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

  • Journal of the Korean academy of pediatric dentistry
  • Abbr : J Korean Acad Pediatr Dent
  • 2018, 45(1), pp.115-122
  • DOI : 10.5933/JKAPD.2018.45.1.115
  • Publisher : The Korean Academy Of Pediatric Dentistry
  • Research Area : Medicine and Pharmacy > Dentistry
  • Received : June 26, 2017
  • Accepted : August 29, 2017
  • Published : February 28, 2018

Inkyung Hwang 1 Yeonju Lee 1 Dohee Sim 1 Yonjoo Mah 2

1이화여자대학교 의과대학 목동병원 소아치과학교실
2이화여자대학교 의학전문대학원 치과학교실

Accredited

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.

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