Done by: Zahra Juma Ahmed AlaswadUni num: 201020500792014-2015

CHERUBISMSynonym:Familial fibrous dysplasia is a synonym for cherubism.

Disease mechanism:Cherbisum is rare, inherited autosomal dominate disease that cause bilateral enlargement of the jaws, giving the child a cherubic facial appearance ,these lesion regress with age .

Clinical feature :Cherubism develops in early childhood between 2 and 6 years of age . the most common presenting sign is a painless, firm , bilateral enlargement of the lower face. Profound swelling of the maxilla may result in stretching of the skin of the check which cause an "eyes raised to heaven" appearance .

Imaging feature:Location: this lesion is bilateral and effects both jaws. When present in only one jaw , the mandible is the most common location . the epicenter is always in the posterior aspect of the jaw , in the ramus of the mandible or the tuberosity of the maxilla. The lesion grows in an anterior direction and in sever case can extend almost to the midline .Periphery: the usually is well defined and in some instance corticated.

Effects of surrounding structure: expansion of the maxilla and mandible can result in sever enlargement of the jaw. Maxillary lesions enlarge into maxillary sinuses. The degree of displacement can be sever, and the tooth buds are destroyed with some lesions. Axial CT of the left mandiblur condoyle you can see the sever expansion and the wispy , ill-defined septa.

Panoramic image shows four lesion in the maxilla and mandible .the epicenter of the lesion are in the maxillary tuberosity and mandibular ramus ,also note the anterior displacement of the unerupted maxillary first molars.

Differential diagnosis:1-central giant cell granuloma2-multiple odontogenic keratocysts in basal cell nevus syndrome

Management:Treatment can be delayed because the cytslike lesions usually become static and fill in with granular bone during adolescence and at the end of skeletal growth. After skeletal growth has stopped , conservative surgical procedure maybe done.Portion of the posteroanterior skull view shows expansion of the mandible

PAGET'S DISEASE

Synonym:Osteitis deformans is a synonym for paget's disease.

Disease mechanism:Paget's disease is a skeletal disorder and essentially a disease involving osteoclasts resulting in abnormal resorption and apposition of poor-quality osseous tissue in one or more bones . is seen most frequently in great Britain and Australia and less often in north America . this disease is an autosomal dominant trait with gentic heterogeneity and may involve paramyxoviral infection, but the etiology for the disease remains unclear.axial CT images of a case of pagets disease involving all of the cranial bones as well as the maxilla and mandible .note increase in bone density and dimension between the internal and outer cortex of the skull

Clinical features:Paget's disease is primarily a disease of late middle and long age. Men is approximately twice than women .

the resulting of poor quality of bone formation is bowing of the legs , curvature of the spine , and enlargement of the skull and the jaws . Separation and movement of the teeth. Patients with this disease may also have ill-defined neurologic pain as the result of bone impingement of foramina and nerve canals .elevated levels of serum alkaline phosphate and hydroxylproline in urine.

Imaging featureLocation: is bilateral ,occurs most often in the pelvis femur, skull , vertebrae and infrequently in the jaws . Note it affects the maxilla about twice than mandible.Coronal CT image demonstrates enlargement of the mandibular ramus

Internal structure:Paget's disease has three radiographic stages :1-an early radiolucent resorptive stage

2-a granular or ground-glass appearance

3-denser more radiopaque appositional late stage creating cotton wool appearance. But these stages are less apparent in the jaws.Edentulous mandible involved with Paget's diseaseOcclusal film show the loss of normal outer cortex and the liner alignment of trabeculae

Effects on surrounding structures:

prominent pagetoid skull bones may swell to three of four times their normal thickness.

In enlarged jaws, the outer cortex may be thinned but remains intact.

The lamina dura may become less evident and may be altered into the abnormal bone pattern .

hypercementosis often develops on few or most of the teeth in the involved jaw. This hypercementosis may be exuberant and irregular , which is characteristic of Paget's disease . The expansion of the mandible and the maintence of a the outer cortical plate

Multiple radiopaque masses in the mandible that have a cotton-wool appearance

Differential diagnosis:

Paget's disease may appear similar to fibrous dysplasia.(the enlargement of involved bone ,elevation of serum alkaline phosphate aid in dif..)

Management:

Paget's disease usually is managed medically at the present time, using calcitonin, sodium etidronate, or bisphosphonates more recently. surgery may be required to correct deformities of the long bone and the fractures. Periapical images of Paget's disease showing exuberant irregular hypercementosis of the root.