IngentaConnect Orofacial Aspects in Noonan Syndrome: 2 Case Report

Authors: de Oliveira Lira Ortega, Adriana¹; de Oliveira Guaré, Renata²; Kawaji, Nilce Samecima³; Ciamponi, Ana Lídia⁴

Source: Journal of Dentistry for Children, Volume 75, Number 1, January-April 2008 , pp. 85-90(6)

Publisher: American Academy of Pediatric Dentistry

Abstract:

Noonan syndrome (NS) is an autosomal dominant disorder characterized by dysmorphic facial features in association with short stature and heart disease. A webbed neck, chest deformities, mental retardation, and bleeding disorders are also frequently associated with this pathology. NS is relatively common, with an estimated incidence of 1 per 1,000 to 1 per 2,500 live births. Many cases have been reported in the dental literature, yet only a few of them describe some details of the oral features. The aim of this paper is to describe 2 cases of Noonan syndrome, emphasizing oral and facial aspects and particularities of the dental treatment in subjects affected by this genetic disease.

Keywords: NOONAN SYNDROME; GENETIC DISEASE; ORAL ASPECTS; MALOCCLUSION; CONGENITAL HEART DISEASE; BLEEDING DIATHESIS

Document Type: Case report

Affiliations: 1: Doctorate in Dental Sciences student, Federal University of São Paulo, Brazil; Group for the Study and Treatment of Special Needs Patients of the Pediatric Dentistry Discipline of the University of São Paulo, Brazil 2: Group for the Study and Treatment of Special Needs Patients of the Pediatric Dentistry Discipline, both at the University of São Paulo, Brazil; Special Needs Patients Discipline, Cruzeiro do Sul University, São Paulo, Brazil 3: University of São Paulo, Brazil 4: Group for the Study and Treatment of Special Needs Patients of the Pediatric Dentistry Discipline, Dediatric Dentistry, University of São Paulo, Brazil

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