Cleft Lip and Palate

Question 1

Which of the following structures arises from the lateral nasal processes during embryologic development?
A) Columella
B) Nasal ala
C) Nasal septum
D) Nasal tip
E) Premaxilla

Answer 1

B) Nasal ala

Question 2

When do nasal structures form?

Answer 2

6th week of gestation

Question 3

What do the nasal pits form from?

Answer 3

The nasal places; these eventually deepen as a result of the formation of the medial and lateral nasal prominences.

Question 4

What do the medial nasal processes form?

Answer 4

The MNP give rise to the nasal tip, columella, philtrum, and premaxilla.

Question 5

What does the nasal septum arise from?

Answer 5

The nasal septum is a downgrowth from the merged medial nasal prominences.

Question 6

What does the frontal nasal process form?

Answer 6

The forehead, the bridge of the nose, and the root of the nose come from the FNP

Question 7

What do the nasal alae derive from?

Answer 7

The lateral nasal processes

Question 8

A newborn is evaluated because of a cleft of the soft and hard palates that extends to the incisive foramen. Which of the following is the most appropriate Veau classification of this cleft?
A) I
B) II
C) III
D) IV

Answer 8

B) II

Question 9

Veau Classification overview

Answer 9

The Veau classification divides the cleft palate into four categories and is a widely utilized scheme for cleft palate classification.

Question 10

Veau Class I

Answer 10

Veau Class I is an incomplete cleft involving the soft palate.

Question 11

Veau Class II

Answer 11

The Veau II cleft involves the hard and soft palate and is limited to the secondary palate.

Question 12

Veau Class III

Answer 12

A Veau III is a complete unilateral cleft lip and palate, where the nasal septum fails to fuse with one palatal shelf.

Question 13

Veau Class IV

Answer 13

A Veau IV is a complete bilateral cleft lip and palate in which both palatal shelves fail to fuse with the nasal septum.

Question 14

The 3-month-old infant shown has a bilateral cleft lip and palate. Which of the following is a goal of the nasoalveolar molding seen in the photograph?
A) Expansion of the alveolar cleft
B) Expansion of the soft tissue of the lip
C) Lengthening of the columella
D) Lengthening of the septum

Answer 14

C) Lengthening of the columella

The alveolar clefts are aligned and narrowed. Generally the septum is in the midline and does not require any lengthening. The upper cleft lip does not require expansion; rather, it requires layered repair with good muscular approximation at the time of the primary lip and nasal repair.

Question 15

What is the purpose of NAM?

Answer 15

NAM allows alignment of the premaxilla with the lateral alveolar segments. Once this is accomplished, nasal stents are placed to lengthen the columella.

Question 16

A 21-year-old man comes to the office for evaluation of a secondary palate fistula following palatoplasty 20 years ago. History includes three unsuccessful attempts at fistula repair using local tissue. Repair using a tongue flap to provide soft tissue to the palate is planned. Blood supply runs primarily in which of the following regions of the tongue?
A) Ventral third
B) Middle third
C) Dorsal third
D) Dispersed throughout

Answer 16

A) Ventral third

For procedures on the tongue, it is imperative to understand the location of the major vascular channels. The primary blood supply is afforded by the lingual arteries. The paired lingual arteries run laterally, primarily within the ventral third of the tongue. Additional vascularity is supplied by the facial and ascending pharyngeal arteries. Vessels that cross from one side to the other exist primarily at the tip with the median septum being relatively avascular.

Question 17

What is the primary blood supply of the tongue?

Answer 17

The primary blood supply is afforded by the lingual arteries. The paired lingual arteries run laterally, primarily within the ventral third of the tongue. Additional vascularity is supplied by the facial and ascending pharyngeal arteries.
Vessels that cross from one side to the other exist primarily at the tip with the median septum being relatively avascular.

Question 18

A 16-year-old boy is referred for consultation regarding treatment of maxillary retrusion and an Angle class III malocclusion. History includes repair of a complete unilateral cleft lip and palate in infancy. Maxillary advancement is planned after completion of facial growth. Completion of facial growth is best determined by which of the following?
A) Chronologic age of the patient
B) Complete eruption of the second molars
C) Hand-wrist x-ray study
D) Serial cephalometric x-ray study

Answer 18

D) Serial cephalometric x-ray study

Serial cephalometric x-ray study is a reliable method to determine completion of maxillofacial growth in adolescents.

Ossification of bones of the wrist and hand is normally the standard for assessing skeletal development. However, its validity in the examination of craniofacial growth has recently come into question. Dental development indicators are not reliable predictors of an individual’s stage of skeletal development.

Question 19

What is the optimal method to determine completion of maxillofacial growth in adolescents?

Answer 19

Serial cephalometric x-ray study

Question 20

Ossification of bones of the wrist and hand vs craniofacial development

Answer 20

Ossification of bones of the wrist and hand is normally the standard for assessing skeletal development. However, its validity in the examination of craniofacial growth has recently come into question.

Question 21

A 16-month-old boy who underwent correction of a complete unilateral cleft lip 2 months ago is brought to the office because his mother is concerned about the appearance of the scar on his lip. Physical examination shows a corrected upper lip with a good pout and contour. The scar is flat, slightly widened, and moderately erythematous. Which of the following is the most appropriate management at this time? 
A ) Continued optimal scar management 
B ) Immediate revision of the scar
C ) Laser resurfacing of the scar 
D ) Revision of the scar in 4 months

Answer 21

A ) Continued optimal scar management

The patient described displays a good result after unilateral cleft lip repair. However, the mother is overly concerned about the appearance of the scar, and she needs to be reassured about the result. She should be reeducated concerning good scar care, which includes use of a strong sunblock, and massage of the scar. Even if the scar were a bad one, the best option at this early stage would be optimal scar care. Revision of scars in children earlier than 12 months is generally not advisable, as they typically continue to improve during this time.

Question 22

How long before revision of a scar in a child can be considered?

Answer 22

At least 12 months, s they typically continue to improve during this time.

Question 23

A 25-year-old woman who is pregnant with her second child comes to the office for consultation regarding the risk that the child will have a cleft lip and palate. She has a history of a cleft lip and palate, and her first child also has a cleft lip and palate. The father has no history of cleft lip or palate. Which of the following best represents the likelihood that the patient's second child will be born with a cleft lip and palate?
A ) 2%
B ) 4%
C ) 5% 
D ) 10%
E ) 14%

Answer 23

E ) 14%

An affected parent with one affected child has a 14% risk for future offspring to have a cleft lip and palate. If both parents are not affected, and their first child has a unilateral defect, the risk would be 2.7% for the next child and 5.4% if the first child had a bilateral defect. If both parents were unaffected and had two affected children, then the risk for the subsequent pregnancy to result in a cleft lip and palate would be 10%

Question 24

Which of the following muscles is used to construct the sphincter during a sphincter pharyngoplasty for the treatment of velopharyngeal insufficiency? 
A ) Levator veli palatini 
B ) Musculus uvulae 
C ) Palatopharyngeus 
D ) Superior constrictor 
E ) Tensor veli palatini

Answer 24

C ) Palatopharyngeus

Question 25

Routine treatment of postoperative velopharyngeal insufficiency

Answer 25

Posterior pharyngeal flap or spincter pharyngoplasty

Question 26

Description of sphincter pharyngoplasty

Answer 26

A sphincter pharyngoplasty is performed by taking the posterior tonsillar pillar, containing the palatopharyngeus muscle, and elevating it inferiorly to superiorly. The elevated posterior tonsillar pillar, pedicled superiorly, is rotated 90 degrees medially, positioned side by side, and sewn into an incision made horizontally on the posterior pharyngeal wall at the level of the adenoid pad.

Question 27

Levator veli palatini in the normal palate

Answer 27

The levator veli palatini muscle, in the normal palate, rests horizontally (coronally) within the middle third of the velum (soft palate) and functions as the motor of the velum. This muscle pulls the soft palate posteriorly and superiorly, allowing for apposition of the free edge of the velum against the posterior pharyngeal wall (velopharyngeal competence)

Question 28

Musculi uvulae anatomy / function

Answer 28

The musculi uvulae arise as paired slips from the palatine aponeurosis and course sagittally in the velar midline, ending in the uvula. The musculi uvulae act as a flexible beam, providing a stiffness-modifying mechanism for the velum

Question 29

Superior pharyngeal constrictor anatomy

Answer 29

The superior pharyngeal constrictor arises from the hamulus and course sagittally along the lateral pharyngeal walls to decussate in the midline of the posterior pharyngeal wall. The superior pharyngeal constrictor is the muscle from the posterior pharyngeal wall that is utilized in the posterior pharyngeal flap.

Question 30

Tensor veli palatini muscle anatomy

Answer 30

The tensor veli palatini muscle arises from the skull base and courses inferiorly and medially around the hook of the hamulus and into the palate as the tensor aponeurosis. It joins the velum (soft palate) to the hard palate.

Question 31

A 2-year-old boy with a cleft palate and recurrent episodes of serous otitis media caused by impaired eustachian tube function is scheduled to undergo cleft repair. In reconstructing the palate, repair of which of the following muscles is most likely to improve eustachian tube function? 
A ) Levator veli palatini 
B ) Palatopharyngeus 
C ) Salpingopharyngeus 
D ) Superior constrictor 
E ) Tensor veli palatini

Answer 31

A ) Levator veli palatini

In cleft palate reconstruction, repair of the levator veli palatini can improve eustachian tube function. Individuals with unrepaired cleft palate suffer chronic otitis media, which can lead to permanent hearing loss. This is thought to be caused by the dysfunction of the eustachian tube. There are several paratubal muscles that are responsible for the opening and closing of the eustachian tube: the tensor veli palatini, the levator veli palatini, and the salpingopharyngeus.

Question 32

What can improve function of the eustachian tube in patients with cleft palates?

Answer 32

Repair of the levator veli palatini

Question 33

Muscles responsible for opening and closing the eustachian tube

Answer 33

There are several paratubal muscles that are responsible for the opening and closing of the eustachian tube: the tensor veli palatini, the levator veli palatini, and the salpingopharyngeus

Question 34

What actions open the eustachian tube?

Answer 34

Contraction of the levator veli palatini with superior and posterior displacement of the levator sling opens the eustachian tube.

Question 35

How is opening of the eustachian tube lost in cleft palate?

Answer 35

In an unrepaired cleft palate, the ability of the levator veli palatini to open the tube is lost because of its abnormal insertion on the posterior hard palate. Repositioning of this muscle during cleft palate repair restores the levator sling, allowing dilation of the eustachian tube.

Question 36

Least important muscle in opening the eustachian tube

Answer 36

The salpingopharyngeus also opens and closes the eustachian tube. However, because of its small size, it is the least important of the paratubal muscles and has minimal functional significance

Question 37

Function of the palatopharyngeus muscle

Answer 37

The palatopharyngeus muscle optimizes velopharyngeal closure. Along with the superior constrictor, it causes medial displacement of the lateral pharyngeal wall.

Question 38

Function of the superior constrictor muscle

Answer 38

The superior constrictor is the main component of the Passavant ridge and functions to bring about medial displacement of the lateral pharyngeal wall through a sphincteric mechanism.

Question 39

A 1-year-old boy is brought to the office for evaluation of cleft lip and palate and congenital pitting of the lower lip. Examination shows complete unilateral cleft lip and palate deformity and small sinuses in the lower lip. Which of the following is the most likely diagnosis?
A ) Pierre Robin sequence
B ) Stickler syndrome
C ) Van der Woude syndrome
D ) Velocardiofacial syndrome
E ) Waardenburg syndrome

Answer 39

C ) Van der Woude syndrome

Question 40

Waardenburg syndrome

Answer 40

Waardenburg syndrome is associated with a white forelock of hair and sensorineural hearing loss. It has been reported to cause cleft lip/palate.

Question 41

The parents of the 3-month-old male infant shown come to the office for consultation. The parents have another child, who also has a cleft lip and palate (CL/P). Neither parent has CL/P. Which of the following best represents the likelihood that their third child will have CL/P?
A ) 2% 
B ) 4% 
C ) 9% 
D ) 17% 
E ) 50%

Answer 41

C ) 9%

The risk of having a child with a cleft lip and palate (CL/P) is dependent on multiple factors. Familial cases have a risk that is dependent on family history, parental involvement, and other sibling involvement. Risks can be categorized as follows:Normal parents, one child with a CL/P: the risk for the next child is 4% (as in the scenario described). Normal parents, two children with a CL/P: the risk for the next child is 9%. Affected parent with a CL/P, no affected children: the risk for the next child is 4%. Affected parent with a CL/P, one child with a CL/P: the risk for the next child is 17%. Affected parent with a CL/P and lip pits who has van der Woude syndrome: the risk for an affected child would be 50%, as this is an autosomal dominant condition

Question 42

A 9-month-old female infant is scheduled to undergo repair of a cleft palate. Repair of which of the following intravelar muscles is necessary to achieve adequate postoperative velopharyngeal competence in this patient?
A ) Levator veli palatini
B ) Musculi uvula
C ) Palatoglossus
D ) Superiorconstrictor
E ) Tensor veli palatini

Answer 42

A ) Levator veli palatini

Question 43

Velum

Answer 43

Soft palate

Question 44

Palatoglossus muscle

Answer 44

The palatoglossus muscle is the muscle found in the anterior tonsillar pillar, arising from the soft palate and inserting into the side and dorsum of the tongues. The palatoglossus muscle elevates the posterior part of the tongue toward the palate and depresses the soft palate toward the dorsum of the tongue.

Question 45

A 31-year-old woman comes to the office with her husband for consultation about the risk that their child will have a cleft lip deformity. Neither of them have a cleft lip deformity, but they have a 2-year-old child with an isolated unilateral cleft lip deformity. Which of the following percentages best represents the risk that this couple will have another child with a cleft lip deformity?
(A)2%
(B)4%
(C)9%
(D)15%
(E)22%

Answer 45

(B)4%

Question 46

Isolated cleft palate is genetically distinct from:

Answer 46

Isolated cleft palate is genetically distinct from isolated cleft lip with or without cleft palate.

Question 47

Family members and likelihood of another child - CL and CL/P vs CP

Answer 47

CL and CL/P vs CP:

1 Child: 4% vs 2%
1 Parent: 4% vs 6%
1 Child 1 Parent: 17% vs 15%
2 Children: 9%

Question 48

A 16-year-old girl who has had repair of bilateral cleft lip, nose, and palate, and a pharyngeal flap has the occlusion shown in the photograph and radiograph. She has 12 mm of negative overjet. Cephalometric evaluation shows no mandibular abnormalities. Which of the following is the most appropriate procedure for aesthetic correction of the malocclusion?
(A)Le Fort I maxillary advancement
(B)Le Fort I maxillary distraction osteogenesis
(C)Mandibular setback with advancement genioplasty
(D)Segmental maxillary advancement
(E)Two-jaw maxillary advancement and mandibular setback

Answer 48

(B)Le Fort I maxillary distraction osteogenesis

For children with orofacial clefting and severe Class III malocclusion, maxillary advancement is the procedure of choice. When there is a large maxillary move to be performed (greater than 6 to 8 mm), and there has been prior surgery resulting in scarring in the region (cleft lip, cleft palate, alveolar bone graft, pharyngeal flap), large movements of the maxilla are challenging and result in significant relapse. n addition, when there is normal shape and position of the mandible, surgery on the mandible is not indicated.

Question 49

Historically, what was done to correct severe Class III malocclusion for children with orofacial clefting?

Answer 49

Historically, because of the difficulty in performing the large required movements of the maxilla, the “difference” of movement would be shared between the maxilla and mandible. For a 12-mm movement, 6 mm of anterior movement of the maxilla would be performed, and the mandible would be set back 6 mm. Today, with the option of mid-face distraction, the appropriate aesthetic large movements of the maxilla can be performed to spare unnecessary surgery on the mandible.

Question 50

A 25-year-old man who underwent repair of unilateral cleft lip and palate comes to the office for consultation regarding a dentofacial deformity. On cephalometric analysis, the SNA angle is 70 degrees (N 81.2 degrees), the SNB angle is 80 degrees (N 77.3 degrees), and 18-mm negative overjet is noted. Which of the following is the most appropriate management for correction of this deformity?
(A)Le Fort I advancement and jumping genioplasty
(B)Le Fort I advancement and sagittal split osteotomy advancement
(C)Le Fort I advancement and sagittal split osteotomy setback
(D)Le Fort II advancement
(E)Le Fort III advancement and mandibular setback

Answer 50

(C)Le Fort I advancement and sagittal split osteotomy setback

The patient shown below has maxillary retrusion and mandibular prognathism, which is most appropriately treated with a maxillary (Le Fort I) advancement and a sagittal split osteotomy (mandibular setback).

The Angle class III malocclusion is demonstrated in photograph, and cephalometric analysis shows overprojection of the mandible and underprojection of the maxilla. Le Fort I advancement also could be attempted but would be difficult for such a discrepancy in occlusion; bimaxillary prognathism would result.

Question 51

Another name for sagittal split osteotomy

Answer 51

Mandibular setback

Question 52

A 24-year-old man comes to the office with his wife to request information about their risk for having a child with cleft lip and palate. The man underwent repair of an isolated cleft lip and palatein infancy. His mother also had an isolated cleft lip and palate and underwent removal of salivary gland pits from her lower lip. The patient’s wife has no family history of cleft lip and palate. Which of the following percentages best represents the likelihood that this couple will have a child with a cleft deformity?
(A)5
(B)10
(C)25
(D)50
(E)80

Answer 52

(D)50

Families with van der Woude syndrome (cleft lip/palate and lip pits) are known to have an autosomal dominant clefting mutation. The patient described has a 50% chance of transmitting the cleft locus to a child.

Question 53

A 24-year-old man who underwent repair of isolated cleft lip and palate in infancy comes to the office with his wife for consultation regarding the risk of their children having cleft deformities because they are planning their first pregnancy. The patient’s family history includes isolated cleft lip and palate in his mother. The patient’s wife has no congenital deformities and has no known family history of cleft palate. Which of the following percentages best represents the chance that this couple will have a child with a cleft deformity?
(A)5%
(B)10%
(C)15%
(D)25%

Answer 53

(A)5%

Although there are cases in which clefting does transmit in an autosomal-dominant or autosomal-recessive fashion, most isolated cleft lip/palate deformities follow a multifactorial model of genetic transmission. Therefore, the risk that this patient will have a child with a cleft is only slightly higher than the general population.

Question 54

Which of the following muscles is typically reoriented during repair of cleft palate?
(A)Levator veli palatini
(B)Muscularis uvulae
(C)Palatoglossus
(D)Palatopharyngeus
(E)Superior pharyngeal constrictor

Answer 54

(A)Levator veli palatini

Question 55

Goals of cleft palate repair

Answer 55

The goals of cleft palate repair during infancy are two-fold. The first goal is to establish a complete, water tight closure of the secondary palate for separation of the oral and nasal cavities. The second goal is to reorient and repair the levator musculature to allow for normal speech formation.

Question 56

Cleft palate occurs during which of the following weeks of fetal development?
(A)1–2
(B)3–4
(C)5–6
(D)7–8
(E)9–10

Answer 56

(D)7–8

Question 57

Embryonic errors that cause cleft palate

Answer 57

Cleft palate, which results from a failure of the palatine shelves to fuse, occurs primarily during weeks 7 to 8.

Question 58

Embryonic errors during weeks 1-2

Answer 58

Cause death

Question 59

Embryonic errors that cause cleft lip

Answer 59

Clefting of the lip occurs during weeks 5 to 6.

Question 60

Embryonic errors that cause ear and eye abnormalities

Answer 60

Ear and eye abnormalities occur during weeks 10 to 12.

Question 61

A 5-year-old boy with submucus cleft palate has velopharyngeal incompetence. Which of the following is the most likely underlying cause?
(A)Incorrectly positioned levator palatine muscles
(B)Incorrectly positioned palatoglossus muscles
(C)Incorrectly positioned palatopharyngeal muscles
(D)Incorrectly positioned tensor palatine muscles
(E)Short soft palate

Answer 61

(A)Incorrectly positioned levator palatine muscles

Question 62

What happens when the levator palatine muscles contract in a cleft patient?

Answer 62

By definition, a submucus cleft palate involves the anomalous insertion of the levator palatine muscles onto the posterior aspect of the hard palate. When contracting, the palate then is forced to elevate in a V-shaped fashion and shorten to some degree.

Question 63

During development of a fetus, a cleft of the lip results when the maxillary process fails to fuse normally with which of the following processes?
(A) Lateral nasal
(B) Frontonasal
(C) Medial nasal
(D) Mandibular

Answer 63

(C) Medial nasal

Question 64

Facial prominences of the developing fetus

Answer 64

These five facial prominences are the frontonasal, paired maxillary, and paired mandibular.

Question 65

Failure of what results in a common cleft of the lip?

Answer 65

A failure of fusion between the maxillary prominence and the medial nasal process results in a common cleft of the lip.

Question 66

The frontonasal prominence gives rise to?

Answer 66

The frontonasal prominence gives rise to the nasal pit or placode, around which develops the medial and lateral nasal processes.

Question 67

A 5-year-old boy who underwent repair of cleft palate via double opposing Z-plasty four years ago has hypernasality indicative of velopharyngeal insufficiency. Direct nasendoscopy shows a coronal closure pattern of the velopharyngeal port and little or no motion of the lateral pharyngeal wall. Which of the following surgical procedures is most appropriate for correction of the velopharyngeal insufficiency?
(A) Augmentation of the posterior pharynx
(B) Inferiorly based posterior pharyngeal flap
(C) Sphincter pharyngoplasty
(D) Superiorly based posterior pharyngeal flap
(E) V-Y pushback palatoplasty

Answer 67

(C) Sphincter pharyngoplasty

Many patients with velopharyngeal insufficiencyafter cleft palate repair have a shortened, scarred velum, resulting in a deficiency in the anterior-posterior coronal closure pattern. Other patients, such as those with velocardiofacial syndrome, have generalized pharyngeal dysfunction with poor lateralpharyngeal wall motion, which contributes to a large central gap that leads to velopharyngeal incompetence.

To correct velopharyngeal insufficiency in this patient, a sphincter pharyngoplasty is most appropriate.

Question 68

What results in velopharyngeal competence?

Answer 68

Velopharyngeal competence results from sufficient apposition of the velar mucosa against the posterior pharyngeal wall and from motion of the lateral pharyngeal wall that causes sphincteric closure of the velopharyngeal port.

Question 69

Causes of VPI after cleft palate repair

Answer 69

Many patients with velopharyngeal insufficiencyafter cleft palate repair have a shortened, scarred velum, resulting in a deficiency in the anterior-posterior coronal closure pattern. Other patients, such as those with velocardiofacial syndrome, have generalized pharyngeal dysfunction with poor lateralpharyngeal wall motion, which contributes to a large central gap that leads to velopharyngeal incompetence.

Question 70

The firstborn child of a Caucasian couple with no abnormalities has bilateral cleft lip and palate. Which of the following percentages best represents the possibility that this couple’s next child will have cleft lip, with or without cleft palate?
(A) 2%
(B) 4%
(C) 10%
(D) 16%
(E) 32%

Answer 70

(B) 4%

The correct answer for the sibling of a child with bilateral cleft lip and palate is approximately 4%, depending on the reference. Regardless, the risk decreases if there is no cleft palate in the proband and/or if the condition is unilateral. The incidence of cleft lip with or without cleft palate for the sibling of a child with bilateral cleft lip but no cleft palate is 6.7%, for the sibling of a child with unilateral cleft lip and palate is 4.9%, and for the sibling of a child with unilateral cleft lip but no cleft palate is 4.0%. If there are two affected children with cleft lip with or without cleft palate, the risk for a third child would be at least 9%.

Question 71

Overall risk of cleft lip with or without cleft palate in a Caucasian population

Answer 71

1:1000

Question 72

In neonates with submucous cleft palate, the zona pellucida results from abnormal morphology of which of the following muscles?
(A) Levator veli palatini
(B) Muscularis uvulae
(C) Palatopharyngeus
(D) Pharyngeal constrictor
(E) Tensor veli palatini

Answer 72

(A) Levator veli palatini

The zona pellucida is formed by parallel bulges of anterior-posterior muscle on either side of the soft palate in the midline. Between these paired and cleft levator veli palatini muscles is a bluish two-layered mucosal bridge, which is the submucous cleft palate. With abnormal morphology, the levator veli palatini muscles insert into the posterior edge of the hard palate, causing Veau’s cleft muscle as is seen in the typical cleft palate. The levator veli palatini muscles originate from the petrous portion of the temporal bone and the medial surface of the auditory tube and insert in the middle soft palate. Inferiorly, they form a V-shaped sling that suspends the velum from the base of the cranium and pull the soft palate up and back.

Question 73

What is used to perform the sphincter pharyngoplasty?

Answer 73

The palatopharyngeus muscle.

The palatopharyngeus muscle originates from the palatal aponeurosis and runs to the posterior pharyngeal pillar. It functions to depress the palate and displace it backward. It is used to perform the sphincter pharyngoplasty.

Question 74

Anatomy of the tensor veli palatini muscle

Answer 74

The tensor veli palatini muscle originates from the scaphoid fossa, medial pterygoid plate, and spine of the sphenoid. It courses inferiorly around the hamulus to form the palatal aponeurosis.

Question 75

During normal development of the secondary palate, elevation of the palatal shelf occurs at approximately how many weeks’ gestation?
(A) 4
(B) 8
(C) 12
(D) 16
(E) 20

Answer 75

(B) 8

In the 7-week-old embryo, the two palatal shelves lie vertically. Starting with the eighth week, the neck straightens from its flexed position, the tongue drops posteriorly, and the shelves rotate superiorly to a horizontal position as they fuse from an anterior to posterior direction.

Question 76

The C flap in the Millard rotation advancement repair of unilateral cleft lip is used to achieve which of the following?
(A) Lengthening of the columella
(B) Lengthening of the lip
(C) Reduction of alar flare
(D) Shortening of the lip
(E) Symmetry in the philtral column

Answer 76

(A) Lengthening of the columella

Question 77

What is the C flap for in repair of unilateral cleft deformity

Answer 77

The C flap is designed to lengthen the columella.

Question 78

Characteristic findings in the nose for unilateral cleft lip deformity

Answer 78

Shortened columella
Deviation and distortion of the septum
Dislocation and slumping of the alar cartilage Flaring of the alar base

Question 79

A 6-year-old boy with velopharyngeal incompetence is scheduled to undergo sphincter pharyngoplasty. He underwent cleft palate repair in infancy. In the sphincter pharyngoplasty procedure, which of the following muscles is elevated in the posterior tonsillar pillar?
(A) Levator veli palatini
(B) Palatoglossus
(C) Palatopharyngeus
(D) Stylopharyngeus
(E) Superior pharyngeal constrictor

Answer 79

(C) Palatopharyngeus

The posterior tonsillar pillar is created by the palatopharyngeus. The palatopharyngeus muscle arises from fibers that commingle with the superior pharyngeal constrictor. It enters the soft palate, where its fibers intermingle with those of the levator. The palatopharyngeus functions to depress the soft palate and provide inward motion of the lateral pharyngeal wall. In a sphincter pharyngoplasty, superiorly based flaps are elevated from the posterior tonsillar pillar, sutured together, and inset into the posterior pharyngeal wall. These flaps contain the palatopharyngeus muscle.

Question 80

Stylopharyngeal muscle

Answer 80

The stylopharyngeal muscle arises from the styloid process. It inserts between the fibers of the superior and middle pharyngeal muscles in the pharyngeal wall.

Question 81

In neonates with isolated cleft palate, which of the following percentages best represents the incidence of additional anomalies?
(A) 10%
(B) 30%
(C) 50%
(D) 70%
(E) 90%

Answer 81

(C) 50%

Isolated cleft palate has an incidence of 1:2000 without ethnic preference and has a higher incidence of associated anomalies than cleft lip and palate (CL/P).

Question 82

Incidence of associated anomalies with CP vs CL/CP

Answer 82

Isolated cleft palate has a higher incidence of associated anomalies (~50%) than cleft lip and palate (CL/P) (10-15%)

Question 83

Percent of isolated cleft palate cases that will fall into established syndromes

Answer 83

Approximately half of the cases of cleft palate with associated anomalies will fall into established syndromes.

Question 84

Incidence of CL/CP vs CP

Answer 84

Patients with CL/P are more common than those with cleft palate alone; the incidence is 1 in 1000 Caucasians, 1 in 2000 African-Americans, and 1 in 500 Asians.

Question 85

Incidence of associated anomalies with CP

Answer 85

50%

Question 86

Incidence of associated anomalies with CL/CP

Answer 86

10-15%

Question 87

A 10-month-old infant with cleft palate is scheduled to undergo repair via the Veau-Wardill-Kilner V-Y technique. Which of the following structures will NOT be directly manipulated during this repair procedure?
(A) Levator veli palatini muscles
(B) Musculus uvulae
(C) Nasal lining
(D) Palatopharyngeus muscles
(E) Tensor veli palatini muscles

Answer 87

(D) Palatopharyngeus muscles

Because cleft palate does not disrupt the palatopharyngeus muscles, they do not need to be repaired.

Question 88

A 10-year-old boy who underwent bilateral cleft lip repair during infancy has tightness of the upper lip. A photograph is shown above. On physical examination, the philtrum is excessively wide and hypoplastic with absence of muscle competence. Redundancy of the lower lip is also  noted. Which ofthe following is the most appropriate method of flap reconstruction?
(A) Abbe flap
(B) Banked fork flaps
(C) Gilles fan flap
(D) Karapandzic flap
(E) Nasolabial flap

Answer 88

(A) Abbe flap

The most appropriate management is reconstruction withan Abbe flap. This lip-switch flap has been designed specifically to create a functional philtrum in patients who have tightness of the upper lip following cleft lip repair.

Question 89

Flap designed to create a functional philtrum in patients who have tightness of the upper lip following cleft lip repair

Answer 89

Abbe flap: This lip-switch flap has been designed specifically to create a functional philtrum in patients who have tightness of the upper lip following cleft lip repair. After the abnormal philtrum is excised, redundant tissue is harvested from the lower lip to replace the resultant defect as an aesthetic subunit. The Abbe flap is pedicled on the submucosal labial artery of the lower lip at the superior edge of the inner free border of the lip. The pedicle is subsequently divided during a delayed second procedure, which is performed a minimum of 10 days to four weeks after flap transfer.

Question 90

Dimensions of the reconstructed philtrum in the adult

Answer 90

In adults, the reconstructed philtrum should be no wider than 10 mm and no longer than 15 mm; however some surgeons advocate using a smaller flap that has a width of 7 to 9 mm at the cupid’s bow and 4 to 5 mm at the labial-columellar junction.

Question 91

What is the Abbe flap pedicled on?

Answer 91

The Abbe flap is pedicled on the submucosal labial artery of the lower lip at the superior edge of the inner free border of the lip.

Question 92

Banked fork flaps

Answer 92

Banked fork flaps were used previously for bilateral lip reconstruction. At the time of primary lip repair, extra tissue on either side of the new philtrum from the prolabium was “banked” within the creases of the alar base to be used as forked flaps for columellar reconstruction at a later date. However, it was subsequently determined that the columella in fact lies within the nasal region and that primary nasoplasty should be performed instead at the time of lip repair to create the columella. In addition to primary nasoplasty, adequate preoperative nasoalveolar molding has been determined to be more appropriate, and banked fork flaps are no longer used.

Question 93

A 5-year-old child who underwent cleft palate repair in infancy has velopharyngeal insufficiency. Nasoendoscopy shows closure of the central velopharyngeal mechanism with residual openings on the left and right. The patient is scheduled to undergo sphincter pharyngoplasty for reconstruction of the pharynx. This procedure involves transposition of which of the following muscles? 
(A) Buccinator
(B) Levator veli palatini
(C) Palatoglossus
(D) Palatopharyngeus
(E) Tensor veli palatin

Answer 93

(D) Palatopharyngeus

Question 94

Which of the following muscles courses around the pterygoid hamulus?
(A) Levator veli palatini
(B) Palatoglossus
(C) Palatopharyngeus
(D) Tensor veli palatini
(E) Uvula

Answer 94

(D) Tensor veli palatini

Question 95

In patients with nasal deformities associated with complete unilateral cleft lip, which of the following best describes the displacement of the ala? 
(A) Lateral, inferior, and anterior
(B) Lateral, inferior, and posterior
(C) Medial, inferior, and posterior
(D) Medial, superior, and anterior
(E) Medial, superior, and posterior

Answer 95

(B) Lateral, inferior, and posterior

Question 96

In patients who have nasal deformities associated with complete unilateral cleft lip, the ala is displaced where?

Answer 96

In patients who have nasal deformities associated with complete unilateral cleft lip, the ala is displaced laterally, inferiorly, and posteriorly. The orbicularis oris inserts onto the alar base abnormally, on the lateral cleft side, and pulls the ala laterally and inferiorly.

Question 97

Why is the ala displaced in complete unilateral cleft lip?

Answer 97

In patients who have nasal deformities associated with complete unilateral cleft lip, the ala is displaced laterally, inferiorly, and posteriorly. The orbicularis oris inserts onto the alar base abnormally, on the lateral cleft side, and pulls the ala laterally and inferiorly. The medial orbicularis inserts on the nasal spine and caudal septum and pulls these structures to the contralateral side. The piriform rim, which normally supports the ala, is also deficient, allowing the ala to be displaced posteriorly on the side of the cleft.

Question 98

A 6-year-old boy who underwent repair of a right-sided unilateral cleft lip and palate in infancy has an unrepaired alveolar cleft. Which of the following best describes the malocclusion on the cleft side in this patient?
(A) Angle class II, division I
(B) Angle class II, division II
(C) Deep overbite
(D) Excessive overjet
(E) Posterior crossbite

Answer 98

(E) Posterior crossbite

A child with an unrepaired alveolar cleft will exhibit collapse of the maxillary arch. The arch is deficient in all dimensions: anteroposterior, transverse, and vertical. Therefore, this child will have posterior crossbite of the maxillary dentition with respect to the mandibular dentition. In patients with crossbite, the lower dentition is positioned labial to the upper dentition; this is the opposite of normal occlusion. The arch form should be corrected orthodontically and dental compensation should be eliminated prior to bone grafting of the alveolar cleft.

Question 99

Malocclusion Class II, division I

Answer 99

Angle class II malocclusion is defined as the mesiobuccal cusp of the maxillary first molar located mesial (anterior) to the buccal groove of the mandibular first molar. In class II, division 1, the lateral incisors are flared labially.

Question 100

Malocclusion Class II, division II

Answer 100

Angle class II malocclusion is defined as the mesiobuccal cusp of the maxillary first molar located mesial (anterior) to the buccal groove of the mandibular first molar. In class II, division 2, the incisors are lingually inclined.

Question 101

Overbite

Answer 101

Overbite describes the distance between the maxillary and mandibular incisors in the vertical plane with the jaws in centric occlusion,

Question 102

Overjet

Answer 102

Overjet describes the distance between the maxillary and mandibular incisors in the horizontal plane with the jaws in centric occlusion.

Question 103

During embryologic development, which of the following structures arises from the lateral nasal processes?
(A) Columella
(B) Nasal bridge
(C) Nasal tip
(D) Nasal ala
(E) Nasal septum

Answer 103

(D) Nasal ala

Question 104

The medial nasal processes give rise to:

Answer 104

The columella, nasal tip, philtrum, and premaxilla.

Question 105

The nasal ale arise from:

Answer 105

The lateral nasal processes

Question 106

A neonate is undergoing evaluation because of airway obstruction. Physical examination shows retrogenia and glossoptosis. Which of the following is the most appropriate initial management of the airway obstruction?
(A) Prone positioning
(B) Orotracheal intubation
(C) Lip-tongue adhesion
(D) Tracheostomy
(E) Mandibular distraction osteogenesis

Answer 106

(A) Prone positioning

Question 107

During embryologic development, which of the following structures arises from the frontonasal processes?
(A) Columella
(B) Nasal bridge
(C) Nasal septum
(D) Nasal tip
(E) Philtrum

Answer 107

(B) Nasal bridge

Question 108

During development, primary cleft palate occurs as a result of unsuccessful fusion of which of the following structures?
(A) Lateral palatine processes and median palatine process
(B) Maxillary prominence and lateral palatine process
(C) Maxillary prominence and mandibular prominence
(D) Medial nasal prominence and nasal septum

Answer 108

(A) Lateral palatine processes and median palatine process

Question 109

How does primary cleft palate occur?

Answer 109

During development, primary cleft palate occurs as a result of unsuccessful fusion of the median palatine process, which forms the primary palate, and the lateral palatine processes, which form the secondary palate.

Question 110

How does secondary cleft palate occur?

Answer 110

Secondary cleft palate occurs following unsuccessful fusion of the lateral palatine processes to each other and with the nasal septum

Question 111

Unsuccessful fusion of the maxillary and mandibular prominences results in:

Answer 111

Unsuccessful fusion of the maxillary and mandibular prominences results in macrostomia

Question 112

A child who had a cleft palate repair in infancy is undergoing sphincter pharyngoplasty for management of velopharyngeal insufficiency. When performing this procedure, which of the following muscles is typically used to create the sphincter?
(A) Levator veli palatini
(B) Musculus uvulae
(C) Palatopharyngeus
(D) Salpingopharyngeus
(E) Tensor veli palatini

Answer 112

(C) Palatopharyngeus

Question 113

Which of the following muscles is/are NOT involved in normal velopharyngeal closure?
(A) Levator palatini
(B) Palatopharyngeus
(C) Superior pharyngeal constrictors
(D) Tensor veli palatini
(E) Uvulus

Answer 113

(D) Tensor veli palatini

Muscles involved in velopharyngeal closure include each of those mentioned above except for the tensor veli palatini. Normal velopharyngeal closure is crucial for production of intelligible speech; any abnormalities in this mechanism can result in hypernasality, nasal emissions, imprecise production of consonants, decreased speech volume, and/or shortness of phrases.

Question 114

Abnormalities in velopharyngeal closure vs speech:

Answer 114

Normal velopharyngeal closure is crucial for production of intelligible speech; any abnormalities in this mechanism can result in hypernasality, nasal emissions, imprecise production of consonants, decreased speech volume, and/or shortness of phrases.

Question 115

Patients with unilateral cleft lip and associated nasal deformities have each of the following findings EXCEPT
(A) attenuation and inferior positioning of the lower lateral cartilage on the side of the cleft
(B) elongation of the philtrum
(C) insertion of the orbicularis oris muscle into the cleft margin and alar wing
(D) outward rotation and projection of the premaxilla
(E) unilateral shortening of the columella

Answer 115

(B) elongation of the philtrum

In patients who have unilateral cleft lip and associated nasal deformities, the premaxilla is rotated and projected outward, and the lateral maxillary element is collapsed and retropositioned. The inferior edge of the septum lies outside of the vomer groove, while the nasal spine is located in the floor of the normal nostril. The affected columella is 25% to 50% shorter than the unaffected side. The lower lateral cartilage is attenuated, and the nasal dome lies separate, below the opposite cartilage. The alar base is flared and rotated outward, and the vestibular lining is deficient on the side of the cleft.

With regard to the lip deformities, the philtrum termination of the orbicularis oris muscle in the lateral lip is shortened at the margin of the cleft; at this point, the muscle inserts into the alar wing. The musclesbetween the philtral midline and the cleft are hypoplastic. Two thirds of the cupid’s bow is preserved, as well as one philtral column and a dimple hollow.

Question 116

A 1-year-old child with Pierre Robin sequence has normal mandibular growth. His jaw deformity is best described as
(A) brachygnathia
(B) hypoplasia
(C) microgenia
(D) micrognathia
(E) retrognathia

Answer 116

(E) retrognathia

Retrognathia, which is defined as posterior displacement of the chin with normal mandibular dimensions, is best used to describe the findingsseen in this patient with Pierre Robin sequence. Other terms such as brachygnathia, micrognathia, congenital mandibular atresia, mandibular hypoplasia, and mandibular hypotrophy have been used in the description of this condition. However, because all of these terms denote abnormalities in mandibular growth, they are frequently used incorrectly; instead, normal mandibular growth is a classic finding of Pierre Robin sequence.

Question 117

In patients who have unilateral cleft lip and associated nasal deformities, the premaxilla is _______________ and the lateral maxillary element is _______________.

Answer 117

In patients who have unilateral cleft lip and associated nasal deformities, the premaxilla is rotated and projected outward, and the lateral maxillary element is collapsed and retropositioned.

Question 118

In patients who have unilateral cleft lip and associated nasal deformities, the inferior edge of the septum _______________ and the while the nasal spine is _______________.

Answer 118

In patients who have unilateral cleft lip and associated nasal deformities, the inferior edge of the septum lies outside of the vomer groove, while the nasal spine is located in the floor of the normal nostril. The affected columella is 25% to 50% shorter than the unaffected side. The lower lateral cartilage is attenuated, and the nasal dome lies separate, below the opposite cartilage. The alar base is flared and rotated outward, and the vestibular lining is deficient on the side of the cleft.

With regard to the lip deformities, the philtrum termination of the orbicularis oris muscle in the lateral lip is shortened at the margin of the cleft; at this point, the muscle inserts into the alar wing. The musclesbetween the philtral midline and the cleft are hypoplastic. Two thirds of the cupid’s bow is preserved, as well as one philtral column and a dimple hollow.

Question 119

In patients who have unilateral cleft lip and associated nasal deformities, the affected columella is:

Answer 119

In patients who have unilateral cleft lip and associated nasal deformities, the affected columella is 25% to 50% shorter than the unaffected side. The lower lateral cartilage is attenuated, and the nasal dome lies separate, below the opposite cartilage. The alar base is flared and rotated outward, and the vestibular lining is deficient on the side of the cleft.

With regard to the lip deformities, the philtrum termination of the orbicularis oris muscle in the lateral lip is shortened at the margin of the cleft; at this point, the muscle inserts into the alar wing. The musclesbetween the philtral midline and the cleft are hypoplastic. Two thirds of the cupid’s bow is preserved, as well as one philtral column and a dimple hollow.

Question 120

In patients who have unilateral cleft lip and associated nasal deformities, the lower lateral cartilage is _________, and the nasal dome ____________.

Answer 120

In patients who have unilateral cleft lip and associated nasal deformities, the lower lateral cartilage is attenuated, and the nasal dome lies separate, below the opposite cartilage. The alar base is flared and rotated outward, and the vestibular lining is deficient on the side of the cleft.

Question 121

In patients who have unilateral cleft lip and associated nasal deformities, the alar base is ____________, and the vestibular lining is ____________.

Answer 121

In patients who have unilateral cleft lip and associated nasal deformities, the alar base is flared and rotated outward, and the vestibular lining is deficient on the side of the cleft.

Question 122

What type of mandibular growth is typical in patients with Pierre Robin sequence?

Answer 122

Retrognathia, which is defined as posterior displacement of the chin with normal mandibular dimensions, is best used to describe the findingsseen in this patient with Pierre Robin sequence. Other terms such as brachygnathia, micrognathia, congenital mandibular atresia, mandibular hypoplasia, and mandibular hypotrophy have been used in the description of this condition. However, because all of these terms denote abnormalities in mandibular growth, they are frequently used incorrectly; instead, normal mandibular growth is a classic finding of Pierre Robin sequence.

Question 123

A 6-year-old boy with a repaired unilateral complete cleft lip and palate presents for an annual cleft team clinic visit. Initiation of palatal expansion is discussed with the child’s parents. Timing for initiation of palatal expansion should be based upon which of the following?
A) Alveolar cleft width
B) Canine eruption
C) Occlusal status
D) Patient age
E) Severity of alveolar collapse

Answer 123

B) Canine eruption

Alveolar bone grafting should be performed during the time of transitional dentition. Specifically, it has the greatest chance for success after the incisors erupt, but before the eruption of the canine. Tooth development/eruption varies from child to child, so there is no set age for bone grafting. Alveolar cleft width will determine the amount of bone grafting, but not the timing. Severity of alveolar collapse will affect the duration of palatal expansion, but not timing. Occlusal status should not play a role in surgical decision-making for alveolar cleft grafting, as it will be addressed later with either orthodontics or jaw surgery after skeletal maturity.

Question 124

A 7-year-old girl with a history of repair of cleft palate is evaluated because of possible velopharyngeal insufficiency. In addition to evaluation of the patient’s speech by trained speech pathologists, which of the following is the most appropriate diagnostic tool?
A) Cine MRI
B) CT scan
C) Examination during anesthesia
D) Lateral cephalogram
E) Nasopharyngoscopy

Answer 124

E) Nasopharyngoscopy

The primary goal of cleft palate repair is normal speech and swallowing. Velopharyngeal competence, the ability to completely close the velopharyngeal sphincter, is required for the normal production of all but the nasal consonants (in English: /m/, /n/, and /ng/). Velopharyngeal insufficiency (VPI) is defined as the inability to completely close the velopharyngeal sphincter. The primary effects of VPI are nasal air escape and hypernasality. Video fluoroscopy and nasopharyngoscopy can detect the sagittal deficiency closure pattern occurring in patients with VPI after cleft palate surgery. Speech articulation errors (i.e., distortions, substitutions, and omissions) are secondary effects of VPI. The result is decreased intelligibility of speech. The velopharyngeal port is bordered anteriorly by the velum, bilaterally by the lateral pharyngeal walls, and posteriorly by the posterior pharyngeal wall. VPI can be diagnosed by both subjective and objective means. Perceptual evaluation of speech by an experienced speech language pathologist is the standard. Multiview video fluoroscopy and nasopharyngoscopy both provide visual information (i.e., closure pattern and closure rating) that is valuable for surgical planning. However, the need to avoid radiation if centers are migrating away from fluoroscopy has caused most cleft centers to migrate to direct nasopharyngoscopy.

MRI is emerging as a technology for evaluating VPI, but it would not be the first choice for diagnostic workup.

Overall, examination during anesthesia in the operating room would likely not be required, but instead a complete examination and nasopharyngoscopy would likely be performed comfortably in the office. Experience with nasopharyngoscopy has grown in most comprehensive cleft centers and has become an invaluable tool for surgical planning.

Lateral cephalogram does not provide a dynamic evaluation of the pharynx.

Question 125

A 4-year-old girl with velocardiofacial syndrome is evaluated for hypernasal speech. She underwent protracted speech therapy after repair of an isolated cleft of the soft palate at age 9 months. Physical examination shows a well-healed palate and trace elevation of the soft palate with phonation. Videofluoroscopy shows poor motion of the velum; adequate lateral pharyngeal wall motion is noted. To improve this patient’s speech, which of the following is the most appropriate management of her velopharyngeal insufficiency?
A) Fat augmentation of the posterior pharyngeal wall
B) Furlow palatoplasty
C) Intravelar veloplasty
D) Reconstruction with a superiorly based pharyngeal flap
E) Sphincter pharyngoplasty

Answer 125

D) Reconstruction with a superiorly based pharyngeal flap

While any of the methods listed can potentially improve speech in the patient described, the lack of significant velar motion coupled with poor lateral pharyngeal wall motion makes pharyngeal flap the most predictable alternative of those listed. Intravelar veloplasty effectively restores and repositions the levator sling, but will not overcome the poor lateral wall motion. Furthermore, it is probable (although not certain) that the muscles were properly positioned during the initial operation. Fat augmentation and Furlow palatoplasty can decrease the coronal gap, but will do little to address the deficient lateral pharyngeal wall mobility. Sphincter palatoplasty has yielded good results in patients with this condition, but poor muscle tone and coronal closure make this method less likely to produce a competent pharyngeal sphincter than a properly designed pharyngeal flap.

Question 126

Velar competence after treatment of velopharyngeal insufficiency with Furlow double-opposing Z-plasty is most strongly correlated with which of the following?
A) Age at the time of procedure
B) Compliance with speech therapy
C) Patient gender
D) Preoperative closure gap
E) Type of cleft

Answer 126

D) Preoperative closure gap

Furlow double-opposing Z-plasty is an effective method of treating velopharyngeal insufficiency associated with submucous cleft palate or following conventional push-back palatoplasty procedures. Several studies suggest that the size of the preoperative velopharyngeal gap, as determined by preoperative nasendoscopy, is the most important determinant of velar competence after Furlow palatoplasty. Thus, patients with a smaller preoperative maximal closure gap were more likely to have a competent velopharyngeal sphincter postoperatively. The procedure has also been reported to be less effective in older children, in overt (versus submucous) clefts, and in patients with certain syndromes such as velocardiofacial syndrome. Nevertheless, these variables are not as important as the preoperative gap. The gender of the patient and compliance with speech therapy do not influence outcomes.

Question 127

A 2-month-old male infant is evaluated for complete unilateral cleft of the lip and palate. Development of which of the following tooth buds is most likely to be impaired in this patient?
A) Central incisor
B) First molar
C) Lateral incisor
D) Premolar
E) Third molar

Answer 127

C) Lateral incisor

Cleft palates can affect tooth development, leading to a variety of dental abnormalities. In general, the upper lateral incisor tooth bud is most commonly susceptible to injury in the area of the cleft in both the deciduous and permanent teeth. Other teeth in the area of the cleft, such as the canines, may be affected as well. The premolar, molar, and central incisor tooth buds are typically too far from the cleft area to be affected.

Question 128

Failure of fusion of which of the following results in the formation of a cleft of the lip?
A) Frontonasal and maxillary prominences during the first 4 to 5 weeks of gestation
B) Frontonasal and maxillary prominences during the first 9 to 11 weeks of gestation
C) Lateral nasal and maxillary prominences during the first 2 to 4 weeks of gestation
D) Medial nasal and maxillary prominences during the first 9 to 11 weeks of gestation
E) Medial nasal and maxillary prominences during the first 6 to 8 weeks of gestation

Answer 128

E) Medial nasal and maxillary prominences during the first 6 to 8 weeks of gestation

Cleft lip and cleft palate are common birth defects that result from a variety of genetic and environmental factors. On average, they occur in 1.7 of every 1000 live-born babies.

The development of the lip and palate in utero involves a complex series of steps that involve cell migration, proliferation, and apoptosis. During the fourth week of gestational development, neural crest cells migrate to the developing craniofacial region of the embryo, where they help in the formation of the frontonasal prominence, the mandibular processes, and the maxillary prominences. Nasal placodes divide the lower portion of the frontonasal prominence into the paired medial and lateral nasal processes. During weeks 6 to 8 of gestation, fusion of the medial nasal prominences with each other and with the maxillary processes forms the upper lip and primary palate. Therefore, Option E is the most appropriate answer regarding the etiology of the baby’s cleft of the lip.

Option D is not appropriate because it states that the failure of fusion occurs during weeks 9 to 11, which is too late in embryological development.

Options A and B are not appropriate because fusion of the frontonasal prominence with the maxillary prominences forms the primary palate.

Option C describes the embryologic formation of an oblique cleft, and the time frame is not appropriate.

Question 129

A 4-year-old girl is referred by her speech therapist because she has persistent nasal air escape with phonation. She underwent isolated repair of the cleft palate in infancy. Physical examination shows a long, mobile palate. No fistula is noted. Nasendoscopy shows good coronal closure with poor lateral pharyngeal wall movement. Which of the following is the most appropriate management?
A) Augmentation of Passavant ridge
B) Continued speech therapy
C) Implantation of a palatal lift prosthesis
D) Posterior pharyngeal flap
E) Sphincter pharyngoplasty

Answer 129

E) Sphincter pharyngoplasty

The patient described has velopharyngeal incompetency (VPI). The inability to adequately close the palate against the pharyngeal walls leads to nasal air escape during speech. This is most common with fricatives such as “s” and “z.” As the degree of incompetence increases, speech errors with plosive sounds become apparent, such as “d” and “p” and “b.”

At the age of 4 years, intervention to correct VPI is appropriate. Speech therapy alone is unlikely to improve hypernasal speech production. A delay in treatment can lead to the development of compensatory misarticulation and worsening speech errors that will be difficult to correct in the future.

The goals of surgery are to eliminate the symptoms of hypernasality and eliminate audible nasal emissions without causing complete obstruction of the velopharyngeal (VP) port, allowing for nasal breathing and nasal resonance. Multiple procedures have been described. Studies indicate that the success of repair depends on selecting the appropriate procedure based on the anatomy and the movement of the VP port.

Sphincter pharyngoplasty involves reduction of the lateral and posterior aspects of the VP ports while maintaining the centric opening. The palatopharyngeus muscle is incised, and a flap is constructed from the posterior tonsillar pillar. These bilateral superiorly based musculomucosal flaps are juxtaposed in the midline of the posterior pharyngeal wall. This procedure is advantageous in that it potentially recreates a functional sphincter, and the incidence of postoperative nasal obstruction is less than that with the pharyngeal flap.

The nasendoscopic examination demonstrates a classic palate closure pattern where the central gap is minimal, and a much larger gap occurs at the lateral ports. Thus, surgery to close the central gap, such as augmentation of Passavant ridge or a posterior pharyngeal flap, will have a lower success rate.

The prosthetic speech bulb is most useful in patients with little or no VP motion. VP movement is essential to surgical success for the VP flap procedure or sphincteroplasty. Patients with little VP movement are good candidates for prosthetic management. A VP speech prosthesis can elevate the velum (lift), fill the residual velopharyngeal gap (obturator), or both (lift-orator).

Question 130

A 2-year-old boy who was recently adopted is brought to the office for evaluation and treatment of cleft of the lip and palate. Physical examination shows involvement of the lip, alveolus, and entire palate. A photograph is shown. He is otherwise healthy with no other congenital anomalies. Which of the following is this patient’s risk of having a child with cleft of the lip? (no lip pits)
A) 1%
B) 5%
C) 10%
D) 15%
E) 50%

Answer 130

B) 5%

The risk of having a child with a cleft of the lip and palate is multifactorial. In familial cases, the risk is dependent on the family history and sibling involvement. In this case, neither the family history nor sibling history is available, so risk calculation is dependent upon the child’s personal history only. For males with an oral cleft, the prevalence of clefts in their first offspring is 4.7%, and for females it is 3.6%. If this child were to have a child with a cleft, then the risk of subsequent children to also have a cleft would be 17%. If this child also had lip pits, then the risk of having a child with a cleft would be 50% (van der Woude syndrome).

Question 131

A 6-year-old boy who underwent repair of cleft palate 5 years ago is brought to the clinic for follow-up. The patient is hypernasal. Nasoendoscopy shows good lateral pharyngeal wall motion and a sagittal closure pattern. Cephalometric analysis shows a posterior gap of 10 mm. Which of the following is the most appropriate management?

A) Intravelar veloplasty
B) Obturator
C) Palatal lift
D)Pharyngeal flap
E) Sphincter pharyngoplasty

Answer 131

D)Pharyngeal flap

Satisfactory lateral pharyngeal wall movement and sagittal or circular velopharyngeal closure patterns should be treated with a pharyngeal flap. A large posterior gap with coronal, circular, or bowtie patterns of closure and good velar elevation, but poor lateral wall motion, should be treated with a sphincter pharyngoplasty. A palatal lift is used in patients with adequate tissue, but poor control of coordination. An obturator is used to assist with closure when there is inadequate palatal tissue. Finally, intravelar veloplasty is used in unrepaired clefts or a submucous cleft with a small posterior gap on closure.

Question 132

A 6-year-old boy with velopharyngeal insufficiency is brought to the office for sphincter pharyngoplasty. History includes repair of a cleft palate as an infant. Innervation of the muscle used to create the sphincter arises from which of the following?
A) Cranial part of the accessory (XI) nerve
B) Glossopharyngeal (IX) nerve
C) Greater and lesser palatine branches of the pterygopalatine ganglion
D) Hypoglossal (XII) nerve
E) Medial pterygoid nerve

Answer 132

A) Cranial part of the accessory (XI) nerve

Sphincter pharyngoplasty for correction of velopharyngeal insufficiency is performed by elevation of myomucosal flaps from the posterior tonsillar pillar, which involves the palatopharyngeus muscle. The palatopharyngeus muscle is supplied by the cranial part of accessory (XI) nerve through the pharyngeal branch of vagus (X) nerve via the pharyngeal plexus. The medial pterygoid nerve innervates the tensor veli palatini. The greater and lesser palatine nerves are branches of the pterygopalatine ganglion which provide sensory innervation to the palate. The hypoglossal (XII) nerve innervates the lingual muscles, with the exception of the palatoglossus, which is supplied by pharyngeal branch of the vagus (X) nerve, via the pharyngeal plexus. A photograph is shown.

Question 133

A 4-year-old girl who has velocardiofacial syndrome is evaluated because of severe velopharyngeal insufficiency. Reconstruction using a superiorly based pharyngeal flap is planned. Which of the following is the most important factor in determining the width of the flap?

A) Ability to close the pharyngeal donor defect
B) Lateral pharyngeal wall motion
C) Length of the soft palate
D) Palatal movement (coronal closure)
E) Position of the carotid arteries in the posterior pharynx

Answer 133

B) Lateral pharyngeal wall motion

Reconstruction with a pharyngeal flap is a highly effective method of treating velopharyngeal insufficiency. This procedure involves elevating a rectangular flap, based superiorly or inferiorly, from the posterior pharynx and insetting it into the soft palate. The posterior raw surface of the flap is typically lined with trapezoidal flaps raised from the nasal side of the soft palate to limit contraction. The flap serves to obstruct air leakage into the nasal passage during speech. Extremely wide flaps can, consequently, also result in obstructive sleep apnea. In such situations, sphincter pharyngoplasties (Hynes) are sometimes used to avoid this potential complication.

The width of the flap is based on the degree of lateral pharyngeal wall motion. Since this creates a static bridge between the soft palate and the nasopharynx, the length of the palate and the degree of palatal movement have no influence on the choice of flap width. Although closing the donor defect expedites healing and decreases pain, this step is not necessary. Medial displacement of the internal carotid arteries as they pass along the posterior pharynx has been described in patients with velocardiofacial syndrome and should be considered when raising the flap. However, this should not impact the ability to raise a flap of sufficient width.

Question 134

A 2-year-old girl is evaluated because of bifid uvula, notching of the posterior hard palate, and midline thinning of the posterior palate. She feeds well and has normal hearing. Which of the following is the most appropriate management at this time?
A)Furlow double-opposing Z-plasty
B)Nasendoscopy
C)Speech evaluation
D)Straight-line veloplasty
E)Veau-Wardill-Kilner push-back palatoplasty

Answer 134

C)Speech evaluation

This patient has physical findings suggestive of a submucous cleft palate: bifid uvula, notching of the posterior hard palate, and a thin midline region in the soft and/or hard palate termed the zona pellucida. The presence of any or all of these features is not required to secure the diagnosis and some patients will have none of them (occult submucous cleft palate). This anomaly is characterized by abnormal attachment of the palatal muscles (usually to the posterior edge of the hard palate) with intact nasal and oral mucosa.

Most patients with submucous cleft palate are asymptomatic; however, about 10 to 15% of affected individuals will demonstrate some degree of velopharyngeal insufficiency (VPI) and hypernasal speech. Other symptoms may include early feeding difficulties and recurrent middle ear effusion/infection. Borderline VPI may improve with speech therapy alone and early operative treatment should be deferred. Patients with severe or frank VPI, or those with less severe VPI who do not respond to speech therapy, will likely require operative intervention to improve speech.

The child in the scenario described is too young to reliably determine the presence of VPI by speech evaluation or diagnostic tests and, therefore, operative treatment of the submucous cleft using Furlow Z-plasty, straight-line veloplasty, or Veau-Wardill-Kilner push-back palatoplasty would be premature and potentially unnecessary. While the diagnosis of VPI can be made by a careful speech evaluation, it is difficult to obtain a reliable evaluation before 2 years of age. Nasendoscopy and videofluoroscopy are very useful to confirm the presence of VPI and to characterize the nature and degree of the deficiency. However, these important diagnostic tests are most informative in a cooperative patient and are rarely used in children younger than 3 years of age. Nasendoscopy could help confirm the presence of a submucous cleft palate, but such information will not change management unless the child develops hypernasal speech. Early evaluation and follow-up with a speech therapist would be appropriate to facilitate and monitor speech development.

Question 135

Which of the following skeletal anomalies of the anterior nasal spine are most commonly found in unilateral cleft lip and palate patients?
A)Deviation to the cleft side and decreased projection of the pyriform aperture and dentoalveolar arch on the cleft side
B)Deviation to the cleft side and increased projection of the pyriform aperture and dentoalveolar arch on the cleft side
C)Deviation to the noncleft side and decreased projection of the pyriform aperture and dentoalveolar arch on the cleft side
D)Deviation to the noncleft side and increased projection of the pyriform aperture and dentoalveolar arch on the cleft side

Answer 135

C)Deviation to the noncleft side and decreased projection of the pyriform aperture and dentoalveolar arch on the cleft side

The inferior border of the bony septum is deviated to the cleft side, while the anterior nasal spine is deviated to the noncleft side. There is decreased sagittal projection of the pyriform sinus and dentoalveolar arch.

Question 136

The L flap used in the rotation-advancement technique of cleft lip repair is implemented to accomplish which of the following?
A) Add bulk to the vermilion
B) Allow for sagittal expansion of the nasal sidewall
C) Lengthen the columella
D) Provide lining in the gingivobuccal sulcus
E) Restore symmetry along the philtral column

Answer 136

B) Allow for sagittal expansion of the nasal sidewall

The L flap is a medially based flap of mucosa from the surface of the lateral lip element. It is used to line the lateral nasal vault between the internal mucosa and the more external hair-bearing skin. A photograph is shown.