Sequential Loss of Mandibular Permanent Incisors in Noonan Syndrome

Abstract

Background: Noonan syndrome (NS) is a multigenic disorder with a highly variable phenotype. Cardiac disorders and a predisposition to neoplasm often require early medical attention. Central giant central lesions (CGCLs) of the jaws are part of the phenotype. Case Report: In a patient with genetically confirmed NS and multiple teeth loss presumably caused by CGCL, careful review of the medical history and radiographic findings made it probable that the cause of tooth loss was cervical root resorption (CRR) of the teeth following long-term orthodontic therapy. Conclusion: CRR is a rare dental disease of unknown origin. However, association with prior orthodontic therapy is well documented. In NS, mandibular lesions can occur which, at first glance, might lead the examiner to assume that it is a CGCL, but on closer analysis, obviously are of non-tumorous origin and should be assessed as coincidental. The report adds relevant information to orthodontic treatment of NS patients.

Clinical Findings in Children with Noonan Syndrome—A 17-Year Retrospective Study in an Oral Surgery Center

“…One of the restrictions of this retrospective study is that it presents the experience of a single research facility. However, it can be argued that, since our department is the leading oral and maxillofacial surgery provider for patients with rare diseases in the country, the participants represent a cross-section of the nationwide population and the obtained results can be comparable to those of other studies on children with NS, including those performed in multiple centers [ 10 , 18 , 19 , 30 , 31 , 32 ]. Another limitation is the small sample size, constituting only of patients who underwent surgical treatment.…”

Recurrent labial xanthoma infection in a patient with Neurofibromatosis-Noonan syndrome: case report and literature review

“…Early diagnosis of this syndrome is highly recommended, because of cardiac issues and neoplasm predisposition [7].…”

“…As a result, several clinical expressions, potentially suggestive of NS, are still not fully known. Several intraoral manifestations have been reported in NS, such as dental anomalies and malocclusion [9], high arched palate [10], distinctive philtrum, full lips [11], radiologic jaw lesion (such as central giants central lesions (CGCLs) [7]).…”