[Embryology and etiology of the Poland and Amazone syndromes]

In 1841 A. POLAND described a rare complex of malformations in the male, characterized a unilateral pectoral muscle defect combined with ipsilateral symbrachydaktyly. If in the female a POLAND Syndrome is associated with ipsilateral hypo- or aplasia of the breast as a leading feature, we propose to name this entity "Amazone Syndrome". Altogether 5 POLAND Syndromes and 40 "Amazone Syndromes" were seen in our department. For the latter a morphological grading into four groups was performed for clinical purposes. The malformation complex of the poland syndrome develops between the 2nd and 5th week of gestation as a result of damage to an upper extremity bud. It is from this extremity-"Anlage" that the muscles of the shoulder girdle and the lateral thoracic wall (with common innervation by the brachial plexus) arise. In early embryonal life the lacteal ridge lies in close proximity to the exrremity bud. The "Amazone Syndrome" can be explained by simultaneous damage to an extremity bud and the lacteal ridge. Its aetiology is not clear. Lack of embryonal material, heredity, and teratogenic damage by medication are discussed. In our patients there was a strong suspicion of unwanted pregnancies with failed induced interruptions. On the other hand some siblings had various other congenital deformities. In conclusion we may state that any inhibition of the normal growth of the upper extremity bud may lead to the POLAND Syndrome, while a simultaneous damage to the extremity bud and the close-by lacteal mound may result in the "Amazone Syndrome.