Department of Plastic Surgery, Sohag University, Sohag, Egypt.
J Craniofac Surg. 2021 Jun 1;32(4):1483-1486. doi: 10.1097/SCS.0000000000007408.
Hemifacial microsomia (HFM) is the second most common congenital anomaly of the face after cleft lip and palate. There is a considerable variability in the extent and severity of affection of the bones and soft tissues of the face arising from these two arches. Different classification systems have been described for HFM with the mandible and/or the ear defects being key features needed for any classification system. Nowadays, the most widely used system is the orbital, mandibular, ear, facal nerve, soft tissue (OMENS) classification, later modified to the OMENS+ to include extracraniofacial manifestations.The aim of this study was to review the demographic characteristics and clinical findings of a group of Egyptian patients with HFM and to correlate the findings with data retrieved from the literature.Thirty-nine patients were identified and included in this study. Right side was predominantly affected in 19 patients (48.7%), 9 patients (23%) had left side affection (23%) and 11 patients (28.3%) had bilateral affection, with overall male-to-female ratio of 1:1.4. The majority of patients had a normal orbit (64%), mildly hypoplastic mandible with functioning temporomandibular joint (58% with type M1 or M2a), normal facial nerve (82%), and minimal to mild soft-tissue hypoplasia (52%). Significant ear deformity affected the bigger percentage of the patients (E3: 44%, E2: 14%)The data demonstrate the phenotypic variability of HFM and suggest a degree of relationship among the components of HFM. Some epidemiological aspects and clinical findings in our study did not always parallel those reported in western literatures. Small sample size is the main limitation of the study. Further, and if possible multi-center, studies are recommended.
半侧颜面短小症(HFM)是继唇腭裂之后的第二种最常见的面部先天畸形。这两种弓在骨骼和面部软组织的受累程度和严重程度上存在相当大的差异。HFM 已经有多种分类系统描述,其中下颌骨和/或耳朵缺陷是任何分类系统都需要的关键特征。如今,最广泛使用的系统是眶、下颌、耳、面神经、软组织(OMENS)分类,后来修改为 OMENS+以包括颅外表现。本研究的目的是回顾一组埃及 HFM 患者的人口统计学特征和临床发现,并将这些发现与从文献中检索到的数据进行相关性分析。
确定了 39 名患者并将其纳入本研究。19 名患者(48.7%)受右侧影响,9 名患者(23%)受左侧影响(23%),11 名患者(28.3%)双侧受累,男女比例为 1:1.4。大多数患者的眼眶正常(64%),下颌骨轻度发育不良伴功能正常的颞下颌关节(58%为 M1 或 M2a 型),面神经正常(82%),软组织轻度至中度发育不良(52%)。显著的耳部畸形影响了较大比例的患者(E3:44%,E2:14%)。
数据显示了 HFM 的表型变异性,并提示 HFM 各成分之间存在一定的关系。我们的研究中的一些流行病学方面和临床发现并不总是与西方文献报道的一致。样本量小是该研究的主要局限性。如果可能的话,建议进行进一步的、多中心研究。