Partial Gigantism of the Face with Neurologic Complications
Although it is a rather rare “embryonic error,” congenital unilateral hypertrophy of the tissues of the face has been described in the medical literature at frequent intervals since 1836. Werner1 in 1905 gave this subject special attention when he reviewed a case of his own and reported his findings on histologic examination of the soft tissues involved in the hypertrophy. A year later Pagenstecher2 described a patient with this deformity and, on performing a cosmetic operation, found that the enlargement involved not only fat and connective tissues but the salivary glands, the mucous membranes, and even the external maxillary artery and facial nerve.
More recently Rushton3 pointed out that the structures chiefly affected in partial gigantism of the face are among those derived from the first branchial arch, but other parts may be involved, such as the nostril, the upper jaw, the zygoma, the frontal bone, and the ear. The increased size of the teeth arising from the latter maxillary process in contrast with those developing from the premaxilla is invariably noted in this condition and should aid in differential diagnosis, for no such dental disparity occurs in simple hyperplasia or in tumors.
It is generally agreed that in this disturbance the involvement is never restricted to one type of tissue or to one organ. In a sense partial gigantism of the face is the antithesis of hemiatrophy of the face, and a neurotrophic influence may be present in both. However, Werner1 emphasized that the hypertrophied regions do not correspond. . .