Pierre Robin sequence typically occurs de novo in most patients and is associated with

hypoplasia (under- development) of the mandible, cleft palate, and defects of the eyes and ears.
Rarely, it is inherited in an autosomal dominant pattern. In the Robin morphogenetic complex,
the initiating defect is a small mandible (micrognathia), which results in posterior displacement
of the tongue and obstruction to full closure of the palatal processes, result- ing in a bilateral cleft
palate (see Figs. 9-39 and 9-40).

(1)

Retardation of mandibular development gives rise to micrognathia of

varying degrees, with accompanying dental malocclusion. Total failure
of development of the mandible (agnathia) is associated with abnormal
ventral placement of the external ears (synotia) (see Fig. 3–21).
(2)