[Antenatal diagnosis of limb body wall complex]

Abstract

The "limb body wall complex" or LBWC is a rare polymalformative syndrome. Two distinct phenotypes of which were recently described: one form with "placento-cranial" adhesion and the other with "placento-abdominal" adhesion. Coelosomia is found in all cases, it variably coexists with encephalic, vertebral, visceral or limb anomalies. Three pathogenesis are proposed: the "early amnion rupture" theory and the "vascular" theory can explain the form with "placento-cranial" adhesion, conversely, the "defective folding process" allows for a better explanation of the form with "placento-cranial" adhesion. These theories do not exclude each other if we accept that this syndrome could be split: the first form would then be the original "LBWC" et could belong to "amniotic band syndrome", the mechanism of which is not precisely known, whereas the second could be named "body stalk syndrome" - "syndrome du cordon court". In any case, this syndrome should be better known so as to be studied and give a prognosis for a later pregnancy, because there is no recurrence.