Orthodontic and surgical management of cleidocranial dysplasia
A 12-year-old Asian boy diagnosed with CCD presented with his father, who had the same condition. The patient's chief complaint was described by his father as "anting normal eruption of his permanent teeth and better alignment of his upper and lower arches." His medical and surgical histories included recurrent otitis media with tubes placed in the tympanic membranes and bilateral femoral osteotomies to correct coxa vara. The boy showed the classical craniofacial features of CCD: broad forehead, hypertelorism, and short upper third of the face (). The initial extraoral examination revealed a retrognathic and vertically deficient maxilla (), resulting in a concave facial profile.\nThe initial intraoral examination revealed a skeletal class III pattern with 9-mm anterior crossbite and 3-mm open bite, moderate-to-severe maxillary crowding, and mild mandibular crowding (). The panoramic radiograph showed nine retained primary teeth, seven supernumerary teeth, four impacted permanent teeth, and no congenitally missing permanent teeth (). The initial lateral cephalogram confirmed the skeletal class III relationship due to maxillary underdevelopment ().\nThe treatment involved five steps and was expected to continue until skeletal maturity, when the underdeveloped maxilla was to be surgically corrected. The first step was to treat multiple primary and newly erupted permanent teeth for caries due to poor oral hygiene.\nAt age 14, nine retained primary teeth (i.e., 53, 62, 63, 74, 73, 72, 82, 83, and 84) were extracted and seven supernumerary teeth were surgically removed under general anesthesia (). All the permanent canines were also removed, because they were determined to be nonretrievable due to severe impaction. The permanent teeth were observed for possible spontaneous eruption over 9 months.\nThe mandibular lateral incisors and maxillary right lateral incisor failed to erupt spontaneously. These teeth were surgically exposed and bonded with brackets and gold chains tied to maxillary and mandibular lingual arches for guided eruption (). Simultaneously, a mesiodens and the maxillary right primary second molar were extracted. Shortly thereafter, full maxillary and mandibular fixed orthodontic appliances were placed for alignment and coordination of the dental arches (). The maxillary right lateral incisor was repositioned in the canine space ().\nTo correct the transverse maxillary discrepancy, a transpalatal arch was used. At skeletal maturity (age 19), the maxilla was advanced and downfractured to correct 6 mm of the sagittal maxillary discrepancy and vertical deficiency (). The postsurgical healing was good with excellent patient compliance and the orthodontic treatment was completed ().\nThe final step was implant placement and prosthodontic rehabilitation. Implants were placed for the maxillary right lateral incisor and left canine and mandibular canines (). The mandibular right canine-premolar space required two separate implants. Because the second molars were the last teeth to develop and the patient had been in treatment for a long time, these teeth were not retrieved. Treatment was completed with the delivery of the final crowns for the implants at age 24.\nThe final extraoral and intraoral photographs are shown in . Fifty-three hard-tissue and soft-tissue landmarks were digitized by an orthodontist using Dolphin software (version 11.5; Dolphin Imaging and Management Solutions, Chatsworth, CA, USA). The pretreatment and posttreatment lateral cephalograms were superimposed to illustrate treatment-induced changes (). Comparison of the ANB, overbite, and overjet values between these time points () showed a dramatic improvement in the sagittal jaw relationship after the maxillary advancement surgery and orthodontic treatment.