Rare Facial Clefts Demystified

Abstract

Rare facial clefts are deformities involving the discontinuity of bones and soft tissues in areas of the face and skull not typically affected by common oral clefts. They can be isolated defects or associated with syndromes. The exact cause of these clefts is not well understood because of their rarity and the complexity of craniofacial embryological development. Tessier descriptive classification system is widely used by surgeons and relates specific anatomical defects to the necessary reconstructive surgery. This system uses the orbit as a primary reference point and numbers clefts from 0 to 14, with an additional number 30 for jaw midline clefts. The surgical treatment of these clefts is challenging because of the almost unlimited number of combinations of defects. There is no standard protocol, and long-term results of surgical techniques are sometimes not well-documented. The main goals of treatment are to restore the craniofacial skeleton and reconstruct soft tissues, focusing on symmetry and placing scars along the lines of aesthetic units.