CONGENITAL NONUNIONS, POSTOPERATIVE DEFECTS AND PALATE DEFORMITIES: ETIOLOGY, CLINICAL FEATURES, SURGICAL TREATMENTS.

Overview

This material primarily reviews cleft lip and palate (CLP) and issues directly related to these anomalies, including secondary deformities and velopharyngeal insufficiency (VPI). Cleft lip and palate deformity can be distinguished from an isolated cleft palate (CP) on the basis of epidemiologic, embryonic, and genetic factors. The etiology of cleft lip and palate or cleft palate is believed to be multifactorial.

The scope of this field precludes in-depth discussion of surgical techniques and controversies. Miscellaneous deformities, such as the Robin sequence, macroglossia, ankyloglossia, and epignathus are briefly highlighted.

The image below depicts embryonic formation of the primary palate.

Illustration depicts fusion of the lateral nasal, medial nasal, and maxillary prominences to form the primary palate.

Two thirds of all cases of clefting involve the lip with or without involvement of the palate, whereas one third of all cases occur as an isolated deformity of the palate. Males predominate within the cleft lip and palate (CLP) group (60-80% of cases), whereas females constitute the majority within the cleft palate (CP) group. Cleft lip and palate deformity is strongly associated with bilateral cleft lips (CLs) (86% of cases); the association decreases to 68% with unilateral cleft lip. The left side is most commonly involved in unilateral cleft lip cases.

Interracial differences exist in the incidence of cleft lip and palate versus cleft palate. The mean incidence of cleft lip and palate is 2.1 cases per 1000 live births among Asians, 1 case per 1000 live births among white people, and 0.41 cases per 1000 live births among black people. A high incidence of the cleft lip and palate is seen in North American populations of Asian descent, such as Indians of the southwestern United States and the west coast of Canada. The incidence of isolated cleft palate is constant among the 3 racial groups at 0.5 cases per 1000 live births.

The incidence of cleft lip and palate also rises with increased parental age, and older mothers with additional parity have an increased incidence of having children with cleft palate.

In relatives of children with cleft lip and palate, the incidence of cleft lip and palate is significantly increased. However, the isolated cleft palate anomaly occurs with the same frequency as that in the general population. Relatives of children with isolated cleft palate also have a higher risk of this anomaly, without an increased risk of the cleft lip and palate deformity.

Overall, 5% of patients with cleft lip and palate and isolated cleft palate have identifiable syndromes. Associated syndromes are more common among patients with isolated cleft palate than among others.

The following image depicts the normal palate anatomy.

Normal anatomy of the palate.

Embryology of Cleft Lip and Palate

The overall development of the palate involves the formation of the primary palate followed by the formation of the secondary palate.At approximately 30-37 days’ gestational age (GA), the primary palate forms by the growth and fusion of the medial nasal, lateral nasal, and maxillary processes (see the image below). The maxillary process, derived from the proximal half of the first arch, grows to meet and fuse with the nasal processes that have grown and moved in association with the olfactory placode. General opinion holds that mesodermal penetration underlies the formation of the primary palate. Mesodermal reinforcement along lines of fusion is important, as epithelial breakdown and clefting is thought to result from the lack of reinforcement.

The secondary palate arises from the 2 palatal shelves, which are initially are in a vertical position because of the interposed tongue. With extension of the head at 7 weeks’ GA and mandibular growth, the tongue is withdrawn, and the palatal shelves can swing into a more horizontal and midline position for fusion and formation of a hard and soft palate (see the following image). The cleft of the hard palate and soft palate is thought to occur because of the intervening tongue, which impedes elevation of the palatal shelves.

Formation of the secondary palate.

Classification of Cleft Lip and Palate

Various classification schemes have been devised in the last 70 years for cleft lip and palate, but few have received widespread clinical acceptance. Four of the more accepted schemes are highlighted below.

Davis and Ritchie classification

The Davis and Ritchie classification divides cleft lip and palate into 2 groups, which subdivided into the extent of the cleft (eg, 1/3, 1/2), as follows:

• Group I – Clefts anterior to the alveolus (unilateral, median, or bilateral cleft lip)
• Group II – Postalveolar clefts (cleft palate alone, soft palate alone, soft palate and hard palate, or submucous cleft)

Veau classification

The Veau classification system divides the cleft lip and palate into 4 groups, which are as follows and illustrated in the image below:

• Group I – Defects of the soft palate only
• Group II – Defects involving the hard palate and soft palate
• Group III – Defects involving the soft palate to the alveolus, usually involving the lip
• Group IV – Complete bilateral clefts

• Veau classification of cleft lip and palate. A: Group I. Defects of the soft palate only. B: Group II. Defects involving the hard palate and soft palate. C: Group III. Defects involving the soft palate to the alveolus, usually involving the lip. D: Group IV. Complete bilateral clefts.

Kernahan and Stark symbolic classification

The Kernahan and Stark classification highlights the anatomic and embryonic importance of the incisive foramen that is formed during weeks 4-7 gestational age (GA). The secondary palate forms the roof of the mouth from the incisive foramen to the uvula during weeks 7-12 GA (see the image below).

This system provides a graphic classification scheme using a Y-configuration, which can be divided into 9 areas, as follows (see also the image below)^[2] :

• Areas 1 and 4 – Lip
• Areas 2 and 5 – Alveolus
• Areas 3 and 6 – Palate between the alveolus and the incisive foramen
• Areas 7 and 8 – Hard palate
• Area 9 – Soft palate

• Kernahan and Stark symbolic classification of cleft lip and palate. R = right; L = left.

International Confederation of Plastic and Reconstructive Surgery classification

The International Confederation of Plastic and Reconstructive Surgery classification system uses an embryonic framework to divide clefts into 3 groups, with further subdivisions to denote unilateral or bilateral cases, as follows:

• Group I – Defects of the lip or alveolus
• Group II – Clefts of the secondary palate (hard palate, soft palate, or both)
• Group III – Any combination of clefts involving the primary and secondary palates

Koul introduced a method for documenting all types of cleft lip and cleft palate for data storage and communication.^[3] This “Expression System” incorporates the actual words for the anatomical structures affected by clefts and can describe accurately and easily, without the need for consulting reference materials, the location and extent of both typical and atypical clefts. The Expression System overcomes several limitations of previous cleft registration methods, and its simplicity and precision benefits all those involved in the care of patients with cleft lip/palate by furthering the interdisciplinary and intradisciplinary approach.

Functional Anatomy of Cleft Lip Palate

Comprehension of the anatomic deformities is central to understanding the principles of their surgical repair. The following section briefly describes the anatomic abnormalities in the patient with cleft lip and palate (CLP) by discussing the muscular, neurovascular, structural, and nasal deformities.

Failure of the muscles to meet their counterparts during embryonic development leads to the functional abnormalities of clefts of the lip and palate.^[4] The nonfunctional substitute attachments lead to atrophy of the muscle units or maladaptive accommodation. Modern cleft lip and palate surgical repair involves detachment of musculature from atypical locations and realignment in a more anatomically functional position.^[4]

Cleft lip

The orbicularis oris muscle is the primary muscle of the lip and can be divided functionally and anatomically into 2 parts (see the image below).^[4] The deep component, in concert with other oropharyngeal muscles, works in swallowing and serves as a sphincter. The superficial component is a muscle of facial expression and inserts into the anterior nasal spine, sill, alar base, and skin to form the philtral ridges.

Muscular defects in unilateral deformity.

In a complete cleft lip (CL), the deep fibers of the orbicularis oris muscle are interrupted by the cleft and end on either side of the defect instead of making their way around the mouth. In addition, the superficial component of the orbicularis oris turns upward, along the margins of the cleft and ends beneath the ala or columella.^[4]

Incomplete cleft lip behaves in a similar manner, except when the cleft is less than two thirds of the height of the lip.^[4] In this case, the fibers of the muscle run along the margins of the cleft, then change direction and run horizontally over the top of the cleft. These muscle fibers are interspersed with connective tissue.

The blood vessels parallel the course of the muscle fibers and run along the margins of the cleft toward the columella or alar base, where they form anastomoses with nearby vessels.

In the bilateral deformity, the anatomic characteristics are determined by the degree of completeness of the cleft and its symmetry. The cleft may involve the primary palate alone or in conjunction with the secondary palate. Although the prolabium varies in size, it is usually retracted and lacks muscle fibers. In addition, the columella is absent and the prolabium appears attached to the top of the nose in some cases. The size and position of the premaxilla vary and effectively can be excluded with a collapse of the alveolar arch.

The extent of nasal deformity associated with cleft lip varies from patient to patient, although it has a characteristic appearance, with the following features:

• Deflection of the nasal tip towards the noncleft side
• Retroplacement of the cleft alar cartilage dome
• Obtuse angle between the medial and lateral crura of the lower lateral cartilage on the cleft side
• Buckling of the ala on the cleft side
• Absence of the alar-facial groove on the cleft side and attachment of the ala to the face at an obtuse angle
• Apparent or real bony deficiency of the maxilla on the cleft side
• Larger nares on the cleft side
• Shorter columella on the cleft side, positioning the entire columella at a slant toward the noncleft side
• Inferior displacement of the medial crus within the columella
• Dislocation of the caudal portion of the septum to the noncleft side from the nasal spine
• Downward rotation of the alar cartilage on the cleft side
• Bilateral deformity in which the nasal tip appears large, flat, and bifid, because both alae are rotated downward and spread apart

Cleft palate

The incisive foramen is the key landmark in the bony palate (see the image below). The premaxilla lies anterior to the incisive foramen and includes the 2 premaxillary bones: the alveolus and the incisors.

The soft-tissue structures in the primary palate include the nasal tip and the upper central lip. The size, composition, and configuration of the premaxilla can vary from full development with the complement of teeth (4 primary and 4 secondary) to underdevelopment with only 2 incisors. If the premaxilla is unrestrained in the intrauterine and neonatal period it can protrude from the arch; the maxillary arches may then collapse and potentially exclude the premaxilla from the arch.

Posterior to the incisive foramen lies the secondary palate, comprising the hard palate and soft palate. The hard palate forms from the palatine processes of the maxilla anteriorly and the palatine bones posteriorly. Posterior to the bony hard palate lies the soft palate.

The soft palate plays an important role in speech and swallowing. Paired muscle on both sides of the midline (see the following image) form the musculature of the soft palate. The levator veli palatini is the most important muscle for the production of speech and velopharyngeal competence. The paired muscles of the soft palate function as a sling from their origin at the undersurface of the temporal bone to their aponeurosis across the midline, as they elevate the soft palate toward the posterior pharyngeal wall.

The palatopharyngeus further supplements the posterior movement of the soft palate. Contraction of the superior pharyngeal constrictor contributes to closure of the velopharyngeal opening at the lateral and posterior pharyngeal wall. The primary function of the tensor veli palatini is to dilate the eustachian tube and to maintain its integrity. The uvular muscle is thought to have a minimal contribution to normal speech.

Muscles of the soft palate.

Clefts of the palate (CPs) are associated with bony, as well as soft-tissue, abnormalities. Clefts of the secondary palate may be isolated or associated with clefts of the primary palate. Although clefts of the secondary palate are midline defects (see the image below), those involving the primary palate are usually asymmetric, with the vomer attached to the noncleft side. The dental arch on the noncleft side usually splays outward due to the lack of restraining force from the lip, and the palate is foreshortened in the anteroposterior direction. In the case of complete bilateral clefts, the entire premaxilla protrudes from the adjacent alveolar ridges. Because of the collapse of the palatine shelves posterior to the premaxilla and its possible rotation, the premaxilla is prevented from rejoining the arch and is left attached solely to the vomer.

Variations of cleft palate.

Soft-tissue defects of the cleft palate include hypoplasia of the velar musculature in addition to anomalous insertions of its muscular components (see the following image). The normal midline insertion and transverse orientation of the levator palatini is substituted by an aberrant longitudinal orientation and insertion along the bony cleft margin and posterior palatine bones. Other palatal muscles are affected similarly. Dysfunction results in speech pathology with velopharyngeal incompetence and in eustachian-tube obstruction with resultant middle-ear effusion, infections, and possible hearing loss.

Underlying defect in the musculature of cleft palate.

Management of Cleft Lip and Palate

Neonatal management, unilateral and bilateral cleft lip repair, presurgical orthodontics, and cleft palate repair are discussed in this section.

Neonatal management

A minority of patients, particularly those with the Robin sequence (see Robin sequence under Selected Mouth and Pharynx Deformities), present with respiratory distress. Securing the airway is the priority in these patients. However, feeding difficulty is the primary problem for most patients with cleft lip and palate (CLP). Although these patients have normal sucking and swallowing reflexes, they have difficulty generating enough negative pressure to nurse adequately. As a result, the baby’s nutrition must be delivered through bottle feeding via nipples with large openings to facilitate the delivery of breast milk or formula.

A multidisciplinary approach is required to assist patients and their families with the comprehensive care of these children. Responsibility for their care is shared by a team of pediatricians, plastic surgeons, otolaryngologists, pedodontists, orthodontists, nurses, speech therapists, audiologists, and social workers.

Unilateral cleft lip repair

Repair of the unilateral cleft lip (CL) is usually performed during the first year of life. Although some surgeons advocate immediate repair, most follow the “rules of 10”: hemoglobin more than 10 g, age older than 10 weeks, and weight more than 10 lb. Patients who satisfy the criteria can better tolerate general anesthesia, and surgeons can perform a more technically accurate surgical repair.

Discussion of the merits of individual surgical procedures for correction of the cleft lip (CL) is beyond the scope of this review. All contemporary procedures use local tissue flaps for reconstruction and closure of the congenital anomaly. Interested readers are urged to refer to surgical atlases or the following:

• Cleft Lip
• Unilateral Cleft Lip Repair

Presurgical orthodontics

Presurgical orthodontics facilitate repositioning of the palatal segments into normal alignment with the use of an appliance.^[5] The simplest device is adhesive tape placed across the cheeks and prolabium of patients with bilateral clefts. Splints can also be used to maintain or adjust the alignment of the premaxilla while the patient awaits definitive cleft lip repair. These appliances have the potential to convert a wide complete cleft lip to an incomplete lip. In addition, preoperative realignment of the segments decreases tension on the wound and incidence of wound dehiscence.

Bilateral cleft lip repair

The bilateral cleft lip deformity is unique, because its management and postoperative results are affected by the status of the premaxillary segment and the degree of symmetry and completeness of the deformity. The goals of surgical correction of a bilateral cleft lip include correction of the cleft lip (CL) and nasal deformity in addition to establishment of a normal relationship between the premaxilla and the alveolar arches. Presurgical orthodontics are used to realign the maxillary arch and premaxilla and to minimize the tension placed on the lip closure.^[5]

El-Kassaby et al (2013) published a retrospective comparative study of nonsyndromic patients with complete bilateral cleft lip and palate.^[6] The patients were divided into 2 groups based on the size and characteristics of the premaxilla: group R for rudimentary premaxilla and group P for prominent premaxilla. The width of the cleft at the hard palate level and the gap between the soft palate and the posterior pharyngeal wall were the most significant parameters in predicting velopharyngeal insufficiency.

For a discussion of surgical procedures used to repair bilateral cleft lip, please refer to plastic surgery texts or the following:

• Cleft Lip
• Bilateral Cleft Lip Repair

Cleft palate repair

The goals of cleft palate (CP) repair include closure of the palatal defect and attainment of normal speech, hearing, dental occlusion, and facial and palatal growth. The timing of surgical correction remains controversial. Factors considered before repair must take into account the known and postulated affects on facial growth and speech development.^{[7, 8]}

The trauma sustained during surgical intervention is thought to play a role in the underdevelopment of the midface. The persistence of a cleft deformity, per se, is not believed to affect normal craniofacial growth. Patients with cleft deformities that are left surgically uncorrected have been observed to have normal maxillary growth.

The development of speech is somewhat independent of craniofacial growth. That vocalization begins with birth is well known. In addition, an intact speech mechanism is required to ensure that the correct neural programming needed for integration of the musculature involved in speech occurs. This process is thought to transpire within the first year of life. Once established, compensatory speech patterns are difficult to change.

Common opinion maintains that although early palatal repair is associated with superior speech and hearing, it has negative effects on facial growth. Operative intervention at a younger age is also technically more challenging because of the small size of the structures and the limitations of the instruments.

Most centers in North America perform palatal closure at age 12-18 months. Patients in this age group have larger anatomy, which facilitates surgical intervention. In addition, common belief asserts that normal speech development is not impeded at this age.

Surgical repair of the cleft palate falls into 2 categories. The first is a single-stage repair involving closure with mucoperiosteal flaps. The second involves a multistage approach in which the soft palate is closed initially, followed by a delayed closure of the hard palate.

In a 2008 retrospective study by Khosla et al concluded that the Furlow Z-plasty yielded excellent speech results for primary cleft palate repair with minimal and acceptable rates of fistula formation, velopharyngeal insufficiency, and the need for additional corrective surgery.

Distraction osteogenesis is a relatively recent technique used for maxillary advancement to correct skeletofacial deformities in older cleft patients. In 2008, Bevillaqua et al published results with this technique on 7 patients with significant anterior movements, which allowed excellent improvements in functional and facial aesthetic outcomes.

Cleft Palate Repair 

Background

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.

History of the Procedure

Rogers and Georgiade expertly reviewed the evolution of cleft palate surgery. 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 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,  Kilner, and Wardill  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 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.

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.

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.

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.

Laboratory Studies

• 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

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

Diagnostic Procedures

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

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 CL/P 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. 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 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  and Salyer independently modified the 2-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 withiormal limits, although 51% required speech therapy before normal speech production could be expected. See the images below.

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 nasal mucosa is performed. The hamulus may be fractured, the muscle is repaired, and the oral mucosa is closed as a separate layer.

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. 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. Many European surgeons now use Perko’s approach of 2-stage palatal closure. 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, 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%.

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. 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. 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. Surgical interventions should be designed to cause minimal disruption of the palate to decrease the severity of subsequent growth problems.

Cleft patients should be managed in a center with a multidisciplinary team. Cleft palate remains a significant challenge for current and future plastic surgeons.

What Is A Cleft Palate?

A cleft palate is a common congenital facial anomaly treated by plastic surgeons. A cleft palate is identified by a gap in the roof of the mouth.  A cleft palate develops in a fetus when the two halves of the palate do not come together and fuse in the middle. In most cases, a cleft lip is also present. Cleft palate causes problems with dental development (see photo), speech, hearing, eating, and drinking. A child may also experience frequent colds, fluid in the ears, sore throat, and problems with the tonsils and adenoids. A cleft palate is different from a cleft lip. A cleft lip affects the upper lip, whereas a cleft palate affects the roof of the mouth. Not all individuals with cleft palate have a cleft lip, and not all individuals with a cleft palate have a cleft lip. It is possible for an individual to have both a cleft lip and a cleft palate. In this article, you will learn what a normal palate looks like. You will also learn about the different types of cleft palates –- complete versus incomplete. Complete indicates that the cleft in the palate involves the entire length of the palate. An incomplete palate involves only the back part of the palate. A cleft palate can also be unilateral or bilateral. Unilateral means the palate has a cleft on one side. Bilateral means there is a cleft on both sides of the palate.

Normal Palate Anatomy

Understanding what a normal palate looks like will help you better understand the anatomy of cleft palate. Mucosa: The mucosa is moist, pink tissue that lines the inside of certain body parts. It lines the nose, mouth, lungs, and the urinary and digestive tracts.  Hard Palate: The hard palate is the bony part of the roof of the mouth. It makes up the front part of the palate. It is in front of the soft palate. You don’t see the bone when you open your mouth because it is covered by the mucosa. With either your tongue or your finger, you can feel when the palate changes from hard to soft.  The hard palate separates the mouth from the nose. Without the hard palate, there is communication between the nasal cavity and the oral cavity. This communication between the two makes speech, eating and drinking difficult. The hard palate keeps food from going up the nose. The hard palate is also important for speaking as it keeps air from going out of the nose instead of the mouth. Soft Palate: The soft palate is the posterior fleshy part of the palate. If you run your tongue from the front of the roof of your mouth to the back, you can feel when the hard palate becomes the soft palate. If you open your mouth and take a deep breath in, you’ll see your soft palate lift. The soft palate moves up and down because of the action of the muscles in the palate.  When there is a cleft in the palate muscle, it does not function and speech is impaired. Specifically, speech becomes difficult to understand because air is going out of the nose instead of the mouth. Additionally, because the soft palate pushes food to the back of the throat when a person is swallowing, eating is more difficult in patients with a cleft of the soft palate. Uvula: The uvula is the part of the soft palate that hangs down the middle in the back of the mouth. In some people, it is very well-defined. Others may have a small one or may not have one at all.  Alveolar Ridge: The alveolar ridge is also known as the “dental arch” or “gums.” The alveolar ridge is where the teeth emerge from. There is an upper alveolar ridge and a lower alveolar ridge.  Primary Palate: The primary palate is the portion of the palate in front of the incisive foramen. It includes the front portion of the hard palate and is triangular in shape. It also includes the four central front teeth and the alveolar ridge.  Secondary Palate: The secondary palate is the back portion of the hard palate (the portion behind the incisive foramen), including the posterior alveolar ridge, and all of the soft palate and uvula.  Incisive Foramen: The incisive foramen is a structure that separates the primary palate from the secondary palate. It is an opening in the bony palate through which the blood vessels and nerves for the palate pass. It is directly behind the two front teeth. In a palate without a cleft, the incisive foramen cannot be seen as it is covered by the mucosa of the palate.

Cleft Palate Classification

         Plastic surgeons classify clefts by their involvement of the primary palate, the secondary palate, or both. Cleft palate classification guides the plastic surgeon, dentist, otolaryngologist, speech therapist, and all others members of the “Cleft Team” in formulating an appropriate treatment plan. However, it may be simpler to think of a cleft of the palate as either “complete” or “incomplete.”

         Complete Cleft Palate A “complete” cleft involves the entire primary and secondary palates. It extends from the uvula all the way into the alveolar ridge. It involves both the primary palate and secondary palate.  A complete cleft palate can be unilateral or bilateral. If the cleft palate is bilateral, both sides may be complete, or one side may be complete and the other side may be incomplete. Incomplete Cleft Palate An incomplete cleft starts at the back of the palate with the uvula and extends forward. It may or may not reach the incisive foramen. In simpler terms, it only involves the secondary palate as it does not extend all the way forward to include the alveolar ridge. The length to which the cleft can extend forward from the uvula varies in severity of appearance. However, any amount of palate clefting can have a detrimental effect on speech development. The various types of incomplete cleft palates are as follows: Bifid Uvula: The least severe of the incomplete clefts in appearance, a bifid uvula is the most common palatal cleft. It is also referred to as a “cleft uvula.” A bifid uvula appears as a splitting or forking of the uvula. It may be very subtle, evidenced only by a small notch, or the uvula may appear as two distinct entities. A bifid uvula in and of itself is not problematic. This occurs in about 2 percent of the population. However, usually a bifid uvula is indicative of a submucosal cleft.  Submucosal Cleft: A submucosal cleft is a cleft that is under the mucosa that lines the roof of the mouth — hence the term “sub.” Because a submucosal cleft is under the mucosa, the only physical indicator of its presence may be a bifid uvula. Even though not seen from the surface, the muscles of the palate are not joined at the midline in a submucosal cleft. This creates an inability to move the palate for some speech sounds. Hence, a submucosal cleft is usually diagnosed when a child has abnormal speech development and a bifid uvula is present.  Soft Palate Cleft: A cleft of the soft palate runs from the tip of the uvula and stops before or at the junction of the soft and hard palate. Not only is it more obvious in its appearance than a submucosal cleft, it creates the same speech problems as a submucosal cleft. The more severe (longer) soft palate clefts are detected at birth due to feeding difficulties. The cleft of the palate makes it difficult for the infant to create a tight oral seal around the nipple. As a result, the infant may not be able to suckle. A partial or shorter soft palate cleft may not show symptoms at birth or may reveal itself as nasal reflux of liquids or foods.  Soft and Hard Palate Cleft: A cleft that involves both the hard and soft palate will include the entire soft palate and any part of the hard palate up to the incisive foramen. The most severe form involves the entire secondary palate, seen as a gap in the palate from the tip of the uvula to the incisive foramen. This is the most overt of the incomplete palate clefts. Similar to the isolated soft palate clefts, the combined soft and hard palate cleft is usually detected at birth because of feeding problems. Speech development will be impaired.  

Cleft Lip & Palate

• INTRODUCTION
• Between the 6th and 12th weeks of fetal gestation, the left and right sides of the face and facial skeleton fuse in the midddle. When they do fail to do so, the result is a craniofacial cleft. Various types of clefts may occur as isolated condition or as part of a syndrome. Since clefts involve the anatomy of the vocal tract, varying effects on speech are common.
• Craniofacial Abnormalities are structural defects of the head and face. These defects may be part of a syndrome that includes other anomalies. Consequences include structuaal and developmentalanomalies.
• Craniofacial – pertains to or involving bothe cranium (skull) and the face.
• Craniofacial Anomalies – Malformations of the cranium and/or face
• INCIDENCE OF CLEFTS
• Accepted estimate of incidence of vert cleft palate not associated with other malformations is 1 in 750 live births.
• Cleft palate without cleft lip occurs more in females (57%) than males (43%), no significant racial differences
• Cleft lip with or without cleft palate occurs twice as many males than females.
• Native Americans (most), Asians, Caucasians, African Americans (least)
• Gender differences may be related to slight differences in timing of embryological development.
• SPEECH PRODUCTION
• 7 Point Man
• Respiratory and laryngeal activities produce voice.
• The vocal signal rises through pharynx or vocal tract.
• The process by which the nasal branch is separated from or coupled with the rest of the vocal tract is referred as velopharyngeal closure.
• Physical characteristics and activity of velopharyngeal region imposes feature of resonance.
• Speech signal with characteristics of phonation and resonance modified by activity of tongue and lips imposes features of place and manner of articulation.
• Voice or Resonance Disorder
• Velopharyngeal Inadequacy should be used as a generic term
• Velopharyngeal Insufficiency for cases for insufficiency of tissue
• Velopharyngeal Incompetence for cases in which movement patterns are inadequate
• ANATOMY OF EXTERNAL FACE & ORAL CAVITY
• Nose
• Lips
• Oral Cavity
• EMBRYOLOGY
• Neural crest theory of development holds that neural crest cells, one type of specialized embryonic cells, give rise to various connective and neural tissues of the skull and face
• The cells migrate at different rates to destined locations
• If the migration fails to occurs, or if there is an absence or inadequacy of related cells, clefts and other facial abnormalities may results.
• The upper lip and premaxilla are formed by merging 3 structures:
• frontonasal process
• right and left nasomedial processes of the maxilla
• Lip closure occurs during 5 or 6th week of embryonic development.
• Palate is formed by union of palatal shelves that develop from the maxillary processes and close during 8th or 9th week of gestation
• Embryonic tongue sits above the mandible between 2 sides of the palate and fusion of the 2 sections of the palate can occur after the mandible and tongue lower of the way. When this sequence of events is interrupted, it results in a condition called Robin sequence.
• Development of the External Face
• Two mandibular processes result in the lower jaw (mandible), lower lip, and chin. By the end of the 4th or 5th week, these structures are formed and fused.
• At 5 weeks, maxillary processes appear on the anterior edge of the mandibular arches. FIG 7-2 (Smith, 1983).
• Two maxillary processes form most of the face, mouth, cheeks, and sides of the upper lip. These processes form most of the face, mouth, cheeks, and sides of the upper lip.
• These processes evolve into most of the hard palate, alveolar ridge, soft palate.
• Nasal Processes (lateral and medial) are prominent at 6 weeks. FIG 7-3 (Smith, 1983).
• Frontonasal Process – develops into the nose, central part of upper face and primary palate.
• Nasolateral Process will form the alae (lateral wings of the nostrils).
• Nasomedial Process will contribute to the formation of several structures: midportion of the nose, upper lip, maxilla, primary palate (entire frontmost portion of the hard palate).
• The nasomedial processes (medial nasal processes) fuses with maxillary processes by week 7.
• By the end of the 7 week, the upper lip and primary palate are formed.
• Development of the Internal Face
• Primary Palate
• Prior to 6th week of development, the primary palate includes that portion of the upper airway that will develop into the lip, alveolar ridge, and the hard palate segment extending back to the incisive foramen ( a hole behind the alveolar ridge through which passes nerve and blood supply to the palate). During 6th week, the separation betweeasal and oral cavities occur. This early structure contains prolabium, premaxilla and four maxillary incisor teeth.
• Secondary Palate (Hard palate 8-10 weeks)
• During the sixth to 10th week of embryological development, the foundation for the hard palate and the floor of the nasal cavities, the palatine shelves, begins their migration toward the midline of the internal face. This movement toward midline coincides with the lowering of the tongue mass which had previously rested within the developing nasal chambers.
• Between 5.5 – 8 weeks, the primary palate and the secondary palate (that portion formed by the joining of the palatine shelves behind the incisive foramen) coordinate their growth to form a single structure: the hard palate. Failure of the shelves to unite with the primary palate or with each other will result in a cleft of the hard palate.
• Soft Palate
• During the 10th to 12th week of orofacial development, the soft palate tissue fuses. Tissue that will form the soft palate’s muscles begins to migrate toward the midline from both sides of the back of the mouth during the 5th to 6th week. By the 10 week this tissue has grown and moved into a horizontal orientation along with the palatine shelves.
• TYPES OF CLEFTS
• Cleft is a separation or space between parts that are normally joined, thus interrupting the continuity of the structure. Although orofacial clefts can be acquired through various traumas, the vast majority of those of the lip and/or hard and soft palates are due to congenital failures of the structures to join. Of the many ways to classify types of congenital cleft (Berlin, 1971), a division into general types is convenient:
• clefts of the lip and/or alveolar process (gum ridge), the embryological primary palate (Figures 9-4, 9-5, Hixon text), and
• clefts of the hard and/or soft palates, the embryological secondary palate (Figure 9-6, Hixon text).
• Cleft Lip – A cleft of the lip involves only soft tissue and extend through the red part of the lip or vermillion border, into the upper portion of the upper lip toward the nostril. If the cleft is unilateral, it commonly occurs on the left side. Patient may have a short columella, which is the strip of tissue between the base and the tip of the nose.
• Cleft Palate – The palate serves as the partition between the nasal and oral cavities. The anterior two-third of the palate make up the hard palate, commonly referred to as the roof of the mouth. The soft palate or velum consists of the posterior one-third of the palate. Isolated cleft palate, without cleft lip, may include all of the hard palate posterior to the incisive foramen and the soft palate; it may involve only a small portion of the posterior part of the soft palate; or it may between these two extremes.
• Alveolar Ridge – A part of the prepalate, partially encircles the hard palate and consists primarily of supporting structures for the teeth.
• Cleft of Prepalate – Median Cleft – clefts of the upper lip is median defect extends to the nose. The missing tissue is the problabium. Bilateral cleft lip with absence of the prolabium or complete median cleft lip.
• Premaxilla Protrusion & Detachment
• Premaxilla – intermaxillary bone
• Prolabium – central prominence of lip
• Submucous Cleft Palates – The palate appears to be structurally intact, but there are both muscular and bony deficits. The defect include a bony notch in the hard palate, a bluish line at the midline of the soft palate, and a bifid uvula.
• Hard Palate – The bony notch can be seen or felt where normally the posterior nasal spine is found along the posterior border of the hard palate. The submucous cleft of the hard palate is not functionally significant.
• Soft Palate – The muscular deficit found in the soft palate often is functionally significant. This cleft is seen as a bluish line through the length of the soft palate. The levator muscles in these cases are often found to be inserted into the hard palate instead of interdigitating (interlock as or like fingers of both hands) to form the normal levator sling. This condition foreshortens the functional soft palate and creates a further complexity which may contribute to velopharyngeal incompetence requiring surgery. The levator muscle elevates the posterior part of the soft palate, the velum, and pulls it slightly backward which effectively closes the nasal from the oral pharynx.
• Unilateral Clefts
• Bilateral Clefts
• COMMUNICATION DISORDERS
• SPEECH DISORDERS
• Good place of articulation and intelligible speech in association with nasal emission and consonant weakness resulting from lack of intraoral breath pressure.
• Compensatory Learned Behaviors – Second pattern involved only nasal escape, but also nasal snort, glottal stops, pharyngeal fricatives, nasal grimace, and other articulatory sibilants.
• MISARTICULATION OF SPEECH SOUNDS
• Reduced intra-oral pressure (McWilliams Audio 1,2)
• Some speakers with VPI seem to accept their loss of intra-oral pressure and continue to articulate as accurately as possible,. These speakers may have weak consonants and audible escape, but placement is accurate. Even intelligibility may be only slightly impaired.
• Sounds Most Frequently Misarticulated
• Most frequently in error with VPI: /s/. In short, velopharyngeal competence is a requirement for adequate consonant production. The first consonant to deteriorate will be /s/. This is the phoneme most frequently in error in individuals with VPI of a marginal or borderline nature. As the incompetence increases, other sibilants and fricatives (f,v,th,sh,z,s,zh) become involved. The symptom is decreased intraoral pressure because of loss of air at the velopharyngeal portal. Still greater incompetence in this critical valve will lead to reduced intra-oral pressure in association with plosives.
• Sounds Most Frequently Misarticulated – McWilliams (1953)
• /s/ – 63%
• /z/ (61%)
• /d/ (48%)
• /ch/ – (44%)
• /p/ – (11%)
• /b/ – (9%)
• /s/ is th espeech sound most frequently and most consistenly misarticulated by cleft palate individuals.
• Substitutions (McWilliams Audio 10)
• Generally sound substitutions are seen in speech problems that are characteristic of young children who are still in the process of learning speech. Some writers have referred to the fact that speakers with cleft palate substitute a nasal consonant for a consonant that requires intraoral pressure (for example, substituting /m/ for /b/). In some instances, this response is regarded as a nasally distorted /b/ rather than the substitution of /m/. Viewed in this context, the speech sound error clearly points to velopharyngeal incompetence rather inappropriate learning.
• Omissions (McWilliams Audio 11)
• Could delete final consonants as a means of avoiding nasal emission, because sounds lacking normal oral air pressure could be interpreetedd as omissions even though some energy marked the final consonants.
• Compensatory Strategies for VPI (McWilliams Audio 12,14)
• Some speakers with VPI seem to accept their loss of intra-oral pressure and continue to articulate as accurately as possible,. These speakers may have weak consonants and audible escape, but placement is accurate. Even intelligibility may be only slightly impaired.
• Others attempt to compensate for their valving deficits by seeking new approaches to consonant articulation. These individuals discover that it is easier to produce a sibilant-like consonant lingua-palatal or pharyngeally than it is to produce it in the front of the mouth and that plosives created glottaly are unaffected by the defective valve.They adopt gross articulation errors, which are referred to as pharyngeal fricative and glottal stops. These compensations are rarely accompanied by nasal escape because that critical valve is bypassed.
• Stop plosives, fricatives, affricates misarticulated cannot manage airstream for development and maintenance of high oral air pressure. Therefore, leading to deterioration of sound (Hixon, 372).
• Hypernasal resonance, nasal air emission, and weak pressure consonants cause phonetic distortions, compensatory gestures cause phonetic substitutions.
• Individuals with clefts tend to substitute non English sounds for English phonemes. Substitute glottal stop (whispered cough) for stop-plosives (p, t, k, b, d, g). Glottal stops are made by closing the glottis, building up pressure within the trachea, and then suddenly reopening the glottis to release a transient puff of air. Substitute pharyngeal fricatives for fricative consonants. Pharyngeal fricatives are produced by positioning the tongue close to the back wall of the throat or by decreasing the side to side diameter of the throat by moving the walls of the pharynx inward. Air is then forced through the constricted pharyngeal airway to produce a turbulent sound.
• Substitution of Glottal Stops – GO BACK TO WITZEL VIDEO
• Speakers with VPI problems demonstrate glottal articulation for plosives, typically back plosives. The interruption of the air stream at the level of the glottis results in a cough-like speech sound that has been labeled the glottal stop /?/.
• Substitution of Pharyngeal Fricative – GO BACK TO WITZEL VIDEO
• Typically, fricatives and affricates are the result of a constriction of the air stream in the anterior portion of the oral cavity (lips, tongue, teeth).
• Speakers with velopharyngeal incompetence are not able to produce the frictiooise because the air pressure escapes from the oral cavity through the velopharyngeal opening. Such speakers sometimes distort fricatives and affricates in order to create the frictiooise by constriction between the tongue and pharyngeal wall or perhaps by constriction of the pharyngeal tube.
• Distortion by Nasal Emission (McWilliams Audio 13,3,4)
• Description -The type of error sometimes referred to as the distortion-nasal is typical of what most listeners regard as “cleft palate speech” and is demonstrated by many cleft palate speakers. The air pressure is emitted through the nostrils during the production of the pressure consonants. If the escape is sufficient in quantity, the nasal emission of air pressure is audible and distorts the acoustic signal of the speech sound.
• Nasal air escape associated with production of consonants requiring high oral pressure. It occurs when air is forced through an incompletely closed velopharyngeal port. Nasal emission may be audible or not.
• RESONANCE
• Hypernasality that occur supraglottally is referred to as “resonance disorders.”
• Voice disorders refers to a problem that occurs at the level of the larynx, a disorder of phonation.
• Hypernasality is not a voice problem that implies phonation.
• Resonance is the vibratory response of a body or air-filled cavity to a freqeuency imposed upon it. (Wood, 1971).
• Individuals with velopharyngeal incompetence have traditionally been described in the literature as having an hypernasal voice quality. In actuality, hypernasality is not a problem associated with phonation. It is the result of alterations that occur when the oral and nasal cavities are coupled when they should be separated by action of the velopharyngeal mechanism.
• Definition – The perception of excessive nasal resonance during the production of vowels. This results from inappropriate coupling of the oral and nasal caviities. Low vowels and vowels iasal consonant contexts are normally somewhat nasalized.
• Factors Responsible for Hypernasality
• Results form inadequate closure of the VP valve during speech, but it may caused by entrance of air into nasal cavity through an open cleft palate or fistula in hard or soft palate.
• Oral Cavity constriction during speech may increase hypernasal resonance by forcing more sound waves into the nasal cavity. This constriction may be the result of :
• restricting mouth opening during speech,
• posterior or superior positioning of the dorsum (upper surface) of the tongue during articulation of some sounds,
• or abnormal positioning or tension of the pharynx.
• Hyponasality is the speech characteristic associated with a reduction iasal resonance with /m,n,ng/ approaching but not matching /b,d/g/.
• a reduction iormal nasal resonance usually resulting form blockage or partial blockage of the nasal airway by any number of causess, including upper respiratory tract infection and a wide obstructiing pharyngeal flap, and moderately large adenoids were present.
• If the nasal airways were completely occluded, speech would be denasal, meaning that nasal air flow associated with /m, n, ng/ would be eliminated and the sound wave altered; the nasal consonants would approach but not match /b, d, and g/.
• VOICE DISORDERS
• Hoarseness
• Hamlet (1973) speculated that glottal tightness might contribute to vocal abuse which would lead to hoarseness, harshness, and vocal nodules secondary to hypernasality. This conclusion appears reasonable in view of the clinical evidence of patients who attempt to control hypernasality by tightening both the respiratory muscles and laryngeal muscles to control air flow before it reaches the crucial velopharyngeal valve.
• Soft Voice Syndrome
• Because of loss of air through the velopharyngeal port, some cleft patient shave difficulty creating a voice that is of sufficient loudness to serve them well in communication or they use reduced loudness as a compensatory strategy. Patients who adopt this voice pattern may have little nasal escape, although their velopharyngeal valving mechanisms are usually deficient. In order to increase loudness, their subglottic pressures must be higher than normal, and there is air loss leading to an increase iasal emission, together with alterations in the perception of hypernasality. This problem is identified on the basis of clinical experience.
• Reduced loudness
• General characteristic of client with repaired cleft palate is the reduced loudness. Aerodynamic and acoustical factors contributes to the reduced loudness rather reduced self confidence or self consciousness.
• For vowels, diphthongs, and many voiced consonants, the problem is related to incomplete velopharyngeal mechanism which allows sounds produced at the larynx to pass through the nasal segment of the upper airway.
• For many consonant sounds of speech, an incompetent velopharyngeal mechanism precludes the adequate buildup of oral air pressure that is required behind airway occlusions and obstructions to generate normal speech sounds (Subtelny, Worth, and Sakuda, 1966). This means that sounds like voiceless stop-plosives, fricatives, and affricates will fail to their normal crispness when produced. Consequently, they are perceived as being too soft. Trying to generate sounds orally in the face of velopharyngeal incompetence is like trying to blow up a tire that has a leak in it.
• Monotone
• Often accompanying the soft-voice syndrome is the monotonous voice with little pitch variation. Patients with problem are often unable to demonstrate pitch variations of more than 3 or 4 tones. This problem is identified on the basis of clinical experience.
• Strangled Voice
• This problem is not one of the more common phonation disorders found inpatients with valving problems, but is encountered often enough to warrant mention here. Strangled voice quality appears to be associated with an attempt to be non-nasal in the presence of VPI. Phonation is associated with extreme tension in the abdominal, diaphragmatic, thoracic, laryngeal, and supraglottal muscles. It is almost as if the person were trying to hold his breath and talk at the same time. The introduction of relaxation results in an increase in hypernasality.
• DELAYED LANGUAGE
• Research suggests that there may a lag in language acquisition during early years of development for many children with orofacial clefts, but as the individual gets older the gap closes. By adulthood, no significant language differences remain.
• Disruption in early experience disruption of oral touch-pressure sensation, oral cavity exploration, perceptual motor deprivation, prolonged deprivation during formative language period and reduced hearing sensitivity.
• Insufficient language stimulation due to low expectations for verbal responsiveness given child’s physical problem.
• Negative reactions of listeners cause child to limit the frequency and elaboration of speech language attempts.
• HEARING DISORDERS
• Hearing disorders are prevalent among individuals with orofacial clefts. These disorders are a result of middle ear dysfunction, involving both ears. Exist in all infants with unrepaired clefts of palate under the age of 2, 70 to 80% with repaired clefts of older children
• Prevalence is due to a disease called otitis media involves an inflammation and/or infection of middle ear system and presence of liquid in middle ear root of otitis media problem is eustachian tube dysfunction muscle to open ET are cleft and do not functioormally consequently ET remains closed, pressure and liquid builds up in ME inflammation and infection occurs middle ear sound transmission system is impaired conductive type of loss occurs