A.B.R. in Nager type acrofacial dysostosis syndrome

Acrofacial dysostosis 1, Nager type

A Case Report: Nager Acrofacial Dysostosis

INTRODUCTION Nager syndrome is a malformation resulting from problems in the development of the first and second branchial arches and limb buds. The cause of the abnormal development of the pharyngeal arches in Nager syndrome is unknown. It is also unclear why affected individuals have bone abnormalities in their arms and legs. Nager syndrome is thought to have an autosomal recessive inheritanc...

Preaxial acrofacial dysostosis (Nager syndrome): a case report

This case report describes the surgical and dental management of a 12-year-old girl with preaxial acrofacial dysostosis also known as Nager syndrome. It highlights the importance of multidisciplinary treatment at an early age and the effect of parental noncompliance on the overall physical and psychological development of a medically compromised patient.

The Nager acrofacial dysostosis syndrome with the tetralogy of Fallot.

A male infant is described with mandibulofacial dysostosis and absent thumbs, consistent with the Nager acrofacial dysostosis syndrome. In addition, the tetralogy of Fallot was present. Major congenital heart malformations occur rarely in this syndrome.

Autosomal recessive inheritance of Nager acrofacial dysostosis.

Nager acrofacial dysostosis is a variant of mandibulofacial dysostosis with severe micrognathia, malar hypoplasia, and radial limb defects. Most cases are sporadic, but autosomal recessive inheritance has been suggested. A family is reported in which two sibs are affected by this syndrome, presenting further evidence for autosomal recessive inheritance. The recognition of this syndrome as a dis...