PALATE STRUCTURE IN HUMAN HOLOPROSENCEPHALY CORRELATES WITH THE FACIAL MALFORMATION AND DEMONSTRATES A NEW PALATAL DEVELOPMENTAL FIELD

In this study we analyzed palate structure in holoprosencephaly and co rrelated it with the facial malformations, Eleven human holoprosenceph alic fetuses (three cyclopic, two ethmocephalic, one cebocephalic, fou r with median cleft lip, and one with short philtrum) at 17-23 weeks o f gestation and three children (age 2 1/2, 6 and 7 years) with a singl e central incisor were studied, Photographic and radiographic methods were used, We found that in holoprosencephaly palate structure is abno rmal, The severity of this malformation decreases with decreasing seve rity of facial malformation Thus, the study shows a close relationship between the facial and the palatal malformation, In all phenotypes th e premaxillary area is malformed, From this region, a fan-shaped field along the midpalatal suture is involved in all facial phenotypes, the fan being broadest in cyclopia and narrowest in the short philtrum ma lformation, A similar fan-shaped field can be discerned in the face, w here the broadest fan also indicates the greatest severity with cyclop ia, and the narrowest fan the least severe median lip malformation, In the palate field, the anteroposterior furrows seemingly demarcate the field, The findings may be of importance for the future evaluation of palatal malformations in children. (C) 1997 Wiley-Liss, Inc.