422 - Dental Management of an Arthrogryposis Multiplex Congenita Patient with Odontoma

Abstract:

Introduction:
Arthrogryposis Multiplex Congenita (AMC) is a rare congenital condition characterized by multiple joint contractures affecting the limbs, spine, and craniofacial structures. Craniofacial manifestations may include micrognathia, facial asymmetry, a high-arched palate, and limited mouth opening. Although odontomas are among the most common odontogenic tumors in children, their occurrence in AMC patients is rarely reported in the literature. 

Case Report:
A 10-year-old girl with a diagnosis of AMC presented with a chief complaint of dental caries. Her medical history included bilateral developmental dysplasia of the hips and patellar dislocation. Craniofacial assessment revealed a dolichofacial growth pattern, micrognathia, facial asymmetry, and restricted mouth opening (22 mm). Intraoral findings included enamel hypoplasia, gingival inflammation, anterior open bite, bilateral crossbite, and multiple carious lesions. Panoramic radiography and CBCT revealed a compound odontoma in the right mandible, displacing the unerupted lower right permanent canine and causing a minor perforation of the inferior mandibular border.
Under general anesthesia, the patient underwent restorative treatment along with conservative surgical enucleation of the odontoma. Postoperative physiotherapy increased the interincisal opening to 34 mm, followed by a growth modification orthodontic phase to support skeletal correction and functional improvement. Follow-up radiographs showed improved positioning of the canine and new bone deposition. 

Conclusion:
This case underscores the importance of early intervention and prevention-focused care, and highlights the essential role of coordinated multidisciplinary teams in managing complex pediatric dental conditions. To our knowledge, this may represent the first documented co-occurrence of compound odontoma in a patient with AMC.