As a member of the cleft team, the plastic surgeon helps to: identify any need for surgical repair of cleft lip, nose and palate defects; and works with the other members of the team to coordinate procedures, depending on the child’s maxillofacial- dental development, as well as speech and hearing development.
The plastic surgeon is often one of the first members of the team to meet with new parents after the birth of a child with a cleft, or even earlier as diagnosis of clefts by prenatal ultrasound becomes more common. The surgeon can provide support and information about what to expect in the coming months and years, giving an overview of surgical repairs that may be necessary as the child grows.
When a child has both cleft lip and palate, the first procedure is usually the lip repair, not only for obvious aesthetic reasons, but also for functional reasons. Restoring normal upper lip anatomy assists the feeding mechanism and molds the premaxilla, guiding it into a more normal anatomical position for later palate repair.
Before describing some of the characteristics of a cleft lip and its repair, it helps to be familiar with some of the common landmarks and anatomy of a non-cleft or normal lip. Cupid’s bow is central to the upper lip, with its peaks delineating the philtrum between the philtral columns. (Figure 1)
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Figure 1. Normal lip anatomy. A) Central vermillion tubercle. B) Philtral column. C) Nasal coumnella |
The demarcation between mucosa and skin of the lip is called the vermilion border. The mucosa or vermilion of the lip is further divided into dry and wet sections. The protuberant vermilion in the midline is referred to as the tubercle. The two nostrils (nares) are separated by the columnella externally and the septum internally. Below the surface, the orbicularis oris muscle encircles the oral aperture, creating a sphincter. The fibers decussate in the midline creating the philtrum Figure 2A).
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Figure 2. Lip musculature. A) Normal musculature with fiber decussation to form the philtral columns. B) Cleft musculature with abnormal muscle insertions into the base of the nose |
In the cleft lip, the orbicularis muscle inserts into the nasal alar base (Figure 2B). Other deformities in the cleft include a small, misshapen, and misplaced alar cartilage, deviated and shortened columnella, septal deviation, and distortion of the nasal tip and nares. The septal deviation often results in airway obstruction. With the interruption of the orbicularis’ progress towards its counterpart, the premaxilla is often displaced by unopposed muscular forces on the non-cleft side. The continuity of the vermilion border is obviously disrupted, with loss of Cupid’s peak and philtral column on the cleft side. Both the skin and vermilion of the lateral cleft lip appear shorter than the non-cleft side. In the complete bilateral cleft lip, the often protuberant prolabium (soft tissue) and premaxillary segment (bone) has no orbicularis muscle component. The nasal tip is broad and short with wide, flattened nostrils. The skin and vermilion of the prolabial segment are short and wide and there is no philtrum or philtral columns.
While cleft lips have been repaired as early as the first few weeks of life, traditional teaching has proposed the Rule of 10s waiting to repair the cleft lip until the child has reached the age of 10 weeks, a weight of 10 pounds, and a hemoglobin of 10. Although exceptions to the rule are many, it helps minimize the risk of general anesthesia, maximize the child’s nutritional status and healing capacity, and facilitates the technical aspects of surgical repair based on the child’s size. Recent advances in the technique of presurgical nasal alveolar molding greatly facilitate anatomic restoration, but delay initial surgery until 4-6 weeks of age.
The basic goal of cleft lip, alveolus, and palate repair, whether for the unilateral or bilateral anomaly, is to restore normal anatomy. The objectives of surgical repair of the cleft lip are two-fold: restore aesthetic ‘normal’ landmarks and reconstruct a functional orbicularis oris. There are many variations in the technique of cleft lip repair. Two of the most common approaches are the rotation-advancement and the triangular flap repair.
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Figure 3. The rotation-advancement (Millard) Lip Repair
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The rotation-advancement repair, (fig. 3) pioneered by D. Ralph Millar, creates two opposing and interdigitating flaps. The medial side of the cleft lip is rotated downward from the columella in order to lower the ’peak’ level to that of the normal side, and the lateral lip is advanced toward the midline into the defect at the base of the columella. A major advantage of the rotation-advancement repair is the placement of scars along the natural anatomic features of the philtral columns and nasal sill.
The triangular flap repair, (fig 4) developed by Tennison and others, uses a triangular flap from the lateral lip, inserted into a notch in the medial side of the cleft, just above the vermilion border, crossing the philtral column as it meets Cupid’s peak. This ‘triangle’ adds length to the shorter cleft side of the lip. While this techniques provides excellent lip length, it comes at the expense of a less natural appearing scar across the columnella.
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Figure 4. The triangular flap repair (Tennyson-Randall) |
Each technique differs in how the short lip is ‘lengthened’ in order to level Cupid’s peaks. In both repairs, the orbicularis is detached from its abnormal position at the alar base and reapproximated in the midline, helping to restore the continuity of the vermilion border and recreate a symmetric Cupid’s bow.
In a bilateral complete cleft lip, both sides of the abnormal orbicularis muscle must be brought together across the prolabial segment. If it appears that either side of the cleft is too wide to close, with risk of muscle dehiscence due to too much tension, a ‘lip adhesion’ can be done, bridging the defect by reapproximating skin only. This allows the short soft tissues to stretch while often ‘molding’ the protuberant premaxilla into a more anatomical position and a narrower defect for later definitive closure. The most common repair is based on Millard’s design.
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Figure 5. The bilateral cleft lip repair design (after Mulliken and Millard) |
Traditionally, the initial cleft nose repair was done as a secondary procedure when the child was older and nose growth had reached its final development. Recently, the trend has been to fix the nose primarily along with the lip. At the time of the lip repair, the alar cartilages can be freed from their abnormal configuration and repositioned to reshape the nasal tip and nostril, restoring better symmetry at an earlier age. A nasal stent may also be used to reshape the nostrils. As the child grows, septoplasty and further revisions may be required to achieve more tip projection and columellar length.
While a cleft palate may not be as obvious or visible as a cleft lip and nose, a palatal defect is more functionally debilitating with respect to feeding, middle ear problems and later speech development. The palate is composed of two parts: the ‘primary’ palate or premaxilla (anterior), and the ‘secondary’ palate (posterior), which includes both the lateral palatal shelves of the hard palate as well as the muscular soft palate. (Fig 6)
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Figure 6. Palate anatomy. A) Skeletal. B) Soft tissue. C) Incisive foramen. D) Postgerior edge of the hard palate. E) Soft palate |
During embryologic development of the secondary palate, the lateral shelves change from a vertical position to a horizontal orientation as the tongue descends, allowing them to meet in the midline and fuse with the premaxilla which has moved into position anteriorly, and the nasal septum descending from above. The anterior portion ossifies while the posterior palate remains mobile. The outer rim of the hard palate where the teeth reside is referred to as the alveolus or alveolar ridge. The premaxilla, carrying the tooth buds of the central incisors, is a part of the alveolar ridge. The hard palate separates the oral cavity from the nasal cavity and is also important for articulation during speech.
The soft palate is comprised of two paired muscles that meet in the midline to form a sling : the tensor veli palatini, and the levator veli palatini. (‘Veli’ is Latin for curtain as it refers to the soft palate in the posterior pharynx). (Fig 7)
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Figure 7. Palate musculature. A) Normal palate. B) Cleft palate. C) Tensor veli palatini muscle D) Hook of Hamulus. E) Levator veli palatini muscle |
This sling is attached to the posterior edge of the hard palate, and acts to close off the nasopharynx, along with the constricting lateral pharyngeal walls, by lifting the soft palate up and back to the posterior pharyngeal wall. Normal speech is dependent upon modulation of nasal air release as further reviewed in chapter X.
As with cleft lips, any disruption during the process of facial development can result in varying degrees of cleft palate, from the subtle clefting of the muscles on in a submucous cleft to clefting of the entire soft and hard palate. Clefting may also occur in combination of hard and soft palate defects. Alveolar clefts may be either unilateral or bilateral, located along the division between primary premaxilla and secondary palatal shelves, extending to the midline of the hard palate, whereas clefts of the soft palate appear only in the midline.
The goal of palate repair is to restore continuity of the hard and soft tissue while recreating the dynamic muscular sling of the soft palate. Palate repair also has significant consequences on subsequent facial growth and dental occlusion.
Repair of the hard palate is accomplished, not by moving the misplaced palatal shelves, but rather by reattaching the mucosal coverings of the bone in two layers (nasal and oral). Because the palatal mucosa is thick and inflexible, it is mobilized from the bone so that the two sides can meet in the midline for a tension-free closure (to avoid dehiscence or fistula formation).
When there is a cleft of the soft palate, the muscles are attached to the posterior edge of the hard palate, rather than meeting in the midline to form a sling. In order to repair the soft palate, the misplaced muscles are detached from the hard palate and the muscle sling is reconstructed across the midline to restore length and mobility for adequate velopharyngeal closure. The reconstruction of the muscular sling is called an intravelar veloplasty. (Figs 8, 9, 10)
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Figure 8. The unilateral palate repair (after Veau). B) Note the greater palatine blood vessels supplying the mucoperiosteal flaps. C & D) Note the intravelar veloplasty repair of the clefted muscles abnormally inserted into the back of the osseus palate. |
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Figure 9. The bilateral palated repair (after Veau) with and intravelar veloplasty. |
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Figure 10. Furlow "double-opposing Z-plasty" palate repair. The Z-plasty adds length to the palate. |
The timing of palate repair is based upon considerations of speech development and the inhibition of subsequent growth by scar. Some surgeons repair the palate in two stages. The two-stage technique closes the soft palate at the time of earlier cleft lip repair followed by hard palate repair at a later date. However, most surgeons repair both the hard and soft palates together in a single stage between the ages of 9 and 18 months.
Persistent velopharyngeal inadequacy following soft palate repair be due to “mechanical” causes related to shortening, postoperative scarring, or poor palatal movement. When speech therapy cannot achieve adequate velopharyngeal closure, a secondary palatal surgery may be necessary to prevent create velopharyngeal adequacy. Pharyngoplasty narrows the area through which air can pass between oral and nasal cavity by joining parts of the soft palate with the posterior pharyngeal wall.
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Figure 11. Pharyngeal Flap. A) A superiorly based flap of posterior pharyngeal wall tissue is designed. B) The flap is reflected superiorly. C) The flap is inset into the cleft defect. D) The defect is closed leaving two lateral ports open for the reduced nasal air escape. |
The pharyngeal flap unfolds the soft palate to increase its length and sews it to a flap swinging forward from the back wall of the throat. (Fig 11)
Air is allowed to pass from mouth to nose through two ports on either side of the flap.
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Figure 12. Pharyngoplasty. Two superiorly based faps are designed and transposed across the midline to reduce the circumference of the nasopharyngeal port and thus nasal excape of air. |
The dynamic sphincter pharyngoplasty uses the palatopharyngeus muscles as extensions of the soft palate on both sides to join with a posterior pharyngeal wall flap, creating a central port between the uvula and the bridging palatopharyngeus flap. (Fig 12).
Pre-surgical Nasoalveolar Molding
Ideally, deficient tissue should be expanded and malpositioned structures should be repositioned prior to surgical correction. Pre-surgical nasal alveolar molding (PNAM) includes not only reduction of the size of the intra-alveolar cleft due to molding of the bony segments, but also the active molding and positioning of the surrounding soft tissues affected by the cleft, including deformed soft-tissue and cartilage in the cleft nose. This is accomplished through the use of a nasal stent that is based on a labial flange of a conventional or molding plate and enters the nasal aperture (Fig 13).
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Figure 13 The nasoalveolar molding device. A) The nasal stents B) The palatal obturating segment. |
The stent provides support and gives shape to the nasal dome and alar cartilages. PNAM may be successfully employed in early management of both unilateral and bilateral cleft anomalies in newborns. In the bilateral cleft, PNAM may be combined with columnellar elongation to create a “neo-columnella” through a non-surgical approach. With close coordination between the pre-surgical phase and the surgical method employed, these techniques greatly improve upon the results usually produced through traditional pre-surgical infant orthopedics. The result is an overall improvement in the aesthetics of the nasolabial complex and the unilateral and bilateral cleft condition, while minimizing the extent of surgery and the overall number of surgical procedures.
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