Surgery first approach for dentofacial deformity correction of a patient with achondroplasia: a case report

Background: Achondroplasia is a characteristically short physical appearance. Of particular interest to this paper is the craniofacial features. Craniofacial features include a hypoplastic midface, enlarged calvaria, frontal bossing, flattened nasal bridge, mandibular prognathism (potentially masking micrognathia) and dental malocclusion including an anterior open bite. Case Description: A 19-year-old male with achondroplasia presented to the University of Alabama Birmingham School of Dentistry with the chief complaint that “he needed to eat and chew better”. The patient said that in addition to his difficulty incising and chewing foods, he also had speech problems related to jaw position. Clinical examination revealed concave facial profile, hypoplastic midfacial soft tissue, adequate distance between the throat and chin. His malocclusion was complicated by a significant anterior crossbite with −9 mm overjet, left side posterior crossbite, and significant deep overbite. The maxillary and mandibular dental midline was coincident with the facial midline. There was no popping, clicking, or crepitation of the temporomandibular joint. The final treatment plan decided was a surgery first approach (SFA) to rehabilitate the patient. This plan included non-extraction treatment, followed by a Le Fort 1 maxillary osteotomy to advance the maxilla, bilateral sagittal split setback with intermaxillary fixation screws. Post-surgical orthodontic treatment with the use of conventional orthodontic brackets was also planned to correct inter-arch discrepancies after the surgery. Conclusions: While SFA has been documented for correction of severe dentofacial deformities including cleft lip and palate, severe malocclusions, and hemifacial microsomia, this case report represents the first time a patient with achondroplasia has had a successful SFA to correct his dentofacial deformity and occlusion. This technique represents a breakthrough in the management of patients with significant facial deformities and the effective use of precious financial and clinical resources.