Result of a family study based on 584 patients with craniostenosis brings some 
answers useful for genetic counselling. For 98 patients (15%) a syndrome is 
associated. Third part of them has Apert syndrome, an other third part has 
Crouzon syndrome, and for the last third more exceptional acrocephalosyndactyly 
syndrome (Saethre-Chotzen, Pfeiffer) or others atypical associations, sometimes 
not yet described, but with an autosomal dominant inheritance. Non syndromic 
craniostenosis involves differently according to the type of join, but the 
localization is the same if recurrence will be happen. Coronal craniostenosis 
seems to be a dominant autosomal character, when scaphocephaly is more often 
sporadic; for both, an autosomal dominant inheritance is not excluded for some 
pedigrees. If the recurrence risk exist in some cases, it is generally well 
accepted by parents on account of the good neurosurgeon prognosis.