Repair of bilateral cleft lip

Principles

Surgical principles, once established, usually endure, whereas surgical techniques frequently change and evolve. The following principles for repair of bilateral cleft lip were induced based on study of the literature and observations of residual deformities :

• 1.

  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.

• 2.

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

• 3.

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

• 4.

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

• 5.

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

Principles 1–4 required definition, interpretation, and confirmation. Principle 5, primary correction of the nasal deformity, is a fundamental change in surgical strategy. The so-called “absent columella” is an illusion; nearby labial tissue need not be recruited to build it. “The columella is in the nose” became the battle cry of the senior author. The columella can be exposed by anatomic positioning and fixation of the lower lateral cartilages and sculpting expanded skin in the soft triangles and upper columella.

Third and fourth dimensions

Analogous to a sculptor working in marble, the surgeon must construct three-dimensional nasolabial features in flesh. Unlike sculpture in stone, the repaired bilateral lip and nasal deformity change with time – there will be normal growth, as well as abnormal alterations of these features. The nasolabial stigmata are attributable to the three-dimensional primary repair and the subsequent fourth-dimensional distortions.

Farkas and colleagues used direct anthropometry to document the normal patterns of nasolabial growth in Caucasians from age 1 to 18 years. Fast-growing nasolabial features attain more than 75% of adult dimensions by age 5 years. For example, nasal height and width develop early, reaching a mean of 77% and 87% of adult size, respectively, by this age. All labial landmarks grow rapidly, reaching approximately 90% of adult proportions by age 5 years. In contrast, tip protrusion and columellar length are slow-growing features; they reach a mean of only two-thirds of adult size by 5 years of age. These differences in nasolabial growth explain the well-recognized nasal deformities and labial misproportions of a repaired bilateral cleft lip. The fast-growing features become overly long or too wide, particularly the interalar and philtral width. In an early study of a small number of patients, it was determined that from time of initial closure to age 5 years, the philtrum widened by a factor of 2.5 at the top and expanded twofold between the peaks of Cupid’s bow. In contrast, nasal tip protrusion and columellar length remain abnormally short following conventional bilateral labial repair.

Applying a posteriori reasoning, the nasolabial features programmed for rapid growth in early childhood must be crafted on a small scale; slow-growing features should be made slightly larger than the normal dimensions for an infant. Construction of the median tubercle is the exception. This normally fast-growing feature reaches 87% of adult height by age 5 years, but after bilateral cleft lip repair, the tubercle lags behind. Therefore, it must be fashioned to be as full as possible, anticipating insufficient growth. Central incisal show is also an unpredictable factor in the fourth dimension. Despite the surgeon’s effort to craft a full median tubercle, augmentation may be necessary after eruption of the permanent central incisors and after the maxilla is in normal sagittal position.

The premaxilla

The projecting premaxilla has long been the stumbling block to closure of the bilateral complete cleft lip. From the Renaissance until the early twentieth century, aggressive surgeons would resect the protruding intermaxillary bone, either shifting cutaneous prolabium upward to elongate the columella or excising it altogether. To retrude the premaxilla, Simon introduced the technique of paring the edges of the prolabium and constructing bilateral labial adhesions. His method is the source of the dubious eponym “Simonart’s bands”. Premaxillary “set-back” by vomerine resection was described in the nineteenth century and continued to be practiced well into the twentieth century by Veau, Brown, McDowell and Byars, Browne, Cronin, and Monroe. The history of this procedure is detailed by Millard in Vol. II of Cleft Craft .

Desault in the late eighteenth century described the use of compressive linen bandages to force the premaxilla backward. These clumsy headcaps and compressive contrivances presaged the development of passive (removable) and later active (fixed) dentofacial orthopedics in the latter half of the twentieth century.

Labial closure

Techniques for labial repair of the bilateral deformity were adapted from those used for the more common unilateral cleft lip. Textbooks and articles well into the mid-twentieth century recommended staged bilateral repair: one side of the cleft (usually the widest) and then the other side. Conspicuous asymmetry was the predictable result. Surgeons believed that the diminutive prolabial element lacked the potential for full growth, and, therefore, had to be lengthened. Thus, various techniques employing rectangular lateral labial flaps inserted beneath the cutaneous prolabium were described by König, Hagedorn and Mirault, and later by Barsky. Insertion of cutaneous lip below the prolabial flap resulted in an unnaturally long lip; this iatrogenic deformity was not well appreciated until the mid-twentieth century. Alternatively, triangular flaps from the lateral labial elements were interdigitated into the prolabial segment to increase philtral height. LeMesurier transposed bilateral quadrilateral flaps below the prolabium; however, later he utilized the entire prolabium without interdigitating flaps. All these methods resulted in curious geometric labial scars (which were often asymmetrical, especially if one side was closed first), a vertically long lip, and a tight lip from side to side. In time, surgeons began to recognize that the philtrum has a remarkable capacity for vertical growth once it is attached to the lateral labial elements. Minor variations on a straight-line type (Veau III) labial closure became the standard design. Although this method diminished elongation of the central lip, the result was typically an abnormally wide, shield- or keystone-shaped philtrum. This was attributed to a failure to attain muscular continuity and to properly design the philtral flap in expectation of growth, especially in the transverse dimension.

How to form the median tubercle is a longstanding dispute. The alternatives are to preserve the prolabial vermilion (resulting in a scar on each side of the median tubercle), retain a tiny strip of central vermilion, or excise it completely and build the tubercle from lateral vermilion–mucosal flaps. If the prolabial vermilion–mucosa is conserved, the free margins of the lateral labial elements tend to hang like swags, flanking a deficient median tubercle that is covered by a thin strip of vermilion and exposed chapped mucosa (“whistling lip deformity”). In profile, the upper lip is flat or convex, lacking a pout. The abnormal contour is further accentuated by an everted lower lip and weak chin. This “cleft lip lower lip deformity” is caused by the child’s struggle to obtain bilabial closure over a protrusive, retroclined, and vertically elongated premaxilla.

Muscular closure

Little attention was given to closure of the orbicularis oris muscle until the middle half of the twentieth century; the early advocates were Schultz, Browne, and Glover and Newcomb. Manchester held to the traditional belief that joining muscle from the lateral elements across the midline would inhibit premaxillary growth. He sutured the orbicularis oris muscle to the central subcutaneous tissue to minimize tension on the premaxilla. Without muscular continuity, a bulge appears on each side of the lip whenever the child puckers. Primary muscular closure is necessary to minimize fourth-dimensional distortions, especially lateral drift of the alar bases and widening of the philtrum. Perhaps equally important, the child is unhappy about being unable to whistle.

With increasing attention to muscular closure in the unilateral deformity, reports began to underscore the importance of orbicularis oris repair in bilateral clefts.

Nasal inattention

Surgeons focused on closure of the bilateral labial cleft and postponed correction of the nasal deformity. Possibly, they were fearful of risking the philtral blood supply or there was concern that manipulation of the nasal cartilages would interfere with growth. The most likely reason was that the short (“absent”) columella was intimidating. Surgeons conceded to the complexity of the bilateral nasal deformity.

Bilateral labial closure produces and accentuates the bilateral cleft lip nasal deformity. The nasal tip is overly broad. The medial crura are pulled into an inferoposterior position; this further dislocates the splayed alar domes and causes the genua to buckle (“recurvatum” deformity) and the alae nasi to flare (“cat’s knees” deformity). The caudal margin of the drooping lower lateral cartilages protrudes into the lateral vestibules producing an oblique ridge or web. Without amends for the vertically long lateral labial elements and normal muscular attachments to the alar bases, an unnatural elevation of the alae nasi occurs, becoming more pronounced when the child smiles. In short, conventional bilateral labial repair worsens the nasal deformity. Furthermore, the deformed and displaced lower lateral cartilages are difficult to correct secondarily.

Secondary nasal correction

Numerous secondary procedures were devised to elongate the “short columella”. The two major technical strategies curiously were published in 1958. The first in print, the Cronin method, involves rotating bipedicled strap flaps from the nostril sills. This procedure results in modest columellar length, and a tendency for loss of the initial gain in height. The second method is the forked flap procedure, devised and popularized by Millard. There are two variations on the forked flap theme. For infants with a wide prolabium, the tines of the forked flap are transposed directly into the columella or banked below the sills and “cashed in” later in early childhood. Alternatively, if the prolabium is relatively narrow, Millard preferred three-stage columellar lengthening: (1) bilateral labial adhesions to expand the prolabium; (2) elevating, rotating, and banking the tines while narrowing the philtrum (around age 18 months); (3) retrieval of the prolabial prongs and elevation, along with the medial crura, to lengthen the columella (at age 2 years).

Millard’s forked flap, like all secondary procedures, causes tertiary distortions that are characteristic of the particular technique. Most secondary methods introduce a nexus of scars across the columellar–labial junction. This causes a transverse nasolabial crease that deepens with smiling and is difficult to surgically correct. Secondary recruitment of tines from each side of the philtrum in an older child usually results in thickened and wide philtral scars. The circumferential philtral scar causes a bulge rather than a dimple. Even in skilled hands, the forked flap procedure results in an unusual-appearing nose: (1) rectangular columella with a broad base and absent waist; (2) sharp columellar–labial angle; (3) abnormally elongated/enlarged nostrils; (4) columellar over-elongation with a disproportionate ratio of nostril length to nasal tip; and (5) downward drift of the columellar base. The medial crura becomes unnaturally positioned in the nasal tip, resulting in a slight break at the columellar–lobular junction. Thus, some surgeons began to wonder whether labial skin belongs in the columella, while others questioned whether the columella was deficient at all. About this time, nasal correction during repair of unilateral cleft lip was becoming more credible. Maybe it would be possible to correct the nasal deformity along with the bilateral labial closure?

Primary repair of the bilateral cleft lip nasal deformity

Nasal dissection of stillborn infants with bilateral labial clefting revealed that the alar domes and middle crura are splayed and subluxed from their normal moorings to the upper lateral cartilages. The columella only appears short because of the malpositioned lower lateral cartilages. Moreover, the investing domal skin is expanded; there is no reason to bring in more tissue. Broadbent and Woolf described a case of primary medial advancement of the alar domes combined with excision of skin from the broad nasal tip. But it was McComb who led the advance toward primary columellar elongation. Initially, he tried elevating a forked flap, followed by second-stage labial closure. He published an assessment 11 years later, including measurements of columellar growth. By this time, however, he was disenchanted with the strategy and presented a new two-stage technique for columellar construction using the tissue in the splayed nasal tip for repair – without a forked flap. In the first stage, an external incision (“flying bird”) opened the nasal tip in order to appose and suspend the splayed genua. His tip incision, which extended almost to the columellar base, was closed V-to-Y fashion, thus shifting the alar margin into the tip and lengthening the upper columella. Long bilateral labial adhesions were part of his first operation; one month later, the lip was reentered for definitive repair. The columellae looked near normal in McComb’s longitudinal assessment at 4 years, and equally good at 18–19 years, although the slight depression caused by the nasal tip scar is noticeable.

The senior author was also focused on early positioning of the lower lateral cartilages. Overly influenced by Millard’s teachings, he banked the tines of the forked flap below the sills during bilateral labial repair. The second stage was a critical departure from Millard, transposing the banked tines intranasally to allow the genua and medial crura to ascend. A vertical nasal tip incision was used to appose the domes, and the lateral crura were suspended to the upper laterals through bilateral rim incisions. Anthropometric measurements in these children showed that nasal length, tip protrusion, and columellar length were within normal limits. But the interalar dimension was overly wide, the mid-columella was broad, and the upper lip was too long. The alar domes often buckled slightly, and there were two instances of nostril stenosis related to the intranasal transposition of the tines. By 1987, it had become obvious that prolabial tines were unnecessary, and the vertical incision in the nasal tip was also abandoned. Primary columellar lengthening and normal nasal tip projection were achieved solely by apposition of the genua, elevation and fixation of the lower lateral cartilages to the ipsilateral upper lateral cartilages, and the sculpting of tissue from the soft triangles and upper portion of the columella.

Other surgeons were also on the trail to primary repair of the primary palate. Trott and Mohan were working in Malaysia, where socioeconomic factors made multistaged bilateral cleft lip repair impractical. They devised a single-stage nasolabial repair using an open rhinoplastic exposure of the dislocated lower lateral cartilages. The prolabial–columellar flap survives on the paired columellar arteries and is dissected on the ventral surface of the medial–middle crura. Cutting and associates described another variation on the open-tip approach following pre-surgical stretching to elongate the columella and nasal lining. The Cutting prolabial–columellar flap is incised along the membranous septum, leaving the medial–middle crura in the flap; the genua and medial crura are apposed by transfixion mattress sutures.

McComb offered a delightful anatomic explanation for the diminutive columella: “… it has been unzipped and its component parts lie within the broad nasal tip”. The old techniques that involved secondary recruitment of prolabial skin into the columella were wrong. The lower lateral cartilages should be placed and fixed in anatomic position followed by trimming and redraping the soft tissues of the nasal tip. No longer would columellae be overly elongated with (often hair-bearing) labial skin, nor would the nasolabial junction be transgressed by a scar that encircled the philtrum.