![]() In the presence of an epibulbar dermoid, vertebral anomalies, and abnormalities beyond the craniofacial region, the term expanded spectrum HFM is the current appropriate term. 1, 2 In patients with bilateral HFM, the deformity is always asymmetrical. ![]() Up to 20% of individuals with HFM have bilateral malformations (craniofacial microsomia) when assessed clinically. If the deformity is bilateral or involves the orbit, the term craniofacial microsomia is sometimes synonymously used. As the child develops and the face matures, the skeletal deformity progresses and becomes more obvious. 1, 2 It varies in phenotypic severity along a spectrum from mild to severe. After cleft lip and cleft palate, HFM is the most common craniofacial malformation. Hemifacial microsomia (HFM) is a congenital facial deformity involving the structures of the first and second pharyngeal arches: maxilla, mandible, external and middle ear, facial and trigeminal nerves, muscles of mastication, and overlying soft tissue. Isolated microtia and HFM could represent a spectrum of expression of the same developmental phenomenon. Thirty-seven patients demonstrated conductive hearing loss, and 1 had sensorineural hearing loss.Ĭonclusions Isolated microtia served as an early clinical marker for asymmetrical facial growth in 40% of the patients. Thirty patients had cranial nerve deficits, and 37 had mandibular asymmetry. The OMENS scores were less than 5 in 24 patients and 6 to 10 in 16 patients. Mean patient age was 9.2 years (range, 6 weeks to 41 years), with male predominance (27 males and 13 females). Results Forty patients (40%) with microtia were determined to have HFM (31 unilateral and 9 bilateral). Main Outcome Measures The OMENS scores, percentage of patients with isolated microtia and undiagnosed HFM, and isolated microtia as an early clinical marker for HFM. Each anatomical abnormality was graded from 0 (normal) to 3 (most severe) (score range, 0-15). The OMENS classification system was used to grade the severity of craniofacial features: orbital deformity, mandibular hypoplasia, ear deformity, nerve (cranial nerve VII) involvement, and soft-tissue deficiency. Interventions All the patients underwent a clinical examination and audiologic evaluation. Patients One hundred consecutive patients with isolated microtia. Setting University-affiliated, tertiary referral clinic for patients with microtia. Objective To determine the proportion of hemifacial microsomia (HFM) in patients with unilateral or bilateral “isolated” microtia.ĭesign Prospective cohort clinical study. Shared Decision Making and Communication.Scientific Discovery and the Future of Medicine.Health Care Economics, Insurance, Payment.Clinical Implications of Basic Neuroscience.Challenges in Clinical Electrocardiography.
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