Congenital isolated absence of the nasal columella : Reconstruction with an internal nasal vestibular skin flap and bilateral labial mucosa flaps

Congenital aplasia of the nasal columella is a very rare anomaly. The deformity is characterized by the isolated absence of the columella from the nasal tip to the root of the philtrum, including the medial crura of the alar cartilages; surrounding structures such as the septum, nose, and upper lip are normal. To the best of our knowledge, only four such cases have been described to date. The embryopathogenesis for this uncommon disease is presently unknown. Our report describes a 14-year-old girl with congenital agenesis of the columella as an isolated anomaly. Her family history was positive for the presence of the same congenital deformity, which also affected her older brother; there was, however, no consanguinity between the parents. The columella defect was reconstructed with an internal nasal vestibular skin flap and bilateral upper labial mucosa flaps. There are many techniques available to repair columella defects, including free grafts from the ear, local flaps from the forehead, face, upper lip, and nose, distant flaps such as tube pedicle flaps, and free flaps from the ear. Each of these techniques has advantages and disadvantages. Because of this, the treatment of columella defects should be individualized.