Cleft Palate Repair Treatment & Management

The Pierre Robin sequence is classically associated with retrognathia, glossoptosis, respiratory distress, and a cleft palate. If untreated, death may result from obstruction by the tongue, which has fallen back in the airway. The most appropriate first step in management is to place the infant in the prone position to allow the tongue to fall forward and clear the trachea.

Orthodontic interventions

The available data suggest that to optimize speech development, some degree of facial growth distortion may need to be accepted. One role of orthodontic intervention is to minimize the severity of the growth disturbance. Interventions vary according to the type of cleft.

Many types of orthodontic appliances have been used in the treatment of patients with cleft palate. In cleft lip/palate, orthodontic appliances can be used to realign the premaxilla into a normal position prior to lip closure. Orthodontic interventions in patients with cleft palate are frequently aimed at maxillary arch expansion, correction of malocclusion, and correction of an often developing class III skeletal growth pattern. The maxillary dental arch contracture may become significant, requiring the surgical repair of the hard palate. Orthodontic interventions may be started early or delayed for several years. When orthodontic manipulation is initiated early, difficulties may occur. Maintaining orthodontic appliances in the infant population may present a challenge unless these appliances are fixed in position.

The beneficial influence of these orthopedic interventions has also been questioned, especially in isolated patients with cleft palate. The most beneficial period for orthodontic interventions in isolated cleft palate may be during the mixed dentition period.

A study by Garland et al indicated that in patients with cleft lip/palate, outcomes from the use of active presurgical devices for closing the alveolar gap do not significantly differ from those for passive devices in terms of facial growth by age 10 years. However, active devices were found to close the alveolar gap more quickly than passive ones. ^[10]

At approximately age 6-8 years, the permanent incisors are erupting. During this period, children are beginning to have social interactions with their peers. The presence of grossly malaligned teeth and severe malocclusion can lead to social isolation. The incisor relation can be corrected and maintained with relatively simple interventions. Patients who undergo palatal arch expansion therapy during this period can benefit from the rapid growth phase. The orthodontic intervention can also proceed with more cooperation from the patient in this age group. Orthodontic management of arch deformities after the permanent dentition has erupted is more limited. The established malocclusion and asymmetry between the maxillary arch and mandibular arch usually require orthognathic surgery.

Timing of palatal closure

The timing of surgical repair of cleft palate remains controversial. The goals of palatal repair include normal speech, normal palatal and facial growth, and normal dental occlusion. Physicians believe that early palate repair is associated with better speech results but early repair also tends to produce severe dentofacial deformities. Randall and McComb as well as Lehman and colleagues consistently reported that children whose palates were repaired at an earlier age appeared to have better speech and needed fewer secondary pharyngoplasties then those whose surgeries had been delayed beyond the first 12 months.

Noordhoff and associates found that children undergoing delayed palatoplasty for cleft palate had significantly poorer articulation skills before the hard palate closure than children of the same age who did not have clefts. These benefits of early cleft palate repair from the standpoint of speech and hearing must be weighed against the increased technical difficulty of the procedure at a younger age and possible adverse effects on maxillary growth.

Numerous studies failed to demonstrate an observable difference in underdevelopment of the palatal arch among children undergoing operations at various ages. The surgical intervention appears to interfere with midfacial growth without regard to the age of the patient at the time surgery is performed.

Bifid uvula occurs in 2% of the population. Although bifid uvula occurs in association with submucous cleft palate, most infants with bifid uvula do not have this problem. The recommended management of a bifid uvula is close observation to ensure that speech develops normally.

Sequence of operations

Multiple protocols for the management of cleft lip/palate have been suggested over the years by various authors. Today, the mainstream of cleft repair calls for closure of the lip at an early age (from 6 wk to 6 mo), followed by closure of the palate secondarily approximately 6 months later. This protocol has little impact on facial development.

When managing a residual alveolar defect and an associated oronasal fistula, the primary goal of surgery is to allow subsequent development of a normal alveolus. Optimal eruption of teeth at the cleft site and development of normal periodontal structures of the teeth adjacent to the cleft occur when bone grafting and final fistula closure are performed prior to eruption of the permanent canine at the cleft site.

A study by Patmon et al indicated that in the United States, in individuals with a complete cleft palate, African-American and Hispanic patients undergo late bone grafting (after age 12 years) for alveolar clefts more frequently than do patients in other racial and ethnic groups. This is despite the fact that in the treatment of alveolar clefts, it is typically preferred that bone grafting be performed between the ages of 6 and 12 years, with lesser results found with later grafting. ^[11]

Choice of operation

The list of surgical techniques used in palatal cleft closure is extensive. The repairs differ depending upon whether the cleft is an isolated cleft palate or part of a unilateral or bilateral cleft lip and palate. The 3 main categories include (1) simple palatal closure, (2) palatal closure with palatal lengthening, and (3) either of the first two techniques with direct palatal muscle reapproximation.

von Langenbeck procedure

The simple palatal closure was introduced by von Langenbeck and is the oldest cleft palate operation in wide use today. The bipedicle mucoperiosteal flaps were created by incising along the oral side of the cleft edges and along the posterior alveolar ridge from the maxillary tuberosities to the anterior level of the cleft. The flaps were then mobilized medially with preservation of the greater palatine arteries and closed in layers. The hamulus may need to be fractured to ease the closure. The von Langenbeck repair continues to be popular because of the simplicity of the operation.

This technique can successfully close moderate-size defects. Modern critics of the von Langenbeck technique cite the unnecessary anterior fistulas it promotes, the insufficiently long palate it produces, and the inferior speech result associated with it.

Trier and Dreyer combined primary Von Langenbeck palatoplasty with levator sling reconstruction (intravelar veloplasty). The authors observed better speech and superior velopharyngeal function following intravelar veloplasty with muscle reconstruction and recommend careful reconstruction of the levator sling at the time of palate repair.

Palatal lengthening - V-Y pushback

Veau's protocol for closure of cleft palate stressed the need for (1) closure of the nasal layer separately, (2) fracture of the hamular process, (3) staged palatal repair following primary lip and vomer flap closure, and (4) creation of palatal flaps based on a vascular pedicle. Kilner and Wardill devised a technique of palatal repair in 1937 that was more radical then Veau's and that ultimately became the V-Y pushback. It includes lateral relaxing incisions, bilateral flaps based on greater palatine vessels, closure of the nasal mucosa in a separate layer, fracture of the hamulus, separate muscle closure, and V-Y palatal lengthening.

The 4-flap technique is similar to the Wardill-Kilner 2-flap technique, except the oblique incisions are more posterior to create 4 unipedicle flaps. The flaps are again mobilized medially and closed. These pushback techniques achieve greater immediate palatal length but at the cost of creating a larger area of denuded palatal bone anterolaterally. The gain in the length of the palate has not been demonstrated to be permanent or translated to improve velopharyngeal function. This approach has been associated with a higher incidence of fistula formation.

Intravelar veloplasty

Several studies have emphasized the necessity of realignment of the muscle in the soft palate. The stratagem was designed to lengthen the palate as well as to restore the muscular sling of the levator veli palatini. Improved velopharyngeal function was sporadically reported. Marsh et al conducted a prospective study of the effectiveness of primary intravelar veloplasty and found no significant improvement in velopharyngeal function.

Double-opposing Z-plasties

In 1986, Furlow described a single-stage palatal closure technique consisting of double opposing Z-plasties from the oral and nasal surfaces. ^{[12, 13]} Use of the double Z-plasty minimized the need for lateral relaxing incisions to accomplish closure. The palate was also lengthened as a consequence of the new position of the velar and pharyngeal tissues. Preliminary data revealed that speech development was excellent, with 86% exhibiting normal speech in Furlow's study. See the image below.

Others have confirmed the improvement in speech development. The closure of the hard palate in Furlow's technique avoids the use of lateral relaxing incisions. The mucoperiosteal flaps are mobilized from the bony hard palate and the palatal defect closed by tenting the flaps across and creating a moderate empty space between the flaps and the bony hard palatal vault. Furlow's technique appears to be quite successful in clefts of limited size. In moderate-size clefts, lateral relaxing incisions may still be required to obtain closure.

Two-flap palatoplasty

Bardach ^[14] and Salyer independently modified the two-flap palatoplasty to combine elements of other operations with some innovative details. The main goals are complete closure of the entire cleft without tension at an early age (< 2 mo), with minimal exposure of raw bony surfaces and the creation of a functioning soft palate. The authors believe that a muscle sling within the soft palate, not velar lengthening, is essential to adequate speech. Morris and colleagues note that 80% of patients treated with this method developed velopharyngeal function within normal limits, although 51% required speech therapy before normal speech production could be expected. See the images below.

In a further modification of the two-flap palatoplasty, the mucoperiosteal flaps are rotated medially. In a study by Black and Gampper, patients who underwent surgery with this technique demonstrated comparatively low rates of velopharyngeal insufficiency and oronasal fistula development (5.7% and 8.6%, respectively). ^[15]

Velar closure - Delayed hard palate closure

Schweckendiek closed the soft palate early (at age 6-8 mo) but left the hard palate open, albeit occluded with a prosthetic plate, until aged 12-15 years. ^[16] In unilateral clefts the soft palate is closed first, followed by lip surgery 3 weeks later. In bilateral clefts one side of the lip is closed first in conjunction with primary veloplasty, with repair of the other side of the lip and the alveolar cleft 3 weeks later. Schweckendiek reports normal jaw development subsequent to this protocol. ^[16] Many European surgeons now use Perko's approach of 2-stage palatal closure. ^[17] Repair of the soft palate occurs at age 18 months and of the hard palate at 5-8 years. Perko found that the remaining cleft in the hard palate does not disturb speech development to a relevant degree.

Several long-term assessments of patients who undergo the Schweckendiek approach or the Zurich approach (as described by Perko) disclosed an unusually high incidence of short palate and poor mobility of the soft palate, with a correspondingly high degree of velopharyngeal insufficiency (VPI). Conversely, facial growth was judged to be quite acceptable in most patients.

Postoperative management

Despite the difference in surgical technique, a general postoperative routine exists. After surgical repair, the child is given either liquids or nothing by mouth until the next day. If not given liquids, hydration is maintained with intravenous fluid. Oximetry is continuously monitored over 24 hours. Pacifiers and toys with sharp edges are avoided. Patients can usually be discharged the day after the operation. The liquid diet is continued for 2-3 days with a soft diet to follow.

The complications of great concern in the immediate postoperative period are bleeding and respiratory distress, ^{[18, 19]} yet the true incidence of these complications is difficult to determine from a review of the literature. Reports of surgical experiences with cleft lip/palate typically mix children and adults, type of cleft, repair technique, timing of the surgery, or sequence of operations.

Some reports suggest that the Wardill-Kilner repair results in greater morbidity than other methods. This technique typically involves increased postoperative bleeding following division of the anterior branch of the greater palatine artery. Epinephrine is routinely injected prior to the incision to allow better visibility and easier control of bleeding. Hemostatic agents can also be used to pack denuded areas of the palate to minimize the amount of bleeding.

Respiratory compromise secondary to obstruction from the palate lengthening or sedation can be life threatening. Airway obstruction was considerably more common after a von Langenbeck procedure with pharyngeal flap.

Other complications, such as wound dehiscence and oronasal fistula, can be difficult to manage. Dehiscence of the palatal closure, as with wound closure in other parts of the body, is usually a result of poor tissue quality and excessive wound tension. The incidence of dehiscence is low, but the incidence of oronasal fistula has been reported as 5-29%.

A retrospective study by Kaye and Che indicated that in patients who undergo primary cleft palate repair, the risk and amount of postoperative weight loss are significantly greater and the weight-recovery rate is slower than in patients in whom primary cleft lip repair is performed. For example, primary cleft palate repair patients had a maximum percentage body weight loss of 9.2%, compared with 6.11% in patients who underwent primary cleft lip repair. Moreover, it took a median 25.37 days and 14.08 days, respectively, for patients in the two groups to regain their preoperative weight. In addition, the investigators found that slower weight recovery in primary cleft palate repair was associated with a 22.5% rate of unintentional fistula/partial dehiscence, while in those who achieved quick palate recovery, the rate was 10.0%. ^[20]

Potential long-term sequelae are discussed below.

Palatal fistula

Fistula as a result of dehiscence of the initial cleft palate repair can be difficult problem. A fistula of sufficient size can lead to significant problems, ranging from oral fluid and food regurgitation into the nasal chamber to speech difficulties secondary to nasal air emission. Factors that may contribute to fistula formation are the type of cleft, type of repair, wound tension, single-layer repair, dead space deep to the mucoperiosteal flap, and, occasionally, unmasking of a nonfunctional fistula with transverse maxillary arch expansion.

Repair requires re-elevation of the mucoperiosteal flaps with the goal of a two-layer closure (a nasal layer and an oral layer). However, the incidence of recurrence after initial fistula closure is high. Faced with recurrence, the surgeon's options extend to pharyngeal flaps, facial artery myomucosal flaps (FAMM), and tongue flaps. When speech disturbance occurs as a result of a fistula of significant size, prosthetic obturation of the fistula (even temporary) can be considered when weighed against repeated failed surgical procedures.

Velopharyngeal incompetence

Morris, in his review of the literature, reported an incidence of velopharyngeal competence of 75%, as defined by the absence of consistent evidence of VPI. No differentiation was made on the type of cleft or the technique of repair. Peterson-Falzone reported 83.4% competence based on the same criteria. ^[21] However, when using the criterion of no nasal emission or hypernasality, the incidence of velopharyngeal competence decreases to 60%.

The analysis of velopharyngeal competence after various techniques is difficult to interpret in the different studies. The anatomy of the cleft has a great degree of variability that is usually not controlled.

A study by Jones et al indicated that in patients with cleft palate, pharyngeal flap repair for velopharyngeal insufficiency does not significantly affect early facial growth. The study, which included 72 patients, compared outcomes of the surgery to results from pharyngoplasty/palatal lengthening or no surgery and found that 12 of 13 craniofacial measures, including maxillary height and projection, did not significantly differ between the groups. Patients underwent surgery at a mean age of 5 years and were evaluated by cephalogram at a mean age of 8 years. ^[22]

The reader is referred to the authors' article on Craniofacial, Pharyngoplasty and Pharyngeal Flaps for further reading.

Growth and morphology

The severity and laterality of the clefts as well as the choice of cephalometric measurements used in the assessment account for much of the variability in the reported effects of clefting in facial growth. Grayson et al studied the net effect of palatal clefts on the facial skeleton as viewed by lateral cephalogram and determined by mean tensor analysis. The authors note reduced facial bone growth in all directions but principally in the horizontal dimension. The effect was most pronounced at the level of the palate and slightly less so in height of the mid face. Vertical facial growth was most restricted in subjects who had clefts of the primary and secondary palate compared with those who had clefts of the secondary palate alone.

Graber was the first to document disturbance of facial growth as a result of palatal surgery. Multiple studies have demonstrated a casual relationship between increased lip pressure from a repaired cleft lip, periosteal denuding and reduced blood flow in the palatine artery during mucoperiosteal flap elevation and the collapse of the dental arch contraction of the arch, and hypoplasia of the maxilla. Even pharyngeal flap surgery was shown to decrease the width and length of the maxillary arch in cleft palate surgery.

The management of a patient with cleft palate is complex. No current universal agreement exists on the appropriate treatment strategy. Several main points should be emphasized. Normal speech should be the most important consideration in the therapeutic plan. Growth disturbance should be minimized but not at the expense of speech impairment because facial distortion can be satisfactorily managed with future surgery, whereas speech impairment can often be irreversible.

The authors believe, as do many others, that repair of cleft palate to establish a competent velopharyngeal sphincter should be completed at age 6-12 months. A study in which Follmar et al examined a large series of palate repairs supports this assertion. The 18 patients in the study who underwent repair after age 18 months had a significantly higher rate of velopharyngeal insufficiency development than did the 183 patients who underwent surgery before 18 months (33% vs 13%, respectively). ^[23]

Surgical interventions should be designed to cause minimal disruption of the palate to decrease the severity of subsequent growth problems, and patients should be managed in a center with a multidisciplinary team.

Improvements on current treatments are always being sought. However, even with continued advances, cleft palate remains a significant challenge for plastic surgeons.