Correction of Secondary Unilateral Cleft Lip and Nose Deformity
H. Steve Byrd, Evan W. Beale
○ Adequate correction of secondary cleft lip and nasal deformities begins with accurate diagnosis of the problem, followed by determination of the underlying anatomic cause.
○ Surgical treatment must address the anatomic elements of soft tissue, muscle, cartilage, and the underlying skeleton and the deficiency, distortion, or excess of each.
○ In a well-balanced but otherwise deficient cleft lip, augmentation of the lip can be performed with a variety of techniques, but microfat grafting is our procedure of choice.
○ Unless a singular and specific underlying cause of a secondary cleft lip deformity is identified, then a major revision with rerotation and advancement is advisable.
○ The primary cleft nasal deformity may be characterized by malposition of the lower lateral cartilage, hypoplasia of the lower lateral cartilage, interruption of the muscle ring across the nasal sill, fixation of the accessory chain of the lateral crus through fibrous connections to the piriform, soft tissue deficiency to the nasal floor, septal deviation, and abnormal muscle insertions at the alar base to the cheek and lip.
○ The surgical steps at primary repair that most influence the outcome of the nasal deformity are (1) adequate release of the lateral crus from its attachments to the piriform and reattachment to the cheek muscles, (2) reconstruction of the muscle ring across the sill and floor, and (3) repositioning of the caudally rotated lateral crus and dome.
○ When primary cleft nasal repair results in (1) adequate lining, (2) release of the lateral ala from the piriform, (3) muscle reconstruction of the sill, and (4) dome repositioning, the secondary surgical correction is commonly limited to alar contour grafting of the lateral crus, caudal septal repositioning, and other routine steps in rhinoplasty.
○ Dorsal deficiency or inadequate tip projection, in association with a deformed ala, indicate a need for rib graft reconstruction.
Major advances have been made in cleft care in the past 50 years. The cleft lip and nose deformity can consistently be transformed, early in life, to a minimal or residual variation of normal. Predictable outcomes for patients with unilateral cleft lip and cleft nasal deformity can be achieved, such that normal appearance and function is a realistic treatment goal.
Despite careful planning and constant effort by the surgeon to achieve the best possible result in the primary cleft repair, secondary cleft deformities are common, occurring in 35% to 75% of patients; the incidence is even higher for cleft nose deformity.1,2 They are referred to, by multiple authors, as the “rule rather than exception.”3,4 Secondary deformities are both intrinsic to the anomaly and iatrogenic.3 Causes include lack of surgeon experience, deficiencies of tissues, individual scar formation, and the effects of facial growth, the last of which cannot be underestimated. The goal of obtaining a “growing repair” that is without deformity initially and that maintains symmetry and balance throughout the patient’s entire growth and development is challenging for even the most experienced cleft surgeon. Furthermore, a growing repair is almost impossible to achieve for a cleft nasal deformity. However, the best treatment for secondary deformities of the cleft lip and nose is prevention at the initial operation. Every effort should be made to achieve a balanced correction of the lip and nose during this first surgery.3
BACKGROUND AND HISTORY
In 1941, James Barrett Brown and Frank McDowell published “Secondary Repair of Cleft Lips and Their Nasal Deformities” in Annals of Surgery,5 as one of the earliest thorough reviews on the topic. Although the techniques used commonly today have evolved greatly, the surgical goals Brown and McDowell set forth are similar to those outlined in this chapter. The following three decades saw a myriad of publications on the subject, many by the leaders of the field, including Millard, Tessier, McIndoe and Rees, Musgrave, and Tajima, among others.6 Techniques evolved to include graft augmentation, lobule complex rotation, and suture suspension techniques.
Knowledge of normal lip and nasal anatomy is essential for an understanding of the distortion caused by a facial cleft. The most important element of a cleft repair is realignment of the muscles. Accurate release and realignment of the muscles comprising the lip and nose are key features in the correction of both the primary and secondary cleft lip and nasal deformity. The ideal lip repair results in symmetrically shaped nostrils, symmetrically placed nasal sills and alar bases, a well-defined philtral dimple and columns, and a natural-appearing Cupid’s bow with a “pout” to the vermilion tubercle. In addition, the repair should result in a functional muscle that, in animation, mimics a normal lip. Although ideally the lip scars approximate natural landmarks, ultimately the eye first focuses on symmetry, and then normal contours of the lip at rest and in animation. The fullness and shape of the free border of the upper lip is a key to these contours.7,8
With improved outcomes in the management of clefts, the repair of the nasal deformity has become more important to families and patients, whose ultimate goal is to restore the face to its full functional and aesthetic capacity. The cleft lip nasal deformity offers a unique challenge to the reconstructive surgeon. Despite the current trend toward nasal reconstruction at the time of primary lip repair, the prevalence of the historic dogma against primary cleft nasal correction results in two classes of patients with secondary cleft nasal deformities: those who underwent primary nasal repair, and those with no primary correction.
In general, patients who have had primary nasal correction are less severely deformed and can be approached with more normal rhinoplasty techniques. Early repair, when achieved with proper release of the tethering forces, has a major role in balancing the muscle forces on the nose and will decrease the degree of lasting nasal deformity. As a result, the algorithm for secondary cleft rhinoplasty varies widely between those patients with and those without primary nasal correction.
Cleft Nose Anatomy
Controversy persists as to whether the primary cleft nasal deformity is secondary to tissue malposition or is associated with tissue deficiencies. McComb9,10 has shown, from his dissections of stillborn fetuses with clefts, that anatomic distortion and malposition is the primary factor in the deformity. This opinion was reinforced by Huffman and Lierie, who proposed that the cleft deformity was due to tissue malposition, and not to a relative size discrepancy.10–13 Despite these published results, our experience in the dissection of stillborn infants with clefts (Fig. 54-1), as well as in open rhinoplasty, suggests that hypoplasia of the lower lateral cartilage complex may be found in association with malposition.14
Malposition is believed to be caused by an imbalance of muscle forces across the cleft. This would explain the deviation of the caudal septum away from the cleft, as well as the lateralization of the cleft dome and the lateral crus.15 Equally significant is the abnormal fixation of the lateral crus to the periosteum of the piriform through a continuation of cartilaginous and fibrous structures.
Hence, we believe that the primary cleft nasal deformity is characterized by some or all of the following components: malposition of the lower lateral cartilage, hypoplasia of the lower lateral cartilage, interruption of the muscle ring across the nasal sill, fixation of the accessory chain of the lateral crus (through fibrous connections) to the piriform rim, soft tissue deficiency to the nasal floor, septal deviation, and abnormal muscle insertions at the alar base to the cheek and lip (Box 54-1).
Fig. 54-1 Dissection of the lower lateral cartilages in a stillborn infant with a unilateral cleft lip shows hypoplasia on the cleft side (right).
• Malposition of the lower lateral cartilage
• Hypoplasia of the lower lateral cartilage
• Interruption of the muscle ring across the nasal sill
• Fixation of the accessory chain of the lateral crus through fibrous connections to the piriform
• Soft tissue deficiency to the nasal floor
• Septal deviation
• Abnormal muscles insertions at the alar base to the cheek and lip
PATIENT SELECTION AND EVALUATION
When analyzing the secondary cleft lip and nasal deformity, multiple aspects of each patient must be considered: the degree of asymmetry, the type of primary cleft repair, the presence of underlying skeletal deformity, and the degree of soft tissue deficiency contributing to the deformity.16–24 Although anthropometric considerations may be used by surgeons while planning and evaluating treatment (sometimes subconsciously), Vegter and Hage25 could find no evidence that patients have an anthropometric interest in their appearance. For example, when asking for secondary correction of the lip, the patient may not want to address the shortness or flatness but rather may want to correct an irregular vermilion border or scar. Although surgeons may not be content with residual stigmata in their patients, the patient ultimately decides whether and which further corrections are indicated. Careful understanding and recording of the patient’s objectives will prevent misjudgments and mistreatment by the surgeon.25
Before planning secondary surgery for the cleft deformity, the surgeon must accurately diagnose all problems associated with the lip, including the lip scar, the status of the orbicularis muscle, the orientation of the vermilion and white roll, the Cupid’s bow, and the mucosa. Skeletal imbalance from maxillary hypoplasia must be identified, because this contributes significantly to the secondary problems commonly seen after initial lip repair.26
Once the deformity has been accurately diagnosed, an attempt to determine the underlying cause is crucial. Although hypoplasia and distortion occur as a result of the malformation and set the stage for many of the commonly seen secondary deformities, poor preoperative design of the primary operation contributes as well. For example, a short lip may be caused by something as simple as a tight contracted scar. On the other hand, underrotation of the initial lip repair or failure to anatomically reunite the underlying orbicularis may be the source of the problem. The design of the secondary surgery should focus on correcting the specific problem and must take into account the soft tissue, muscle, and underlying skeleton. Addressing all short lips with the same operation is not sufficient.
All features of the unrepaired primary cleft nasal deformity may characterize the secondary cleft nasal deformity, plus the added distortion induced by growth.27–29 Lesser degrees of the primary deformity may be seen, depending on what was done at the time of lip repair. The three surgical steps at primary repair that most influence the outcome of the nasal deformity are:
- Adequate release of the lateral crus from its attachments to the piriform and reattachment to the cheek muscles
- Reconstruction of the muscle ring across the sill and floor
- Repositioning of the caudally rotated lateral crus and dome
The Cleft Ala and Tip
The major defect of unrepaired or inadequately repaired cleft lip nasal deformity concerns the position of the ala.30–32 The ala lies caudal and lateral to the contralateral side and is tethered by a pathologic attachment of the accessory chain to the piriform aperture.29 The ala rests on an underdeveloped maxilla, which partly accounts for lowering of the alar base and horizontal nostril seating. The ala may be underdeveloped, hypoplastic, and weak, exhibiting a convoluted shape (Fig. 54-2). This contributes further to lowering of the dome on the cleft side. The clefted orbicularis muscle ring across the nasal sill places unequal muscular pull on the cleft and noncleft sides and adds to the nasal asymmetry and deformity. Malfunction of the cleft ala external valve may be caused by the position of the cleft alar base, imbalanced muscular pull, or abnormal attachment of the cheek muscles to the lateral crus. Because of the abnormal ala, the columella is foreshortened and lies obliquely, with its base directed away from the cleft side.
The septum may be displaced from the vomerine groove, and the cartilaginous portion may be buckled, both of which contribute to nasal tip deviation and narrowing or obstruction of the nasal airway. The caudal septum is displaced away from the cleft, and dorsal septal curvature is present. Clinically significant posterior bony airway obstruction involving the vomer and ethmoid is common.
The Skeletal Framework
The nasal bones are often widened, both at the dorsum and at the frontal process of the maxilla. The dorsum may be low, normal, or overprojecting. Deviation may affect the bony and cartilaginous segments. Generally, midvault curvature is present, with collapse on the concave side and fullness on the convex side (Fig. 54-3).
Fig. 54-2 The unrepaired alar cartilage is weak and contributes to a collapse of the ala.
Fig. 54-3 Midvault curvature can be seen with concavity on the cleft side and convexity on the noncleft side.
Obstruction of the Airway
Aside from the narrowing expected from the deviated septum and bony obstruction, nasal rhinometry has demonstrated statistically significant findings of smaller airways in patients with cleft deformity compared with patients without cleft deformity. Furthermore, external valve malfunction may add to the airway problem.
Analysis of Cleft Nasal Deformities
The key points of our analysis, which influence the algorithm of repair, are as follows:
- Was primary cleft nasal repair done?
A. Was the lateral crus released from the piriform?
B. Is the nasal lining deficient?
C. Was muscle reconstruction across the nasal sill accomplished?
D. Is the external valve patent and functional?
E. Was malposition of the lateral crus and dome corrected?
- Is tip projection adequate?
- Is the cleft lateral crus deformed by a persisting alar crease or buckle?
- Is the alar base recessed and tethered to the piriform?
- Are the piriform and maxilla hypoplastic?
- Is projection of the bony dorsum deficient, normal, or overprojecting?
Age of Intervention
Determining the age to surgically intervene is an important component of a successful outcome. The timing of the surgical intervention should be predicated on the severity of the deformity, the psychosocial and functional effect of the deformity on the child, the stage of facial growth, and the expected effects of such growth on the repair.33 The need for, and timing of, additional surgical procedures (including cleft palate repair, pharyngeal flap, alveolar cleft repair with bone graft, maxillary LeFort I advancement, and rhinoplasty) may also contribute to timing of revisional surgery. The most common age for revisional soft tissue surgery in our practice is 5 to 6 years of age. Occasionally, when a failed primary nasal repair results in severe nasal stigmata, a nasal tip plasty is performed. Such occurrences are almost always in association with severe hypoplasia of the lower lateral cartilage complex, require ear cartilage augmentation, and are generally repaired before the child enters second grade. The goal is to achieve a socially acceptable appearance by the second grade (7 to 8 years of age), when peer interactions change from awareness and curiosity to harmful teasing and ostracism. Revisional surgery may occur at an early age in patients who have been adopted and whose primary repair was performed in another country. Persistent significant deformity in these patients is suggestive of an improper repair, and surgical revision should be performed at least 1 year after the primary procedure.
Another common period for surgical correction is during early adolescence. Post-pubescent development may enhance the lip deformity through asymmetric growth, threatening the child’s self-esteem at this vulnerable age. We plan secondary cleft lip nasal repair after the age of 14 years in girls and after the age of 16 years in boys. The goal is to wait for the post-pubertal growth spurt to be completed in the anterior septum and in the bony dorsum. We do not favor repetitive rhinoplasty procedures throughout the course of childhood, because the final long-term result is greatly compromised. Lip revision may be combined with the definitive rhinoplasty in adolescence if the deformity is minor. Major lip revision (such as rerotation and advancement) should not be combined with rhinoplasty, because the edema and distortion associated with the rhinoplasty can make the lip deformity difficult to precisely correct.
Secondary Unilateral Cleft Lip Deformities
Deformities of the Vermilion Border, White Roll, and Cupid’s Bow
The vermilion border, the distinct convex white roll, and the precise relationship between these two structures are critical in lip repair (Fig. 54-4). The vermilion border at the level of the cleft repair may be malaligned or appear peaked because of a short vertical dimension to the lip (see Fig. 54-4, A). Mild deformities of the vermilion are commonly seen early, within the first 6 months after the rotation advancement repair.34 This is attributable to a degree of scar contracture, which usually resolves with time. Encouraging the parents to provide active massage and silicone sheeting of the scar to facilitate scar resolution and softening of the repair is beneficial. In general, we defer evaluating for revisional surgery on these children until preschool age. In cases in which a minor vermilion mismatch or widened white roll scar are present, we perform a diamond- or keel-shaped excision of the scar.
Reconstruction of the Cupid’s bow and the white roll is crucial in preserving the aesthetic nature of the lip; even slight alterations or misalignments of these areas are overtly noticeable. The white roll is a structure that cannot be duplicated surgically. We no longer advocate Millard’s white roll triangular flap as part of the primary repair, because it may introduce cutaneous and dry vermilion scarring above and below the inset that is noticeable and difficult to correct. However, in secondary cases of white roll separation, in the presence of good cutaneous lip scars, it can be used to not only reconstruct the white roll, but also gain some lip length.
Vermilion notching is a common secondary deformity in patients with cleft lip. The free border of the lip is a composite structure consisting of the orbicularis oris and the overlying tissues, namely subcutaneous fat, vermilion, and mucosa. A deficiency of all or one of these structures is responsible for the vermilion notch.7
Careful analysis of the deformity may reveal misalignment of the profundus muscle, vermilion, or labial mucosa, as well as fullness or hypoplasia of the medial or lateral segment. Malalignment without significant tissue deficit can be corrected by readjustment of available local tissue. This may involve a complete revision of the lip with mobilization and realignment of the superficialis and profundus components of the muscle. Realignment of the vermilion red line can be achieved by advancing the lateral segment excess into a medial segment red-line incision or back-cut.