title: A cephalometric analysis of patients with recessive dystrophic epidermolysis bullosa.
abstract: Patients diagnosed with Recessive Dystrophic Epidermolysis Bullosa (RDEB) suffer from severe growth inhibition due to reduced food intake as a result of severe oropharyngeal and esophageal blistering. This investigation examined the patterns of facial growth in a group of 42 children with RDEB for whom lateral skull radiographs were available. The differences between RDEB patients and patients with normal cephalometric values were also assessed. Lateral skull radiographs were digitized and a number of orthodontic indices were compared to the published normal values. The RDEB patients examined demonstrated smaller maxillae than normal (length 41.3 +/- 2.9 mm compared to 47.4 +/- 2.5 mm) and smaller mandibles than normal (length 82.3 +/- 6.1 mm compared to 93.1 +/- 4.2 mm). This impaired growth may result from reduced food intake or severe orofacial scarring associated with RDEB. This contributes significantly to dento-alveolar disproportion and dental crowding and puts patients at increased risk of dental caries.
authors.affiliation: Department of Paediatric Dentistry, Eastman Dental Institute for Oral Health Care Sciences, University College London, UK.
authors.forename: Hemal
authors.initials: H
authors.lastname: Shah
authors.forename: Fraser
authors.initials: F
authors.lastname: McDonald
authors.forename: Victoria
authors.initials: V
authors.lastname: Lucas
authors.forename: Paul
authors.initials: P
authors.lastname: Ashley
authors.forename: Graham
authors.initials: G
authors.lastname: Roberts
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002508:Cephalometry; D002648:Child; D002675:Child, Preschool; D002921:Cicatrix / Q000150:complications / Q000209:etiology; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications* / Q000503:physiopathology*; D005260:Female; D006130:Growth Disorders / Q000209:etiology*; D006801:Humans; D008297:Male; D008445:Maxillofacial Development; D015588:Observer Variation; D011502:Protein-Energy Malnutrition / Q000150:complications* / Q000209:etiology*; D018709:Statistics, Nonparametric; D014313:Trismus / Q000150:complications / Q000209:etiology