AÇBİD 13th International Congress-2019, Antalya, Türkiye, 26 Nisan 2019, ss.67
Noonan Syndrome(NS) is an autosomal dominant, variably expressed, a multisystem disorder with an estimated prevalence of 1 in 1000-2500. Most affected
individuals have characteristic facial features that evolve with age; a broad,
webbed neck, short stature, feeding difficulties, sternal deformity, pubertal delay,
cryptorchidism, etc. Frequently, ectopic unerupted teeth are involved by lesions.
Here we report a case who is diagnosed with NS with various outcomes in the oral
and maxillofacial region such as multilocular radiolucencies in total mandible but its
condylar processes. She also revealed expansions in her mandibular angle regions
which have been suspected as Cherubism previously. Because of her failure to thrive and
pubertal delay, the patient was consulted to the Department of Orthodontics for her
misalignment. During her follow-ups, she got an infection on her left retromolar region
which was treated with antibiotherapy only and healing was uneventful. Radiographic
evaluations showed better calcification process in radiolucent areas during her treatment.
Oral and maxillofacial symptoms of NS is not discussed in the literature because of its highly
rare to encounter. However, this is the first case report that gives information and insights for
an NS patient whose prognosis is followed-up clinically and radiologically for 3 years.