the face being the representation of an individual , any defects associated with it is amplified to affect the individual suffering in various aspects of life . in the various spectrums of defects affecting the maxillofacial region congenital defects are a group of disorders that affect the individual since birth and the afflicted person struggles with its sequel and its treatment phases almost all throughout their life . cleft lip and palate patients are such individuals who spend almost their entire childhood and a significant portion of their early adulthood in obtaining and undergoing treatment for their condition . these patients undergo a significant amount of physical and psychological difficulties during the course of their treatment . a sub group of these cleft lip and palate patients is the median facial dysplasia ( mfd ) , it has an incidence rate of 2% which was first described by noordhoff and cheng , this incidence rate was stated by them in their extensive study , they also described this subgroup in detail as having a summation of characteristic craniofacial defects which include a short prolabium , absent cupid 's bow , absent labial frenum , hypoplastic premaxilla , absent maxillary central and lateral incisor on cleft side , deficient septal cartilage , and nasal spine . a unique feature is the absence of any gross brain abnormalities , which is the distinguishing feature of this condition from holoprosencephaly sequence . 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 . the 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 . the most important factor in reconstruction of the cleft lip in cases of mfd is the identification of the surgical reference points of chelioplasty [ figure 1a ] 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 [ figure 1b ] . the measurements of 5a and 5b are also noted for quantifying the shortening of the cleft side lip . ( a ) normal chelioplasty markings , ( b ) markings for chelioplasty of median facial dysplasia the 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 2a and b ] . ( a ) pre - operative left unilateral median facial dysplasia cleft lip , ( b ) post - operative view the middle third of the face is the most complex skeletally , composed partly of the skull base and incorporating both the nasal extension of the upper third and part of the masticator apparatus . mfd embryologically occurs due to defects in the development of the median portion of anterior neural plate . in the 3 week of gestation , the primitive tissues of trilaminar embryo give rise to the notochord and pre - cordal mesoderm . at the same time these tissues are responsible for the ultimate development of the brain and midline facial structures . pre - cordal mesoderm has a dual role , its first role is in formation of the median skeleton of face and second role is in induction and differentiation of rostral neural ectoderm and thereby prosencephalic development . therefore , whenever pre - chordal mesoderm is defective mid facial and prosencephalic development may be arrested at any stage , a developmental error at this phase results in holoprosencephaly and mid facial defects . the etiology of mfd has not been identified , which is the case with any rare disorder a hypothesis has been suggested which could be mechanical , genetic or environmental teratogens that can cause defects in pre - chordal neural crest cells which can arrest development of midline structures and prevent organogenetic cleavages of the prosencephalon . the diagnosis of mfd is established when a patient presents with mid facial deficiencies in a unilateral or a bilateral cleft lip with or without accompanying cleft palate , and without any clinically discernable cerebral anomalies . these patients of mfd have a normal intelligence level but have poor potential for midface growth . noordhoff and cheng initially coined the term median facial dysgenesis , this term was later changed to mfd because dysplasia means developmental malformation while dysgenesis suggests abnormal morphogenesis . van der meulen classified these malformations based on embryology and the region involved , he also recommended that the term dysgenesis should be changed to dysplasia and the terms median and cerebro - facial were appropriate for regional orientation . noordhoff and cheng in their landmark series of more than 2900 patients of cleft lip and palate over a period of 14 years in the taiwanese population had identified 59 patients with varying degree of mfd , following which the same population group were evaluated for maxillary growth pattern by liao et al . amongst the various indian literatures on cleft lip and palate only a single case report by aggarwal identified a total of three cases of mfd , but with no further information on the management of the associated cleft lip / palate or other related facial defect in the report . the identification of this sub group of cleft lip and palate is important as they pose a problem in reconstruction ; the role of the chelioplasty procedure is very important and has to be performed with meticulous accuracy to properly approximate the sparse mid facial soft tissue structures .
median facial dysplasia ( mfd ) is a distinct and unique disorder of the craniofacial region that is characteristic of deficient mid facial structures with the addition of a unilateral or bilateral cleft lip with or without a cleft palate . 
 a cleft lip which is associated with mfd whether it is unilateral or bilateral does not represent a typical cleft lip and poses some challenges in reconstruction . 
 the management of such cleft lip has been rarely discussed in previous literatures ; the main obstacle comes in identifying the anatomical landmarks to establish an esthetic reconstruction of the lip . 
 however , reasonably good result can be achieved when the adjacent and distant anatomical structures are correlated to aid the reconstruction .