J Korean Acad Pediatr Dent.  2018 Feb;45(1):115-122. 10.5933/JKAPD.2018.45.1.115.

Oral Features in a Child with Noonan Syndrome : A Case Report

• Hwang I
• Lee Y
• Sim D
• Mah Y

• KMID: 2430322
• DOI: http://doi.org/10.5933/JKAPD.2018.45.1.115

Abstract

Noonan syndrome is characterized by distinctive facial features, short stature, and congenital heart disease. It is a congenital genetic disorder with a prevalence of between 1/1,000 and 1/2,500 in both genders. An 11-year-old boy with Noonan syndrome visited the hospital with an ectopically positioned tooth. A pulmonary stenosis was diagnosed and his growth and development were delayed. In many cases of this diseases there is obvious hemostasis, which he was not experiencing. His facial appearance showed characteristic features of Noonan syndrome. The patient showed a dental class II relationship, labioversion of the upper anterior teeth, and a shallow overbite. Radiographic examination revealed that the upper right canine was ectopically positioned, which led to root resorption of the upper right lateral incisor. A lateral cephalometric radiograph revealed a craniofacial pattern that was within normal limits. Surgical opening and button attachment on the impacted upper right canine were performed and traction was applied on the impacted tooth using a removable appliance. This patient was mildly affected by Noonan syndrome and showed some dental problems. However, few studies have reported the oral characteristics of Noonan syndrome despite its high incidence. Thus, this case report describes the oral features and management of Noonan syndrome.

Keyword

Noonan syndrome; Dental; Oral; Features; Characteristic

MeSH Terms

Child*
Growth and Development
Heart Defects, Congenital
Hemostasis
Humans
Incidence
Incisor
Male
Noonan Syndrome*
Overbite
Prevalence
Pulmonary Valve Stenosis
Root Resorption
Tooth
Tooth, Impacted
Traction