Characterization of Treatment Modalities for Patients With Syndromic Craniosynostosis in Relation to Degree of Midface Hypoplasia and Patient's Age Using Longitudinal Follow-Up Data.

ABSTRACT: The purpose of this study was to investigate the type and frequency of use of treatment modalities (Tx-Mods) in patients with syndromic craniosynostosis (SC) using longitudinal follow-up data. A total of 28 patients with SC (24 Crouzon, 2 Apert, and 2 Antley-Bixler syndromes), who were treated at the Department of Orthodontics, Seoul National University Dental Hospital, Seoul, South Korea between 1998 and 2020, was included. According to the degree of midface hypoplasia (MH) at the initial visit (T1), the patients were divided into the mild-MH (78°≤SNA < 80°, n = 8), moderate-MH (76≤SNA < 78°, n = 7), and severe-MH (SNA < 76°, n = 13) groups. T1-age and Tx-Mods, including cal-varial surgery (CALS), orthopedic treatment (OPT), fixed orthodontic treatment, and midface advancement surgery in childhood (MAS-child) and adulthood (MAS-adult), were investigated. Complexity of MAS-adult was graded as follows: 0, no surgery; 1, orthognathic surgery; 2, distraction osteogenesis (DOG); 3, combination of distraction osteogenesis and orthognathic surgery. Then, statistical analysis was performed. Percentage distribution of Tx-Mods was 71.4% in CALS, 21.4% in MAS-child, 42.9% in OPT, 100% in fixed orthodontic treatment, and 89.3% in MAS-adult. 92.9% of patients underwent MAS more than once. The number of MAS increased according to the severity of MH ( P < 0.05). The complexity of MAS-adult increased as T1-age and severity of MH increased (all P < 0.05); whereas it decreased when CALS and OPT were performed (all P < 0.05). However, MAS in childhood did not guarantee the avoidance of additional MAS in adulthood ( P > 0.05). These findings may be used as basic guidelines for successful treatment planning and prognosis prediction in patients with SC.

Characterization of Phenotypes and Treatment Modalities in Patients With Treacher-Collins Syndrome.

ABSTRACT: The purpose of this study was to investigate the phenotypes and treatment modalities (Tx-Mod) in patients with Treacher-Collins syndrome (TCS) using a TCS severity index (TSI). The sample consisted of 14 Korean TCS patients treated at the Department of Orthodontics, Seoul National University Dental Hospital during 1998 to 2019. The TSI was calculated by adding the scores from the number of deformity-involved midface structures (eye, ear, zygoma) and the degree of mandibular hypoplasia (Pruzansky-Kaban type, gonial angle, Sella-Nasion-B point angle). The deformity-involved midface structure, degree of mandibular hypoplasia, oral manifestations, dental phenotypes, and Tx-Mod types were investigated using descriptive statistics. The TSI classified the subjects into 2 mild, 6 moderate, and 6 severe cases. The severity of ear and zygoma deformities, degree of condylar hypoplasia (Pruzansky-Kaban type), clockwise-rotated morphology (gonial angle) and retrusive position (Sella-Nasion-B point angle) of the mandible, and frequency of oral/craniofacial cleft, openbite, congenitally missing tooth and impacted tooth showed a tendency of increase from mild to severe TCS cases. After growth observation (78.6%), diverse combinations of Tx-Mods were applied except for functional appliance therapy. Surgical procedures for eye, ear, and zygoma reconstruction were performed on all patients (100%), whereas fixed orthodontic treatment, mandibular distraction osteogenesis, and orthognathic surgery were performed on 50% of patients. Surgical procedure for hearing improvement was the most frequent Tx-Mod (78.6%). The main desires of TCS patients were to obtain the facial esthetics in the midface and to improve hearing function. The TSI might provide a primary data for individualized diagnosis and treatment planning.

Characterization of phenotypes and predominant skeletodental patterns in pre-adolescent patients with Pierre-Robin sequence.

OBJECTIVE: To investigate the phenotypes and predominant skeletodental pattern in pre-adolescent patients with Pierre-Robin sequence (PRS). METHODS: The samples consisted of 26 Korean pre-adolescent PRS patients (11 boys and 15 girls; mean age at the investigation, 9.20 years) treated at the Department of Orthodontics, Seoul National University Dental Hospital between 1998 and 2019. Dental phenotypes, oral manifestation, cephalometric variables, and associated anomalies were investigated and statistically analyzed. RESULTS: Congenitally missing teeth (CMT) were found in 34.6% of the patients (n = 9/26, 20 teeth, 2.22 teeth per patient) with 55.5% (n = 5/9) exhibiting bilaterally symmetric missing pattern. The mandibular incisors were the most common CMT (n = 11/20). Predominant skeletodental patterns included Class II relationship (57.7%), posteriorly positioned maxilla (76.9%) and mandible (92.3%), hyper-divergent pattern (92.3%), high gonial angle (65.4%), small mandibular body length to anterior cranial base ratio (65.4%), linguoversion of the maxillary incisors (76.9%), and linguoversion of the mandibular incisors (80.8%). Incomplete cleft palate (CP) of hard palate with complete CP of soft palate (61.5%) was the most frequently observed, followed by complete CP of hard and soft palate (19.2%) and CP of soft palate (19.2%) (p < 0.05). However, CP severity did not show a significant correlation with any cephalometric variables except incisor mandibular plane angle (p < 0.05). Five craniofacial and 15 extra-craniofacial anomalies were observed (53.8% patients); this implicated the need of routine screening. CONCLUSIONS: The results might provide primary data for individualized diagnosis and treatment planning for pre-adolescent PRS patients despite a single institution-based data.