Definition and clinical picture
A not uncommon familial, presumably autosomal-dominant disorder almost exclusively affecting the jaw bones in children between the ages of 2 to 4 years.
Symptoms: Symmetric maxillary and mandibular enlargement. The alveolar processes are extremely thickened resulting in disturbed occlusion, displacement of tooth germs, and delayed tooth eruption. Facial expression reminiscent of baroque cherubs with upward gaze due to elevation of the orbital floors.
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Proof - Histopathological findings
Radiographically: bony enlargement with cystic cavitation is pathognomonic.
Histologically: Vascular and fibrous connective tissue with numerous multi-nuclear giant cells without bony trabeculae.
Above all, fibrous dysplasia and other "granulomatous jaw disorders" (histologically
Treatment and prognosis
There is a gradual reduction in the rate of bony enlargement towards puberty, with the lesions disappearing slowly after puberty. If the deformity is significant, surgical treatment may be necessary: curettage and bony remodelling. Abnormal tooth positions will require orthodontic treatment
Horch, H. H.
Praxis der Zahnheilkunde 10,1