Orthodontic Treatment in Conjunction with Twin-bock Treatment and Growth Hormone Therapy in Silver Russell Syndrome

Silver-Russell syndrome (SRS) is a very rare genetic disorder characterized by intrauterine growth retardation, short stature, and typical craniofacial abnormalities including micrognathia. While growth hormone (GH) therapy in children with SRS significantly improves somatic growth, functional orthopedic treatment can also be effective in adolescents with mandibular deficiency. We report the effects of Phase 1 functional orthopedic treatment of a twin-block appliance in conjunction with GH administration in a 9-year-old boy with GH deficiency and SRS, and the result of the subsequent Phase 2 orthodontic treatment.

Silver-Russell syndrome, growth hormone, twin-block appliance

1. Eggermann T, Meyer E, Ranke MB, Holder M, Spranger S, Zerres K, et al. Diagnostic proceeding in Silver-Russell syndrome. Mol Diagn;9:205-9. 2005.

2. Silver HK, Kiyasu W, George J, Deamer WC. Syndrome of congenital hemihypertrophy, shortness of stature, and elevated urinary gonadotropins. Pediatrics;12:368-76. 1953.

3. Russell A. A syndrome of intra-uterine dwarfism recognizable at birth with cranio-facial dysostosis, disproportionately short arms, and other anomalies (5 examples). Proc R Soc Med;47:1040-4. 1954.

4. Saal HM, Pagon RA, Pepin MG. Reevaluation of Russell-Silver syndrome. J Pediatr;107:733-7. 1985.

5. Tanner JM, Lejarraga H, Cameron N. The natural history of the Silver-Russell syndrome: a longitudinal study of thirty-nine cases. Pediatr Res;9:611-23.1975.

6. Hansen KK, Latson LA, Buehler BA, Latson LA. Silver-Russell syndrome with unusual findings. Pediatrics;79:125-8. 1987.

7. Wollmann HA, Kirchner T, Enders H, Preece MA, Ranke MB. Growth and symptoms in Silver-Russell syndrome: review on the basis of 386 patients. Eur J Pediatr;154:958-68. 1995.

8. Kotilainen J, Holtta P, Mikkonen T, Arte S, Sipila I, Pirinen S. Craniofacial and dental characteristics of Silver-Russell syndrome. Am J Med Genet;56:229-36. 1995.

9. Rakover Y, Dietsch S, Ambler GR, Chock C, Thomsett M, Cowell CT. Growth hormone therapy in Silver Russell syndrome: 5 years experience of the Australian and New Zealand Growth database (OZGROW). Eur J Pediatr;155:851-7. 1996.

10. Kjellberg H, Beiring M, Albertsson Wikland K. Craniofacial morphology, dental occlusion, tooth eruption, and dental maturity in boys of short stature with or without growth hormone deficiency. Eur J Oral Sci ;108:359-67. 2000.

11. Bergman A, Kjellberg H, Dahlgren J. Craniofacial morphology and dental age in children with Silver-Russell syndrome. Orthod Craniofac Res;6:54-62. 2003.

12. Clayton PE, Cianfarani S, Czernichow P, Johannsson G, Rapaport R, Rogol A. Management of the child born small for gestational age through to adulthood: a consensus statement of the International Societies of Pediatric Endocrinology and the Growth Hormone Research Society. J Clin Endocrinol Metab;92:804-10. 2007.

13. O’Brien JE, Sadeghi-Nejad A, Feingold M. Growth hormone deficiency in a patient with Silver-Russell syndrome. J Pediatr;93:152-3.1978.

14. Kisnisci RS, Fowel SD, Epker BN. Distraction osteogenesis in Silver Russell syndrome to expand the mandible. Am J Orthod Dentofacial Orthop;116:25-30. 1999.

15. Davies TI, Rayner PH. Functional appliance therapy in conjunction with growth hormone treatment. A case report. Br J Orthod;22:361-5. 1995.

16. Hwang CJ, Cha JY. Orthodontic treatment with growth hormone therapy in a girl of short stature. Am J Orthod Dentofacial Orthop;126:118-26. 2004.

17. Cantu G, Buschang PH, Gonzalez JL. Differential growth and maturation in idiopathic growth-hormone-deficient children. Eur J Orthod;19:131-9. 1997.

18. Simmons KE. Growth hormone and craniofacial changes: preliminary data from studies in Turner’s syndrome. Pediatrics;104:1021-4. 1999.

19. Pirinen S. Endocrine regulation of craniofacial growth. Acta Odontol Scand;53:179-85. 1995.

20. de Zegher F, Du Caju MV, Heinrichs C, Maes M, De Schepper J, Craen M, et al. Early, discontinuous, high dose growth hormone treatment to normalize height and weight of short children born small for gestational age: results over 6 years. J Clin Endocrinol Metab;84:1558-61. 1999.

21. Suzuki S, Itoh K, Ohyama K. Local administration of IGF-I stimulates the growth of mandibular condyle in mature rats. J Orthod;31:138-43. 2004.

22. Peacey SR, Shalet SM. Insulin-like growth factor 1 measurement in diagnosis and management of acromegaly. Ann Clin Biochem;38:297-303. 2001.

23. Singleton DA, Buschang PH, Behrents RG, Hinton RJ. Craniofacial growth in growth hormone-deficient rats after growth hormone supplementation. Am J Orthod Dentofacial Orthop;130:69-82. 2006.

24. O’Brien K, Wright J, Conboy F, Appelbe P, Davies L, Connolly I, et al. Early treatment for Class II Division 1 malocclusion with the Twin-block appliance: a multi-center, randomized, controlled trial. Am J Orthod Dentofacial Orthop;135:573-9. 2009.

25. Baccetti T, Franchi L, Toth LR, McNamara JA, Jr. Treatment timing for Twin-block therapy. Am J Orthod Dentofacial Orthop;118:159-70. 2000.

26. Davies PS, Valley R, Preece MA. Adolescent growth and pubertal progression in the Silver-Russell syndrome. Arch Dis Child;63:130-5. 1988.

27. Ioannidou-Marathiotou I, Sluzker A, Athanasiou AE. Orthodontic management of silver-russell syndrome. A case report. Open Dent J;6:131-6. 2012.

28. Kotilainen J, Pälvi H, Taipo M, Sirpa A, Iikka S, Sinikka P. Craniofacial and dental characteristics of Silver-Russell syndrome. Am J Med Genet;56:229-36. 1995.

29. Ito RK, Vig KW, Garn SM, Hopwood NJ, Loos PJ, Spalding PM, et al. The influence of growth hormone (rhGH) therapy on tooth formation in idiopathic short statured children. Am J Orthod Dentofacial Orthop;103:358-64. 1993.