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Clinical treatment of oral manifestations of Beckwith-Wiedeman syndrome in a child

  • Aldo Giancotti1,*,
  • Gaia Romanini1
  • Raffaella Docimo1
  • Claudio Arcuri1

1Department of Orthodontics, Univeristy of Rome "Tor Vergata", Ospedale Fatebenefratelli, Isola Tiberina

DOI: 10.17796/jcpd.27.4.6356k0231w213386 Vol.27,Issue 4,October 2003 pp.377-380

Published: 01 October 2003

*Corresponding Author(s): Aldo Giancotti E-mail: giancott@uniroma2.it

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Abstract

Orthodontic treatment in a young patient with Beckwith-Wiedeman Syndrome (BWS) is reported and a multidisciplinary approach to the management of this syndrome is reviewed. The patient presented with a tendency to Class III malocclusion, an open-bite and a slight macroglossia, which was treated at an early age by glossotomy. It was decided to monitor growth without treatment and to wait for the best time to begin therapy. It was based only on functional rehabilitation, without any fixed appliances, in which optimum intercuspation of the teeth and the skeletal Class I relationship was achieved and maintained after the retention period of three years. In conclusion the treatment of BWS patients requires a multidisciplinary approach that includes orthodontics, orthopaedics and surgical interven-tion. It is also necessary to underline the significance of diagnosis at an early age and timely treatment to reduce the development of dento-skeletal alterations.

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Aldo Giancotti,Gaia Romanini,Raffaella Docimo,Claudio Arcuri. Clinical treatment of oral manifestations of Beckwith-Wiedeman syndrome in a child. Journal of Clinical Pediatric Dentistry. 2003. 27(4);377-380.

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https://www.jocpd.com/articles/10.17796/jcpd.27.4.6356k0231w213386

References

1. Bischoff FZ, Feldman GL, McCaskill C, Subramanian S, Hughes MR, Shaffer L. Single cell analysis demonstrating isodi-somy and Beckwith-Wiedeman syndrome. Hum Mo Genet 4: 395- 99, 1995.

2. Richard III CW, Boehnke M, Berg DJ et al.A radiation hybrid map of the distal short arm of human chromosome 11 containing the Beckwith-Wiedeman and associate embryonal tumor disease loci. Am J Hum Genet 52: 915-21, 1993.

3. Elliott M, Bayly R, Cole T, Temple IK, Maher ER. Clinical features and natural history of Beckwith-Wiedeman syndrome: presentation of 74 new cases. Clin Genet 46: 168-174, 1994.

4. Engström W, Lindham S, Schofield P. Wiedeman-Beckwith Syndrome. Eur J Pediatr 147: 450-7, 1988.

5. McManammy DS, Barnett JS. Macroglossia as a presentation of the Beckwith-Wiedeman syndrome. Plast Reconstr Surg 75: 170-6, 1985.

6. Weng EY, Mortier GR, Graham Jr JM. Beckwith-Wiedeman syndrome: an update and review for the primary paediatrician. Clinical Paediatrics 6: 317-26, 1995.

7. Wiedeman HR. Tumor and hemihypertrophy associate with Wiedeman-Beckwith’s syndrome. Eur J Pediatr 141: 129, 1983.

8. Rimmel FL, Shapiro AM, Shoemaker DL, Kenna MA. Head and neck manifestations of Beckwith-Wiedeman syndrome. Otolaryngol Head Neck Surg 113: 262-5, 1995.

9. Thornburn MJ, Miller CG, SmithRead CH. Exomphalos-Macroglossia-Gigantism syndrome in Jamaican patients. Am J Dis Child 119: 316-21, 1970.

10. Beckwith JB. Macroglossia, omphalocele, adrenal cytomegaly, gigan-tism and hyperplastic visceromegaly. Birth Defects 5: 188-196, 1969.

11. Friede H, Figueroa AA. The Beckwith-Wiedeman syndrome: a longitudinal study of the macroglossia and dentofacial complex. J Craniofac Genet Dev Biol Suppl 1: 179-87, 1985.

12. Kamogashira K, Ito T, Nakagawa M, Kawagoe H, Ichikawa K, Matsumoto M. Orthodontic findings of a case of Beckwith-Wiedeman syndrome. J Jpn Orthod Soc 43: 564-72, 1984.

13. Malagola C, Barbato E, Fordellone P. Sindrome di Beckwith-Wiedeman: un raro caso. Dental Cadmos 1: 74-9, 1990.

14. Ruffino S, Frigerio M, GalvagnoG, Bosco G. Il problema della macroglossia nel lattante affetto da BWS: descrizione di un caso. Minerva Stomatologica 34, 1985.

15. Preda GE, Pasetti P, Caggiula S. Sindrome di Beckwith- Wiedeman: presentazione di un caso clinico. Dental Cadmos 16: 104-7, 1989.

16. Slater AD. Primary macroglossia. Clin Pediatr 7: 357- 63, 1968.

17. Farkas JG, (ed) Anthropometry of the head and face. 2nd ed. New York, Raven, p.244-333, 1994.

18. Moore ES et al. Heterogeneity in Wiedeman Beckwith Syndrome: anthropometrics evidence. Am J Med Gen 90: 283-90, 2000.

19. Sokoloski PM, Ogle RG,Waite DE. Surgical correction of macroglos-sia in Beckwith-Wiedeman syndrome.J Oral Surgery 36:212-15,1978.

20. Rizer FM, Schechter GL, Richardson MA. Macroglossia: etiologic considerations and management techniques. Inter J Paediatric Otorhinolaryngology 8: 225-36, 1985.

21. Kveim M, Fisher JC, Jones KC, Gruer B. Early tongue reduction for Beckwith-Wiedeman macroglossia. Ann Plast Surg 14: 142-4, 1985.

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