Cleft Lip Rhinoplasty

Jonathan M. Sykes

INTRODUCTION

All patients with a cleft lip deformity have an associated deformity of the nose. The surgical repair of the cleft nasal defect is performed at the time of the primary cleft lip operation at approximately 10 weeks of age. The goal of the primary rhinoplasty is to lessen the secondary deformity and to allow the nose to grow in a symmetric fashion. A secondary rhinoplasty is sometimes required, and is preferably performed in late adolescence, after facial skeletal growth is complete. Successful cleft rhinoplasty requires a structural approach that rebuilds the nasal infrastructure. The deformity associated with congenital clefting of the lip and nose requires restoration and repositioning of multiple layers of tissue. The nose must be reconstructed on a stable foundation to achieve symmetry, to preserve function, and to correct facial deformity.

In order to properly treat the cleft lip nasal deformity, one must understand the characteristic defects of the nose associated with a unilateral or bilateral cleft lip. This chapter describes the nasal abnormalities associated with cleft lip and outlines the operative techniques and timing of the surgery to correct the cleft nasal deformity.

HISTORY

Ultrasound techniques and advances in perinatal medicine allow many children with a cleft lip and nasal deformity to be diagnosed in utero. Early detection of facial irregularities helps the surgeon counsel the child’s parents and plan for surgical treatment. It is important for families to understand prenatal and hereditary risk factors that may have resulted in the cleft deformity. A geneticist can elicit a family history of any craniofacial abnormalities or syndromes, evaluate genetic risk factors, and provide counseling to families.

Upon learning that their child has a cleft lip and nasal deformities, many parents are anxious to expedite reconstructive surgery. Prenatal, birth, and postnatal histories are of considerable importance in the evaluation of a patient who may undergo future surgical interventions. Further, ensuring adequate growth and weight gain before surgery is paramount. The goal weight prior to surgery is 10 pounds. Cleft lip and cleft nasal deformity are often associated with a cleft palate, which may cause difficulties with nursing. Specialized feeding techniques using bottles with customized nipples, such as the McGovern nipple, facilitates nursing and subsequent improvement in nutrition and weight gain. Nurses especially trained in working with cleft lip and palate patients are a good resource for parents and the surgeon.

PHYSICAL EXAMINATION

The physical examination of a patient with a cleft lip nasal deformity should include an assessment of the severity of the cleft lip along with any other associated defects, such as the presence of an associated cleft palate or cleft alveolus. Additionally, it is important in any child with a congenital cleft malformation involving the lip,
nose, or palate to examine the patient for any associated syndromes, abnormalities, or deformities. Patients who have syndromic clefting should be carefully examined by a knowledgeable pediatric geneticist to identify any potential systemic anomalies.

FIGURE 37.1 Unilateral cleft lip nasal deformity. An infant is shown with a complete left cleft lip, cleft palate, and cleft nasal deformity. The left nostril is displaced inferiorly, laterally, and posteriorly; the nasal tip is flattened, asymmetric, and poorly defined; the floor of the nose is absent.

Patients who have undergone primary surgical treatment for clefting of the lip with an associated nasal deformity may require a secondary nasal surgery, usually in late adolescence. The nature of the secondary cleft rhinoplasty operation depends on the identification of nasal deformities that have persisted following the original repair. Several nasal features must be noted in the physical exam, including alar position, columellar height, tip definition, symmetry, nasal tip projection, and the position of the nasal septum.

Unilateral Cleft Lip Nasal Deformity

A unilateral cleft lip nasal deformity results from a lack of bony support for the nasal base. Depending on the severity of the defect, a cleft nasal deformity can include a common cavity from the nasal floor into the mouth, depression and widening of the ala on the cleft side, flattening of the nasal dome, underprojection of the nasal tip on the cleft side, and deficient bone in the alveolus and maxilla (Fig. 37.1). The features of a unilateral cleft lip nasal deformity are summarized in Table 37.1.

Bilateral Cleft Lip Nasal Deformity

Patients with a bilateral cleft lip deformity have a range of severity in their oral and nasal defects. The common sequelae of a bilateral cleft lip nasal deformity are a shortening of the columella; a wide, flattened appearance of the nasal tip; and a substantial loss of nasal tip support (Fig. 37.2). Clefting and bone loss in the maxillary eminence and bilateral alveolar ridges are important to note. Dentition and alignment of the incisor and canine teeth can be significantly impacted by clefting of the alveolus. Stunted midfacial growth can occur in patients with bilateral cleft lip deformities. Nasal symmetry is often present if the severity of the bilateral cleft lip deformity is equal on both sides. The features of the bilateral cleft lip nasal deformity are summarized in Table 37.2.

Secondary Nasal Deformity

Although the original nasal deformity associated with congenital cleft lips are characteristic, secondary cleft nasal deformities exhibit more variability. Secondary cleft nasal deformities are related to three factors: (1) the original congenital malformation, (2) any repositioning and surgical scarring resulting from prior surgical intervention, and (3) alterations of the nose related to growth. At the time of evaluation for secondary rhinoplasty,
the septum has not typically been corrected with a previous procedure and has characteristics associated with either the unilateral or bilateral cleft lip deformity. In the unilateral cleft lip nasal deformities, the septum is caudally deviated to the noncleft side and posteriorly deviated to the cleft side. In symmetric bilateral cleft lip nasal deformities, the septum is often wide but is relatively in the midline.

TABLE 37.1 Physical Examination Findings of the Unilateral Cleft Lip Nasal Deformity

Nasal tip	Medial crus of lower lateral cartilage is shortened on cleft side Lateral crus of lower lateral cartilage is lengthened on cleft side Total length of the lower lateral cartilage on the cleft side equal to the noncleft side The nasal tip on the cleft side has a flattened appearance and is displaced laterally
Columella	Appears shortened on the cleft side Columellar base is deflected to noncleft side (from orbicularis oris muscle contraction)
Nostril	Has widened, flattened, horizontal appearance
Floor of the nose	Occasionally absent
Nasal septum	Deviated caudally to the noncleft side and posteriorly to the cleft side Deviation typically does not cause nasal airway obstruction
Alar base	Displaced laterally, inferiorly, and posteriorly

FIGURE 37.2 Bilateral cleft lip nasal deformity. A: A male infant is shown with a congenital bilateral symmetric cleft lip in addition to clefting of the secondary palate. He has a mild nasal deformity associated with his bilateral cleft lip deformity and a notched alveolus. B: A male infant with a complete bilateral cleft lip and cleft in the anterior palate is shown. The patient has a more severe nasal deformity than the patient shown in (A) and suffers from a shortened columella, absent nasal floor bilaterally, and an overly protruding premaxilla.

Secondary deformities of the septum and nasal tip can be addressed during definitive septorhinoplasty. In a unilateral cleft lip patient, the nasal tip is underprojected on the cleft side. In bilateral cleft lip nasal deformities, the tip is underprojected bilaterally. The underprojection of the nasal tip is often associated with a deficiency of internal and external skin. Because the underlying deformity is deficient bony skeleton on the cleft side, there is typically inadequate support to the alar base (Fig. 37.3).

INDICATIONS

Patients with a cleft lip will often require surgery to correct associated deformities of the nose that are not addressed primarily with the initial cleft lip repair. The goal of initial repair is to create symmetry and improve nasal tip projection, in addition to closing connections between the floor of the nose and the mouth.

Secondary cleft lip rhinoplasty is performed to correct aesthetic and functional problems of the nose. Indications for revision surgery include a widened nasal base, persistent columellar shortening, poor tip definition, a flattened nasal dorsum, alar asymmetry, and the desire for scar revision. Timing for secondary rhinoplasty is preferably when a patient’s facial growth is complete. Performing an intermediate rhinoplasty before the completion of facial growth may be warranted in school-age children or adolescents with severe nasal deformities that are socially hindering and a source of ridicule.

CONTRAINDICATIONS

Infants who are underweight or malnourished or who suffer from global illness or syndromic sequelae may not be good candidates for any elective primary surgery on a cleft lip nasal deformity. Secondary definitive
rhinoplasty, including major revisions, should be avoided in children and young adolescents who are expected to have further facial growth. Operating on the nose too early in children and adolescents may damage growth plates and create problematic scarring, stunting midfacial growth or nasal growth. If Class III malocclusion is present in patients who have a cleft lip and nasal deformity (Fig. 37.4), orthognathic surgery for malocclusion should precede their planned definitive secondary cleft rhinoplasty.

TABLE 37.2 Physical Examination Findings of the Bilateral Cleft Lip Nasal Deformity

Nasal tip	The medial crura portion of the lower lateral cartilage is shortened bilaterally The lateral crura portion of the lower lateral cartilage is shortened bilaterally The lateral crura are caudally displaced The domes are widened, leading to a flat tip with poor definition
Columella	Short Wide columellar base
Nostril	Horizontal shape bilaterally
Floor of the nose	Usually absent bilaterally
Nasal septum	The septum is midline if the severity of the bilateral cleft lip is equal; if one side is more severe, the septum deviates to the less affected side
Alar base	Widened with excess show of internal nasal lining

FIGURE 37.3 Skeletal changes in a unilateral cleft deformity. The septum is deviated caudally to the noncleft side and posteriorly to the cleft side. The nasal floor is absent, and there is a common cavity between the mouth and the nose.

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