Median facial dysplasia: A rare craniofacial syndrome and the surgical management of associated cleft lip
A 9-month-old female baby presented with a false median cleft with hypotelorism, on further examination she had median cleft lip with rudimentary prolabium, poorly defined cupids bow, lack of upper labial frenum, flattened nose with widened nostrils and deficient collumella. The pre-maxilla seemed to be rudimentary in this patient, hence a lack of anterior alveolus was seen, but with no evidence of a cleft palate or alveolus. The dental occlusion of the patient could not be assessed as the teeth had not erupted.\nThe patients’ history revealed that she was a full-term normal delivery, with no known complications during pregnancy and no significant history of teratogenic exposure or familial history of cleft was present. The patient seemed to be of a normal intellect judging by the childs understanding and orientation of surroundings and had her normal milestones achieved with respect to the age. The diagnosis was made in the presence of the typical findings of deficient mid facial structures and absence of any clinically appreciable gross brain abnormalities.\nThe most important factor in reconstruction of the cleft lip in cases of MFD is the identification of the surgical reference points of chelioplasty [] which is a cumbersome process as the columella is deformed or poorly defined, hence the aid of adjacent midline structures such as the midpoint of nasal columella can be used as a guide to locate IS (Cupid bow summit point, Point 1), similarly the CPHR (Cleft Philtrum Horizontal Right, Point 2) is traced towards the non-cleft side, an equidistant point is marked as CPHL (Cleft Philtrum Horizontal Left, Point 3) on the cleft side based on the measurement between IS and CPHR. The CPHL’ (Point 3’) is marked based on the maximum thickness of the vermilion and verified by transferring the measurements of CHR (Cleft Horizontal Right point) -CPHR to CHL (Cleft Horizontal Left point) -CPHL’ []. The measurements of 5A and 5B are also noted for quantifying the shortening of the cleft side lip.\nThe incision using the reference points, C flap is developed till the midline of columella, and a back cut is given hence in that process developing a thick columella by dropping down a thick C flap, this provides the deformed or a poorly developed columella a structure. The reference points are further used as guide for dissection of the muscles and reorientation with its counterpart on the non-cleft side [Figure and ].