Cleft lip repair

Brief introduction

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  The multidisciplinary approach is essential to the satisfactory treatment of the cleft patient including: surgeons, orthodontists, speech pathologists, pedodontists, prosthodontists, otolaryngologists, social workers, psychologists, as well as a photographer.

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  The techniques presented for the unilateral cleft lip repair are based on the experience of the members of the Chang Gung Craniofacial Center over a period of 30 years in a Chinese population. They have also been tested in other racially diverse centers. The improved outcomes result from an integrated approach with presurgical management, surgical refinements, and postsurgical maintenance.

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  It is generally believed that isolated cleft palate is a genetic entity distinct from unilateral cleft lip with or without cleft palate.

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  This conclusion arises from both epidemiologic studies and the fact that embryologic events leading to cleft lip/palate and cleft palate occur at somewhat different times (3–7 weeks versus 5–12 weeks).

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  It has long been assumed that both genetic and epigenetic factors play important roles in the etiology of clefts, and this is supported by the varying incidence of clefting with ethnicity, geographic location, and socioeconomic conditions.

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  The incidence of cleft lip/palate in white newborns is approximately 1 in 1000 ( Table 11.1 ); isolated cleft palate occurs in about 0.5 in 1000.

  Table 11.1

  Incidence of cleft lip/palate in differing ethnic groups

  Data from Wyszynski DF, Beaty TH, Maestri NE. Genetics of nonsyndromic oral clefts revisited. Cleft Palate Craniofac J. 1996;33:406–417. Vieira AR, Orioli IM. Candidate genes for nonsyndromic cleft lip and palate. ASDC J Dent Child. 2001;68:229, 272–279.

  Ethnicity	Incidence per 1000 births
  American Indian	3.6
  Japanese	2.1
  Chinese	1.7
  White	1.0
  African American	0.3

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  While there are more than 250 syndromes associated with orofacial clefting, most cases occur as an isolated abnormality, so-called non-syndromic cleft lip/palate.

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  In a large review of their center's experience, Rollnick and Pruzansky identified other malformations in 35% of cleft lip/palate patients and 54% of cleft palate patients.

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  Cleft lip/palate has an unequal gender distribution, favoring boys over girls, whereas this relationship is reversed in cleft palate only.

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  Cleft lip/palate affects the left side more often.

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  A common question from parents pertains to their risk of having another child with a cleft lip/palate:

  • Unaffected (i.e., non-cleft) parents who have one child with cleft lip/palate have an estimated recurrence risk of 4%, increasing to 9% if two children are affected.

  • If one parent is affected, the risk of having a child with cleft lip/palate is also 4%, increasing to 17% if there is already both an affected parent and an affected child.

  • As the degree of familial relationship increases, recurrence risk decreases: first-, second-, and third-degree relatives have 4%, 0.7%, and 0.3% risk, respectively.

  • Recurrence risk increases with the severity of the cleft.

Preoperative considerations

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  With the assistance of ultrasound, prenatal diagnosis of cleft lip is usually made after 16–20 weeks’ gestation.

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  Three-dimensional ultrasonography has improved the accuracy of cleft lip diagnosis and is helpful for prenatal counseling because parents can visualize the face of the fetus clearly.

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  A genetic diagnosis and evaluation for other systemic conditions should be done at the time when a prenatal diagnosis of the cleft is made.

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  The newborn cleft baby should have a pediatric evaluation, and parents are counseled about feeding and given information for subsequent care and treatment.

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  Depending on surgical preference, presurgical nasoalveolar molding (NAM) may be started at 2 weeks or even earlier.

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  If utilized, it usually takes 3–4 months before the completion of the NAM.

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  There are several different treatment plans for surgical correction of the cleft lip deformity ( Figs. 11.1 and 11.2 ) ; however, generally, timing is as follows:

  • Alveolar/NAM:

  • If utilized, typically begins by 2 weeks of age.

  
  

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  Lip repair typically occurs around 3 months of age:

  • With presurgical NAM, a definitive cheiloplasty is done at the age of 3–5 months, when the alveolar gap is narrowed and nasal deformity is improved.

  • When presurgical orthopedics is not available or if the child is older than 3 months, a definitive cheiloplasty with nasal correction is performed.

  • If there is a wide cleft (>12–15 mm) and an associated tissue deficiency, a nasolabial adhesion cheiloplasty is done at 3 months, followed by a definitive cheiloplasty at about 9 months.

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  If the child has an associated cleft palate, a palatoplasty is typically performed at 9–12 months.

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  Timing of alveolar bone grafting relates to the eruption of the central incisor and canine and is frequently determined by the orthodontist usually at the age of 7–11 years.

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  Early intervention for velopharyngeal insufficiency is done as soon as possible on the basis of speech evaluation and nasopharyngoscopy.

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  Secondary correction of nasal deformities and orthognathic surgery, when indicated, are delayed until facial growth is complete.

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  Bilateral cleft lip presents in three major anatomic forms ( Fig. 11.3 ) :

  • Bilateral symmetrical complete (50%).

  • Bilateral symmetrical incomplete (25%).

  • Bilateral asymmetrical (complete/incomplete) (25%).

  

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  The extent of the palatal cleft usually corresponds to the severity of the labial clefts.

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  Minor-form cleft lip extends 3–5 mm above the normal Cupid's bow peak, i.e., 50% or less of the normal cutaneous labial height.

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  Other features are deficient vermillion on medial side of the cleft, cutaneous groove and muscular depression, hypoplastic median tubercle, and minor nasal deformity.

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  Microform cleft lip is characterized by a notched vermillion–cutaneous junction in which the Cupid's bow peak is elevated less than 3 mm above normal.

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  Other features are the same as in a minor form, but they are less obvious.

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  Nasal deformities include small depression of the sill, slightly slumped alar genu, and 1–2 mm lateral displacement (and often under-rotation) of the alar base.

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  Mini-microform cleft lip is distinguished by a disruption of the white roll (vermillion–cutaneous junction) without elevation of the Cupid's bow peak.

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  Usually there is a notch of the free mucosal margin. Muscular depression (particularly noticeable below the nostril sill) is variable as is the cleft nasal deformity.

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  This detailed subcategorization of the contralateral side in an asymmetrical bilateral cleft lip is important because the extent of vermillion–cutaneous disjunction determines the operative strategy.

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  Synchronous bilateral nasolabial repair is indicated for a contralateral incomplete cleft lip, including a minor form.

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  Correction of a contralateral microform or mini-microform is usually deferred until closure on the greater side.

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  Alignment of the three maxillary elements sets the skeletal stage for synchronous bilateral nasolabial repair and minimizes the nasolabial distortions that occur during rapid growth of early childhood.

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  After retrusion and centralization of the premaxilla, the philtral flap can be designed in proper proportions, the nasal tip cartilages can be anatomically positioned, and the alveolar clefts can be closed, which stabilizes the maxillary arch and usually eliminates oronasal fistulas.

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  There are two dentofacial orthopedic strategies: passive and active.

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  Passive strategies include NAM devices.

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  Active strategies include the Latham appliance ( Fig. 11.4 ) .

  

Anatomical/technical pearls

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  Areas of vital concern to the surgeon: the amount of tissue medial to the base of the ala, the vertical height of the lateral lip, the horizontal length of the lateral lip, and the epidermal extension from the columella onto the premaxilla.

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  The discrepancy between the height from the central point of the base of columella to the two peaks of the Cupid's bow is critical for leveling of the Cupid's bow.

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  Vertical length of the lip is more important aesthetically compared with the horizontal length.

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  Therefore, vertical length is seldom sacrificed for horizontal length columella and nasal floor skin.

The vermillion beneath the cleft-side Cupid's bow is always deficient compared with the counterpart vermillion width on the non-cleft side ( Fig. 11.5 ) .

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  Inadequate reconstruction of this deficient vermillion will result in free border deformities as seen in a straight-line vermillion closure.

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  The vermillion medial to the base of the philtral column fits into the deficient vermillion beneath the Cupid's bow.

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  The key concepts of the rotation advancement surgical technique in the unilateral cheiloplasty are as follows:

  • Mohler rotation incision.

  • Mucosal flaps for nasal floor reconstruction, correction of mucosal deficiency in piriform area.

  • Eliminate the perialar incision on advancement flap, limiting scars around the ala base and nostril floor.

  • Mobilization of alar base.

  • Nasal floor reconstruction with complete mucosal closure.

  • Muscle release and reconstruction to simulate the philtral column.

  • Anchoring of advancement flap to nasal septum for centralizing the Cupid's bow.

  • Correction of central vermillion deficiency with triangular vermillion flap from lateral lip.

  • Semi-open rhinoplasty with a reverse-U incision on the cleft side and rim incision on the non-cleft side.

  • Atraumatic dissection to release the fibrofatty tissue from lower lateral cartilages (LLCs).

  • Advancement and fixation of the cleft side LLC to the non-cleft side LLC and to the skin in an overcorrected position.

  • Definition of the alar-facial groove with alar transfixion sutures.

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  The following principles for repair of bilateral cleft lip were induced based on study of the literature and observations of residual deformities:

  • Maintain nasolabial symmetry . Even the slightest differences between the two sides of the lip and nose will become more obvious with growth. Symmetry is the one advantage a bilateral cleft lip has over its unilateral counterpart. It must be maintained.

  • Secure muscular continuity . Construction of a complete oral ring permits normal labial function, eliminates the lateral bulges, and minimizes later distortion of the philtrum and interalar widening.

  • Design the philtral flap of proper size and shape. The philtrum rapidly elongates and widens, particularly at the columellar–labial junction.

  • Construct the median tubercle using lateral vermillion-mucosal elements. There is no white roll in the prolabium. Retained vermillion lacks normal coloration and fails to grow to full height.

  • Position the slumped/splayed LLCs and sculpt excess soft tissue in nasal tip and columella . These maneuvers are necessary to establish normal nasal tip projection and columellar length/width.

Operative techniques