All patients with a cleft lip deformity have an associated nasal deformity that varies in degree of severity. A three-dimensional understanding of the anatomy of the cleft nose aids surgeons in selecting the proper technique for repair. Analysis and performance of orthognathic surgery should be done before nasal surgery to optimize the overall result. Goals of the secondary rhinoplasty include relief of nasal obstruction, creation of symmetry and definition of the nasal base and tip, and management of nasal scarring and webbing. Septal reconstruction in the cleft nose is a key maneuver in cleft rhinoplasty.
Key points
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A three-dimensional understanding of the anatomy of the cleft nose aids surgeons in selecting the proper technique for repair.
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Advantages of early surgical intervention include minimizing the deformity as the child grows and lessening asymmetries to allow optimal nasal growth.
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Analysis and performance of orthognathic surgery should be done before nasal surgery to optimize the overall result.
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Goals of the secondary rhinoplasty include relief of nasal obstruction, creation of symmetry and definition of the nasal base and tip, and management of nasal scarring and webbing.
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Septal reconstruction in the cleft nose is a key maneuver in cleft rhinoplasty.
Introduction
The nasal deformity associated with congenital cleft lip is a complex defect that results in significant aesthetic and functional problems. The defect involves all tissue layers, including the bony platform of the nose, the inner nasal lining, the cartilaginous infrastructure, and the external skin. The extent of the deformity varies with the degree of lip abnormality; it may be unilateral or bilateral and subtle or complete.
In many patients with congenital clefts, the secondary nasal deformity is minimal. However, the appearance of the nose in some patients with clefts is often the feature that is the most noticeable to the observer. The variability of the secondary cleft nasal deformity is related to the original deformity, scarring from previous surgeries on the lip and nose, and changes related to growth. In addition, many patients with clefts have significant nasal obstruction and functional problems.
The goal of complete care of the cleft nasal deformity is to minimize functional problems and to maximize the appearance of the nose. This goal requires the surgeon to have an understanding of the pathophysiology of clefting, and the three-dimensional nature of the cleft nasal deformity. This article discusses the anatomy and pathophysiology of the cleft lip nasal deformity and the timing of the various repairs needed, and provides a philosophic understanding of a selection of techniques currently used to repair the cleft nasal deformity.
Introduction
The nasal deformity associated with congenital cleft lip is a complex defect that results in significant aesthetic and functional problems. The defect involves all tissue layers, including the bony platform of the nose, the inner nasal lining, the cartilaginous infrastructure, and the external skin. The extent of the deformity varies with the degree of lip abnormality; it may be unilateral or bilateral and subtle or complete.
In many patients with congenital clefts, the secondary nasal deformity is minimal. However, the appearance of the nose in some patients with clefts is often the feature that is the most noticeable to the observer. The variability of the secondary cleft nasal deformity is related to the original deformity, scarring from previous surgeries on the lip and nose, and changes related to growth. In addition, many patients with clefts have significant nasal obstruction and functional problems.
The goal of complete care of the cleft nasal deformity is to minimize functional problems and to maximize the appearance of the nose. This goal requires the surgeon to have an understanding of the pathophysiology of clefting, and the three-dimensional nature of the cleft nasal deformity. This article discusses the anatomy and pathophysiology of the cleft lip nasal deformity and the timing of the various repairs needed, and provides a philosophic understanding of a selection of techniques currently used to repair the cleft nasal deformity.
Anatomy and embryology of the cleft nasal deformity
During normal development, the paired median nasal processes fuse to form the premaxilla, philtrum, columella, and nasal tip. The bilateral maxillary processes form the lateral aspects of the upper lip. Cleft lip deformities result from a failure of the fusion of the median nasal processes with the maxillary processes. Interruption of this embryonic process creates malformation of some or all of the upper lip, central alveolus, and primary palate. The extent of the associated cleft nasal deformity is related to the extent of the interruption of the normal developmental fusion process.
The characteristic unilateral and bilateral cleft nasal deformities can occur along a spectrum of severity. In patients with incomplete cleft lips, these nasal deformities are less pronounced. Even though the nasal defects may be subtle, there is always a nasal abnormality associated with cleft lips.
Unilateral Cleft Lip Nose Deformity
In patients with complete, unilateral cleft lip, the maxilla on the cleft side is deficient. Because of this, the alar base on the cleft side does not fuse in the midline and is positioned more posterior, lateral, and inferior than the alar base on the noncleft side. Consequently, the lateral crus of the lower lateral cartilage (LLC) on the cleft side is lengthened and the medial crura is shortened in relation to the LLC on the noncleft side. The septum is attached to the noncleft maxilla inferiorly, which causes the septum to be deviated to the noncleft side caudally, and bowing dorsally toward the cleft side. The attachment of the upper lateral cartilage to the LLC is affected by the change in position of the LLC, which effectively weakens the scroll region and causes compromise of the internal nasal valve. In addition, the abnormal insertion of the orbicularis oris muscle causes an asymmetric pull on the caudal septum. This pull also adds to the characteristic anterior septal deflection to the noncleft side ( Fig. 1 ).
Bilateral Cleft Lip Nose Deformity
In patients with complete, bilateral cleft lip, the maxilla is deficient bilaterally, which allows the prolabium to have unopposed anterior growth. The alar bases are displaced in a more posterior, lateral, and inferior position than occurs without clefting. The deficient skeletal base leads to longer lateral crura of the LLC bilaterally and short, splayed medial crura. This creates an underprojected, broad, and flat nasal tip. The columella is short because of the malposition of the prolabium and the shortening of the medial crura. The short columella makes the broad and snubbed nasal tip even more pronounced. Insertion of the septopremaxillary ligament is usually symmetric, thereby causing no alteration in the anterior septum/columella unit. Bilateral insertion of the orbicularis oris musculature into the alar base contributes to the widening of the nose and flattening of the LLC ( Fig. 2 ).
Treatment
Timing of the Cleft Nasal Repair
The decision to perform early nasal surgery on children with clefts is based on several factors. These factors include the extent of the deformity and the potential scarring and impact of the procedure on nasal growth. Advantages of early surgical intervention include minimizing the deformity as the child grows, lessening asymmetries to allow optimal nasal growth, and creating favorable conditions for future surgery.
Historically, controversy has existed as to whether primary tip rhinoplasty was a positive influence on the eventual appearance of the nose in patients with clefts. Major septal work and cartilaginous dissection has been thought to negatively affect nasal growth. However, no experimental or clinical studies have ever proved that minor manipulations (without resection) of the nasal tip or nasal base interfere with future nasal growth. For these reasons, most contemporary surgeons agree that the ideal repair of a cleft nasal deformity is performed in 2 stages. The first includes alteration in the nose at the time of lip repair (primary rhinoplasty), delaying a definitive repair until the patient has completed facial growth (secondary rhinoplasty). In female patients, secondary rhinoplasty is generally performed around 15 to 17 years of age, and in male patients at approximately 16 to 18 years of age.
Presurgical Nasoalveolar Molding
Presurgical nasoalveolar molding (PNAM) can be used in patients with wide or very asymmetric clefts to (1) reposition the malaligned alveolar segments, (2) narrow the cleft gap, (3) improve nasal tip symmetry in unilateral clefts, (4) elongate the columella, and (5) expand the nasal soft tissues in bilateral clefts ( Fig. 3 ). PNAM uses an intraoral alveolar molding device with nasal molding prongs. This technique requires a dedicated orthodontist and a motivated family that understands the treatment goals. If properly used, PNAM can lessen the tension across the lip wound and lessen the nasal deformity. Primary rhinoplasty can then be performed to improve nasal appearance and optimize nasal growth.
Primary Rhinoplasty
The purpose of primary rhinoplasty is to close the anterior nasal floor, to relocate the displaced alar base, and to bring early symmetry to the nasal base and tip. This approach allows for both a functional and aesthetic improvement without jeopardizing nasal and facial growth.
After the cleft lip incisions are made and the primary lip dissection is completed, the muscle and soft tissues of the alar base are separated from their maxillary attachments. The malpositioned alar base is freed by creating an internal alotomy at the anterior head of the inferior turbinate. If adequate soft tissue dissection of the alar base is performed, the cleft alar base can be repositioned (during closure) in the optimal three-dimensional position.
The LLC on the cleft side is then dissected from its cutaneous attachments by creating a medial and a lateral tunnel just superficial to the LLCs. These subcutaneous tunnels are connected and allow the cleft LLC to be repositioned in a more symmetric fashion. Care is taken not to violate the vestibular skin, avoiding the complication of secondary adhesions and nostril stenosis.
Primary cleft rhinoplasty begins with closure of the nasal floor and sill. This closure is first started with reapproximation of the musculature of the nasal base, which allows the cleft alar base to be reconstructed in a manner that mirrors the noncleft alar base. Closure of the nasal sill is performed with 5-0 chromic catgut sutures. It is important not to narrow the sill too much. A nasal base that is too wide is easy to narrow secondarily, whereas a stenotic sill is difficult to widen later.
The other component of primary cleft rhinoplasty is to reposition the cleft nasal tip into a more projected, symmetric position. After the nasal sill is reestablished and the lip is repaired in a layered fashion, the cleft LLC is repositioned. This step is achieved with internal mattress or tie-over external bolsters. The new dome has a lengthened medial crus and a shortened lateral crus. The resulting nasal tip is more symmetric, defined, and projected ( Fig. 4 ).
