Cherubism in a 4-year-old boy managed with tumor curettage, mandibular osteotomies and repositioning

Abstract

Cherubism is a rare disease characterized by significant loss of medullary bone which is replaced by excessive amounts of fibrous tissue growth within the mandible and maxilla. We present a case of a 4-year-old boy with a rapidly enlarging mandible and maxilla, causing significant change in the facial contour, malocclusion and phonation difficulties. He was treated with aggressive tumor curettage, lateral mandibular cortex osteotomies with medial repositioning. This allowed obliteration of the enlarged medullary space and restoration of the normal mandibular anatomy. At 12 months postoperatively, the patient had significant improvement in facial contour, normal outward appearance, and stable dentition.

Keywords

Cherubism / mandibular osteotomy / tumor curettage / Piezo Electric bone cutter / surgical treatment for cherubism

References

[1] Jones WA.Familial multilocular cystic disease of the jaws.Am J Cancer1933;17:946-50

[2] Jones WA.Further observations regarding familial multilocular cystic disease of the jaws.Brit J Radiol1938;11:227-41

[3] Jones WA,Pritchard J.Cherubism-familial fibrous dysplasia of the jaws.J Bone Joint Surg Br1950;32-b:334-47

[4] Schultze-Mosgau S,Wiltfang J.Cherubism: clinical evidence and therapy.J Craniofac Surg2003;14:201-6; discussion 7-8

[5] Raposo-Amaral CE,Warren SM,Amstalden EM,Raposo-Amaral CM.Two-stage surgical treatment of severe cherubism.Ann Plast Surg2007;58:645-51

[6] Baskin B,Ray PN.Cherubism. GeneReviews(®). GeneReviews is a registered trademark of the University of Washington, Seattle.1993;Seattle (WA)University of Washington

[7] Papadaki ME,Levine MA,Kaban LB.Cherubism: best clinical practice.Orphanet J Rare Dis2012;7 Suppl 1:S6 PMCID:PMC3359956

[8] Deshmukh R,Deo PN.Nonfamilial cherubism: a case report and review of literature.J Oral Maxillofac Pathol2017;21:181 PMCID:PMC5406808

[9] Prescott T,Rustad CF,Geirdal AO,Jensen JL.Characterization of a Norwegian cherubism cohort; molecular genetic findings, oral manifestations and quality of life.Eur J Med Genet2013;56:131-7

[10] Ueki Y,Santanna C,Maulik C,Ninomiya C,Peters H,Rhee-Morris L,Kreiborg S,Reichenberger E.Mutations in the gene encoding c-Abl-binding protein SH3BP2 cause cherubism.Nat Genet2001;28:125-6

[11] Jain V.Radiographic, CT and MRI features of cherubism.Pediatr Radiol2006;36:1099-104

[12] Kau CH,English JD,Wong ME.The surgical and orthodontic management of cherubism in a growing child.J Craniomaxillofac Surg2012;40:229-33

[13] Meng XM,Yu GY.Clinicopathologic study of 24 cases of cherubism.Int J Oral Maxillofac Surg2005;34:350-6

[14] Shokri A.Cherubism: an unusual study with long-term follow-up.J Craniofac Surg2016;27:e511-2

[15] Dukart RC,Polte HW.Cherubism: report of case.J Oral Surg1974;32:782-5

[16] 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 Endod2000;90:765-72

[17] Tsodoulos S,Antoniades K.Cherubism: a case report of a three-generation inheritance and literature review.J Oral Maxillofac Surg2014;72:405.e1-9