Overall, 52.3% of children had their first relapse within 1 year from disease onset. Children with onset age <12 years were more likely to have an earlier first relapse (p=0.030), despite showing no difference in time to immunosuppression compared with those aged 12-18 years at onset. At the cohort median disease duration of 79 months, 34.3% had developed permanent visual disability, 20.7% EDSS score 4 and 25.8% cognitive impairment. Visual disability was associated with white race (p=0.032) and optic neuritis presentations (p=0.002). Cognitive impairment was predicted by cerebral syndrome presentations (p=0.048), particularly if resistant to steroids (p=0.034).Conclusions: Age at onset, race, onset symptoms and resistance to acute therapy at onset attack predict first relapse and long-term disabilities. The recognition of these predictors may help to power future paediatric clinical trials and to direct early therapeutic decisions in AQP4-IgG NMOSD. [ABSTRACT FROM AUTHOR]

Intramedullary magnetic lengthening nails represents a marked advance in the process of limb lengthening, reducing the complications associated with other methods, and improving patient's comfort. However, one important limitation is its lengthening ability, especially when it comes to short bones. Previous reports have shown techniques that may allow further lengthening but at a cost of having to carry out repeated procedures, requiring the use of other devices, and submitting the patient to long-winded surgical procedures. This report presents a novel technique to achieve lengthening beyond the initial reach of the nail, in a simple and quick procedure, without the need of implanting any additional devices., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Calcified aponeurotic fibroma is a rare benign fibrous tumour with characteristic histopathological features commonly observed in children and adolescents. It usually involves the most distal part of the extremities. Despite being a benign tumour, it has a high risk of local recurrence. We report our experience treating four children with calcifying aponeurotic fibroma who underwent surgical excision of the tumour. Two of them developed a local recurrence: one required a surgical revision, whereas the other currently continues to be under observation. The incidence found over a 5-year period (2010-2015) may lead us to consider that this condition is not as rare as reported and may often be misdiagnosed.

Traditional systems of hemiepiphysiodesis are based on the application of asymmetrical compression to the physis to correct angular deformities. The guided growth method claims to act as a tension plate avoiding compression. The aim of this study was to confirm or refute this claim. Twenty-four White New Zealand rabbits were subjected to a proximal tibial hemiepiphysiodesis using either staples or a plate and two-screws method. Both methods succeeded in producing deformity. The initial existent histological differences between systems became less apparent after 6 weeks of hemiepiphysiodesis, when histological results were very similar. The findings suggest that the eight-plate system produces, like staples, compression of the physis, but the forces are applied more gradually.