Denouement and Discussion
Oral-Facial-Digital Syndrome
Manifestations
Facial characteristics include shortening of the outer portion of the nostrils and nasal septum, lateral displacement of the inner canthi, broad nasal bridge, evanescent facial milia, frontal bossing, and flattening of the mid-portion of the face. Oral manifestations consist of cleft tongue and a pseudocleft in the middle of the upper lip. The hard palate is cleft laterally at the bicuspid level into an anterior and posterior segment. In addition, there is often a complete but asymmetrical cleft of the soft palate. Thickened, hyperplastic frenula traverse the upper and lower mucobuccal folds and obliterate the mucobuccal sulcus at several sites. The tongue is lobulated and may be bifid, trifid, or tetrafid, and the frenum is short and hypertrophied. Small, pedunculated, whitish, hamartomatous masses are attached to the dorsum of the tongue and, in some cases, the tongue is incompletely differentiated from the normal tissues of the