Cleft Lip and Palate
Travis T. Tollefson
INTRODUCTION
Comprehensive management of the patient with a cleft lip and palate requires a collaborative multidisciplinary team of specialists, typically including facial plastic or general plastic surgeons, otologists, speech and language pathologists, nurses, geneticists, orthodontists, dentists, oral surgeons, audiologists, pediatricians, and social workers. Orofacial clefting is the most common craniofacial birth defect; surgical management of this issue requires meticulous soft tissue technique, in which mere millimeters of error in infancy may result in permanent cleft stigmata. Producing a satisfactory aesthetic result depends upon careful attention to the skin and soft tissue, nasal cartilages, teeth, and skeletal components, which must be addressed sequentially during the child’s growth and development. Concomitantly, speech and swallowing function are rehabilitated through painstaking restoration of dental and palatal structures.
An orofacial cleft represents failure of fusion of the lip, nasal sill, alveolus, or palate, which may occur in a spectrum of combinations of unilateral or bilateral deformities. The etiology of orofacial clefting is not well understood, but the condition results from an interruption in the complex craniofacial developmental pathway. Clefts may develop as complete or incomplete lip defects, complete or incomplete palate defects, or a combination that may span all the way from the nasal sill to the uvula (Fig. 36.1). Lesser manifestations of orofacial clefting are termed “microform,” “occult,” “minor,” or “forme fruste” (aborted form).
A variety of classification schemes have been suggested, the first in 1938 when Veau described his system: group A includes defects of the soft palate l only; group B includes defects of the hard and soft palate not extending anterior to the incisive foramen; group C includes defects extending through the entire palate and the alveolar ridge; and group D includes complete bilateral cleft lip. This general framework may be useful in discussion; however, further understanding of the developmental pathways that lead to orofacial clefting has simplified classification of the palatal cleft based on whether the primary palate (structures anterior to the incisive foramen, including the lip, premaxilla, and anterior septum) or the secondary palate (structures posterior to the incisive foramen, including the lateral palatine shelves, soft palate, and uvula) is involved.
A comprehensive classification scheme should identify involvement of the primary and secondary palates as well as lip and nasal deformities so that these areas may be addressed specifically during management. I prefer to determine initially whether the cleft is typical or atypical. The atypical craniofacial clefts were described in the landmark 1976 article by Tessier, who outlined a classification scheme for orofacial clefts that present with atypical orientations, such as the number 7, macrostomia due to a cleft at the commissure of the lip (Fig. 36.2). Extension of these clefts through the soft tissues may involve the maxilla, orbit, and skull base. For example, a bilateral Tessier number 4 cleft extends from the upper lip through the nasolacrimal duct and into the lower eyelid at the medial canthus (Fig. 36.3).
Typical orofacial clefts are described by laterality of the lip cleft (unilateral or bilateral) and its degree. The cleft lip may be complete (through the lip and nasal sill), incomplete (orbicularis oris and skin are intact for at least three-fourths of the length of the lip), or microform (characterized by a philtral skin
groove, minor nasal deformity, orbicularis oris discontinuity, and a notched vermilion-cutaneous junction with disruption extending to no more than one-fourth of the labial height, as measured from the normal peak of the Cupid’s bow to the nasal sill) (Fig. 36.4). The cleft alveolus may be complete or notched. Independent of the cleft lip type, the cleft palate is described as unilateral (one palatal shelf is attached to the nasal septum) or bilateral.
groove, minor nasal deformity, orbicularis oris discontinuity, and a notched vermilion-cutaneous junction with disruption extending to no more than one-fourth of the labial height, as measured from the normal peak of the Cupid’s bow to the nasal sill) (Fig. 36.4). The cleft alveolus may be complete or notched. Independent of the cleft lip type, the cleft palate is described as unilateral (one palatal shelf is attached to the nasal septum) or bilateral.
 FIGURE 36.1 The spectrum of incomplete cleft palate includes defects ranging from isolated uvular bifidity to palatal clefting as anterior as the incisive foramen: (A) intraoral view of an incomplete soft palate cleft shows extension just to the hard palate; (B) another child’s unrepaired cleft palate extends through the hard palate. The adenoid pad is seen between the two sides of the cleft uvula. |
 FIGURE 36.2 Atypical orofacial cleft, Tessier No. 7, demonstrating macrostomia (A) preoperatively, (B) after soft tissue repair, and (C) left lower eyelid coloboma and ocular dermoids prior to removal. |
 FIGURE 36.3 Photograph of a child with bilateral Tessier No. 4 orofacial clefts (A) preoperatively and (B) after subunit repair to recreate lower eyelids and continuity of the mouth; maxillary clefting will require future bone grafting. |
 FIGURE 36.4 The features of a typical right microform unilateral cleft lip shown in (A) frontal view and (B) base view, including notched mucosa, elevated Cupid’s bow peak, furrowing of the philtral column, thinning of the dry vermilion medially, widening of the nasal base, hypoplasia of the orbicularis oris muscle, and asymmetry of the nostril. |
In this chapter, I will outline the following: multidisciplinary management of cleft lip and palate, physical examination findings, and potential associated syndromic and nonsyndromic comorbidities. Preoperative planning will be reviewed, along with presurgical nasoalveolar molding (PNAM), and the indications and contraindications for repair of the surgical cleft. Preferred techniques for unilateral and bilateral cleft lip repair and palatoplasty will be described. Postoperative management will be reviewed with emphasis on prevention of complications.
HISTORY
The role of a multidisciplinary team is to address the conditions that coexist with orofacial clefts, ranging from difficulties with speech and swallowing to Eustachian tube dysfunction and audiologic issues to orthodontic, dental, and orthognathic problems. Care of the child born with a cleft lip and/or palate begins with a consultation soon after birth, during which the multidisciplinary team focuses on teaching the mothers effective feeding techniques and emphasizes the importance of appropriate weight gain. In each patient, a comprehensive history is obtained that includes familial, prenatal, and delivery events. Attention is dedicated to the presence of additional physical findings as well as the possibility of a syndromic or sequence event. Consultations with neonatal intensivists, geneticists, and additional specialists are made on a case-by-case basis. With each patient, specialized care commonly continues through the teenage years and into early adulthood, involving regular team meetings and sequential intervention, both operative and nonoperative.
PHYSICAL EXAMINATION
The presence of an orofacial cleft may be identified on routine prenatal ultrasound at 18 to 20 weeks of gestation. Three-dimensional ultrasound technology has increased the detection rate of cleft lip, but not isolated cleft palate. Because of this, expectant parents with a positive ultrasound are now presenting for prenatal facial plastic surgery consultation. Preparation for and understanding of the surgical repairs through educational consultation may help allay parental concerns. Prenatal consultation also allows the surgeon to begin assessment for preoperative therapies, such as PNAM, which requires frequent clinic visits and significant parental commitment.
The neonate is examined for cleft lip and cleft palate immediately after birth. Identification of the subtle microform cleft lip or submucous cleft palate is facilitated with training and experience in identifying muscle abnormalities that lack obvious epithelial defects. A thorough examination of the head and neck begins with assessment and palpation of the continuity of the upper lip and nares. The maxillary alveolus is palpated for a notch or cleft.
 FIGURE 36.5 Lower lip pitting seen in Van der Woude’s syndrome. |
Improved visualization of the soft palate may be achieved by placing the child supine in the parent’s lap and carefully extending the neck. The neonate often opens his or her mouth and spontaneously protrudes the tongue, giving a good view. A tongue depressor and light should also be used to assess the palate and uvula. A bifid uvula or zona pellucida in the soft palate should prompt palpation for a hard palate notch. The lower lips are inspected for nodular lip pits as seen in Van der Woude’s syndrome (Fig. 36.5). The oral commissure and cheeks are evaluated for atypical clefting or auricular remnants found in oculoauriculovertebral spectrum (Fig. 36.6). The shapes and positions of the ears are inspected, looking for microtia or other features of hemifacial microsomia. The eyelids may also demonstrate colobomata or notches.
INDICATIONS
The typical indications for proceeding with repair of the cleft lip are difficulty feeding, poor weight gain, and airway obstruction. Although poor weight gain may indicate feeding difficulty, the surgeon and pediatrician must also consider cardiac or other defects leading to failure to thrive. Breastfeeding is encouraged, though formula supplementation is common. Postnatal weight loss of up to 10% of birth weight is normal, but should be regained in 2 weeks. Thereafter, at least 1 ounce of weight gain per day indicates adequate feeding. Nasogastric tube feeding is seldom required if a feeding nurse specialist can counsel the mother effectively. Neonates with cleft palate have difficulty creating suction; they should be positioned upright to limit nasal regurgitation. Specialized nipples, such as the Haberman nipple (Fig. 36.7), control the flow rate from a bottle and may limit the infant’s fatigue while feeding.
 FIGURE 36.6 Oculoauriculovertebral spectrum, demonstrating hemifacial microsomia, left anotia, and (A) a repaired unilateral complete cleft lip and palate, (B) 3-D CT scan showing the left unrepaired alveolar cleft and left mandibular ramus reconstruction with rib grafting. |
 FIGURE 36.7 Infant with a bilateral cleft lip and palate feeding with a Haberman nipple, while the PNAM appliance is in place. The cylindrical nipple on the bottle may be squeezed to control flow. |
CONTRAINDICATIONS
Repair of an orofacial cleft may be contraindicated until airway obstruction is addressed. Airway obstruction due to the tongue is seen in a small proportion of infants with orofacial clefts, with Pierre Robin sequence being the most common cause (Fig. 36.8). Initial treatment includes prone positioning, nasopharyngeal trumpet, nasal continuous positive airway pressure, or endotracheal intubation. Definitive surgical intervention may include tracheostomy, mandibular distraction osteogenesis, or tongue-lip adhesion.
PREOPERATIVE PLANNING
The typical timeline for each of the discrete steps of orofacial cleft management is shown in Figure 36.9. Cleft lip repair is often performed at 3 to 5 months of age. For infants with cleft palate, bilateral myringotomy and tympanostomy tube placement is followed by behavioral audiometry prior to the cleft palate repair, performed between 10 and 14 months of age. Speech and language pathology assessment and therapy are initiated after vocabulary develops (3 to 5 years old) with emphasis on identifying velopharyngeal dysfunction. A secondary speech surgery may be required to address hypernasality at this time. Superiorly based flap pharyngoplasty, sphincter pharyngoplasty, and Furlow double-opposing Z-palatoplasty are procedures frequently used to limit nasal air escape; when employing these techniques, the surgeon must be aware of the potential to produce obstructive sleep apnea from excessive nasal obstruction.
In the presence of a typical unilateral or bilateral cleft lip and palate, dental and orthodontic care should be initiated early. Prior to the eruption of the maxillary canines at around 7 to 10 years of age, an orthodontist will begin dental preparation for the alveolar bone graft. The bone graft is often harvested from the iliac crest at approximately 10 years of age. Orthognathic surgery for correction of dentofacial malocclusion is delayed
until skeletal growth is complete, which occurs earlier in females than in males. Subsequently, definitive cleft septorhinoplasty will address the nasal deformity in the teenage years.
until skeletal growth is complete, which occurs earlier in females than in males. Subsequently, definitive cleft septorhinoplasty will address the nasal deformity in the teenage years.
 FIGURE 36.8 Infant with Pierre Robin sequence demonstrating microgenia, glossoptosis, and cleft palate with external mandibular distraction device. |
 FIGURE 36.9 Timeline for comprehensive management of cleft lip and palate showing surgical procedures on the bottom and nonsurgical treatment above, from birth to adulthood. |
Cleft Lip Repair
During the initial consultation, weight gain and feeding are assessed. The upcoming surgical procedures and future multidisciplinary management are discussed. Wide cleft lips may require one of several presurgical preparations including: lip taping, oral appliance use (Latham device), PNAM, 2-staged repair with a primary lip adhesion, or delayed repair to allow tissue growth. Daily lip taping may affect soft tissue expansion. Standard protocols use an adhesive strip or tape to appose the lip edges. The cheeks are protected with a skin barrier that requires routine replacement due to lip wetting from feeding (Fig. 36.10). The Latham appliance actively repositions the premaxilla but is not used frequently. Concerns with potential maxillary growth inhibition continue to fuel the debate regarding the appropriate use of presurgical premaxillary positioning.
 FIGURE 36.10 Infant with bilateral cleft lip and palate shown (A) preoperatively, (B) during lip taping, and (C) 1 week after surgical repair, with silicone nasal stents taped in place. |
 FIGURE 36.11 Intraoral impression of the maxilla of an infant with a bilateral cleft lip and palate shown before (left) and after (right) nasoalveolar molding repositioned the premaxilla posteriorly. |
Presurgical Nasoalveolar Molding
Greyson and Cutting introduced and developed the principles of PNAM, including the addition of nasal prongs to the traditional intraoral alveolar molding device. Alveolar molding will bring the maxillary alveolar segment or segments into contact with the premaxilla in both unilateral and bilateral cleft lip and palate. Further objectives of the PNAM technique are to bring the cleft lip segments closer together, expand the columellar mucosa and skin, and improve symmetry of the nasal tip. Parental compliance should be assessed before initiating a PNAM treatment program, which should begin within the first several weeks after birth.
In cases of a wide unilateral or bilateral cleft lip, alveolus and palate, presurgical orthodontic treatment with PNAM is started at the second office visit (Fig. 36.11). A specialized orthodontist fabricates the PNAM appliance; a maxillary impression is taken around the third postnatal week, after which, the mold is used to create an acrylic nasoalveolar molding appliance. The orthodontist adjusts the appliance every week by removing hard acrylic and adding soft acrylic (Permasoft denture liner, Dentsply International, Chicago, IL). Nasal prongs may be added to the appliance and positioned just under the soft tissue triangle of the nostril (Fig. 36.12). The stents are adjusted by adding soft acrylic to help create a tissue-expanding effect on the columellar skin, simultaneously reorienting the nasal tip. The orthodontist adapts the appliance over the next several months to reposition the alveolar arches, delaying the definitive cleft lip repair until approximately 4 to 5 months of age. The alveolar segments may be repositioned to come in contact prior to lip closure, potentially allowing closure of the alveolar cleft with a gingivoperiosteoplasty.
 FIGURE 36.12 Infant with a right complete unilateral cleft lip and palate (A) preoperatively and (B) during PNAM with the appliance and nostril prong in place; (C) another infant with bilateral cleft lip and palate shown preoperatively, (D) with PNAM appliance, and (E) after repair in a frontal view showing the enhanced medial lip tubercle and (F) profile view showing the effect of primary rhinoplasty on the nasolabial relation. |
Staged Repair
In the severely wide cleft lip, some surgeons choose to use a lip adhesion technique to bring the mucosa and lip skin together without dissection of orbicularis oris muscle. The second-stage definitive cleft lip repair is then delayed for several months until the orthopedic forces created by the lip adhesion move the lip and alveolar segment into closer proximity. Another way to stage repair of the bilateral cleft lip deformity is to address each side individually. The main drawbacks to this approach are the increased potential for asymmetry and difficulty in reconstituting an intact orbicularis oris sphincter over the premaxilla.
SURGICAL TECHNIQUE
Unilateral Complete Cleft Lip
After induction of general endotracheal anesthesia, the tube is taped to the midline of the chin to avoid distortion of the upper lip and nasal tip, and the eyes are protected with occlusive dressings. Local anesthetic (1% lidocaine with 1:100,000 epinephrine) is infiltrated under the upper lip, into the supraperiosteal planes of the maxilla, in order to block the infraorbital nerves. The oral commissures and midline buccal sulcus are injected, taking care to avoid edema at the lip edges. Low volume injection into the plane between the lower lateral cartilages and skin-soft tissue envelope (SSTE) of the nasal tip is performed through standard marginal injection sites.
Lip Markings and Flap Design
Initial markings are placed at the subnasale and lateral alar base, by tattooing with a 30-gauge needle and methylene blue (Fig. 36.13). The junction of the columella and the upper lip is marked, followed by the midline
philtral column at the vermilion-cutaneous (v-c) junction. Approximately 2 to 3 mm lateral to this, the Cupid’s bow peaks on the cleft and noncleft sides are marked.
philtral column at the vermilion-cutaneous (v-c) junction. Approximately 2 to 3 mm lateral to this, the Cupid’s bow peaks on the cleft and noncleft sides are marked.
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