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

Korean Journal of Orthodontics 2021³â 51±Ç 5È£ p.337 ~ 345
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¾çÀÏÇü ( Yang Il-Hyung ) - Seoul National University School of Dentistry Department of Orthodontics
Á¤ÁöÇõ ( Chung Jee-Hyeok ) - Seoul National University Children¡¯s Hospital Department of Plastic and Reconstructive Surgery
ÀÌÇõÁØ ( Lee Hyeok-Joon ) - Seoul National University School of Dentistry Department of Orthodontics
Á¶ÀϽĠ( Cho Il-Sik ) - Private practice
ÃÖÁø¿µ ( Choi Jin-Young ) - Seoul National University School of Dentistry Department of Oral and Maxillofacial Surgery
ÀÌÁ¾È£ ( Lee Jong-Ho ) - Seoul National University School of Dentistry Department of Oral and Maxillofacial Surgery
±è¼®È­ ( Kim Suk-Wha ) - Seoul National University College of Medicine Department of Plastic and Reconstructive Surgery
¹é½ÂÇР( Baek Seung-Hak ) - Seoul National University School of Dentistry Department of Orthodontics

Abstract


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.

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Pierre?Robin sequence; Phenotype; Skeletodental pattern

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SCI(E)
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KoreaMed