Orthodontic treatment protocol of Ehlers-Danlos syndrome type VI. 2006

Tulin Arun, and Didem Nalbantgil, and Korkmaz Sayinsu
Department of Orthodontics, Yeditepe University Dental School, Goztepe, Istanbul, Turkey.

Ehlers-Danlos syndrome (EDS) type VI is an autosomal recessive disorder of the connective tissue characterized by joint hypermobility, muscle hypotonia, scoliosis, and ocular fragility. In this case report, an EDS type VI patient with a skeletal and dental Class III malocclusion is presented and the clinical approach to his orthodontic problem is emphasized. A 17-year-old male patient presenting some major and minor symptoms of the syndrome was referred to our orthodontic department for diagnosis and treatment. The typical clinical signs confirmed the diagnosis of EDS type VI. He was a skeletal and dental Class III malocclusion patient (both mandibular protrusion and maxillary retrusion) with a noncontributory family history. He had severe crowding in the lower and upper dental arches with retruded incisors. His first treatment plan included orthognathic surgery, but because of the risks of bleeding and poor healing, we elected to treat the patient without surgery.

UI MeSH Term Description Entries
D008297 Male Males
D008313 Malocclusion, Angle Class III Malocclusion in which the mandible is anterior to the maxilla as reflected by the first relationship of the first permanent molar (mesioclusion). Angle Class III,Habsburg Jaw,Hapsburg Jaw,Prognathism, Mandibular,Underbite
D010155 Palatal Expansion Technique An orthodontic method used for correcting narrow or collapsed maxillary arches and functional cross-bite. (From Jablonski's Dictionary of Dentistry), Maxillary Expansion,Palatal Expansion Technic,Expansion, Maxillary,Palatal Expansion Technics,Palatal Expansion Techniques,Technic, Palatal Expansion,Technique, Palatal Expansion
D002508 Cephalometry The measurement of the dimensions of the HEAD. Craniometry
D004535 Ehlers-Danlos Syndrome A heterogeneous group of autosomally inherited COLLAGEN DISEASES caused by defects in the synthesis or structure of FIBRILLAR COLLAGEN. There are numerous subtypes: classical, hypermobility, vascular, and others. Common clinical features include hyperextensible skin and joints, skin fragility and reduced wound healing capability. Cutis Elastica,Ehlers Danlos Disease,Ehlers-Danlos Disease,Danlos Disease, Ehlers,Disease, Ehlers Danlos,Disease, Ehlers-Danlos,Ehlers Danlos Syndrome,Syndrome, Ehlers-Danlos
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D014096 Tooth, Supernumerary An extra tooth, erupted or unerupted, resembling or unlike the other teeth in the group to which it belongs. Its presence may cause malposition of adjacent teeth or prevent their eruption. Molar, Fourth,Teeth, Supernumerary,Fourth Molar,Fourth Molars,Molars, Fourth,Supernumerary Teeth,Supernumerary Tooth
D014097 Tooth, Unerupted A normal developing tooth which has not yet perforated the oral mucosa or one that fails to erupt in the normal sequence or time interval expected for the type of tooth in a given gender, age, or population group. Teeth, Unerupted,Unerupted Teeth,Unerupted Tooth

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