The cleft lip nasal deformity is a complex, three-dimensional problem that challenges any rhinoplasty surgeon. The extent of the nasal deformity is related to the severity of the original cleft malformation and ranges from mild to severe.
The secondary cleft nasal deformity is related to several factors: (1) the original deformity, (2) any interim surgery performed on the nose, lip, and alveolus, and (3) growth pattern of the nose and midface. The decision to perform nasal surgery is determined by the amount of functional breathing issues and aesthetic concerns of the patient. This chapter describes the nasal deformities associated with congenital clefting and outlines the timing and techniques used to correct these deformities.
Anatomy of the Cleft Nasal Deformity
The nasal deformities associated with congenital unilateral cleft lips have been well described and are consistent. The extent of the typical nasal deformity is related to the degree of deficiency of alar base support on the cleft side. The typical characteristics of the unilateral cleft lip nose are described in Table 34-1 .
|Horizontal orientation on cleft side|
|Displaced laterally, posteriorly, and inferiorly|
|Caudal deflection to the noncleft side and posterior deviation to the cleft side|
The hallmark of the unilateral cleft lip nasal deformity is a three-dimensional asymmetry of the nasal tip and alar base ( Figure 34-1 ). The columella and caudal nasal septum always deviate to the noncleft side, secondary to an asymmetric, unopposed pull of the orbicularis oris muscle. The cleft alar base is asymmetric and the cleft ala is displaced laterally, inferiorly, and posteriorly to its noncleft counterpart. The nasal tip is also asymmetric, with the cleft side lower lateral cartilage (LLC) having a shorter medial crus and longer lateral crus than the LLC on the noncleft side ( Figure 34-2 ). The weakened and malpositioned cleft side LLC produces a nostril that is wide and horizontally oriented.
The nasal septum in the unilateral cleft lip nose is deviated caudally to the noncleft side, and is bowed posteriorly to the cleft side ( Figure 34-3 ). In a study of 140 nasal septums in patients with unilateral lip clefting, Crockett and Bumstead found that the bony septum was deviated into the cleft airway in 80% of these patients. Interestingly, most patients with clefting do not complain of nasal obstruction despite having significant deviation and abnormality of the nasal airway. In all likelihood, this tolerance of major abnormalities of the nasal septum can be attributed to the fact that cleft patients become accustomed to their nasal airway anatomy and associated impaired nasal breathing from a very young age.
The etiology of the asymmetry of the cleft alar base is a lack of skeletal support on the cleft side alar base. Deformities of the bony skeleton near the pyriform aperture result in inadequate support of the alar base both medially (at the columella) and laterally (at the alar-facial groove). The lack of medial and lateral support causes introversion of the nasal ala and webbing of the nasal vestibule. The contour of the lateral crus of the LLC is often concave, secondary to the lack of medial and lateral support. Introversion of the cleft ala is defined as a posterior and inferior malposition of the cephalic border of the lateral crus of the LLC. The combination of the lack of skeletal support and the malposition of the cleft LLC causes weakness of the external nasal valve, often further compromising nasal airflow in the cleft patient.
The middle third of the nose in the unilateral cleft lip nasal deformity is also characterized by weakness of the upper lateral cartilages (ULCs) and malposition of these cartilages. Again, this weakness results from inadequate skeletal support and is often manifest by concave ULC. This weakness typically affects the internal nasal valve on the cleft side.
Bilateral Cleft Nasal Deformity
The bilateral cleft lip nasal deformity is also caused by a lack of skeletal support. The bilateral cleft lip nose is usually not grossly asymmetric. Of course, if a marked difference exists on the two sides of the lip, there can be gross asymmetry of the cleft nasal tip and alar base in the bilateral cleft lip patient.
The bilateral cleft lip nose is characterized by a lack of skin and cartilaginous support in the nasal tip. The columella in bilateral deformities is typically short and there is inadequate projection of the nasal tip. The extent of columellar shortening is related to the size, shape, and position of the prolabium and to the severity of the cleft deformity ( Figure 34-4 ). An abnormal junction between the columella and the central aspect of the upper lip is usually present. Characteristics of the bilateral cleft lip nasal deformity are listed in Table 34-2 .
|Horizontal orientation bilaterally|
|Displaced laterally, posteriorly, and inferiorly|
|Usually absent bilaterally|
|In a complete bilateral cleft lip and palate, the septum is midline; however, if cleft on one side is incomplete, the septum deviates toward the less affected side.|
The medial crura of the LLCs are short, and the lateral crura are both long in bilateral clefts ( Figure 34-5 ). This results in underprojection of the nasal tip, and displacement of the alar bases in a posterior, lateral, and inferior location when compared to noncleft patients. If one side of the lip is more involved than the other side, the short columella is typically deviated toward the less involved side, pulling the tip toward that direction. The nostrils in bilateral cleft lip patients are more horizontal than those in noncleft patients. The nasal septum is usually midline, being deviated caudally to the less involved side if asymmetry exists. The middle nasal third exhibits poor cartilaginous support, compromising the internal nasal valve and affecting functional nasal breathing.
Timing of Cleft Nasal Repair
Cleft nasal reconstruction can be divided into primary and secondary repairs. Primary rhinoplasty refers to nasal surgery performed at the time of the initial cleft lip repair. Secondary rhinoplasty refers to any cleft nasal surgery performed after the initial cleft lip repair. Secondary cleft rhinoplasty may be further subdivided into intermediate repairs, usually performed during childhood, and definitive repairs, occurring at the time of full nasal growth.
The decision to perform surgery on patients with cleft deformities is the product of many factors. These include the fact that the child with a nasal malformation is exposed to ridicule during childhood. This philosophy is counterpointed by the surgeon’s knowledge that nasal and midfacial growth is not completed until the mid-to-late teen years. Early surgical intervention is thought to interfere with subsequent nasal growth. The philosophy that early nasal surgery affects subsequent nasal growth is supported by the experimental work of Sarnat and Wexler and Bernstein, who demonstrated nasal growth inhibition in animals after aggressive resection of the nasal septum and overlying mucoperichondrium.
Although traditional philosophy avoids aggressive early nasal surgery fearing nasal growth inhibition, there is a growing trend toward primary cleft nasal repair. In many centers, rhinoplasty at the time of initial cleft repair is the accepted surgical treatment. This philosophy recognizes that maximizing nasal tip projection and nasal and alar base symmetry during lip repair allows the nose to grow in a symmetric fashion. For this reason, most contemporary cleft surgeons perform primary nasal repair on the tip and alar base.
Presurgical Management of the Cleft Nasal Deformity
The traditional approach to management includes a single stage lip repair at 3 months of age, palatoplasty at approximately 1 year of age, alveolar bone grafting at 9 to 11 years, and definitive rhinoplasty after full facial growth has been reached. Nonsurgical repositioning of alveolar segments serves to lessen tension across the lip wound, improve nasal tip symmetry in wide unilateral clefts, and elongate the columella and expand the nasal soft tissues in bilateral clefts. There have been many reports of presurgical devices designed to decrease the cleft gap and minimize the eventual lip and nasal deformity. Presurgical nasoalveolar molding (PNAM) uses an intraoral alveolar molding device with nasal molding prongs ( Figure 34-6 ). Successful use of any presurgical orthopedic devices requires a team approach—a dedicated orthodontist, a flexible and patient surgeon, and an involved, responsive, cooperative, and compliant family ( Figure 34-7 ). If properly performed, PNAM can provide soft tissue expansion and mold the nasal infrastructure, thereby decreasing the nasal deformity.