Frontonasal dysostosis, retarded growth, and mental
development. Brachycephaly, broad notched nasal tip, cleft lip/palate, and
macrostomia. Polysyndactyly,
Frontonasal dysostosis, retarded growth, and mental
development. Brachycephaly, broad notched nasal tip, cleft lip/palate, and
macrostomia. Polysyndactyly, camptodactyly, and hypoplasia of the distal
phalanges of the hands. Iliac hypoplasia, short legs, and hypospadias.
Type I: Postaxial Polysyndactyly, Frontonasal Dysostosis, and Cleft
Lip/Palate; Cleft Lip/Palate with Frontonasal Dysostosis and Postaxial
Type II: Naguib-Richieri-Costa Syndrome; Richieri-Costa-Montagnoli Syndrome;
Hypertelorism, Hypospadias, and Polysyndactyly Syndrome.
The term dysostosis refers to a defect in the ossification process,
which results in malformations of individual bones arising either isolated
or multiple. Some patients also exhibit axial involution. The dysostosis
predominantly involves the extremities. The most common forms are syndactyly
or polydactyly, which may occur in combination with craniofacial or other
abnorm