|Year : 2013 | Volume
| Issue : 1 | Page : 43-47
A Trivedi, SD Gupta, S Sharma, R Mehta
Department of Oral Medicine and Radiology, Guru Nanak Dev Dental College, Sunam, Punjab, India
|Date of Web Publication||27-May-2013|
Department of Oral Medicine and Radiology, Guru Nanak Dev Dental College, Sunam, Punjab
Source of Support: None, Conflict of Interest: None
| 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
| Introduction|| |
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|| |
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.
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.
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.
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|| |
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).
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].
The maxillary topographic view revealed similar radiolucency in the left posterior region [Figure 8].
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].
The posteroanterior view of the skull revealed multilocular radiolucencies extending bilaterally on both sides of the mandible [Figure 10].
The paranasal sinus view revealed slight haziness in the left maxillary antrum suggestive of left sinus involvement [Figure 11].
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].
Thus, based on these investigations, the final diagnosis of cherubism was made.
| Discussion|| |
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. , The exact etiology is unknown, but heredity plays an important role. , 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.  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.  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. 
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. ,, 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.
Serum calcium, phosphorus and alkaline phosphatase usually within normal limits.
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.
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[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]