Oral Manifestations of Noonan Syndrome: Review of the literature and a report of four cases

Abstract

Noonan syndrome (NS) was described by Noonan and Ehmke as a multi-system disorder, which is typically evident at birth. The incidence of this syndrome is estimated to be one per 2500 to one per 1000 and affects both genders. While the clinical manifestations of NS have been well documented, the oral manifestations have not been extensively discussed. The purpose of the present article is to (a) review the oral manifestations of NS reported in the literature, and (b) describe four cases (three females and one male) of NS, who presented with short stature, cardiac problems and various oral findings. Based on these cases, we conclude that many oral anomalies may have possible relationships with NS, which require multidisciplinary treatment planning and timely management. The importance of oral findings in NS has largely gone unnoticed and it is essential to consider oral manifestations as scoring criteria in the diagnosis of NS.