23. Cleft Palate Repair I by Alex Campbell

Cleft Palate Repair

23.

A cleft palate has tremendous aesthetic and functional implications for patients in their social interactions, particularly on their ability to communicate effectively and on their facial appearance. 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.

Incidence The incidence of cleft lip/palate (CL/P) 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 CL/P have other anomalies at birth.

Inheritance Patterns In 25% of patients, there is a family history of facial clefting, which does not follow either a normal recessive or dominant pattern. The occurrence of 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 CL/P, 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 CL/P has other affected first-degree

Etiology The causes of cleft palate appear to be multifactorial. Some instances of clefting may be due to 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 with several teratogenic drugs. 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. Relevant Anatomy The bony portion of the palate is a symmetric structure divided into the primary and secondary palate based on its embryonic origin. 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. 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 pharyngeal constrictor, musculus uvulae, palatopharyngeus, palatoglossus and tensor veli palatini. The three muscles that appear to have the greatest contribution to the velopharyngeal function are the musculus uvulae, levator veli palatine and superior pharyngeal constrictor. The musculus 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 the superior pharyngeal constrictor, aids in the opposition of the velum against the posterior pharyngeal wall to form the competent sphincter. The palatopharyngeus displaces 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 palatiniʼs 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 the tensor tympani with which it shares its innervation, is to improve the ventilation and drainage of the auditory tubes.

Â In a 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 the 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 six muscles, the prevailing theory attributes most of the contribution to the velopharyngeal competence to the levator veli palatini.

Clinical Findings The pathologic sequelae of cleft palate can include airway issues, feeding and nutritional difficulties, abnormal speech development, recurrent ear infections, hearing loss and facial growth distortion. Airway Problems The infant with Pierre Robin sequence 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. Feeding Difficulty The communication between the oral and nasal chamber impairs the normal sucking and swallowing mechanism of the cleft infants. Food particles can reflux into the nasal chamber. Breastfeeding is usually not successful unless milk production is abundant. Speech Abnormalities Speech abnormalities are intrinsic to the anatomic derangement of cleft palate. The velopharyngeal mechanism is essential in production of nonnasal sounds and is a modulator of the airflow in the production of other phonemes that require nasal coupling. The manipulation of the velopharyngeal mechanism, if not successfully learned during early speech development, can permanently impair normal speech. Â

Â Middle Ear Disease The disturbance in anatomy associated with cleft palate affects the function of the eustachian tube orifices because the abnormal insertion of the tensor veli palatini that prevents satisfactory emptying of the middle ear. Recurrent ear infections have been implicated in the hearing loss of patients with cleft palate that may worse the speech. The reposition of the muscles levator and the tensor veli palatini improves function of these muscles, improves ventilation of the middle ear, decreases serous otitis and 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. Facial Growth Abnormalities Multiple studies have demonstrated that the cleft palate maxilla has some intrinsic deficiency of growth potential. This growth potential is further impaired by surgical repair. Any surgical intervention performed prior to completion of full facial growth can have 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 blamed for restriction of maxillary expansion. The growth disturbance is usually prognathic appearance and discrepant occlusion relationship between the maxilla and the mandible. 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 CL/P. High among the associated anomalies are those affecting the circulatory and skeletal systems.

Submucous Cleft Palate Three diagnostic signs of submucous cleft palate: bifid uvula, notching of the posterior border of the hard palate, and muscular diastasis of the soft palate with an intact mucosal layer. Majority Asymptomatic. 15% will develop velopharyngeal insufficiency. Presurgical Orthopedics Goals: Guide the cleft segments into approximation Manipulation of neonatal arch prior to surgical intervention Mold nasal deformities Retrain palatal growth 3 types: Active (Latham-type) retained in mouth with pins in the maxilla

Passive with straps

Passive without straps (NAM)

Surgical Goals and the Benefits of Repair § § § §

The broad goal of cleft palate treatment is to separate the oral and nasal cavities looking for normalize feeding and decrease regurgitation and nasal irritation. Repositioning of the soft palate musculature to anatomically recreate the palate and to establish normal speech. Minimize restriction of growth of the maxilla in both sagittal and transverse dimensions. Improve eustachian tube function and ultimately to hearing.

Essential features for satisfactory soft palate function § Adequate length § Adequate mobility with appropriate resting muscle tension § Midline dorsal convexity that conforms to the dorsal surface of the pharyngeal wall.

Relative Contraindications There are no absolute contraindications for cleft palate repair. Relative contraindications include concurrent 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. Timing of Palatal Closure Controversial. The goals of palatal repair include normal speech, normal palatal and facial growth and normal dental occlusion. Early palate repair: § Better speech results, but early repair also tends to produce severe dentofacial deformities. § Increased technical difficulty of performing the procedure at a younger age and possible adverse effects on maxillary growth. Delayed Repair § Prevent maxillary growth disturbance § Higher Velopharyngeal dysfunction The surgical intervention appears to interfere with midfacial growth without regard to the age of the patient at the time of repair. Bifid uvula occurs in 2% of the population. Although this can occur in association with a 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. Current recommendations § Orthopedic appliance birth - 3 mo § Closure of lip: >3 months/6 months § Closure of palate: >9-12 months § Gingivoperiostoplasty + bone graft: prior to eruption of the permanent canine teeth at the cleft site Cleft Palate Surgery: Techniques The three main categories include: (1) simple palatal closure; (2) palatal closure with palatal lengthening; and (3) either of the first two techniques with direct palatal muscle reapproximation.

Von Langenbeck Palatoplasty § Creation of bipedicle mucoperiosteal flaps with preservation of the anterior part and the greater palatine arteries. § Relaxing incision laterally § Levator detached from insertion § Flaps are then mobilized medially and closed in layers. § The hamulus may need to be fractured to ease the closure. § Close moderate-sized defects. § Disadvantages: o Unnecessary anterior fistulas o No lengthening of palate o Poor speech result: VPI 60%

Veau-Wardill-Kilner: Palatal Lengthening or V-Y Pushback Veauʼs protocol (1) Closure of the nasal layer separately (2) Fracture of the hamular process (3) Staged palatal repair, following primary lip and vomer flap closure (4) Creation of palatal flaps based on a vascular pedicle. Kilner and Wardill (1937): a more radical palatoplasty that became the V-Y pushback. § 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 § V-Y palatal lengthening.

Pushback techniques achieve greater immediate palatal length but at the cost of creating a larger area of denuded palatal bone anterolaterally: Higher incidence of fistula formation.

Intravelar Veloplasty • Designed to lengthen the palate and restore the muscular sling of the levator veli palatini. • Repair of cleft of velum • Realignment of the muscle in the soft palate • Used with vomer flaps and pharyngoplasties Double-Opposing Z-Plasties Described by Furlow in 1986. § Double opposing Z-plasty from the oral and nasal surfaces § Levator included in each posteriorly based mucosal flap § Reorients levator muscles § Palatal lengthening § Hard palate closed with vomer flap § Difficult in wide clefts; transverse shortening inherent in Z-plasty § Perhaps best reserved for secondary procedures

Two-Flap Palatoplasty Bardach (1984) and Salyer • Main goals are complete closure of the entire cleft without tension • Two mucoperiosteal flaps based posteriorly on the greater palatine neurovascular bundle • Reposition of the flaps to their original position • Muscle sling is essential to adequate speech

Vomer Flaps in Palatoplasty Propoed by Veau and Plessier in 1932. Flaps of vomerine mucosa can be useful in the closure of particularly wide clefts and bilateral clefts Concern regarding their effect on facial growth: impair maxillary growth.

Postoperative Management Arm splints in recovery room are applied to prevent the child from disrupting the wound by placing his fingers in his mouth. Parentʼs instructions § It is very important to provide appropriate nutrition after surgery. § Your baby may breast feed normally and immediately after surgery. § To feed your child using other method: Carefully, using a baby bottle, cup, syringe, or the side of a spoon, drop fluid and/or pureed food into the mouth. § Do not allow the spoon to touch the roof of the mouth. Do not allow your child to chew on anything. § After each feeding, give your child water to drink to rinse the mouth area out and to keep it clean. Have your child drink water during the day to rinse the mouth. § Brush your childʼs teeth two times per day with clean water and toothpaste taking special care not to disturb the wound § Do not allow your child to place any objects in mouth, including fingers. Diet: • First week: Full liquid diet. • Second Week: Soft diet of foods that DO NOT require chewing • Third Week: Any food that is not hard or crunch.

Early Complications § Bleeding and respiratory distress. § Dehiscence of the palatal closure: result of poor tissue quality and excessive wound tension. § Oronasal fistula Long-Term Complications §

Palatal Fistula: prealveolar, alveolar and postalveolar. o Problems: food passing into the nasal chamber, speech difficulties secondary to nasal air emission. o Factors that may contribute to fistula: type of cleft, type of repair, wound tension, single-layer repair, dead space below the mucoperiosteal flap and maxillary arch expansion.

Velopharyngeal Incompetence §

16% in large reoperation

studies

need