Case Senario
8 year old boy presented with bilateral painless swelling of both cheeks, noted by his mom over the last 5 months. O/E: migrated retained primary with spacing, no permanent teeth erupted. OPG: radiolucency bilateral body, angle and ramus of the mandible.
Differential Diagnosis
Aneurysmal bone cyst Ameloblastoma Odontogenic keratocyst Central giant cell granuloma Central Odontogenic Fibroma Primary bone neoplasm Acute osteomyelitits of the Jaws Brown Tumor Odontogenic Myxoma Cherubism
Odontogenic Keratocyst
Swelling is the most common presenting complaint; however, Asyptomatic and found incidentally on dental X-rays. Usually Unilateral multiple = Gorlin Goltz syndrome Gorlin Goltz Syndrome
Multiple Odontogenic Keratocyst Basal Cell Carcinoma Skeletal, dental, opthalmic and Neurological Abnormalities Intracranial ectopic calcification of falx cerebri Facial Dysmorphism
Multiple = Ehlers danlos Syndrome: hereditary disorder of the CT related to collagen metabolism
Ameloblastoma
Rare, benign tumor of Odontogenic epithelium most common type of Odontogenic Epithelial Tumor Mandible > Maxilla Presence of unerupted teeth, painless, unilateral Sound of bone cracks when palpated and this phenomenon is referred to as "Egg Shell Cracking" or crepitus, an important diagnostic feature
Brown Tumor
The brown tumor is a bone lesion that arises in settings of excess osteoclast activity, such as hyperparathyroidism. It is not a true neoplasm. However, it may mimic a true neoplasm bone deposition followed by additional resorption can expand beyond the usual shape of the bone, involving the periosteum thus causing bone pain. characteristic brown coloration results from hemosiderin deposition into the osteolytic cysts.
Odontogenic Myxoma
an uncommon benign odontogenic tumor arising from embryonic connective tissue associated with tooth formation. found in patients ranging in age between 10 and 50 years specifically between 25 and 35 years of age Mandible > Maxilla Painless, slowly enlarging expansion of the jaw with possible tooth loosening or displacement "soap bubble" or "honeycomb" appearance
Cherubism
Cherubism is a rare disease of autosomal dominant inheritance characterized by painless, frequently symmetrical, enlargement of the jaws as a result of the replacement of bone with fibrous tissue. The disease is also called familial fibrous dysplasia of the jaws. Cherubism is usually diagnosed in children aged 2 to 7 years, with the observation of exacerbation of its manifestations within the first 2 years after diagnosis and of stabilization or even regression after puberty. Boys are more affected than girls at the proportion of 2 : 1.
Clinical Findings
Clinically, cherubism is characterized by bilateral enlargement of the mandible and/or maxilla, causing a rounded face and swollen cheeks accompanied by upwardlooking eyes. Orbital involvement may appear late in affected individuals. This condition gives the patient the appearance of cherubs. Significant displacement of teeth Failure of permanent teeth to erupt
Radiographic Appearance
Expansion of the buccal and lingual cortical plates is seen on occlusal and postero-anterior views Mandibular body and rami are frequently involved Enlargement of the maxilla is at the expense of the maxillary sinuses
Radiographic Apperance
It is almost always bilaterally symmetrical with an epicentre in the ramus of the mandible or tuberosity of maxilla. Lesions are usuallywell-defined, expansile, with fine trabeculae forming a multilocular pattern
Biopsy
Biopsy and histopathologic examination are not required in most cases to establish the diagnosis of cherubism. However, when performed, numerous osteoclast-like multinucleated giant cells in a moderately loose fibrous stroma are present.
Treatment
Self-limiting condition that improves over time Treatment depends on the individual patients functional and esthetic needs Early surgical intervention is contraindicated because it appears to predispose to recurrences, investigators prefer to wait until the end of puberty Treatment, if necessary, consists of recontouring the bone for cosmetic reasons after the lesions have stabilized in size. Surgery is only indicated in cases characterized by impaired speech, chewing or swallowing difficulties, or with the presence of major deformities that may cause psychological problems for the patient.