[Maxillofacial abnormalities of syndromes of the 1st branchial arch]. 1987

J F Tulasne, and Y Manach, and C Hamann
Service O.R.L., Hôpital Necker Enfants Malades, Paris.

Hemifacial microsomia and Treacher Collins syndrome are the most frequent among the first and second branchial arch syndromes to include anomalies of the facial skeleton. Hemifacial microsomia is almost always unilateral and is characterised by hypoplasia or agenesis of the mandibular ramus. Treacher Collins syndrome is always bilateral and involves specifically the zygomatic bones. The treatment of skeletal anomalies is seldom started before 10-12 years of age, except in severe form with major aesthetic impairment. Such cases are treated at the beginning of school age, on and after 6-8 years. Several operations, including osteotomies of the jaws and bone grafts, are usually necessary to achieve an acceptable result.

UI MeSH Term Description Entries
D007569 Jaw Abnormalities Congenital absence of or defects in structures of the jaw. Abnormalities, Jaw,Abnormality, Jaw,Jaw Abnormality
D010027 Osteotomy The surgical cutting of a bone. (Dorland, 28th ed) Osteotomies
D001934 Branchial Region A region, of SOMITE development period, that contains a number of paired arches, each with a mesodermal core lined by ectoderm and endoderm on the two sides. In lower aquatic vertebrates, branchial arches develop into GILLS. In higher vertebrates, the arches forms outpouchings and develop into structures of the head and neck. Separating the arches are the branchial clefts or grooves. Branchial Arches,Branchial Clefts,Pharyngeal Arches,Visceral Arches,Branchial Arch,Branchial Grooves,Pharyngeal Arch,Pharyngeal Clefts,Pharyngeal Grooves,Visceral Arch,Arch, Branchial,Arch, Pharyngeal,Arch, Visceral,Arches, Branchial,Arches, Pharyngeal,Arches, Visceral,Archs, Pharyngeal,Branchial Cleft,Branchial Groove,Branchial Regions,Cleft, Branchial,Cleft, Pharyngeal,Clefts, Branchial,Clefts, Pharyngeal,Groove, Branchial,Groove, Pharyngeal,Grooves, Branchial,Grooves, Pharyngeal,Pharyngeal Archs,Pharyngeal Cleft,Pharyngeal Groove,Region, Branchial,Regions, Branchial
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes
D015050 Zygoma Either of a pair of bones that form the prominent part of the CHEEK and contribute to the ORBIT on each side of the SKULL. Cheek Bone,Jugal Bone,Malar Bone,Zygomatic Arch,Arches, Zygomatic,Zygomatic Arches,Arch, Zygomatic,Bone, Cheek,Bone, Jugal,Bone, Malar,Bones, Cheek,Bones, Jugal,Bones, Malar,Cheek Bones,Jugal Bones,Malar Bones,Zygomas
D016025 Bone Transplantation The grafting of bone from a donor site to a recipient site. Grafting, Bone,Transplantation, Bone,Bone Grafting

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