Wh. Arnold et al., CRANIOFACIAL SKELETAL DEVELOPMENT IN 3 HUMAN SYNOPHTHALMIC HOLOPROSENCEPHALIC FETUSES, ANNALS OF ANATOMY-ANATOMISCHER ANZEIGER, 180(1), 1998, pp. 45-53
In three human fetuses with synophthalmic holoprosencephaly (8, 14, 23
wks. p.c.) and two normal human fetuses (9 and 13 wks. p.c.) the anat
omy of the cranial base, facial cranium and their relation to the noto
chord was studied using serial histological sections and computer aide
d three-dimensional reconstruction methods. Mesethmoidal cartilage dif
ferentiation was variably deficient in all three holoprosencephalic ca
ses. The premaxillary bones were rudimentary with missing tooth buds.
The development of the sphenoid bone was defective in two of the holop
rosencephalic cases (8, 14 wks. p.c.). The notochord terminated normal
ly within the sphenoid body in all investigated cases. Our results ind
icate that in holoprosencephaly there is a general defect in the midli
ne cranial cartilage differentiation rostral to the notochord.