Noonan syndrome in 12-year-old male: Case report and orthodontic management of the occlusion

Abstract

SummaryBackground/Aim: Noonan syndrome (NS) is an autosomal dominant disorder, caused by mutations on genes located on the long arm of chromosome 12. The condition has no sex or race predilection and its incidence is 1 per 1,000 – 2,500 live births. Individuals affected with Noonan syndrome have distinctive facial features, hypertelorism, short stature, congenital heart disease; mainly pulmonary stenosis and hypertrophic cardiomyopathy, chest deformities, variable learning disabilities and mental retardation. Orofacial findings in Noonan syndrome may be high-arched palate, micrognathia, dental malocclusion and articulation difficulties.Case report: The present article reports on a male case of 12 years old, referred for treatment in the orthodontic office. Despite the difficulties of hyperactivity, the light mental delay and the gag reflex, the treatment was completed satisfactorily with fixed orthodontic appliances in 15 months. Both the patient and his parents were happy with the results. The patient is presently undergoing the retention period of this orthodontic treatment.Conclusions: Despite the difficulties of treating a child with a genetic syndrome for his/her malocclusion, the reported case presented in this article proves that it is always worth trying for the benefit of the patient.

Cephalometric Evaluation of Children with Short Stature of Genetic Etiology. A Review

“…The external ear position in individuals with Noonan syndrome does not differ significantly from that of healthy controls [41]. The mandibular incisors seem to be labially inclined in most reports [61,64], but not all of them [60,62].…”

“…However, the length of the jaws seems to be reduced. There are reports of class I malocclusion [60][61][62]. Several reports indicate a posteriorly placed mandible [15,[63][64][65].…”

“…c) Vertical plane: Most reports indicate a downwards mandibular growth pattern with hyperdivergent planes and increased vertical angles [15,[62][63][64]66]. Nevertheless, cases of a horizontal growth pattern [60], an increased vertical overjet [62,63] and a decreased vertical overjet [61,65] have been reported.…”