The current article reviews the pertinent literature on the management of cleft lip and palate. We review the commonly used surgical techniques for repair, adjunctive options for treatment, clinical outcomes, complications, and concerns to consider. Throughout the discussion, we state the level of evidence where applicable and identify areas for future study.
Key points
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The repair of unilateral cleft lip is performed using a rotation-advancement, geometric, straight-line, or hybrid technique.
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For bilateral cleft lip repair, most surgeons use either the Millard or Mulliken technique, and their variations.
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Most cleft centers perform cleft lip repair at the age of 3 to 5 months.
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Presurgical infant orthopedics, which can include nasoalveolar molding, is used before definitive cleft lip repair.
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For cleft palate repair, the 2-flap palatoplasty and Furlow double-opposing Z-plasty are most commonly used.
Introduction
At an estimated prevalence of 16.86 cases per 10,000 live births, isolated cleft palate, as well as cleft lip with or without cleft palate, is the most common congenital orofacial malformation in the United States. Children with cleft anomalies may experience a multitude of physical and developmental challenges. There also may be psychosocial and emotional concerns for the patients and their families. As such, comprehensive care for the patient with cleft lip and/or palate requires an interdisciplinary team. The guidelines for team care outlined by the American Cleft Palate Association recommend team members that may include anesthesiology, audiology, genetics, neurosurgery, nursing, ophthalmology, oral maxillofacial surgery, orthodontics, otolaryngology–head and neck surgery, pediatrics, pediatric dentistry, physical anthropology, plastic surgery, prosthodontics, psychiatry, psychology, social work, and speech-language pathology. Although every specialty may not be represented, the quality of care is augmented through collaborative discussion and coordination of care.
Broadly speaking, orofacial cleft anomalies may be unilateral or bilateral and involve the lip, the palate, or both. Although there have been considerable publications on this topic, most are single-surgeon/center experience papers or are retrospective in nature. As a result, the cleft lip–cleft palate literature regarding the clinical and surgical decision points lacks consensus. This review article seeks to define the typical management plans, describe the various viewpoints, and suggest recommendations based on the levels of evidence ( Table 1 ) on the management of cleft lip and palate. The article is organized to address management of the techniques, timing, outcomes, and complications starting with cleft lip, and then addressing the same in cleft palate management.
| Level I | High-quality, properly powered and conducted randomized controlled trial, systematic review, or meta-analysis of these studies |
| Level II | Well-designed controlled trial without randomization; prospective comparative cohort trial |
| Level III | Retrospective cohort study, case-control study, or systematic review of these studies |
| Level IV | Case series with or without intervention; cross-sectional study |
| Level V | Expert opinion, case reports, or bench research |
Introduction
At an estimated prevalence of 16.86 cases per 10,000 live births, isolated cleft palate, as well as cleft lip with or without cleft palate, is the most common congenital orofacial malformation in the United States. Children with cleft anomalies may experience a multitude of physical and developmental challenges. There also may be psychosocial and emotional concerns for the patients and their families. As such, comprehensive care for the patient with cleft lip and/or palate requires an interdisciplinary team. The guidelines for team care outlined by the American Cleft Palate Association recommend team members that may include anesthesiology, audiology, genetics, neurosurgery, nursing, ophthalmology, oral maxillofacial surgery, orthodontics, otolaryngology–head and neck surgery, pediatrics, pediatric dentistry, physical anthropology, plastic surgery, prosthodontics, psychiatry, psychology, social work, and speech-language pathology. Although every specialty may not be represented, the quality of care is augmented through collaborative discussion and coordination of care.
Broadly speaking, orofacial cleft anomalies may be unilateral or bilateral and involve the lip, the palate, or both. Although there have been considerable publications on this topic, most are single-surgeon/center experience papers or are retrospective in nature. As a result, the cleft lip–cleft palate literature regarding the clinical and surgical decision points lacks consensus. This review article seeks to define the typical management plans, describe the various viewpoints, and suggest recommendations based on the levels of evidence ( Table 1 ) on the management of cleft lip and palate. The article is organized to address management of the techniques, timing, outcomes, and complications starting with cleft lip, and then addressing the same in cleft palate management.
| Level I | High-quality, properly powered and conducted randomized controlled trial, systematic review, or meta-analysis of these studies |
| Level II | Well-designed controlled trial without randomization; prospective comparative cohort trial |
| Level III | Retrospective cohort study, case-control study, or systematic review of these studies |
| Level IV | Case series with or without intervention; cross-sectional study |
| Level V | Expert opinion, case reports, or bench research |
Cleft lip
Overview
A typical orofacial cleft can be classified by laterality , extent , and severity . The laterality (left, right, asymmetric/symmetric bilateral) is noted with the unilateral deformity being more common than the bilateral. The extent of the cleft lip is variable and can include the cleft alveolus, which can 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. The extent of the cleft is classified as complete ( Fig. 1 ), incomplete ( Fig. 2 ), or microform ( Fig. 3 ). In the complete cleft, there is disruption of the lip’s mucosal up to the nasal floor with the associated nasal deformity. There is a spectrum of incomplete clefting, ranging from vermilion notching to near-complete disruption of the lip with a remaining Simonart band. An incomplete bilateral cleft lip can be quite asymmetric ( Fig. 4 ). The severity of the cleft lip width can make the repair more difficult because of wound tension. Management of the more severe cleft lip often requires a more prolonged presurgical preparation period (eg, presurgical infant orthopedics [PSIO]).
In the complete unilateral cleft lip, there is an external and upward rotation of the medial segment of the premaxilla and an internal and posterior rotation of the lateral segment. Fibers of the orbicularis oris muscle attach medially to the base of the columella and laterally to the alar base. The nasal septum is dislocated from the vomerian groove with a shortening of the columella. The alar cartilage of the cleft side is deformed such that the medial crus is displaced posteriorly and the lateral crus is flattened over the cleft.
In the complete bilateral cleft lip deformity, the premaxilla and prolabium are entirely separate from the lateral lip and maxillary segments. As a result, the premaxilla protrudes past the lateral segments. The prolabium can vary in size and lacks the normal philtral structure of a central groove and philtral ridges. The vermilion cutaneous junction and cutaneous (white) roll are often deficient. In a completed bilateral cleft lip, the prolabium does not contain orbicularis oris muscle. The nasal deformity associated with bilateral cleft lip is a shortened columella, flattened nasal tip, and alar hooding. Flaring of the alar base is common with inadequate alar base repair.
Surgical Techniques
Unilateral cleft lip
The objective of cleft lip repair is to approximate the medial and lateral lip elements with preservation of natural landmarks, align a functional concentric orbicularis, and to establish symmetry and proportionality. Unilateral cleft lip repair designs can be divided into 3 schools, which include (1) straight-line closure, (2) geometric, and (3) rotation-advancement techniques. The most common technique used to repair a unilateral cleft lip is the Millard rotation-advancement flap, as well as its modifications, including the Noordhoff vermilion flap and the Mohler modification. There are few studies that compare the outcomes of various cleft lip repair techniques. Holtmann and Wray (1983) studied patients randomized to receiving either the Millard rotation-advancement repair or the triangular (geometric) cleft lip repair, as described by Randall and colleagues (Level II evidence). They did not find any significant differences in esthetic outcomes between the 2 groups. Chowdri and colleagues (1990) also compared the Millard and Randall techniques in a randomized study (Level I evidence). Similar to Holtmann and Wray, no differences were found in outcomes and both techniques were recommended in the repair of cleft lip.
There has been debate regarding whether the extent that the orbicularis oris muscles should be extensively released from the aberrant insertions on the maxilla to facilitate cleft lip repair. Some have felt that excessive dissection and a tense approximation of the muscular elements will lead to maxillary growth disturbance. However, there is no evidence at present that muscular reconstruction leads to growth disturbance (Level IV evidence). In addition, the prevailing theory is that reconstructed musculature encourages normal and symmetric facial skeletal growth. Two studies have suggested that muscular reconstruction leads to improved facial development (Level II evidence). Although additional evidence is needed to conclude definitively regarding muscular reconstruction, it does seem to be associated with improved functional and esthetic outcomes.
Bilateral cleft lip
There are a few approaches to the repair of bilateral cleft lip. One approach is a 2-stage repair with columellar elongation as the second procedure between the ages of 1 and 5 years (Level V evidence). Alternatively, a 1-stage approach with primary rhinoplasty at the time of cleft lip repair has been advocated for symmetric cases (Level IV–V evidence).
The severely wide bilateral cleft lip with significantly projected premaxilla may necessitate with a staged cleft lip repair, PSIO, delayed repair, or premaxillary setback. In grossly asymmetric clefts or when a prolabium is less than 6 mm in height, a lip adhesion is performed, followed by a delayed definitive cleft lip repair, after the adhesion has successfully brought the soft tissue elements and maxillary arches closer together (Level V evidence). Presurgical infant orthopedics, which includes nasoalveolar molding, is effective at decreasing the severity of the cleft width by applying orthopedic forces to the maxillary arches and premaxilla with an oral appliance. Where presurgical infant orthopedics is ineffective or unavailable, premaxillary setback with vomer osteotomy can be performed with caution. The risks of devascularizing the premaxilla as well as maxillary growth inhibition should be considered (Level IV evidence). Most North American surgeons use the Millard and Mulliken bilateral cleft lip techniques or a variation thereof. Similar to unilateral cleft lip repair, there is insufficient evidence to suggest the superiority of one technique over another.
Timing
There are advocates for cleft lip repair over a range of time frames, from the neonatal period to 5 to 6 months of age (Level V evidence). Intrauterine repair has been piloted using animal models based on the potential benefit of no scar formation (Level V evidence); however, this has not been seriously pursued in humans, as the theoretic benefits do not outweigh the risks of exposing both the mother and fetus to this procedure. Neonatal repair also has been investigated for the similar reasons of minimizing scar formation and potentially allowing molding of the nasal cartilages due to the intrauterine exposure to maternal hormones. Earlier repair also has the proposed benefits of facilitating maternal-child bonding; however, studies have not been able to substantiate this (Level V evidence).
In the absence of an obvious benefit with earlier repair, most surgeons adhere to the conventional rule of 10’s. Specifically, surgery is deferred until the child is 10 pounds in weight, at or after 10 weeks of age, with a hemoglobin concentration of 10 g/dL. This increases the safety of undergoing anesthesia. It also has been argued to improve esthetic results, as waiting allows for the lip musculature to grow (Levels IV and V evidence).
Other Therapeutic Options
Presurgical infant orthopedics and nasoalveolar molding
Evidence supporting the use of PSIO is conflicting. This can likely be attributed to sparse evidence to definitively suggest a presurgical method is superior to another. Existing studies fail to use consistent outcome measures, which have partially driven the development of Eurocleft and Americleft research groups. Two systematic reviews that examine the utility of PSIOs concluded that there is insufficient evidence to suggest an improvement in maxillary arch form/facial growth/occlusion, motherhood satisfaction, infant feeding/nutritional status, or speech (Level II evidence).
Nasoalveolar molding (NAM) is a type of PSIO that incorporates the intraoral appliance with nostril prongs to improve the cleft nasal deformity ( Fig. 5 ). There is more supportive evidence for PSIO due to the beginning of intraoral devices decades before NAM. Studies have shown that when instituted at 1 week of age and continued for 3 to 4 months, NAM is effective in approximating the cleft as well as improving the nasal deformity. Specifically, patients undergoing NAM treatment experienced improved nasal alar symmetry, columella lengthening, and nasal tip projection (Levels II to V evidence). The counter arguments include nasal relapse and maxillary growth constriction. A recent review concluded that there is some evidence for its use in the unilateral cleft population in improving nasal symmetry (Level III evidence). Although randomized controlled trials at multi-institutional levels are lacking, there is evidence that NAM should be incorporated into the routine management of both unilateral and bilateral clefts. In a phone survey that contacted 89% of North American cleft centers, more than one-third of the centers offer NAM as an adjunct to surgical repair of unilateral and bilateral cleft lip.
Lip adhesion
Lip adhesion surgery can be performed in unilateral and bilateral cleft lip. It is performed before definitive surgery, typically before 3 months of age. The rationale is that it applies orthopedic pressure on the underlying maxilla, thereby narrowing the cleft for the definitive repair (Level V evidence); however, the evidence is limited and there is the potential disadvantage of additional scarring (Level IV evidence).
Alveolar bone grafting
Primary alveolar bone grafting is typically performed at approximately 8 to 10 years of age. Some centers graft the alveolar cleft at age of 5 to 7 years, before the eruption of the permanent canines so as to improve bone height, dentofacial esthetics, and function (Level IV evidence). Performing a primary graft in children younger than this is associated with the risk of insufficient alveolar bone volume. Bone grafting in older children may be associated with an increased risk of failure, as healing occurs more slowly and there is increased donor site morbidity (Level II evidence). Iliac crest cancellous bone harvest is the standard, but other donor sites and off-label use of bone-morphogenetic protein have been described. More rarely described is the use of a split-rib technique with minimal maxillary dissection used for primary alveolar bone grafting, but the risks of maxillary growth restriction if performed too early must be considered (Level IV evidence).
Primary rhinoplasty
A paradigm shift to include primary rhinoplasty at the time of cleft lip repair has been noted over the past few decades (Level V evidence). Given the complexity of the nasal deformities associated with cleft lip, definitive rhinoplasty has and still is typically deferred until after adolescence and full skeletal growth (Level V evidence). The rationale for minimal primary rhinoplasty during infancy was concern that significant change would occur during adolescent growth, necessitating repeat surgery. There was also the theoretic risk of excessive scar tissue that would interfere with nasal growth. Finally, patients with cleft lip often require orthognathic surgery, which should precede definitive rhinoplasty.
Arguments against delaying rhinoplasty until adolescence are that waiting may lead to a worsened nasal deformity as well as symptoms of nasal obstruction and increased rates of revision surgery (Level IV evidence). It also may be associated with psychological stress, given that patients will have to live with the unrepaired deformity until adolescence. Over the past 3 decades, various investigators have published on their experiences with primary cleft rhinoplasty, demonstrating that stable long-term results can be achieved with minimal growth disturbance (Level III–IV evidence). Therefore, some evidence does exist to support primary rhinoplasty in improving nasal appearance and function. A recent study showed that more than half of North American cleft surgeons do perform a limited rhinoplasty at the time of primary lip repair.
Postoperative nasal stents
Nasal stents have been used for the goal of preventing secondary deformities with healing and scarring following primary repair ( Fig. 6 ). There have been case series, as well as one prospective study, demonstrating improved alar symmetry in those who underwent postoperative internal nostril stenting (Level IV evidence). The limitations of using nasal stents include poor patient tolerance, possible airway distress in the case of stent dislodgement, and pressure ulcers. Currently, there are no randomized controlled trials examining the benefits of postoperative nasal stenting.
Clinical Outcomes
There is significant variation among studies in measuring and reporting outcomes after cleft lip repair. Some investigators have used clinical photographs with subjective scoring, whereas others use 3-dimensional imaging or anthropometry. The heterogeneity among patient populations, surgical techniques, and outcome assessment strategies make comparisons across studies difficult.
One outcome measure that can be used to gauge the success of cleft lip repairs is the rate of revision surgery. In a review of 50 consecutive patients with bilateral cleft lip with either a cleft palate or cleft alveolus, Mulliken and colleagues found a nasolabial revision rate of 33% in the cleft lip and palate group (Level IV evidence). In the cleft lip and alveolus group, the revision rate was 12.5%. In a review of 750 patients with unilateral cleft lip, secondary reconstruction was performed in approximately 35% of patients (Level IV evidence). The highest revision rates were reported by the Eurocleft study, which assessed the practice patterns and outcomes of 5 cleft centers in Northern Europe (Level II evidence). Four centers provided revision rate data. One center reported a lip revision rate of 4%, and the remaining reported rates from 63% to 69%. For revision rates specific to nasal reconstruction, Mehrotra and Pradhan reported a second rhinoplasty rate of 10% after primary rhinoplasty at the time of cleft lip repair (Level IV evidence).
Although revision rates provide a quantifiable method of gauging outcomes, it must be interpreted with caution. The decision to undertake revision surgery is family and surgeon-dependent. As such, the undertaking of revision surgery may be as reflective of these preferences as it is of the esthetic and functional outcomes from the primary repair. Furthermore, higher revision rates as an indicator of poorer outcome may not be accurate, as a child undergoing multiple revisions may actually have a final result that is more esthetically and functionally pleasing than a child who does not undergo any revisions.
Complications and Concerns
Wound complications
In a recent retrospective review of 3108 cases, Schonmeyr and colleagues reported an overall short-term complication rate of 4.4% (Level IV evidence). In 0.5% of these cases, the complication was severe enough to warrant revision surgery. The most common early postoperative complications were wound dehiscence and/or infection, which were 4.3% in the previously mentioned study. This was consistent with the rates of 2.6% to 4.6% reported by other studies (Level IV evidence). Complete clefts and bilateral clefts were both significantly associated with wound dehiscence (Level IV evidence). Other complications included stitch granuloma (0.2%) and pressure necrosis (0.05%).
Maxillary growth
Concern also has been raised regarding cleft lip repair and effects on maxillary growth. There are various hypotheses for how lip repair can lead to maxillary retrusion. Some postulate that pressure from a repaired lip restricts maxillary growth (Level V evidence). Maxillary growth restriction theoretically could be greater in complete cleft lip-palate as the maxillary segments would be less able to withstand the restrictive forces (Level IV evidence). In a review of 82 patients with unilateral cleft lip, alveolus, and palate, lip repair was found to be associated with maxillary retrusion (Level IV evidence). Those with more severe defects were found to have greater retrusion. In a prospective study of 22 patients with unilateral cleft lip and palate, lip repair was found to cause transverse narrowing of the maxilla without any effects on sagittal growth (Level IV evidence).
Cleft palate
Overview
A cleft deformity can occur in both the primary and secondary palates. Clefts of the primary palate range from an alveolar notch to those that extend through the hard and soft palates. Clefts of the secondary palate range from a bifid uvula to clefts that extend to the incisive foramen. The soft palate consists of 5 muscles that are responsible for velopharyngeal closure, including the musculus uvulae, the palatoglossus, the palatopharyngeus, the tensor veli palatini, and the levator veli palatini. The levator veli palatini is the primary muscle involved in velopharyngeal closure. Normally, it originates from the Eustachian tube and inserts anteromedially onto the tensor aponeurosis, along with the tensor veli palatine. In the cleft palate, the levator muscles insert aberrantly onto the posterior edge of the hard palate. Contractions of the palatal muscles therefore become ineffective at closing the velopharynx.
Surgical Techniques
The goals of cleft palate repair include closure of the soft palate and reorientation of the levator veli palatini to obtain normal velopharyngeal closure and speech. Closure of the hard palate cleft separates the oral and nasal cavities. There are numerous techniques for cleft palate repair and there is significant variation in treatment protocols across cleft centers.
One of the oldest procedures performed is the von Langenbeck palatoplasty. With this technique, bipedicled mucoperiosteal flaps are raised off of the hard palate. The cleft edges are incised and both nasal and oral mucosa are medialized. The biggest drawback to this technique is that it does not add additional length to the soft palate (Level V evidence). Other techniques have been designed to improve velopharyngeal function by lengthening the velum. One such technique is the Veau-Wardill-Kilner palatoplasty, which is a variation of the V-Y pushback. Mucoperiosteal flaps are raised and retropositioned. This lengthens the velum but leaves a large area of exposed hard palate anteriorly, which heals by secondary intention. Variations of the V-Y pushback technique have fallen out of favor because of poor maxillary growth outcomes (Level V evidence).
Two-flap palatoplasty ( Fig. 7 ) was first introduced in 1967 by Bardach. Large mucoperiosteal flaps based on the greater palatine vasculature are raised. Closure is layered to minimize tension, with approximation of the nasal and then oral mucosa. The soft palate musculature is then repaired via an intravelar veloplasty (IVV). IVV involves releasing the levator veli palatini from its aberrant attachment to the posterior hard palate. Among cleft surgeons, consensus is that IVV does improve velopharyngeal function and may reduce rates of secondary speech surgery; the drawbacks include additional operative time and devascularizing the muscle. The muscle fibers are then reapproximated in the transverse direction to establish the palatal muscular sling. Since its introduction, studies on the effectiveness of IVV have had conflicting but overall supportive results. Marsh and Galic prospectively studied 51 patients randomized to receive or not receive IVV during cleft palate repair. In this study, IVV was not associated with improved speech (Level II evidence). On the contrary, a retrospective study of 213 patients showed that IVV improved speech and decreased the rate of secondary velopharyngeal insufficiency (Level IV evidence). Neither study found an increased rate of complications with IVV. Other studies also have found improved speech and velopharyngeal function with IVV (Levels I and II evidence).
