Denouement and Discussion Marie-Sainton Syndrome, Mutational
Dysostosis Frontal, parietal, and occipital bossing forming a
large globular-shaped skull. Aplasia of the entire clavicle or
its midportion allowing the shoulders to approximate anteriorly.
An autosomal dominant with high penetrance and variable
expressivity. HeadFrontal, parietal, and occipital bossing
forming a large, globular-shaped skull. Fontanel is widely open
at birth and remains so until adulthood. Sutures are also widely
open. Wormian bones are present.ClaviclesThere is symmetrical
aplasia or hypoplasia of the cartilaginous part of the bone. The
midportion of the clavicle is most commonly absent. The sternal
and acromial ends may be present. The shoulders can be
approximated anteriorly.Oral CavityThe deciduous teeth are
normal, but the permanent teeth may be absent, delayed in
eruption, or have defective enamel and deficient roots. The
palate is high arched with an