Median facial dysplasia in unilateral and bilateral cleft lip and palate: a subgroup of median cerebrofacial malformations.

The median facial dysplasia group of patients is a unique, distinct, definable group characterized by mid-line facial deficiencies in the presence of a unilateral or bilateral cleft lip with or without cleft palate and without clinically detectable anomalies of the brain. This group of patients comprised 2 percent of new cleft lip and palate patients. The midline facial deficiencies in median facial dysplasia are characterized by a poorly defined Cupid's bow, absence of the labial frenulum and anterior nasal spine, deficient columella, and poorly developed septal cartilage and premaxilla. One of the central incisors may be absent or rudimentary. There are no definable gross abnormalities of the brain. The results of intelligence tests of median facial dysplasia patients have a normal distribution and are not significantly different from those of a noncleft normal population. Unoperated and operated median facial dysplasia patients all have typical midfacial growth disturbances below the norm for their ages. The classification of median facial dysplasia patients as well as other median cerebrofacial anomalies, the embryologic basis of these deformities, and the clinical results of their treatment are helpful in understanding some of the problems related to the treatment of the cleft lip and palate patient. Median facial dysplasia patients demonstrate an inherent potential for poor midfacial growth. Deficiencies of soft tissue such as a lack of the Cupid's bow make it difficult to reconstruct the lip and nose satisfactorily.