• Document: Orthodontic Treatment Protocol of Ehlers-Danlos Syndrome Type VI
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Case Report Orthodontic Treatment Protocol of Ehlers-Danlos Syndrome Type VI Tulin Aruna; Didem Nalbantgilb; Korkmaz Sayınsuc Abstract: 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 di- agnosis of EDS type VI. He was a skeletal and dental Class III malocclusion patient (both man- dibular 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. (Angle Orthod 2006;76:177–183.) Key Words: Ehlers-Danlos syndrome; Connective tissue disorder; Missing frenula; Class III malocclusion INTRODUCTION creasing clinical experience. Some forms are uncom- Ehlers-Danlos syndrome (EDS) is a group of gen- mon (EDS types IV, VI, VII, and VIII),7 whereas others eralized disorders characterized by abnormalities of have been found in only a few families (EDS types IX the connective tissue leading to fragility of the skin and and X).4 blood vessels, hyperextensibility of the skin, and joint EDS type VI is an autosomal recessive disorder.6,8 hypermobility.1–3 The prevalence is about 1 in 5000 The major diagnostic criteria are general joint hyper- births.4 The classification of the syndrome is according mobility, muscle hypotonia, scoliosis, and ocular fra- to the major and minor diagnostic criteria. On the basis gility. In addition to these symptoms, the minor diag- of these clinical symptoms, there are at least 10 types nostic criteria are tissue fragility, atrophic scars, easy of the syndrome. However, about half of the patients bruising, arterial rupture, tricuspid insufficiency, mar- with EDS do not fit into 1 of the 10 types.5 In 1997, fanoid habitus, microcornea, considerable radiological Beighton et al6 revised the classification because of osteopenia, and family history.3,6,8,9 In cases with a pro- recent developments in the elucidation of the biochem- gressive kyphoscoliosis, thoracic cage deformity and ical and molecular bases of EDS, together with in- hypotonia lead to a decrease in pulmonary function and favor recurrent episodes of pneumonia, relative a Professor, Department of Orthodontics, Yeditepe University coronary insufficiency, and early death.10 The diagno- Dental School, Istanbul, Turkey. sis is made by finding decreased amounts of hydroxy- b Research Assistant, Department of Orthodontics, Faculty of lysine in skin and confirmed by low levels of lysyl hy- Dentistry, Yeditepe University Dental School, Istanbul, Turkey. droxylase measured in cultured dermal fibroblasts.4,8 c Assistant Professor, Department of Orthodontics, Yeditepe University Dental School, Istanbul, Turkey. Absence of the inferior labial and lingual frenula was Corresponding author: Didem Nalbantgil, DDS, Msc, Depart- found to be an oral manifestation associated with clas- ment of Orthodontics, Faculty of Dentistry, Yeditepe University, sical and hypermobility types of the syndrome.11 Other Bagdat cad. 238, Goztepe, Istanbul, Turkey dental symptoms include fragile oral mucosa, early on- (e-mail: dnalbantgil@yeditepe.edu.tr) set of periodontal defects, unusual dental crown anat- Accepted: March 2005. Submitted: March 2005. omy, dental fractures, stunted roots or dilacerations, Q 2006 by The EH Angle Education and Research Foundation, aberrant dentinal tubules, pulpal vascular lesions and Inc. Based on a paper presented at the IXth International Sym- denticles, hypermobile temporomandibular joint (TMJ), posium on Dento

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