Noonan Syndrome and 3 Years Follow-Up


Ünsalan A. E. , Yücesoy T. , Kılıç B. , Kılıç E.

AÇBİD 13th International Congress-2019, Antalya, Turkey, 26 April 2019, pp.67

  • Publication Type: Conference Paper / Summary Text
  • City: Antalya
  • Country: Turkey
  • Page Numbers: pp.67

Abstract

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.