Patients may look normal in the first years of
life. The initial changes are characterized by unilateral fullness of the
cheeks, most often starting during the second or third year of life (but
later onset is possible). Eventually, both mandibular rami and angles are
involved, along with the maxilla. The growth rate is fastest during the
first 2 years, then slows down and finally regresses during puberty.
Hypertelorism is a constant sign. There is also an association with
hyperplasia of cervical lymph nodes. The teeth are often loose and
irregularly positioned. Extragnathic skeletal involvement is rare.
Conservative management is recommended because it is a benign, self-limited
condition. However, curettage of tissue hindering nasal breathing or
function of the tongue may be indicated. Surgery may be requested for
cosmetic reasons. Patients are mentally normal.