McCune-Albright syndrome

Alternative names:

Albright syndrome; osteitis fibrosa cystica; polyostotic fibrous dysplasia

Definition:

A disease of unknown cause affecting the bones and pigmentation of the skin, and causing premature sexual development.

Causes, incidence, and risk factors:

The hallmark of McCune-Albright syndrome is premature puberty in the female which may begin at variable ages. Sexual development is disordered in that menses may begin before the appearance of breast or pubic hair development. Early sexual development in the male is less common than the female.

These children have abnormal fibrous dysplasia (abnormal fibrous development) in the bone that can lead to fractures or deformities. Deformity may appear in the facial bones causing cosmetic problems. In addition, the children have cafe-au-lait spots, which may be present from birth. Bone changes in the skull can lead to blindness or deafness.