No abstract

No abstract

Children with a mucopolysaccharidosis or mucolipidosis suffer progressive disability of the hands, particularly in relation to dysfunction of the median nerve. This is an increasing problem because bone-marrow transplantation has dramatically improved survival without apparently changing the musculoskeletal manifestations. We have reviewed 48 children with these syndromes who required carpal tunnel decompression, recording symptoms, signs, radiological, electrophysiological and operative findings, histology and upper-limb function. In these children the carpal tunnel syndrome differs from that seen in adults. Symptoms are rare but signs such as decreased sweating, pulp atrophy, thenar wasting and manual clumsiness are much more common. At operation, the flexor retinaculum was thickened and a mass of white tenosynovium engulfed the flexor tendons. Most patients had some definite nerve constriction with a thickened epineurium.Functional improvement was seen after early decompression, with some benefit from simultaneous tendon release. Regular physiotherapy helped to maintain increased hand movement.We describe our assessment protocol, the physiotherapy and operative regime and the standard functional review which helps to maximise function in the hands and upper limbs of these children.

This study corroborates a previous study by the same authors that showed equally good results in clinical outcome and gait between the conventional single radius and medial pivot designs under stringent testing conditions. Cite this article: 2018;(1 Supple A)100-B:76-82.

Recurrent patellofemoral instability is a disabling condition, attributed to a variety of anatomical aetiologies. Trochlear dysplasia, patella alta, an increased tibial tubercle trochlear groove distance of greater than 20 mm and soft tissue abnormalities such as a torn medial patellofemoral ligament and inadequate vastus medialis obliquus are all factors to be considered. Management of this condition remains difficult and controversial and knowledge of the functional anatomy and biomechanics of the patellofemoral joint, a detailed history and clinical examination, and an accurate patient assessment are all imperative to formulate an appropriate management plan. Surgical treatment is based on the underlying anatomical pathology with an aim to restore normal patellofemoral kinematics. We summarise aspects of assessment, treatment and outcome of patellofemoral instability and propose an algorithm of treatment.