Early predictors of disability of paediatric-onset AQP4-IgG-seropositive neuromyelitis optica spectrum disorders.

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.

Innovative treatment for pes cavovarus: a pilot study of 13 children.

Background and purpose - Pes cavovarus (PCV) is a complex deformity, frequently related to neurological conditions and associated with foot pain, callosities, and walking instability. The deformity has the tendency to increase during growth. Orthotic treatment is ineffective and surgery may be troublesome. We present the preliminary results of a new mini-invasive surgical technique for correction of this foot deformity. Patients and methods - We operated on 13 children (24 feet), age 7-13 years. In 7 children the deformity was neurological in origin. The surgical technique included a dorsal hemiepiphysiodesis of the 1st metatarsal, and a plantar fascia release. The clinical deformity, hindfoot flexibility, and foot callosities, together with a radiological assessment (Meary angle, calcaneal pitch, and talo-calcaneal angle), was done pre- and postoperatively. At final check-up, after a median of 28 months (12-40), the Oxford Ankle Foot Questionnaire for children (OXAFQ-C) was used to assess patient satisfaction. The primary outcome was the hindfoot varus correction. Results - All the operated feet improved clinically and radiologically. Heel varus improved from a mean 6° preoperatively to 5° valgus postoperatively. In those children where treatment was initiated at a younger age, full correction was achieved. Footwear always improved. Interpretation - This treatment may offer a less aggressive alternative in the treatment of PCV in young children and may eventually reduce the amount of surgery needed in the future.

Retrograde Percutaneous Drilling for Osteochondritis Dissecans of the Head of the Talus: Case Report and Review of the Literature.

Osteochondral lesions of the talus might be a more common cause of pain than previously recognized, especially among those involved in athletic activities. However, the location of an osteochondral lesion on the talar head is much less common than such lesions localized to the dome of the talus and can pose diagnostic difficulties. We present the case of a 14-year-old soccer player who complained of longstanding pain in his left foot. After unsuccessful conservative treatment consisting of rest and bracing, he was ultimately treated with retrograde percutaneous drilling of the talar head performed by a medial approach. This was followed by casting and non-weightbearing for 6 weeks, after which physical therapy was undertaken. He was able to return to full activity and remained asymptomatic during a 5-year observation period. Although rare, osteochondritis dissecans of the talar head should be considered in young athletes with persistent foot pain that is unresponsive to reasonable therapy.

Maximizing length with precice nail: a novel technique.

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.

How to manage pes cavus in children and adolescents?

Pes cavus in its different forms is not a pathological entity, but rather the manifestation of multiple diseases.Cavovarus, a form of cavus foot, should never be considered a physiological deformity. A neurological condition should always be excluded.The evolution of pes cavovarus is unpredictable because of the large number of conditions involved in its aetiology, as well as their variable degree of expression. About 66% of cavovarus feet are the result of subtle neurological diseases, which only become evident later in life.Although surgery may not change quality of life, recent studies suggest that it may improve foot posture and reduce walking instability.The aim of treatment is to preserve a painless, plantigrade, mobile foot. Management consists of correcting bone deformity while preserving movement, and the wise use of rebalancing techniques. Arthrodesis should only be a salvage procedure. Cite this article: EFORT Open Rev 2021;6:510-517. DOI: 10.1302/2058-5241.6.210021.

Calcifying aponeurotic fibroma in children: our experience and a literature review.

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.

Histological differences between various methods of hemiepiphysiodesis: is guided growth really different?

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.