MIDLINE DEFECTS OF THE OROFACIODIGITAL-SYNDROME TYPE-VI (VARADI-SYNDROME)

The orofaciodigital syndromes (OFDS) represent a spectrum of anomalies oi the palate, cranium, hands, and feet. Varadi syndrome, designated OFDS type VI, is a rare disorder that is additionally characterized by cerebellar anomalies. the following report is of a patient with OFDS VI and characteristic multiple midline defects: median deft lip and pa late, lingual cleft with nodules, and midline brain malformation. In a ddition, this case is uniquely associated with the presence of midline (metopic and sagittal) craniosynostoses as well. It is unusual that d eformities which result from premature fusion of cranial vault sutures would appear synchronously in a syndrome based on the concept of fail ure of fusion or coalescence of facial growth centers. The midline rep resents an independent developmental field, whereby CNS defects and mi dline anomalies can present concurrently.