Hemifacial Hyperplasia

Description

The criteria for the hemifacial type of congenital hypertrophy are (1) unilateral enlargement of the viscerocranium bounded superiorly by the frontal bone (not including the eye), inferiorly by the inferior border of the mandible, medially by the midline of the face, and laterally by the ear, the pinna being included within the hypertropic area, and (2) enlargement of all tissues--teeth, bone, and soft tissue--within this area (Rowe, 1962).

Clinical Features

Rowe (1962) reported 4 cases of hemifacial hypertrophy and reviewed 30 previously reported cases. The left and right sides were almost equally affected. Histologic examination in one case demonstrated that the enlargement resulted from an increased number of cells rather than an increase in cell size.

Burchfield and Escobar (1980) described a family in which several members showed mandibular asymmetry and maxillary hypoplasia. Several instances of male-to-male transmission were observed. This was classified by the authors as facial hemihypertrophy. The proband had deviation of the mandible to the right and 'a severe crippling malocclusion.'

Also see 141350.