HEMIFACIAL HYPOPLASIA AND HYPOMELANOSIS OF ITO

We present three cases of hemifacial hypoplasia associated with hypome lanosis of Ito. The facial deformity is often severe with marked soft tissue shortage and underlying skeletal hypoplasia posing difficulty i n reconstruction. The external ear is relatively uninvolved, although a degree of hypoplasia is usually present. The hallmark of hypomelanos is of Ito is linear depigmentation of skin often associated with asymm etric abnormalities. It is a heterogenous disorder due to chromosomal mosaicism, but cytogenetic confirmation of the diagnosis may be diffic ult. The relationship between mosaicism and anatomical asymmetry is di scussed.