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

Yıl 2018, Cilt: 5 Sayı: 2, 157 - 163, 01.08.2018

Elif Dilara Şeker , Türker Yücesoy , Ahmet Yağcı

https://doi.org/10.15311/selcukdentj.337302

Öz

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.

Anahtar Kelimeler

Anterior Cross-bite, Chromosome Deletion, Orthodontic Treatment

WILLIAMS SENDROMLU HASTANIN ORTODONTİK TEDAVİSİNİN SONUÇLARI VE TEDAVİDEKİ ZORLUKLAR

Öz

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.

Anahtar Kelimeler

Anterior Çapraz Kapanış, Kromozom Delesyonu, Ortodontik Tedavi

Kaynakça

• 1. Williams JCP, Barratt-Boyes BG, Lowe J. Supravalvular aortic stenosis. Circulation 1961;24(6):1311-8.
• 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. 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. Kelly JR, Barr ES. The elfin facies syndrome. Oral Surg, Oral Med, Oral Pathol 1975;40(2):205-18.
• 5. Jones KL, Smith DW. The Williams elfin facies syndrome: a new perspective. Pediatr 1975;86(5):718-23.
• 6. Baum BJ, Cohen MM. Agenesis and tooth size in the permanent dentition. Angle Orthod 1971;41(2):100-2.
• 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. Udwin O, Yule W. A cognitive and behavioural phenotype in Williams syndrome. J Clin Exp Neuropsychol 1991;13(2):232-44.
• 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. 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. 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. 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. 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. Samanli ÜB, Sarioglu A, Saltlk L, Ertugrul A. Williams Sendromlu Çocuklarda Klinik ve Kardiyovasküler Bulgular. Türk Kardiyoloji Dern Arş 1997;25:375-81.
• 15. Morris CA, Leonard CO, Dilts C, Demsey SA. Adults with Williams syndrome. Am J Med Genet 1990;37(6):102-7.
• 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. 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. 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. 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. 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. Hertzberg J, Nakisbendi L, Needleman H, Pober B. Williams syndrome-oral presentation of 45 cases. Pediatr Dent 1994;16:262.
• 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. Proffit W, Fields Jr H. Contemporary Orthodontics. St. Louis: Mosby–Year Book: Inc; 1993.