Cleft lip

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.

Fig. 29.3 Normal lip landmarks and features.

Fig. 29.4 The lip and nose are surrounded by a host of musculature that plays a role in its structure and function, and must be taken into consideration when correcting the cleft nose deformity.

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).

Fig. 29.5 Cleft anatomy and abnormal muscle insertions.

Evaluation

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 deformities ^33,³⁴; 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).

Fig. 29.6 Elements of analysis of secondary cleft lip deformities.

Patient selection

Timing

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.

Scarring

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.

Fig. 29.7 (A–H) Illustration of the vest-over-pants closure, which buries de-epithelialized dermis underneath adjacent epidermis to add regional soft-tissue bulk.

Lip

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.

Wide lip

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.

Video2

Vermillion

Thin lip

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.