Unfavourable results in the repair of the cleft lip.

INTRODUCTION: Unfavorable results in unilateral and bilateral cleft lip repair are often easy to spot but not always easy to prevent as to treat. We have tried to deal with the more common problems and explain possible causes and the best possible management options from our experience. UNILATERAL CLEFT LIP REPAIR: Unfavorable results immediately after repair involve Dehiscence and Scaring. Delayed blemishes include vermillion notching, a short lip, deficiency in the height of the lateral vermillion on the cleft side, white roll malalignment, oro-vestibular fistula, the cleft lip nose deformity, a narrow nostril and a "high-riding" nostril. We analyze the causes of these blemishes and outline our views regarding the treatment of these. BILATERAL CLEFT LIP: Immediate problems again include dehiscence as also loss of prolabium or premaxilla. Delayed unfavorable results are central vermillion deficiency, a lip that is too tight, bilateral cleft lip nose deformity, problems with the premaxilla and maxillary growth disturbances. Here again we discuss the causation of these problems and our preferred methods of treatment.
CONCLUSION: We have detailed the significant unfavorable results after unilateral and bilateral cleft lip surgery. The methods of treatment advocated have been layer from our own experience.

INTRODUCTION

In all aspects of plastic surgery, there is an equal measure of art and science. However in the repair of the cleft lip, art seems to take the upper hand. In art, there is no perfection: You go through experiences in art, every time reaching a subtly higher plane of achievement. Consistent perfection eludes the craftsman; the love affair is never consummated and therefore it never dies. How often do we hear experienced master Surgeons say, “Ah! If only “this” or “that” were right, you would never have known that this child suffered from a cleft lip at all!” This article intends to deal with the “this” or “that” in the outcome of cleft lip surgery.

Unfavourable results in unilateral and bilateral cleft lip repair lead one into a deep ocean of blemishes, easy to spot, but not always easy to prevent or treat.

What we have performed in this article is to try and deal with the more common problems, explain our views regarding their causation and define the best possible management, mostly from our personal experiences over these many years, never forgetting Sir Harold Gillies’ dictum: “Diagnose before you treat.”[1]

We shall approach the unfavourable results following unilateral and bilateral cleft lip repairs separately. In each of these, the unfavourable result may be immediate or delayed.

UNILATERAL CLEFT LIP REPAIR

Immediate

Dehiscence

This is not very common in unilateral cleft lip repair. The main reason is either tension in the repair or improper suturing of the orbicularis muscles. Trauma or infection may be rarer causes. In cases where the modality of pre-operative orthodontics has not been used, we mobilise the lateral lip element extensively in a sub-periosteal plane up to the infraorbital foramen superiorly and the zygomatic eminence laterally. Further release may be obtained by scoring on the under surface of the periosteum if required. With such extensive mobilisation, the width of the cleft ceases to be a daunting factor anymore. If one encounters dehiscence, adequate debridement is necessary. Infection must be controlled and secondary suturing done.

Scarring

This depends on three factors:

Intrinsic strain, which depends on the tightness of the suturing

Extrinsic strain, which is the tension with which the tissues are brought together

The Inherent reaction of the individual to surgical trauma.

The surgeon has control over the first two factors, but not over the third.

In the early period following the repair, most children exhibit a scar contracture with a pulled up Cupid's bow and a vermillion notch on the cleft side. However, with time, this settles in most cases. Only when the initial rotation has been inadequate or the lateral lip element is too short, will this contracture persist. We always perform an adequate rotation with an ample back-cut in every patient. Salyer[2] use a small Z plasty above the Cupid's bow to get the Cupid's bow points on both sides at the same level. We avoid this as the resulting scar is across the Langer's lines and shows badly. Besides, cutting across Langer's lines is against first surgical principles.

We believe that while placing the cinch suture we must get a good bite on the paranasal muscles. The perialar incision enables a better placement of this suture. However, often the perialar scar shows badly while the rest of the lip scar may be hardly evident. Hence, we now restrict the extent of the advancement incision and do not proceed too far laterally.

When the contracture persists [Figure 1], a re-rotation is required secondarily. Millard himself advocated a waiting period of at least a year before embarking on correction in such cases.[3]

Figure 1

Persistent contracture following unilateral cleft lip repair

Delayed

Vermillion notching

This is a discontinuity in the free border of the vermillion. This may be central (“whistle deformity”) or lateral along the line of the scar. The latter is more common in unilateral lips and hence is dealt with here [Figure 2].

Figure 2

Notch on the vermillion

This may be caused by:

Inadequate rotation

Inversion of the sutured edges

Orbicularis Oris marginalis muscle deficiency

Straight line scar contracture.

The senior author has evolved a protocol that is followed in all patients during primary repair.[4] This involves:

An adequate rotation of the medial element with an ample back-cut to bring down the raised Cupid's bow point level with its counterpart on the non-cleft side [Figure 3]

Figure 3

Adequate rotation with back-cut, bringing the cupid's bow points at the same level

Undermining the skin and mucosal edges prevents their inversion [Figure 4]

Figure 4

Undermining the skin and mucosa

To avoid a deficiency of muscle bulk in the vermillion, an excess of muscle cuff is retained in both the medial and lateral lip elements while paring the lip. This muscle is then built up with at least three 6-O Nylon sutures [Figure 5]

Figure 5

Ample muscle is retained both medially and laterally to prevent deficiency in muscle bulk

Straight line scar contracture is best prevented by a Z plasty. If this is done on the skin of the body of the lip as is done in the triple wedge technique of C. Balakrishnan or in Fisher's technique,[5] we believe that it violates Langer's lines, and the scar invariably shows. Hence we perform a Z Plasty on the mucosa away from Noordhoff's red line. This Z should be placed carefully-neither too close to the red-line (it would then show), nor too far inside on the mucosa (when it would not serve the purpose) [Figure 6].

Figure 6

Z plasty on mucosa, away from the red line

With such a meticulous approach, we have been able to consistently avoid a vermillion notch in our patients [Figure 7].

Figure 7

Notch-free Vermillion using the Charles Pinto centre protocol: (a) Pre-operative and (b) post-operative

Another popular technique of vermillion correction has been developed by Noordhoff and Chen.[6] All the tissues are richer on the non-cleft side except the vermillion, which is richer on the cleft-side. Thus, there is a mismatch. To correct this, Noordhoff retained a V shaped extension of the tissue on the cleft side while paring the lip, and inset this into a transverse incision made on the medial lip element. Though Noordhoff made the incision at the junction of the wet and dry mucosa (the red line), we believe that it is more apt to make the cut in the dry mucosa as one of the main aims of this technique is to obtain a better colour match of the vermillion on the two sides, especially in patients with a fair complexion.

We use the same protocol that we use for notch prevention for notch correction also [Figure 8a and b]. A re-rotation is done. Undermining of the skin and the mucosa follows. Scar tissue obtained during the scar excision is not discarded, but de-epithelialized and retained as a filler to provide fullness at the vermillion if necessary. A Z plasty is done on the mucosa as in the primary procedure.

Figure 8

Revision Cutaneous Millard's procedure and notch correction procedure: (a) Pre-operative and (b) post-operative

A lip too short

This may result when the rotation is inadequate or when the lateral lip is too short in height.

In all our unilateral cleft lip patients, we perform an adequate rotation with an ample back-cut (not violating the philtral column on the non-cleft side).

If there is an obviously short lateral lip height, the lateral point on the cleft side may be moved laterally by 1 mm and superiorly (into the nostril base) by 1 mm. A gain of 2 mm is really quite substantial in these small babies. Mulliken and LaBrie[7] has shown that there is a good growth in the transverse dimension of the lateral lip element, which will thus compensate for the more lateral placement of the lateral point.

However, Fisher[5] has designed a technique to address the lateral lip length deficiency by a constant placement of the lateral point and by adjusting the incision medial to this to gain adequate length.

In patients with inadequate rotation, a revision Millard procedure is done with a back-cut to align the Cupid's bow points. If the rotation required is minimal, the incision is confined to the skin and the subcutis, sparing the muscle. This has been termed the Cutaneous Millard Procedure by the Senior Author and has been performed in this unit for many years now [Figure 8].

In patients in whom there is bunching up of the muscles laterally due to improper approximation of the muscles, after adequate undermining, the muscles are built up with 5-O polypropylene sutures.

Where more substantial rotation is required, a full-fledged Revision Millard Procedure is carried out, with the incision extending through the muscle and mucosa as well [Figure 9a and b].

Figure 9

Revision Millard's procedure: (a) Pre-operative and (b) post-operative

Deficiency in the height of the lateral vermillion on the cleft side

This is a common problem noted in many unilateral cleft lip patients with a gross alveolar disparity pre-operatively. We have seen this in various types of primary procedures and not just the Millard's procedure. Hence, we believe that this is an inherent deficiency.

We are unaware of any surgical measure to prevent the occurrence of this problem. However, the centres utilizing pre-surgical orthodontics like Nasoalveolar Molding do not seem to encounter such a problem. This is the impression when one observes the results presented from these units. It is probable that by bringing the medial and lateral bony shelves in alignment, the discrepancy in the lateral vermillion height is overcome.

When one is faced with this situation during follow-up, there are two options available to treat these patients:

The Gillies’ half Cupid's bow procedure [Figure 10a and b]. Excision of a triangular strip of skin immediately above the white roll is performed. A wedge of muscle may be excised from the medial aspect. This technique requires meticulous craftsmanship so that the resulting scar will not be visible

Figure 10

Gillies half Cupid's bow (a) incision marked (b) completed procedure

V to Y mucosal advancement with muscle build-up [Figure 11a–c].

Figure 11

(a) V-Y mucosal advancement procedure with muscle build up: Incision marked on mucosa. (b) V flap raised and muscle built up. (c) Sutured flap (V-Y)

This is our preferred method as there is no scarring on the skin. It can be used to correct fairly large deficiencies of the lateral vermillion. We mark the V with the broad base superiorly towards Noordhoff's red line and the apex towards the labial sulcus. The mucosal flap is raised including a layer of muscle for improved vascularity. The underlying muscle is closely inspected for the exact area of the deficiency and adequate muscle build up is carried out using 5-O nylon plicating sutures in more than one layer if necessary. Care is taken to avoid any distortion of the lip from overenthusiastic muscle build up. This technique has given us consistent and sustained results [Figure 12a and b].

Figure 12

Correction of lateral vermillion deficiency. (a) Before correction and (b) after correction

White roll malalignment

Minor grades of this can be corrected by a Z plasty [Figure 13a and b].

Figure 13

White roll malalignment correction by Z plasty: (a) Incision marked and (b) after completion

Orovestibular fistulas

These happen because of a breakdown of the nasal floor repair at the vestibular sulcus, or because the nasal floor has not been repaired. A two layered closure has to be done secondarily if this occurs.

The cleft lip nose

It is the nose that separates the men from the boys. The cleft lip and nose are situated in the centre of the face and one half of each is begging for comparison with the other, and nothing short of absolute and perfect symmetry satisfies the patient or the surgeon. This perfection is seldom achieved. In search of perfection, a host of exponents have described various methods of lip repair;[8] the very fact that there is still no consensus among them shows that absolute perfection remains and may remain a pipe dream.

The deformities in the unilateral cleft lip nose have been well-studied.[9] These include: The cleft side alar dome is depressed, the clef side ala is buckled inward, the nostril is wider, the hemi-columella is shorter, the nasal septum is deviated anteriorly towards the non-cleft side [Figure 14].

Figure 14

Cleft lip nose with classical deformity

It is possible to minimise the extent of the secondary deformity in a child with a unilateral cleft lip by correcting the nasal deformity to some extent at least during the primary repair. This correction may take the form of an open or a closed technique. At our centre the senior author developed a method of nasal correction more than 40 years ago, well before other surgeons like Anderl et al.,[10] and which is also much more aggressive. This has taken the form of a closed alar cartilage shift by dissecting the alar cartilages free from the overlying skin of the nose dorsally [Figure 15].

Figure 15

Closed alar cartilage dissection medially and laterally

Simultaneously, a radical septal repositioning is done by detaching the septum from all its bony attachments-to the maxillary crest, vomer and the perpendicular plate of the ethmoid. The mucoperichondrium is separated off the underlying septal cartilage on both sides. The cartilage is scored on its non-cleft side to make it flail and to get rid of its bow-stringing effect. A sliver of cartilage is also excised from the inferior aspect and a wedge removed from the anterior aspect so that the tip may spring up for better projection [Figure 16].

Figure 16

Septal cartilage. Shaded areas are excised

Finally, the cartilage is fixed to the newly reconstructed nasal floor with 5-0 polypropylene sutures [Figure 17].

Figure 17

Fixing the septal cartilage to the reconstructed nasal floor

Septal repositioning has been described by Anderl et al.[10] and Smahel et al.[11] and long-term studies by them have not shown any detrimental effect on nasal growth from this early septal intervention.

A cleft lip nose reconstructed as above does not show any of the major stigmata usually associated with such patients. However, many of these patients do still develop a drop of the soft triangle on the cleft side [Figure 18].

Figure 18

Soft triangle droop

We correct this at about 5-6 years (pre-school age), using an open structure anterior rhinoplasty to avoid the child being ridiculed by peers at school. We use a Tajima reverse U incision[12] on the non-cleft side, and a V to Y advancement at the base of the columella for columellar lengthening [Figure 19].

Figure 19

Tajima reverse U on the left side. Inner rim incision on the right side and V at the base of columella

Once the upper and lower lateral cartilages have been exposed on both sides, the cleft side lower later cartilage is hitched to the non-cleft side upper lateral cartilage through the septum with a 4-0 nylon mattress suture [Figure 20].

Figure 20

Sutural technique of rhinoplaty. The cleft side lower lateral cartilage is sutured to the ipsi- and contra-lateral upper lateral cartilages; inter-domal, and inter-medial crural sutures are also shown

We believe that this is the key suture and that the anchoring of this stitch through the septum is important to provide stability over a prolonged period of time. The cleft side lower lateral is also hitched to the ipsilateral upper lateral cartilage. Inter-domal mattress sutures bring the domes together and narrow the tip. The two medial crura are hitched together for columellar support. We have been following the above sutural technique for the past 9 years and the results have been gratifying [Figure 21a and b].

Figure 21

Preschool rhinoplasty (a) pre-operative and (b) post-operative

We believe that these patients will not need a definitive rhinoplasty at a later date as we have corrected both the nasal septal and alar cartilaginous deformities.

We also use a similar sutural technique for adult patients with cleft lip nasal stigmata that we come across [Figure 22a and b].

Figure 22

Adult rhinoplasty (a) preoperative and (b) post-operative

In most of these patients, we are able to obtain the necessary correction without the use of onlay alar cartilage grafts. We do resort to the use of septal cartilage grafts for the tip as well as supports for the columella, if necessary. It has been our observation that adult patients who have had their primary repair at our centre require secondary septal correction less frequently than patients from other centres where no primary septal work has been done.

If there is any need for alteration of the bony structure of the nose, it is done at 16 years of age.

The narrow nostril

Another unfavourable outcome following cleft lip repair is a narrow nostril. When there is adequate nostril volume, we have used a Y to V procedure for widening the nostril base. However, when the nostril volume is reduced overall, it becomes very difficult to correct. Various methods have been tried-Potter's V to Y release,[13] septal flaps, etc. Rarely, even full thickness grafts have been used after release of the web. However, we have not found the results of any of these procedures very encouraging. The senior author still uses Pinto's Z plasty on the web [Figure 23].[14]

Figure 23

Pinto's Z plasty for the alar web

The “high-riding” nostril

In patients who have a wide antero-posterior alveolar disparity between the medial and lateral bony segments, that have not been addressed pre-operatively, there is a possibility of the cleft side nostril base lying on a higher horizontal plane than its non-cleft side counterpart [Figure 24].

Figure 24

High riding nostril

Pre-surgical orthodontics will eliminate the alveolar disparity and hence the chances of a high riding nostril will be eliminated. When such pre-surgical moulding is not available, we have used an unequal Z plasty as advocated by Jackson [Figure 25].[15]

Figure 25

Jackson's unequal Z plasty

In these, the lateral limb of the Z is at right angles to provide a greater release. Medially the Z is at a 45° angle. Thus, the Z is unequal with respect to its angles. When the alveolar disparity is gross, we use two such Z plasties for better release. This has reduced the incidence of the high riding nostril in our patients.

Once a high riding nostril occurs, treating it secondarily seldom produces perfect results. The nostril can be brought down by a form of Z plasty [Figure 26a and b].

Figure 26

Correction of high riding nostril by Z plasty: (a) Z plasty incision and (b) after completion

A cinch suture anchoring the paranasal muscles to the anterior nasal spine seems to produce more consistent results. The results are not always predictable.

BILATERAL CLEFT LIP REPAIR

Dehiscence occurs more commonly with bilateral lips than unilateral ones, probably due to the stretch of the repair over the protruded premaxilla in patients without pre-surgical orthodontics. Even mild trauma like the banging of the head of the child against the shoulder of the parent may cause disruption of the repair. Prevention is by meticulous and thorough mobilization sub-periosteally as mentioned for the unilateral lip repair. The Veau suture (with 4-0 polypropylene) may help in adding strength to the repair [Figure 27].

Figure 27

Veau suture for the bilateral cleft lip

Millard used the Logan's bow with all of his bilateral lip repairs.[16]

Once dehiscence has occurred secondary suturing is done after thorough debridement and control of infection.

Loss of prolabium/premaxilla

With the onset of surgical techniques bringing the lateral orbicularis oris muscles together in the mid-line after raising the prolabial skin flap, rarely, there can be an loss of the prolabium, and even entire premaxilla due to vascular compromise. Methods of repair like that of Trott and Mohan[17] involving primary rhinoplasty can cause damage to the branches of the Anterior Ethmoidal arteries.[18]

A strip of mucosa must be preserved over the vomer in the mid-line during the nasal floor closure. The nasal layer on either side of the vomer is raised, leaving this strip of vomerine mucoperiosteum intact to preserve the vascularity of the premaxilla.

Central vermillion deficiency (“whistle deformity”)

This is more common in bilateral cleft lips, especially with repair over a severely protruded premaxilla.

When there is good presurgical alignment of the premaxilla with both the lateral maxillary segments, an adequate advancement of the lateral lip elements from both sides is easier and there is no central vermillion deficiency. The lateral turn-down flaps are used to form the central vermillion after turning down the prolabial mucosa. The latter should never be used for the vermillion of the prolabium. If it is, there is a scalloping of the edges which is unsightly.

Treatment involves one of the following courses:

If there has been inadequate advancement of the lateral lip with a wide phitrum, then a revision bilateral lip repair with advancement of both the lateral lip elements medially and vermillion muscle build up with 5-0 nylon sutures will do the trick.

If the central vermillion notch is modest and enough advancement has already been achieved, then we use a V to Y mucosal advancement with muscle build up with 5-0 nylon as we have described for lateral vermillion deficiencies [Figure 28a and b].

Figure 28

Central vermillion notch correction by V-Y mucosal advancement and muscle build up: (a) Pre-operative and (b) post-operative

In more severe cases of central notching, filler like dermal fat graft will be necessary. One must over-correct with the fat graft as there is always some resorption, the extent of which is unpredictable.

A lip too tight

This happens when too much of the lateral lip elements have been discarded, often due to repeated lip advancements to correct central vermillion deficiencies. The best reconstruction available in this instance is a central shield-shaped Abbe flap [Figure 29] as recommended by Millard.[19] The Senior Author has on occasion used a ‘W’ shaped Abbe flap, which is technically more demanding [Figure 30].

Figure 29

Shield shaped Abbe (a) before and (b) after the procedure

Figure 30

W shaped Abbe flap. (a) Incision and (b) after completion

The bilateral cleft lip nose

The classical bilateral cleft lip nose deformity involves a short columella, divergent lower lateral cartilages and wide nostril bases. Primary correction commenced with the onset of the concept that the columella is in the nose.[20] Harold McComb[21] was one the pioneers of this concept. Mulliken[22] used bilateral alar rim incisions and an interdomal suture. Once the cartilages have been brought together, the columella also lengthens with time and secondary columellar lengthening procedures are avoided.

When primary nasal correction has not been resorted to, a columellar lengthening procedure is required secondarily. We use a V to Y columellar lengthening at the base of the columella. Bilateral Tajima reverse U incisions are used to get better nostril shape and symmetry [Figure 31].

Figure 31

Bilateral Tajima revision U incision and V-Y elongation of columella-incision

The underlying cartilages are built up with non-absorbable sutures-the lower lateral cartilages are hitched to both the ipsilateral and contralateral upper lateral cartilages with 4-0 Ethilon [Figure 32]. A cephalic trim is done on both the lower lateral cartilages to narrow the tip. Interdomal mattress sutures bring the domes together. The medial crura are hitched together. With such a technique, we get consistent and sustained results [Figure 33a–d].

Figure 32

Intra-operative picture showing the sutures hitching each lower lateral cartilages to the ipsi- and contra-lateral upper lateral cartilages with 4° nylon sutures

Figure 33

Rhinoplasty for correction of a bilateral cleft lip nose. (a and c) Before. (b and d) After

Other techniques of columellar lengthening include the Millard's forked flap[23] and the Cronin's method.[24] The former gives excellent columellar lengthening, but the resultant lip scarring is often unsightly and has long been given up at our centre for this reason.

Premaxilla

A protuberant premaxilla hampers primary reconstruction of a bilateral cleft lip. Many centres now routinely use pre-surgical orthodontics to set it back. Where it has not retruded and remains prominent, it is unsightly and needs to be set back after lateral expansion with orthodontics.

In rare cases, the protruding premaxilla is so unsightly that a vomerine osteotomy is required to set it back. However, such an osteotomy should be delayed at least until after the cessation of growth of the mid-face, which happens at about 8 years.[25] Earlier osteotomies will lead to severe maxillary regression and are to be discouraged.

Damage to growth of the maxilla

We believe that sub-periosteal mobilisation of the lateral lip causes less maxillary regression than extra periosteal mobilisation. The frequency and extent of maxillary regression is more with regard to bilateral cleft lips, as the extent of mobilisation is much more. Pre-surgical orthodontics minimises the mobilisation required. It remains to be seen if it translates into less regression in the long run.

When it does occur, modalities like maxillary distraction or Le Forte I osteotomies are required, sometimes in conjunction with mandibular osteotomies.

CONCLUSION

The authors would like to stress that for each of the modalities suggested for the correction of blemishes in the cleft lip surgery, there is always another method and often there are multiple procedures described as each textbook undergoes a new edition. The ancient adage that ‘There are more ways of skinning a cat than one’ is very true in cleft surgery. In the ultimate analysis, the cleft surgeon remains a ‘seeker’ and must find his own way to diminish his unfavourable results and to solve them when the need arises. The aim as in all art is to achieve perfection-if there is such a thing as perfection in art.