Abstract
Systemic and local conditions involving the craniofacial skeleton are uncommon. Given similarities between pathologic entities, diagnosis and treatment can be challenging. Surgical and/or pharmacologic management during skeletal growth affects treatment decisions. In this chapter, we will discuss several maxillofacial bony conditions, their presentation, and management. We will also discuss therapeutic options with associated risks and benefits.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Kaban LB, Dodson TB. Management of giant cell lesions. Int J Oral Maxillofac Surg. 2006;35(11):1074–5.
Papadaki ME, Troulis MJ, Kaban LB. Advances in diagnosis and management of fibro-osseous lesions. Oral Maxillofac Surg Clin North Am. 2005;17(4):415–34.
Chuong R, Kaban LB, Kozakewich H, Perez-Atayde A. Central giant cell lesions of the jaws: a clinicopathologic study. J Oral Maxillofac Surg. 1986;44(9):708–13.
Neville BW, Damm DD, Allen CM, Bouquot JE. Giant cell lesions. In: Oral and maxillofacial pathology, Neville, Damm, Allen, Bouquot editors. New York: WB Saunders; 2004, pp. 611–620.
Susarla SM, August M, Dewsnup N, Faquin WC, Kaban LB, Dodson TB. CD34 staining density predicts giant cell tumor clinical behavior. J Oral Maxillofac Surg. 2009;67(5):951–6.
Dewsnup NC, Susarla SM, Abulikemu M, Faquin WC, Kaban LB, August M. Immunohistochemical evaluation of giant cell tumors of the jaws using CD34 density analysis. J Oral Maxillofac Surg. 2008;66(5):928–33.
Kaban LB, Mulliken JB, Ezekowitz RA, Ebb D, Smith PS, Folkman J. Antiangiogenic therapy of a recurrent giant cell tumor of the mandible with interferon alfa-2a. Pediatrics. 1999;103(6 Pt 1):1145–9.
Chuong R, Kaban LB. Diagnosis and treatment of jaw tumors in children. J Oral Maxillofac Surg. 1985;43(5):323–32.
Kaban LB, Troulis MJ, Wilkinson MS, Ebb D, Dodson TB. Adjuvant antiangiogenic therapy for giant cell tumors of the jaws. J Oral Maxillofac Surg. 2007;65(10):2018–24.
Papadaki ME, Lietman SA, Levine MA, Olsen BR, Kaban LB, Reichenberger EJ. Cherubism: best clinical practice. Orphanet J Rare Dis. 2012;7 Suppl 1:S6.
Jones WA. Familial multilocular cystic disease of the jaws. Am J Cancer. 1933;17:946–50.
Jones WA. Further observations regarding familial multilocular cystic disease of the jaws. Br J Radiol. 1938;11:227–41.
Von Wowern N. Cherubism: a 36-year long-term follow-up of 2 generations in different families and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2000;90(6):765–72.
Chacon GE, Ugalde CM, Jabero MF. Genetic disorders and bone affecting the craniofacial skeleton. Oral Maxillofac Surg Clin North Am. 2007;19(4):467–74, v.
Thompson N. Cherubism: familial fibrous dysplasia of the jaws. Br J Plast Surg. 1959;12:89–103.
McClendon JL, Anderson DE, Cornelius EA. Cherubism—hereditary fibrous dysplasia of the jaws: II. Pathologic considerations. Oral Surg Oral Med Oral Pathol. 1962;15(Suppl):17–38.
Ueki Y, Tiziani V, Santanna C, Fukai N, Maulik C, Garfinkle J, Ninomiya C. doAmaral C, Peters H, Habal M, Rhee-Morris L, Doss JB, Kreiborg S, Olsen BR, Reichenberger E. Mutations in the gene encoding c-Abl-binding protein SH3BP2 cause cherubism. Nat Genet. 2001;28(2):125–6.
Tiziani V, Reichenberger E, Buzzo CL, Niazi S, Fukai N, Stiller M, Peters H, Salzano FM, Raposo doAmaral CM, Olsen BR. The gene for cherubism maps to chromosome 4p16. Am J Hum Genet. 1999;65(1):158–66.
Noonan J, Ehmke D. Associated noncardiac malformations in children with congenital heart disease. J Pediatr. 1963;63:468–70.
Lee SM, Cooper JC. Noonan syndrome with giant cell lesions. Int J Paediatr Dent. 2005;15(2):140–5.
Cohen Jr MM, Gorlin RJ. Review Noonan-like/multiple giant cell lesion syndrome. Am J Med Genet. 1991;40(2):159–66.
Tartaglia M, Zampino G, Gelb BD. Noonan syndrome: clinical aspects and molecular pathogenesis. Mol Syndromol. 2010;1(1):2–26.
Lee JS, Tartaglia M, Gelb BD, Fridrich K, Sachs S, Stratakis CA, Muenke M, Robey PG, Collins MT, Slavotinek A. Phenotypic and genotypic characterisation of Noonan-like/multiple giant cell lesion syndrome. J Med Genet. 2005;42(2):e11.
Jafarov T, Ferimazova N, Reichenberger E. Noonan-like syndrome mutations in PTPN11 in patients diagnosed with cherubism. Clin Genet. 2005;68(2):190–1.
Beneteau C, Cavé H, Moncla A, Dorison N, Munnich A, Verloes A, Leheup B. SOS1 and PTPN11 mutations in five cases of Noonan syndrome with multiple giant cell lesions. Eur J Hum Genet. 2009;17(10):1216–21.
Edwards PC, et al. Bilateral central giant cell granulomas of the mandible in an 8-year-old girl with Noonan syndrome (Noonan-like/multiple giant cell lesion syndrome). Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005;99(3):334–40.
Lee JS, FitzGibbon EJ, Chen YR, Kim HJ, Lustig LR, Akintoye SO, Collins MT, Kaban LB. Clinical guidelines for the management of craniofacial fibrous dysplasia. Orphanet J Rare Dis. 2012;24(7 Suppl 1):S2.
Chen YR, Wong FH, Hsueh C, Lo LJ. Computed tomography characteristics of non-syndromic craniofacial fibrous dysplasia. Chang Gung Med J. 2002;25(1):1–8.
Schwindinger WF, Francomano CA, Levine MA. Identification of a mutation in the gene encoding the alpha subunit of the stimulatory G protein of adenylyl cyclase in McCune-Albright syndrome. Proc Natl Acad Sci U S A. 1992;89(11):5152–6.
Weinstein LS, Shenker A, Gejman PV, Merino MJ, Friedman E, Spiegel AM. Activating mutations of the stimulatory G protein in the McCune-Albright syndrome. N Engl J Med. 1991;325(24):1688–95.
Park BY, Cheon YW, Kim YO, Pae NS, Lee WJ. Prognosis for craniofacial fibrous dysplasia after incomplete resection: age and serum alkaline phosphatase. Int J Oral Maxillofac Surg. 2010;39:221.
Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69:412.
Cohen Jr MM. Vasculogenesis, angiogenesis, hemangiomas, and vascular malformations. Am J Med Genet. 2002;108:265–74.
Mulliken JB, Fishman SJ, Burrows PE. Vascular anomalies. Curr Probl Surg. 2000;37(8):520–76.
Kaban LB, Mulliken JB. Maxillofacial vascular anomalies. In: Pediatric oral and maxillofacial surgery. Kaban LB, Troulis MJ, editors. Philadelphia: WB Saunders; 2004, pp. 259–285.
Kaban LB, Mulliken JB. Vascular anomalies of the maxillofacial region. J Oral Maxillofac Surg. 1986;44:203.
Finn MC, Mulliken JB, Glowacki J. Congenital vascular lesions: clinical application of a new classification. J Pediatr Surg. 1983;18:894.
Abramowicz S, Padwa BL. Vascular anomalies in children. Oral Maxillofac Surg Clin North Am. 2012;24(3):443–55.
Kohout MP, Hansen M, Pribaz JJ, Mulliken JB. Arteriovenous malformations of the head and neck: natural history and management. Plast Reconstr Surg. 1998;102:643–54.
Steiner, RD; Pepin, MG, Byers, PH, Pagon, RA, Bird, TD, Dolan, CR, Stephens, K, Adam, MP (2005). Osteogenesis Imperfecta. PMID 20301472. http://www.ncbi.nlm.nih.gov/books/NBK1295. Retrieved Nov 28, 2012.
Rosén A, Modig M, Larson O. Orthognathic bimaxillary surgery in two patients with osteogenesis imperfecta and a review of the literature. Int J Oral Maxillofac Surg. 2011;40(8):866–73.
Tashima H, Wattanawong K, Ho CT, Wen-Ching-Ko E, Nguyen A, Lo LJ. Orthognathic surgery considerations for patients with undiagnosed type I osteogenesis imperfecta. J Oral Maxillofac Surg. 2011;69(8):2233–41.
López-Arcas JM, Chamorro M, Del Castillo JL, Cebrián JL, Palacios E, Burgueño M. Osteogenesis imperfecta and orthognathic surgery: case report and literature review. J Oral Maxillofac Surg. 2009;67(5):1128–32.
Turer CB, Lin H, Flores G. Prevalence of vitamin D deficiency among overweight and obese US children. Pediatrics. 2013;131(1):e152–61.
Wacker M, Holick MF. Vitamin D—effects on skeletal and extraskeletal health and the need for supplementation. Nutrients. 2013;5(1):111–48.
Ross AC, Manson JE, Abrams SA, Aloia JF, Brannon PM, Clinton SK, et al. The 2011 report on dietary reference intakes for calcium and vitamin D from the Institute of Medicine: what clinicians need to know. J Clin Endocrinol Metab. 2011;96(1):53–8. PMCID: 3046611.
Rizzoli R, Boonen S, Brandi ML, Bruyere O, Cooper C, Kanis JA, et al. Vitamin D supplementation in elderly or postmenopausal women: a 2013 update of the 2008 recommendations from the European Society for Clinical and Economic Aspects of Osteoporosis and Osteoarthritis (ESCEO). Curr Med Res Opin. 2013;29:305–13.
Prentice RL, Pettinger MB, Jackson RD, Wactawski-Wende J, Lacroix AZ, Anderson GL, et al. Health risks and benefits from calcium and vitamin D supplementation: Women’s Health Initiative clinical trial and cohort study. Osteoporos Int. 2012;24(2):567–80.
Vanstone MB, Oberfield SE, Shader L, Ardeshirpour L, Carpenter TO. Hypercalcemia in children receiving pharmacologic doses of vitamin D. Pediatrics. 2012;129(4):e1060–3.
Winzenberg T, Shaw K, Fryer J, Jones G. Effects of calcium supplementation on bone density in healthy children: meta-analysis of randomised controlled trials. BMJ. 2006;333(7572):775. PMCID: 1602024.
Zhu K, Prince RL. Calcium and bone. Clin Biochem. 2012;45(12):936–42.
Candelas G, Martinez-Lopez JA, Rosario MP, Carmona L. Calcium supplementation and kidney stone risk in osteoporosis: a systematic literature review. Clin Exp Rheumatol. 2012;30(6):954–61.
Spence LA, Weaver CM. Calcium intake, vascular calcification, and vascular disease. Nutr Rev. 2013;71:115–22.
Falk MJ, Heeger S, Lynch KA, DeCaro KR, Bohach D, Gibson KS, et al. Intravenous bisphosphonate therapy in children with osteogenesis imperfecta. Pediatrics. 2003;111(3):573–8.
Rauch F, Travers R, Plotkin H, Glorieux FH. The effects of intravenous pamidronate on the bone tissue of children and adolescents with osteogenesis imperfecta. J Clin Invest. 2002;110(9):1293–9. PMCID: 151613.
Ward LM, Rauch F, Whyte MP, D’Astous J, Gates PE, Grogan D, et al. Alendronate for the treatment of pediatric osteogenesis imperfecta: a randomized placebo-controlled study. J Clin Endocrinol Metab. 2011;96(2):355–64.
Sellmeyer DE. Atypical fractures as a potential complication of long-term bisphosphonate therapy. JAMA. 2010;304(13):1480–4.
Leet AI, Collins MT. Current approach to fibrous dysplasia of bone and McCune-Albright syndrome. J Child Orthop. 2007;11:3–17. PMCID: 2656698.
Liamis G, Milionis HJ, Elisaf M. A review of drug-induced hypocalcemia. J Bone Miner Metab. 2009;27(6):635–42.
Amanata N, He LH, Swain MV, Little DG. The effect of zoledronic acid on the intrinsic material properties of healing bone: an indentation study. Med Eng Phys. 2008;30:7843–7.
Kim TY, Ha YC, Kang BJ, Lee YK, Koo KH. Does early administration of bisphosphonate affect fracture healing in patients with intertrochanteric fractures? J Bone Joint Surg Br. 2012;94(7):956–60.
Kidd LJ, Cowling NR, Wu AC, Kelly WL, Forwood MR. Bisphosphonate treatment delays stress fracture remodeling in the rat ulna. J Orthop Res. 2011;29(12):1827–33.
Sharma D, Ivanovski S, Slevin M, Hamlet S, Pop TS, Brinzaniuc K, et al. Bisphosphonate-related osteonecrosis of jaw (BRONJ): diagnostic criteria and possible pathogenic mechanisms of an unexpected anti-angiogenic side effect. Vasc Cell. 2013;5(1):1.
Maines E, Monti E, Doro F, Morandi G, Cavarzere P, Antoniazzi F. Children and adolescents treated with neridronate for osteogenesis imperfecta show no evidence of any osteonecrosis of the jaw. J Bone Miner Metab. 2012;30(4):434–8.
Malmgren B, Astrom E, Soderhall S. No osteonecrosis in jaws of young patients with osteogenesis imperfecta treated with bisphosphonates. J Oral Pathol Med. 2008;37(4):196–200.
Schwartz S, Joseph C, Iera D, Vu DD. Bisphosphonates, osteonecrosis, osteogenesis imperfecta and dental extractions: a case series. J Can Dent Assoc. 2008;74(6):537–42.
Report of the Council of Scientific Affairs. Expert panel recommendations: dental management of patients on oral bisphosphonate therapy. American Dental Association, June 2006. http://www.ada.org/prof/resources/topics/osteonecrosis.asp. Accessed Jan 11, 2013.
Ruggiero SL, Dodson TB, Assael LA, Landesberg R, Marx RE, Mehrotra B, American Association of Oral and Maxillofacial Surgeons. American Association of Oral and Maxillofacial Surgeons position paper on bisphosphonate-related osteonecrosis of the jaws—2009 update. J Oral Maxillofac Surg. 2009;67(5 Suppl):2–12.
Shroff R, Beringer O, Rao K, Hofbauer LC, Schulz A. Denosumab for post-transplantation hypercalcemia in osteopetrosis. N Engl J Med. 2012;367(18):1766–7.
Boyce AM, Chong WH, Yao J, Gafni RI, Kelly MH, Chamberlain CE, et al. Denosumab treatment for fibrous dysplasia. J Bone Miner Res. 2012;27(7):1462–70.
Watanabe A, Yoneyama S, Nakajima M, Sato N, Takao-Kawabata R, Isogai Y, et al. Osteosarcoma in Sprague–Dawley rats after long-term treatment with teriparatide (human parathyroid hormone (1–34)). J Toxicol Sci. 2012;37(3):617–29.
Mason JJ, Williams BO. SOST and DKK: antagonists of LRP family signaling as targets for treating bone disease. J Osteoporos. 2010;2010:460120. PMCID: 2951123.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer Science+Business Media New York
About this chapter
Cite this chapter
Susarla, S.M., Jacobsen, C.M., Abramowicz, S. (2014). Pediatric Maxillofacial Conditions and Drugs. In: Klein, G. (eds) Bone Drugs in Pediatrics. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-7436-5_11
Download citation
DOI: https://doi.org/10.1007/978-1-4899-7436-5_11
Published:
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4899-7435-8
Online ISBN: 978-1-4899-7436-5
eBook Packages: MedicineMedicine (R0)