Median facial cleft syndrome: A case report

Congenital Cleft Lip and Palate is a common birth defect with an incidence of 1 in 600 to 800 live births. This defect usually affects either the left, right or in some cases both sides of the lip and is called a Tessier Type 3 cleft. Clefting of the face in the midline is exceedingly rare and such a case of a Median Facial Cleft Syndrome is being reported which occurs in nearly 1 in 1,000,000 live births. This was first described by Bechard in 1823 and can be sporadic or part of an inherited syndrome. The child had a median cleft of the upper lip, nose, and palate of a severe, complete variety, with absent philtrum of the upper lip, premaxilla, columella, nasal septum and vomer.

Median Cleft of the Lip: Its Significance and Surgical Repair

Median cleft lip is a midline vertical cleft through the upper lip in the absence of a prolabial remnant. This may occur as a sporadic event or be part of an inherited sequence of anomalies. A failure of formation or fusion of the medial nasal prominences derived from the frontonasal prominence is ultimately responsible for this aberration. Two categories of dysplasia are associated: (1) frontonasal deformity associated with hypotelorism and (2) median facial cleft syndrome associated with hypertelorism. A patient presents with median cleft lip, mild bifid nose, and hypertelorism. Following surgical reconstruction, a good result is achieved. The embryology, implications for associated abnormalities, and surgical technique for treating these cases are discussed.