29 Secondary deformities of the cleft lip, nose, and palate
The etiology of secondary cleft deformities is multifactorial. The most influential factors leading to the development of a secondary deformity are the type and severity of the cleft, as well as the primary repair technique. Poor preoperative analysis and choice of technique will always lead to a secondary deformity. Matching the deformity to procedure takes a great deal of experience and flexibility by the surgeon. This experience and level of attention to detail can greatly affect results and the need for secondary correction. Tips and tricks learned by a single surgeon over a lifetime of performing cleft surgery can never truly be conveyed verbally or on paper. Lastly, because essentially all patients are operated on within the first year of life, the long period of dramatic growth and the resultant scar after the repair of the cleft structures both profoundly affect the end result. Delaying the definitive repair until a child has reached adolescence or adulthood may minimize the bony growth disturbance; however, early repair of the cleft deformity is mandatory for functional development and psychosocial benefit. Unrestrained growth of the cleft margins may result in even more severe secondary deformities, as the repaired structures guide growth in an anatomic fashion. It is ideal to allow the facial structures to develop in a normal relationship to one another in the intended “functional matrix.” Interestingly, this debate may become less important, as we learn more about whether these growth disturbances actually result from surgical insult or are simply sequelae of an intrinsic bony genetic defect. Correction of the unilateral cleft lip deformity was once thought to restrict the growth of the midface; however, based upon more recent animal studies, the hypoplasia may be secondary to an intrinsic growth deficit.1–3
Secondary procedures are, in many ways, more difficult than primary repairs. This is why preventing secondary deformities is of the utmost importance. Unlike the relatively uniform nature of the initial deformity, secondary problems are widely varied in both their appearance and etiology. Additionally, secondary procedures, by definition, involve previously operated on and heavily scarred tissue. Consequently, learning the techniques described in this chapter, while avoiding a “cookie-cutter” approach by intraoperatively tailoring the procedure to correct the defect uncovered after scar release, both allow the surgeon to express creativity and ultimately yields the best results. Even in the best of hands, the initial insult to the tissue can forever contribute to secondary deformities.
Great advances have been made in cleft care over the past decades. The Millard rotation advancement revolutionized surgical lip repair, and the Furlow double opposing Z-plasty provided another excellent option for palatal repair. However, many less glamorous advances have prevented secondary deformities, decreasing the lasting stigmata of cleft surgery, and leading to improved aesthetic outcomes.
Respecting growth and understanding that early results are not always indicative of long-term appearance are extremely important. However, we have also learned that soft-tissue deformities can be successfully addressed early with lasting results, and, if needed at all, the early correction often simplifies and improves the outcome of later revisions. It may be tempting to think “it’ll settle out” or “I’ll just fix it at the next surgery” when a minor contour deformity is noticeable in the operating room during primary repair, but we have learned that addressing these types of mild deformities early, at the time of initial repair, prevents the need for later revision.
The cleft side frequently requires lengthening of the philtral column. Simply recognizing the discrepancy, if present, and addressing it during primary repair can avert a peaked/notched vermillion requiring revision.
Passive molding, although extremely time- and labor-intensive for the orthodontist, provides a remarkable adjunct to a well-performed surgery and can make an otherwise good result an outstanding result, preventing the need for revision.
Historically, the nose was off limits during primary lip repair. Thinking that early intervention would not last and that attempted repair might harm growth potential or make future rhinoplasty more difficult, the cleft nose deformity was not addressed until facial skeletal maturity was reached. Additionally, modern rhinoplasty techniques (described later) were only beginning to develop, and, contemporary techniques of that era ultimately did not correct the deformities. However, with the advent of new (sometimes cleft nose-specific) techniques, we and many others have begun addressing the nasal deformity at the time of primary lip repair, with improved results. During primary lip repair, quilting sutures prevent lidding/plica vestibularis. Reorientation and repositioning of the lower lateral cartilage (LLC) after adequate release corrects the alar base deformity. These small improvements add up to a markedly improved nasal appearance, and, more importantly, simpler and better results at the time of definitive rhinoplasty.
Lastly, in addition to the failure of fusion, clefted musculature and tissue are aberrantly anchored and must first be completely taken down or disinserted prior to reorientation. This technique creates a stable framework of anatomically aligned structures, providing a platform to prevent relapse into the cleft during healing and scar formation, yielding better results and decreasing the need for secondary revision.
Taken in isolation, each technique only generates small improvements, but when combined, has a dramatic affect on overall results.
Basic science/disease process
Wound healing and growth
Secondary deformities occur for a number of reasons, including scarring, physical growth, and in some cases, technical error with the primary repair. Scarring affects individuals to varying degrees, and whether related to genetics or ethnic heritage, it can have profound results on aesthetic outcomes. The scar contraction can distort the fragile anatomic landmarks of the lip and nose, while excess scar tissue deposition can lead to unsightly irregularities in contour and color. Additionally, physical growth is often referred to as the “fourth dimension” in cleft lip and palate surgery. The growth of the osseocartilaginous skeleton and soft tissues of the face is difficult to predict, and can very visibly affect outcomes. The original cleft lip surgery occurs months after birth with careful approximation and alignment of landmarks and facial structures in three dimensions; however, the human face undergoes rapid periods of growth from birth to 5 years of age and also during puberty, with final osseocartilaginous facial growth in normal children ending with the cessation of puberty. The subsequent changes in aesthetic outcomes that this “fourth dimension” will have are difficult to predict. Finally, technical error or poor technique selection can play a role in outcomes. Together, these factors allow secondary deformities of the cleft lip and palate to be the rule, and not the exception (Figs 29.1, 29.2).
Fig. 29.1 (A–C) Even with perfect surgical technique, postoperative scarring and inflammation vary, resulting in a broad spectrum of secondary deformities.
Secondary deformity management is an integral part of treating the cleft lip and/or palate deformity. Patients with cleft lip and palate will undergo, on average, at least one nose and lip revision when followed into adolescence, with a significant number of individuals requiring multiple interventions.4–6
The reported incidence rates of palatal fistulas have varied greatly, with citations ranging from 0 to 76%; however, caution should be used when interpreting this data as definitions vary and asymptomatic fistulas are often excluded. Interestingly, over the past 5 years, reported palatal fistula rates have decreased. Using a variety of surgical techniques, authors report fistula rates between 0 and 12.9%, with 0–8.1% being symptomatic.7–19
Several studies have looked at the rates of fistula formation, and several strong factors have been identified. Studies have repeatedly demonstrated that surgeon experience is a major predictor of postoperative fistula rates.7,20,21 Murthy et al. reviewed one surgeon’s lifetime experience and noted that over 80% of his palatal fistulas occurred in the first 10 years of practice.7 Additionally, Cohen et al. reported on three independently functioning surgeons: two had performed over 45 cleft palate repairs each and had fistula rates of 15% and 18%, while the third surgeon performed only 19 cleft palate repairs, and reported fistula rates of 63%.21
Studies have also demonstrated that the width of the initial palatal defect, the width of the palatal shelves, and ratio of the cleft width to the posterior arch width were significant predictors of palatal fistulas postoperatively.21 Finally, other risk factors continue to be debated, including initial Veau classification of the defect, type of closure, age at repair, and gender.
Fistulas, regardless of size, represent a complex problem, as they are indicative of a significant scarring along a tense palatal closure. This is demonstrated by relatively high recurrence rates reported historically following fistula repair (25–100%)11,21,22–31; however, more recent data have demonstrated that fistula recurrence rates of 0–25%7,11,23–25 are now being achieved.
Underlying each cleft lip and/or palate is a variable degree of skeletal deformity and hypoplasia. In a portion of these cases, the deficiency is mild and can be treated with orthodontics alone. On the other hand, for unilateral clefts, the reported incidence of orthognathic surgery varies between 26% and 48.5%, and for bilateral clefts is 24–76.5%.21,26,27 The need ultimately varies based on threshold for performing orthognathic surgery.
Normal lip anatomy
The lips’ surface can be divided into three portions: cutaneous, dry vermillion, and wet vermillion (Fig. 29.3). Immediately underlying these superficial layers is a thin layer of connective tissue and fat, followed by a group of muscles which give the upper lip many of its structural and functional features (Fig. 29.4). Other important aesthetic features include the contour and volume of the lips, provided by soft-tissue bulk and configuration.28–31 Finally, the upper gingivobuccal sulcus plays an important role in the appearance and movement of the lip with animation.
Cleft lip anatomy
In the cleft lip, the anatomy is both disrupted and diminutive.32 In general, the changes can be understood by dividing the discussion into the topics of: landmarks (philtral columns, white roll, cupid’s bow), vermillion, and muscular continuity (Fig. 29.5).
It is useful to determine, either through medical records and/or clinical assessment, the type of primary repair and if any secondary procedures have been performed. When evaluating secondary deformities, it is helpful to have a conceptual framework in place to evaluate the lip in a systematic matter. Objective methods have been described to evaluate secondary deformities33,34; however, in day-to-day practice, these can be quite cumbersome. It helps, though, to take the essential elements from these to categorize deformities into five broad areas: scarring, lip, vermillion, muscle, and buccal sulcus.
Scarring should be evaluated for signs of immaturity (redness, firmness), contour changes, and for distortion of local structures. The degree and stage of scarring will dictate an early versus late intervention. Severe and worsening scarring may require an acute intervention (steroid injections), while stable or improving scars are best handled with close monitoring until full maturation.
Lip deformities include abnormal dimensions or landmark distortions of the philtral columns, cupid’s bow, and lateral lip segments. The deformities include a short lip (philtral column on the cleft side is vertically shorter than the noncleft side), a long lip (philtral column on cleft side is vertically longer than the noncleft side), tight upper lip (decreased width between cupid’s bow peaks and/or disparity in anteroposterior projection of the upper lip in relation to lower lip), wide upper lip (increased horizontal width between cupid’s bow peaks), short lateral lip (cleft-side lateral lip segment is horizontally shorter than the lateral lip segment on the normal side), philtral column distortion, and cupid’s bow distortion.
Vermillion deformities include thin or thick lip segments, vermillion mismatches (between the wet and dry vermillion), vermillion notching or border malalignment (between the white roll and vermillion), and the whistle deformity (median tubercle paucity resulting in nonapposition of upper and lower lip segments at rest). From a frontal view, the prominence and volume of the median tubercle should also be assessed. From a profile view, the lip should be evaluated for the break seen just above the white roll, as well as the anterior–posterior relationship between the upper and lower lip.
Deformities involving the muscle may result from inadequate muscle reapproximation during the primary repair or subsequent dehiscence. Orbicularis oris muscular dehiscence will present as bulging on either side of the lip repair with animation or a short and widened lip scar. However, since muscle reconstruction has become a standard part of the primary repair, the muscular deformities commonly seen now are more likely a result of inadequate disinsertion of the aberrant muscular attachments. Incomplete dissection of the aberrant attachments leads to more mild deformities, including tethering of the nasal ala, and a subtle relapse to the prior classic cleft appearance. While the orbicularis oris has always received great focus in cleft lip repair, the cleft lip and nose deformity involves a complex network of muscles in the oronasal region. Dissections performed by the authors have demonstrated that the lateral nasalis is also aberrantly inserted in the primary deformity and is often unaddressed during the primary repair. Additionally, the importance of creating a nasal floor and nostril sill during primary repair and subsequent secondary procedures cannot be overemphasized in achieving lasting results and preventing relapse of lateral and elevated alar position.
The buccal sulcus should be examined to determine how free the lip is from the maxilla. The deep aspect of the sulcus should extend up to the region of the columella–lip junction. If the lip is tethered, this can result from scar contracture or from a true paucity of tissue related to the initial deformity.
Any of the previously mentioned deformities can occur concomitantly, and as such, require the surgeon to prioritize treatment based on the importance and realistic potential benefit to the patient. The ultimate goal is a surgical plan that yields the best result, in the fewest number of operations (Fig. 29.6).
Proper intervention focuses on addressing functional impairments, as well as aesthetic deformities that could affect psychosocial development. To do the latter, one must have a solid understanding of how and when psychosocial factors affect outcomes, as well as the normal growth of the face. At about 6 years of age, the risk of teasing by peers increases substantially and may be a source of great distress for the cleft patient and the family. Consequently, all patients should be evaluated for secondary surgery at the age of 4 or 5 before the start of kindergarten. Decisions regarding timing are predicated on the severity of the problem relative to function and appearance, and balanced against both the emotional and physical maturity of the patient. As children progress into adolescence, they become more capable of expressing opinions about appearance, and their wishes should play an increasingly significant role in surgical decision-making.
Growth plays a role in outcomes, and studies have shown that roughly 87–93% of growth-related changes in labial landmarks occur by age 5.35 Thereafter, certain growth-related changes are more predictable; however, scarring and other factors continue to affect long-term outcomes.
While the corrected cleft lip deformity often looks its best at the first office visit, the process of scar maturation can result in significant color and contour abnormalities, distortion of adjacent mobile landmarks, and shortening of the lip dimensions. While these changes may necessitate a secondary correction, there are actions that can be taken during the healing process to minimize the severity of its sequelae. Parents should be advised to massage the scar with the lotion of their choice (vitamin E, cocoa butter, zinc, or Mederma), and utilize sun protection for at least 1 year to prevent persistent hyperpigmentation. These acute interventions are beneficial, not only for scar improvement, but also psychologically, as they involve the family in the care of the child. While silicone sheeting and hypoallergenic taping may be beneficial in the treatment of scars,36 they may be difficult to maintain on the upper lip of a child and therefore yield varying results. Intralesional steroid injections may be started if there is evidence of hypertrophic or keloid scarring, or if the patient is at high risk for either of these. If the scar continues to be problematic after optimal conservative care, and a minimum of 12–18 months has been allowed for scar maturation, consideration should be given to a surgical revision.
Several surgical options exist for the treatment of secondary cleft lip scarring. The appropriate treatment selection should take into consideration the characteristics of the scar. Dermabrasion is a potential option for scars that are elevated. If scars are hypertrophic and have widened significantly, treatment with excision in the shape of a diamond or ellipse facilitates a straight-line closure. If the scar is depressed or overlies a philtral column a skin-only excision with a vest-over-pants closure (Fig. 29.7), or other bulking techniques (see philtral column distortion section, below) will be helpful.
A vertically short lip can be caused by scar contraction, a primary deficiency of prolabial soft tissues, and/or inadequate primary rotation and advancement. A mild degree of shortening is not uncommon after repair because of scar contraction; however, this may improve over time. During the scar maturation process, patients can perform scar management, as discussed previously. If the deformity persists for more than 1 year or after the scar has matured, then consideration should be given to a surgical procedure.
The difference in lip height between normal and repaired sides is precisely measured to determine the amount of lengthening necessary. In patients with minor deformities (<2–3 mm), the shortness may be treated by a diamond-shaped scar excision or a unilimb Z-plasty to lengthen the philtrum; however, for lip shortening greater than 2–3 mm, these techniques may be insufficient. For this degree of lip shortening, one option is to perform a standard Z-plasty to lengthen the philtral column; however, it is important to remember that this technique places additional scars on the upper lip. For more significant degrees of shortening, or if additional scars from a Z-plasty are not preferable, the entire repair should be taken down and repeated (Fig. 29.8).
Fig. 29.8 This patient demonstrates a short lip deformity, notched vermillion, and white roll discontinuity secondary to hypertrophic scarring. The patient subsequently underwent complete take-down of the repair, with repeat rotation advancement. (A) Preoperative photo; (B) 1-week postoperative photo; (C) 3–4-month postoperative photo.
When a short philtral column persists due to underrotation of the flap, it is usually because of minimizing the backcut, and will always lead to vermillion notching and a vertically oriented, noncontinuous white roll. It is recommended to trade a slightly larger backcut/scar (even onto the columella and back down the normal philtral column) to obtain the necessary rotation.
This deformity is rarely seen because of decreasing use of the Tennison repair; however, it can still occur for other reasons, such as overrotation of the medial lip segment. When addressing this deformity, it is tempting simply to excise lip tissue from beneath the alar base in an effort to “hitch up” the elongated cleft side. Experience has demonstrated that this does not yield acceptable results because of the underlying action of the orbicularis and gravity pulling down on the incision. Even suspension with permanent sutures fixed to bone is not always predictable. It is more preferable to take down the entire repair and excise tissue in all dimensions to correct the deformity.
Options for management of the horizontally tight lip deformity include fat injections to the upper lip and reduction of the lower lip through a wedge excision of inner lip tissue, reducing lip bulk disparity. More significant deformities, however, are best treated with the Abbé flap.
The Abbé flap transfers full-thickness lip elements to the upper lip in a two-stage procedure by transfer of a lower lip segment based on the inferior labial vessels. The pedicled flap is then left in place for 2–3 weeks, before division and inset (Fig. 29.9). While this flap is more commonly used with secondary bilateral cleft lip deformities, it can be used when there is: (1) decreased anteroposterior projection of the upper lip (particularly helpful in reducing the disparity in tissue volume between the upper and lower lip); (2) excess scarring of the central aesthetic unit; and (3) a significantly narrowed or shortened central aesthetic unit. Additionally, the flap provides a pseudodimple for the philtrum, a tuft of hair in males, and continuity of surface landmarks (Fig. 29.10). This procedure, however, should not be the first choice, because of donor site morbidity, increased risk associated with two procedures, and patient discomfort associated with a 2–3-week period of flap delay between stages.
Fig. 29.9 (A–D) The Abbé flap allows for the transfer of full-thickness lower lip elements to the upper lip in cases of: (1) decreased upper lip anteroposterior projection; (2) excess scarring of the central aesthetic unit; and (3) significantly narrowed or shortened central aesthetic unit.
Fig. 29.10 A patient who was born with a bilateral cleft lip deformity (A, B) before and (C, D) 3 years after Abbé flap to correct a scarred and constricted philtrum and a tight lip. (E, F) A bilateral cleft lip patient with a short lip bilaterally accompanied by a paucity of central vermillion, and a tight upper lip. (G, H) Nine-year postoperative results post Abbé flap.
The horizontally wide lip can be caused by a number of factors. It is most frequently seen in bilateral clefts where the prolabial philtral segment was designed too wide at the time of initial operation. It can also be seen when persistent tension across the neophiltrum, secondary to a protruding maxilla or underlying orbicularis muscle function causes horizontal expansion of the prolabial soft tissue. The solution is excision of the excess philtral tissue, taking care to remember the tenets of lip surgery: meticulous approximation of the orbicularis oris and accurate approximation of philtral landmarks. Regardless of age at the time of the secondary procedures, the philtrum should be made smaller than the final desired size in anticipation of subsequent stretching.
Short lateral lip
A decrease in the horizontal width of the lateral upper lip segment is a common deformity, resulting from the need to achieve an optimal cupid’s bow. This decrease in width of the lateral lip segment results after excision of its diminutive medial portion. Traditionally, the short lateral lip segment has been said to “stretch”; however, in our experience, this is not the case. Ultimately, though, intact central landmarks and good lip contour are more important than symmetry of the lateral lip segment.
Philtral column distortion
The philtral column can be affected by excessive scarring, short length, or a lack of prominence. In the case of excess scarring, the methods described in the section on scarring, above, can improve aesthetic outcomes. Shortened philtral columns can be addressed using methods described in the section on vermillion notching, below. Methods to recreate the philtral column, in general, involve either the addition or overlapping of soft tissue to add soft-tissue elevation. Fat grafts (free or dermal) have been used by some to augment the philtral column (Fig. 29.11), and a “vest-over-pants” closure is an option to create the philtral column using local tissues. The latter method involves excision of the prior scar, with subsequent burial of the underlying dermis underneath the adjacent dermis to provide the bulk for the philtral column. Additional methods have been described, including using mattress sutures to reapproximate and evert the orbicularis, as well as vertical interdigitation of the orbicularis.37
Fig. 29.11 (A, B) These images demonstrate the concept behind free fat injections. Free fat is injected into a stab incision made near the oral commissure, and tunneled into the upper lip.
Key principles of any procedures involving the region of the philtral columns include limited subcutaneous undermining, enough to allow a layered closure, and concomitantly releasing dermal orbicularis oris attachments that would place additional tension on the wound. Care should be taken, however, to limit subcutaneous undermining to less than 5 mm, and not to cross the contralateral philtral column or central philtral dimple, thereby preventing landmark distortion and/or vascular compromise.
Cupid’s bow distortion
Significant abnormalities of the cupid’s bow in unilateral cleft lip deformities are usually the result of misalignment or notching, and can be corrected by local tissue rearrangement techniques, such as diamond excisions and Z-plasties. With more severe or complicated distortions, it may be necessary to take down the previous lip repair and to repeat rotation advancement. When there is a paucity of healthy and unscarred tissue for reconstruction, as seen after bilateral cleft lip repair, full-thickness lip elements can be acquired from the lower lip via the Abbé flap.
The goals for treating a thin lip deformity are to increase the amount of vermillion show, improve anteroposterior projection of the upper lip, and replace contour landmarks (the break point existing 3–4 mm above the upper lip vermillion and the prominent median tubercle). These goals are even more important in the female population, as increased vermillion show and volume are associated with increased attractiveness.29,30 In cases of patients with adequate vermillion but a paucity of volume, fat grafting becomes an excellent option. This can be performed with either free fat injections or via a dermal fat graft.
Free fat grafting can be performed by harvesting adipose tissue from the lateral thigh or the periumbilical region, processing the fat, and subsequently injecting this into the upper lip via small stab incisions, just medial to the oral commissures (Figs 29.11 and 29.12). In addition to increasing volume, there is much recent interest and reports of fat grafting improving the quality and color of overlying skin in scarred or radiated beds. While the procedure is simple in nature, it can become difficult if there is a significant amount of scar, making tunneling and multiple small-volume passes difficult. The authors have had more success fat grafting unscarred beds, as expanding the contracted, tight scar is quite challenging even with repeated injections.
Fig. 29.12 (A, B) This patient has a preoperative paucity of vermillion centrally, as well as a thin lateral lip. After fat injections to the upper lip, the patient now has increased vermillion show and upper lip fullness. Fat grafting is a potent method to achieve these aesthetic outcomes, especially within the female population.
Dermal fat grafts are composite grafts of dermis and subcutaneous fat that can be harvested and inserted en bloc via an intraoral incision into the upper lip. With the rapidly increasing success and interest in free fat grafting, the use of dermal fat grafts has dramatically decreased.
Bilateral clefts often have greater tissue deficiency than unilateral clefts, and subsequently have a greater risk of secondary deformity. Although local tissue rearrangement, as described before, may suffice for minor cases of vermillion deficiency, the deficiency in bilateral cleft lips is often more severe, requiring soft tissue that the adjacent areas cannot provide. These situations are best treated with an Abbé flap.