Home | About Us | Editorial Board | Current Issue | Archives | Search | Instructions | Subscription | Feedback | e-Alerts | Login 
Journal of Indian Society of Pedodontics and Preventive Dentistry Official publication of Indian Society of Pedodontics and Preventive Dentistry
 Users Online: 1919  
 
  Print this page Email this page   Small font sizeDefault font sizeIncrease font size


 
  Table of Contents    
CASE REPORT
Year : 2013  |  Volume : 31  |  Issue : 1  |  Page : 43-47
 

Nonfamilial cherubism


Department of Oral Medicine and Radiology, Guru Nanak Dev Dental College, Sunam, Punjab, India

Date of Web Publication27-May-2013

Correspondence Address:
A Trivedi
Department of Oral Medicine and Radiology, Guru Nanak Dev Dental College, Sunam, Punjab
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-4388.112408

Rights and Permissions

 

   Abstract 

Cherubism is a non neoplastic progressive heredity disease affecting the jaw bones. It is a benign disease with a characteristic symmetrical involvement of the maxilla and mandible. It usually affects children before 5 years of age, with painless progressive swelling of the cheeks, frequently associated with dental malformations. Here, we are presenting a case report of a 12-year-old boy suffering from cherubism.


Keywords: Familial multilocular disease of the jaws, fibrous swelling of the jaws, familial fibrous dysplasia of the jaws, familial multilocular cystic disease of the jaws, hereditary fibrous dysplasia of the jaws


How to cite this article:
Trivedi A, Gupta S D, Sharma S, Mehta R. Nonfamilial cherubism. J Indian Soc Pedod Prev Dent 2013;31:43-7

How to cite this URL:
Trivedi A, Gupta S D, Sharma S, Mehta R. Nonfamilial cherubism. J Indian Soc Pedod Prev Dent [serial online] 2013 [cited 2019 Jul 22];31:43-7. Available from: http://www.jisppd.com/text.asp?2013/31/1/43/112408



   Introduction Top


Cherubism is a benign disease with a characteristic symmetrical involvement of the maxilla and mandible. It was first described by Jones in 1933 as a "familial multilocular disease of the jaws" in three siblings who appeared as though they were "looking towards heaven." This inspired him to call the condition "cherubism", to describe the round appearance of the cheeks, typical of cherubs, resulting from jaw hypertrophy. Affected children usually present before 5 years of age, with painless progressive swelling of the cheeks, frequently associated with dental malformations.


   Case Report Top


A 12-year-old male child of non-consanguineous parents, youngest of three siblings, presented with painless progressive swelling of bilateral cheeks since 2 years [Figure 1]. There was no history of similar disease in any of the siblings or the parents of the affected child. History of presenting illness revealed that swelling was noticed 2 years back as a small-sized swelling present on both sides of the face, which slowly increased in size to attain the present size. There was history of difficulty in chewing due to malaligned teeth. There was no history of any trauma, pain, pus discharge, blood discharge, fever, paresthesia, anorexia and weight loss.
Figure 1: Front view of the patient

Click here to view


Extraoral examination revealed diffuse swelling present on both the right and the left sides of the face. Superoinferiorly, the swelling on both the right and left sides extended below the ala-tragus line up_to the lower border of the mandible and, mesiodistally, starting from the nasolabial fold and going up_to the posterior border of the ramus of the mandible. Skin over the swelling was normal except for the multiple, small papules that were appreciated on the right side of the cheek [Figure 2] and [Figure 3]. On palpation, the swellings were bony hard in consistency and nontender, with no overlying rise in temperature. Single left submandibular lymph node was palpable, measuring approximately 1.5 cm in diameter, firm in consistency, nontender and mobile.
Figure 2: Right lateral view of the patient

Click here to view
Figure 3: Left lateral view of the patient

Click here to view


Intraoral examination revealed that the patient was in mixed dentition stage, with number of teeth present being 21. Fifty-three, 75 and 85 were retained. Occlusion was deranged. Diffuse swelling extending from the 32, 75 area and going up_to the distal surface of 37 was appreciated in the left lower back teeth region with obliteration of the buccal and lingual vestibule with respect to 75, 36, 37. Obliteration of the buccal vestibule was observed with respect to 85, 46, 47. The left side of the palate was obliterated with midline shift [Figure 4],[Figure 5] and [Figure 6]. Inspectory findings were confirmed by palpation and the swellings were bony-hard in consistency.
Figure 4: Midline shift towards right side

Click here to view
Figure 5: Expansion of buccal cortical plate

Click here to view
Figure 6: Midline shift of palate towards right

Click here to view


Based upon the history and clinical examination, a provisional diagnosis of cherubism was given, with the differential diagnosis of mumps, fibrous dysplasia, bilateral masseter hypertrophy, Paget's disease, infantile cortical hyperostosis and human immunodeficiency virus (HIV) parotitis.


   Investigations Top


Blood investigations

Serum calcium, serum phosphate, serum alkaline phosphatase, SGOT, SGPT, HIV, HBV and HCV were conducted and all were within the normal range except serum alkaline phosphatase, which was slightly toward the higher side (134IU/L).

Radiographic investigations

A mandibular cross-sectional occlusal view revealed bilateral soap bubble- like radiolucencies in the posterior region extending from 34 up-to 37 and 44 up-to 47 [Figure 7].
Figure 7: Mandibular cross-sectional occlusal view

Click here to view


The maxillary topographic view revealed similar radiolucency in the left posterior region [Figure 8].
Figure 8: Maxillary cross sectional occlusal view

Click here to view


The panoramic radiography revealed generalized bilateral- multicystic- lesions affecting the mandible and the left side of the maxilla. The condyles and coronoid process on both sides were involved. Multiple impacted teeth were seen within the body of the mandible [Figure 9].
Figure 9: Orthopantomogram

Click here to view


The posteroanterior view of the skull revealed multilocular radiolucencies extending bilaterally on both sides of the mandible [Figure 10].
Figure 10: Postero anterior mandible view

Click here to view


The paranasal sinus view revealed slight haziness in the left maxillary antrum suggestive of left sinus involvement [Figure 11].
Figure 11: Paranasal sinus view

Click here to view


Computed tomography confirmed the presence of multilocular cystic lesions affecting the body, rami, coronoid and condyle of the mandible. Similar lesions were also seen in the left side of the maxilla , indenting the maxillary sinus [Figure 12],[Figure 13] and [Figure 14].
Figure 12: Computed tomography scan – frontal view

Click here to view
Figure 13: Computed tomography scan- 3 D reconstruction

Click here to view
Figure 14: Computed tomography scan – axial scan

Click here to view


Thus, based on these investigations, the final diagnosis of cherubism was made.


   Discussion Top


Cherubism is a disease of childhood that usually presents before the age of 5 years, most often between 12 and 36 months, with males affected more commonly. [1],[2] The exact etiology is unknown, but heredity plays an important role. [3],[4] Mesenchymal alteration during the development of the jaw bones as a result of reduced oxygenetion secondary to perivascular fibrosis has been suggested as a possible pathology. [5] It progresses until puberty, and shows partial or complete spontaneous involution in adulthood. Masticatory, speech and swallowing difficulties are present. The mandibular angle, ascending ramus, retromolar region, and posterior maxilla are the most common sites affected. The coronoid process may be involved, but the condyles are always spared. [6] However, in our case, condyles were also involved. Vast majority of the cases occur only in the mandible, but in our case, the left side of the maxilla was involved. Intraorally, a hard, nontender swelling is palpated in the affected area. Premature exfoliation of the primary dentition occurs and the permanent dentition is often defective, with absence of numerous teeth, displacement of tooth follicles and lack of eruption of those present. [7]

Radiographic appearance

Multiple, well-defined, multilocular cystic radiolucencies of the mandible, less often of the maxilla, with expansion and thinning of the cortical plate, occur with displacement of the inferior alveolar canal. [8],[9],[10] Teeth appear as if floating in the cystic-appearing spaces. Physiologic resorption of the primary root is accelerated, resulting in early exfoliation. Ectopic eruption of some permanent teeth occurs, causing impaction of others.

Lab findings

Serum calcium, phosphorus and alkaline phosphatase usually within normal limits.

Treatment

No active treatment is required in most of the cases. Extraction of teeth in the involved area and surgical contouring of the expanded lesions can be performed after skeletal growth is completed. Radiation therapy is contraindicated in most of the cases. Medical therapy like Calcitonin is also tried, but is without appropriate results.

 
   References Top

1.Victor T, Rogério D, André G, Alexandre B, Cristiane F. Cherubism: Case report and literature review. Rev-de Clín- Pesq- Odontol 2004;45:342-4.  Back to cited text no. 1
    
2.Jain V, Gamanagatti SR, Gadodia A, Kataria P, Bhatti SS. Nonfamilial Cherubism. Singapore Med J 2007;48:e253.  Back to cited text no. 2
    
3.Sarda D, Kothari P. Cherubism in siblings: A Case report. Indian Soc Pedod Prev Dent 2007;25:27-9.  Back to cited text no. 3
    
4.Khalifa MC, Ibrahim RA. Cherubism. J Laryngol Otol 1988;102:568-70.  Back to cited text no. 4
    
5.Ozkan Y, Varol A, Turker N, Aksakalli N, Basa S. Clinical and radiological evaluation of cherubism: A sporadic case report and review of the literature. Int J Pediatr Otorhinolaryngol 2003;67:1005-12.  Back to cited text no. 5
    
6.Gomes MF, de Souza Setubal Destro MF, de Freitas Banzi EC, dos Santos SH, Claro FA, de Oliveira Nogueira T. Aggressive behaviour of cherubism in a teenager: 4-years of clinical follow-up associated with radiographic and histological features. Dentomaxillofac Radiol 2005;34:313-8.  Back to cited text no. 6
    
7.Regezi JA, Sciubba J. Oral pathology: Clinical-pathologic correlations. 2nd ed. Philadelphia: Saunder; 1993. p. 345-54.  Back to cited text no. 7
    
8.Yamaguchi T, Dorfman HD, Eisig S. Cherubism: Clinicopathologic features. Skelet Radiol 1999;28:350-3.  Back to cited text no. 8
    
9.Kalantar Motamedi MH. Treatment of cherubism with locally aggressive behavior presenting in adulthood: Report of four cases and a proposed new grading system. J Oral Maxillofac Surg 1998;56:1336-42.  Back to cited text no. 9
    
10. Beaman FD, Bancroft LW, Peterson JJ, Kransdorf MJ, Murphey MD, Menke DM. Imaging characteristics of cherubism. AJR Am J Roentgenol 2004;182:1051-4.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14]



 

Top
Print this article  Email this article
 

    

 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (5,014 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Investigations
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed2626    
    Printed65    
    Emailed3    
    PDF Downloaded213    
    Comments [Add]    

Recommend this journal


Contact us | Sitemap | Advertise | What's New | Copyright and Disclaimer 
  2005 - Journal of Indian Society of Pedodontics and Preventive Dentistry | Published by Wolters Kluwer - Medknow 
Online since 1st May '05