title: Oro-palatal dysplasia Bettex-Graf--clinical findings, genetic background, treatment.
abstract: Oro-palatal dysplasia Bettex-Graf is an extremely rare syndrome consisting of microstomia, U-shaped cleft palate and micrognathia. Only two affected families have been reported before. We present the clinical findings, treatment and 13 year follow-up in a patient with this rare syndrome. The possible linkage to the fragile site 16q22 has been supported, contrary to earlier statements of its non-pathogenic character. The analysis of clinical symptoms and reference to the literature suggests, that ankyloglossia is a part of oropalatal dysplasia, whereas hypodontia is associated with the cleft itself. The authors postulate that a 20mm intercommissural distance allows acceptable function without the need for surgical correction.
authors: Department of General Dentistry, Pomeranian Medical University, Szczecin, Poland. jjo@sci.pam.szczecin.pl
authors: Joanna
authors: J
authors: Janiszewska-Olszowska
authors: Elżbieta
authors: E
authors: Gawrych
authors: Aldona
authors: A
authors: Dydyk
authors: Ewa
authors: E
authors: Studniak
authors: Anna
authors: A
authors: Biaduń-Popławska
authors: Stanisław
authors: S
authors: Zajączek
mesh_terms: D000848:Anodontia / Q000473:pathology; D002508:Cephalometry / Q000379:methods; D002872:Chromosome Deletion; D025063:Chromosome Disorders / Q000235:genetics; D002885:Chromosomes, Human, Pair 16 / Q000235:genetics; D002972:Cleft Palate / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D019465:Craniofacial Abnormalities / Q000235:genetics; D002658:Developmental Disabilities / Q000235:genetics; D005500:Follow-Up Studies; D008040:Genetic Linkage / Q000235:genetics; D006330:Heart Defects, Congenital / Q000235:genetics; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008310:Malocclusion / Q000628:therapy; D001523:Mental Disorders / Q000235:genetics; D008844:Micrognathism / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D008865:Microstomia / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D009061:Mouth Mucosa / Q000637:transplantation; D009297:Nasal Mucosa / Q000601:surgery; D010156:Palatal Muscles / Q000601:surgery; D010521:Periosteum / Q000637:transplantation; D013524:Surgical Flaps / Q000637:transplantation; D014059:Tongue / Q000002:abnormalities