Atypical facial clefts: Tessier number 3 and 4 clefts.

PURPOSE OF REVIEW: Tessier number 3 and 4 clefts result from failed fusion of facial processes during embryogenesis, and cause functional, psychosocial, and cosmetic morbidity. Given their rarity and heterogeneity, they present a unique challenge to the reconstructive surgeon, with limited literature for guidance. The purpose of this update is to summarize Tessier number 3 and 4 clefts with a focus on recent literature and expert opinion. RECENT FINDINGS: The incidence of atypical facial clefts has been estimated between 1.4 and 4.9 per 100 000 live births. Several retrospective chart reviews have been published in recent years; however, the epidemiologic data remains limited. Surgical management must be individualized and guided by classic reconstructive principles. The goal of surgery is to return the three soft tissue components (lip, nasomalar, and eyelid) to their proper anatomical location. SUMMARY: Tessier number 3 and 4 clefts are rare, demonstrate a wide spectrum of clinical presentation, and remain challenging to gain a breadth of experience for any single surgeon. They are classified based on their location along well defined anatomical axes. Component repair is performed with attention to the lip, nasomalar, and eyelid regions to restore facial symmetry and function.