Treatment Considerations in Hutchinson-Gilford Progeria Syndrome: A Case Report

Hutchinson-Guilford progeria syndrome is an extremely rare condition classified as one of the premature ageing syndromes. This case presents a 16-year-old Israeli female patient, suffering from a variant of Hutchinson-Guilford progeria with a history of treatment with oral biphosphnates. The patient presented with typical cranial and facial features of the syndrome including delayed teeth eruption and root development probably due to insufficient jaw growth and severs retrognatic position of the maxilla and mandible. Orthodontic treatment considerations are described along with those required in light of the previous treatment by oral biphosphonates. All primary teeth were extracted in three appointments while creating as minimal trauma as possible to the surrounding tissue and alveolar bone. For now, the patient refuses to begin the orthodontic treatment course. There are no limitations to conduct any dental procedures in progeria patients, however, extreme caution must be exercised during oral surgery due to the inelasticity of tissues and dermal atrophy. Orthodontic procedure commencement should be early enough to manage the delayed development and eruption of teeth. Patients taking oral biphosphonates should be advised of this potential complication. If orthodontic treatment is considered appropriate, plans should be assessed and modified to include compromises.

Hutchinson-Guilford progeria, Orthodontics, Biphosphonates

1. Dyer CA, Sinclair AJ. The premature ageing syndromes: insights into the ageing process. Age Ageing; 27: 73-80,1998.

2. Batstone MD, Macleod AW. Oral and maxillofacial surgical considerations for a case of Hutchinson-Gilford progeria. Int J Paediatr Dent; 12: 429-432,2002.

3. DeBusk FL. The Hutchinson-Gilford progeria syndrome. Report of 4 cases and review of the literature. J Pediatr; 80: 697-724,1972.

4. Domingo DL, Trujillo MI, Council SE, Merideth MA, Gordon LB, Wu T et al. Hutchinson-Gilford progeria syndrome: oral and craniofacial phenotypes. Oral Dis; 15: 187-195,2009.

5. Pollex RL, Hegele RA. Hutchinson-Gilford progeria syndrome. Clin Genet; 66: 375-381,2004.

6. Fong LG, Ng JK, Meta M, Cote N, Yang SH, Stewart CL et al. Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice. Proc Natl Acad Sci U S A; 101: 18111-18116,2004.

7. Goldman RD, Shumaker DK, Erdos MR, Eriksson M, Goldman AE, Gordon LB et al. Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome. Proc Natl Acad Sci U S A; 101: 8963-8968,2004.

8. Martin GM. Genetic modulation of senescent phenotypes in Homo sapiens. Cell; 120: 523-532,2005.

9. Gordon LB, McCarten KM, Giobbie-Hurder A, Machan JT, Campbell SE, Berns SD et al. Disease progression in Hutchinson-Gilford progeria syndrome: impact on growth and development. Pediatrics; 120: 824-833,2007.

10. Hasty MF, Vann WF, Jr. Progeria in a pediatric dental patient: literature review and case report. Pediatr Dent; 10: 314-319,1988.

11. Wesley RK, Delaney JR, Litt R. Progeria: clinical considerations of an isolated case. ASDC J Dent Child; 46: 487-492,1979.

12. Yu QX, Zeng LH. Progeria: report of a case and review of the literature. J Oral Pathol Med; 20: 86-88,1991.

13. Palsson SR, Kjaer I. Morphology of the mandibular canal and the angulation between the mandibular and mental canals in dry skulls. Eur J Orthod; 31: 59-63,2009.

14. McNamara JA, Jr. A method of cephalometric evaluation. Am J Orthod; 86: 449-469,1984.

15. Suri L, Gagari E, Vastardis H. Delayed tooth eruption: pathogenesis, diagnosis, and treatment. A literature review. Am J Orthod Dentofacial Orthop; 126: 432-445,2004.

16. Fukuchi K, Katsuya T, Sugimoto K, Kuremura M, Kim HD, Li L et al. LMNA mutation in a 45 year old Japanese subject with Hutchinson-Gilford progeria syndrome. J Med Genet; 41: e67,2004.

17. De Sandre-Giovannoli A, Bernard R, Cau P, Navarro C, Amiel J, Boccaccio I et al. Lamin a truncation in Hutchinson-Gilford progeria. Science; 300: 2055,2003.

18. Eriksson M, Brown WT, Gordon LB, Glynn MW, Singer J, Scott L et al. Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome. Nature; 423: 293-298,2003.

19. Kurol J. Early treatment of tooth-eruption disturbances. Am J Orthod Dentofacial Orthop; 121: 588-591,2002.

20. Smith RJ, Rapp R. A cephalometric study of the developmental relationship between primary and permanent maxillary central incisor teeth. ASDC J Dent Child; 47: 36-41,1980.

21. Crepin S, Laroche ML, Sarry B, Merle L. Osteonecrosis of the jaw induced by clodronate, an alkylbiphosphonate: case report and literature review. Eur J Clin Pharmacol; 66: 547-554,2010.

22. Filleul O, Crompot E, Saussez S. Bisphosphonate-induced osteonecrosis of the jaw: a review of 2,400 patient cases. J Cancer Res Clin Oncol; 136: 1117-1124,2010.

23. Ryan P, Saleh I, Stassen LF. Osteonecrosis of the jaw: a rare and devastating side effect of bisphosphonates. Postgrad Med J; 85: 674-677,2009.

24. Sedghizadeh PP, Stanley K, Caligiuri M, Hofkes S, Lowry B, Shuler CF. Oral bisphosphonate use and the prevalence of osteonecrosis of the jaw: an institutional inquiry. J Am Dent Assoc; 140: 61-66,2009.

25. Truong SV, Chang LC, Berger TG. Bisphosphonate-related osteonecrosis of the jaw presenting as a cutaneous dental sinus tract: a case report and review of the literature. J Am Acad Dermatol; 62: 672-676,2010.

26. Vassiliou V, Tselis N, Kardamakis D. Osteonecrosis of the jaws: clinicopathologic and radiologic characteristics, preventive and therapeutic strategies. Strahlenther Onkol; 186: 367-373,2010.

27. Brown JJ, Ramalingam L, Zacharin MR. Bisphosphonate-associated osteonecrosis of the jaw: does it occur in children? Clin Endocrinol (Oxf); 68: 863-867,2008.

28. Chahine C, Cheung MS, Head TW, Schwartz S, Glorieux FH, Rauch F. Tooth extraction socket healing in pediatric patients treated with intravenous pamidronate. J Pediatr; 153: 719-720,2008.

29. Schwartz S, Joseph C, Iera D, Vu DD. Bisphosphonates, osteonecrosis, osteogenesis imperfecta and dental extractions: a case series. J Can Dent Assoc; 74: 537-542,2008.

30. Edwards BJ, Hellstein JW, Jacobsen PL, Kaltman S, Mariotti A, Migliorati CA. Updated recommendations for managing the care of patients receiving oral bisphosphonate therapy: an advisory statement from the American Dental Association Council on Scientific Affairs. J Am Dent Assoc; 139: 1674-1677,2008.

31. Khan AA, Sandor GK, Dore E, Morrison AD, Alsahli M, Amin F et al. Canadian consensus practice guidelines for bisphosphonate associated osteonecrosis of the jaw. J Rheumatol; 35: 1391-1397,2008.

32. Rinchuse DJ, Sosovicka MF, Robison JM, Pendleton R. Orthodontic treatment of patients using bisphosphonates: a report of 2 cases. Am J Orthod Dentofacial Orthop; 131: 321-326,2007.

33. Schwartz JE. Ask us: Some drugs affect tooth movement. Am J Orthod Dentofacial Orthop; 127: 644,2005.

34. Ghoneima AA, Allam ES, Zunt SL, Windsor LJ. Bisphosphonates treatment and orthodontic considerations. Orthod Craniofac Res; 13: 1-10,2010.

35. Zahrowski JJ. Bisphosphonate treatment: an orthodontic concern calling for a proactive approach. Am J Orthod Dentofacial Orthop; 131: 311-320,2007.

36. Wagner M, Katsaros C, Goldstein T. Spontaneous uprighting of permanent tooth germs after elimination of local eruption obstacles. J Orofac Orthop; 60: 279-285,1999.