CARPAL-TUNNEL SYNDROME IN THE MUCOPOLYSACCHARIDOSES AND MUCOLIPIDOSES

Children with a mucopolysaccharidosis or mucolipidosis suffer progress ive disability of the hands, particularly in relation to dysfunction o f 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 child ren with these syndromes who required carpal tunnel decompression, rec ording symptoms, signs, radiological, electrophysiological and operati ve 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 wastin g and manual clumsiness are much more common, At operation, the flexor retinaculum was thickened and a mass of white tenosynovium engulfed t he flexor tendons, Most patients had some definite nerve constriction with a thickened epineurium. Functional improvement was seen after ear ly decompression, with some benefit from simultaneous tendon release, Regular physiotherapy helped to maintain increased hand movement. We d escribe our assessment protocol, the physiotherapy and operative regim e and the standard functional review which helps to maximise function in the hands and upper limbs of these children.