Correction of Secondary Bilateral Cleft Lip and Nose Deformities

Laura A. Monson, Edward P. Buchanan, Larry H. Hollier, Jr.

KEY POINTS

○ The key to an excellent lip repair and revision is an understanding of the normal and abnormal anatomy.

○ Revisions of the lip and soft tissues of the nose can be performed any time. Full septorhinoplasty (including osteotomies) should not be done until cessation of maxillary growth and completion of orthognathic surgery.

○ The Abbé flab is rarely needed for central vermilion deficiency; often local tissue rearrangement is adequate.

○ A complete release and repair of the orbicularis muscle is the foundation to a good bilateral lip repair.

○ The bilateral cleft nose often lacks adequate support, and rib graft is usually needed at the time of definitive rhinoplasty.

Proper treatment of patients with bilateral cleft lip deformities is an effort that challenges even the most experienced cleft surgeon. Diagnosing the problem (including understanding the original repair technique) is paramount during the initial stages of care. Recognition of common deformities will help facilitate the surgical planning process and assist the surgeon during future procedures. Timing of intervention depends on the severity of the deformity, the age and subjective distress of the patient, and the expectations of the parents. Although many of the secondary procedures devised for unilateral clefts can be adapted and used for bilateral defects, some inherent characteristics in the secondary bilateral cleft lip and nose deformity must be taken into consideration because they affect treatment management and outcomes. These include paucity of central lip tissue; an inadequate or diminutive columella; and a limited blood supply to the prolabium. With patience and surgical care, successful correction of secondary cleft lip deformities can be achieved.

TIMING OF SURGERY

Although parents typically want to have their child’s deformity corrected as soon as possible, the bilateral cleft lip by its nature and complexity may necessitate more than one surgical procedure.1 Many cleft surgeons agree that one of the main roles of the primary procedure is prevention of unnecessary secondary procedures. The primary repair of a bilateral cleft lip is a significant challenge; complications can arise during the revision of a poorly repaired bilateral cleft lip.

There is no absolute rule for the precise timing of secondary procedures; however, major cleft centers generally agree regarding the timing for revision surgery for bilateral cleft lip and nose.²

The preschool years (age 4 to 5 years) are a common time to begin secondary revisions.^3–7 These include correction of the vermilion border and lip scars. Although the need for early septoplasty to relieve significant airway obstruction has been documented, nasal correction at this age is limited to repositioning of the lower lateral cartilages.⁸ The timing of canine eruption will determine the appropriate time for correction of alveolar defects. Further lip revision can be performed at the same time if feasible. Definitive septorhinoplasty with osteotomies and dorsal revision is often performed following cessation of facial growth and after any maxillary realignment and alveolar bone grafting is completed.^7–13 The patient should be emotionally mature enough to participate in the decision-making process associated with the septorhinoplasty as the final step in surgical correction. Although minor adjustments of vermilion fullness or deficiency can be performed at any time, if the child’s initial repair was truly inadequate, we advocate for a complete re-repair at any age.

SECONDARY LIP DEFORMITIES

Vermilion Deficiency

Deficiency of midline vermilion is primarily associated with bilateral clefts and is the most common form of vermilion deformity.¹⁴ The whistle deformity can be adequately corrected by reapproximation of the orbicularis oris muscle.¹⁴ The cause of the deformity can be both the lack of normal musculature and mucosa in all dimensions and an inappropriate primary repair.¹⁵ All but the mildest of incomplete bilateral clefts have a relative excess of lateral vermilion tissue, known as festoons (Fig. 55-1). In mild cases, various local tissue rearrangements may compensate or camouflage the deficiency.^4,8,12 For mild deformities, a mucosal V-Y advancement can be used to augment the central vermilion deficiency, lengthen the short upper lip, and narrow the alar base¹⁴ (Fig. 55-2). For more substantial defects, the main issue is usually inadequate primary repair, and the vermilion deficiency will be accompanied by a widened philtrum, deficient columella, and widened nasal base. The proper correction will involve a complete takedown and re-repair (Fig. 55-3). When the vermilion deficiency is the only defect, fat augmentation (injected micrografts or full-thickness dermal fat grafts) is our preferred method for correction of mild to moderate central vermilion deficiency.

Fig. 55-1 Festooning, a relative excess of lateral vermilion tissue.

Fig. 55-2 V-Y mucosal roll-down for whistle lip deformity.

Fig. 55-3 In a patient with a significant whistle deformity, takedown and re-repair are shown.

Fig. 55-4 Constructing an Abbé flap.

Abbé Flap

When local measures of tissue rearrangement have been exhausted, more severe whistle deformities and horizontally deficient lips may require an Abbé flap.16 It was commonly used for severe postsurgical cleft lip deformities, including patients whose premaxilla and prolabium had been completely discarded at a previous operation.^13,17 Currently, the Abbé flap is used less often because of improvements in primary repair techniques. It is the only available procedure that can reconstruct a natural-looking philtrum, Cupid’s bow, and central tubercle.¹⁸ In addition, the donor site simultaneously corrects the relative excess vermilion projection, or “pout,” from the lower lip, thus restoring balance to the entire aesthetic unit. This surgical technique is the best available for addressing significant upper lip tissue deficiencies.

When designing the flap dimensions, emphasis should be placed on reconstructing the entire philtral unit. This will release the abnormal tightness and provide a balanced profile (Fig. 55-4). The flap should preferably be harvested from the center of the lower lip and placed in the aesthetic center of the upper lip.^3,18 This facilitates the appearance of a central vermilion tubercle and Cupid’s bow.¹⁸ It should also be designed slightly smaller than the intended neophiltrum. This provides two benefits: (1) it facilitates closure of the lower lip donor site, and (2) it allows for stretching of the flap once in position. Most horizontally tight lips are vertically short, as well. Vertical lip length should be maximized to match the neophiltrum by moving the apices of the lateral lip elements laterally. Finally, we recommend dividing the flap on postoperative day 10 to 14. To ensure viability, the surgeon can compress the native labial artery pedicle and check capillary refill of the inset flap.

Vermilion Excess

Although the bilateral complete cleft lip, at its foundation, is characterized by displacement and deficiency of tissue, the primary repair commonly results in relative tissue excess. In fact, the most common secondary deformity is a “relative” excess of mucosa of the lateral labial elements or festoons^19,20 (see Fig. 55-1). This is nearly always caused by insufficient medial advancement of the vermilion during the primary repair and, depending on the severity, can be corrected by re-advancement or local tissue rearrangement in the form of Z-plasties within the vermilion (Fig. 55-5).

Fig. 55-5 This patient with a small deformity of the wet-dry junction required scar revision and vermilion Z-plasty.

Fig. 55-6 Takedown and re-repair of a patient with a previous Manchester repair with significant whistle deformity.

The Manchester Repair

The vermilion excess that accompanies a primary Manchester repair is unsightly, dry, and flaky vermilion from the original prolabium. If the patient has had this type of initial repair, a lack of orbicularis oris repair with all the resulting stigmata is also likely. The only option if the patient has had a previous Manchester repair is to completely discard that vermilion and to perform a complete lip revision as if the patient had only had a previous lip adhesion procedure (Fig. 55-6).

Lip Scar

Often, the most noticeable and striking stigmata of a repaired bilateral cleft lip is an unsightly scar. A poor scar is one that even after 18 months remains hyperpigmented, raised, or foreshortened. Contributing factors to poor scarring at the time of cleft repair include inadequate orbicularis oris muscle release and repair, a protruding premaxilla, and excessive cleft width. All of these factors result in a tight initial lip closure and lead to extreme tension and subsequent widening of the lip scar over time. Permanent sutures used at the time of primary repair taken with excessively wide bites and left in for more than a few days will result in a scar with cross-hatching, as well. Although some surgeons believe that the cutaneous lip should never be reopened for revision, we advocate full-thickness scar excision from the prolabial side of the repair and muscle reapproximation if continuity is lacking 3,21,22 (Fig. 55-7). An adequate muscle repair is the basis for successful, permanent correction of widened and prominent scars.²³ Ideally, application of presurgical alveolar molding and premaxillary repositioning will cause the secondary tight lip scar deformity to fade over time. The desired end result is for the scars to simulate the philtral columns.²⁴