DIFFICULTIES AND TREATMENT OUTCOMES OF ORTHODONTIC THERAPY OF A PATIENT WITH WILLIAMS SYNDROME

Abstract

Williams Syndrome (WS) is a rare congenital and progressive multisystem disorder which is described with specific systemic disorders, skeletal and dental abnormalities and tongue thrusting. A 13-year-old patient applied to our clinic with complaining dental caries, malocclusion and tongue thrusting. The patient was treated with a full fixed standard edgewise appliance named as Mini Master Series from American Orthodontics (Central Islip, N.Y., USA), 0.018 inch bracket slots) for 2 years and 8 months.

Despite poor oral hygiene, frequent appointments helped to complete the orthodontic treatment of the patient without caries formation. Also extraction of first molars with uncertain prognosis provided social benefits when it was compared to healthy premolar extractions. This case report presents the successful results of the orthodontic therapy and difficulties of an orthodontic treatment of a patient who was diagnosed with WS.

Keywords

• Anterior Cross-bite,Chromosome Deletion,Orthodontic Treatment

WILLIAMS SENDROMLU HASTANIN ORTODONTİK TEDAVİSİNİN SONUÇLARI VE TEDAVİDEKİ ZORLUKLAR

Abstract

Williams Syndrome (WS) nadir görülen ve progresif bir şekilde ilerleyen multi-sistem bir sendrom olup özel sistemik hastalıklarla beraber, iskeletsel ve dental anomalilere ek olarak dil itme durumunun izlenebildiği bir hastalık tipidir. Bu vaka raporunda kliniğimize malokluzyon ve dil itme şikayetiyle başvuran WS sendromlu hastanın tedavisi sırasında karşılaşılan zorluklar ve tedavi planlaması anlatılmaktadır. Hasta standart edgewise braketler(Mini Master Series from American Orthodontics (Central Islip, N.Y., USA),0.018 inch braket slot) ile yaklaşık 2 yıl 8 ay tedavi edilmiştir. Bu vaka raporunda, WS tanısı ile takip edilen hastanın ortodontik tedavisi süresince karşılaşılan zorluklar ve bunlara rağmen elde edilen başarılı sonuçların paylaşılması hedeflenmiştir. Hastanın ortodontik tedavisi kötü oral hijyenine rağmen sık randevu aralıkları sayesinde çürük kaviteleri oluşmadan tamamlanmıştır. Ayrıca hastanın sağlıklı premolar dişlerinin ağızda tutularak prognozu belirsiz molar dişlerinin çekimi sosyal olarak hastaya avantaj sağlamıştır.

Keywords

• Anterior Çapraz Kapanış,Kromozom Delesyonu,Ortodontik Tedavi

References

1. 1. Williams JCP, Barratt-Boyes BG, Lowe J. Supravalvular aortic stenosis. Circulation 1961;24(6):1311-8.
2. 2. Beuren AJ, Schulze C, Eberle P, Harmjanz D, Apitz J. The syndrome of supravalvular aortic stenosis, peripheral pulmonary stenosis, mental retardation and similar facial appearance. Am J Cardiol 1964;13(4):471-83.
3. 3. Dupont B, Dupont A, Bliddal J, Holst E, Melchior J, Ottesen O. Idiopathic hypercalcaemia of infancy. The elfin face syndrome. Danish Med Bull 1970;17(2):33-46.
4. 4. Kelly JR, Barr ES. The elfin facies syndrome. Oral Surg, Oral Med, Oral Pathol 1975;40(2):205-18.
5. 5. Jones KL, Smith DW. The Williams elfin facies syndrome: a new perspective. Pediatr 1975;86(5):718-23.
6. 6. Baum BJ, Cohen MM. Agenesis and tooth size in the permanent dentition. Angle Orthod 1971;41(2):100-2.
7. 7. Morris CA, Demsey SA, Leonard CO, Dilts C, Blackburn BL. Natural history of Williams syndrome: physical characteristics. Pediatr 1988;113(2):318-26.
8. 8. Udwin O, Yule W. A cognitive and behavioural phenotype in Williams syndrome. J Clin Exp Neuropsychol 1991;13(2):232-44.

9. 9. Ewart AK, Morris CA, Atkinson D, Jin W, Sternes K, Spallone P, et al. Hemizygosity at the elastin locus in a developmental disorder, Williams syndrome. Nature Genet 1993;5(1):11-6.
10. 10. Nickerson E, Greenberg F, Keating MT, McCaskill C, Shaffer LG. Deletions of the elastin gene at 7q11. 23 occur in approximately 90% of patients with Williams syndrome. Am J Hum Genet 1995;56(5):1156-61.
11. 11. Lowery MC, Morris CA, Ewart A, Brothman LJ, Zhu XL, Leonard CO, et al. Strong correlation of elastin deletions, detected by FISH, with Williams syndrome: evaluation of 235 patients. Am J Hum Genet 1995;57(1):49-53.
12. 12. Tarjan I, Balaton G, Balaton P, Vajo Z. The role of dental evaluation and cephalometric analysis in the diagnosis of Williams–Beuren syndrome. Wien Klin Wochenschr 2005;117(5):226-8.
13. 13. Habersack K, Grimaldi B, Paulus GW. Orthodontic–orthognathic surgical treatment of a subject with Williams–Beuren syndrome—a follow-up from 8 to 25 years of age. Eur J Orthod 2007;29(4):332-7.
14. 14. Samanli ÜB, Sarioglu A, Saltlk L, Ertugrul A. Williams Sendromlu Çocuklarda Klinik ve Kardiyovasküler Bulgular. Türk Kardiyoloji Dern Arş 1997;25:375-81.
15. 15. Morris CA, Leonard CO, Dilts C, Demsey SA. Adults with Williams syndrome. Am J Med Genet 1990;37(6):102-7.
16. 16. Vieira GM, Franco EJ, Rocha DFP da, Oliveira LAd, Amorim RFB. Alternative treatment for open bite Class III malocclusion in a child with Williams-Beuren syndrome. Dental Press J Orthod 2015;20(1):97-107.
17. 17. Karmiloff-Smith A, Grant J, Ewing S, Carette M, Metcalfe K, Donnai D, et al. Using case study comparisons to explore genotype-phenotype correlations in Williams-Beuren syndrome. J Med Genet 2003;40(2):136-40.
18. 18. Pober BR, Morris CA. Diagnosis and management of medical problems in adults with Williams–Beuren syndrome. Paper presented at: Am J Med Genet C Semin Med Genet 2007;145:280-90.
19. 19. Bedeschi MF, Bianchi V, Colli AM, Natacci F, Cereda A, Milani D, et al. Clinical follow‐up of young adults affected by Williams syndrome: Experience of 45 Italian patients. Am J Med Genet 2011;155(2):353-9.
20. 20. Axelsson S, Kjær I, Heiberg A, Bjørnland T, Storhaug K. Neurocranial morphology and growth in Williams syndrome. Eur J Orthod 2005;27(1):32-47.
21. 21. Hertzberg J, Nakisbendi L, Needleman H, Pober B. Williams syndrome-oral presentation of 45 cases. Pediatr Dent 1994;16:262.
22. 22. Kelly, J. E. , M. Sanchez , and L. E. Van Kirk . An assessment of the occlusion of the teeth in children. Washington, DC: National Center for Health Statistics, US Public Health Service, DHEW Pub No (HRA) 74–1612, Series 11, No 130; 1973.
23. 23. Proffit W, Fields Jr H. Contemporary Orthodontics. St. Louis: Mosby–Year Book: Inc; 1993.