Zur Klinik, Symptomatologie, Pathogenese und Erbpathologie des Klippel-Trénaunay-Weber-schen Syndroms

Abstract

SUMMARYThe Klippel-Trénaunay-Parkes Weber-syndrome (K.T.W.) is a congenital malformation with partial gigantism (osteohypertrophy), naevi and varicose veins in the fully developed trias of symptoms. According to arterio- and phlebographic findings varicose veins are usually the result of mal-and deformations of the deep venous system or arteriovenous anastomosis, rarely of the arterial system alone.In a critical consideration of all hypotheses about aetiology and pathogenesis of the K.T.W.-syndrome is discussed that there is no satisfactory explanation for the assumption that the syndrome is a exogenous malformation.It is pointed out that so far there have been no family investigation of a greater extent. The statistic survey of the existing family investigations showes that the K.T.W.-syndrome is most variable in its phaenotypical manifestation and also has a strong intra-and interfamiliar variability. Heredopathologically is suggested that apart from a weak (?) principal gene secondary genes are essential for the manifestation. The mode of inheritance may be either dominant or recessive. The existence of various biotypes is considered probable. It is proposed to count the K.T.W.-syndrome into the group of the ectome-sodermal malformations (Oehler), the hamartomata (Wohlwill), the phacomatoses (van der Hoewe) or the neuro-cutaneous syndromes. 2 own cases are described.