Korean J Orthod.  2020 Jan;50(1):33-41. 10.4041/kjod.2020.50.1.33.

Distribution and phenotypes of hemifacial microsomia and its association with other anomalies

• Yang IH
• Chung JH
• Yim S
• Cho IS
• Lim SW
• Kim K
• Kim S
• Choi JY
• Lee JH
• Kim MJ
• Baek SH

• KMID: 2468639
• DOI: http://doi.org/10.4041/kjod.2020.50.1.33

Abstract

OBJECTIVE
To investigate the distribution and phenotypes of hemifacial microsomia (HFM) and its association with other anomalies.
METHODS
This study included 249 Korean patients with HFM, whose charts, photographs, radiographs, and/or computed tomography scans acquired during 1998-2018 were available from Seoul National University Hospital and Dental Hospital. Prevalence according to sex, side involvement, degree of mandibular deformity, compensatory growth of the mandibular body, and Angle's classification, and its association with other anomalies were statistically analyzed.
RESULTS
Prevalence was not different between male and female patients (55.0% vs. 45.0%, p > 0.05). Unilateral HFM (UHFM) was more prevalent than bilateral HFM (BHFM) (86.3% vs. 13.7%, p < 0.001). Although distribution of the Pruzansky-Kaban types differed significantly in patients with UHFM (I, 53.0%; IIa, 18.6%; IIb, 24.7%; III, 3.7%; p < 0.001), no difference was observed in occurrence between the right and left sides (52.6% vs. 47.4%, p > 0.05). Among patients with BHFM, prevalence of different Pruzansky-Kaban types on the right and left sides was greater than that of the same type on both sides (67.6% vs. 32.4%, p < 0.05). Despite hypoplasia of the condyle/ramus complex, compensatory growth of the mandibular body on the ipsilateral side occurred in 35 patients (14.1%). Class I and II molar relationships were more prevalent than Class III molar relationships (93.2% vs. 6.8%, p < 0.001). Forty-eight patients (19.3%) had other anomalies, with 50.0% and 14.4% in the BHFM and UHFM groups (p < 0.001).
CONCLUSIONS
Patients with HFM require individualized diagnosis and treatment planning because of diverse phenotypes and associations with other anomalies.

Keyword

Distribution; Phenotype; Hemifacial macrosomia; Association with other anomalies

MeSH Terms

Congenital Abnormalities
Diagnosis
Female
Goldenhar Syndrome*
Humans
Male
Malocclusion
Molar
Phenotype*
Prevalence
Seoul

Figure

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  Figure 1 Distribution of sex and side involvement in patients with hemifacial microsomia (HFM). The chi-square goodness-of-fit test was performed (***p < 0.001).

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