Cherubism

Emma Boylan

Mary Wyers, MD

Children's Memorial Hospital

Abstract

2 year old

Keywords

1601 cherubism fibrous dysplasia


Publication Date: 2010-03-17

History

2 year old with facial swelling

Findings

Symmetric expansile remodeling of the right and left maxilla and mandible, with sparing of the mandibular rami and condyles. There is thinning of the cortices of the involved bones and a lucent to intermediate density matrix with scattered foci of dense sclerosis.

Diagnosis

Cherubism

DDx

craniofacial fibrous dysplasia, familial gigantoform cementoma

Discussion

Cherubism is a rare condition in which the maxillary and mandibular bones are replaced by fibrous tissue, causing painless symmetric facial swelling in children. Onset is typically between 2-4 years of age, and the swelling tends to spontaneously regress as the onset of puberty. The condition takes its name from the observable facial swelling and retraction of lower eyelids, which give patients a “cherubic” appearance. Although the lesions are benign, their growth can cause impairment in speech and mastication, as well as dental complications.

Diagnosis is primarily clinical and radiological; CT and MRI are also useful in determining the extent of the inflammatory tissue and creating an individual plan for the preservation of teeth. Radiologically similar conditions include brown tumors, craniofacial fibrous dysplasia, and central giant-cell granuloma; these can be eliminated on the basis of cherubism’s characteristic symmetry. Familial gigantoform cementoma is also a close radiological mimic of cherubism. However, the location of familial gigantoform cementoma, which may extend to the orbits and nasal septum, is inconsistent with cherubism which exclusively involves maxillary and mandibular bone.

Because the lesions are benign and tend to regress with significant bone remodeling into the third decade of the patient’s life, cases of cherubism may be treated conservatively. In some cases limited resection may be useful to improve function or for cosmetic reasons. There are also case studies which suggest that periodic surgery during childhood and adolescence may be useful in treating and preventing dental complications. This child did undergo surgical limited resection.

References

  1. Beaman FD, LW Bancroft, JJ Peterson, et al. “Imaging Characteristics of Cherubism.” American Journal of Roentgenology [serial online]. 2004;182:1051-1054. Available at: http://www.ajronline.org/cgi/reprint/182/4/1051. Accessed 12 March 2010.
  2. Holst AI, U Hirschfelder, S Holst. “Diagnostic potential of 3D-data-based reconstruction software: an analysis of the rare disease pattern of cherubism.” The Cleft Palate-Craniofacial Journal [serial online]. 2009;46:215-219. Available at: http://proquest.umi.com/pqdweb?index=0&did=1669698411&SrchMode=1&sid=1&Fmt=6&VInst=PROD&VType=PQD&RQT=309&VName=PQD&TS=1268412303&clientId=71862. Accessed 12 March 2010.
  3. Jain V, R Sharma. “Radiographic, CT and MRI features of cherubism.” Pediatric Radiology [serial online]. 2006;36:1099-1104. Available at: http://www.springerlink.com/content/wr21854031nq136k/fulltext.pdf. Accessed 10 March 2010.
  4. Mortarello C, L Bello, AG Lucchina, A Pucci. “Diagnosis and treatment of familial cherubism characterized by early onset and rapid development.” Journal of Craniofacial Surgery [serial online]. 2009;20(1):116-120. Available at: http://ovidsp.tx.ovid.com. Accessed 12 March 2010.
  5. Nicolae C, BR Olsen. “Unexpected matrix diseases and novel therapeutic strategies.” Cell and Tissue Research [serial online]. 2010;399:155-165. Available at: http://www.springerlink.com/content/p551572r780l1453/fulltext.pdf. Accessed 10 march 2010.

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