Hypertelorism and the median cleft face syndrome. An embryological analysis.

Abstract

A microscopical study of the early and late development of the face was performed in 77 human embryos and fetuses. After the transformation of both nasal placodes, via nasal grooves, into the nasal tubes the ectoderm of the face is closed superficially and the early development of the face (less than or equal to 17 mm crown-rump length (C-RL)) is terminated. Between the nasal tubes the internasal groove is present. Furthermore these embryos show physiologically a flat nose and hypertelorism. During the late development (greater than or equal to 17 mm C-RL) of the face the internasal groove disappears due to the outgrowth and differentiation of the nasal septum in the frontocaudal direction. Simultaneously (17-27 mm C-RL) the distance between the eyes decreases relatively, because of a relative lag in transverse growth. The differentiation of the facial mesenchyme into bone centres starts in the same period. From this embryological point of view the major anomalies of the median cleft face syndrome (hypertelorism--orbital as well as interorbital--and cranium bifidum occultum, median cleft nose, median cleft prolabium and median cleft premaxilla), can be classified as secondary or late, i.e., differentiation, defects.