title: Shape of the craniofacial complex in children with Turner syndrome.
abstract: The shape of the craniofacial complex was established in 69 children with Turner syndrome aged between 3.5 and 16.6 years. The children had not been treated with growth hormone (GH) or anabolic steroids. On a standardized lateral roentgenencephalogram 13 linear and 7 angular variables were measured. Data of all variables were available from normal Dutch children for comparison. The main abnormalities were located in the cranial base and in the mandible and consisted of a short posterior cranial base, all increased cranial base angle and a short, retrognathic and posteriorly rotated mandible. The maxilla was smaller than normal and also slightly posteriorly rotated. The abnormalities were already present in young children with Turner syndrome. Indications were found that in Turner syndrome interstitially as well as appositionally growing cartilage is affected. The changes in the maxilla can be explained in various ways. They may be due to defective growth of the nasal cartilage or to a disorder in the intramembranous ossification of the maxilla or they may be adaptive to the changes in the cranial base and the mandible. From this study it can be concluded that patients with Turner syndrome exhibit several craniofacial abnormalities, probably due to a cartilage disorder.
authors.affiliation: Department of Paediatrics, University Hospital Nijmegen, The Netherlands.
authors.forename: C
authors.initials: C
authors.lastname: Rongen-Westerlaken
authors.forename: E
authors.initials: E
authors.lastname: vd Born
authors.forename: B
authors.initials: B
authors.lastname: Prahl-Andersen
authors.forename: B
authors.initials: B
authors.lastname: Rikken
authors.forename: V
authors.initials: V
authors.lastname: Teunenbroek
authors.forename: N
authors.initials: N
authors.lastname: Kamminga
authors.forename: I
authors.initials: I
authors.lastname: vd Tweel
authors.forename: B J
authors.initials: BJ
authors.lastname: Otten
authors.forename: H A
authors.initials: HA
authors.lastname: Delamarre vd Waal
authors.forename: N M
authors.initials: NM
authors.lastname: Drayer
mesh_terms: D000293:Adolescent; D000365:Age Determination by Skeleton; D002508:Cephalometry; D002648:Child; D002675:Child, Preschool; D005147:Facial Bones / Q000473:pathology*; D005260:Female; D006801:Humans; D008334:Mandible / Q000473:pathology; D008437:Maxilla / Q000473:pathology; D008445:Maxillofacial Development; D012886:Skull / Q000473:pathology*; D014424:Turner Syndrome / Q000235:genetics / Q000473:pathology*; D014960:X Chromosome