[A complex malformation of the first and second branchial arch and of the first bran-hial arch and of the first branchial cleft. Dysostosis temporo-spheno-facialis (author's transl)].

A complex malformation of the first and second branchial arches and of the first branchial cleft is described; there was also involvement of the axial skeleton and the limbs. The syndrome has been described by us as dysostosis temporo-sphenofacialis and could not be classified with any previously described entity. The reason for this was due to the imcomplete description of previously published cases, but also the large number of possible combinations of changes in various organs which can occur. In order to understand the condition more clearly, the aetiology and pathogenesis of abnormalities of the face and skull have been considered in detail. Adequate and accurate radiographic examination is considered as a pre-requisite to surgery, which can be cosmetic or improve hearing, and which may have good results.