Management of Bilateral Temporomandibular Joint Ankylosis and Class 2 Malocclusion in a Patient With Nager Syndrome

Nager syndrome is a rare hereditary syndrome characterized by severe mandibulofacial and pre-axial upper limb anomalies. Patients with Nager syndrome often exhibit syndromic Pierre-Robin sequence secondary to their hypoplastic mandible and require mandibular distraction osteogenesis to prevent dependence on a tracheostomy or gastric tube. This procedure, however, has been associated with temporomandibular joint (TMJ) ankylosis. TMJ ankylosis is a highly debilitating condition that dramatically impairs patients’ function and can result in facial asymmetry in affected children during periods of rapid facial growth. Surgical intervention is warranted in patients who fail physiotherapy; however, there is no gold standard treatment for surgically correcting TMJ ankylosis in patients with Nager syndrome. Herein, we detail the surgical management of TMJ ankylosis and class 2 malocclusion in a patient with Nager syndrome.

PROSTHETIC TREATMENT OF A PATIENT WITH NAGERACROFACIALDYSOSTOSIS: A CASE REPORT

Introduction: Nager syndrome, alsoknown as Nager acrofacialdysostosis, was first described by Nager and de Reynierin 1948. It is a rare syndrome resultingfromdevelopmentalabnormalities of the first and second branchial arches and ismostlysporadic. The prevalenceisunknown about 100 cases have been published up to now. The purpose of this report is to present a case of Nager syndrome wherewewere able to achieve a conventionalcomplete denture despite the many challenges due to the complexity of the clinical case. Case Report: A 27-years-old female patient wasreferred to our service of RemovableProsthodontics at Casablanca UniversityHospital Center complaining of difficulty in speaking and chewing. The patient presentedseveralcraniofacial anomalies including facial atrophy, maxillomandibularhypoplasia, flat nasal bridge, associated to defectsupper and lowerlimbs. Based on these craniofacial characteristics and the coexistingupper and lowerlimbpreaxial anomalies, a diagnosis of Nager syndrome wasconfirmed.The intra-oral examinationrevealed an edentulous mandibular arch and carious, mobile and hypomineralizedmaxillaryteeth, the patient presents a severemicrognatiawith a skeletal Cl III whichcomplicates the prosthetic management of the patient. Therealization of a complete denture has improved the comfort and function of the patient. Conclusion: Nager Syndrome is a condition with a ratherheavyclinical situation and its management must bemultidisciplinary, the psychology and comfort of these patients isoftenaffected. Oral care has improvedaesthetics, function and the quality of life in this patient with Nager syndrome.

Craniofacial Syndromes Part I: Craniofacial Growth and Development, Craniosynostosis Syndromes, Craniofacial Microsomia, and Craniofacial Dysostoses

Craniofacial syndromes are a diverse group of congenital disorders primarily affecting structures of the head and face. Recent genetic advances have improved our ability to diagnosis specific syndromes, understand the molecular basis for abnormal embryogenesis, and anticipate future treatment needs. This is the first of a two-part series exploring the most common craniofacial disorders. This article will provide the embryologic and developmental foundation necessary to understand congenital craniofacial pathology. Clinical characteristics and molecular genetics needed to make an accurate diagnosis and formulate a treatment plan will be detailed for craniosynostosis syndromes, craniofacial microsomia, and craniofacial dysostoses. This review contains 13 figures, 4 tables, and 42 references. Keywords: craniofacial embryology, craniofacial growth, craniosynostosis, Apert syndrome, Crouzon syndrome, Pfieffer syndrome, craniofacial microsomia, hemifacial microsomia, Treacher Collins syndrome, Nager syndrome