Hemifacial Microsomia: Clinical Features and Associated Anomalies

Abstract

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 OMENSþ 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.

The etiology, clinical features, and treatment options of hemifacial microsomia

“…There is no discernible variation in distribution between the left and right sides of the skull, and the male‐to‐female ratio is roughly 1.09:1 (Cohen et al, 2017; Xu et al, 2015). HFM patients tend to have unilateral facial deformities, but 20% to 30% of patients have bilateral deformities (Allam, 2021).…”

Using a New Deep Learning Method for 3D Cephalometry in Patients With Hemifacial Microsomia

“…Its incidence is approximately 0.017% to 0.028% 1 . The clinical manifestations of HFM are mainly presented by the same side of the auricle, facial bone (maxilla, mandible, zygomatic, and temporal bone), and surrounding soft tissue and nerve dysplasia 2 . Hemifacial microsomia diagnosis, classification, and treatment often require auxiliary cephalometric measurements.…”

“…1 The clinical manifestations of HFM are mainly presented by the same side of the auricle, facial bone (maxilla, mandible, zygomatic, and temporal bone), and surrounding soft tissue and nerve dysplasia. 2 Hemifacial microsomia diagnosis, classification, and treatment often require auxiliary cephalometric measurements. In the traditional 2D cephalography, it is difficult to clarify the degree and classification of the HFM because of the overlap of bone shadow.…”

Discrepancy in Mandibular Medullary Cavity on Different Sides: More Hints Towards Understanding Hemifacial Microsomia

“…Its definition or essential clinical findings vary between sporadic and syndromic phenotypes, yet there has been a wide consensus on the presence of mandibular and auricular hypoplasia on at least 1 side. 1 Although there are numerous additional symptoms mentioned in literatures or the modified OMENS classification system for HFM, namely malformation of the maxilla, 2 orbits, eyes, facial soft tissues, 3 and even abnormalities of the brain, nerves, the heart, kidneys, and the spine. 4 Patients, especially those of severe HFM classified into Pruzansky-Kaban types Ⅱ and Ⅲ, have to live with asymmetrical or distorted facial appearances since their birth, suffering from consequent occlusional dysfunction and impacted social fitness.…”

“…Hemifacial microsomia (HFM) is one of the most extensive congenital craniofacial deformities. Its definition or essential clinical findings vary between sporadic and syndromic phenotypes, yet there has been a wide consensus on the presence of mandibular and auricular hypoplasia on at least 1 side 1. Although there are numerous additional symptoms mentioned in literatures or the modified OMENS classification system for HFM, namely malformation of the maxilla,2 orbits, eyes, facial soft tissues,3 and even abnormalities of the brain, nerves, the heart, kidneys, and the spine 4.…”