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基于1998年至2018年期间从单一大学医院获取的数据,韩国颅面裂患者的分布、病变累及情况、表型及相关异常

Distribution, side involvement, phenotype and associated anomalies of Korean patients with craniofacial clefts from single university hospitalbased data obtained during 1998-2018.

作者信息

Chung Jee Hyeok, Yim Sunjin, Cho Il-Sik, Lim Seung-Weon, Yang Il-Hyung, Ha Jeong Hyun, Kim Sukwha, Baek Seung-Hak

机构信息

Department of Plastic and Reconstructive Surgery, Seoul National University Children's Hospital, Seoul, Korea.

Department of Orthodontics, School of Dentistry, Seoul National University, Seoul, Korea.

出版信息

Korean J Orthod. 2020 Nov 25;50(6):383-390. doi: 10.4041/kjod.2020.50.6.383.

Abstract

OBJECTIVE

To investigate the distribution, side involvement, phenotype, and associated anomalies of Korean patients with craniofacial clefts (CFC).

METHODS

The samples consisted of 38 CFC patients, who were treated at Seoul National University Dental Hospital during 1998-2018. The Tessier cleft type, sex, side involvement, phenotype, and associated anomalies were investigated using nonparametric statistical analysis.

RESULTS

The three most common types were #7 cleft, followed by #0 cleft and #14 cleft. There was no difference between the frequency of male and female. Patients with #0 cleft exhibited nasal deformity, bony defect, and missing teeth in the premaxilla, midline cleft lip, and eye problems. A patient with #3 cleft (unilateral type) exhibited bilateral cleft lip and alveolus. All patients with #4 cleft were the bilateral type, including a combination of #3 and #4 clefts, and had multiple missing teeth. A patient with #5 cleft (unilateral type) had a posterior openbite. In patients with #7 cleft, the unilateral type was more prevalent than the bilateral type (87.0% vs. 13.0%, p < 0.001). Sixteen patients showed hemifacial microsomia (HFM), Goldenhar syndrome, and unilateral cleft lip and palate (UCLP). There was a significant match in the side involvement of #7 cleft and HFM (87.5%, < 0.01). Patients with #14 cleft had plagiocephaly, UCLP, or hyperterorbitism. A patient with #30 cleft exhibited tongue tie and missing tooth.

CONCLUSIONS

Due to the diverse associated craniofacial anomalies in patients with CFC, a multidisciplinary approach involving a well-experienced cooperative team is mandatory for these patients.

摘要

目的

研究韩国颅面裂(CFC)患者的分布、患侧、表型及相关畸形情况。

方法

样本包括1998年至2018年期间在首尔国立大学牙科学院接受治疗的38例CFC患者。采用非参数统计分析方法对Tessier裂型、性别、患侧、表型及相关畸形进行研究。

结果

最常见的三种类型依次为7号裂,其次是0号裂和14号裂。男女发病率无差异。0号裂患者表现为鼻畸形、骨缺损、上颌前部牙齿缺失、正中唇裂和眼部问题。1例3号裂(单侧型)患者表现为双侧唇裂和牙槽突裂。所有4号裂患者均为双侧型,包括3号裂和4号裂的组合,且有多颗牙齿缺失。1例5号裂(单侧型)患者有后牙开 。7号裂患者中,单侧型比双侧型更常见(87.0%对13.0%,p<0.001)。16例患者表现为半侧颜面短小畸形(HFM)、Goldenhar综合征和单侧唇腭裂(UCLP)。7号裂与HFM的患侧有显著匹配(87.5%,<0.01)。14号裂患者有斜头畸形、UCLP或眶距增宽。1例30号裂患者表现为舌系带过短和牙齿缺失。

结论

由于CFC患者存在多种相关的颅面畸形,对于这些患者,由经验丰富的合作团队采用多学科方法是必不可少的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e50f/7642224/2428dd9a3df8/KJOD-50-383-f1.jpg

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