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

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

Affiliations
  • 1Department of Plastic and Reconstructive Surgery, Seoul National University Children’s Hospital, Seoul, Korea
  • 2Department of Orthodontics, School of Dentistry, Seoul National University, Seoul, Korea
  • 3Private Practice, Pohang, Korea
  • 4Department of Orthodontics, School of Dentistry, Chonnam National University, Gwangju, Korea
  • 5Department of Orthodontics, Dental Research Institute, School of Dentistry, Seoul National University, Seoul, Korea
  • 6Department of Plastic and Reconstructive Surgery, Seoul Metropolitan Government-Seoul National University Boramae Medical Center, Seoul, Korea
  • 7Department of Plastic and Reconstructive Surgery, College of Medicine, Seoul National University, Seoul, Korea

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%, p < 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.

Keyword

Craniofacial clefts; Distribution; Phenotype; Associated anomalies
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