Abstract

We read with interest the article by N. Van Meir and L. De Smet on ‘Carpal tunnel syndrome in children‘, in the October 2003 issue of ‘Acta Orthopaedica Belgica' (4). The authors should be congratulated for their excellent manuscript A literature search had been made and the underlying aetiology of carpal tunnel syndrome (CTS) in children has been reviewed by the authors. They state that macrodactyly, ‘a benign localised form of gigantism', is another unusual cause of CTS in children. We think that this could be part of other asymmetric overgrowth syndromes such as Proteus syndrome, rather than being a localised gigantism. In the aetiology of CTS, the authors could have mentioned Proteus syndrome, which is characterized by macrodactyly of hands and/or feet, skin naevi and subcutaneous-internalperipheral nerve hamartomas (2, 3). Proteus syndrome is also of interest because of clinical overlap with the Klippel Trenaunay syndrome (1).<br /><h1>Authors' reply</h1>We have read the comments from Dr Saliha Senel with great interest. The correct diagnosis of overgrowth syndromes is challenging. There is an overlap between isolated macrodactyly, Proteus syndrome and Klippel Trenaunay. In the cases reported by us and those found in the literature, macrodactyly did not appear to be part of the full blown Proteus syndrome, mainly due to the absence of other characteristic features. There is however a continuing discussion about the relationship between nerve compression and macrodactyly, as some authors believe that the nerve overgrowth is the cause of the overgrowth of the soft tissues. In contrast, we never saw hypertrophic nerves in Proteus syndrome.