Dental and Skeletal Characteristics and Behavioral Aspects of the Patient with Floating-Harbor Syndrome Compared with Twin Sister

J. Lim, G. Kim, Jaesik Lee, Soonhyeun Nam, Hyunjung Kim
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Abstract

Floating-Harbor syndrome (FHS) is a rare genetic disorder. This report introduced in a patient with FHS. Distinctive facial characteristics, severe skeletal class 3 malocclusion with underdeveloped maxilla and protruded mandible, congenital missing teeth, microdontia and ectopic positions of maxillary teeth were presented in the patient. In his twin sister, mild skeletal class 3 malocclusion with protruded mandible was observed but congenital missing teeth and microdontia were not observed. High-arched palate, narrow V-shaped maxillary arch compared to wide and ovoid mandibular arch and inverse relationship between the maxillary and mandibular intermolar width resulted in posterior crossbite were confirmed by model analysis of the patient. These were not observed in the twins. Behaviorally, poor cooperation during dental treatment because of mental retardation was observed in the patient.
浮港综合征患者与孪生姐妹的牙骨特征及行为特征比较
浮港综合征(FHS)是一种罕见的遗传性疾病。本报告介绍了一例FHS患者。患者面部特征明显,骨骼3级错颌畸形严重,上颌发育不全,下颌骨突出,先天性缺牙,小牙畸形,上颌牙位异位。在他的孪生姐妹中,观察到轻度骨骼3级错颌,下颌骨突出,但未观察到先天性缺牙和小齿。通过对患者的模型分析,证实了上颌高弓、窄v型上颌弓相对于宽卵形下颌骨弓以及上颌与下颌磨牙间宽度成反比关系导致后牙合的发生。这些在双胞胎中没有观察到。在行为上,由于精神发育迟滞,患者在牙科治疗过程中配合不良。
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