47

William Manchester wrote, “No greater problem exists in the whole field of surgery than the successful treatment of a patient suffering from complete, bilateral cleft lip and palate.”1 Although colleagues in other surgical specialties would likely take umbrage with this statement, Manchester’s commitment to these children is clear. He warned that any surgeon who attempts repair of a bilateral cleft lip assumes an onerous responsibility, because “failure to achieve its objectives can result in a lifelong human tragedy that secondary procedures can only partially alleviate.”

I agree with Manchester and all devoted surgeons before me who have struggled to achieve a normal appearance in a child born with a bilateral cleft lip. Surgeons must have passion, precision, patience, and perseverance to undertake this operation and be willing to accept the obligation for periodic evaluation of their results. In a professional lifetime, surgeons have the privilege of following only a small number of these patients into adulthood. These children should not suffer because of a surgeon’s technical wanderings and misadventures. The principles that should be followed in repair of bilateral cleft lip are established. The techniques have evolved in such a way that the results should be equivalent to those for unilateral cleft lip.²

Philosophers tell us that those who do not know history are likely to repeat its mistakes. So before taking a scalpel to an infant with bilateral cleft lip, practitioners have an obligation to learn the lessons of the past.

HISTORY OF BILATERAL CLEFT LIP REPAIR

The projecting premaxilla has always been an obstacle to closure of a bilateral cleft lip. From the Renaissance until the early twentieth century, surgeons either manually in-fractured the premaxilla or summarily excised it, either preserving the prolabium as the central lip or shifting it upward to form the columella. Our more conservative surgical forbearers struggled with ingenious methods of external compression to force the premaxilla into alignment.³ These efforts to reposition the premaxilla presaged the development of passive (removable) and later active (fixed) palatal devices in the middle of the last century.

Simon was the first to pare the edges of the prolabium and construct bilateral labial adhesions to retrude the procumbent premaxilla. His method evolved to the eponym Simonart’s band, often incorrectly applied to a tiny bridge of tissue spanning the upper section of a cleft lip.⁴ Premaxillary surgical setback to permit labial closure was advocated by Veau, Brown, and Cronin.³ Preoperative manipulation of the premaxilla and the concerns about possible deleterious effects on midfacial growth continues to be controversial.

Early surgeons often repaired a bilateral cleft lip in stages, one side first and then the other. The result was loss of symmetry, the one advantage a bilateral cleft lip has over a unilateral cleft lip. A misconception existed that the diminutive prolabium lacks growth potential. Thus various techniques were used to introduce rectangular or triangular flaps from the lateral labial elements to augment philtral height. These procedures resulted in unsightly geometric scars and a central lip that was tight and too long. Variations on a straight-line repair minimized elongation but resulted in an abnormally wide, bowed, or shield-shaped philtrum, especially if the muscular ring was not repaired. Closure of the orbicularis muscle was usually not possible over a procumbent premaxilla, and some surgeons believed that muscular closure would inhibit midfacial growth. For years, debate existed over how to construct the median tubercle: whether to preserve prolabial vermilion or excise and rebuild it with lateral vermilion-mucosal flaps.

Surgeons were puzzled by the distorted nose in children with bilateral cleft lip. They knew that the short columella does not have the capacity to grow, but conceded to the complexity of the nasal deformity and deferred correction. There also was concern that manipulation of the tip cartilages would interfere with nasal growth. Numerous secondary procedures were devised, all based on the apparent need to recruit adjacent tissue to lengthen the columella. Two basic strategies for columellar elongation became popular. The Cronin technique5 involves rotation of bipedicled straps from the nostril sills. This gives only modest columellar length; often the procedure has to be repeated. The other method, the forked-flap technique of Millard³ was once used widely, but has fallen out of favor in most centers. A recent survey found that this procedure is still practiced by 23% of surgeons.⁶ Like all secondary procedures, the forked-flap method causes peculiar tertiary distortions. In retrospect, these secondary procedures were based on the incorrect concept of a need to import soft tissue to build the columella but ignored the dislocated lower lateral cartilages. Primary labial closure accentuates the bilateral nasal deformity by pulling the medial crura inferoposteriorly, further dislocating the domes, and buckling the genua into recurvatum. In short, conventional bilateral lip repair makes the nasal deformity worse.⁷ In addition, deformed and malpositioned lower lateral cartilages are difficult to correct secondarily.

PRINCIPLES

Lessons From Bilateral Cleft Nasolabial Deformities

The nasolabial stigmata after bilateral cleft lip repair are obvious, even at a conversational distance (Fig. 47-1). The lip is too long, is flat, and lacks normal protrusion. Upon closer inspection, the philtrum is wide, bowed, and undimpled. The median tubercle is thin and chapped, particularly if prolabial vermilion mucosa was retained. The free mucosal margins of the lateral labial elements hangs like festoons, and there is excessive dental show (“whistling lip” deformity). The nasal tip is flat and broad, the nostrils have an oblique axis, and the ala nasi are flared (“cat’s knees deformity”). The squatty columella is the centerpiece of these distorted nasolabial features. The snubbed nasal tip and tight upper lip are even more obvious in profile and further accentuated by an everted lower lip and weak chin. This “cleft lip-lower lip deformity” is caused by the child’s struggle for bilabial closure over the abnormally positioned premaxilla, which is protruded at its base, retroclined inferiorly, and vertically overgrown.8 As the child puckers, a muscular bulge appears on each side of the lip, and the alar bases rise when the child smiles.⁸

Fig. 47-1 Stigmata of repaired bilateral complete cleft lip and palate. This 4-year-old boy had labial closure during infancy and columellar lengthening (forked flap) at 2 years of age at a major university center. His nasal width is 35 mm (normal is 28 mm); columellar length is 5.5 mm (normal is 6.9 mm); the philtrum is bowed; and the peak-to-peak measurement of the Cupid’s bow is 14 mm (normal is 8 mm). Also, note the preserved prolabial vermilion mucosa and the thin median tubercle.

The following principles for repair of bilateral cleft lip and correction of the nasal deformity were induced by study of the literature and analysis of the nasolabial distortions that remain using conventional techniques⁷:

1. Preserve symmetry, because even a minor nasolabial difference between the two sides will magnify with growth.
2. Achieve muscular continuity to construct the orbicular ring, eliminate the lateral muscular bulges, and minimize philtral expansion.
3. Design the philtral flap of proper size and shape, because this central labial element will bow and elongate.
4. Construct the median tubercle from the lateral labial elements, because the prolabial vermilion has an abnormal color, lacks a white roll, and will not grow normally.
5. Position the slumped or splayed lower lateral cartilages to form the nasal tip and columella.

Principles 1 through 4 are generally accepted, whereas principle 5 represents a fundamental change in approach to the bilateral cleft lip nasal deformity. Currently, 50% of surgeons report they practice some form of primary nasal correction.⁶

Synchronous Bilateral Nasolabial Repair

Surgical history teaches that complex congenital anomalies that initially required staged correction can, with experience, be repaired in one operation and usually more successfully. This lesson applies to correction of the bilateral cleft lip and nasal deformity.

A few surgeons worked independently toward the goal of synchronous bilateral cleft nasolabial repair. Intraoperative observation and nasal dissection of a stillborn infant with bilateral cleft lip have shown that the alar domes are splayed and caudally dislocated from their normal position overlying the upper lateral cartilages.^7,9 Broadbent and Woolf¹⁰ described a single case of primary medial advancement of the alar domes along with vertical excision of redundant skin in the nasal tip. McComb was in the forefront of primary columellar elongation. He initially tried elevating a forked flap as a first-stage procedure, but became disenchanted with this technique by the time he published his assessment 11 years later.^9,11 Next, he proposed a two-stage primary nasal repair, minus the forked flap, and reviewed his early results 4 years later.^12,13 McComb was clearly on the right track.

I was also struggling to primarily position the lower lateral cartilages in repair of bilateral cleft lip. Initially, I banked the tines of a forked flap, and at a second stage retrieved and rotated the tines intranasally to allow the genua and medial crura to ascend. A vertical tip incision provided access for elevation of intradomal fat and apposition of the domes, and the lateral genua were suspended to the upper lateral cartilages through bilateral rim incisions.⁷ This approach evolved until, by 1987, it became obvious that banked prolabial tines were unnecessary. It was possible to repair a bilateral cleft lip and nasal deformity in a single stage, including construction of the columella.^14,15 The vertical tip incision was abandoned because, with experience, I was able to elevate and secure the lower lateral cartilages through only bilateral rim incisions. Caudal extension of the rim incisions was necessary to sculpt the tip and upper columella.¹⁶

Another approach to the displaced lower lateral cartilages is to elevate a prolabial-columellar flap, based on the paired columellar arteries. Trott and Mohan¹⁷ dissected this flap on the ventral surface of the cartilages. Cutting et al18 described a slightly different open-tip approach. They incise the prolabial-columellar flap along the membranous septum so that the medial and middle crura are in the flap; the genua are apposed with a transvestibular mattress suture.

The principle of primary correction of the bilateral cleft nasal deformity is established; however, the techniques continue to evolve. Tissue from the lip does not need to be secondarily recruited, because “the columella is in the nose.”¹⁶ Synchronous nasolabial repair should be the standard procedure. Children with bilateral clefts no longer need to grow up with stigmata because their surgeon practices antiquated methods.

Four-Dimensional Problems for Three-Dimensional Minds

A surgeon who undertakes repair of a bilateral cleft lip and nasal deformity is different from a sculptor working in stone. It is not enough to visualize the three-dimensional aspects of size, shape, and proportions of the infantile face. The lip and nose must be constructed in anticipation of the changes that occur with growth. These features do not follow the normal patterns in a growing child with a repaired bilateral cleft lip.

Normal nasolabial growth patterns in whites from 1 to 18 years of age have been documented by anthropometry.¹⁹ Nasolabial dimensions grow rapidly and reach more than two thirds of adult size by age 5 years; however, two exceptions are columellar length and nasal tip protrusion. This differential rate of growth correlates with the typical stigmata of a repaired bilateral cleft lip and nasal deformity. The fast-growing features become overly long or wide (the nasal length and width and the philtral dimensions, particularly the distance between the ridges and the peaks of the Cupid’s bow). In contrast, the two slow-growing features remain relatively short (columellar length and nasal protrusion).

The surgeon must make the necessary adjustments in three-dimensional construction of the nasolabial features in expectation of these fourth-dimensional changes. Features programmed for rapid growth and distortion are crafted on a small scale, whereas slow-growing features, those that will never reach normal dimensions, should be constructed on a slightly larger than normal size for an infant.^9,20,21 An exception to these guidelines is construction of the median tubercle. Although it is a fast-growing structure in the normal lip, it lags behind in children with a repaired bilateral cleft lip. Thus the median tubercle should be made as full as possible in anticipation of normal show of the secondary dentition at rest and with smiling.

MANAGEMENT

Preoperative Dentofacial Orthopedics

Three- and four-dimensional nasolabial repair cannot be achieved if the premaxilla is procumbent. Two strategies for preoperative premaxillary manipulation exist: passive and active. A passive appliance is retained by undercuts and dental adhesive; the configuration of the molding plate is altered, by adding or subtracting material, so as to selectively apply pressure on the maxillary elements. A passive appliance maintains, but does not change, the anterior or posterior maxillary width. This can be a problem, because sometimes the lesser maxillary segments collapse so there is insufficient space for the premaxilla. Whenever a passive plate is used, an external force is needed to retract the premaxilla. Bilateral labial adhesion was first described by Simon⁴ in the mid-nineteenth century and sometimes still used in conjunction with a passive plate.²² Labial adhesions are prone to dehisce because there is no muscle in the cutaneous prolabium. For many years, another way to retroposition the premaxilla was to apply traction; the infant was fitted with a head cap, to which an elastic band was attached by hooks and eyes. A strip of tape can be used for the same purpose.

Cutting and Grayson described a more sophisticated variation of active-passive dentofacial orthopedics they call nasoalveolar molding.18,23,24 Passive alveolar molding is done first; once the gap is reduced to less than 5 mm, active nasal molding begins. The nostrils are pushed upward by prongs on an acrylic extension attached to the palatal plate. To further stretch the columella, a horizontal band of soft denture material is added across the nasolabial junction, and a vertical tape is placed from prolabium to appliance to give a downward counterforce. At the same time, the premaxilla is gradually retracted by serial application of tape across the outrigger to the cheeks or to the labia. The tapes are changed daily by the parents; the apparatus must be adjusted weekly to modify the alveolar molding plate. Tape is not used to secure the type of plate and nasal extension described by Bennun and Figueroa.²⁵ Instead, the acrylic intraoral plate lies loosely in the mouth, and by lingual movement during suction and swallowing, force is transmitted through a flexible spring to a nasal extension fitted with a silicone “bumper.” All external traction techniques tend to focus pressure inferior to the basilar premaxilla, causing lingual inclination and bowing of the vomer. Furthermore, it can be difficult to centralize a severely rotated premaxilla using a passive plate and external traction.

The most commonly used active-type dentofacial orthopedic device is based on the prototypical design by Georgiade et al²⁶ and later refined and popularized by Millard and Latham.²⁷ A plaster model of the upper jaw is made during early infancy. The appliance, which is custom made according to Latham’s design, is inserted with the infant under general anesthesia. The acrylic plates are pinned to the maxillary shelves. A looped wire is passed through the neck of the premaxilla, well anterior to the premaxillary-vomerine suture. An elastic chain on each side is connected to the transpremaxillary wire, looped under a pulley in the posterior-superior section of the appliance, and attached to cleats at the anterior edges of the maxillary plate (Fig. 47-2). Each day the parents turn a ratcheted screw in the middle of the device to expand the anterior palatal segments. There is no posterior expansion. The dentist or orthodontist tightens the elastic chains to retract the premaxilla. This requires postinsertion visits at 1, 3, and 5 weeks, then every 2 weeks until the operative date. Usually 6 to 8 weeks are required to align the premaxilla between the expanded palatal segments. Latham’s fixed appliance is most effective in correcting the premaxilla in the anterior-posterior plane; the movement is more retroclination than retroposition. The teeth become retroclined, and the vomer may bend slightly. This appliance can also rectify premaxillary rotation; however, there is little effect on vertical position of the premaxilla.

An enduring belief is that intervention might interfere with midfacial growth and should not be used in the habilitation of children born with cleft lip and palate. Critics of active premaxillary orthopedics have shown long-term evidence for minor midfacial retrusion.^28,29 Other longitudinal studies failed to document serious inhibition of anterior maxillary growth in children managed with active dentofacial orthopedics.^30,31 Whatever the type of preoperative premaxillary manipulation and whatever the operative technique, primary repair of bilateral cleft lip and palate causes some inhibition of vertical and forward maxillary growth. The major goals of repair of bilateral cleft lip and palate are attractive nasolabial appearance and normal speech. These goals should not be forgotten in attempts to minimize restriction of maxillary growth. Midfacial retrusion is predictably corrected by maxillary advancement after completion of growth with additional aesthetic benefits to nasal, labial, and malar projection.

Whatever the possible effect on facial growth, presurgical dentofacial orthopedics is essential to set the stage for synchronous primary repair of the primary palate. Alignment of the maxillary segments permits design of the philtrum in proper proportions, facilitates nasal correction, allows closure of the alveolar gaps, stabilizes the upper arch, and permits closure of the oronasal fistulae. Furthermore, early retropositioning of the premaxilla minimizes nasolabial distortion as the child grows.

Fig. 47-2 A, Latham appliance. B, Before insertion of the appliance. C, Six weeks after dentofacial orthopedic manipulations.

TECHNIQUE

Synchronous bilateral cleft labionasal repair should be the first, and probably the only, procedure performed in a given day. There should be no committee meetings, patients waiting in the office, or other obligations; this is the most important day of the patient’s life since birth.

Markings

While standing at the head of the table, the surgeon elevates the nostrils with a double-ball retractor and the philtral flap is drawn using a sharpened toothpick dipped in brilliant green dye. The midpoint at the columellar-labial junction (subnasale [sn]) is noted, and dots are placed 2 mm apart (crista philtri superior [cphs]–cphs) at the base of the flap. The philtral flap is designed to be 6 to 7 mm in length (sn–labiale superius [ls]) and 3.5 to 4.5 mm from peak-to-peak of the Cupid’s bow (crista philtri inferior [cphi]–cphi). The sides of the philtral flap are drawn slightly biconcave, and on each flank, a tiny rectangular flap is delineated that will be deepithelialized and incorporated to simulate philtral ridges. The columellar base flaps are drawn to the prolabial cutaneous-mucosal junction.

Fig. 47-3 A, Markings for synchronous repair of bilateral cleft lip and nasal deformity. Open circles denote tattooed points. B, Anthropometric points. (al, Ala nasi; c, highest point of columella nasi; cphi, crista philtri inferior; cphs, crista philtri superior; ls, labiale superius; n, nasion; prn, pronasale; sbal, subalare; sn, subnasale; sto, stomion.)

The corresponding Cupid’s bow–peak points on the lateral labial elements are positioned to allow for sufficient white roll for the handle of the bow and ample vermilion height for the median tubercle. The alar base flaps are drawn at the junction with the lateral labial element, and the line of incision is extended inside the nostrils along the vestibular mucosal-cutaneous junction (Fig. 47-3, A).

After markings are completed, record the anthropometric measurements for the nasal features: length (n-sn), width (al-al), projection (sn-prn), and columellar height (sn-c); and for the labial features: philtral flap dimensions (cphs-cphs, cphi-cphi, sn-ls), total labial height (sn-sto), and thickness of the median tubercle (ls-sto) (Fig. 47-3, B).

The labial elements and nose are infiltrated with xylocaine and epinephrine. After a sufficient waiting period, a 30-gauge needle, fitted on the end of a cotton-tipped applicator, is used to tattoo the anatomic points that must be preserved during the dissection, including the vermilion-mucosal line in the lateral elements. Tattoo punctures made before injection of epinephrine tend to bleed excessively, blurring the lines.

Dissection

All the lines are lightly incised, the philtral flanking tabs are deepithelialized, and the excess prolabial skin is excised. The philtral flap is elevated in the loose areolar plane, extending up to the anterior nasal spine.

The lateral labial flaps are disjoined from the alar bases, and the basilar flaps are freed from the piriform attachments. The white line–vermilion–mucosal flaps are incised and separated from the lower edge of the muscular layer, stopping just short of the tattooed lateral Cupid’s bow point. The mucosal incisions in the anterior gingivolabial sulci are extended to the premolar region. With a double hook on the muscular layer and a protecting ring finger on the infra-orbital rim, the surgeon widely releases the lateral labial elements off the anterior maxillae in the supraperiosteal plane (Fig. 47-4, A). The orbicular muscular layers are dissected within the lateral labial elements in the subdermal and submucosal planes (Fig. 47-4, B).

Closure

Repair of the lip and nose is integrated, beginning in the lip, next to the nose, back to the lip, and completed in the nose. The following sequence of steps is suggested as a guide. Minor deviations from this sequence are permissible, but major changes can disrupt and interfere with the construction.

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