Dental Management in a Patient with Turner Syndrome with Dental Anomalies : A Case Report = 치아형태이상을 가진 터너 증후군 환자의 치과적 관리

다국어 초록 (Multilingual Abstract)

Turner syndrome (TS) is a chromosomal disorder caused by monosomy of the X chromosome, with complete or partial absence of the second sex chromosome. Anomalies of root morphology have been found to occur more often in patients with TS, which make endodontic treatment challenging and requires special handling. The patients with TS may also have systematic problems such as cardiac or renal malformations, so in treating these patients it is important for clinicians not only to be aware of the characteristic intraoral findings, but also to make the patients have regular dental check-ups to prevent oral complications in advance. An 12-year-old girl who had been diagnosed with TS at the age of 10 years was referred due to discomfort in the bilateral mandibular premolar regions. Dens evaginatus and taurodontism were detected in all the mandibular premolars characteristically. The bilateral mandibular first premolars had three roots and the bilateral mandibular second premolars had periapical lesion with two roots. Due to the complexity of the root canal anatomy, root canal treatment were completed with a dental microscope to ensure adequate visualization. After 2 years of regular follow-up examinations, there were no clinical sign or symptom associated with the teeth, and no periapical lesion, was found. This case report describes the characteristic oral features and dental management of TS patients.

참고문헌 (Reference)

1 Garn SM, "X-linked inheritance of tooth size" 44 : 439-441, 1965

2 Saenger P, "Turner's Syndrome" 335 : 1749-1754, 1996

3 Knickmeyer RC, "Turner syndrome: advances in understanding altered cognition, brain structure and function" 25 : 144-149, 2012

4 Batch J, "Turner syndrome in childhood and adolescence" 16 : 465-482, 2002

5 Varrela J, "Tooth crown size in human females with 45,X/46,XX chromosomes" 33 : 291-294, 1988

6 Mayhall JT, "Tooth crown size in 46,X,i(Xq) human females" 36 : 411-414, 1991

7 Midtbo M, "Tooth crown size and morphology in Turner syndrome" 52 : 7-19, 1994

8 Midtbo M, "Tooth crown size and morphology in Turner syndrome" 52 : 7-19, 1994

9 Cleghorn BM, "The root and root canal morphology of the human mandibular second premolar: a literature review" 33 : 1031-1037, 2007

10 Cleghorn BM, "The root and root canal morphology of the human mandibular first premolar: a literature review" 33 : 509-516, 2007

11 Gonzalez L, "The patient with Turner syndrome: puberty and medical management concerns" 98 : 780-786, 2012

12 Tsesis I, "Taurodontism: an endodontic challenge. Report of a case" 29 : 353-355, 2003

13 Varrela J, "Taurodontism in females with extra X chromosomes" 9 : 129-133, 1989

14 Varrela J, "Taurodontism in 45,X females" 69 : 494-495, 1990

15 MidtbMidtbo M, "Skeletal maturity, dental maturity, and eruption in young patients with Turner syndrome" 50 : 303-312, 1992

16 Varrela J, "Root morphology of mandibular premolars in human 45,X females" 35 : 109-112, 1990

17 Varrela J, "Root morphology of mandibular premolars in human 45,X females" 35 : 109-112, 1990

18 Kusiak A, "Root morphology of mandibular premolars in 40 patients with Turner syndrome" 38 : 822-826, 2005

19 Townsend G, "Reduced tooth size in 45,X (Turner syndrome) females" 65 : 367-371, 1984

20 Saenger P, "Recommendations for the diagnosis and management of Turner syndrome" 86 : 3061-3069, 2001

21 Gotzsche CO, "Prevalence of cardiovascular malformations and association with karyotypes in Turner's syndrome" 71 : 433-436, 1994

22 Horowitz SL, "Palatal abnormalities in the syndrome of gonadal dysgenesis and its variants and in Noonan's syndrome" 38 : 839-844, 1974

23 Szilagyi A, "Oral manifestations of patients with Turner syndrome" 89 : 577-584, 2000

24 Lopez ME, "Oral and clinical characteristics of a group of patients with Turner syndrome" 94 : 196-204, 2002

25 Ho VB, "Major vascular anomalies in Turner syndrome : prevalence and magnetic resonance angiographic features" 110 : 1694-1700, 2004

26 Lin AE, "Further delineation of aortic dilation, dissection, and rupture in patients with Turner syndrome" 102 : e12-, 1998

27 Joseph M, "Endodontic treatment in three taurodontic teeth associated with 48,XXXY Klinefelter syndrome: a review and case report" 105 : 670-677, 2008

28 Varrela J, "Effect of 45,X/46,XX mosaics on root morphology of mandibular premolars" 71 : 1604-1606, 1992

29 Faggella A, "Dental features in patients with Turner syndrome" 7 : 165-168, 2006

30 Mazzanti L, "Congenital heart disease in patients with Turner's syndrome. Italian Study Group for Turner Syndrome (ISGTS)" 133 : 688-692, 1998

31 Sybert VP, "Cardiovascular malformations and complications in Turner syndrome" 101 : E11-, 1998

32 Turner EK, "A case of probable ovarian agenesis with Turner syndrome in a female child aged seven months" 41 : 39-42, 1954

33 Bharti R, ""Taurodontism" an endodontic challenge: a case report" 51 : 471-474, 2009