Cleft Palate

It was Kilner who said, “Ask not for a spatula and torch to check your cleft palate repair, but listen to your patient speak.” By this obvious but profound statement, he drew the cleft surgeons’ attention to the fact that gone are the days of breakdowns and fistulae and that if your child does not speak well, your operation is a failure, for such a child would be out of the mainstream of life forever. In spite of the advances in technique and execution, experienced cleft surgeons all over the world still struggle to obtain perfect speech in a large percentage of cases.


Introduction
It was Kilner who said, "Ask not for a spatula and torch to check your cleft palate repair, but listen to your patient speak." By this obvious but profound statement, he drew the cleft surgeons' attention to the fact that gone are the days of breakdowns and fistulae and that if your child does not speak well, your operation is a failure, for such a child would be out of the mainstream of life forever. In spite of the advances in technique and execution, experienced cleft surgeons all over the world still struggle to obtain perfect speech in a large percentage of cases.
In view of the obvious difficulties of intraoral surgery, it is not surprising that cleft lips were repaired way before anyone tried to repair a cleft palate. Credit for the first successful repair of a cleft palate goes to Le Monnier, a French dentist from Roven. Le Monnier in 1766 cauterized to freshen the cleft edges and sutured them successfully [1].
Cleft surgery then passed through a stormy period of uncertainty in the hands of giants like Ferdinand von Graefe [2], Roux, Dupuytren [3], Dieffenbach [4], and Warren [5]. Mucosal flaps were used, and even lateral osteotomies were tried to move the hard palate medially. In 1859, almost a hundred years after the first hard palate was repaired, von Langenbeck [6] emphasized the need to raise mucoperiosteal bipedicled flaps to repair a palate. Langenbeck procedure was a fundamental breakthrough, and with this major advance, breakdowns of the palate were reduced. In its wake refinements followed. In 1931, Victor Veau [7] parted from the bipedicled flap to single pedicled flaps based on the greater palatine vessels. Veau stressed the importance of repairing the nasal lining of the palate and the need to lengthen it.
In 1937, Kilner [8] working in Oxford and Wardill [9] working in Newcastle on Tyne independently used a four flap procedure for the complete cleft palate.
The weakness of this procedure was a high fistula rate at the junction of the four flaps. In the 1940s, Dorrance [10], Cronin [11], and others used various flaps to lengthen the palate. In 1966 Millard [12] introduced his island flaps based on the greater palatine arteries to lengthen the nasal lining. Ravin Thatte [13] went a step further and took two island flaps one for lining and one for cover and used a tongue flap on the denuded hard palate. All these procedures had their day but were short lived in popularity. However, special mention must be made of the buccal myomucosal flap originally called the cheek flap by Padgett [14]. This flap was revived 30 years later by Murari Mukherjee [15] of Calcutta. He changed the direction of the flap. Recently Ian Jackson has introduced muscle into this mucosal flap to make it more robust, and he calls it a myomucosal flap. He uses it in all his palates to lengthen the nasal lining by 1.5 cm and believes that as a result he achieves better speech. Robert Mann [16] does a Furlow's and coves the residual raw areas with two buccal flaps. His rationale is that there is deficiency of tissue in the palate and therefore, on first plastic surgical principles, bring in tissue from outside. This may become the philosophy of the future. (Figs. 72. 1 and 77.1)

Embryology
The primary palate is derived from the intermaxillary segment derived from the frontonasal and medial nasal prominences. The main part of the definitive palate is formed by two shelf-like outgrowths from the maxillary prominences. These outgrowths, the palatine shelves, appear in the sixth week of development and are directed obliquely downward on each side of the tongue (Fig. 73.1a and b).
In the seventh week, however, the palatine shelves ascend to attain a horizontal position above the tongue and fuse, forming the secondary palate (Fig. 73.2a and b). Anteriorly, the shelves fuse with the triangular primary palate, and the incisive foramen is the midline landmark between the pri-mary and secondary palates. At the same time as the palatine shelves fuse, the nasal septum grows down and joins with the cephalic aspect of the newly formed palate (Fig. 73.3a and b).
The secondary palate lies posterior to the incisive foramen and consists of the bony hard palate anteriorly and the soft palate posteriorly, terminating at the uvula. The hard palate consists of the palatine bony shelves on either side, attached to the vomer in the midline. The soft palate consists of a sandwich of muscles enveloped by oral and nasal mucosa.

Muscles
The muscles of the soft palate include intrinsic muscle, i.e., musculus uvulae, and the insertions of several extrinsic muscles. Apart from this, large amount of.glandular material is present in the anterior inferior portion of the velum and an anterior aponeurosis. Extrinsic muscles of the velum include the tensor veli palatine, levator veli palatini, palatopharyngeus, palatoglossus, and fibers of the superior constrictor. Pharyngeal muscles usually described as having functional role in velopharyngeal movement include the superior constrictor and the salpingopharyngeus (Fig. 73.4). Following is a tabular summary for the muscles of palate and their abnormal anatomy in a cleft palate (Table 73.1).
From functional standpoint, it is apparent that the levator veli palatini muscle is the principal and quite possibly the only muscle to function for elevation of the velum in speech. The differences between the normal and the cleft arrangement of the muscles of velopharyngeal closure occur because the muscles extending toward the central line of the soft palate cannot attach themselves in the midline of the velum so they insert at some substitute points. These points prevent the muscles from becoming fully functional, and therefore their development is retarded. With the preservation of normal origins, the atypical insertions and hypoplasia of the muscles are the main pathological features in the cleft palate. The abnormal insertions of levators in clefts illustrate that the function of these muscles in cleft palate is almost opposite to that in normal one. While the muscles of both sides normally join in the raphe to form a sling lifting the palate upward, in cleft palate each muscle pulls its own half of the soft palate in an entirely different direction, i.e., superolaterally, causing further widening of the cleft.

Vascular Supply (Fig. 73.5a and b)
The internal maxillary artery gives off the descending palatine artery, which in turn gives off several branches to the tonsils and soft palate. It then passes through the posterior palatine foramen, just above the periosteum, and proceeds forward close to the alveolar margin on each side as the greater major palatine artery to the incisive fossa. At that point it sends terminal branch through the incisive foramen to anastomose with the terminal branch of the sphenopalatine artery. The vascular supply of importance is discussed under two headings, anterior and posterior palate.

Vascular Supply of the Anterior Palate/ Premaxilla
The blood supply to the anterior alveolar process of the maxilla comes from the arterial complex composed of the major palatine, anterior and superior alveolar, and branches of the sphenopalatine artery. The posterior septal artery arises from the sphenopalatine artery in the roof of the nasal cavity and courses down the groove of the vomer to the incisive foramen. In the complete bilateral cleft lip and palate, the union of the superior labial arteries is lacking; thus they do not contribute to the blood supply of the philtrum. Also the anastomosis of the posterior septal artery with the major palatine artery is absent. Therefore premaxilla and philtrum must derive their blood supply from the posterior septal artery and to some degree from the lateral and terminal branches of the anterior ethmoid vessels which pass through the columella. There is usually one well-developed vessel on either side of the premaxilla in the region where the incisive foramen should have been. Each of these vessels moves anteriorly and inferiorly into   In a cleft child, as there is obviously no continuity across the midline due to the cleft, there are abnormal attachments of the levator muscle to the palatopharyngeus muscle posteriorly, to the edge of the cleft medially, to the tensor veli palatini, and to the posterior edge of the hard palate anteriorly the philtrum and forms an arcuate anastomosis across the midline in the inferior part of the philtrum.

Vascular Supply of Posterior Palate
The greater palatine artery supplies the oral surface of the hard palate and gives off a few fine branches which perforate the horizontal plate of the maxilla to supply the nasal mucosa. It also sends twigs to the gingiva and the palatoglossal arch. The lesser palatine artery supplies the anterior half of the oral surface of the soft palate. A branch of the facial artery, the ascending palatine artery, is the largest vessel entering the soft palate. It ascends on the lateral side of the superior constrictor muscle to turn downward and forward into the soft palate, between the tensor and levator palati giving small branches to these muscles. Twigs from the tonsillar and ascending pharyngeal arteries also reach the soft palate. In the secondary palate, the existence or absence of cleft makes little difference to the vascular pattern. The palatal mucoperios- teum is detached from its bony base in the palatal pedicle flap, and as a result the recurrent bony branches are severed. These branches result in bleeding from their cut stumps at the bony surface. This may be judged insignificant at the time of surgery; however, after the flap is repositioned, should bleeding continue, blood may pool beneath the repositioned flap [17].

Classification and Presentations of Cleft Palate
Many of the historical and contemporary classifications have been discussed in brief in the Chap. 72 on cleft lip. However, Veau's classification for palate is the most practical and therefore is mentioned again here.

2)
Clefts of the palate that are less extensive in magnitude include the submucous cleft palate and the occult submucous in cleft palate. Sommerlad has devised a grading system for the submucous cleft palate with three points for each of the above criteria [18]. A lesser score in this system naturally denotes more trivial clefting. However, paradoxically a lower score reflects a poorer prognosis for speech according to this study. An occult submucous cleft does not exhibit any of Calnan's criteria but shows a trough-like depression on the superior surface of the soft palate as seen in nasoendoscopy. Croft believed that in these patients, the musculus uvulae is absent. It is suspected when a child with a normal palate speaks with nasal emission.

General Examination
A routine per-oral examination of the neonate reveals the cleft of the palate with or without cleft lip. The extent of clefting is noted. General examination is mandatory as it is important to identify syndromic patients. These are more common in isolated cleft palate patients [19]. Pierre Robin sequence is a fairly common presentation with micrognathia or retrognathia (Fig. 73.6 and b). As the bony abnormalities are believed to be the cause of the non-descent of the tongue in utero leading to the clefting of the palate, this is now believed to be a sequence and not a syndrome. Syndromic patients include Treacher Collins syndrome, Goldenhar syndrome, etc.

Clinical Assessment of the Cleft
The first clinical visit usually happens soon after birth. The extent cleft is assessed, i.e., the length of the palate and the width of the cleft. Randall has classified cleft palate on the basis of length into four types [20]. Clefts may be partial or complete. A partial cleft of the secondary palate is confined to the soft palate ( Fig. 73.7). A complete cleft includes both the soft and the hard palate up to the incisive foramen ( Fig. 73.8). In patients with bilateral cleft lips, the associated cleft palate usually presents with a central vomer and clefting on either side of the vomer (Fig. 73.9).
A pediatric assessment is made for other anomalies, weight of the baby is noted, and feeding instructions are given during this visit. A squeezable feeding bottle is usually advisable to overcome child's difficulties in sucking. The child is fed with the head held slightly higher. Parents are counselled about the need for immunization. They are also explained about the need for surgery and are shown results of the procedure on similar patients. It is very important to allay their anxiety as they are very often crestfallen on seeing the deformity in their baby. Subsequent visits, possibly at monthly intervals, aim to assess the growth and development of the baby. Associated cardiac or other anomalies if present need investigation.

Preoperative Factors (Box 73.1)
1. Timing and Age of the Patient. The dilemma regarding the timing of repair is between earlier repair with better prognosis for speech, but worse prognosis for maxillary growth, and late repair which is exactly the opposite. Most surgeons today agree that the cleft palate is to be operated close to 1 year. Some prefer 9 months, where others wait for up to a year and 4 months. At our center we have been operating on these children at about 11 months of age. The Oslo school of surgeons [21,22] devised a protocol of operating on the lip with the soft palate in the first stage and operating on the hard palate much later. In some centers, the palate has been repaired ©Association of Oral and Maxillofacial Surgeons of India   first at initial presentation, and the cleft lip is repaired, subsequently, in view of the fact the children may not be brought back for treatment of the palate once the lip is repaired [23].

Airway.
Airway management is important at the time of birth in cleft palate patients, as most of isolated cleft palates may be associated with a smaller airway at birth. This may be due to associated sequence or syndromes, such Pierre Robin sequence or Treacher Collins syndrome. Such patients might require emergency tracheostomy or tongue lip adhesion to secure airway. Definitive management for mandibular advancement and cleft palate repair can be undertaken at a later stage.

Feeding.
As mentioned already, the parents are counselled about feeding practices during the first visit itself. Expressed breast milk is to be preferred. Special feeding bottles like the Haberman feeding bottle are used when the common methods like the use of simple squeezable bottles are not effective. As a cleft child swallows air along with the milk, frequent burping is essential to avoid regurgitation of feeds. 4. Anesthesia.
The operation is performed under general anesthesia with an endotracheal tube. RAE tubes are preferred for cleft palate patients. Previously, Oxford red rubber tubes were used. These were well adapted to the needs of the cleft children but often caused laryngeal edema presumably due to the latex in the tubes. At our center we have given up the use of these otherwise very useful tubes.
Preoperatively, the child must be assessed by a good pediatrician and also by a senior anesthetist. Respiratory infections are common in these children and must be treated if significant. X-ray of the chest is usually done to rule out heart or lung anomalies. Cardiology clearance is required when there are suspected cardiac anomalies. We have come across one case with a congenital absence of the lung.
The anesthetist should be experienced in pediatric anesthesia. In patients with Pierre Robin sequence or other anomalies like shoulder or spinal anomalies, difficult intubation is to be anticipated, and appropriate equipment such as intubating endoscopes, etc. should be at hand, and the anesthetist must be familiar with its use.  [24,25].

Principles and Methods of Repair
The major objectives of a cleft palate operation are: • To produce anatomical closure of the defect.
• To create an apparatus for development and production of normal speech. • To minimize the maxillary growth disturbances.

Box 73.2 Methods of Cleft Palate Repair
Methods of repair palate and above the nasal mucosa in the soft palate. A dental syringe with a short-beveled needle is very useful for this purpose.
Surgical procedure: At our center, we use the two longflap technique popularized in India by Charles Pinto and the world over by Bardach (Fig. 73.13). The technique was originally used by Veau. The cleft edges are pared (Fig. 73.14).
The lateral incision is made from the maxillary tuberosity area toward the retromolar area posteriorly. The pterygoid hamulus is dissected, fractured, and detached (Fig. 73.15a  and b).
This aids in mobilization of the nasal layer subsequently. However, there are many surgeons nowadays who do not believe in fracturing the hamulus. It has, however, been shown in different studies that fracture of the hamulus does not cause any deleterious effects on hearing [26].
The lateral incision is then carried anteriorly and the oral mucoperiosteal flap is lifted off till the medial edge of the hard palate using Kilner's palate elevators (Fig. 73.16a  and b).  An incision is then made on the medial border of the hard palate, and the flap is also divided anteriorly taking care to secure the end of the greater palatine vessels. The oral flap is then raised till the posterior border of the hard palate. Laterally, the greater palatine vessel is identified and skeletonized, after incising the cone of periosteum which binds the vessel behind it (Fig. 73.17a, b, c and d).
After the hamulus is fractured, the medial pterygoid plate is denuded of any soft tissue attachments (Fig. 73.18).
Similarly, the posterior border of the hard palate is also cleared of muscular attachments (Fig. 73.19a and b).
A Cumine Scaler is introduced over the bare medial pterygoid plate and then turned anteriorly to separate the nasal layer from the hard palate in one clean sweep. This dissection is then completed on a deeper plane with a Wallace's finisher (Fig. 73.20a and b).
This practice of separating the nasal layer from behind forward was originally introduced by Kilner [8] but has somehow not been followed widely. It is much easier than the alternative method of separating the nasal layer from the hard palate starting anteriorly. Very often this results in tearing of the nasal layer.
Similar dissection is performed on the other side also, raising a long oral mucoperiosteal flap. The vomer flap is raised (Fig. 73.21) and used when accessible and necessary.
Rarely, the vomer is receding and cannot be used. In a bilateral cleft lip patient, the vomer is in the midline, and a central incision on the vomer is made to raise two mucoperiosteal flaps, one on each side, to be sutured to the corresponding side in nasal layer of the hard palate.   Fig.73.15 (a, b) Fracture of the pterygoid hamulus

Dissection of the Soft Palate
The early techniques of cleft palate repair only detached all false attachments of muscles from the posterior border of the hard palate (Fig. 73.22).
Surgeons like Braithwaite [27] and Kriens [28] emphasized the importance of the dissection and retroposition of the levator veli palati muscle. This was popularized by Sommerlad [29] and Tambewekar in India.
Infiltration of lignocaine with 1:200,000 adrenaline is made between the nasal mucosa and the muscle (Fig. 73.23).
A transverse incision is made on the aponeurotic layer just beyond the hard palate (Fig. 73.24).
Then the muscle is dissected off the underlying mucosa leaving behind enough tissue on the nasal layer, to avoid its tearing during suturing. Also, a narrow layer of muscle is retained along the cleft edges to hold the sutures. The muscle is dissected till just short of the uvula. The muscle contains the levator veli palati mostly. However, it also contains other muscles like the palatopharyngeus ("muscle of Veau") ( Fig. 73.25).
The extent of dissection of the muscle on the soft palate varies. The authors [30] only separate it from the nasal layer. Others like Sommerlad [29] also separate it off the oral layer. However, if there is any suspicion of injury to the greater palatine vessels during dissection, then the levator muscle should not be dissected free of the oral mucosa.

Suturing
Suturing commences on the nasal layer from anteriorly, proceeding backward. The sutures are not tied but held on the Kilner suture carrier.

Uvula
The last suture on the nasal layer is a mattress suture (Fig. 73.26) [30].
Next, an apical mattress suture is placed on the tip of the uvula, and this suture is left long and held on an artery forceps, thus turning the uvula over, providing access to the nasopharyngeal aspect of the uvula. Two or three simple sutures are placed between the mattress suture and the apical stitch ( Fig. 73.27a).
Then two or three mattress sutures are placed on the oral aspect of the uvula (Fig. 73.27b).
The nasal layer sutures are then tied from behind forward.
"A" suture: this is the suture placed at the junction of the hard and soft palate. It goes through the nasal mucosa and later crisscrosses into the oral mucosa, resembling the letter A, but is like the figure of "8" when tied.
Anterior sutures are also tied but retained long. Suturing of the oral layers then proceeds from behind forward, with every suture picking up a bite on the nasal layer to obliterate dead space. The dissected levator mus- cle is also included in these oral sutures. Some surgeons close the levator muscle as a separate layer. The anterior sutures are then brought through the oral layer also, helping in anchoring the oral layer. In the absence of the anchoring, the oral layer may sag postoperatively causing a "fallen palate." Laterally the raw areas are sutured with interrupted sutures without tension. This helps in hemostasis (Fig. 73.28).
Previously, this area used to be packed with different materials, but there was often bleeding from the areas on removal of the pack postoperatively.
Buccal pad of fat harvested through a stab incision on the buccal aspect bilaterally has also been used to fill large lateral raw areas. (Box 73.3)

Early Complications
Hemorrhage This is often from the apices of the long flaps, from the cut ends of the greater palatine vessels. Arterial bleeding points need to be ligated or cauterized using diathermy. Small venous bleeding areas can be controlled by the use of pressure with fingers or by the use of crushed ice or topical hemostatic agents like tranexamic acid.

Respiratory Obstruction
This may be from tongue fall and can be avoided by use of a tongue stitch at the time of palate repair. The tongue stitch has to be placed well posteriorly and must include a good bulk of the tongue tissue to avoid the sutures from tearing through.
When a wide cleft palate is closed, the child may have difficulty in learning to breathe adequately through the nose. Laryngeal edema due to endotracheal tube-related trauma may present with a hoarse cry and, in severe cases, chest retraction. Early detection is important, and this can be reversed by intravenous steroids and nebulization.

Breakdown of the Repair
It may result in fistulas or complete breakdown of the repair. The most common cause of these, especially at the junctional area, is suturing of the cleft edges under tension due to inadequate mobilization.

Long-Term Complications
Long-term complications include poor speech. Adequate muscle repair has helped in better speech results. Nonetheless, speech is an enigma that has not been adequately understood and mastered. The variables like width of the cleft, the gap between the uvula and the pharynx, and the adequacy of the muscle repair all have a bearing on the speech results. Despite all the advancements in the repair of the cleft palate, no surgeon can truly claim to achieve perfect speech results in all patients consistently.

Long-Term Results
The long-term evaluation of cleft palate patients should include the presence or absence of a fistula, the quality of speech, and the growth parameters of the maxilla, i.e., whether there is maxillary hypoplasia.
At present, many centers are able to produce fistula-free palates in a majority of patients. Tension-free closure of the palate and meticulous suturing are the key factors in the prevention of fistulas. A review of literature reveals a huge range in the incidence of fistula after cleft palate repair. The range is from 2.6 to 58%. In between these extremes, many have reported around 10%, some 15 and 23% [31,32].
The emphasis on good levator veli palatini dissection and repair has led to improved speech results overall. However, one can never perfectly predict the speech outcome, as speech itself is an enigma. Probably as a result of the variation in the presentation, mode of repairs and age at the time of repair, etc., the published literature notes a VPI rate of 5.9-70% [32].
Maxillary growth continues to be a problem. Operations like these of Robert Mann show promise in this direction. Again, owing to variability involved in the type and extent of cleft, the time of repair and the nature of the surgery, the need for maxillary advancement may range from 10 to 40% in non-syndromic cleft patients [32].

Secondary Repair and Revision
The age old saying that primary surgery is the best chance for the surgeon to obtain optimal results is very apt in the repair of the cleft palate. A badly repaired cleft palate sometimes leaves behind a grossly scared palate with paucity of tissue. Small soft palate fistula can be repaired by freshening of the edge and two-layered closure. Small hard palate fistulas can be repaired by local turn over flaps for lining and oral mucoperiosteal flaps for the oral layer. Larger ones and those with poor speech will require revision palate repair. When there is gross deficiency of tissue, flaps like the tongue flaps, or temporalis musculocutaneous flap, facial artery muscle mucosal (Famm) flap may be necessary [33,34].
Secondary palate repair is also required for velopharyngeal incompetence (VPI). A detailed discussion about the management of such incompetence is beyond the scope of this chapter. Diagnosis of VPI is by speech assessment, nasoendoscopy, or videofluoroscopy. Once a diagnosis is made, treatment modalities will include redo palate repair, sphincter pharyngoplasty, or flap pharyngoplasties, depending on whether it is the palate, lateral wall (palatopharyngeus) or the posterior wall, or a capacious pharynx as seen on investigations. Subsequent interventions will be required for maxillary hypoplasia when significant. This is again out of the scope of this chapter. In brief, anterior maxillary distraction is done if the maxillary advancement required is significant and the facial bones are still growing. Some of these do require a LeFort 1 osteotomy later on if there is relapse.
When the growth is completed, then a LeFort 1 osteotomy is required when there is significant maxillary hypoplasia. When the advancement required is more than in 1 cm, bijaw surgery is needed, with a LeFort 1 advancement in the maxilla and a bilateral sagittal split osteotomy of the mandible.

Recent Techniques
The use of the buccal mucosal flap in primary repair was pioneered by Padgett [14] and then used extensively by Murari Mukherjee [15]. Later Ian Jackson incorporated buccinator muscle into the flap and used it on the nasal layer transverse to provide for lengthening of the soft palate.
Furlow's Double Opposing Z plasty described by Furlow [35] is increasingly used both for primary and secondary repair of cleft palates. This illustrated in the figure (Fig. 73.29a, b, c and d). Robert Mann has described the extensive use of the double opposing "Z" plasty with the buccal flaps over the nasal and/or oral layers to avoid lateral incisions and to promote for better bony growth. This is probably the method of the future as it shows promise of good speech and good maxillary growth. However, extensive suturing of the buccal flaps is involved and this is not the optimal surgery for the novice to try. The Mann procedure is for the experienced cleft surgeons [36].

Case 1
This is a patient with a partial cleft of the secondary palate ( Fig. 73.30). The hard palate is intact. The cleft palate was repaired at the age of 11 months using the Veau-Wardill V-to-Y repair and radical muscle dissection in the soft palate. Subsequently the child is undergoing regular follow-up and speech assessment.

Case 2
This is a child with a complete cleft of the primary and secondary palate on the (Fig. 73.31). The cleft lip was repaired at 6 months of age using the Millard's rotation advancement technique. At 11 months, the cleft palate was repaired using the two long-flap procedure popularized in India by Charles Pinto, the mentor of the senior author, and, worldwide, by Bardach. The child is now being subjected to constant follow-up and speech therapy.

Disclosure
Authors have no financial conflicts to disclose.