Cleft Palate Repair 

Updated: Jun 05, 2020
Author: Pravin K Patel, MD; Chief Editor: Jorge I de la Torre, MD, FACS 

Overview

Practice Essentials

The presence of cleft palate has both aesthetic and functional implications for patients in their social interactions, particularly on their ability to communicate effectively and on their facial appearance with or without involvement of the lip. Midfacial skeletal growth may be affected by the surgical repair of the palate. The treatment plan focuses on two areas: speech development and facial growth. Speech development is paramount in the appropriate management of cleft palate. Many surgical techniques and modifications have been advocated to improve functional outcome and aesthetic results. The most controversial issues in the management of cleft palate are the timing of surgical intervention, speech development after various surgical procedures, and the effects of surgery on facial growth. The major goals of surgical intervention are normal speech, minimizing growth disturbances, and establishing a competent velopharyngeal sphincter.[1]

Management of cleft lip and palate

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.

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 three 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.

History of the Procedure

Rogers and Georgiade expertly reviewed the evolution of cleft palate surgery.[2, 3] The first record of a palatal operation dates to 500 AD and was prompted by inflammation of the uvula. In 1552, Houlier proposed suturing palatal clefts and 12 years later Ambroise Pare illustrated obturators for palatal perforations. In 1764, Le Monnier, a French dentist, successfully repaired a cleft velum with a few sutures and hot cautery of the edges. von Graefe, 50 years later, produced inflammation of the velar margins before bringing them together in his palate suture and is credited with performing the first velar repair of a cleft in 1816. JC Warren performed the first velar closure in America in 1824.

In 1828, Dieffenbach enhanced the surgical treatment of cleft palate by introducing hard palatal mucosa elevation to allow the closure of hard palatal cleft. von Langenbeck (1859) proposed the creation of a bipedicle mucoperiosteal flap that can be mobilized medially to close the palatal cleft. The improved vascular supply of the mucoperiosteal flap significantly decreased the incidence of dehiscence. See the image below.

The von Langenbeck repair. Two bipedicle mucoperio The von Langenbeck repair. Two bipedicle mucoperiosteal flaps are 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 are 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.

With the ability to successfully close the palate, concern about palatal function was raised. It was evident by this time that the short and immobile palate impaired the speech capability of patients with cleft palate. Veau,[4] Kilner,[5] and Wardill[6] described the unipedicled mucoperiosteal flap based posteriorly on the greater palatine artery that pushed the flap posteriorly to lengthen the palate. The scarring of the denuded bone areas anteriorly and laterally was suspected as the cause of facial growth retardation posteriorly.

In 1994, Schweckendiek advocated the use of a 2-stage cleft palate closure. The soft palate was closed early, with closure of the hard palate delayed until several years later. The rationale for the 2-stage procedure was to provide improved velopharyngeal function during the initial speech development and to accomplish the closure of the hard palate after the cleft narrows with facial growth. Anatomic muscle realignment has also been postulated as essential in improving postoperative velopharyngeal function.

A topic for debate has been over the treatment of the alveolar cleft that accompanies the cleft palate. The rationale for its closure includes stabilizing the maxillary arch, providing support for tooth eruption and postsurgical orthodontics, closing oronasal fistulae, and improving the aesthetics of the mid face and nose. The current trend is toward secondary bone grafting at the time of mixed dentitia, with early (primary) grafting potentially proving detrimental to midfacial growth.

Much discussion has centered over the role and timing of presurgical appliances. Both the hard palate and the alveolus can be molded with passive molds and active devices, with the shared ultimate goals of facilitating surgical repair and providing an improved long-term outcome in both facial form and palatal function.

These historic developments in the treatment of the cleft palate underlie the existing controversies still found today.

Problem

Classification

Numerous classifications have been suggested over the years. Kernahan's classification system is the most common one used. This "striped Y" classification has been almost universally adopted for its simplicity and usefulness. See the image below.

Millard modification of Kernahan striped-Y classif Millard modification of Kernahan striped-Y classification for cleft lip and palate. The small circle indicates the incisive foramen; the triangles indicate the nasal tip and nasal floor.

Embryology

The embryogenesis of the palate can be divided into two separate phases: the formation of the primary palate followed by the formation of the secondary palate. Palatal development begins at approximately day 35 of gestation with the emergence of facial processes. The fusion of the medial nasal process (MNP) with the maxillary process (MxP), followed by the lateral nasal process (LNP) with the MNP, completes the formation of the primary palate. Failure of fusion or breakdown of fusion of the processes results in a cleft of the primary palate. The genesis of the secondary palate begins at the completion of the primary palate formation. The secondary palate arises from the bilateral shelves that develop from the medial aspect of the MxP. The two shelves meet in the midline with the elevation of the shelves. As the shelves move superiorly, the fusion process begins. Interference in the fusion leads to clefting of the secondary palate.

Epidemiology

Frequency

The incidence of cleft lip/palate by race is 2.1/1000 in Asians, 1/1000 in whites, and 0.41/1000 in blacks. Isolated cleft palate shows a relatively constant ratio of 0.45-0.5/1000 births. The foremost type of clefting is a bifid uvula, occurring in 2% of the population. The second most frequent type is a left unilateral complete cleft of the palate and prepalatal structures. Midline clefts of the soft palate and parts of the hard palate are also common. Complete clefts of the secondary palate are twice as common in females as in males while the reverse is true of velar clefts. About 7-13% of patients with isolated cleft lip and 11-14% of patients with cleft lip/palate have other anomalies at birth. A comprehensive review of syndromes associated with cleft lip/palate is beyond the objectives of this article. For more information, see Medscape Reference article Reconstructive Surgery for Cleft Palate.

Recurrence risks for clefting deformities do not correspond to any Mendelian pattern of inheritance and it would appear that clefting is inherited heterogeneously. This observation is supported by evidence from studies of twins that indicate the relative roles of genetic and nongenetic influences of cleft development. For isolated cleft palate and combined cleft lip and palate, if the proband has no other affected first- or second-degree relatives, the empiric risk of a sibling being born with a similar malformation is 3-5%. However, if a proband with a combined cleft lip and palate has other affected first-degree relatives, the risk for siblings or subsequent offspring is 10-20%.

Etiology

In 25% of patients, there is a family history of facial clefting, which does not follow either a normal recessive or dominant pattern. The condition appears to be multifactorial. Some instances of clefting may be because of an overall reduction in the volume of the facial mesenchyme, which leads to clefting by virtue of failure of mesodermal penetration. In some patients, clefting appears to be associated with increased facial width, either alone or in association with encephalocele, idiopathic hypertelorism, or the presence of a teratoma. The characteristic U-shaped cleft of the Pierre Robin anomaly is thought to be dependent upon a persistent high position of the tongue, perhaps associated with a failure or delay of neck extension. This prevents descent of the tongue, which in turn prevents elevation and a medial growth of the palatal shelves.

The production of clefts of the secondary palate in experimental animals has frequently been accomplished by drug administration. Agents commonly used are steroids, anticonvulsants, diazepam, and aminopterin. Phenytoin and diazepam may also be causative factors in clefting in humans. Infections during the first trimester of pregnancy, such as rubella or toxoplasmosis, have been associated with clefting.

An international study (Democratic Republic of the Congo, the Philippines, Vietnam, and Honduras) by Figueiredo et al indicated that parental risk factors for oral clefts include older maternal age, pregestational hypertension, gestational seizures, a lower maternal and paternal education level, and paternal tobacco smoking, as well as a family history of oral clefts.[7]

Pathophysiology

The pathologic sequelae of cleft palate include feeding and nutritional difficulties, recurrent ear infections, hearing loss, abnormal speech development, and facial growth distortion. The communication between the oral and nasal chamber impairs the normal sucking and swallowing mechanism of the cleft infants. Food particles reflux into the nasal chamber.

The abnormal insertion of the tensor veli palati prevents satisfactory emptying of the middle ear. Recurrent ear infections have been implicated in the hearing loss of patients with cleft palate. The hearing loss may worsen the speech pathology in these patients. Evidence that repair of the cleft palate decreases the incidence of middle ear effusions is inconsistent. However, these problems are overshadowed by the magnitude of the speech and facial growth problems.

Speech abnormalities are intrinsic to the anatomic derangement of cleft palate. The facial growth distortion appears to be, to a great extent, secondary to surgical interventions. Along with an intact hard palate, an intact velopharyngeal mechanism is essential in production of high pressure consonants and the oral resonance of vowels. The velopharyngeal mechanism must also remain open to some degree to accomplish nasal resonance of m, n, and ng. With connected spontaneous speech, oral and nasal speech contexts are rapidly coarticulated, resulting in the complex need for millisecond timing of velopharyngeal closure and opening, depending on the speech targets.[8]

Experimentation with this velopharyngeal activity begins with babbling and continues through the early language learning years. Without sufficient velopharyngeal function during this early learning period, compensatory strategies may be adopted, with consonants produced in the pharynx to create pressure buildup. These compensatory strategies are difficult to change, and always require speech therapy intervention, often with the need for additional surgical reconstruction.

Multiple studies have demonstrated that the cleft palate maxilla has some intrinsic deficiency of growth potential. This intrinsic growth potential varies from isolated cleft of the palate to complete cleft lip and palate. This growth potential is further impaired by surgical repair. Any surgical intervention performed prior to completion of full facial growth can have significant deleterious effects on maxillary growth. Disagreement exists as to the appropriate timing of surgery to minimize the harmful effects on facial growth and on what type of surgical intervention is most responsible for growth impairment. The formation of scar and scar contracture in the areas of denuded palatal bones are most frequently blamed for restriction of maxillary expansion.

The growth disturbance is exhibited most prominently in the prognathic appearance during the second decade of life despite the normal appearance in early childhood. The discrepant occlusion relationship between the maxilla and the mandible is usually not amenable to nonsurgical correction.

Presentation

The following 4 points should be emphasized in the presentation of an infant with cleft lip/palate.

Feeding

Although a child with cleft palate may make sucking movements with the mouth, the cleft prevents the child from developing normal suction. However, in general, swallowing mechanisms are normal. Therefore, if milk or formula can be delivered to the back of the child's throat, the infant feeds effectively. Breastfeeding is usually not successful, unless milk production is abundant, but most infants can bottle feed with more specially designed bottles and nipples.

Airway

The infant with Pierre Robin anomaly or other conditions in which the cleft palate is observed in association with a micrognathia or retrognathic mandible may be particularly prone to upper airway obstruction.

Middle ear disease

The disturbance in anatomy associated with cleft palate affects the function of the eustachian tube orifices. Parents and physicians should be aware of the increased possibility of middle ear infection so that the child receives treatment promptly if symptoms arise.

Associated deformities

The surgeon must always keep in mind that in as many as 29% of patients, the child with cleft palate may have other anomalies. These may be more commonly associated with isolated cleft palate than with cleft lip/palate. High among the associated anomalies are those affecting the circulatory and skeletal systems.

Indications

Children born with a cleft palate should undergo surgical repair unless otherwise contraindicated. The main goal is to perform a functional repair of the soft palate musculature through the repositioning of the abnormally-oriented and attached muscles. This anatomic repair attempts to facilitate the development of normal speech. While separation of the oral and nasal cavities is advantageous to normalize feeding and decrease regurgitation and nasal irritation, it is not absolutely necessary for feeding.

Palate repair with repositioning of the palatal musculature may be advantageous to eustachian tube function and ultimately to hearing. Because the levator and the tensor veli palatini have their origins along the eustachian tube, repositioning improves function of these muscles, improves ventilation of the middle ear, and decreases serous otitis, which further decreases the incidence of hearing abnormality. Palate repair alone does not usually completely correct this dysfunction and additional therapy frequently includes placement of ear tubes as necessary.

Relevant Anatomy

The bony portion of the palate is a symmetric structure with division based on the embryonic origin into the primary and secondary palate. The premaxilla, alveolus, and lip, which are anterior to the incisive foramen, are parts of the primary palate. Structures posterior to it, which include the paired maxilla, palatine bones, and pterygoid plates, are part of the secondary palate. The severity of the clefting of the bony palate varies from simple notching of the hard palate to clefting of the alveolus. See the image below.

Anatomy of the palate. Anatomy of the palate.

The palatine bone is located posterior to the maxilla and pterygoid lamina. It is composed of horizontal and pyramid processes. The horizontal process contributes to the posterior aspect of the hard palate and becomes the floor of the choana. The pyramidal process extends vertically to contribute to the floor of the orbit.

Even though the bony defect is important in the surgical treatment of cleft palate, the pathology in the muscles and soft tissues has the greatest impact on the functional result. Six muscles have attachment to the palate: levator veli palatini, superior constrictor pharyngeus, uvulus, palatopharyngeus, palatoglossus, and tensor veli palatini. The 3 muscles that appear to have the greatest contribution to the velopharyngeal function are the uvulae, levator veli palatini, and superior constrictor pharyngeus. The uvulae muscle acts by increasing the bulk of the velum during muscular contraction. The levator veli palatini pulls the velum superiorly and posteriorly to appose the velum against the posterior pharyngeal wall. The medial movement of the pharyngeal wall, attributed to superior constrictor pharyngeus, aids in the opposition of the velum against the posterior pharyngeal wall to form the competent sphincter. The palatopharyngeus displace the palate downwards and medially.

The palatoglossus is mainly a palatal depressor that plays a role in the production of phonemes with nasal coupling by allowing controlled airflow into the nasal chamber. The tensor veli palatini does not contribute to the movement of the velum. The tensor veli palatini's tendons hook around the hamulus of the pterygoid plates and the aponeurosis of the muscle inserts along the posterior border of the hard palate. The muscle originates partially on the cartilaginous border of the auditory tubes. The function of the tensor veli palatini, similar to tensor tympani with which it shares similar innervation, is to improve the ventilation and drainage of the auditory tubes.

In cleft palate, the aponeurosis of the tensor veli palatini, instead of attaching along the posterior border of the hard palate, is attached along the bony cleft edges. All the muscles that attach to the palate insert onto the aponeurosis of this muscle. Thus, the overall length of the palate is shortened. The abnormality in the tensor veli palatini increases the incidence of middle ear effusion and middle ear infection.

The muscle sling of the levator veli palatini is also interrupted by cleft palate. The levator does not form the complete sling. The medial portion of each side attaches to the medial edge of the hard palate. Thus, in patients with cleft palate, the effectiveness of the velar pull against the posterior pharyngeal wall is impaired. Of the 6 muscles, the prevailing theory attributes most of the contribution to the velopharyngeal competence to the levator veli palatini.

Contraindications

No absolute contraindications exist for the repair of cleft palate.

Relative contraindications include current illness or other medical condition that can interfere with general anesthesia, possible compromise of the airway in a child with a preexisting airway problem (such as severe micrognathia), severe developmental delay, or a short life expectancy because of other severe illnesses.

 

Workup

Laboratory Studies

See the list below:

  • Routine laboratory studies are noncontributory in otherwise healthy infants with cleft palate. Some centers obtain a blood count as a routine study before performing surgery on a child with cleft palate. The authors do not find this necessary unless some other associated medical conditions coexist.

Imaging Studies

See the list below:

  • Routine imaging studies are noncontributory in otherwise healthy infants who undergo primary cleft palate repair.

Diagnostic Procedures

See the list below:

  • Early collaboration with an audiologist and an otolaryngologist, including examination and early audiologic assessment, prevents long-term hearing deficits.

 

Treatment

Medical Therapy

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.

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.

Surgical Therapy

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.

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.[9, 10] 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.

Double-opposing Z-plasties. Furlow's single-stage Double-opposing Z-plasties. Furlow's single-stage palatal closure technique consisting of double opposing Z-plasties from the oral and nasal surfaces. The double Z-plasty minimizes the need for lateral relaxing incisions to accomplish closure. The palate is lengthened as a consequence of the new position of the velar and pharyngeal tissues.

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[11] 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.

Two flap palatoplasty. After lateral relaxing inci Two flap palatoplasty. After lateral relaxing incisions are performed, bilateral flaps are elevated based on greater palatine vessels.
Two-flap palatoplasty (continued). Closure of the Two-flap palatoplasty (continued). Closure of the nasal mucosa is performed. The hamulus may be fractured, the muscle is repaired, and the oral mucosa is closed as a separate layer.

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).[12]

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.[13] 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.[13] Many European surgeons now use Perko's approach of 2-stage palatal closure.[14] 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.

Follow-up

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.

Complications

The complications of great concern in the immediate postoperative period are bleeding and respiratory distress,[15, 16] 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%.[17]

Outcome and Prognosis

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.[18] 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.[19]

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.

Future and Controversies

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).[20]

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.

 

Guidelines

Guidelines Summary

Treatment for patients who are born with facial clefts is ideally accomplished through an interdisciplinary staff of professionals at a dedicated cleft/craniofacial center. The American Cleft Palate-Craniofacial Association has provided guidelines for such teams to help ensure the coordination and consistent administration of care, with evaluations and treatments properly sequenced in view of a patient’s developmental, medical, and psychological needs. Those guidelines are as follows[21] :

  • A designated patient coordinator on the team should facilitate its function and efficiency, as well as the coordination of care and treatment-plan assistance for patients and families/caregivers
  • The minimum core of the team must include speech-language pathology, surgery, and orthodontics professionals
  • The team must have access to psychology, social work, audiology, genetics, general and pediatric dentistry, otolaryngology, and pediatrics/primary care professionals
  • A surgeon trained in transcranial cranio-maxillofacial surgery must be part of the team, which must also have access to a psychologist involved in neurodevelopmental and cognitive assessment; access for referral to a neurosurgeon, an ophthalmologist, a radiologist, and a geneticist is also necessary
  • The team must provide the patient and family/caregiver with oral and written information about evaluation and treatment procedures
  • The patient and family/caregiver should be encouraged by the team to participate in the treatment process
  • Families/caregivers should receive team assistance in locating resources for financial help necessary to meet patient needs
  • Team care must be sensitive and flexible with regard to linguistic, cultural, and ethnic diversity among patients and their families/caregivers
  • The team must include or have available for referral social workers and psychologists who can see to the psychological and social needs of the patient and family/caregiver
  • The team must have a cognitive development evaluation mechanism
  • When necessary, cognitive psychometric testing must be administered to patients aged 4 or older whose craniofacial condition requires transcranial surgery
  • The team should self-assess its performance and make improvements accordingly