cherubism is an inherited , fibro - osseous condition characterized by firm , painless swelling of the jaws , and was first described by jones in 1933 .
clinically cherubism is characterized by fullness of the cheeks and jaw bones that results in a round face with retraction of the lower eyelids and exposure of the sclera below the irises ; the 
heaven look  produced is suggestive of a cherub and gave rise to the name of this condition .
the radiographical appearance is characterized by bilateral , multilocular radiolucent lesions , which often begin near the angle of the mandible and spread to the ramus and body of the mandible .
the presence of eosinophilic , collagenous material around small capillaries in histopatholgical sections is of value in the diagnosis of cherubism .
a 25-year - old male patient of non - consanguineous parents reported to our department with a complaint of bilateral painless swelling of both jaws .
later he noticed progressive increase in the swelling in his jaws which continued till it attained the present size .
the size of the swelling had been stable for the past year and had not regressed or increased . on extra - oral examination , the swellings appeared ovoid in shape , with well - defined borders .
the swelling was more prominent in the parasymphysis and body of the mandible and molar regions .
eyes were upturned with sclera visible below the iris giving an  eye raised to heaven  appearance .
there was a difference in the ocular levels , right eye being at a slightly higher level [ figure 1 ] .
obliteration of buccal sulcus of mandible was noticed in the molar regions . in the maxilla multiple swellings
were seen , which were hard , sessile , non - tender , and covered with pink mucosa .
extra - orally the swellings appeared ovoid in shape , with well - defined borders .
the orthopantamograph revealed multiple impacted teeth in the mandible , with multiloculated osteolytic lesions involving the entire body as well as the ramus of the mandible sparing the condyles [ figure 2 ] .
a 3-dimensional computed tomography showed multiloculated cystic lesions affecting the body and rami of the mandible and also the maxilla .
the lesion caused marked expansion of the bones , with a multifocal cortical breakthrough [ figure 3 ] .
the orthopantamograph revealed multiple impacted teeth in the mandilble ( red arrows ) with multiloculated osteolytic lesions involving mandible and maxilla ( blue arrows ) .
3-dimensional computed tomography scan showing multiloculated cystic lesions affecting the body and rami of the mandible ( blue arrows ) and maxilla with raised orbital floor ( red arrows ) .
biopsy to obtain specimen from both the jaws in the alveolar region was performed under local anesthesia.microscopy revealed highly cellular stroma with many multinucleated giant cells .
the cellular stroma showed spindle cell fibroblasts with vesicular nuclei arranged in fascicles , whorled pattern and irregularly in few areas .
the giant cells were unequally distributed and were of different sizes with varied number of nuclei resembling osteoclasts .
there were few blood vessels and areas of extravasated blood and minimal amount of collagen was seen .
microscopy revealed highly cellular stroma consisting of plenty of multinucleated giant cells ( black arrow ) .
the cellular stroma consists of spindle cell fibroblasts with vesicular nuclei arranged in fascicles ( blue arrow ) . the grotesque appearance and the parent 's insistence on esthetic improvement compelled the surgeon to attempt cosmetic surgical recontouring of the jaws under general anaesthesia .
the entire mandible and maxilla was degloved intraorally . the exposed lesion tissue consisted of multiple locules with reddish hue and semi - hard consistency .
buccal surfaces of maxilla and mandible were decorticated and curetted as much as possible to provide optimal cosmetic improvement . impacted and loose teeth were removed .
patient had no recurrence of the lesion during the 2-year follow - up period [ figure 6 ] .
the orthopantamograph revealed multiple impacted teeth in the mandible , with multiloculated osteolytic lesions involving the entire body as well as the ramus of the mandible sparing the condyles [ figure 2 ] .
a 3-dimensional computed tomography showed multiloculated cystic lesions affecting the body and rami of the mandible and also the maxilla .
the lesion caused marked expansion of the bones , with a multifocal cortical breakthrough [ figure 3 ] .
the orthopantamograph revealed multiple impacted teeth in the mandilble ( red arrows ) with multiloculated osteolytic lesions involving mandible and maxilla ( blue arrows ) .
3-dimensional computed tomography scan showing multiloculated cystic lesions affecting the body and rami of the mandible ( blue arrows ) and maxilla with raised orbital floor ( red arrows ) .
biopsy to obtain specimen from both the jaws in the alveolar region was performed under local anesthesia.microscopy revealed highly cellular stroma with many multinucleated giant cells .
the cellular stroma showed spindle cell fibroblasts with vesicular nuclei arranged in fascicles , whorled pattern and irregularly in few areas .
the giant cells were unequally distributed and were of different sizes with varied number of nuclei resembling osteoclasts .
there were few blood vessels and areas of extravasated blood and minimal amount of collagen was seen .
microscopy revealed highly cellular stroma consisting of plenty of multinucleated giant cells ( black arrow ) .
the cellular stroma consists of spindle cell fibroblasts with vesicular nuclei arranged in fascicles ( blue arrow ) .
the grotesque appearance and the parent 's insistence on esthetic improvement compelled the surgeon to attempt cosmetic surgical recontouring of the jaws under general anaesthesia .
the entire mandible and maxilla was degloved intraorally . the exposed lesion tissue consisted of multiple locules with reddish hue and semi - hard consistency .
buccal surfaces of maxilla and mandible were decorticated and curetted as much as possible to provide optimal cosmetic improvement . impacted and loose teeth were removed .
patient had no recurrence of the lesion during the 2-year follow - up period [ figure 6 ] .
no cause and effect relationship with trauma , infection , or hemorrhage has ever been verified .
anderson suggested that a genetically induced biochemical abnormality stimulates the giant cell lesions characteristic of cherubism .
a molecular pathogenesis of cherubism has been proposed ; sh3bp2 gene mutations cause dysregulation of the msx-1 gene , which is involved in regulating mesenchymal interaction in craniofacial morphogenesis .
clinically the lesion of cherubism produces painless , typically slow expansion of the affected area of jaw .
the facial expansion maybe quite mild or may result in considerable deformity . more extensive involvement may result in enlargement and widening of the alveolar bone .
progressive involvement of the anterior maxillary segment bone stretches the skin and the lower eyelid beneath the orbit , exposing the lower sclera .
the developing permanent teeth in the areas of involvement may be displaced , malformed , or absent .
history , clinical course , and the findings in our case corresponds to the classic features of cherubism .
irregular , multilocular , well - defined , translucent , cystic spaces causing expansion of bone and sparing only a thin layer of cortex are usually seen .
the teeth may be displaced , unerupted , or appear to be floating in the cyst - like spaces . in the mandible ,
the inferior alveolar canal maybe displaced and lesion may involve the alveolar process , the angle and ramus .
it may advance toward the incisors and obliterate the sigmoid notch , but the condyles may be spared .
periosteal new bone formation is never present . in the maxilla the lesion is similar and
the maxillary antra may be completely obliterated only to become normally pneumatized as the lesion regress .
seward and hankey have proposed a grading system based on the radiographical location of the lesions in the jaws .
it is as follows : grade 1 : involvement of bilateral mandibular molar regions and ascending rami , mandible body , or mentis .
grade 2 : involvement of bilateral maxillary tuberosities ( in addition to grade 1 lesions ) and diffuse mandibular involvement .
grade 3 : massive involvement of the entire maxilla and mandible , except the condyles .
grade 4 : involvement of both jaws , including the condyles . according to ramon and engelberg grade 4 lesions
our case could possibly be classified as above grade 3 cherubism as there was huge deformity of the mandible , especially the bilateral posterolaterosuperior extension of maxillary lesions with slight elevation of orbital floor .
the lesions of cherubism are not distinctive histologically and are difficult to differentiate from other giant cells containing fibro - osseous lesions , making diagnosis dependent on the clinical findings impossible .
hamner histochemically demonstrated this perivascular cuffing to be collagen protein and felt its presence was of value in the diagnosis of cherubism .
surgical treatment appears to be unnecessary for grade 1 and 2 cases , in the absence of secondary disturbances .
curettage appears to be necessary in more aggressive cases ( grade 3 ) , to reduce maxillofacial deformity that occurs after puberty .
dukart et al . , found that surgical curettage and recontouring performed during a period of rapid growth of cherubism lesions not only offer a favorable immediate result but also arrests active growth of remnant lesions while stimulating bone regeneration .
the rationale of calcitonin administration is that it inhibits the osteoclastic activity of the giant cells .
radiation therapy is ineffective and contraindicated in view of the risk of osteoradionecrosis , interference with dentofacial growth and development , and the effect on future surgical procedure . in our case ,
the patient was an adolescent affected psychologically by his grotesque appearance , which was also a concern for his parents .
therefore , a conservative surgical curettage and recontouring of the jaws was performed under general anesthesia to address his esthetic concern .
two years of postoperative follow - up showed a sustained esthetic improvement achieved by surgery of the grade 3 lesion .
 cherubism is a rare non - neoplastic hereditary disease related to genetic mutations characterized by symmetrically swollen cheeks , particularly over the angles of the mandible , and an upward turning of the eyes . 
 the affected mandible and maxilla begin to swell in early childhood , and gradually increase until the age of puberty . apparently , surgical intervention is unnecessary unless significant functional , esthetic , or emotional disturbances develop . in the present paper , 
 we report a case of cherubism , with classic features that was classified as grade 3 , managed with surgery and followed up for 2-years after treatment . 