Familial Multilocular Cystic Disease of the Jaws

Familial multilocular cystic disease of the jaws, the title of this paper, is a provisional name, given to describe an unusual condition occurring in three children, members of the same family, of the Hebrew race, aged six, five, and four years (Fig. 1). It is characterized by marked fullness of the cheeks and jaws and a slight upward turning of the eyes, revealing white lines of sclera beneath. There is also noticeable swelling of the submaxillary regions. The full round cheeks and the upward cast of the eyes give the children a peculiarly grotesque, cherubic appearance. As a matter of fact, they are active, intelligent children, showing a child's normal interest and curiosity as to their surroundings. Both jaws are felt as hard, protuberant masses bulging outward to form a bilateral painless swelling of the face. The teeth are irregularly placed and many are missing. The oldest boy possesses only nine teeth at present, while the little girl has ten. The alveolar ridges are extremely wide, and in the upper jaw this gives rise to a narrow V-shaped palate. There is no evidence of local infection in the mouth or nose, and the tonsils are not enlarged. The submaxillary lymphatic glands are chronically enlarged in all three children. At the present time the greatest degree of enlargment is seen in the youngest. Five months ago that distinction belonged to the little girl.