CONGENITAL ANOMALIES AND PEDIATRIC PLASTIC SURGERY
CHAPTER 19 CLEFT LIP AND PALATE: EMBRYOLOGY, PRINCIPLES, AND TREATMENT
RICHARD A. HOPPER
Cleft lip and palate are the most common congenital craniofacial anomalies. Successful treatment requires technical skill, knowledge of the abnormal anatomy, and appreciation of three-dimensional facial aesthetics. Cleft care requires a collaborative multidisciplinary team. Through self-scrutiny, honest evaluation of the results, and a great deal of imagination, plastic surgeons continue to advance cleft care.
An understanding of head and neck embryology is helpful in the appreciation of the wide spectrum of the cleft lip and palate phenotype. The cranial portion of the human embryo develops early, with the three germ layers (ectoderm, mesoderm, and endoderm) forming in the beginning to middle of the third week of gestation. The ectoderm layer gives rise to the cutaneous and neural systems, with differentiation starting at 20 days. The interaction between ectoderm-derived components at the crest of the neural fold gives rise to a unique cell population of neural crest cells (NCCs). NCCs have the unique ability to remain pluripotent despite their single germ layer origin. NCCs migrate along cleavage planes between germ layers and within the mesoderm to differentiate at their final destination into connective, muscle, nervous, or endocrine tissue, as well as pigment cells.
NCCs that migrate ventro-caudal from the crest come into contact with the pharyngeal endoderm and mesoderm core that surrounds the six aortic arches. This results in a series of mesenchymal swellings termed branchial arches in the fourth week. The six paired branchial arches decrease in size from cranial to caudal. Although the first and largest arch, the mandibular arch, is primarily responsible for development of the anatomy that includes the lip and palate, the fourth arch is responsible for the pharyngeal constrictor, the levator veli palatini, and the palatoglossus muscles, which play a role in the problems and treatments associated with cleft palate. Each branchial arch gives rise to a nerve along with the associated muscles. This muscle–nerve relationship is maintained regardless of the functional interaction of the differentiated structures. Although the tensor veli palatini and levator veli palatini work in close coordination in the mature normal palate and are pathologically tethered through the aponeurosis of the tensor tendon in patients with cleft palate, they retain their distinct innervation based on their embryologic origin. The levator veli palatini, as a fourth arch derived muscle, is innervated by the fourth arch derived superior laryngeal branch of the vagus (cranial nerve X). The fourth arch derived palatoglossus and pharyngeal constrictors have similar innervation. The tensor veli palatini alone, as a derivative of the first arch, is innervated by the trigeminal nerve (cranial nerve V).
The first branchial arch and the mesenchyme ventral to the developing forebrain are responsible for the three named prominences that give rise to the face, mouth, neck, larynx, pharynx, and nasal cavities (Figure 19.1). The first branchial arch contributes the paired maxillary and mandibular prominences, which fuse to form the lateral and caudal components of the primitive stomodeum or mouth. A central process formed by the proliferation of mesenchyme ventral to the forebrain creates the frontonasal prominence (FNP), which forms the cranial portion of the stomodeum. It is important to note that the FNP and its derivatives are not formed by branchial arches, but rather originate from distinct mesenchyme ventral to the branchial arches. These five facial prominences (two paired and one unpaired) are separated by external grooves, but the mesenchyme of all five is continuous, such that unobstructed migration of mesenchymal cells can occur around the stomodeum. Coordinated fusion and communication between these five prominences are essential for normal lip and palate development.
Development of the human face occurs between the 4th and 10th weeks (Figure 19.1B–H). The nasal placodes develop as bilateral thickenings on the surface ectoderm of the infero-lateral aspect of the FNP by the end of the fourth week (Figure 19.1C). As the placodes elevate, medial and lateral nasal prominences develop around the depressed central nasal pit. Medial migration of the maxillary prominence from the first arch effects medial migration of the nasal prominences, such that when they fuse together, the stomodeum is no longer in continuity with the nasal pit, creating the nasal–oral separation (Figure 19.1G).
The medial nasal prominences form the philtrum and Cupid’s bow region of the upper lip, the nasal tip and septum, and the premaxilla back to the incisive foramen. The lateral nasal prominences form the nasal alae. The maxillary prominences form the lateral lip elements that normally fuse with the philtrum derived from the medial nasal prominence. A failure of fusion of a lateral lip element (maxillary prominence) with the philtrum (medial nasal prominence from the FNP) results in a unilateral cleft lip. If both maxillary prominences fail to fuse, a bilateral cleft lip will result. With a failure of fusion to the maxillary prominence, the growth of the medial placode elements (prolabium, premaxilla, and septum) is unbalanced, resulting in the central protrusion seen in a cleft patient.
The formation of the palate is also a result of interaction between the FNP and maxillary prominences. The two medial nasal prominences of the FNP merge to form the median palatine process, which develops the primary palate, whereas the lateral palatine processes derived from the maxillary prominences form the secondary palate (Figure 19.2). During the eighth week, the lateral palatine processes change from their initial vertical orientation to horizontal, within a period of hours. The developing mandible protrudes in synchrony to allow the tongue to descend and leave room for palate fusion. Fusion occurs in both the axial and sagittal planes, with the median palatine process and two lateral palatine processes fusing to form the palate, and the nasal septum descending from the FNP to join the fusion and separate the two nasal cavities (Figure 19.2C–F). Fusion involves focal degeneration of the leading epithelial edges in a process felt to represent “programmed cell death.” Once fused, the mesenchyme of the primary palate and anterior secondary palate ossify into the hard palate, whereas the posterior secondary palate forms muscle to create the dynamic soft palate.
FIGURE 19.1. Illustrations of the progressive stages of the development of the human face. From gestational age of 28 days (A) through 10 weeks (G) there is staged and progressive fusion of the frontonasal (purple), maxillary (orange) and mandibular (blue) prominences.
When there is normal fusion between the FNP and maxillary prominences creating a normal lip and alveolus, but there is lack of fusion between the lateral palatine processes of the opposing maxillary prominences, an isolated cleft of the secondary palate occurs. If, however, the maxillary prominences fuse appropriately, creating a normal secondary palate, but the FNP and maxillary prominences do not fuse, then a cleft lip and cleft of the primary palate will occur. The variety of fusion patterns between these two pathologic scenarios results in the plethora of cleft lip and palate combinations described later in the chapter.
Epidemiology and Etiopathogenesis
Among the cleft lip and palate population, the most common diagnosis is cleft lip and palate (46%), followed by isolated cleft palate (33%) and isolated cleft lip (21%). The majority of bilateral cleft lips (86%) and unilateral cleft lips (68%) are associated with a cleft palate. Unilateral clefts are nine times as common as bilateral clefts and occur twice as frequently on the left side than on the right. Males are predominant in the cleft lip and palate population, whereas isolated cleft palate occurs more commonly in females. In the Caucasian population, cleft lip with or without cleft palate occurs in approximately 1 in 1,000 live births. These entities are twice as common in the Asian population, and approximately half as common in African Americans. This racial heterogeneity is not observed for isolated cleft palate, which has an overall incidence of 0.5 per 1,000 live births.
Both environmental teratogens and genetic factors are implicated in the genesis of cleft lip and palate. Intrauterine exposure to the anticonvulsant phenytoin is associated with a 10-fold increase in the incidence of cleft lip. Maternal smoking during pregnancy doubles the incidence of cleft lip. Other teratogens, such as alcohol, anticonvulsants, and retinoic acid, are associated with malformation patterns that include cleft lip and palate, but have not been directly related to isolated clefts.
Genetic abnormalities can result in syndromes that include clefts of the primary or secondary palates among the developmental fields affected. More than 40% of isolated cleft palates are part of malformation syndromes, compared with less than 15% of cleft lip and palate cases. The most common syndrome associated with cleft lip and palate is van der Woude syndrome with or without lower lip pits or blind sinuses. Microdeletions of chromosome 22q resulting in velocardiofacial, DiGeorge, or conotruncal anomaly syndromes are the most common diagnoses associated with isolated cleft palate. Although there is a recognized genetic component to nonsyndromic cleft lip and/or palate, it appears to be multifactorial. Among other recent studies, a meta-analysis of 13 genome scans by Marazita et al.1 revealed multiple cleft lip/palate genes on 16 chromosomal regions.
FIGURE 19.2. A. Sketch of a sagittal section of the embryonic head at the end of the sixth week showing the median palatine process, or primary palate. B, D, F, and H. Drawings of the roof of the mouth from the 6th to 12th weeks illustrating development of the palate. Thebroken lines in (D) and (F) indicate sites of fusion of the palatine processes. The arrows indicate medial and posterior growth of the lateral palatine processes. C, E, and G. Drawings of the frontal sections of the head illustrating fusion of the lateral palatine processes with each other and the nasal septum, and separation of the nasal and oral cavities.
Parents of a child with a nonsyndromic cleft, or a family history of clefting, always ask about the risk of clefts in subsequent pregnancies. The risk depends on whether the proband has a cleft lip alone (CL), cleft lip with cleft palate (CLP), or a cleft palate alone (CP). If the family has one affected child or parent with CLP, the risk of the child of the next pregnancy having CLP is 4%. If two previous children have CLP, the risk increases to 9%, and if one parent and one child were previously affected, the risk to children of subsequent pregnancies is 17%. For families with a child having CP, the risk of CP to children of subsequent pregnancies is 2%, 6% if one parent has CP, and 15% if one parent and one previous child have CP.
Surgical Evaluation and Classification
The newborn infant with a cleft is ideally evaluated by the cleft team in the first weeks of life. The increasing number of clefts detected by prenatal imaging allows early preparation of the family and introduction to the treatment plan. Patients with cleft lip and/or palate are not a homogenous group. As mentioned above, they can be divided into CL, CP, and CLP; however, the surgical treatment plan requires a more complex classification scheme. The cleft lip deformity is typically divided into unilateral or bilateral, and then subdivided into complete, incomplete, or microform based on the pattern of embryonic fusion described earlier. The width of the cleft deformity and the degree of alveolar arch collapse also play a part in surgical planning, as these directly relate to the degree of associated nasal deformity and the tension and difficulty of the repair. The associated nasal deformity is similarly categorized as mild, moderate, or severe. Mild nasal deformity is characterized by a lateral displacement of the alar base with normal alar contour, minimal columella shortening, and normal dome projection. Moderate nasal deformity has lateral and posterior displacement of the alar base, columella deficiency, and a depressed dome with mild separation of the interdomal space. Severe nasal deformity has an underprojecting alar dome with complete collapse of the lower lateral cartilage and a severe deficiency of columella height with a dramatic interdomal separation. Severe nasal deformities often have a reversed curvature to the alar rim. The nasal deformity is secondary to a three-dimensional distortion of the lower lateral cartilage, described by some as the “tilted tripod.” It is not caused by hypoplasia or deficiency of the cartilage itself.
If a cleft palate is present, it is surgically classified as unilateral, bilateral, or submucous. A submucous cleft results from epithelial fusion of the soft palate, but lack of the programmed mesenchymal fusion described in the embryology section. The width of the cleft is noted as it affects the difficulty of closure.
Although most surgeons use the descriptive classification of cleft deformities during the initial assessment of a patient, other classification systems are often used for outcome research and record keeping. Kernahan and Stark’s “stripped Y” diagrammatic classification scheme and its modifications continue to be used in many cleft centers. It recognizes the embryologic division of the primary (lip and alveolus) and secondary palates at the incisive foramen. Kriens presented a palindromic acronym organization of cleft deformities. The acronym LAHSHAL denotes the bilateral anatomy of lip (L), alveolus (A), hard (H), and soft (S) palates, by convention from right to left. Lowercase letters represent incomplete clefts of the structure; a period denotes no cleft. A bilateral cleft lip with a complete unilateral cleft of the secondary palate, with incomplete clefting of the lip and alveolus on one side would be represented as LAHSal. This system is currently used for the outcomes registry of the American Cleft Palate and Craniofacial Association (ACPA).
Microform Cleft Lip. The microform cleft (Figure 19.3A) is characterized by a furrow or scar transgressing the vertical length of the lip, a vermilion notch, imperfections in the white roll, and varying degrees of vertical lip shortness. Nasal deformity may be present and is sometimes more extensive than the associated deformity of the lip. Surgery is generally indicated but is approached cautiously to avoid a surgical deformity worse than the congenital defect. If there is isolated disruption of the orbicularis oris sphincter, it can be repaired through an intraoral approach.
Unilateral Incomplete Cleft Lip. Unilateral incomplete clefts (Figure 19.3B) are characterized by varying degrees of vertical separation of the lip, but they all have in common an intact nasal sill. They typically require the same surgical technique as a complete cleft lip in order to repair the underlying muscle malposition, with the associated distortion of the septum, alar base, and lip. If the nasal sill skin is normal, and the nasal lining intact, one of the challenges of the incomplete cleft is to elevate the nasal lining from the underlying alveolar cleft to allow repositioning of the alar base while preventing a nasolabial fistula. As with complete clefts, the best time to address the associated nasal and septal deformity is at the time of the primary lip repair.
Unilateral Complete Cleft Lip. Unilateral complete clefts (Figure 19.3C) are characterized by disruption of the lip, nostril sill, and alveolus (complete primary palate). Since there is no skin bridge connecting the alar base to the footplates of the lower lateral cartilages of the nose, unopposed pull of the orbicularis oris muscle results in a more severe nasal deformity than seen in an incomplete cleft lip. The alar base is displaced inferior and posterior, the ipsilateral lower lateral cartilage of the nose is stretched and the natural contour deformed, and the floor of the nasal septum is displaced into thenon-cleft nostril, collapsing the nasal tip support. The critical factors for evaluating unilateral complete clefts are the position of the lesser and greater alveolar segments, the vertical height of the lateral lip element, and the degree of associated nasal deformity. The alveolar (maxillary) segments assume one of four positions: (a) narrow–no collapse; (b) narrow–collapse; (c) wide–no collapse; (d) wide–collapse. “Wide” is determined by an alveolus position lateral to the desired alar base position (i.e., with lip closure the alar base is medial to the alveolus and thereby sitting in the cleft). “Collapse” refers to a palatal displacement of the lateral maxillary segment as predicated by the arch configuration of the medial, non-cleft dental ridge.
Clefts characterized as “narrow–no collapse” with minimal nasal deformity may be treated with presurgical taping to prevent widening of the cleft with growth and feeding, prior to a primary cleft lip repair with primary tip rhinoplasty. Clefts characterized as “narrow–collapse” or “wide–collapse” may benefit from presurgical molding to create the desired arch form, alveolar contact, and nasal anatomy at the time of surgery. Clefts characterized as “wide–collapse” or “wide–no collapse” must be assessed closely by the dental members of the cleft team. If they feel that these cases are deficient in arch mesenchyme, presurgical orthopedics is used to align the arch segments by correcting the collapse, but not to close the alveolar cleft since this will result in a constricted or perhaps locked in arch. External taping can be used to correct the alar base position over the maintained arch form. The use of presurgical orthopedics or aggressive presurgical taping has eliminated the need for preliminary lip adhesion surgery. The primary benefit of a balanced arch configuration at the time of primary lip repair is decreased tension on the lip repair. A secondary benefit is the reduction of alar discrepancy.
Complete Bilateral Cleft Lip. The most obvious aspect of a complete bilateral cleft is the protruding premaxilla (Figure 19.3D). Because of the lack of connection of the premaxilla with the lateral palatal shelves, the premaxilla has not been “reined back” into alignment with the lateral arch segments during fetal development. At the time of birth, the premaxilla protrudes on a vomerine stem. Uncontrolled growth at the premaxillary suture results in over-projection of the premaxilla, with or without rotation and angulation of the segment. Just as the premaxilla is not reined back by the lateral palatal shelves, the lateral palatal shelves are not pulled forward by their attachment to the premaxilla. Without the intervening premaxilla to maintain arch width, the lateral palatal shelves collapse toward the midline. The severity of this disruption of arch morphology varies, and will dictate the tension on the repair, the degree of dissection required, and, ultimately, the final aesthetic result unless it is corrected prior to lip repair. Presurgical orthopedics is employed to achieve this correction prior to surgery.
FIGURE 19.3. The clinical spectrum of cleft lip deformities. A. Microform cleft lip. B. Unilateral incomplete cleft lip. C. Unilateral complete cleft lip. D. Bilateral complete cleft lip. E. Bilateral incomplete cleft lip. F. Hybrid incomplete and complete bilateral cleft lip.
The anterior nasal spine is poorly formed or absent in the bilateral cleft lip deformity, resulting in a retruded area under the base of the septal cartilage and recession of the footplates of the medial crura. The footplates of the lower lateral cartilages are displaced posterior and laterally, which in turn pulls the normal junction (genu) of the medial and lateral crura apart resulting in a broad, flat nasal tip. The recession of the medial crural footplates along with lateralization of the domes and deficient skin produces the typical “absent columella” deformity. The most anterior and inferior extent of the frontonasal process, which normally contributes to the skin between the philtral columns of the lip, forms a wide, short disk, called a prolabium, that appears to hang directly from the nasal tip skin. In conventional techniques, the linear distance from the inferior tip of the prolabium to the nasal tip is inadequate to reconstruct both the central upper lip and columella length. This vertically limited tissue is used to create the central lip element at the cost of inadequate columella length and tip projection. A major benefit of nasoalveolar molding (NAM) is the ability to lengthen both the columella skin and the prolabium prior to surgery, creating enough skin to reconstruct the central lip length without compromising nasal tip projection.
Incomplete Bilateral Cleft Lip. Occasionally, bilateral clefts are incomplete with a near-normal nose, a normally positioned premaxilla, a skin bridge across one or both nasal floors, and clefts involving only the lip (Figure 19.3E). In such circumstances, a rotation-advancement approach, or a triangular flap approach similar to that used in unilateral repairs, can be used either in a single-stage or a two-stage operation. In two-stage repairs one side is closed first, allowed to heal, and then the other side is repaired a short time later. Symmetry is difficult to achieve with a staged approach, and we prefer a single-stage procedure with a bilateral straight-line technique as described later in the chapter. Patients with a complete cleft on one side and an incomplete cleft on the other present a surgical challenge(Figure 19.3F). These cases have the asymmetric nasal deformity of a unilateral complete cleft lip and the paucity of lip tissue of a bilateral cleft. If there is a discrepancy in columella height between the two sides, we will consider a rotation-advancement repair on the complete side to increase columellar length and a straight-line closure on the incomplete side.
Cleft Lip and Palate
The primary palate consists of the lip, alveolus, and anterior palate back to the incisive foramen. The secondary palate consists of the hard and soft palates from the incisive foramen back to the uvula. The presence of a cleft palate introduces feeding difficulties, concerns regarding speech development, and the possibility of impaired facial growth. The width of a primary palate cleft and the degree of collapse are typically increased in the presence of a cleft of the secondary palate. The family is counseled about the increased number of surgical operations that will be required if a cleft palate is present: primary cleft palate repair with intravelar veloplasty; possible secondary surgery on the palatopharyngeal muscle sling, such as a sphincteroplasty or pharyngeal flap; and possible orthognathic surgery at skeletal maturity. The abnormal attachment of the muscles of the soft palate in a cleft palate alters the tension on the pharyngeal drainage of the Eustachian canal, increasing the incidence of ear infections. Myringotomy and grommet tube placement is performed in the majority of infants at the time of either the lip repair or the palate repair to prevent the development of hearing abnormalities.
Isolated Cleft Palate
The infant with isolated cleft palate is examined carefully for manifestations of the Pierre Robin sequence (micrognathia and glossoptosis leading to airway obstruction). The cause of the cleft palate in the Pierre Robin sequence is thought to be mechanical obstruction of the lateral palatine processes as they swing from a vertical to horizontal orientation during palate fusion, and not because of failure of the fusion process secondary to “programmed cell death.” The micrognathia and associated glossoptosis causes this obstruction, resulting in the characteristic wide “horseshoe” cleft palate. Since the associated findings are caused by a “domino effect” starting with the micrognathia, and not a shared etiology, the condition is considered a sequence and not a syndrome. If the Pierre Robin sequence is present, the majority of cases can be treated with positioning and anti-reflux medications. In more severe cases, treatment may include nasopharyngeal airway protection, gavage feedings, and apnea monitoring. A small percentage of Pierre Robin patients require surgical intervention such as tongue–lip adhesion, distraction lengthening of the mandible, or tracheostomy. Because of airway concerns, palatoplasty may be delayed for several months in Pierre Robin patients compared with other cleft palate closures.
Submucous Cleft Palate
The submucous cleft palate is traditionally defined by a triad of deformities: a bifid uvula, absence of the posterior nasal spine resulting in a notched posterior hard palate, and muscular diastasis of the velum resulting in a zona pellucidum. Submucous clefts vary considerably, however, and muscular diastasis can occur in the absence of a bifid uvula. The majority of patients with submucous cleft palate are asymptomatic. Approximately 15% of patients will develop velopharyngeal insufficiency (VPI). VPI correlates with short palatal length, limited mobility, and easy fatigability of the palate. Because the majority of patients with submucous cleft palate remain asymptomatic, a non-operative approach is recommended until speech can be adequately evaluated, which is typically after 3 years of age.
Multidisciplinary Cleft Care
Individuals born with cleft lip and or palate require coordinated care from multiple specialties to optimize treatment outcome. The national standard is in a center with a multidisciplinary cleft team, dedicated to treating cleft-related issues from birth to adulthood. Typical members of a cleft team include an audiologist, dentist, geneticist, nurse, nutritionist/dietitian, oral surgeon, orthodontist, otolaryngologist, pediatrician, plastic surgeon, psychologist, social worker, and speech pathologist (Table 19.1). Attentive team care in the first few months of life will increase the success of primary surgeries by preparing the infant and family medically, physically, and psychologically.
The emphasis is on coordination of subspecialized experts to minimize the number of surgeries performed while maximizing the benefit.
The goal of presurgical orthopedics is to adjust the cleft anatomy such that the surgery is minimized, and the result is optimized. One of the most common and time-tested forms of presurgical orthopedics is early and persistent lip taping in the first month of life up to the time of the primary cleft lip repair. Due to the deformability of infant soft tissue, this gradual force can cause a progressive decrease in cleft width.
More elaborate orthopedic devices involve appliances, which are either active or passive. Generally, active appliances use an acrylic plate and controlled forces, sometimes from extra-oral traction (bonnet with straps), to move the maxillary alveolar segments into approximation. One of the best known active appliances is the pin-retained variety used by Latham2, which is designed to exert a forward force to the lesser posterior segment of the unilateral cleft maxilla. It consists of a two-piece maxillary splint that overlies the palatal shelves and is retained by short medial pins. An expansion screw connecting the two pieces can be moved to adjust the width of the lateral palatal segments. An orthodontic elastic chain is used to retract the premaxilla. By adjustment of these independent controls, the premaxilla is brought back into its proper position in the arch before the primary repair. The Latham device requires a surgical procedure to introduce and remove it.
Passive appliances generally consist of an alveolar molding plate made of a hard outer shell and a soft acrylic lining. By gradual alteration of the tissue surface of the acrylic plate, the alveolar segments are gently molded into the desired shape and position by the direction of alveolar growth. The devices allow continued growth by a passive molding action without permitting medial movement of the buccal segments. Once the segments are in proper position, early lip repair and bone grafting can be performed. This passive molding approach has evolved into the contemporary technique of NAM.
Nasoalveolar Molding. The goal of NAM is not only to minimize the alveolar cleft width through passive acrylic plate molding but also to minimize the associated nasal deformity. The principle is that the high degree of plasticity and lack of elasticity in neonatal cartilage is caused by high levels of hyaluronic acid, a component of the proteoglycan intercellular matrix, as a result of high estrogen levels from exposure to maternal estrogen. During the first 2 to 3 months after birth, active soft tissue and cartilage molding can take place through the application of persistent gentle external forces. This phenomenon was used by Matsuo and Hirose for treating the nasal deformity associated with a unilateral cleft lip with intact nasal floor, and then later developed by Grayson and Cutting3 into the powerful current clinical tool applicable to all forms of clefting.
The technique of NAM starts shortly after birth, with an impression of the intraoral cleft defect using an elastomeric material in an acrylic tray. A conventional molding plate is constructed on the maxillary study model from clear orthodontic resin. The molding plate is applied to the palate and alveolar processes and secured through the use of surgical adhesive tapes applied externally to the cheeks and to an extension from the oral plate that exits the horizontal labial fissure (Figure 19.4). The molding plate is modified at weekly intervals to gradually approximate the alveolar segments. This is achieved through the selective removal of acrylic from the region into which one desires the alveolar bone to grow (“negative sculpting”). At the same time, soft denture liner is added to line the appliance in the region from which one desires the bone to be moved. The ultimate goal of this sequential addition and selective removal of material from the inner walls of the molding plate is to align the alveolar segments and achieve closure of the alveolar gap. The effectiveness of the molding plate is enhanced by adequately supporting the appliance against the palatal tissues and by taping the left and right lip segments together between clinical visits. Once the alveolar cleft is 5 mm or less, the nasal changes of NAM can be achieved by the use of a nasal stent rising from the labial vestibular flange of the acrylic intraoral molding plate. The shape of the nostrils and alar rims is carefully molded through gradual modifications to the shape and position of the nasal stents. A successful NAM result will result in the affected alar rim curving upward into a normal position with a presurgical “pinch test” (Figure 19.4). This result will greatly minimize the dissection required to create alar rim symmetry at the time of surgery. For bilateral cleft lip deformities, the nasal stents are bilateral, with a joining bridge that creates a fulcrum at the columella–labial angle. The prolabium is lengthened over this fulcrum using a vertical tape from the prolabial skin to the molding plate, while the columella is lengthened by the opposing upward pull of the nasal stents. This vertical stretch is critical to create enough skin length to both reconstruct the upper lip and create a columella at the time of primary bilateral cleft lip repair.
Iatrogenic deformities can be inadvertently created by the NAM practitioner. Close communication with the surgeon during the course of molding is important in order to minimize this risk. The more common NAM deformities include effacement of the Cupid’s bow anatomy due to over-stretching of this region during taping, over-lengthening of the lateral lip element from an inferior vector of taping on this skin, and a “mega-nostril” deformity from over-elevation of the alar rim before the gap between the alar base and columella base has been appropriately narrowed.
Effect of Presurgical Orthopedics on Facial Growth. One of the most controversial issues surrounding presurgical orthopedics in infants is a possible negative effect on maxillary growth. Ross4showed in a multicenter study that there is no difference in facial growth between cleft patients treated with or without presurgical orthopedics. On the other hand, Robertson5, in a 10-year follow-up study by a single surgeon, demonstrated that better facial growth was achieved in patients treated with this technique than in control subjects. In another long-term single-surgeon study, Lee et al.6showed that maxillary growth was not inhibited in patients aged 9 to 13 years who had previously undergone presurgical NAM and primary gingivoperiosteoplasty (GPP). In contrast, Berkowitz7 has been openly critical of the Latham and Millard technique of presurgical Latham-type orthopedics, periosteoplasty, and lip adhesion. He reports a higher incidence of anterior and buccal crossbite at 3, 6, 9, and 12 years of age after the procedure when compared with no presurgical orthopedics without GPP. Millard8 reviewed this same clinical database and also reported a higher incidence of anterior crossbite in the POPLA group, but a lower incidence of buccal crossbite. He noted that the two groups had different orthodontic treatment protocols by different orthodontists and that this could have a confounding effect on the results. The variability in the orthopedic and surgical techniques used in all these studies precludes a global conclusion on how to settle this controversy.
Primary Unilateral Cleft Lip Repair
Numerous methods have been described for repair of the cleft lip deformity. A variety of techniques are still practiced in different parts of the world and can usually be identified by the scar pattern. All repairs have in common the use of a lateral lip flap to fill a medial deficit, focusing on correcting the relative shortness of the medial lip element. For example, the LeMesurier repair involves a lateral, quadrilateral flap, whereas the Tennison repair and Rose-Thompson repair employ a lateral triangular flap. These two techniques introduce the flap into the lower half of the medial lip, whereas the Trauner and Millard techniques introduce the lateral flap in the upper half. Most current techniques in North American focus on variations of the triangular flap technique of Randall-Tennison,9,10 or the rotation flap technique of Millard11–13.
There is no agreement on the ideal timing and the technique of repair among established and experienced cleft surgeons. This underscores the fact that more than one treatment plan is acceptable and that comparable outcomes can be achieved with different philosophies. Successful approaches have in common a surgeon who is knowledgeable about the variation in abnormal anatomy among clefts, is comfortable with the details and limitations of the various technique, and is able to combine these two qualities to achieve the optimum surgical result.
FIGURE 19.4. Nasoalveolar molding (NAM) of the unilateral cleft deformity. A. Unilateral left complete cleft lip and palate in a newborn. B. Infant wearing nasoalveolar molding plate with nasal extension. The projecting button seen in the mouth is used to secure the plate to the patient’s cheeks with tape and elastics. The nasal extension is not added to the molding plate until the alveolar cleft is less than 5 mm wide so as to avoid overstretching the nostril. C. Presurgical result of the same patient after a course of NAM. The alveolar segments and premaxilla are aligned, the cleft-side columella is lengthened, the alar bases are in a more symmetrical position, and the cleft alar rim is curved. D. Immediate post-surgery result of primary lip repair with nasal dissection. E. Nasal appearance at 3 years. Although there is a slight drop in alar rim elevation on the cleft side, acceptable symmetry has been maintained.
The author employs a modification of the technique initially described by Mohler, which, in turn, is based on the technique of Millard.12 Compared with the traditional Millard technique, this technique minimizes the alar base skin incisions and places the back-cut used to rotate the medial lip element at the base of the columella instead of the upper lip. With these modifications, the upper lip scar parallels the contralateral philtrum instead of curving across the philtral groove. The remainder of this section focuses on the modified Mohler technique used by the author.
Timing and Treatment Planning. Whenever possible, all complete unilateral cleft lips undergo preoperative NAM at our institutions. Presurgical orthodontic treatment is initiated in the first or second week following birth, with the maximum response occurring during the first 6 weeks. The primary lip repair is scheduled when the patient is approximately 12 weeks of age, at which time closure of the anterior nasal floor and a primary tip rhinoplasty are also performed. If the alveolar segments are appropriately aligned and <2 mm apart, the family is offered a GPP at the time of the surgery. Bone grafts are not employed with early closure of the alveolus. If collapse is present or the gap is too wide, the GPP is deferred.
Correction of the nasal deformity in unilateral clefts is coupled with the rotation-advancement repair along with early anterior septal repositioning. We believe that it is important to minimize the number of secondary surgeries to the nose during the growth phase to minimize scarring and to optimize the final result of a formal open rhinoplasty in adolescence or thereafter.
Anesthesia. General anesthesia is used for all stages of lip repair. A straight, cuffed endotracheal tube is taped to the chin by the surgeon to avoid distortion of the lower lip and alteration of landmarks. The eyes are protected with occlusive patches, and a throat pack is inserted. After marking of landmarks and incisions, an equal mixture of 0.5% lidocaine and 0.25% bupivacaine with 1:200,000 epinephrine is injected in the planned dissection planes of the lip, in the supraperiosteal plane of the cleft-side maxilla, and between the skin and cartilage of the planned nasal dissection. Accurate injection with a minimal volume of fluid maximizes hemostasis and facilitates dissection. An infraorbital nerve block with bupivicaine is used to minimize the early need for analgesics post-surgery.
Surgical Technique. The markings for the modified Mohler rotation-advancement repair used by the author are applied as shown in Figure 19.5. The points in red are tattooed with a 25G needle dipped in gentian violet ink such that they are preserved during the course of the operation. The depth of the Cupid’s bow on the medial lip segment is marked as point 1, with point 2 being the white roll at the height of the Cupid’s bow on the non-cleft side, and point 3 being equidistant on the cleft side. Ideally, the distance between each point should be approximately 2.5 mm, for a final Cupid’s bow width of 5 mm; however, this can be adjusted based on the patient’s anatomy. Point 4 is selected by a number of considerations, the least important of which is the traditional technique of matching the distance from the commissure to Cupid’s bow on the non-cleft side. Instead, it is selected by matching the vermilion and white roll thickness, or bulk of the lateral lip segment with that of the medial site at the Cupid’s bow peak. This point should coincide as closely as possible with the point on the white roll that intersects the arc of a line drawn from the alar base whose length equals the vertical lip height of the non-cleft side (the height from point 4 to point 5 equals that from point 6 to point 2 when the lateral lip is gently straightened without excessive traction). The vertical incision of the lateral lip segment that will be approximated to the medial segment to reconstruct the philtral ridge originates from point 4, crossing perpendicular to the white roll, then curves sharply toward point 7 at the nasal sill. The triangle formed by points 4, 5, and 7 is isosceles, with the height from point 4 to point 7 equaling that from point 4 to point 5. It is important that the base of this isosceles triangle (line 5-7) does not violate the nasal sill. It is deceptive how much of the nostril base is pulled onto the upper lip by the muscle deformity. As a consequence, if line 5-7 is placed in the visible crease that forms in this region, once the muscles have been released and repaired, the incision will fall up into the nostril floor, obliterating the natural fullness found in the part of the alar base. A good rule of thumb is to make line 5-7 such that it is perpendicular to the vermilion border found between points 4 and 7. Although this inferior slope of the incision will appear non-intuitive, once the lateral lip is brought into position, the line 5-7 will become horizontal, and the fullness under the alar base will be preserved.
Point 4 can be chosen on most cleft lips using these two guidelines. In some cases, however, the lateral lip element is vertically deficient, resulting in a point 4 that is too laterally displaced (too close to the commissure) to achieve a minimal tension repair. In these cases, the arc of the lateral vertical incision from point 4 to point 7 can be increased to lengthen the course between the two points. Another option, although rarely required, is to transpose a 2-mm triangular flap from the medial lip element under the C flap into a back-cut at point 4 (not shown in Figure 19.5).
Care is taken during marking to mark the wet–dry red lip border as well as the white roll and vermilion border. The dry vermilion in the medial lip element often diminishes in height as it approaches the cleft, being less than that on the opposing lateral lip element. A small triangle of dry red lip from the lateral lip element with a height that equals the deficiency is created and inset into a back-cut at the wet–dry junction in the medial lip element. If the dry vermilion deficiency is not addressed in this fashion, the patient will have central lip dryness and scabbing from the exposed wet red lip as they age.
FIGURE 19.5. Unilateral cleft lip repair. A. Markings for unilateral complete primary cleft lip repair. M, medial mucosal flap; L, lateral mucosal flap; C, central cutaneous flap. The purple lines mark planned incisions. The dotted light blue line marks the intranasal lateral wall release if an L-flap is to be used as shown in Figure 19.6. The dotted green line marks the posterior lateral nasal wall release to be used if an L-flap is not needed. See text for details. B. Intracleft view of a patient with a unilateral cleft lip and palate, showing the wet–dry vermilion markings and the intraoral markings to align the lip margin during closure of the oral mucosa.
For the medial lip segment incisions, point 8 is chosen as the location of the back-cut of the C flap. Unlike the traditional Millard repair, this point is located approximately 1 mm up on the columella and three-fifths along the width of the columella, toward the non-cleft side. This allows the back-cut scar to be hidden at the base of the columella, instead of on the upper lip. It also creates a vertical scar that mirrors the non-cleft philtral ridge and does not violate the philtral groove. The incision from point 3 to point 8 is the vertical philtral incision of the medial lip segment and defines the non-cleft border of the C flap. Unlike the traditional Millard repair, this incision has only a slight medial curvature in order to create a vertical philtrum. The curvature can be adjusted based on the curvature of the non-cleft philtral ridge. The cleft border of the C flap (from point 3 as it heads up into the nose) parallels the junction of the medial lip skin and the oral mucosa. It is important not to include any mucosa in the C flap, as it will be rotated into the base of the columella to fill the skin deficiency after downward rotation of the medial lip segment. This inferior incision from point 3 extends into the nose, along the natural demarcation between the oral and nasal mucosa, to create a small septal mucosal flap that will be used to repair the nasal floor back to the incisive foramen and prevent a nasolabial fistula.
Points 3 and 4 on the white roll are tattooed with needle and ink to facilitate alignment at the end of the repair. The author finds that when two marks are used to identify each of these points, there is less chance of error in alignment of the vermilion border. One mark is placed on the height of the white roll and one at the top of the white roll. The other areas that are tattooed include the following: (1) the wet–dry red lip junction on each side of the cleft, since this is effaced during surgical swelling; (2) points 10 and 11 inside the nostril that are marked equidistant from points 5 and 6, such that these two points will be symmetric once appropriate rotation of the alar base has been achieved; and (3) points 12 and 13 (not shown) that lie inside the lip on the wet mucosa, equidistant from the wet–dry junction, with point 12 being directly under point 3 on the undersurface of the lip, and point 13 being under point 4. These latter marks facilitate symmetric intraoral closure of the mucosa when suturing and allow a symmetric full-thickness cut through the muscle of both red lips from points 3 and 4 to these two inside mucosa points.
The lip is then infiltrated with lidocaine and epinephrine as described above (see Anesthesia). After the skin incisions are complete with a micro-knife, the red lip portions of the medial and lateral segments are everted to equal fullness, and a no. 11 blade is used to transect the red lip mucosa and marginal component of the orbicularis oris between points 3 and 12 and 4 and 13, respectively. The superior labial arteries are identified and cauterized. The anterior border of the L-flap is marked by the incision from points 4 to 7. The posterior border of the L-flap starts at point 13 and parallels incision 4-7 until it enters into the nose at the level of the palatal shelf, following the natural demarcation between nasal and oral mucosa. This mucosal incision is extended as far posterior as possible. At this point, the decision is made whether the L-flap is required to expand the lateral nasal wall. In most clefts, the L-flap can be avoided to avoid any anterior intranasal incisions or flaps. The lateral nasal wall mucosa can be undermined as a subperiosteal superiorly based flap which is back-cut vertically as far posterior in the nose as possible (point 9, Figure 19.5), behind the piriform rim (green dotted line in Figure 19.5). In this fashion, the lateral nasal wall movement needed to elevate the displaced alar base anterior and superior occurs through opening of the back-cut inside the nose, leaving the exposed lateral nasal wall bone to re-mucosalize. However, in severe clefts and many bilateral clefts, the alar base–columella base discrepancy is too great to leave a back-cut over the bone, in which case the back-cut is made just behind the lateral crus of the lower lateral cartilage and the L-flap is inset and sutured into this more anterior defect (blue dotted line in Figure 19.5; Figure 19.6). When the L-flap is elevated, it is a posterior based mucosal flap pedicled off the lateral nasal wall, posterior to the lateral crus of the lower lateral cartilage. The base of the L-flap is left thick by dissecting in the subperiosteal plane on the piriform aperture. The L-flap is not inserted until the muscle release described below has been completed and the alar base is mobile.
FIGURE 19.6. Nasal lining release and inset of L-flap. A. The constricted lateral nasal lining is released with an incision behind the lateral crus as marked by the dotted green line in Figure 19.5. The mucosal L-flap is elevated from the lateral lip element. B. The L-flap is pedicled off the lateral nasal wall and inset into the lining defect to support the new position of the alar base. C. The inferior edge of the inset L-flap can now be brought across the cleft and sutured to a minimal vomer flap to close the nasolabial fistula and bring the alar base into a more symmetric position.
With elevation of the L- and M-flaps in a submucosal plane, the underlying orbicularis muscle can be judiciously separated from the overlying skin and underlying mucosa. With dissection of the muscle of the medial lip segment, care must be taken not to violate the midline of the philtrum to avoid distorting the natural groove. The red lip mucosa and white roll are not separated from the underlying marginal component of the orbicularis oris muscle in order to permit normal animation of this area. The nasal and perioral components of the orbicularis oris muscle are separate at the exposed muscle edge of the lateral lip segments. The separation of these two components is a judgment call to decide how much muscle will be used to create the bulk under the alar base, and how much will be needed to create the transverse fibers of the upper lip.
The medial lip segment is lengthened and rotated inferiorly by sequentially releasing the skin with a back-cut at the base of the columella described above, then the muscle with a separation of the nasal and perioral components of the orbicularis oris, followed by the mucosa at the frenum. Care is taken not to fully release the frenum if possible to avoid creating a long-lip deformity. At the end of the medial lip segment release and rotation, points 1, 2, and 3, the landmarks of the Cupid’s bow, should be aligned horizontally with minimal inferior traction and no distortion of the columella–labial angle.
Angled nasal tip scissors are used to dissect between the footplates of the lower lateral cartilages by accessing them underneath the C flap. A vertical incision is made through the nasal mucosa in the area of the membranous septum between the anterior edge of the cartilaginous septum and the posterior edge of the ascending limb of the lower lateral cartilage within the cleft-side nostril. This releases the cleft-side lower lateral cartilage footplate, allowing differential elevation of this cartilage and associated nasal tip relative to the non-cleft side. Scissor dissection then continues between the ascending limbs of the lower lateral cartilages, over the nasal tip, and along the alar component of the cleft-side lower lateral cartilage. The skin is carefully separated from the lower lateral cartilage over the alar rim to allow the skin envelope to redrape when the cartilage is repositioned. This dissection pocket between the cartilage and overlying skin is extended up to the upper lateral cartilage (ULC) of the non-cleft side. This continuous dissection plane between the non-cleft upper lateral and cleft lower lateral cartilages will later be used to place subcutaneous Tajima suspension sutures to adjust the alar rim contour.
The final dissection involves releasing the abnormal attachments of the cleft alar base to allow tension-free approximation across the cleft. An upper gingivobuccal sulcus incision is performed on the cleft side and continued as a supra-periosteal dissection over the face of the maxilla. Through this incision, the abnormal fibrous attachments of the cleft-side accessory nasal cartilages to the lateral piriform aperture are released. The dissection of the nasal component of the orbicularis oris muscle from the oral component continues with this exposure directly under the skin of the alar crease. If these two muscle flaps are separated up to the overlying dermis, then when they are repaired to their partners across the cleft, the natural appearing triangular depression forms at the height of the nasolabial crease just under the alar base. Some surgeons advocate creating an incision directly along the alar crease to achieve this same effect, but the author believes that this additional scar can be avoided through a careful subdermal dissection. All areas are checked carefully for hemostasis before the closure begins.
Closure begins with the nasal floor. If used the L-flap is rotated, trimmed, and sutured into the defect created in the lateral nasal lining when the cleft alar base is advanced into the appropriate position (Figure 19.6). With execution of the dissection described above, the alar base should be able to be brought into symmetric position with the non-cleft alar base in all three dimensions, without undue tension. As the alar base is brought into position, the inferior edge of the lateral nasal wall flap lining is sutured to the opposing septal mucosal flap created from the intranasal incision extending from point 3 to close the nasal floor from the nasal sill back to the incisive foramen. At the end of this nasal floor closure, the posterior displacement of the cleft alar base should be corrected. Closure of the nasal floor to the incisive foramen at the time of primary lip repair will avoid any oronasal or nasolabial communication after the remaining nasal floor reconstruction during the later cleft palate repair. If this detail is omitted from the lip repair, the child will be forced to deal with an anterior nasolabial fistula until closure can be performed at the time of secondary alveolar bone grafting.
Lip construction is achieved by everting the red lip on either side of the cleft to even fullness and then advancing and closing the lateral lip segment mucosa to the medial lip segment mucosa. The M-flap can be rotated into the defect from the releasing back-cut at the frenum if necessary, or it can be used to augment the labial sulcus. After the lip mucosa is closed, the white roll should be aligned across the cleft, and the red lip should have equal fullness. If the lateral red lip is thin, the lateral lip flap had not been adequately advanced toward the midline during the mucosa closure and the lip is inverted.
A sound muscle reconstruction forms the foundation of a good cleft lip repair, creating a nasal component that supports the cleft alar base, a transverse oral component that gives natural animation and length, and a marginal component that allows symmetry of the red lip. The nasal component of the orbicularis oris is repaired first. A 4-0 polydioxanone suture (PDS) is used to secure the nasal component of the orbicularis that had been dissected subdermal under the alar base as previously described to the muscle and periosteum in the region of the anterior nasal spine. The point of suture on the medial lip differs from patient to patient and requires different rotations of the nasal muscle, and different vertical placement, to achieve symmetry with the non-cleft alar base. The perioral components of the medial and lateral lip segments are approximated across the cleft using buried horizontal mattress sutures of 5-0 vicryl to create a philtral ridge and construct the oral sphincter. Once the oral and nasal components have been differentially advanced into their desired positions, it is important to then secure these two muscle flaps together with a buried vicryl suture under the cleft nasal lining repair. If this is not done, the lip muscles can separate from the nasal repair, either lengthening the lip with time, or providing insufficient support to the alar base. The vertical skin incision of the lip is closed with buried 5-0 resorbable monofilament dermal sutures, followed by sparse, non-strangulating, 6-0 interrupted nylon sutures. Care is taken to ensure that the tattooed marks of the white roll on either side of the cleft are aligned.
To address the residual deformities of the nose, a retractor is used to slightly overcorrect the cleft alar rim and underlying lower lateral cartilage in an advanced and superior position. This slides the released cleft lower lateral cartilage footplate toward the nasal dome in relation to the non-cleft side. A series of 4-0 PDS transfixion sutures are used to secure this new relationship of the ascending limbs of the lower lateral cartilages to the anterior septum. This elevation of the cleft-side alar rim and lengthening of the columella leaves a defect from the back-cut at the base of the columella. The C flap is trimmed to fit and rotate into this defect. The rotation point of the C flap creates a natural flare to the base of the columella of the cleft nostril.
The final sculpting of the nostril shape is achieved with a 4-0 PDS subcutaneous Tajima suspension suture. The needle enters the nasal surface of the cleft lower lateral cartilage at the point of desired elevation, enters into the previously described subcutaneous nasal tip dissection pocket, exits into the non-cleft nostril at the level of the ULC, and then returns on its path, such that tightening of the suture will elevate the cleft alar rim. Lateral alar cinch sutures of 4-0 PDS can also be used to contour the lateral alar rim and nasal lining in the new position, by exiting and entering the same percutaneous hole in the alar groove. The number of suspension and cinch sutures required will depend on the degree of the deformity. With good NAM results, the degree of nasal dissection and number of sutures required are minimized. With this approach, the lip and nasal deformities can be addressed in a single surgery.
Unilateral Incomplete Cleft Lip Operative Technique. The unilateral incomplete cleft lip deformity is treated with the same surgical technique and dissection that was described for the complete cleft lip, but with a few modifications. Failure to address all the lip and nasal abnormalities in the incomplete cleft lip with the same detail paid to the wide complete cleft will result in a suboptimal result.
Compared with the complete cleft lip repair, the incomplete cleft repair does not involve intranasal incisions. If possible, the nasal sill is not violated by the vertical incision. If the nasal base is wide compared with the non-cleft side, a small wedge can be removed from the nasal sill to create symmetry. If any nasal sill is resected, it is vital that the excision be minimal, because over-resection with scarring will result in the recalcitrant micronostril deformity.
The L-flap and M-flap are not required for the incomplete cleft lip repair because the nasal floor is intact. To correct the alar base malposition, the abnormal attachments of the nasal cartilages to the piriform aperture must be released as in the complete cleft technique, but the nasal floor lining must also be dissected free from the piriform rim. The thin nasal floor is firmly attached to the edge of the piriform opening and can easily be perforated if care is not taken. Failure to release the nasal lining from the underlying bone will make it impossible to mobilize the alar base into the desired advanced and medial position.
The nasal deformity is addressed with the same dissection as the complete cleft; however, the vertical nasal lining incision behind the ascending limb of the cleft lower lateral cartilage is not available for improved access to the nasal tip. Angled nasal tip scissors are used to access the nasal tip between the footplates of the lower lateral cartilages; if necessary, the nasal tip can be approached laterally from the supraperiosteal maxillary dissection plane.
Microform Cleft Operative Technique. The critical factor when evaluating the microform cleft is the vertical height of the lip. If the vertical height of the affected side approximates that of the normal side, imperfections in the vermilion along the skin furrow can be eliminated with an elliptical excision and a straight-line repair. Triangular flaps of the white roll and vermilion can be used to balance the closure.
When the vertical difference exceeds 1 to 2 mm, the modified Mohler rotation-advancement repair described above (see Unilateral Incomplete Cleft Lip Operative Technique) is used. The additional scar underneath the sill and columella is preferable to a loss of definition in the involved philtral column, which invariably results with straight-line closure when the elliptical excision is extended to provide the desired lengthening.
The correction of a very mild nasal deformity is deferred in the microform cleft requiring a straight-line repair, as the repair does not necessitate a perialar incision. If the deformity remains minimal, treatment is postponed until late adolescence, when a definitive rhinoplasty is performed. With a moderate nasal deformity and with mild deformities requiring a rotation-advancement lip repair, correction of the nasal deformity is carried out with the lip repair.
Primary Septoplasty. The anterior cartilaginous deformity associated with unilateral cleft lip involves displacement of the inferior footplate away from the midline of the anterior nasal spine into the piriform floor of the non-cleft nostril. The deformity is believed to be secondary to the unopposed pull of the nasal component of the orbicularis oris on the non-cleft side. The curvature of the cartilage associated with this displacement causes a tilting of the septum and columella, resulting in decrease in tip support and nasal projection.
A recent publication has reported on the benefit of correction of the septal deviation at the time of the primary cleft lip repair.14 We have been practicing this technique at our institution for the past 5 years and have found the same benefits. Prior to muscle repair, the anterior septum can be approached under the medial crura of the lower lateral cartilages. It is important not to strip the mucoperiosteum from the vertical premaxillary suture located a few millimeters behind the anterior septal edge. Sharp dissection is performed on the inferior surface of the curved septal cartilage, releasing the fibrous bands that hold it to the non-cleft piriform floor. Once released, the immature cartilage can be straightened and repositioned on the other side of the anterior nasal spine. Unlike adult cartilage, the cartilage does not need to be scored to change the memory of the curvature. In most cases, the repositioned septum is stable in its new position, but a 5-0 PDS suture can be used to secure the base to the periosteum. Successful repositioning of the caudal septum corrects the columella tilting and lifts the nasal dome.
Postoperative Care. Although we previously used soft arm restraints for 2 weeks after lip repair, we no longer find them necessary and have had no problems with self-inflicted dehiscence. Care must be taken not to overuse morphine in these patients, since it is one of the most common root causes of series of sentinel events in these patients.
We do not impose restrictions and allow return to the preoperative routine immediately. Diet is advanced to full-strength formula or breast milk on the day of surgery to pacify the infant. Some surgeons have described a preference to feed with a catheter-tip syringe fitted with a small, red, rubber catheter for the first 10 days postoperatively to minimize strain on the muscle and skin sutures and to avoid trauma to the repaired velum.
Suture line care consists of regular cleansing with half-strength hydrogen peroxide followed with a light coating of antibiotic ointment. Sutures are removed on or after the third postoperative day. After suture removal, taping and silicone scar gel is encouraged. Parents are told to expect firmness in the lip scar and temporary shortening across the repair that generally becomes maximum 4 to 6 weeks after surgery. Scars typically soften between 3 and 6 months postoperatively. If there is early evidence of aggressive hypertrophic scar formation, intralesional injection of a dilute steroid can be considered, but is rarely required.
Primary Bilateral Cleft Lip Repair
Bilateral cleft lip repair presents different challenges from unilateral cleft lip repair. Although the lip repair is made more difficult by the deficiency of skin and muscle overlying the premaxilla, it is the associated bilateral nasal deformity that has been a continuing challenge to correct. The treatment of the complete bilateral cleft and associated nasal deformity remains in transition. Only recently, because of NAM, have the results of one-stage primary bilateral cleft lip and nose repair begun to approach those of unilateral cleft lip and nose repair. Previous multistage techniques often produced a lip and nose that were still quite abnormal, with a confluence of scars at the lip–columella junction, a broad nasal tip, an unstable premaxilla, and often large nasolabial fistulas. Results fell short of ideal because the condition was viewed as a purely cutaneous deformity. Over the past decade, techniques advocated by McComb, Mulliken,15 Cutting, Trott, and others have recognized the importance of addressing the contribution of the nasal tip cartilages and lip muscle to the cleft deformity. Previous techniques such as the banked forked flap procedure and Cronin procedure focused on using the width of the prolabium to repair the cleft lip with the least amount of tension, and then at a second stage, after the lip has relaxed, use the lip tissue to treat the up-to-now ignored columella and nasal anatomy. Current techniques instead place the focus on how to get the muscle and cartilage in appropriate anatomic position, such that the growth and molding forces that occur in the years after lip and nose repair result in a progressive improvement, instead of deterioration of form and function. This shift from a skin-based to a cartilage- and muscle-based paradigm has produced a number of techniques with improved outcomes.
Manchester- Versus Millard-Based Techniques. There are two general methods for constructing the central lip vermilion. One involves using the mucosa visible on the inferior aspect of the prolabial skin to form the central vermilion, such as used in the Manchester repair. The original Manchester repair did not create an orbicularis oris sling across the upper lip, but instead sutured the muscle to the edges of the premaxilla. As there was no muscle under the prolabium or within the buccal mucosa, this approach did not provide sufficient bulk to serve as the central lip vermilion and resulted in an abnormal appearance with animation of the central upper lip. A number of techniques have been described to address this limitation, including bringing strips of muscle across this area from the lateral lip, and de-epithelializing the buccal mucosa or subcutaneous tissue from the lateral sides of the prolabium and folding them behind the inferior prolabial mucosa. An advantage of using the prolabial or buccal mucosa to create the central vermilion is that very little bulk of the lateral segment of the vermilion is required, thereby decreasing the tension required for closure across the cleft. The disadvantages of this technique are that (a) there are two parallel scars across the red lip, (b) the central red lip does not have sufficient bulk, resulting in a whistle deformity, and (c) the central buccal mucosa does not possess the same minor salivary gland distribution as the lateral vermilion tissues, often resulting in a dry, chapped, central vermilion segment. The author has needed to revise the lips of numerous older patients with Manchester-type repairs suffering from these problems, converting them to a more functional Millard-type repair.
A second approach is to use the vermilion tissue from the lateral lip segments to create the central vermilion as a variation of the technique described by Millard. The muscle of the lateral lip elements rotates down with the full-thickness vermilion flaps and can create a satisfactory central vermilion construction with a single vertical scar in the red lip under the depth of the Cupid’s bow.
Surgical Technique. The author uses a modified Millard-type repair for bilateral complete lip. The considerations when making the lateral lip markings are similar to those described for the unilateral cleft lip repair (Figure 19.7). The major difference is that point 4 is not located at the same level of the cut through the red lip, but is instead located above the white roll, 2.5 mm lateral to the red lip transaction. This creates a 2.5-mm wide flap on each lateral lip element consisting of red lip and white roll. These two flaps are used to reconstruct the central white roll and red lip inferior to the prolabium.
For the prolabium skin markings, the prolabium is gently placed under traction to find the central vertical axis. Point 1 is placed at this center axis directly above the mucosa–skin junction. No mucosa from the prolabium is used for the anterior lip repair, but is instead used for the sulcus repair of the anterior vestibule. Points 2 and 3 are at the same skin–mucosa junction, 2.5 mm on each side of point 1. A small curvilinear incision is made between the three points to create a scar that simulates the Cupid’s bow. Points 4 and 5 are located at the desired columella–labial crease, just below the natural flare of the base of the columella. Placing these points too high to try to gain more prolabial length will result in distortion of the natural fullness at the base of the columella. The lateral incisions from points 2 and 3 follow the mucosa–skin border, with care taken not to include any mucosa. They extend into the nose, under the footplates of the lower lateral cartilages, and along the demarcation of the nasal and palate mucosa. As described with the incision extending from point 3 into the nose in the unilateral cleft lip repair, this extension of the incision into the nose creates a limited superiorly based nasal septal flap that is later sutured to the lateral nasal wall flap to repair the nasal floor. The medial incisions from points 2 and 3 stop below the columella at points 4 and 5.
The lateral lip dissection, including the decision to use an L-flap or a posterior nasal wall back-cut, is the same as previously described in the unilateral cleft lip repair. Attention to dissecting the oral and nasal components of the orbicularis oris, releasing the accessory cartilages of the nose to the piriform rim, and the use of an upper gingivobuccal sulcus incision to mobilize the lateral lip is also shared. Once the lateral lip elements have been dissected and the alar bases and lateral nasal linings mobilized, attention is shifted to the prolabial dissection.
Bilateral cleft lip dissection places the prolabial skin at risk for necrosis. Meticulous dissection and good judgment is required to avoid this devastating potential complication. Once elevated, the superiorly based prolabial flap is perfused retrograde from the nasal blood supply through the ascending columella vessels. The columella vessels can be destroyed during the flap elevation, the nasal tip dissection and sutures can disrupt the retrograde flow from the nasal dorsum, and the lateral muscle dissection under the alar creases can cut the angular branch of the facial artery contribution to the overall nasal perfusion.
After the skin incisions are made, the prolabial flap bordered by points 2, 3, 4, and 5 is elevated off the underlying mucosa. The dissection is therefore submucosal and not subdermal. The superiorly based mucosal flap, M, will later be used to reconstruct the anterior vestibule. From underneath the elevated prolabial skin flap, scissor dissection is performed between the footplates of the lower lateral cartilages over the genu of the lower lateral cartilages and up to the ULCs bilaterally. This subcutaneous nasal dissection is the same as described in the unilateral technique, but is performed bilaterally.
As with the unilateral repair, the first reconstruction is the nasal floor. The L-flap or lateral nasal mucosal flap is swung across the cleft and sutured to the septal flap using 5-0 vicryl sutures. Care is taken to advance the alar bases during this nasal floor closure to avoid restricting their appropriate placement. For the intraoral repair, the thinned mucosal flap (M) elevated off the prolabium is draped over the premaxilla and quilted down to the periosteum of the exposed anterior surface. This creates a new sulcus edge at the level of the columella base, which is anatomic. The lateral oral mucosa flaps are then advanced across the clefts and secured at the midline to this new sulcus in the region of what would be the anterior nasal spine. If this creation of a neosulcus is not performed, the central oral mucosa will prolapse over the central teeth and create a long-lip deformity with redundant mucosa. Once the nasal lining, intraoral lining, and sulcus have been repaired, the alar bases are brought into their appropriate position by suturing the opposing dissected nasal muscle flaps to the periosteum of the upper premaxilla. It is important to suspend this subnasal muscle sling high, just above the recreated oral sulcus, in order to support the nose. With the alar bases in position, the oral muscle flaps are brought over the premaxilla and sutured to each other. Although this can be the most challenging part in repairing wide clefts, it is essential to get muscle continuity across the lip. The oral and nasal muscle flaps are then secured to each other under the nose. It is even more important to do this in the bilateral repair to prevent the tight transverse oral muscle sling from separating from the secured nasal muscle sling. If separation occurs, the lip muscles slip over and inferior to the premaxilla, creating a long-lip deformity. The marginal lip muscle is then repaired and the opposing white roll flaps sutured under the prolabium. A z-plasty can be placed at the wet–dry vermilion junction of each lateral lip flap to create a tubercle. The prolabial flap is thinned as much as possible given the vascularity, and when insetting, a deep suture is placed in the sagittal plane, from the dermis to the nasal sling muscle. This creates a columella–labial angle and prevents the obliteration of this angle when the prolabial flap is inset. The forked flaps created lateral to lines 2-5 and 3-4 are not banked, but are instead trimmed to fit the remaining defect under the nose at the base of the columella.
FIGURE 19.7. Repair of unmolded bilateral cleft lip deformity. A. Markings and landmarks for bilateral cleft lip repair technique. P, prolabial flap; L, lateral mucosal flap; M, medial mucosal flap. See text for details. B. Bilateral complete lip before surgical repair. C. Immediate postoperative result in the same patient. D. Nine months after the operation. There is good symmetry with minimal labial scars. The width of the prolabium and interdomal space have slightly increased at the expense of the columella height.
The postoperative care of the bilateral cleft patient is the same as for the unilateral cleft patient.
Complications Following Cleft Lip Repair
A retrospective review of 23 institutions in the Pediatric Health Information System database reported that 1.4% had a serious medical complication (primarily airway related) in the first 24 hours after surgery and that 1.9% had an unscheduled readmission post-discharge. Although most primary cleft lip repairs are routine elective procedures, the rate of early serious complications was associated with medical comorbidities and a surgeon with lower clinical volumes.16
Lip adhesion is still occasionally used for wide clefts by surgeons not working with a team or those patients with lateral maxillary collapse that does not respond to presurgical maxillary orthopedics. We have not used it in our institution, even with wide clefts that have elected not to undergo presurgical molding. Some supporters of lip adhesion cling to the belief that it improves maxillary arch alignment and enables a more predictable correction of the cleft nasal deformity in select patients. The improved nasal results are thought to be secondary to improved alar base arch support, which reduces the strain and relapse tendency for the mobilized lower lateral cartilage.
The adhesion is classified as a straight-line muscle repair and begins with the complete marking of a rotation-advancement cheiloplasty. An L-flap is elevated from the lateral segment beginning approximately 3 mm medial to the Cupid’s bow peak. This flap length provides adequate tissue for nasal release. The flap is turned 90° into the nasal release along the lateral floor of the nose, which follows the piriform rim and the lateral portion of the nasal bones. A contiguous, maxillary sulcus incision is made through this nasal mucosal incision, and the lateral lip and cheek muscle mass is elevated in continuity from the maxilla and piriform aperture. The L-flap is sutured into the nasal defect, and the lateral lip element is advanced medially for closure.
An M-flap is also raised 3 mm from the Cupid’s bow peak to maintain symmetry of repair. The mucosal flap is based on the maxillary alveolus and is turned into the alveolar cleft to augment closure. All dissection is maintained outside the margins for primary lip repair. No medial muscle dissection is done at this stage.
Closure is achieved with sutures placed in the undissected orbicularis layer along the paired margin and is reinforced with a chromic catgut mucosal closure between the M-flap and the lateral lip mucosa. Skin is generally closed with interrupted 5-0 chromic catgut, with sutures placed outside the markings for definitive cheiloplasty. The adhesion effectively closes the nasal sill and upper two-thirds of the lip. The forces from the overlying muscle closure have an immediate effect on the position of the alveolar segments.
Primary Cleft Palate Repair
Although cleft lip and cleft palate surgeries are linked by a shared patient population, and both require a complete understanding of the abnormal anatomy by the surgeon, they are surprisingly different. A cleft lip repair is an artistic, flexible technique tailored to the unique three-dimensional anatomy of each child, whereas a cleft palate repair is a technical recipe, the success of which depends on precise and atraumatic execution. Following a cleft lip repair, the parents appreciate the hours of work of the surgeon because of the visible incisions and facial difference, whereas following a cleft palate repair, the key portions of the operation, namely the nasal closure and the intravelar veloplasty, are hidden in the mouth by the transposed oral flaps. The success of the cleft lip repair can be predicted at the end of the operation; results of the cleft palate repair take years to assess and cannot be evaluated definitively until the commencement of speech and completion of facial growth. Despite the lack of surgical glamour associated with a palatoplasty, the patient with a cleft palate requires multidisciplinary evaluation and treatment, a technically sound operation, and standardized postoperative care to achieve the desired results while minimizing the potentially severe complications.
Timing of Surgery. The optimum timing of cleft palate repair balances the benefit of normal velopharyngeal function to optimize speech development against the potential disadvantage of impaired facial growth secondary to early surgical trauma. Graber’s description in the late 1940s of restricted maxillary growth following early palate closure was accompanied by a recommendation to delay surgery until 4 to 6 years of age. Because of the deleterious implications of this recommendation on speech development, the conventional timing for cleft palate repair was arbitrarily set at 18 to 24 months as a compromise between speech and facial growth. The current consensus, based on an increased understanding of speech development, is that cleft palate repair should be completed before 18 months of age; however, there is no general agreement regarding the earliest that surgery can be performed. Since Graber’s earlier work, there have been a number of studies indicating that impaired maxillary growth in cleft patients is independent of cleft palate repair and may result from the lip repair alone or may be an intrinsic phenomenon.
Results from previous retrospective studies examining the effect of timing of cleft palate repair on speech development are inconsistent and are compromised by small study numbers and potentially confounding variables. The one thing that the surgical community agrees on is that long-term, well-designed prospective studies are required before the optimum timing of cleft palate repair can be determined.
There are currently two common approaches to the timing of cleft palate repair in North America: (a) two-stage repair, with the soft palate repair and veloplasty performed at the time of lip adhesion or primary lip repair, and the hard palate repaired before 18 months, or delayed further with the use of an obturator; and (b) single-stage repair around the age of 11 to 12 months. Our center practices the latter approach, delaying the surgery until the time when the child starts to demonstrate the introduction of plosives (b, d, and g) in their speech. It is at this time that they require an intact velopharyngeal sphincter to continue with normal speech mechanics. In children with airway issues, such as those associated with micrognathia of Pierre Robin sequence, the procedure can be delayed until age 14 to 18 months to allow further mandible growth and to decrease the chance of postoperative airway compromise.
Cleft Palate Repair Technique. The two common cleft palate repair techniques are a two-flap palatoplasty with intravelar veloplasty as a modification of the technique described by Veau, Wardill, and Kilner (the “Oxford” palatoplasty), or a single-stage two-flap palatoplasty with Furlow double-opposing z-plasty to achieve the levator repositioning and lengthening of the palate. Both techniques share a common approach to the hard palate and the goal of creating transverse orientation of the reconstructed levator sling.
The patient is placed in the supine position, with a shoulder roll to extend the neck. A number of mouth retractors have been designed for the operation, but all retract the lips and tongue, open the jaws, and keep the endotracheal tube out of the operative site. Care must be taken not to hyperextend the neck, not to strangulate the tongue, and not to bruise the lips. The mouth and nasal cavities are cleaned with normal saline and a small throat pack is placed. The hard and soft palates and the nasal septum are infiltrated with lidocaine and epinephrine, avoiding injection directly around the greater palatine vascular pedicle. With pressure, the mucoperiosteum can be hydrodissected from the hard palate with the injection to facilitate elevation of the flaps.
The lateral aspect of the mucoperiosteal flaps are incised at the junction between the hard palate mucosa and the attached gingiva, and then the anterior portions of the flap are elevated from the hard palate. With a curved elevator, through this lateral incision, the nasal mucosa can be elevated from the lateral nasal wall on the cleft side and posterior nasal spine on the non-cleft side in continuity with the oral flaps. The medial aspects of the flaps are released along the length of the cleft from alveolus to the tip of uvula, following the visible junction between the oral and nasal mucosa. Care must be taken not to leave the nasal flaps deficient. The oral flaps can always be mobilized to the midline, whereas the mobility of the nasal flaps is limited if they are cut too short. For the Veau technique, the anterior tips of the mucoperiosteal flaps are released to increase visualization of the greater palatine pedicle, which is carefully preserved and dissected circumferentially. For the Langenbeck technique, the anterior attachment is left intact, such that the flaps are bi-pedicled to improve vascularity and decrease dissection in the region of the premaxillary suture. The disadvantage of the Langenbeck technique is that it may leave an anterior fistula behind the alveolus unless care is taken to raise a gingivolabial flap to close this portion. The visibility of the pedicle is also decreased.
Two structures tether the oral mucoperiosteal flaps and limit their mobilization across the cleft at the level of the posterior nasal spine. The first is the greater palatine pedicle, and the second is the abnormal attachment of the levator veli palatini and tensor palatini muscles to the posterior hard palate. A number of techniques have been described for lengthening of the pedicle, including osteotomies of the foramen to release the pedicle from the bone, or circumferential release of the periosteal cuff around the pedicle. One or both of these techniques may be required to achieve tension-free closure of the oral lining. The pedicle dissection should be performed before release of the muscle from the posterior hard palate. If the pedicle is compromised during the dissection, the muscle attachments are required to perfuse the mucoperiosteal flaps.
The nasal lining is then separated from the muscles of the soft palate using sharp fine scissors. There is no reliable dissection plane within the first 2 or 3 mm of the cleft edge, and we prefer to leave this edge of the nasal lining flap thick to help with suturing. Immediately beyond the cleft edge, however, there is a defined, gray, smooth dissection plane. The dissection continues laterally to the lingual surface of the medial pterygoid plate. The nasal lining is dissected off the medial pterygoid plate in a subperiosteal plane down to the skull base so that the nasal flaps can be approximated across the cleft with minimal tension.
The final stage of the dissection is the intravelar veloplasty, which is essential for normal speech development. As described by Sommerlad,17 the normal velum consists of the levator muscle in the middle 40% and the tensor aponeurosis in the anterior 33%. In the cleft palate anomaly, the two muscles are closely related, with the tensor aponeurosis attaching to the posterior border of the hard palate and the levator inserting at the margins of the cleft in the anterior half of the velum. The abnormal attachment of the tensor can be directly visualized at the posterior shelf of the hard palate as obliquely oriented fibers. The fibers are sharply released from the edge of the hard palate, and the tensor tendon is divided medial to the hamulus. This allows mobilization of the levator muscle, so that it can be retrotransposed across the cleft.
For an intervelar veloplasty, the levator is dissected sharply from the overlying palatoglossus on its oral surface as described by Cutting, or the oral lining is dissected under the submucous glands as described by Sommerlad. In either technique, care must be taken to leave a thick, well-perfused oral mucosa flap that can be advanced medially across the cleft, independent of the posterior-medial rotation of the levator sling. The repositioning of the levator sling from an oblique orientation to a transverse one not only serves to create an intact circumferential levator–pharyngeal sphincter for nasopharyngeal closure and speech but also serves to lengthen the soft palate. After nasal closure, the mobilized levator sling is transposed across the cleft. The tension of the muscle repair is based on surgeon experience. A repair that is too tight can lead to a decreased nasopharyngeal aperture and potential postoperative airway compromise. A repair that is too loose will compromise function of the levator sling during speech. We repair the muscle sling with approximately three buried horizontal mattress sutures of 3-0 vicryl.
In the primary Furlow technique, the levator transposition occurs through double-opposing z-plasties of the nasal and oral flaps, with the levator muscle left attached to the posterior-based flap on each surface, and the anterior-based flaps being mucosa and submucosa only (Figure 19.8). The release on each flap extends toward the hamulus, such that when transposed, the posteriorly based muscle–mucosa flaps become transversely oriented, and the long axis of the z-plasty shifts from transverse to sagittal, thereby lengthening the soft palate. An additional benefit of the Furlow technique is felt to be the raising of the nasal lining superiorly during the flap transposition, such that the soft palate is in a more functional position relative to Passavant’s ridge on the pharynx.
The palate is repaired sequentially: the nasal closure from anterior to posterior, followed by the oral closure from posterior to anterior. In a bilateral cleft of the secondary palate, bilateral mucosal flaps are elevated from the caudal edge of the vomer with a midline incision and sutured to the opposing lateral nasal flaps using buried interrupted 5-0 vicryl sutures. The posterior extent of the vomerine flaps is at the posterior nasal spine. At this point, the nasal closure continues with direct approximation of the nasal lining of the soft palate across the cleft back to the uvula. With a unilateral cleft, only one vomerine flap is required. If there is an associated cleft of the primary palate, the nasal lining is repaired as far anteriorly as possible. Ideally, the nasal floor should have been repaired by the earlier cleft lip repair back to the incisive foramen, such that oronasal separation can be completed at the time of the palate repair. This saves the child the inconvenience of an anterior oronasal fistula during the years after the palate repair, and also makes a secondary alveolar bone graft easier and potentially more successful as nasal closure has already been achieved.
FIGURE 19.8. Double-opposing z-plasty closure of a unilateral cleft of the primary and secondary palates. A. Design of the incisions. Dotted black lines mark the oral surface incisions; dotted grey lines mark the nasal surface incisions. The location of the greater palatine pedicle is in the region of the blue circle. Dotted blue lines mark the posterior aspect of the hard palate. The tensor tendon as it crosses over the hamulus to fuse with the levator aponeurosis is marked with an “X.” B. The levator muscle is left attached to the mucosa of the posteriorly based flaps in both the oral and nasal linings and the z-plasties are transposed. C. Transposition of the double z-plasty changes the orientation of the levator muscle from oblique para-sagittal to transverse. This recreation of the levator sling creates a functional soft palate for velopharyngeal competence.
Various techniques have been described for uvuloplasty, including bilateral Y incisions and truncating the tip of the uvula to create a broad raw surface. None are ideal. With wide cleft repairs under increased tension, the uvula tends to widen at the base and decrease in projection. All techniques have in common accurate eversion of the mucosal lining of the uvula and repair of the muscle bundle at the base of the uvula to decrease postoperative widening and prevent fistula formation.
Oral closure is achieved using either 4-0 chromic or 5-0 vicryl vertical mattress sutures. Two 3-0 chromic sutures are used to grasp the underlying nasal lining closure as part of the mattress suture. These close the dead space between the oral and nasal lining. Horizontal mattress sutures of 3-0 chromic are also used to secure the anterior tips of the mucoperiosteal flaps directly to the back of the alveolus. In a bilateral cleft, the flaps are also secured to the posterior aspect of the premaxilla, where a very limited mucoperiosteal dissection is performed after an angled blade has created a transverse cut to create an edge to receive a suture. The original description of the two-flap palatoplasty included a “pushback” to lengthen the palate, which left the anterior hard palate exposed. This pushback technique has been discontinued following evidence of impaired facial growth and the resulting large anterior fistula and is unnecessary for lengthening if a proper levator muscle transposition is performed.
Meticulous hemostasis is essential during the cleft palate repair. If there is any sign of oozing from the flaps or lateral defects, the bleeding is stopped prior to waking the patient. Some surgeons suture absorbable hemostatic material in the lateral defects, but recognize that this does not replace surgical hemostasis. Any blood that has collected in the oropharynx is suctioned. The patient is placed in soft arm restraints, and the endotracheal tube is not removed until spontaneous breathing and purposeful movement is established. We recommend postoperative oxygen saturation monitoring and close observation in the recovery room for 1 to 2 hours prior to discharge to the ward. Intensive care unit (ICU) level care may be indicated in syndromic or other complex patients.
Complications Following Cleft Palate Repair. Compli-cations of cleft palate repair include bleeding, respiratory obstruction, infection, dehiscence, and oronasal fistula formation. Significant postoperative bleeding is rare, but if it occurs, it requires re-intubation and exploration for hemostasis. Respiratory obstruction is also rare in the absence of excessive bleeding, but is life-threatening. The airway is monitored carefully in the recovery room and only after adequate assessment should the baby be transferred to the floor. We recommend oxygen saturation monitors to be employed on the floor or the patient can be monitored in an ICU setting if the airway is tenuous or the patient is syndromic. Monitors alone are not a fail-safe prophylaxis. They are only as good as the response of personnel to the alarm. Pain control should be handled by experienced staff, as overmedication with narcotics can easily lead to respiratory arrest in these patients. Infants with Pierre Robin sequence or other congenital anomalies affecting the airway are at highest risk for airway problems.
Palatal fistulas may present as asymptomatic holes or may cause such symptoms as speech problems, nasal regurgitation of fluids, or difficulty with oral hygiene. The most common locations are at the region of the incisive foramen, the posterior nasal spine, and the uvula. Fistula rate has previously been reported at 10% to 15%, but in experienced hands is now 5% or less. Meticulous surgical technique to create intact, well-perfused flaps that are carefully approximated across the cleft with minimal tension is the best prophylaxis against fistula formation. The use of biomaterials, such as acellular cadaveric human dermis, has been described as a reinforcing layer on top of the nasal closure for wide clefts. With a well-executed technique this should rarely, if ever, be indicated. Symptomatic oronasal fistulas are treated early with local mucosal flaps. Asymptomatic oronasal fistulas may be left unrepaired until the time of another surgical procedure such as alveolar bone grafting.
Operative Treatment of Velopharyngeal Insufficiency
Speech and Language Development. All children born with a cleft palate require examination by a speech pathologist at regular intervals to allow timely intervention if a significant delay develops in receptive or expressive language. The diagnosis and workup of language difficulties require the multidisciplinary involvement of the speech pathologist, audiologist, otolaryngologist, psychologist, and pediatrician, as the delay is not always secondary to mechanical problems of the velopharynx. Other potential contributing factors include hearing difficulties, abnormal speech habits, psychosocial delay, and tongue restriction. VPI is the inability to achieve closure of the velopharyngeal port during sustained speech. The most common cause of VPI is a cleft of the secondary palate; however, other less common causes include submucous cleft palate, neuromuscular abnormalities, adenoidectomy, and congenital VPI of unknown etiology. Once other causes of language delay have been ruled out, a formal VPI workup is performed to diagnose the underlying dynamics of the velopharynx and to recommend appropriate treatment.
Velopharyngeal Insufficiency. Intelligible speech production requires reliable and voluntary function of the velopharyngeal valve that controls communication between the oral and nasal cavities. The valve is closed by contraction of the pharyngeal muscles that advance the lateral and posterior pharyngeal walls, as well as the levator sling that pulls the soft palate (velum) posteriorly. If this palatopharyngeal sling is incompetent, abnormal coupling of the nasal and oral cavities occurs, which results in hypernasality, nasal emission, imprecise consonant production, decreased vocal intensity (loudness), and short phrases. These are the typical signs of VPI, which may be caused by either a structural defect or a physiologic dysfunction.
Tissue deficiency, pharyngomegaly, and neurogenic paresis of the velopharynx can all cause VPI. Not all patients who exhibit glottal stops, pharyngeal fricatives, or nasal emission have, however, VPI. Learned articulatory compensations such as glottal stops and pharyngeal fricatives may be confused with velopharyngeal dysfunction. Phoneme-specific nasal emission is often confused with VPI, even though no resonance disorder exists. Other aspects of phonatory, articulatory, and prosodic breakdowns may be unrelated to the competency of the velopharyngeal valve. If opening of the velopharyngeal valve, instead of closing, is the problem, abnormal uncoupling of the nasal and oral cavities results in hyponasality. This can be found in individuals with hypertrophic adenoid tissue and must be recognized before considering surgical intervention. Nonsurgical treatments of VPI include speech therapy, prosthetic management with speech bulb or palatal lift appliances, and posterior pharyngeal injections or implants. The next section focuses on the surgical treatment of VPI.
Preoperative Velopharyngeal Insufficiency Evaluation. The goal of surgical intervention in patients with VPI is to provide a mechanism for functional speech. The design of the surgical procedure is based on the velopharyngeal anatomy and the function, which is determined by a series of clinical and radiographic tests. Clinical examination includes a formal recording of the child’s speech before, during, and after therapeutic intervention. Typical speech samples include isolated phonemes, words, phrases, and non-nasal reading passages with the nares occluded and unoccluded to detect acoustic differences associated with cul-de-sac resonance. Dynamic study of the pharynx by multiview videofluoroscopy and nasopharyngoscopy is usually indicated. This test provides information regarding the posterior and superior movement of the velum as well as the degree of medial excursion of the lateral pharyngeal walls during speech. In patients who have been referred from another center, intraoral examination and nasopharyngoscopy will determine if an intravelar veloplasty was performed at the time of cleft palate repair and if the levator sling is functioning appropriately. With these tests, the VPI team can determine if the problem is of insufficient length and/or excursion of the velum, sagittal orientation of the levators, or poor excursion of the pharynx, which will determine whether correction requires a secondary palate lengthening procedure such as a Furlow palatoplasty or if pharyngeal surgery is indicated.
After pharyngeal flap surgery, patients are followed closely by both the surgeon and the speech pathologist. Clinical evaluations and tape recordings are obtained at least every 3 months for the first year and then annually for 3 to 5 years. Periodic acoustical analyses with the sound spectrograph are used to monitor speech characteristics postoperatively and should validate more subjective, perceptual ratings in judging the success of surgery.
Pharyngeal Surgery for Velopharyngeal Insufficiency
The nonvelar surgical management of VPI usually consists of pharyngeal flap or sphincter pharyngoplasty. Both have been shown to be efficacious.
Pharyngeal Flaps. Pharyngeal flaps may be superiorly or inferiorly based. Most studies in the literature have not found a difference on speech outcome between the two dissections. The mucosal flaps are raised from the posterior pharyngeal wall and attached to the soft palate so as to create a midline obstruction of the oral and nasal cavities between two lateral openings (ports). The amount of lateral pharyngeal wall motion will determine how wide the flap needs to be to achieve velopharyngeal competence. If the flap is too narrow, hypernasality will persist from the inability of the lateral pharyngeal walls to close the ports on either side of the flap. If the flap is too wide, passive occlusion of the lateral port can occur, and the patient will develop mouth breathing, hyponasality, and possibly obstructive sleep apnea. Hogan18 popularized the concept of lateral port control based on his appreciation of the previous work by Warren et al. in the 1960s. These pressure-flow studies demonstrated that oropharyngeal air pressure decreases markedly when the port cross section exceeds 10 mm2, whereas nasal escape of air is audible above 20 mm2. Sprintzen et al. introduced the concept of “tailor-made” flaps based on preoperative evaluation of lateral pharyngeal excursion.19
The technique of pharyngeal flap surgery involves longitudinal incisions through the mucosa and muscle down to the fascia on each side of the posterior pharyngeal wall. Dissection is continued along the fascia. A superiorly based flap is transversely incised inferiorly and raised to a level above the palatal plane, which usually corresponds to 1 to 2 cm above the tubercle of the atlas. An inferiorly based flap is incised just below the adenoid pad. The flap is usually inset with turn-back flaps on the nasal side of the uvula, with or without opening the midline palate repair. The turn-back flaps from the nasal mucosa are used to line the raw surface of the pharyngeal flap to minimize postoperative contraction. The pharyngeal donor defect of the flap is closed primarily. In patients with velocardiofacial syndrome, the internal carotid arteries can have an anomalous course that approaches the midline. The pharynx is observed and palpated carefully for any abnormal pulsations in the region of the proposed flap. Some authors have advocated preoperative computerized tomography or magnetic resonance imaging angiograms in these select patients.
Complications following pharyngeal flap surgery are considerable compared with those of primary cleft lip and palate repairs. The Hospital for Sick Children in Toronto published retrospective data from a 7-year period in 1992 and reported an 8.2% risk of bleeding, a 9.1% risk of airway obstruction, and a 4.1% risk of sleep apnea. Five percent of their cohort required eventual surgical revision of the flap. With changes instituted by this group based on their review, including closer observation and monitoring, increased education, and decreased number of surgeons performing the procedure, the total rate of complications decreased from 11% to 3.2%. Bleeding decreased to 1.4%, airway obstruction to 3.2%, and hospital stay decreased from 5.8 to 3.8 days. These two valuable reports emphasize the potential complications associated with pharyngeal surgery, and the benefit of constant vigilance and quality improvement at all centers.
As expected, sleep apnea or upper airway obstruction is a potential complication of an operation whose purpose is to decrease the velopharyngeal airway. Although studies report up to a 35% incidence of abnormal polysomnograms following pharyngeal flap surgery, the vast majority of these patients resolve within 5 months. Lesavoy20 concluded that “the surgeon may sometimes need to accept some transient upper airway obstruction to achieve correction of velopharyngeal insufficiency.”
Sphincter Pharyngoplasty. The sphincter pharyngoplasties performed today are modifications of either the Hynes or the Orticochea techniques. In both techniques, the sphincter is constructed from bilateral superiorly based flaps raised from the posterior tonsillar pillars, including mucosa and the palatopharyngeus muscle. In the Hynes pharyngoplasty, the flaps are transposed to the midline and inset into a defect created by a transverse incision at the level of the flap base. In the Jackson modification of the Orticochea technique, the flaps are sutured together with a small, superiorly based, posterior pharyngeal flap.21 Subsequent authors and studies have emphasized that the level of the sphincter is the most important predictor of success in both of these techniques. The pharyngeal constriction must be high, at the level of palatopharyngeal closure. The tightness of the pharyngoplasty can be controlled by the degree of overlap of the tonsillar flaps.
The procedure achieves both static and dynamic reduction in the velopharyngeal port with no disruption of the velum. It is ideal when there is poor medial excursion of the lateral pharyngeal walls and a short anteroposterior component of velar competency. It has the advantage of allowing revision if necessary by re-elevating the flaps and adjusting the tightness of the sphincter.
In both sphincteroplasties and pharyngeal flaps, the adenoid pad can limit the superior dissection and placement of the obstruction. For these patients, the otolaryngologist on the cleft team is consulted to determine if an adenoidectomy should be performed first in order to optimize the subsequent sphincter pharyngoplasty or pharyngeal flap placement.
Pharyngeal Flap Compared with Sphincter Pharyngo- plasty. Studies comparing the two pharyngeal surgeries have not documented a significant difference in speech outcome. Both techniques have advantages and disadvantages and potential complications and require an experienced surgeon for success. The results of a prospective international multicenter randomized trial of 97 patients treated with either pharyngeal flap or pharyngoplasty were reported in 2005.22 Although early (3 months) elimination of hypernasality was achieved in twice as many patients treated with a flap, there was no significant difference between the two techniques after 1 year in terms of resonance, nasalance, endoscopic outcomes, and surgical complications. Sleep apnea rarely resulted from either procedure.
Treatment of the Alveolar Cleft
The preconference symposium of the 2004 ACPA annual meeting focused on treatment of the alveolar cleft. Three approaches were presented and debated: (a) early alveolar bone grafting in the first year of life with autogenous rib cortical graft as a separate operation; (b) presurgical NAM with primary GPP at the time of primary lip repair; and (c) secondary alveolar bone grafting as a separate operation during mixed dentition with autogenous iliac crest cancellous graft. No conclusions regarding the superiority of one technique over another could be drawn at the end of the symposium. Each approach has been studied by its proponents to provide data justifying its use. Secondary bone grafting at the time of mixed dentition remains the traditional and the most common technique for treatment of the alveolar cleft, and as such remains the standard by which other techniques are compared (Figure 19.9).23 The ideal treatment for the alveolar cleft would be a minimal surgical intervention performed without an additional anesthetic, with no donor-site morbidity, and no detrimental effect on facial growth or dental eruption. To date, NAM with primary GPP comes closest to this goal. In this technique, after NAM treatment has decreased the alveolar cleft size to less than 2 mm and the edges are parallel, a very limited subperiosteal dissection is performed inside the alveolar cleft as described by Millard. Small flaps are then raised to create a closed tunnel of periosteum joining the exposed facing bone edges of the alveolar cleft. Bone forms in this tunnel to close the gap without the need of grafting. GPP requires presurgical orthopedics by a trained team and has been reported to have a 60% chance of avoiding secondary bone grafting. However, GPP requires further evaluation to confirm that it has no detrimental effect on maxillary growth or on the developing tooth buds.
Recombinant human bone morphogenic protein-2 (rhBMP2) is a mitogen that has been demonstrated to stimulate osteoblastic activity and induce bone nodule formation in animals. It has been approved by the U.S. Food and Drug Administration for clinical use in human spine fusion procedures and has been shown to decrease non-union, donor-site morbidity, and operating time over autogenous grafting in this population. More recent clinical applications have been on patients undergoing alveolar augmentation and implant placement, and early trials are now underway at individual centers for the treatment of alveolar clefts. The risk–benefit profile of rhBMP2 in these patients will remain unknown for the next decade. Concerns include an inflammatory response, heterotopic ossification, potential long-term carcinogenesis, and a detrimental effect on normal dental ligament development. Patient selection should therefore be based on enrollment in an institutional review board approved trial with appropriate consent and evaluation, including oversight by an independent data safety monitoring board.
FIGURE 19.9. Unilateral alveolar bone graft. A. Markings for the superiorly based mucoperiosteal flap. The inferior tip of the flap consists of attached gingival to resurface the oral lining of the cleft. Stenson’s duct is marked with a blue dot. The dotted line marks a previous scar from the primary lip surgery. B. The anterior flap has been elevated and the intracleft flaps have been used to close the oral lining as well as the nasal lining. C. A bone tamp is used to gently pack autogenous cancellous bone chips from the iliac crest from the incisive foramen to the labial surface of the cleft. D. Advancement and closure of the flap over the bone graft. The advancement is facilitated by a deep periosteal release.
Orthodontic and Orthognathic Treatment Following Cleft Palate Repair
Studies on unrepaired cleft palates in developing countries suggest that the surgical intervention of cleft palate repair impairs future maxillary growth. Some individuals with cleft palate may also have an intrinsic limited growth potential. Decreased maxillary width and the resulting lingual crossbite are common and are managed by orthodontic maxillary expansion with a fixed appliance. Once expansion is completed, the optimum time for bone grafting is chosen according to the stage of canine development. If the graft is performed too early, it can result in bone resorption as a consequence of a lack of mechanical stimulation from a tooth. If the graft is performed too late and the erupting canine root does not have sufficient bone support, the tooth may be lost.
Maxillary retrusion or midface hypoplasia resulting in an Angle class III occlusal relationship with anterior crossbite can be managed in childhood with a distraction device such as a Delaire mask to aid horizontal growth, but eventually requires orthognathic advancement. A plan of treatment is formulated on the basis of clinical examination, photographs, cephalometric studies, and dental models. If surgery is indicated, presurgical orthodontics is required to align dental arches and to eliminate crowding and dental compensations. Le Fort I maxillary advancement is performed at the time of epiphyseal closure at skeletal maturity, approximately age 16 years for a girl and 18 years for a boy. Large advances greater than 1 cm can be difficult as a result of restriction from palatal scarring related to palate repair or previous pharyngeal flaps and are prone to relapse. Adding distraction osteogenesis to the Le Fort I osteotomy allows larger advancements and, in general, prevents relapse.
Polley and Figueroa have studied and popularized Le Fort I maxillary distraction osteogenesis using an external cranial halo-based device. Intraoral devices are now also available that are attached across the osteotomy and are activated through a trans-mucosal arm. Once the permanent teeth have descended below the osteotomy site, the traditional Le Fort I osteotomy is performed and the distraction device is applied. The segment can then be advanced at a rate of 1 mm a day to the desired position of overcorrection. This is followed by a period of wearing the device without advancement during which ossification occurs within the osteotomy and consolidates the new maxillary position. The original consolidation period of 8 weeks for the external device has been decreased to 2 to 3 weeks by the use of a removable, elastic-traction, traditional, orthodontic face mask that is attached to the oral splint after removal of the halo. Polley and Figueroa’s studies demonstrated stability of large maxillary advancements with distraction osteogenesis. In addition there was minimal detrimental effect on speech.
Secondary Cleft Lip and Nose Surgery
Increased understanding of the primary cleft anatomy and an improvement in the technique of primary repair have reduced the severity of residual deformities and the need for secondary corrections. Perfection in a single surgery, however, remains elusive. The goals of early cleft lip and nose reconstruction are that the cleft be undetectable by peers at conversational distance by school age to minimize psychosocial stigmata and that an optimal final surgical result using up-to-date techniques be complete by skeletal maturity. This is rarely achieved by one surgery in wide, complete clefts, resulting in the need for secondary cleft lip and nose procedures.
Each secondary surgery is approached with the following guidelines in mind:
• Identify the primary repair that has been performed to appreciate how it will affect the planned revisions.
• Recognize the optimum age to achieve the surgical goals. If the child is too young, small, temporizing procedures are performed to minimize the deformity and scarring until the definitive procedure can be performed.
• Find the normal landmarks and return them to their normal positions.
• Do not remove any tissue until certain that it will not be useful.
• Treat each case individually—there is no routine secondary procedure.
• Use the basic plastic surgery principle of transferring tissue from areas of excess to areas in need.
• Replace the lost tissue with similar tissue when prior surgery, growth, or the lack of growth is responsible for the deficiency.
Indications for Surgery. The indication for a secondary surgical procedure is a correctable deformity given the age of the patient, which if not repaired, will remain or will result in psychosocial or functional problems. The surgeon must recognize that there are four perspectives of anatomic abnormalities to be considered: those of the surgeon, those of the patient, those of the parent, and those of peers or other members of society. Which perspective is predominant affects both the indication for surgery and the chance of a successful outcome.
When addressing a patient with a secondary deformity, it is first necessary to recognize the cause of the deformity. Steffensen24 outlined reasonable requirements for lip repair: (a) accurate skin, muscle, and mucous membrane union; (b) proper rotation of the deflected medial and lateral orbicularis oris muscle into a horizontal position; (c) a symmetric nostril floor and nostril tip; (d) an even vermilion border with reproduction of the Cupid’s bow; (e) slight eversion or pouting of the central upper lip; and (f) a minimal scar.
Timing of Secondary Repair. As mentioned above, the goals of secondary repair of cleft lip and nasal deformities are that the cleft be undetectable by a peer at conversational distance by school age and that an optimal final surgical result is complete by skeletal maturity. To achieve this, we perform presurgical NAM in early infancy, followed by a primary lip repair with repositioning of the nasal cartilages when the patient is approximately 3 months old and defer any revisions until just prior to school age. At that time, any indicated lip revisions are completed to facilitate the child’s interaction with peers in a school environment. In the case of an obvious residual nasal deformity, such as that following repair of an unmolded wide bilateral cleft, a minor nasal tip rhinoplasty through limited intranasal incisions can be offered. The optimal time to complete the nasal reconstruction, however, is in adolescence, when a formal open rhinoplasty with cartilage grafting, septoplasty, and/or osteotomies can be done. If orthognathic surgery is anticipated, the final rhinoplasty is best deferred until after this is complete, as the appearance of the nose will change following repositioning of the bone that supports the nasal base.
Muscle Secondary Deformities. As described earlier in the chapter, the key to a successful cleft lip repair requires full release of the abnormal attachments of the orbicularis muscle and repositioning of the marginal, oral, and nasal components to create oral continuity and competence. When discontinuity is present in a unilateral cleft repair, a subcutaneous groove or trough appears and the scar contracture, which is normally seen only in the first few months after a repair, persists. The groove is more readily apparent on lip animation, with bulging of the lateral muscle segments caused by unbalanced contraction. In a bilateral repair, lack of muscle continuity will result in a grossly widened prolabium, unrestricted premaxillary growth, lack of upper lip animation, and widened interalar distance (Figure 19.10). Secondary deformities of the muscle require recreation of the cleft defect, and appropriate repositioning of the different functional layers.
Vermilion Deficiency and Irregularities. The most common irregularity is a “whistle notch” deformity. Notching is usually caused by inadequate approximation of the marginal component of the orbicularis oris muscle within the red lip. Deficiency of the free edge of the lip can often be treated by reopening of the inferior incision, symmetric eversion of the medial and lateral lip elements, and accurate layered approximation of the muscle. A z-plasty in the wet vermilion can also minimize recurrence of the deformity. The width of the dry vermilion should also be measured. If there is deficiency of the lip above the wet–dry junction, then a combination of triangular inset flaps or z-plasty may be required to achieve and even thickness. If wet mucosa is left above the wet–dry junction, scabbing will result. The labial frenum should always be examined if there is a red lip contracture to ensure that it is not contributing to the problem. If the lip is excessively thick on the cleft side, a transverse ellipse can be excised at the wet–dry junction.
Short Upper Lip. Deformities of the unilateral lip repair are mainly asymmetries and disproportions. One of the most readily visible deformities is an asymmetry between the vertical heights of the peaks of the Cupid’s bow. If the Cupid’s bow is not level, the cause should be identified and a surgical solution created. Vertical shortening of the cleft lip scar is not uncommon in the first few months following surgery, but should settle within a year postoperatively.
A short lip following unilateral repair refers to a diminished vertical distance from the Cupid’s bow white roll to the base of the columella, the alar base, or both. The most common cause of the short lip is inadequate lengthening at the primary repair. Careful evaluation is required to determine if the vertical deficiency is primarily cutaneous, muscular, mucosal, or all of them. The deficiency typically involves the medial lip element, but in some secondary cases the lateral lip is also short. If the alar base had not been adequately released and repositioned at the primary surgery, the nasal deformity can also mask some of the vertical deficiency of the upper lip. The distance from the alar bases to the Cupid bow white roll should be compared with each other and then with the distance from the midpoint of the columella base. If the cleft-side alar base is still displaced inferiorly, and the upper lip is also short, then a full revision is required with recreation of the defect, release and repositioning of the alar bases and muscle, and lengthening of the lip either through a re-rotation of the medial lip and advancement of the lateral lip similar to the primary rotation repair, or with a combination of triangular inset flaps from lateral to medial. If the alar bases are symmetric, however, and the deficiency is limited to the lip alone, the surgeon needs to determine if the oral component of the orbicularis is appropriate. If not, the lip must again be opened and reconstructed. If the muscle sphincter is correct, then a limited skin repair can be performed.
If a straight-line repair was performed primarily, it will not interfere with a subsequent rotation-advancement revision, which will advance the alar base medially and lengthen the columella on the cleft side. The ideal indication for rotation-advancement following a straight-line repair includes the following: (a) the philtral scar on the cleft side is short; (b) the Cupid’s bow is pulled up toward the nostril; (c) the nostril floor is wide; and (d) the ala is displaced laterally and downward.
A short upper lip following a Millard-type rotation repair usually requires revision with recreation of the defect and repeat rotation-advancement. Simple re-rotation and advancement of skin only, without complete takedown of the muscular repair, should be reserved for minimal deficiencies. Additional lengthening may be obtained by adding a z-plasty or triangular inset flap placed close to the sill of the nostril or just above the white roll, so that it is not readily apparent.
For symmetrically short upper lips following a bilateral repair, again the relative contribution of the muscle, skin, and mucosa must be determined. Lengthening of the skin of the central upper lip typically involves advancing the lateral lips toward the midline. The most frequent secondary deformity of the bilateral cleft lip is paucity of the central lip. The thin central vermilion (whistle deformity) is more commonly seen after a Manchester-type repair, where the central lip has been corrected with abnormal prolabial mucosa that is deficient in bulk and often dry or flaking. The single-stage Millard-type bilateral repair, in which the red lip component of the lateral segments creates the central vermilion, usually leads to better symmetry and a fuller vermilion tubercle. If a whistle deformity is present following a Manchester-type repair, the best treatment is often to convert the repair to a Millard-type pattern. Bilateral Burrow triangle excisions are made above the white roll of the lateral lip; the width of the prolabium is decreased; and vermilion, marginal muscle, and white roll from the lateral lip elements are brought under the prolabial skin (Figure 19.10).
Long Upper Lip. The long lip is more commonly found in bilateral than unilateral clefts. It is usually secondary to a failure to resuspend the nasal muscle sling and oral sphincter up to the anterior nasal spine at the time of the primary repair. This results in the transverse oral muscle descending below the protruding premaxilla, similar to a low belt line over a protuberant abdomen. This not only causes a long-lip deformity but also flattens the columella–labial angle and removes the molding force of the muscle below the premaxilla such that it remains prominent. To correct this, the entire lip needs to be opened and proper primary lip repair performed.
In other cases of long upper lip in both unilateral and bilateral cleft lips, the problem is not an increase of vertical height, but rather a prolapse of the intraoral vestibule. If the vestibule sulcus had not been suspended in an anatomic position at the time of the primary repair, then the wet mucosa falls down, covering the maxillary teeth. This is often accentuated when the patient smiles and the mucosa is squeezed against the labial surface of the teeth. Patients referred with this secondary deformity are often misdiagnosed with redundant mucosa and undergo transverse resection. If this is performed, the patient will end up with an obliteration of the anterior sulcus and the lip tethered to the attached gingival of the premaxilla. To distinguish a prolapsed sulcus from redundant mucosa, a cotton tip applicator is placed behind the lip and pushed up to simulate the appropriate sulcus height. If this corrects the deformity, then resuspension and recreation of the sulcus is required and not mucosal resection.
In rare cases when there is isolated vertical excess of upper lip skin following unilateral or bilateral repair, a transverse resection of skin under the nose can be performed followed by resuspension of the oral muscle into an elevated position.
Tight Upper Lip. A tight upper lip that cannot be corrected sufficiently with local flaps requires a donation of tissue from the lower lip via an Abbe flap. This is uncommon in unilateral repairs, but can be required following a bilateral repair that had complications. The Abbe flap improves the balance between the upper and lower lips by bringing comparatively excessive tissue from the pouting lower lip to the tight upper lip that is deficient of tissue. The scar on the upper lip can be excised or alternatively it can be used to lengthen the columella, creating a central defect in the upper lip. A full-thickness flap is designed centrally on the lower lip to reconstruct the aesthetic subunit of the upper lip philtrum. The donor defect on the lower lip should not violate the mental crease. The flap is rotated on a mucosal bridge containing an intact labial artery and vein that are found at the level of the vermilion border on the lingual (inner) side of the lip. The pedicle is divided after 10 to 14 days, and the flap is inset. The white roll of the flap segment must line up perfectly with that of the lateral lip elements. Up to one-third of the lower lip can be harvested while still achieving primary closure of the donor defect. If the muscle sphincter of the upper lip is in continuity, the Abbe flap can be designed as a skin/mucosal flap to wrap around the native orbicularis muscle.
FIGURE 19.10. Secondary bilateral cleft lip repair with functional muscle sphincter reconstruction. A. At the time of the primary repair, a functional muscle reconstruction had not been achieved resulting in widened prolabium, scars, and interalar distance. Oral competence and animation was also limited. B. Surgical markings for reduction of the prolabial width. The white roll underneath the new prolabium will come from the lateral lip elements. C. Dissection and release of the three components of the orbicularis oris muscle. The upper forceps is grasping the nasal component and the lower forceps is grasping the oral component. The marginal component remains attached to the red lip mucosa. D. Surgical result in the same patient 5 years later. With a functional muscle repair, the prolabial width is maintained and the scars are favorable.
Premaxillary Setback. The complete bilateral deformity is characterized by protrusion of the premaxilla and collapse of the lateral alveolar segments. Following repair of the orbicularis oris at the time of the primary repair, the segments are typically naturally molded by the muscle tension. In rare cases, persistent premaxillary protrusion may occur. With the help of the team orthodontist, the decision is made whether a premaxillary setback is required as an orthognathic procedure. Premaxillary setback should only be performed by an experienced surgeon, as the vascular supply of the premaxilla is precarious and loss of the entire premaxilla and central teeth can occur. In some cases, the lip repair has formed a constricting band superior to the premaxilla forcing the premaxilla inferiorly. Not only does the premaxilla continue to project, but its severe inferior malposition may result in the incisor teeth biting into the lower gingivobuccal sulcus. In this circumstance, resection of a short section of vomer stem with repositioning of the premaxilla, mucosal repair, and alveolar bone grafting may be required. Premaxillary setback and repositioning should only be performed with the guidance of an orthodontist to plan for future dental rehabilitation and facial contour aesthetics.
Secondary Cleft Lip Nasal Repair
If the alar base position, the nasal component of the orbicularis, and the nasal floor have been appropriately corrected and repaired at the time of the primary surgery, the majority of the most challenging secondary deformities seen in adolescence can be avoided. After an inadequate primary repair, the next most common cause of severe nasal deformities is multiple, repeated open nasal procedures throughout childhood, leading to heavy scarring, poor vascularity, and decreasing returns with each operation. The over-operated nose can be a devastating deformity for a teenage cleft patient, with few options available to restore nasal form and function.
Any nasal surgery prior to adolescence should be limited to repositioning of the muscle and cartilage such that natural nasal growth will ameliorate the majority of the deformity. Resection of cartilage and cartilage grafting should be avoided in the growing nose, except in cases of severe iatrogenic secondary deformities.
The literature is replete with numerous approaches to secondary repair of the cleft lip nasal deformity. Many older techniques are still useful in certain circumstances, but should be used within the current paradigm of a systematic anatomic evaluation of the deformity followed by an equally systematic treatment plan. Just as techniques first used in the treatment of cleft patients formed the basis of the aesthetic rhinoplasty, many of the techniques that have recently evolved in aesthetic plastic surgery have been adopted by cleft surgeons. Each component of the deformity must be addressed in an orderly manner: skeletal base, nasal dorsal bone and cartilage, nasal tip cartilage, and, finally, the skin envelope.25
Skeletal Base. Like all facial structures, the nose is supported by the underlying skeleton. The cleft deformity is not restricted to skin and cartilage. In the unilateral deformity, the piriform rim under the ipsilateral alar base is deficient of bone and is retrusive. During the primary cleft lip surgery, the abnormal attachment of the nasal accessory cartilages to the piriform rim is released in order for the alar base to be moved anterior, medial, and superior into the desired position. Because of the lack of skeletal support, the alar base on the cleft side can, in some cases, become retropositioned with growth, even following an appropriate primary correction. If the patient is undergoing secondary alveolar bone grafting at the time of mixed dentition, this is the best time to augment the deficient piriform rim with autogenous cancellous onlay bone graft. The bone graft will elevate and support the alar base to achieve symmetry and provide a stable base for the remainder of the nasal reconstruction in the teenage years.
In the bilateral cleft deformity, the anterior nasal spine is absent, and the footplates of the lower lateral cartilages rest on the muscle repair over the premaxilla. Prior to the definitive secondary rhinoplasty, the position of the premaxilla must be assessed. If the patient has not yet undergone orthodontic treatment, the premaxilla can be retrusive or protrusive. Both deformities will affect the appearance of the nose and should be corrected before a rhinoplasty is undertaken. In the unfortunate event that the premaxilla is absent, either because of inappropriate resection or iatrogenic loss, prosthetic replacement is needed to provide a base support for the nose and lip.
A number of cleft patients will require orthognathic surgery following orthodontics because of midface retrusion. Ideally, the definitive rhinoplasty should be delayed until after the maxillary advancement has been completed. The Le Fort segment contains the anterior nasal spine, which will affect the columella–labial angle and nasal tip projection.
Nasal Dorsal Bone and Cartilage. The unilateral cleft lip nasal deformity often includes a deviated bony and cartilaginous nasal septum with or without deviation of the nasal bones. If the nasal bony pyramid is symmetric, it can be mobilized as a “monobloc” and centralized. If the pyramid is asymmetric, independent movements of the nasal bones will be required. We use a 3-mm osteotome percutaneously to control the nasal osteotomies.
The deviated nasal septum can be treated with a septoplasty, using sutures and scoring to straighten the nasal passage, or, alternatively, with a submucosal resection if cartilage graft is required for the nasal tip, leaving a 1-cm dorsal and ventral L strut for support. In both cases, the base of the septum is mobilized and centralized using a permanent suture through the periosteum of the nasal spine. If the septal cartilage is too weak to support the new position, onlay strut grafts are used to reinforce the nasal tip projection.
As with any rhinoplasty, the preoperative evaluation includes an intranasal examination. In the cleft deformity, the ULC is inferiorly displaced. This can clearly be seen on intranasal examination of an infant undergoing primary repair. An associated collapse of the internal nasal valve between the septum and ULC with nasal obstruction on inspiration can be treated with spreader grafts using either a closed or open technique. This can also increase the width of the middle third of the nose to improve symmetry in unilateral cleft deformities. The spreader graft can also be extended to create increased support for the lower lateral cartilages of the nasal tip.
In severe bilateral cleft deformities that have undergone numerous previous procedures, a cantilevered rib graft may be required. This is often indicated in patients with saddle nose deformities from over-resection of the cartilaginous septum and an associated flattened nasal pyramid.
Nasal Tip Cartilages. The medial and lateral crura of the alar cartilages in the secondary deformity are often displaced posteriorly on the cleft side, with the lateral crus displaced laterally. This causes collapse of the nasal tripod, alar rim hooding, and lateralization of the genu of the nasal dome. Older techniques transposed subsections of the displaced cartilages and are rarely indicated. The current consensus is to reposition the entire lower lateral cartilage structure using an open tip rhinoplasty. Unlike a non-cleft rhinoplasty, simple repositioning with nasal tip sutures is typically insufficient to correct the cleft deformity. After the native cartilage framework is reconstructed, autogenous cartilage grafting is required to strengthen the new position and to augment the nasal tip projection. Graft augmentation is rarely indicated before adolescence.
In the unilateral deformity, a “springboard” non-anatomic alar batten graft is often required to maintain alar rim curvature. The graft is harvested from the septum and is anchored in a subcutaneous pocket at the alar base. The graft is then bent under mild tension over the lower lateral cartilage and secured to the nasal dome. The lateral crus is then secured to the undersurface of the graft. The graft is non-anatomic and should follow the alar rim from base to top, instead of heading posteriorly in the lateral nostril. The springboard effect of the graft will create and maintain the desired alar rim curvature when the skin is re-draped.
In the bilateral cleft lip nasal deformity, the displaced lower lateral cartilages are addressed in a fashion similar to that described above. Compared with the unilateral deformity, however, the nasal tip projection and support is more deficient. Columella cartilage strut grafts or septal extension grafts as described by Byrd26 are required to provide support to the nasal tip construct. After medialization of the genu of the lower lateral cartilages to the midline to create a nasal dome, multiple onlay tip grafts are usually required to achieve the desired shape and projection.
FIGURE 19.11. Early open nasal tip rhinoplasty for bilateral cleft lip deformity. A. At age 4, the patient’s nasal growth was following a pattern of progressive relative columella shortening, increased nasal tip width, and decreased alar height. There was palpable divergence of the nasal domes. Without early treatment, this deformity would pose a significant surgical challenge at the time of the definitive teen age rhinoplasty. B. Open nasal dissection was performed limited to the lower lateral cartilages, leaving the intradomal fat attached to the skin envelope. The interdomal fat was not removed. C. Using 5-0 PDS interdomal sutures (Ethicon, Somerville, NJ), the genu of the lower lateral cartilages we approximated and the skin redraped. D. Five years later, the subsequent nasal growth and lengthened the columella and maintained alar height. The adaptation of the skin envelope will be favorable for the definitive rhinoplasty at maturity.
Skin Envelope. A principal argument for an aggressive primary rhinoplasty in infancy is the frustration associated with attempts to correct the deformed, deficient, and scarred skin envelope of a secondary or tertiary rhinoplasty. All cleft surgeons have experienced the satisfaction of constructing a formidable cartilage framework, only to see it compromised under compression when the skin is re-draped. The delicate anatomy of the natural soft triangle and nasal dome cannot be created with current secondary techniques, but should remain our goal.
In teenage bilateral cleft patients if nasal growth has taken place without the genu of the lower lateral cartilages approximated, the columella skin will be either short or almost absent, and the nasal tip will be wide and flat. Re-draping this deformed skin envelope over a cartilage reconstruction to create a natural columella is difficult. In order to avoid this added challenge, performing a limited tip rhinoplasty just prior to the nasal growth spurt can close the interdomal space such that the subsequent growth lengthens the columella skin and minimizes the cutaneous deformity faced in the definitive rhinoplasty in adolescence or thereafter. This surgery can be performed at age 4 to 5 but is limited to patients with palpable divergence of the nasal domes (Figure 19.11).
In the unilateral deformity, the deformed skin envelope often overhangs the nostril apex. The Tajima “inverted U” nostril apex incision can help to address this problem. The skin flap left attached to the inferior edge of the lower lateral cartilage turns over to form the inner lining of a constructed soft triangle. A similar turnover flap approach can be used secondarily if the underlying cartilage is already in the correct position.
In the bilateral cleft lip nasal deformity, the skin envelope is deficient vertically, from the nasal tip to the base of the columella. When closing the open rhinoplasty, the relatively lax lateral tip skin is advanced toward the nasal tip when the rim incisions are closed, in order to create sufficient skin for the nasal tip and columella closure. In severe deformities, however, the skin envelope is too tight to drape over the cartilage construct with tension-free closure at the columella incision. Techniques that borrow skin from the upper lip, such as a V-Y advance, result in scarring at the lip–columella junction but may be required. Techniques that borrow from the horizontal laxity of the nasal tip skin, such as the McComb and Brauer alar lift incisions, result in additional scars on the nasal tip. Both approaches therefore have limitations and no ideal alternative currently exists.
Nostril Stenosis. Nostril stenosis, or a “micronostril deformity,” can be one of the most difficult late complications associated with cleft lip repair. It is considerably easier to narrow a nostril than to enlarge it. In general, any circumferential nasal lining incision is associated with a high incidence of nostril stenosis. Intranasal z-plasties or composite grafts can be used if there is a localized constriction. If the patient has a micronostril with constricted nasal floor and a medial displacement of the alar base, a small inferiorly based nasolabial flap can be used to correct both deformities. Long-term postoperative use of nasal stents is required to minimize the chance of recurrence, but, unfortunately, is limited by patient compliance. An active nostril expander using a small jackscrew has been described by the group in Miami. In a compliant patient, this may be the best option available.
Many plastic surgeons were drawn to their surgical specialty after seeing a cleft lip repair. Cleft care stands out as a rare opportunity to have a huge impact on an infant’s future psychosocial well-being and to follow these children over the formative years of their lives. Once in practice, the cleft surgeon is reminded of the success, as well as of the failure, of his/her primary operations for years to come. Modern cleft surgical techniques, preoperative orthodontics, and specialized multidisciplinary team care enable us to achieve more consistent favorable primary surgical results. Repair of secondary deformities, especially those involving the nose, remains a challenge and is still best treated by preventative surgery at the time of the primary repair. Recent “inductive” techniques, such as NAM and distraction osteogenesis, have improved care over the past decade, and as comparable advances in plastic surgery occur in the future, a child born with a cleft can look forward to fewer operations with better aesthetic and functional results.
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