• Title/Summary/Keyword: Anterior open bite

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DENTAL TREATMENT FOR A PATIENT WITH TREACHER COLLINS SYNDROME : CASE REPORT (Treacher Collins 증후군 환아의 치과 치료: 증례보고)

  • Kim, Minji;Song, Ji-Soo;Shin, Teo Jeon;Hyun, Hong-Keun;Kim, Young-Jae;Kim, Jung-Wook;Lee, Sang-Hoon;Jang, Ki-Taeg
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.15 no.1
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    • pp.79-83
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    • 2019
  • Treacher Collins syndrome(TCS) is an autosomal craniofacial development disorder which results from mutations in the gene TCOF1. Major features include midface hypoplasia, micrognathia, microtia, conductive hearing loss. Oral manifestations are characterized by cleft palate, shortened soft palate, malocclusion, anterior open bite and enamel hypoplasia. The purpose of this presentation is to describe the interesting aspects of dental treatment of a patient with TCS. A 6-year-old boy with TCS visited Seoul National University Dental Hospital for dental caries. Multiple caries was observed from clinical and radiographic examination. Because of multiple caries and behavior management ploblem, dental treatment under general anesthesia was planned. Treatment of posterior teeth was performed and some primary teeth were extracted. General anesthesia was induced and maintained with sevoflurane, nitrous oxide and oxygen. Under general anesthesia, successful dental procedure was done. Considering behavior management problem and medical condition of patient with TCS, general anesthesia can be useful.

MANAGEMENT OF LESCH-NYHAN SYNDROME PATIENTS WITH SELF-MUTILATION BEHAVIOR USING THEIR TEETH : CASE REPORTS (레쉬니한 증후군(Lesch-Nyhan syndrome) 환자의 치아와 연관된 자해행동의 관리: 증례보고)

  • Lee, Ji-Mi;Lee, Sang-Ho;Lee, Nan-Young;Jih, Myeong-Kwan
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.14 no.2
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    • pp.97-101
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    • 2018
  • Lesch-Nyhan syndrome is a rare X-linked recessive genetic disorder. During purine metabolism, the hypoxanthine guanine phosphribosyl transferase (HGPRT) enzyme is deficient causing phosphoribosyl transferase to accumulate and resulting in excessive uric acid. Clinical symptoms include hypercalcemia, choreoathetosis, spasticity, mental retardation, and self-injury to lips, tongue or fingers. This results not only in pain caused by the self-injury but also secondary infection of the wound site and the esthetic damage of the soft tissue defect. Dental treatments include conservative methods using intraoral appliances such as soft mouthguards, fixed lip bumpers, and occlusal guards, and invasive methods such as extraction of all teeth or forming an artificial anterior open bite. We report two cases of Lesch-Nyhan syndrome patients with self-mutilation behavior; one was treated with a preservative method using a soft mouthguard, and the other was treated with extraction of all teeth.