Etiology • Autosomal-dominant, fibroblast/giant cell–containing
condition • May be secondary to somatic mutation, mapping
to chromosome 4p16.3 • No associated metabolic or biochemical alterations noted • Possible linkage/association with Noonan’s syndrome
condition • May be secondary to somatic mutation, mapping
to chromosome 4p16.3 • No associated metabolic or biochemical alterations noted • Possible linkage/association with Noonan’s syndrome
Clinical Presentation • Early signs in childhood • Bilateral, symmetric enlargement of mandible • Maxillary involvement less common and less prominent • Dental arch/occlusal discrepancies may be noted. • Unerupted teeth often noted • Facial features include lower-third fullness and scleral
exposure at a forward resting gaze.
Radiographic Findings • Symmetric, multiloculated, expansile radiolucencies of
mandibular body and ramus • Impacted/displaced teeth common • Thinned cortices with scalloped medullary margins • Older patients may exhibit maturation with bone fill in some
areas but with preservation of expanded bony profile.
Diagnosis • Clinical appearance • Radiographic findings
Differential Diagnosis • Central giant cell granuloma (multiple) • Fibrous dysplasia • Langerhans cell disease (histiocytosis X) • Hyperparathyroidism • Multiple odontogenic keratocysts
Treatment • Variable, ranging from cosmetic recontouring to local curettage
early in lesion development • Active surgical intervention should be deferred until after the
pubertal growth spurt, if possible.
Prognosis • Stability usually noted by end of skeletal growth • Often regresses into adulthood, but variably so
early in lesion development • Active surgical intervention should be deferred until after the
pubertal growth spurt, if possible.
Prognosis • Stability usually noted by end of skeletal growth • Often regresses into adulthood, but variably so