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, d efinable group characterized by midline facial deficiencies in the pre sence of a unilateral or bilateral cleft lip with or without cleft pal ate and without clinically detectable anomalies of the brain. This gro up of patients comprised 2 percent of new cleft lip and palate patient s. The midline facial deficiencies in median facial dysplasia are char acterized by a poorly defined Cupid's bow, absence of the labial frenu lum and anterior nasal spine, deficient columella, and poorly develope d septal cartilage and premaxilla. One of the central incisors may be absent or rudimentary. There are no definable gross abnormalities of t he brain. The results of intelligence tests of median facial dysplasia patients have a normal distribution and are not significantly differe nt from those of a noncleft normal population. Unoperated and operated median facial dysplasia patients all have typical midfacial growth di sturbances below the norm for their ages. The classification of median facial dysplasia patients as well as other median cerebrofacial anoma lies, the embryologic basis of these deformities, and the clinical res ults of their treatment are helpful in understanding some of the probl ems related to the treatment of the cleft lip and palate patient. Medi an facial dysplasia patients demonstrate an inherent potential for poo r midfacial growth. Deficiencies of soft tissue such as a lack of the Cupid's bow make it difficult to reconstruct the lip and nose satisfac torily.