Single stage repair of the median cleft lip deformity in holoprosencephaly

A single stage repair of the median cleft lip in holoprosencephaly is presented. The use of a superiorly-based pedicle from the nasal lobule/columellar remnant for columellar creation and a free skin graft from excess horizontal lip tissue for philtral reconstruction, in addition to medial rotation of the lateral lip segments, permits complete repair in one procedure. Such an approach offers the advantage of a reduction in number of procedures and anaesthetic exposure in the severely medically compromised holoprosencephalic patient.