Anatomic landmark approach to reconstruction of asymmetric midline cleft lip due to Pai syndrome

Midline clefts of the upper lip are rare, and it is therefore important that surgeons have access to a methodical approach for when these presentations are encountered. We adapted principles of the anatomic subunit approximation for unilateral cleft lip, to the repair of midline clefts. The overt use of anatomic landmarks to define the repair results in a design that inherently adjusts to varying degrees of clefts and can accommodate asymmetries. The “measure twice, cut once” style is an advantage to new surgeons and to surgeons who seldom encounter this presentation. We describe the details of surgical repair in the context of a patient with Pai syndrome and associated nasal hamartomas that resulted in nasolabial asymmetry. This is the first report of surgical outcome following treatment of Pai syndrome and includes early and 5-year follow-up. The system of repair that we describe is applicable to both symmetric and asymmetric midline clefts.