Summary:
The author summarizes hitherto assembled experience with the clinical and genetic characteristics of Poland’s
and Möbius syndrome. Five selected case-records with this disease and the sequence of the Poland-Möbius syn-
drome are presented. Another case-record is devoted to an allied syndrome, hypoglossia-hypodactyly, found in
a spontaneously aborted fetus. For establishment of a more accurate symptomatology, an irreplaceable place is
held by anthropometric examination; for objectifying the asymmetry of the chest the so-called cyrtogram, the
chest circumference recorded by means of a wire, is valuable. From the aspect of genetic counseling, preconcep-
tion care is always provided to mothers from families with reproductive intentions, as well as ultrasonographic ex-
amination of the fetus in areas of assumed acral symptomatology (signaling phenotype). In two families
ultrasonography was used for prenatal diagnosis. Invasive prenatal diagnosis by amniocentesis was employed in
a family with Möbius syndrome. In these families dermatoglyphs have certain common characteristics, such a ten-
dency towards simple patterns. In the wider family of one of our patients we detected in a cousin Parkes-Weber-
Klippel-Trenaunay’s syndrome, which may indicate common vascular predisposing factors.
Key words:
Poland’s syndrome, Möbius syndrome, vascular disruption sequence, genetics, dermatoglyphs
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