52. The Surgical Principles of Treatment of a Cleft Lip (unilateral)
- Introductory note: The formation of the Cupid’s bow with a dimple on top and the two columns on its either side has remained an embryological mystery. Perhaps at some time in the future a continuous, safe, real time monitoring of the foetus in its first three months may reveal the way it forms. Recently a craft has landed on the head of a comet to find out about its structure and also its origin. One wonders what will be unveiled first, the origin of the comet or the formation of the Cupid’s bow. If a comet has fascinated mankind in general the Cupid’s bow has fascinated artists across the ages. In recent times plastic surgeons have surgically altered the upper lip to improve the Cupid’s bow and the central tubercle (Fig. 1-4) (please see para 2). But alas historically surgeons who attempted to surgically treat congenital clefts of the lip paid scant attention to the Cupid’s Bow and the dimple on its top as they went about repairing this defect, until Millard came on the scene (please see paragraph 3).
Some interesting questions may be pertinent. Does a hare after which the deformity was named because of its exposed pre-maxilla look ugly? Does the innocent smile of an infant with a cleft enchant the observer? And lastly, did Mona Lisa as drawn by Da Vinci have a proper Cupid’s bow with an attractive dimple on top? Or is it her enigmatic half smile that bewitches us? As is said, beauty is in the eyes of the beholder (Fig. 5-7). But we must not digress because clefts of the lip need treatment. The following paragraphs restrict themselves to the surgical treatment of the congenital unilateral clefts of the lip.
- Anatomy: In addition to the Cupid’s bow, there are other features to the upper lip which need to be noted. As mentioned earlier, a white line runs along its upper border which dips as it courses along the Cupid’s bow. The mucosa of the lip has a brown and a red part. The brown part is comparatively dry and the red part is wet because the population of minor salivary glands is higher in this area. The brown part occupies most of the visible part of the upper lip (Fig. 8) but yet is commonly called vermilion (red) for convenience. In modern women this coloration is almost never visible because of the use of lipstick (Fig. 3, 4). Also in the centre of the brown part (vermilion) halfway up its breadth lies a tiny prominence called a tubercle which varies in size in each individual. The dimple between the two columellar pillars in males usually has fewer hair follicles as compared to the rest of the lip (Fig. 8). In unilateral clefts the white line which borders the cleft both on the cleft and the non-cleft side becomes indistinct in its upper part nearer the nose (Fig. 10). These features are important and must be borne in mind during reconstruction. The orbicularis oris muscle which lies horizontally across the normal lip (Fig. 9) runs obliquely on either side of the cleft including in the vermilion and gets attached to the nearest fixed point usually the periosteum of the alveolar bone (Fig. 10). In restoring the muscles horizontally it needs to be detached from this fixed point when an operation for cleft is performed. The nose on cleft side is flared and droops to varying degrees depending upon the severity of the cleft (Fig. 10). The detailed nature of this nasal deformity is discussed in the next chapter of this blog.
- History: Millard (USA) in 1958, in a general surgery journal at that, first explained that it was not necessary to impinge on the area of the central dimple on the non-cleft side, where two of the three landmarks, that is the highest point of the Cupid’s bow on the non-cleft side and its lowest point, are present and intact. A curved incision high up under the columella from an imagined equidistant third point of the Cupid’s Bow (see fig. 10) of the nose will drop the lip to its horizontal position, albeit creating a defect in the process under the columella. This defect can then be filled by advancing the skin and muscle from the cleft side. The method is popularly called as rotation advancement technique because the non-cleft side is rotated down and the skin on the cleft side is advanced (Fig. 11 and 13). This was a big change from the methods practiced till then, those of Le Mesurier (Canada) which brought in a quadrangular flap to fill the defect made in the philtral area very near the vermilion above the white line, or that of Tennyson (USA) which brought in a triangular flap in the same area (Fig. 12, 14, 15). Earlier to that the cleft was closed by just paring the edges (Rose, England) or, at the most, the Cupid’s bow was marked but no great attention was paid to rotating it or bringing it down at the time of paring the cleft edges and suturing them (Peet, England).
- Rotation Advancement Technique: Basic Steps (Fig. 18-23)
- The problems with the Rotation Advancement Technique: Millard’s rotation advancement technique first met with resistance as usually happens when any new method is introduced and was then grudgingly accepted. Millard, an understudy of the legendary Sir Harold Gilles for some time then set about with Messianic zeal to prove that his method was a panacea for all manner of unilateral clefts through his book Cleft Craft, vol. 1 (Fig. 24-25). This unusual and extraordinary book is fascinating among other things because Millard noticed the difficulties that he encountered in some of his cases and how he set about to sort them out. The problem was, while his method of rotating the lip by an incision high up under the nose was rational and aesthetic, enough material was not always present on the lateral or the cleft side to fill the defect particularly in its vertical dimension. Therefore he extended the advancement flap into the nose and borrowed some skin from the nostril floor or included a part of the alar base to increase the vertical dimension of the advancement flap. Additionally the point on the white line on the cleft side can be marked a few millimeters away to increase the height on the lateral or the cleft side (Fig. 26-29). All this in popular parlance is called ‘cheating’ but is helpful in filling the defect created on the non-cleft side but takes away from the exact geometry that was the basis of the rotation advancement technique and highlighted an element of deficiency on the cleft side.
- Reconfiguration of the abnormal muscles: Irrespective of what method is employed to fill in the defect on the non-cleft side to bring the lip to its horizontal position, the flaps used for filling this defect, however small, are muco-musculo-cutaneous flaps (this was many a year before a musculo-cutaneous flap was formally described). The consequences of the interposition of these flaps was vividly illustrated by Millard himself with drawings in which it was apparent that the abnormality of the oblique disposition of the orbicularis muscle in clefts was best corrected with his rotation advancement technique (Fig. 30-33).
- The increase in the height on the cleft side. The potential of a quadrangular (Le Mesurier) or a triangular flap (Tennyson) from the lateral or the cleft side is much greater for substituting tissue to fill the defect created on the non-cleft side, because the vertical height of these flaps is converted into the horizontal (Fig. 34-35). Whether the interruption of these small flaps mars the function of the muscle in a reconstructed lip remains a moot point.
What is more, because the flaps are located immediately above the Cupid’s Bow, the bow cannot peak or does not tend to return to its oblique position because of the interposition of the flap. In the rotation advancement technique the scar leads straight from the nose to the lip margin and may contract aided sometimes by lack of tissue on the lateral or the cleft side. It would seem that many surgeons now tend to get the best of both worlds by performing rotation advancement technique and also introducing a small triangular flap just above the Cupid’s Bow (Fig. 36).
- The back-cut and the release of the frenum. While the oblique disposition of the orbicularis oris on either side of the cleft are shown in figures in many a book dealing with the subject of clefts, the figures do not happen to be identical. For example, the figures in Millard’s Cleft Craft and in the book Management of Cleft Lip and Palate edited by ACH Watson are an example of this variation (Fig. 37-38).
Also the dynamic effects of the release of muscle from its abnormal attachment is not predictable and the severity or the lack of the obliquity of the Cupid’s bow is not really a guide to the amount of release that a given incision will achieve as marked on the skin. The rotation advancement technique of Millard is therefore fittingly called the ‘cut as you go’ technique in which the defect is created first, if necessary with a back cut (Fig. 39), if and when required. However in a patient placed horizontally the Cupid’s bow does not drop by gravity and its newly created horizontal position is subjective and is aided by holding the release tissue with a stitch or a forceps and dragging it to find out if it offers any resistance. Notice figures 20, 21 (case 1) in this chapter to show how the lip curls back in spite of a recent release. Most literature on the subject fails to mention if the frenum is included in releasing the lip from its oblique position (Fig. 40).
It is a common experience of several plastic surgeons who the compiler of these short notes interviewed, that cutting the frenum has a dramatic effect on the release of the abnormally oblique Cupid’s bow. This stands to reason because the frenum constitutes the muco-muscular component of the oblique deformation.
- Matching the vermilion: In cases of clefts the interrupted vermilion lies on either side of the cleft. Theoretically they together should be equal to the thickness of the normal lip in the centre. But during the formation of the cleft that arithmetic may not always turn out to be true. The white line too shows inconsistency as it fades away while ascending to the nose. When the lip is pared, the additional vermilion on either side of the cleft remains attached to the two sides at the lower end as it comes down and is used to reconstruct the central third of the lip. Generally this vermilion tissue appears greater on the cleft side as it gets bunched up hanging without any support. The vermilion on the medial or the non-cleft side in unilateral clefts is however anchored in the philtral area and therefore appears thinner. The general tendency is to use the lateral or the cleft side vermilion to give adequate thickness in the centre of the lip by going across the midline to bolster any deficiency if present below the Cupid’s bow (Fig. 41,42). Occasionally the excess vermilion is de-epithelialised and buried to give bulk to the lip (Fig. 43). In a minority of cases the medial or the non-cleft side vermilion is in excess and is pulled across to the lateral side (Fig. 44). Either way a criss-cross scar results beginning with a V in the Vermilion then a triangular flap above the Cupid’s bow and the V shaped advancement flap high up under the nose.
- Case 1: Operative steps for matching the vermilion and post-op results
- The 1 mm white line flap. Millard however disagrees and objects to these local maneuvers and insists that symmetrical cylinder shaped well matched parts of the vermilion are always available and a 1 mm flap of the white line can be employed across the point at the mucocutaneous junction of the repair to camouflage the discontinuity at that point.
- The C flap. While repairing a unilateral cleft lip by the rotation advancement technique a flap is created near the columella of the nose based superiorly (Fig. 51). Traditionally it is called a C-flap because the two main flaps, rotation and advancement are A and B. This flap is variable in size depending on the nature of the cleft. Originally in the Millard rotation advancement technique a part or the whole of the flap was used across the nostril floor above the advancement flap but later Millard used it to increase the height of the columella on the cleft side the rationale being that part of the columella on the cleft side is short. Whether this deficiency is apparent or actual is a matter of debate. Be that as may, if that flap is partly or fully used, in the nostril floor then altogether more than one Z-plasty is created along the newly created column on the cleft side and probably helps to restrict the contraction of the resultant scar (Fig. 52).
- Primary treatment of the nasal deformity. Having discussed an outline of the repair of the cleft vermilion and the skin (and muscle) a few lines are added here about the correction of the nasal deformity in unilateral clefts at the time of the primary repair. The subject will be discussed in detail in the next chapter. Broadly speaking the wider the cleft, the greater is the nostril flare. Also if the cleft extends through the alveolus and beyond, the alar base may be placed posteriorly because the alveolar arch might have collapsed. It appears certain that the rotation advancement technique (of Millard) is best suited to correct the nasal deformity because the nose can be moved medially together with the advancement flap which is also moving in the same direction. Though there is an incision along the upper part of the advancement flap and this flap and the nose do not remain a single unit as they move, this incision is helpful in dissecting out the abnormal position of the ala to free it and then move in the same direction as the flap (Fig. 53-62).
- Case 2:
- A combination of more than one method. The preceding paragraphs only glimpse at the problems surrounding the surgical treatment of a unilateral cleft of the lip. Each cleft is different and the defect created in the process of bringing the Cupid’s Bow to its horizontal position varies in its dimension. The variation is present on the cleft side as well in terms of the tissue available to fill the aforementioned defect. While it is ideal that the defect is created under the columella and is then filled at that location as in the Millard technique this ideal may not always be feasible, forcing the surgeon to create a defect lower down above the Cupid’s bow and fill it with a triangular flap from the lateral side. If this is not done primarily it may be needed at the time of the secondary repair, when the Cupid’s bow gets pulled upwards (called peaking) either by way of a scar contracture or because of shortage in substituting the tissue from the cleft side.
- Discussion. Nitin Mokal, a leading craniofacial and cleft surgeon in India is on record to have stated that there is not an operated cleft lip that he has seen which cannot be improved with a secondary correction. That is perhaps why books or chapters dealing with the subject have more pages on treatment of secondary deformities rather than the primary repair. The probable exceptions are some very minor clefts or those operated clefts where patients are asked if they had been in an accident involving the face. The latter question is somewhat satisfying to the plastic surgeon who dreads what is called the “post-cleft lip surgery face”. The reasons for this state of affairs can perhaps be summarized as below:
- The markings for surgery in clefts follow what is known as plane geometry. The reality is in fact three dimensional and Euclidean. Just to give two examples, the prolabium on the non-cleft side sits atop a convex structure called the pre-maxilla and in many clefts the lateral or the cleft side lies posterior to this prolabium because the alveolar arch on that side is collapsed and takes some time to come to a proper alignment. In many cases this perfection is never achieved, not for lack of effort but because of inherent deficiency.
- To add to the Euclidean dilemma, surgery is performed on a structure which encloses a dynamic element, that of a muscle. The release of an abnormally disposed muscle creates unpredictable defects which do not necessarily match surface markings. Also the released muscles do not always give up their inherent spring like the perpetually curved tail of the dog creating post-operative pulls which cannot be predicted.
- The responsibility of filling defects on the non-cleft side when it is brought to its horizontal position falls on the cleft side or the lateral side which is likely to be deficient to start with. After all, clefts are caused by failure of fusion but this failure may be caused by an inherent deficiency. On this background to stick to only one method under all circumstances can lead to unfavourable results. Surgeons must be flexible and resort to more than one method at different times to create a pleasing, post-operative lip.
- As the child grows both deficiency and distortion may get magnified. This is particularly true of the nose in which the septum can rarely (and justifiably) be treated in childhood. The deformation of the septum has a cascading, deforming effect beginning with the dome, then up to the base of the alar cartilage which might be sitting on a deeper or posterior foundation,that is on the posteriorly displaced alveolar arch.
- While in the post-surgical appearance the scars in the skin may attract attention, mobilization of the lateral or the cleft side particularly in wide clefts and then suturing of muscles under tension also exacerbates scars which may mar results.
- In treating clefts of lips surgeons probably try to create the perfect standard lip. What is actually going to happen is, these babies are going to resemble the faces that they have inherited from their parents as time goes by.
- Some case studies: Case 3
- Postscript: The results presented above are random cases from the practice of the compiler of these short notes. Fortuitously these patients presented during the time this chapter was being written and a byline is therefore added at the end of this chapter. All photographs of the results are taken by the parents of the patients with their phone cameras and are therefore not dressed up. These results cannot by any means be called perfect and are probably not very different from cases operated elsewhere in India. They are however inferior to the results shown in international meetings by leaders in this field. Two alibis or excuses can be given in this regard. For one, the orthodontic corrections in those cases are far superior to those in India and therefore the operated lips sit on a better platform. The other reason is the use of splints in the nostrils which improve or rather do not allow deterioration in the results of primary cleft lip rhinoplasties. The introduction of passive, slow, naso-alveolar moulding pre-operatively (as opposed to rapid pre-surgical infant orthopaedics) are now dramatically improving the pre-surgical condition of the cleft in terms of width and therefore the consequent dissection at the time of surgery is reduced and leads to less scarring and tension. All this is expected to be covered in the next chapter. It also has to be admitted that this chapter includes only two basic surgical techniques, a triangular flap in the upper one-third of the lip (rotation advancement) or in the lower one-third of the lip (Tennyson). The number of variations described by surgeons of these two methods is mind boggling and the proverb ‘each unto his own’ probably applies most to surgeons in this field. There are any number of surgeons who are known to say “I use a slightly different flap here which has improved my results”.
- Other important information. Clefts of the lip are best operated as early as feasible and the old rule of around 100 days after birth with a hemoglobin level of 10 gm and a weight of 10 pounds (between 4-5 kg) is quite safe. By this time the child is out of the neonatal period of adjustments to the extra-uterine environment. In those cases where the cleft has been diagnosed during pregnancy, counseling helps a great deal in convincing parents that a good outcome is almost always possible with proper all round care and surgery. Many a time only a cleft of the lip is seen in the ultrasound examination and the parents must be informed that a cleft of the palate may accompany the cleft in the lip not picked up by the ultrasound. In syndromic clefts, patients might opt for an abortion depending on the anomaly and here a clinician should take a somewhat neutral position explaining in detail what lies in store for the child and the parents. Ethical and religious considerations also play a part here. Genetic counseling is available at various centres and can help parents make a decision particularly in syndromic cases. If in clefts of the lip alone feeding is not a problem, either directly from the breast or with supplementary top-up feeds. Clefts of the lip and the palate, both unilateral and bilateral, pose some problems which can be surmounted first by giving small feeds at frequent intervals (dropper or a nozzle). Breast milk can be expressed and given in this manner and, if it is inadequate, can be supplemented by a formula feed. At all times a pediatrician must be involved in the treatment of the baby for its general health and to help with the nature and quantity of feeds as well as to plan for the immunization schedule so that surgery can be planned. In a vast majority infants adjust splendidly to their feeding schedule. The mothers are taught in advance about waiting for the baby to burp before the next feed and also put the baby in the prone position and even holding it by its legs with the head down if there is coughing, gagging or vomiting. One of the principal reasons for early surgery when clefts extend into the palate is that the restoration of the muscular sling of the orbicularis oris helps mould the alveolar arch on the cleft side and reduces the size of the cleft. Keeping the baby turned on the side of the cleft both while awake and asleep may reduce the cleft but documentary evidence is lacking of the effectiveness of this maneuver. For some time in the past an operation called lip adhesion, of merely suturing the mucosa and skin, was in vogue to reduce the width of the cleft. Not many centers now appear to be practicing this technique, certainly not in India at this time, partly because the adhesion did not always succeed due to wound dehiscence. The subjects of closure of the alveolar cleft up to the incisor foramen or even beyond, orthodontic treatment to realign the alveolar arches and the correction of nasal deformity in its entirety primarily will be covered in the next chapter.
Acknowledgments: Bharati Khandekar, plastic surgeon, my senior associate and coordinator for the Smile Train Project at Godrej Hospital gave valuable inputs while drafting this chapter and was a key colleague when the two patients shown in this blog were operated. My sincere thanks are due to her. Nisheet Agni, the youngest of the team members, and a maxillofacial surgeon, at the Godrej Hospital in the Smile Train project, helped with intra-operative suggestions and took some of the photographs. Vaishali Ahire, the paediatric anaesthesiologist in the unit was of invaluable help and also took some photographs.
– Ravin Thatte
The frequently imparted plastic surgery principle that each defect and deformity is different and therefore its solution is unique to the situation has been stretched too far and applied rather vicariously to this chapter on the treatment of cleft lip. In my experience of forty years Millard’s radical rotation and advancement technique has rarely failed to give good results and even in the very small numbers where the technique failed a revision was successful by repeating the Millard technique and the fault had lain with the surgeon rather than the method. In fact the Millard technique can be applied as a secondary procedure where a lower triangular or quadrangular flap has been used in an earlier surgery. To the fellows or trainees who request that I demonstrate to them the lower triangular or quadrangular flap for the clefts of the lip, my answer is always a firm ‘no’ because these methods impinge on the philtral dimple and runs across the local (prominent) Langers’ line also called the philtral column. Only rarely for the barest minimum cleft near the vermillion or for a microform cleft, do I use a straight line repair. The Millard radical rotation and advancement technique has a longish learning curve both for the surgeon and the trainee but once the technique is fine-tuned, it is like a dream come true for a plastic surgeon and what is more it can be combined with septal and alar cartilage correction which saves lot of secondary surgery in later years.
– H.S. Adenwala (Head of CJT Pinto Centre for Plastic Surgery, Jubilee Mission Hospital, Trichur, Kerala, India).