51 Cleft Lip or Palate

Question 1

Who cares for children with cleft lip and palate?

Answer 1

Who cares for children with cleft lip and palate?

It is generally agreed that a multidisciplinary team best treats children with cleft lip and palate. This team is usually composed of a diverse group of clinicians, including otolaryngologists, plastic surgeons, pediatric dentists, orthodontists, occupational therapists, pediatricians, speech therapists, audiologists, social workers, geneticists, psychologists, and feeding specialists/nutritionists. Each team member provides expertise in an area needed in the treatment of children who are born with a cleft.

Question 2

Are there guidelines regarding the care of children with cleft lip and/or palate?

Answer 2

Are there guidelines regarding the care of children with cleft lip and/or palate?

A report about children with special needs issued in 1987 by the Surgeon General of the United States stressed that the care of children with clefts should be comprehensive, coordinated, culturally sensitive, specific to the needs of the individual, and readily accessible. The Maternal and Child Health Bureau recognized that children with clefts and/or other craniofacial anomalies have special needs and in 1991 provided funding to the American Cleft Palate-Craniofacial Association (ACPA) to develop standards for their health care. As part of these parameters of care, it has been recommended that treatment of cleft and craniofacial conditions occur in a team setting. In 1993, the ACPA released Parameters for Evaluation and Treatment of Patients with Cleft Lip/Palate or Other Craniofacial Anomalies. These parameters have been revised several times, most recently in 2009, and serve as a basis for teams to achieve and maintain accreditation.

Question 3

Summarize the guidelines for the cleft palate team.

Answer 3

Summarize the guidelines for the cleft palate team.

• The team should consist of an operating surgeon, orthodontist, speech-language pathologist, and at least one additional specialist from otolaryngology, audiology, pediatrics, genetics, social work, psychology, and general pediatric or prosthetic dentistry, who meet face to face at least six times per year to evaluate and develop treatment plans for the team’s patients.
• The team should evaluate at least 50 patients per year.
• The team should have at least one surgeon who operates on at least 10 primary cleft lips and/or palates per year.
• The team should coordinate treatment and ensure that a primary care physician evaluates each patient.
• The team should ensure that its members attend periodic continuing education programs about cleft lip and palate.

Figure 51-1 demonstrates example times/ages when specific concerns are most often addressed. -Example timelines for cleft lip and palate care as provided by a multidisciplinary team.

Question 4

Describe the difference in frequency of clefting in regard to race and gender.

Answer 4

Describe the difference in frequency of clefting in regard to race and gender.

The overall reported frequency of children being born with a cleft lip and/or palate is approximately 1 : 700. A higher frequency of cleft lip and palate occurs in Native Americans, those of Asian descent, and those of Latin American descent (1 : 400). The lowest frequency is reported in African Americans (1 : 1500 to 2000). Cleft palate alone is fairly consistent among ethnic groups at 1 : 2000. There is a male predominance in cleft lip and palate, and a female predominance in cleft palate alone.

Question 5

What are some of the causes of cleft lip/palate?

Answer 5

What are some of the causes of cleft lip/palate?

Clefts can be generally classified as syndromic or nonsyndromic. Single gene transmissions, chromosomal aberrations, teratogenic effects, or environmental exposures can cause syndromic clefting. Over 400 syndromes are associated with cleft lip/palate.

Nonsyndromic clefts have a non-Mendelian inheritance pattern. There is not a clear understanding of the factors involved in the occurrence of cleft lip/palate. Concordance rates in monozygotic and dizygotic twins are 40% to 60% and 5%, respectively. These findings indicate a major genetic component, but environmental factors are also implicated. Recurrence rates for cleft lip/palate and isolated cleft palate range from 1% to 16% in cases of families with children born with nonsyndromic cleft lip and/or palate.

Question 6

Name some of the more common syndromes in which cleft lip/palate is a characteristics.

Answer 6

Name some of the more common syndromes in which cleft lip/palate is a characteristics.

• Apert’s syndrome
• Stickler’s syndrome
• Treacher Collins syndrome
• 22q11 deletion syndrome (previously named velocardiofacial syndrome, Sphrintzen syndrome, or DiGeorge complex syndrome)
• Van der Woude syndrome
• Goldenhar syndrome or hemifacial microsomia

Question 7

What is Pierre Robin or Robin (pr. Rō-bāń) sequence?

Answer 7

What is Pierre Robin or Robin (pr. Rō-bāń) sequence?

Pierre Robin sequence was first described in the early 1800’s but bears the name of Robin, a French stomatologist who wrote extensively on and drew attention to the constellation of findings beginning in 1923. It is usually described as micrognathia (small mandible), relative glossoptosis (tongue of normal size, but relatively large compared to the small mandible), and airway obstruction. The constellation of findings is thought to occur from a single embryologic event, which occurs between 6½ to 10 weeks of embryologic development resulting in a small mandible. The relative macroglossia causes the tongue to sit high and posterior in the oropharynx, leading to upper airway obstruction at birth. A wide U-shaped cleft palate is present in most, but not all, patients due to inability of the palate to close normally because the tongue impedes it. Robin sequence is rarely isolated and can occur in a variety of craniofacial syndromes.

Question 8

What is the ratio of cleft lip to cleft lip and palate?

Answer 8

What is the ratio of cleft lip to cleft lip and palate?

Cleft lip and palate is the most common occurrence, accounting for 50% of patients. Left unilateral cleft lip and palate is the most common, followed by right unilateral cleft lip and palate, and then bilateral cleft lip and palate. Cleft palate alone occurs in 35% and is more often syndromic than cleft lip and palate or cleft lip alone. Cleft lip alone occurs in 15%.

Question 9

Distinguish between complete cleft lip and incomplete cleft lip.

Answer 9

Distinguish between complete cleft lip and incomplete cleft lip.

The distinction between complete and incomplete cleft lip is controversial. Generally, a complete cleft lip is defined as a cleft with muscular diastasis of the orbicularis oris. This condition can usually be best determined by observing nostril symmetry or appearance with facial movement. A complete cleft may be present with a Simonart’s band.

Question 10

What is a Simonart’s band?

Answer 10

What is a Simonart’s band?

A Simonart’s band is a thin remnant of tissue in the floor of the nasal vestibule bridging the medial and lateral lip elements across the cleft. The tissue may consist of skin and/or mucosa and subcutaneous tissue with or without a small amount of muscle fibers. The origin of the term is obscure, but many attribute it to Pierre Joseph Cécilien Simonart, a Belgian obstetrician (1817–1847).

Question 11

What is the primary palate? The secondary palate?

Answer 11

What is the primary palate? The secondary palate?

The primary and secondary palates are separated by the incisive foramen. The primary palate consists of the lip, alveolar arch, and palate anterior to the incisive foramen (the premaxilla). The secondary palate consists of the soft palate and the hard palate posterior to the incisive foramen (Figure 51-2).

Question 12

How is the primary palate formed?

Answer 12

How is the primary palate formed?

Primary palate formation occurs between weeks 4 and 7. The development of the primary palate is largely complete prior to formation of the secondary palate. During week 4 the frontonasal prominence forms, including nasal placodes. The nasal placode consists of ectodermal thickenings located on the lateral aspect of the prominence. By week 5 the frontonasal prominence elevates and forms medial and lateral nasal prominences around the nasal placode. Next, the placode invaginates and forms nasal pits. During weeks 6 and 7, the maxillary prominences enlarge and grow medially. This growth forces the medial nasal prominences toward the midline. With the fusion of both medial nasal prominences, the tip of the nose, central upper lip, and philtrum of the upper lip are formed. The lateral portion of the upper lip and maxilla are formed by fusion of the medial nasal prominence and maxillary prominence. Finally, the nasal alae are formed by fusion of the lateral nasal prominences with the maxillary prominence.

Question 13

How is the secondary palate formed?

Answer 13

How is the secondary palate formed?

Formation of the secondary palate occurs between weeks 6½ and 10 in three stages: growth, shelf elevation, and fusion. First, outgrowths from the maxillary processes extend vertically downward along the tongue. Next, the shelves quickly assume a horizontal position above the tongue. The palatal shelves then fuse, forming an intact secondary palate.

Question 14

Describe the embryologic failures in regard to the formation of a cleft lip and palate.

Answer 14

Describe the embryologic failures in regard to the formation of a cleft lip and palate.

A cleft lip is due to failure of fusion between the medial nasal prominence and the maxillary prominence, lateral nasal prominence, or both. Cleft palate formation is hypothesized to result from one of the following: defects in palatal shelf growth, delayed or failed shelf elevation, defective shelf fusion, failure of medial epithelial seam cell death, or failure of mesenchymal consolidation and differentiation.

Question 15

What is a submucous cleft? How is it diagnosed?

Answer 15

What is a submucous cleft? How is it diagnosed?

A submucous cleft is a muscular diastasis in the palate with intact overlying mucosa. A submucous cleft is classically characterized by the triad of bifid uvula, a midline furrow along the length of the soft palate due to abnormal muscle insertion (zona pellucida), and a notch in the posterior margin of the hard palate. Submucous cleft palate can usually be seen on physical examination or nasopharyngoscopy. A midline furrow of the nasal surface of the posterior palate seen during phonation is a classic endoscopic finding.

Question 16

List some characteristics of the classic cleft nose deformity.

Answer 16

List some characteristics of the classic cleft nose deformity.

• Shortened columella with its base angled to the noncleft side
• Nasal spine deviation to the noncleft side, with a similar deflection of the caudal septum toward the noncleft side and compensatory hypertrophy of the cleft side inferior turbinate
• The lower lateral cartilage of the nose on the cleft side is rotated or displaced laterally in the nasal tip with the medial crura collapsed inferiorly and the lateral crura collapsed and buckled.
• Deflection of the nasal tip toward the cleft side due to the above deficiencies
• Relative stenosis or collapse of the nasal valve on the cleft side
• Hypoplastic maxilla on the cleft side, causing lateralization of the alar base and widening of the nares
• Broad nasal dorsum
• Horizontal as opposed to vertical nostril orientation, as seen on basal view

Question 17

What specific role does the otolaryngologist play in the care of a child with cleft lip/palate?

Answer 17

What specific role does the otolaryngologist play in the care of a child with cleft lip/palate?

In some institutions, the otolaryngologists perform all surgical procedures involving cleft lip/palate repair, correction of velopharyngeal insufficiency (VPI), alveolar bone grafting, rhinoplasty, and orthognathic surgery. In other institutions, the plastic surgeon performs some or all of the previously listed procedures except for management of ear disease associated with cleft palate. Oral and maxillofacial surgeons may be involved in or perform alveolar bone grafting and orthognathic surgery procedures. A pediatric otolaryngologist should be involved with the complex feeding and airway issues that often occur in children born with cleft lip and/or palate.

Question 18

List the initial priorities for managing a newborn with cleft lip/palate.

Answer 18

List the initial priorities for managing a newborn with cleft lip/palate.

As in any newborn, airway management is of primary importance. Feeding and achieving adequate nutrition is the second priority for any newborn, including infants with cleft lip/palate (see Question 20).

Question 19

Discuss airway management in newborns with cleft lip/palate.

Answer 19

Discuss airway management in newborns with cleft lip/palate.

For a child born with Pierre Robin sequence or more complex craniofacial anomalies, airway management is of much greater concern. Such children may have few signs of upper airway obstruction; however, severely affected children may require immediate attention. Intervention may be as simple as prone positioning, which is most effective in nonsyndromic cases. Surgical techniques for management are more often required in syndromic patients and may include glossopexy or tongue-lip adhesion (primarily of historical interest, since its effectiveness is controversial), nasal airway placement, mandibular distraction osteogenesis, or tracheostomy tube placement.

Question 20

Describe the approach to feeding in infants with cleft lip/palate.

Answer 20

Describe the approach to feeding in infants with cleft lip/palate.

Patients with cleft lip only may require little or no intervention, and many are able to breast-feed or use regular bottle nipples. Children with cleft palate or cleft lip/cleft palate are at a disadvantage, given the anatomic and functional deficits caused by a cleft. The inability to generate negative pressure within the oral cavity secondary to palatal insufficiency can lead to the expenditure of too much energy for feeding, long feeding times, and subsequent poor weight gain, or even dehydration. Strategies developed to assist these children include special feeding systems (e.g., Medela Special Needs Feeder, formerly called Haberman feeder, Mead Johnson squeeze bottle, Pigeon feeder, Dr. Brown’s nipple/bottle) and the fabrication of a palatal obturator or prosthetic. The obturator not only acts to aid in feeding but can also be used to help reposition the protruded premaxilla, lengthen the columella, reposition lateral maxillary segments, and reshape the nostril (see Question 24).

Question 21

Discuss the underlying pathophysiology of middle ear disease in a child with cleft lip/palate.

Answer 21

Discuss the underlying pathophysiology of middle ear disease in a child with cleft lip/palate.

Studies since the 1960s have implicated eustachian tube dysfunction as the main cause of ear disease. Multiple investigations have supported the hypothesis that the eustachian tube in a cleft child is unable to open properly and ventilate the middle ear because the muscles of the palate are involved in eustachian tube physiology. When these muscles are abnormal, such as in a cleft palate, they cannot function normally to open and close the eustachian tube.

Question 22

What percentage of children with cleft palate have middle ear disease? How is it treated?

Answer 22

What percentage of children with cleft palate have middle ear disease? How is it treated?

Virtually all (≥90%) children younger than 2 years of age with an unrepaired cleft have an effusion of the middle ear. Persistence of an effusion in young children leads to variable levels of hearing loss. Hearing loss during early childhood may lead to difficulties in speech and language development. The majority of centers treating children with clefts recommend tube placement during the first year of life or sooner if effusions become infected or hearing is markedly impaired. During either cleft lip or palate repair, the first set of tubes is often placed.

Question 23

When is the optimal time to perform a cleft lip repair?

Answer 23

When is the optimal time to perform a cleft lip repair?

Commonly, the cleft lip is repaired between the ages of 2 and 6 months. After 10 weeks of age (corrected for prematurity if necessary) there is a decrease in respiratory complications following general anesthesia. The classic “rule of 10s” is often used at many centers. This rule requires children to be at least 10 weeks old, to weigh at least 10 pounds, and to have a hemoglobin level of 10 mg/dL. Although these criteria are commonly used, they are largely of historical interest and are not based on scientific data. Efficient feeding, proven weight gain, and good general health are much more important factors in successful surgical repair. Obviously, other congenital anomalies, such as congenital heart disease, must take precedence over cleft repair.

Question 24

What is presurgical nasoalveolar molding (NAM)?

Answer 24

What is presurgical nasoalveolar molding (NAM)?

Presurgical nasoalveolar molding (NAM or pNAM) is basically presurgical orthodontics. An anterior palatal obturator is fabricated. The obturator may help in feeding. The obturator is then progressively modified to move the lateral maxillary segments. A protuberant premaxilla can be moved posteriorly, and attachments can be added to lengthen the short columella. Nasoalveolar molding is most beneficial in wide complete bilateral clefts of the lip and palate, but increasing use is being seen in unilateral clefts.

Question 25

What is a cleft lip adhesion?

Answer 25

What is a cleft lip adhesion?

In certain cases of wide unilateral or bilateral cleft lip, some surgeons perform a cleft lip adhesion. This procedure is a staged lip repair in which the first stage (adhesion) involves reapproximating medial and lateral lip elements and the orbicularis oris muscle. The first stage converts a complete cleft, either unilateral or bilateral, to a more easily repaired incomplete cleft. The second stage then uses one of the techniques in Question 26 as the formal lip repair.

Question 26

List the techniques used for formal repair of unilateral cleft lip.

Answer 26

List the techniques used for formal repair of unilateral cleft lip.

• Millard rotation-advancement repair: The medial lip element is rotated inferiorly and the lateral lip element is advanced into the resulting upper lip defect. The columellar flap is then used to lengthen the columella or create the nasal sill.
• Tennison-Randell repair: The medial lip element is lengthened by introduction of a triangular flap from the inferior portion of the lateral lip element.
• Hagedorn-LeMesurier repair: A quadrilateral flap developed from the lateral lip element is introduced to lengthen the medial lip element.
• Rose-Thompson repair: Curved or angled paring of the cleft margins is used to lengthen the lip as a straight-line closure.
• Skoog repair: The medial lip element is lengthened through the introduction of two small triangular flaps developed from the lateral lip element.

Question 27

List the techniques used for formal repair of bilateral cleft lip.

Answer 27

List the techniques used for formal repair of bilateral cleft lip.

• Millard’s repair involves complete elevation of the prolabium and reconstitution of the orbicularis across the premaxilla. In addition, Millard banked lateral segments of the prolabium as “forked flaps” that were meant to add columellar height at a later stage. The banking of fork flaps has recently fallen out of favor with more emphasis on primary columellar lengthening and rhinoplasty.
• Veau repair: The Veau operation is a straight-line closure without elevation of the prolabial skin and correspondingly without any attempt to restore the continuity of the orbicularis oris, which is not favored because this results in incompetence of the oral sphincter.
• Manchester repair: Manchester preferred to maintain the prolabial vermilion to create the cupid’s bow and tubercle, but similarly to Veau, Manchester did not repair the orbicularis because he thought it would create an overly tight lip.

Question 28

Describe recent developments in nasal and lip repair.

Answer 28

Describe recent developments in nasal and lip repair.

In recent years, significant contributions by McComb, Mulliken, Nakajima, Nordhoff, and Cutting have integrated the correction of the associated nasal deformity with simultaneous lip repair that appears to achieve adequate primary columellar lengthening and nasal tip projection. Greyson and Cutting have introduced presurgical molding of the nasal tip and columella with acrylic outriggers, orthodontic elastics, and tapes attached to a palatal appliance (see Question 24).

Question 29

When should cleft palates be repaired?

Answer 29

When should cleft palates be repaired?

Timing of cleft palate repair is much more controversial than that of cleft lip repair. The goals of cleft palate repair include attaining normal long-term speech and avoiding deleterious effects on future facial growth. Patients with unrepaired clefts have the least amount of abnormal growth, but the speech impact of this approach is unacceptable. Recent research into speech development supports cleft palate repair before the age of 12 months but for optimum outcomes some centers recommend closure much earlier, at 7 to 12 months of age. Early repair avoids compensatory techniques that may impair speech and be difficult to unlearn. Studies have shown that children with clefts repaired before 12 months have improved speech outcomes compared with children who undergo repair closer to 24 months. Long-term follow-up has found no significant impact on growth in children with earlier repaired clefts.

Question 30

Name the more common methods of cleft palate repair.

Answer 30

Name the more common methods of cleft palate repair.

• Two-flap palatoplasty
• Wardill-Kilner V-Y advancement
• Von Langenbeck palatoplasty
• Furlow double-opposing Z-plasty

Question 31

List possible postoperative complications of cleft palate repair.

Answer 31

List possible postoperative complications of cleft palate repair.

• Bleeding
• Fistula (5% to 35%)
• VPI
• Postoperative upper airway obstruction

Question 32

What is velopharyngeal insufficiency?

Answer 32

What is velopharyngeal insufficiency?

VPI is hypernasality during speech or reflux of saliva or food into the nasopharynx during swallowing. VPI occurs when the nasopharynx and oropharynx are not successfully separated by complete palatal closure during certain speech sounds or during swallowing. Persistent VPI occurs in about 10% to 40% of children after cleft palate repair.

Question 33

How is VPI treated?

Answer 33

How is VPI treated?

An experienced speech and language pathologist should evaluate the presence, specific cause, and severity of VPI, often in conjunction with an imaging technique such as video nasopharyngoscopy or video fluoroscopy. Both medical and surgical methods for correction of VPI are available. Medical management includes speech therapy and oral appliances that aid in correcting the underlying problem. The majority of children undergo speech therapy before being referred for surgical management. Surgical management includes a variety of procedures aimed at attaining separation of the oropharynx from the nasopharynx during speech. Specific procedures include the pharyngeal flap, sphincter pharyngoplasty, Furlow palatoplasty, and posterior pharyngeal wall augmentation.

Question 34

What are concerns as children reach older ages?

Answer 34

What are concerns as children reach older ages?

As previously stated, the treatment of cleft lip and palate is best directed by a multidisciplinary team. Figure 51-1 demonstrates example times/ages when specific concerns are addressed.

• As children reach elementary school age, dental and speech needs become more of a concern.
• In late elementary school and early middle school, orthodontic and alveolar cleft repair are usually addressed.
• In high school age patients, orthodontics and secondary cosmetic surgical procedures are addressed.
• At skeletal maturity (16 to 18 years in females, 18 to 21 years in males), orthodontics and orthognathic surgical procedures are addressed.

Question 35

What are adult concerns for patients with cleft lip and palate anomalies?

Answer 35

What are adult concerns for patients with cleft lip and palate anomalies?

It is the goal of team care that the majority of functional and cosmetic concerns will be address by the time a patient reaches adult age. In some instances, other circumstances prevent the address of all concerns by this time. Functional and cosmetic concerns can be addressed at any age, even in adults. Many adults born with cleft and craniofacial disorders will choose medical and/or surgical treatments to address these concerns.

Question 36

How is rhinoplasty approached in the child with cleft lip/palate?

Answer 36

How is rhinoplasty approached in the child with cleft lip/palate? Controversy

The approach to correction of the cleft nose is controversial. Some experts recommend immediate or primary correction with cleft lip repair, whereas others who are concerned with potential disrupted growth recommend a much later repair when patients are in their late teens. Advocates of primary repair found that early repair creates better symmetry and hence symmetric growth. They also claim there is much less psychological stress with earlier repair. Advocates of later definitive repair claim that they avoid any potential disturbances in growth, produce less scarring, and avoid multiple surgeries due to unexpected changes caused by growth. Recently there has been increased support for limited, primary rhinoplasty at the time of initial lip repair.

Question 37

What are the advantages of fetal repair of cleft lip/palate?

Answer 37

What are the advantages of fetal repair of cleft lip/palate? Controversy

Fetal surgery began in 1981 for life-threatening anomalies (e.g., diaphragmatic hernia). As fetal surgical techniques have improved, the scope of fetal surgery has expanded into the head and neck area. Securing the airway in cases of laryngeal atresia and large cervical/facial tumors has garnered the most interest. On the fringe of this interest, and highly controversial, is the desire to correct cleft lip/palate deformities in utero. The proposed advantages of this undertaking include scar-free wound healing if performed at the proper time, as well as interruption and correction of the associated facial maldevelopments that result from cleft lip/palate.

Question 38

What are the disadvantages of fetal repair?

Answer 38

What are the disadvantages of fetal repair? Controversy

The disadvantages involve the risks to fetus and mother during the procedures. Until the limitations of fetal surgery are overcome (e.g., preterm labor, fetal demise, technical limitations), mainstream medicine will not accept such a procedure to repair a nonlethal deformity.