CL & CP, CL Nose, VPI Flashcards

Question

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 1

The correct response is Option E. Retrognathia, which is defined as posterior displacement of the chin with normal mandibular dimensions, is best used to describe the findings seen 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. In addition, the surgeon should be aware that the growth potential in patients with retrognathia and posterior displacement of the chin on external manipulation is excellent. Pierre Robin sequence is also characterized by respiratory obstruction and glossoptosis. Microgenia is defined as abnormal development in the region of the mental symphysis. However, this term can be differentiated from micrognathia because all of the mandibular components do not have to be involved; instead, some patients have an isolated small chin deformity with normally-sized jaw components. Microgenia can also occur in conjunction with mandibular hypoplasia or mandibular prognathism.

Answer 2

The correct response is Option E. Over **90% of patients have chronic infection of the middle ear prior to repair.** As a result of this fact, 100% of patients with cleft palate should be screened for hearing loss and for fluid in the middle ear due to eustachian dysfunction. There is emerging evidence and controversy with regard to treatment of the tensor veli tendon and the hamulus. However, this controversy and the tensor tenopexy, tensor transection, or fracture of the hamulus and their effects on the eustachian tube are beyond the scope of the question. However, what is clear, is that the cleft patient population is at risk for chronic infection of the middle ear. Unfortunately, if this condition is unrecognized, elements of preventable hearing loss will occur.

Answer 3

The correct response is Option B. This patient has significant hypernasality in the face of a prior straight-line cleft palate repair, but does not have an oronasal fistula. Improving hypernasal speech in patients with prior cleft repairs can be addressed with several different techniques, including sphincter pharyngoplasty, posterior pharyngeal flap, and conversion Furlow palatoplasty. This particular patient has vaulting of the velum, indicative of anterior placement of the levator veli palatini muscles, and also appears to have good palatal elevation. Both of these make him a promising candidate for a conversion Furlow palatoplasty. Posterior pharyngeal flap surgery would be less preferable, given his sleep apnea type symptoms and good palatal mobility. Further speech therapy would be unlikely to provide the patient with any significant improvement in hypernasality. Although tonsillectomy may help with his sleep apnea type symptoms, it is unlikely to provide the patient with significant speech improvement. Although posterior pharyngeal flap surgery is an option in patients with hypernasal speech following palatoplasty, this patient has a history of sleep apnea type symptoms and a mobile velum. Posterior pharyngeal flap would be preferable in a patient with poor motion of the velum, a coronal velopharyngeal port defect, and good lateral wall motion. The use of a palatal lift device can be beneficial in patients with velopharyngeal insufficiency and poor palatal motion who are not candidates for or do not wish to undergo surgery.

Answer 4

The correct response is Option B. 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.

Answer 5

The correct response is Option B. 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%. The overall risk of cleft lip with or without cleft palate in a Caucasian population is 1:1000. In a first-degree relative, that risk is multiplied by 40; in a second-degree relative, the risk is multiplied by 7; and in a third-degree relative, the risk is multiplied by 3. However, the risk is increased when more than one relative is affected, if the family member has a severe form of the disorder, if the family member is of the sex least likely to be affected, or if the parents are consanguineous. For one affected parent, the risk of having one child with cleft lip with or without cleft palate is 4%, and the risk for a second child increases to 17%.

Answer 6

The correct response is Option A. 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. The other muscles listed are unrelated to submucous cleft palate. The muscularis uvulae runs longitudinally along the medial palate from the tensor aponeurosis to the uvula. During speech, it functions as a flexible beam, lifting and bending the palate back and modifying the stiffness of the palate. 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. The superior pharyngeal constrictor muscle is a continuation of the posterior buccinator and tongue. This muscle curves back and up, ending in a tendinous median raphe attached to the occipital bone. It serves to pull the lateral and posterior pharyngeal walls medially, narrowing the pharynx. In patients with velopharyngeal insufficiency, a horizontal bend in this muscle elevates the mucosa to form ridge at the junction of the nasopharynx and oropharynx. In patients with cleft palate, this hypertrophied area is called Passavant ridge. 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. It acts to tighten the palate so the tongue has a firm surface against which to create a bolus of food. It is primarily involved in swallowing, rather than in speaking.

Answer 7

The correct response is Option E. The primary goal of nasoalveolar molding (NAM) is to reposition and approximate the alveolar segments and reshape the nasal cartilage. Financial analysis shows that NAM costs significantly less than lip adhesion, but this a labor-intensive process for the family. It is not designed to address palatal collapse. There is anecdotal information on impact of feeding, but this is not the principal goal as customized bottles work well in these children. There is no impact on midface growth in either place based on studies with more than 18 years, follow-up. Finally, the biggest benefit of NAM in the bilateral cases is the correction in columella height–even more than in unilateral cases.

Answer 8

The A flap is marked on the medial lip element and is the rotation flap. This flap is designed as a gentle curve from the height of Cupid’s bow on the cleft side of the medial cleft element to the junction of the philtrum and columella. This incision approaches the normal philtral column as much as needed to de-rotate and level Cupid’s bow. If more rotation is needed to level Cupid’s bow, a back-cut is made, but the normal philtral column is not violated. This incision is used in philtral subunit reconstruction and leveling Cupid’s bow. The B flap is made on the non-cleft, lateral lip element and is the advancement flap. This flap is the “wedge” that is placed in the defect created above the rotation flap as the medial lip element is de-rotated to level Cupid’s bow. This flap is marked by first finding the point on the white roll that will correspond to the height of Cupid’s bow on the medial cleft element. This point is found where the fullness of the lateral lip element begins to fade. A line is drawn from this point cephalad and is the same length as the normal philtral column as well as the marked edge of the A (rotation) flap. The C flap or columellar flap is made from the tissue medial to the A-flap incision. This small flap is rotated into the columella, lengthening the cleft side of the columella. It is also sewn to the D flap (alar base flap). The D flap or alar base flap is cut along the alar-facial groove, and this incision corresponds to the cephalad portion of the edge of the B flap (advancement flap). This alar groove incision is made as small as possible and long enough to allow differential rotation of the D flap and the B flap. The L flap is the leading edge of the lateral lip that can be used to fill the nasal lining defect. The M flap is the medially based flap from the rotation.

Answer 9

The correct response is Option A. 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.

Answer 10

The correct response is Option D. Errors occurring during the main embryonic period are responsible for major craniofacial anomalies. Cleft palate, which results from a failure of the palatine shelves to fuse, occurs primarily during weeks 7 to 8. Errors during weeks 1 to 2 result in death of the embryo. Clefting of the lip occurs during weeks 5 to 6. Ear and eye abnormalities occur during weeks 10 to 12.

Answer 11

The correct response is Option B. The primary disadvantage of iliac crest bone grafting is postoperative pain in the donor site, as there is no donor site morbidity with demineralized bone matrix (DBM). Cost for the increased operating room time for the iliac crest bone graft cancels out the increased cost for DBM. Primary artery bypass grafting is associated with a negative impact on mid face growth that is not seen with secondary bone graft regardless of the material used in the defect. An iliac crest bone graft increases operating room duration but not enough to cause any significant morbidity. Failure rates of DBM and iliac crest bone grafting for alveolar defects are equivalent.

Answer 12

The correct response is Option B. The genetics of the cleft lip (CL) deformity, with or without a cleft palate (CL/P) or an isolated cleft palate deformity (CP), are complicated and do not follow a typical mendelian inheritance pattern. The cause is thought to be multifactorial, involving both genetic and environmental factors. Most researchers agree that there is a genetic component to the development of the CL/P or CP deformities. This is supported by studies in cleft monozygotic twins, in whom the concordance rate is 30% to 60% compared with 1% to 4.7% in dizygotic twins. Families with a member with a CL/P or CP deformity have a higher risk than the general population of having another child with a similar deformity. The increased risk is outlined in the table below.

Answer 13

The answer is C: 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. Many patients with velopharyngeal insufficiency after 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 22q11.2 deletion syndrome, have generalized pharyngeal dysfunction with poor lateral pharyngeal 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. In this procedure, the posterior tonsillar pillars (palatopharyngeus muscles) are bilaterally dissected from the tonsillar fossae and rotated 90 degrees medially. Then they are affixed in an overlapping fashion against the posterior pharyngeal wall. Because the palatopharyngeus muscles are a continuation of the soft palate and lateral pharyngeal walls, this procedure narrows the entire pharyngeal port in a sphincteric fashion and augments the posterior pharyngeal wall. In a patient with little or no motion of the lateral pharyngeal wall, augmentation of the posterior pharynx is not appropriate. Although it may decrease the anterior-posterior coronal deficiency, it does nothing to treat the poor lateral wall motion. In addition, augmentation of the posterior pharynx has been attempted with multiple materials (including fat, Teflon, and silicone) in the past, without success. Today, the procedure has all but been abandoned. Likewise, a posterior pharyngeal flap (whether inferiorly based or superiorly based) is not appropriate because it may not completely correct the hypernasality associated with velopharyngeal insufficiency. This is true because it does not allow the lateral pharyngeal walls to move medially and seal off the lateral ports between the pharyngeal flap and lateral pharyngeal walls. V-Y pushback palatoplasty would not adequately correct the problem.

Answer 14

Correct answer is option A. 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%.

Answer 15

Correct answer is option B. The lateral maxillary prominences elevate, and then approach the midline, with fusion of the palatal shelves from anterior to posterior at about day 50 of embryonic development. The medial and lateral nasal prominences, frontonasal prominence and the mandibular prominence contribute to development of facial structures.

Answer 16

Correct answer is option A. 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. Laser resurfacing has not been shown to be an effective early modifier of scar outcomes.

Answer 17

The answer is D: 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. The levator palatini muscles are of primary importance in pharyngeal closure. These muscles join in the midline to form a sling in the normal palate, inserting into the palatal aponeurosis. Their mechanism of action is to pull the middle third of the soft palate superiorly and posteriorly, resulting in contact with the pharyngeal walls. In contrast, patients with cleft palate have abnormal insertion of the levator palatini on the posterior aspect of the hard palate. Cleft palate repair recreates the "normal" muscle sling by reorienting the muscles horizontally through a procedure known as intravelar veloplasty. If normal pharyngeal closure is not achieved following cleft palate surgery, additional procedures may be considered to allow for a more complete velopharyngeal closure. Sphincter pharyngoplasty or reconstruction with pharyngeal flaps is most frequently performed. The palatopharyngeus muscles pull the soft palate posteriorly, while the superior pharyngeal constrictors move the lateral and posterior pharyngeal walls. The uvulus muscle enhances velopharyngeal closure by thickening the uvula. Although the tensor veli palatini attaches to the eustachian tubes, acts to tense the soft palate, and is important for eustachian tube function, it does not provide an important contribution to velopharyngeal closure.

Answer 18

The correct response is Option B. 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. In addition, when there is normal shape and position of the mandible, surgery on the mandible is not indicated. Historically, because of the difficulty in performing the large required movements of the maxilla, the €œdifference € 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.

Answer 19

The correct response is Option E. 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. The Angle classification of occlusion is based on the relationship of the mesiobuccal cusp of the maxillary first molar to the buccal groove of the mandibular first molar. In patients with class I (normal) occlusion, the mesiobuccal cusp of the maxillary first molar lies in the buccal groove of the mandibular first molar. 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. This classification of malocclusion has two divisions; in class II, division 1, the lateral incisors are flared labially, and in class II, division 2, the incisors are lingually inclined. In Angle class III malocclusion, the mesiobuccal cusp of the maxillary first molar lies distal (posterior) to the buccal groove of the mandibular first molar and is located instead in the buccal groove of the mandibular second molar. Overbite describes the distance between the maxillary and mandibular incisors in the vertical plane with the jaws in centric occlusion, and overjet describes the distance between the maxillary and mandibular incisors in the horizontal plane with the jaws in centric occlusion.

Answer 20

The correct answer is C. In this child who is scheduled to undergo sphincter pharyngoplasty, the palatopharyngeus muscle should be incorporated within the flap. The technique of sphincter pharyngoplasty originally involved the bilateral transposition of salpingopharyngeus muscle flaps from the lateral pharyngeal walls. This process has been further developed to incorporate palatopharyngeal myomucosal flaps with intact neurovascular supply. These flaps are elevated from the posterior tonsillar pillar and inset into the posterior wall of the nasopharynx where it joins the palate, creating a smaller dynamic velopharyngeal sphincter. This newly developed procedure decreases the risk for reduction of the nasal airway, which may result in hyponasality and sleep apnea. The levator veli palatini muscles arise from the eustachian tube and sphenoid bone and insert into each other in the midline of the soft palate. These muscles elevate and posteriorly displace the soft palate against the posterior nasal wall during speech. This action closes the velopharyngeal port and opens the eustachian tube. The palatoglossus muscles form the anterior tonsillar pillars, which elevate the base of the tongue. The tensor veli palatini muscles arise from the medial pterygoid plate and eustachian tube, pass around the hamulus, and insert into each other in the midline of the soft palate. During swallowing, these muscles tense the soft palate, opening the eustachian tube and allowing the tongue to push the food bolus posteriorly. The uvular muscles arise from the palatine aponeurosis and the posterior nasal spine and insert into the uvula. These muscles are longitudinally oriented, cylindrical, paired structures located on the nasal side of the soft palate. Contraction during speech results in bulging of the soft palate with velopharyngeal closure. The uvula is lifted and bent backward.

Answer 21

The correct response is Option D. 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.

Answer 22

The correct response is Option A. One of the characteristic findings in the unilateral cleft deformity is a shortened columella. The C flap is designed to lengthen the columella. Other findings in the unilateral cleft nose include deviation and distortion of the septum (corrected with presurgical orthodontics), dislocation and slumping of the alar cartilage (corrected by dissecting the medial part of the cartilage and constructing a medial crus by suturing to the normal side), and flaring of the alar base (corrected with the alar cinch procedure).

Answer 23

The correct response is Option A. When repairing clefts of the palate, performing an intravelar veloplasty or reconstructing the levator veli palatini muscle within the soft palate (velum) is very important for adequate postoperative velopharyngeal competence. In the normal palate, the levator veli palatini muscle rests horizontally (coronally) within the middle third of the velum (soft palate) and functions as the motor of the velum, pulling the soft palate posteriorly and superiorly, allowing for apposition of the free edge of the velum against the posterior pharyngeal wall (velopharyngeal competence). In the cleft palate, the levator veli palatini muscle is €œclefted € and courses sagittally, in an anterior-posterior direction, attached abnormally to the posterior edge of the hard palate, the tenor aponeurosis, and the superior constrictor. The muscular uvuli arise as paired slips from the palatine aponeurosis and course sagittally in the velar midline, ending in the uvula. The musculi uvuli act as a flexible beam, providing a stiffness-modifying mechanism for the velum. 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. The superior pharyngeal constrictor arises from the hamulus and courses 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. The tensor veli palatini muscle arises from the skull base, courses inferiorly and medially around the hook of the hamulus and into the palate as the tensor aponeurosis, joining the velum (soft palate) to the hard palate.

Answer 24

The correct response is Option D. 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. Similar mendelian genetic models would apply to other scenarios. Although there are cases in which clefting is not transmitted in an autosomal dominant or recessive fashion, most cases of isolated cleft lip/palate follow a multifactorial model of genetic transmission.

Answer 25

The correct response is Option D. 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.

Answer 26

The correct response is Option C. Nasoalveolar molding (NAM) is the fabrication and adjustment of a custom intraoral appliance that narrows the cleft, aligns the cleft segments, shapes existing nasal structures, and expands deficient nasal tissues. Bilateral cleft lip and palate is associated with a nasal deformity that is defined by a short columella. The premaxilla is typically protuberant if the cleft is complete. NAM allows alignment of the premaxilla with the lateral alveolar segments. Once this is accomplished, nasal stents are placed to lengthen 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.

Answer 27

The correct response is Option C. This patient has severe maxillary retrusion and mid face hypoplasia in the setting of significant velopharyngeal deficiency. These two issues are at odds with one another. Advancing her mid face with a standard Le Fort I will worsen her velopharyngeal dysfunction (VPD), while addressing her VPD with a pharyngeal flap will make advancing her maxilla challenging. Only anterior segmental maxillary distraction will maintain her current velopharyngeal anatomy but allow for improved mid face projection. A pharyngoplasty can be performed relatively easily at a later date or as a secondary procedure. Performing a mandibular setback is not appropriate because she has normal mandibular projection.

Answer 28

The correct response is Option D. Establishing orbicularis oris continuity is a goal in both unilateral and bilateral cleft lip repair. Centralizing the columella is performed only in unilateral cleft lip repair. The prolabium is only apparent in bilateral cleft lips. Preparing the projecting premaxilla is done only in bilateral cleft lip repairs. Creating a rotation advancement flap is performed only in unilateral cleft lip repair.

Answer 29

The correct response is Option B. The bifid uvula is a marker for a submucous cleft palate, but neither the bifid uvula or the submucous cleft palate are an indication for surgery in isolation. Children with a cleft palate will often have hearing loss, but this is not addressed with VPI procedures. Snoring will likely be made worse by the VPI procedure and is not an indication for this procedure.

Answer 30

The correct response is Option D. 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. The levator palatini muscles are of primary importance in pharyngeal closure. These muscles join in the midline to form a sling in the normal palate, inserting into the palatal aponeurosis. Their mechanism of action is to pull the middle third of the soft palate superiorly and posteriorly, resulting in contact with the pharyngeal walls. In contrast, patients with cleft palate have abnormal insertion of the levator palatini on the posterior aspect of the hard palate. Cleft palate repair recreates the "normal" muscle sling by reorienting the muscles horizontally through a procedure known as intravelar veloplasty. If normal pharyngeal closure is not achieved following cleft palate surgery, additional procedures may be considered to allow for a more complete velopharyngeal closure. Sphincter pharyngoplasty or reconstruction with pharyngeal flaps is most frequently performed. The palatopharyngeus muscles pull the soft palate posteriorly, while the superior pharyngeal constrictors move the lateral and posterior pharyngeal walls. The uvulus muscle enhances velopharyngeal closure by thickening the uvula. Although the tensor veli palatini attaches to the eustachian tubes, acts to tense the soft palate, and is important for eustachian tube function, it does not provide an important contribution to velopharyngeal closure.

Answer 31

The correct response is Option A. 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. Families with Van der Woude syndrome, for example, are known to have an autosomal-dominant clefting mutation. If this were the case, this patient would then have a 50% chance of transmitting the cleft locus to a child. Similar Mendelian genetic models would apply to other scenarios.

Answer 32

The correct answer is posterior repositioning of the muscles of the soft palate. Both techniques attempt to place the muscle in an anatomically correct position.

Answer 33

The correct response is Option C. A cleft palate and craniofacial team accredited by the American Cleft Palate-Craniofacial Association (ACPA) must have, as a minimum core, health care providers from the speech-language pathology, surgery, and orthodontics specialties. These providers must participate in team meetings as appropriate for specific patient needs. The participation of these individuals should be documented in each patient’s team reports. The ACPA team must also have access to professionals in the disciplines of psychology, social work, audiology, genetics, general and pediatric dentistry, otolaryngology, and pediatrics/primary care. However, these providers are not considered core providers.

Answer 34

The correct response is Option D. Because cleft palate does not disrupt the palatopharyngeus muscles, they do not need to be repaired. Cleft palate repair aims to eliminate the oronasal fistula and optimize the function of the soft palate. The fistula is closed by creating a lining for the nasal and oral sides of the fistula. The abnormal attachments of the levator veli palatini and tensor veli palatini muscles to the hard palate are released, and the muscles are repaired in the midline, giving them a more functional transverse orientation. Finally, the musculus uvulae is repaired in the midline. This muscle provides bulk on the upper surface of the soft palate during velopharyngeal closure. Although the palatopharyngeus muscles are involved in velopharyngeal closure, they are not disrupted by cleft palate.

Answer 35

Correct answer is option B. Lateralization of the alar base in the unilateral cleft lip deformity is primarily due to two forces. When the primary palate is affected, the posterior collapse of the alar base leads to posterior and lateral displacement of the alar base. In addition, there are muscular forces at play which can also affect the position of the alar base. In the unaffected child, the force of the zygomaticus major is balance by the intact orbicularis oris sphincter. In the cleft lip deformity, the orbicularis oris is disrupted resulting in an imbalance of force to the lateral upper lip on the cleft side. This leads to an unopposed action of the zygomaticus major and lateralization of the upper lip and associated alar base. It is this unopposed muscular action that explains how the alar base can become lateralized in patients with a unilateral cleft lip and an intact upper alveolus.

Answer 36

The correct response is Option C. The palatopharyngeus muscle is one of the two structures (arches) that surround the oropharyngeal tonsils. Its action is to pull the pharynx upward during swallow to help separate the oropharynx from the nasopharynx. A sphincter pharyngoplasty transposes bilaterally the palatopharyngeal muscles to create an even greater barrier from the oropharynx to the nasopharynx to decrease velopharyngeal insufficiency (VPI). The hyoglossus muscle is an extrinsic tongue muscle and, like the stylohoid muscle, has one of its attachments to the hyoid bone. Neither of these muscles is commonly used to treat VPI. The levator veli palatini is an important muscle to mobilize in primary hard palate reconstruction in cleft cases, but not in secondary situations where the muscle is not functional. The pharyngeal constrictors are used in pharyngeal flaps and are primarily employed in secondary situations with a sagittal closure pattern.

Answer 37

Correct answer is option B. 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%. The overall risk of cleft lip with or without cleft palate in a Caucasian population is 1:1000. In a first‐degree relative, that risk is multiplied by 40; in a second‐degree relative, the risk is multiplied by 7; and in a third‐degree relative, the risk is multiplied by 3. However, the risk is increased when more than one relative is affected, if the family member has a severe form of the disorder, if the family member is of the sex least likely to be affected, or if the parents are consanguineous. For one affected parent, the risk of having one child with cleft lip with or without cleft palate is 4%, and the risk for a second child increases to 17%.

Answer 38

The correct response is Option A. Bilateral cleft lip repair is more commonly symmetric and is usually repaired as a one-stage repair around 3 to 6 months of age. Asymmetrical bilateral cleft lip repair can be done in one stage or two stages, and the sequencing has been controversial. However, two recent studies show convincing evidence that even for asymmetrical bilateral cleft lip repairs, a one-stage repair leads to overall better symmetry and cleft lip repair outcomes. Therefore, a two-stage bilateral cleft lip repair is incorrect. Cleft palate repair is typically performed closer to 9 to 12 months of age, therefore, cleft palate repairs at 6 to 9 months of age are less typical and irrelevant, as the two-stage repair is considered not optimal for this asymmetric bilateral cleft lip scenario.

Answer 39

The answer is C: 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. Many patients with velopharyngeal insufficiency after 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 22q11.2 deletion syndrome, have generalized pharyngeal dysfunction with poor lateral pharyngeal 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. In this procedure, the posterior tonsillar pillars (palatopharyngeus muscles) are bilaterally dissected from the tonsillar fossae and rotated 90 degrees medially. Then they are affixed in an overlapping fashion against the posterior pharyngeal wall. Because the palatopharyngeus muscles are a continuation of the soft palate and lateral pharyngeal walls, this procedure narrows the entire pharyngeal port in a sphincteric fashion and augments the posterior pharyngeal wall. In a patient with little or no motion of the lateral pharyngeal wall, augmentation of the posterior pharynx is not appropriate. Although it may decrease the anterior-posterior coronal deficiency, it does nothing to treat the poor lateral wall motion. In addition, augmentation of the posterior pharynx has been attempted with multiple materials (including fat, Teflon, and silicone) in the past, without success. Today, the procedure has all but been abandoned. Likewise, a posterior pharyngeal flap (whether inferiorly based or superiorly based) is not appropriate because it may not completely correct the hypernasality associated with velopharyngeal insufficiency. This is true because it does not allow the lateral pharyngeal walls to move medially and seal off the lateral ports between the pharyngeal flap and lateral pharyngeal walls. V-Y pushback palatoplasty would not adequately correct the problem.

Answer 40

The correct response is Option E. Bone morphogenetic protein-2 (BMP-2) works via osteoinduction to stimulate osteoprogenitor cells to differentiate into osteoblasts that begin new bone formation. Randomized controlled trials comparing BMP-2 to bone graft controls in alveolar cleft reconstruction have shown variable results, with most showing equivalence. An analysis of 17 randomized controlled trials of BMP-2 showed that severe prolonged edema was the most common complication, in some cases requiring steroid treatment or reoperation or leading to wound dehiscence. The use of recombinant human bone morphogenetic protein-2 (rhBMP-2) in patients younger than 18 years of age remains off-label. Concerns have been raised about a potential increase in malignancy, infection, and heterotopic ossification (HO) with the use of BMP-2 in spinal surgery. However, in these randomized controlled trials, HO and malignant transformation were not observed and rates of infection were no different. There is no difference in the rate of mucosal atrophy between these techniques.

Answer 41

The correct response is Option E. The patient has the three classic clinical features of submucous cleft palate. The overall incidence of overt submucous cleft palate is exceedingly rare (0.02 to 0.08%). Although there is some ongoing debate regarding the timing of surgical management, it is agreed that only a small number of patients will need surgical intervention for good speech outcomes. Velopharyngeal insufficiency (VPI) refractory to speech therapy is the most widely accepted indication for surgical correction. Speech cannot be adequately assessed until age 2.5 years at the earliest, and speech therapy cannot be initiated before this age. A typical course of management is diagnosis of VPI and initiation of speech therapy for 6 to 12 months. When therapy is found to be refractory, surgical intervention is planned. Surgical options are: excision with primary closure of the submucous cleft, pharyngeal flap, palatal pushback, palate repair with intravelar veloplasty, Furlow Z-plasty, or combined techniques. The approach is often tailored to the patient. In this case of a 6-month-old patient, it is too early to consider surgical intervention. The patient should be followed by a cleft palate team. When VPI is diagnosed by the speech therapist, therapy should be initiated, and if improvement is refractory to therapy, surgery should be planned. Nasoendoscopy can be used as a preoperative assessment to plan for surgery. There is no role for palatal elevators in the management of the submucous cleft palate. Speech outcomes are best if surgery is performed before age 7 years. Before age 7, 84% of patients go on to develop normal speech or occasional hypernasality versus only 64% of patients have an equivalent outcome when the operation is performed after age 7 years.

Answer 42

The correct response is Option A. This cleft involves both the soft and posterior hard palate, but does not extend into the alveolus or primary palate (Veau class II). Based on several recent studies, including a large systematic review, the rate of fistula formation following a Furlow repair of a Veau class II palatal cleft is 5% or less.

Answer 43

The answer is B: All patients with suspected VPI should have a thorough intraoral exam. Unrepaired oronasal fistula after cleft palate repair is an important cause of VPI to rule out prior to pursuing other interventions on the palate or pharynx.

Answer 44

The correct response is Option E. 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).

Answer 45

The correct response is Option A. 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. In contrast, secondary cleft palate occurs following unsuccessful fusion of the lateral palatine processes to each other and with the nasal septum. Because the lateral palatine process is derived from the maxillary prominence, these two structures cannot fuse. Unsuccessful fusion of the maxillary and mandibular prominences results in macrostomia. Failure of fusion of the maxillary prominence with the medial nasal prominence causes cleft lip (CL). Fusion of the medial nasal prominences leads to development of the nasal septum; therefore, these structures cannot fuse to each other. The premaxilla, nasal tip, and the philtrum and cupid's bow of the upper lip are also formed from fusion of the medial nasal prominences; the lateral portion of the upper lip is formed from the first branchial arch.

Answer 46

The correct response is Option C. 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.

Answer 47

The correct response is Option E. 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%.

Answer 48

Please note: Upon further review, this item was not scored as part of the examination. The correct response is Option E. Studies using three-dimensional CT scanning have demonstrated five consistent findings in unilateral cleft lip nasal deformity: The columellar base is deviated to the noncleft side The cleft-side alar base is more posterior than the noncleft-side alar base The noncleft-side alar base is farther from the midline than the cleft-side alar base The cleft-side piriform margin is farther posterior than the noncleft-side piriform margin The angle made by the medial and lateral crura would be more obtuse on the cleft side

Answer 49

The correct response is Option A. Within the normal soft palate, the levator veli palatine muscle forms a dynamic sling that elevates the velum toward the posterior pharyngeal wall during the production of certain sounds. In children born with cleft palate, there is a division of the musculature of the velum into separate muscle bellies with abnormal insertions along the posterior edge of the hard palate. The goals of cleft palate repair during infancy are twofold. The first goal is to establish a complete, watertight 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. The muscularis uvulae is also repaired in cleft palate repair. However, this muscle is typically not reoriented. Instead, it is simply approximated in the midline.

Answer 50

The A flap is marked on the medial lip element and is the rotation flap. This flap is designed as a gentle curve from the height of Cupid’s bow on the cleft side of the medial cleft element to the junction of the philtrum and columella. This incision approaches the normal philtral column as much as needed to de-rotate and level Cupid’s bow. If more rotation is needed to level Cupid’s bow, a back-cut is made, but the normal philtral column is not violated. This incision is used in philtral subunit reconstruction and leveling Cupid’s bow. The B flap is made on the non-cleft, lateral lip element and is the advancement flap. This flap is the “wedge” that is placed in the defect created above the rotation flap as the medial lip element is de-rotated to level Cupid’s bow. This flap is marked by first finding the point on the white roll that will correspond to the height of Cupid’s bow on the medial cleft element. This point is found where the fullness of the lateral lip element begins to fade. A line is drawn from this point cephalad and is the same length as the normal philtral column as well as the marked edge of the A (rotation) flap. The C flap or columellar flap is made from the tissue medial to the A-flap incision. This small flap is rotated into the columella, lengthening the cleft side of the columella. It is also sewn to the D flap (alar base flap). The D flap or alar base flap is cut along the alar-facial groove, and this incision corresponds to the cephalad portion of the edge of the B flap (advancement flap). This alar groove incision is made as small as possible and long enough to allow differential rotation of the D flap and the B flap. The L flap is the leading edge of the lateral lip that can be used to fill the nasal lining defect. The M flap is the medially based flap from the rotation.

Answer 51

cORRECT ANSWER IS OPTION c. Nasoalveolar molding (NAM) is the fabrication and adjustment of a custom intraoral appliance that narrows the cleft, aligns the cleft segments, shapes existing nasal structures, and expands deficient nasal tissues. Bilateral cleft lip and palate is associated with a nasal deformity that is defined by a short columella. The premaxilla is typically protuberant if the cleft is complete. NAM allows alignment of the premaxilla with the lateral alveolar segments. Once this is accomplished, nasal stents are placed to lengthen 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.

Answer 52

Correct answer is option A. A recent claims database analysis of 30-day complications after speech surgery demonstrated that respiratory complications (4.3%) were the most common after pharyngeal procedures, followed by need for critical care (2.7%), dehiscence (2.3%), and bleeding (1.4%). There were no reported infections.

Answer 53

The answer is A: By definition, a submucous cleft palate involves the anomalous insertion of the levator palatine muscles onto the posterior aspect of the hard palate. In normal anatomy, these muscles would be oriented in a transverse fashion. When contracting, the palate then is forced to elevate in a V‑shaped fashion and shorten to some degree. Nevertheless, not all children will develop speech difficulty or velopharyngeal incompetence. The tensor palatine muscles and posterior tonsillar pillars (the palatopharyngeal muscles) are in relatively normal location. Their function is impacted by the incorrectly positioned levator. Children with a submucus cleft have normal palate length.

Answer 54

The correct response is Option B. 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.

Answer 55

Correct answer is option E. 1. David DJ, Bagnall AD. Velopharyngeal incompetence. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders; 1990;4:2903-2921. 2. Randall P, LaRossa D. Cleft palate. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders; 1990;4:2723-2752.

Answer 56

The answer is D. The best choice of the listed options for a patient with a prior palatal lengthening procedure and VPI with a sagittal closure pattern is posterior pharyngeal flap. A sphincter pharyngoplasty would be a better choice for a coronal closure pattern in the setting of poor lateral wall motion. Pharyngeal augmentation has been tried with a variety of synthetic and autologous materials, but data about long-term effectiveness and safety is lacking. This patient’s hypernasal speech is unlikely to improve with continued speech therapy alone in the setting of her demonstrated anatomic VPI.

Answer 57

The correct response is Option E. 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.

Answer 58

The correct response is Option C. 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). Approximately half of the cases of cleft palate with associated anomalies will fall into established syndromes. 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. Associated anomalies in children with CL/P is approximately 10% to 15%. The type of associated anomalies would include musculoskeletal, cardiac, and neurological. Both cleft lip and cleft palate are controlled by multifactorial inheritance. Therefore, there is no distinctive pattern of inheritance within a single family. The risk to first-degree relatives can be estimated as the square root of the population risk. The risk is much lower for a second-degree relative. Higher chance of occurrence is also seen with greater degree of severity in the affected relative. The risk for development in a sibling of an affected child increases if more than one family member has the condition.

Answer 59

The correct response is Option C. The developing fetus has five facial prominences that are populated by neural crest cells. These five facial prominences are the frontonasal, paired maxillary, and paired mandibular. The frontonasal prominence gives rise to the nasal pit or placode, around which develops the medial and lateral nasal processes. A failure of fusion between the maxillary prominence and the medial nasal process results in a common cleft of the lip.

Answer 60

Correct answer is option A. The most appropriate management is reconstruction with an 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. 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 labialcolumellar junction. 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. The Gilles fan and Karapandzic flaps are oral circumference advancement flaps that were originally designed for lower lip reconstruction, but are also used for upper lip defects. These flaps rotate around the commissure and narrow the oral aperture. They are not designed for reconstruction of the philtrum as an aesthetic subunit. The nasolabial flap is a transposition flap that is derived from the nasolabial crease. It is most often used for reconstruction of the nasal alar subunit and has limited indications for lip reconstruction. It is not typically used for aesthetic subunit reconstruction of the philtrum.

Answer 61

The correct response is Option A. 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. Laser resurfacing has not been shown to be an effective early modifier of scar outcomes.

Answer 62

The correct response is Option D. Although all of the teeth listed have been described as being affected in patients born with cleft lip and palate, studies demonstrate that the permanent lateral incisor is most prone to be affected in the area of the cleft. It is frequently congenitally missing. It may also be hypoplastic or demonstrate a delay in root development and eruption. The other teeth can all be affected by alveolar clefting, but not to the degree to which the lateral incisor is affected. The lateral incisor is congenitally absent in up to 56% of patients with unilateral cleft lip and palate.

Answer 63

The correct response is Option B. 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.

Answer 64

The answer is C. In this child who is scheduled to undergo sphincter pharyngoplasty, the palatopharyngeus muscle should be incorporated within the flap. The technique of sphincter pharyngoplasty originally involved the bilateral transposition of salpingopharyngeus muscle flaps from the lateral pharyngeal walls. This process has been further developed to incorporate palatopharyngeal myomucosal flaps with intact neurovascular supply. These flaps are elevated from the posterior tonsillar pillar and inset into the posterior wall of the nasopharynx where it joins the palate, creating a smaller dynamic velopharyngeal sphincter. This newly developed procedure decreases the risk for reduction of the nasal airway, which may result in hyponasality and sleep apnea. The levator veli palatini muscles arise from the eustachian tube and sphenoid bone and insert into each other in the midline of the soft palate. These muscles elevate and posteriorly displace the soft palate against the posterior nasal wall during speech. This action closes the velopharyngeal port and opens the eustachian tube. The palatoglossus muscles form the anterior tonsillar pillars, which elevate the base of the tongue. The tensor veli palatini muscles arise from the medial pterygoid plate and eustachian tube, pass around the hamulus, and insert into each other in the midline of the soft palate. During swallowing, these muscles tense the soft palate, opening the eustachian tube and allowing the tongue to push the food bolus posteriorly. The uvular muscles arise from the palatine aponeurosis and the posterior nasal spine and insert into the uvula. These muscles are longitudinally oriented, cylindrical, paired structures located on the nasal side of the soft palate. Contraction during speech results in bulging of the soft palate with velopharyngeal closure. The uvula is lifted and bent backward.

Answer 65

The correct response is Option E. Cleft lip with or without palate (CLP) has different epidemiologic characteristics and presentation, compared with isolated cleft palate only (CPO). CLP has a higher incidence (1 in 700, versus 1 in 1500), higher male predominance (M:F 2:1, versus F:M 3:2), incidence varies by ethnicity (Asian:Caucasian:African 4:2:1, versus no ethnicity difference), and less syndromic association (15% versus 50%). Cleft lip is always associated with some degree of cleft nose deformity, whereas CPO does not involve the nose. Therefore the correct response is that cleft lip/palate is less likely to be associated with a syndrome.

Answer 66

The correct response is Option C. 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%. -Normal parents, two children with a CL/P: the risk for the next child is 9% (as in the scenario described.) -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.

Answer 67

The correct response is Option D. With a “bow tie” pattern seen on the nasoendoscopy, the patient is an ideal candidate for sphincter pharyngoplasty, which will bring the lateral walls in more centrally, so the velum and pharynx can close off the velopharyngeal port. Pharyngeal flap surgery is ideal for patients who have a large central gap or sagittal closure pattern caused by palatal hypotonia or shortened palatal length. Palatal soft tissue augmentation is ideal for patients with a very small central gap following adenoidectomy. A palatal lift appliance is typically used in patients with adequate palatal length, but inadequate motor function. The patient described in this scenario has excellent motor function of the velum, but poor lateral wall motion. Speech therapy does not correct the anatomic deficiency in this patient population.

Answer 68

The correct response is Option C. Le Fort I advancement and lengthening can result in dramatic changes to facial appearance. These include increased midfacial projection and fullness, increased upper lip vermilion fullness, decreased upper lip height, decreased depth of the nasolabial folds, and increased tooth show in repose and smile. Additionally, as the bony platform of the caudal piriform region is advanced, the alar base widens, and the tip rotates cephalad, increasing the nasolabial angle. It is important to counsel patients on this expected outcome preoperatively so that they are fully informed of the facial changes they will experience.

Answer 69

The correct response is Option A. The most appropriate management is reconstruction with an 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. 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. 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. The Gilles fan and Karapandzic flaps are oral circumference advancement flaps that were originally designed for lower lip reconstruction, but are also used for upper lip defects. These flaps rotate around the commissure and narrow the oral aperture. They are not designed for reconstruction of the philtrum as an aesthetic subunit.

Answer 70

The correct response is Option B. Patients with cleft lip and palate frequently have absence of teeth in the alveolar cleft or teeth that may be grossly abnormal or that erupt at an inappropriate angle and require removal. The lateral incisors are most commonly affected, although central incisors and canines may also be affected. Alveolar bone grafting during the mixed dentition phase restores adequate bone support for subsequent placement of an endosseous titanium implant, to which a permanent crown may then be attached. Many studies have reported the efficacy and safety of this approach. This has become the dentofacial prosthetic treatment of choice for the replacement of a single tooth due to its appearance, functionality, and longevity. A removable partial denture is one that rests on the surrounding soft tissues of the alveolar ridge and palate. Although aesthetics may be reasonable, it may cause irritation of the surrounding soft tissues and may produce movement during function. It is often a temporary solution at best. A three-unit fixed partial denture is a prosthesis which spans the gap produced by the missing tooth by anchoring to the adjacent two teeth. However, the abutment teeth often require reduction to permit fixation of the prosthesis. While certainly longer lasting than a removable partial denture, a fixed partial denture will need to be replaced periodically, and therefore would not be the best choice for this young patient. A palatal obturator is a prosthesis used to treat a residual oronasal fistula by physically blocking air escape during speech. This decreases hypernasality. It rests on the soft tissues of the palate and may anchor to the alveolar ridge or teeth. It does not, however, play a role in dental restoration. Nasoalveolar molding is a prosthesis-based treatment used early in life, typically for wide clefts, prior to repair of the lip and palate, but it is not used for dental restoration.

Answer 71

The correct response is Option B. Patients with cleft palate who are late-presenting and have undergone previous procedures that were performed out of the preferred sequence can be especially challenging cases. This patient, with a bilateral cleft lip and palate, has only had her lip and palate repaired. Yet she has significant speech, skeletal, and soft tissue sequelae of her cleft including unrepaired anterior hard palate fistulae and alveolar fistulae, inadequate central lip fullness (whistle deformity), a wide and poorly projected nose, mid face hypoplasia, and velopharyngeal insufficiency. Sequencing procedures to address these issues is crucial to having favorable surgical outcomes. Addressing speech is the patient's main concern and should be performed first. This includes closing of the oronasal fistula along with a premaxillary setback followed by a pharyngoplasty, if necessary. Alveolar bone grafts should be performed to stabilize the maxillary dental arch and minimize tooth injury. This should be followed by a lip revision to establish the lip-tooth relations prior to undergoing Le Fort I advancement. Finally, a rhinoplasty can be performed once the maxilla has been advanced.

Answer 72

Correct answer is option D. McComb, Salyer and others have demonstrated that manipulation of the lower lateral cartilages as the time of infancy is not detrimental to future nasal growth. As the blood supply to the nose is robust, several surgical approaches can be used to access the lower lateral cartilages during the time of primary cleft rhinoplasty. These exposures include a medial approach, lateral approach or nostril rim incisions. An open rhinoplasty is not typically used in primary cleft rhinoplasty however this technique is often used in secondary cleft rhinoplasty.

Answer 73

The correct response is Option A. The first branchial arch contributes to the formation of the mandible, the tragus, and the anterior helix. The first branchial cleft gives rise to the external auditory canal. The second branchial arch contributes to the formation of the majority of the external ear–the antitragus, remainder of the helix, antihelix, and crura all arise from the second branchial arch. The second branchial cleft is typically obliterated during development, but may persist in the form of a second branchial cleft cyst. Reference(s) 1. Adams A, Mankad K, Offiah C, Childs L. Branchial cleft anomalies: a pictorial review of embryological development and spectrum of imaging findings. Insights Imaging. 2016 Feb;7(1):69-76. 2. Parada C, Chang Y. Mandible and tongue development. Curr Top Dev Biol. 2015;115:31-58.

Answer 74

The correct response is Option A. The patient in this question has had multiple palate surgeries and severe midface hypoplasia. One of the sequelae of severe midface hypoplasia is obstructive sleep apnea, which is confirmed by the patient's abnormal polysomnogram. The best treatment for this patient is a trial of CPAP. Although midface advancement surgery (either conventionally or with distraction) is often used to address obstructive sleep apnea, this patient is a poor candidate for the surgical options provided. The patient is in mixed dentition, and a Le Fort I level surgery would risk permanent injury to his unerupted adult teeth. Although a Le Fort III osteotomy would avoid injury to tooth roots, it would also advance his infraorbital rims, which are not affected in patients with cleft lips. A tracheostomy will bypass his midface level obstruction; however, it is associated with significant cost, burden of care, and a 1% annual mortality risk and should be avoided if less invasive options exist.

Answer 75

The correct response is Option D. 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.

Answer 76

The correct response is Option E. A Le Fort I procedure is needed in 25 to 40% of patients with cleft lip and palate, and velopharyngeal insufficiency (VPI) is often seen postoperatively. Preoperative soft palatal length and postoperative pharyngeal depth have been associated with the need for a VPI procedure. In a 17-year-old, the normal soft palatal length is 32 mm and the normal nasopharyngeal depth is 24 mm. A significant velopharyngeal gap of greater than 5 mm cannot be adequately corrected with a Furlow palatoplasty or sphincter pharyngoplasty. Poor lateral wall motion alone would be a finding that supports a sphincter pharyngoplasty. The Le Fort I advancement distance has not been directly related to the need for a specific type of procedure. References

Answer 77

The correct response is Option D. Velopharyngeal port (VP) function results from the coordinated effort of the musculature of the velum and pharyngeal wall. The levator veli palatini muscle is the key muscle responsible for elevation of the soft palate, which is necessary for VP closure. In patients with a cleft palate or submucous cleft palate, clefting of the muscle and its abnormal insertions result in loss of the normal levator sling that elevated the palate. Reconstruction of this levator sling through an intravelar veloplasty (IVVP) is a critical component of palatoplasty procedures to ensure optimal VP function and speech development. In its normal anatomic relationship, the levator veli palatini muscle has a muscular origin from the petrous temporal bone, the carotid canal, and cartilaginous Eustachian tube. The muscle forms a muscular sling by joining with its paired contralateral muscle running in a transverse orientation in the middle 50% of the velum. The pathologic orientation of the levator muscle is seen in a cleft palate. The muscles are not transversely oriented. Instead of joining with the contralateral levator muscle in the midline, the muscles are instead oriented more sagittally and insert into the posterior edge of the hard palate and tensor aponeurosis laterally. The tensor veli palatini muscle originates from the greater wing of the sphenoid and lateral aspect of the cartilaginous and membranous Eustachian tube. The majority of its fibers pass around the pterygoid hamulus as a tendon and insert into the tensor aponeurosis, which occupies the anterior 20 to 30% of the velum. This muscle acts as a shock absorber for the velum and primary dilator of the Eustachian tube. The palatoglossus muscle is also a paired muscle of the velum that descends in the anterior faucial pilar anterior the palatine tonsil and inserts into the dorsolateral aspect of the posterior tongue. It contributes to velar depression and glossal elevation. The palatopharyngeus muscle originates from the posterior palate and runs posterior to the tonsillar fossa. These muscles are often used for sphincter pharyngoplasty.

Answer 78

The correct response is Option E. Lip adhesion is the surgical version of nasoalveolar molding (NAM) in terms of accomplishing decreased cleft width and alveolar alignment; however, it does not do much for the nasal contour. The primary advantage of lip adhesion surgery is that it minimizes the burden on the family. It costs more, potentially can create more scar, and has a higher complication rate in the form of either scar or wound dehiscence. There is no proven clinical outcome advantage, and some would argue that because NAM also addresses the nose, the outcome is better, but that will take additional studies to validate.

Answer 79

The correct response is Option E. The above patient scenario is typical of a cleft lip and palate patient with missing lateral incisor on the cleft side and significant maxillary growth restriction leading to class III malocclusion. There are multiple options for surgical correction of the deformity described, however they must address the patient's complaints of malocclusion, address the missing dental unit, and provide a lasting reconstruction. The best option is a two-piece Le Fort I advancement with a surgical canine substitution. This allows restoration of the dental arch and closure of the space, with the canine filling the lateral incisor spot. Postoperatively, the prosthodontist will recontour the canine so it appears like a lateral incisor. This patient is likely skeletally mature at 17 years old (14 to 16 years for females, 16 to 18 years for males). Bilateral sagittal split osteotomy would address the malocclusion without addressing the mid face deficiency. However, there is not enough bone stock for a standard osseointegrated implant, which usually requires at least 10 to 15 mm of alveolar height. Although a fixed bridge will address the missing tooth, neither it nor alveolar transport distraction will fix her malocclusion.

Answer 80

The correct response is Option B. 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).

Answer 81

Correct answer is option D. The alar base is displaced posteriorly and laterally in the both the unilateral and bilateral cleft lip nasal deformity. This displacement is due to the posterior collapse of the lesser segment, combine with the unopposed action of the zygomaticus major on the lateral upper lip and associated alar base.

Answer 82

The correct response is Option B. Van der Woude syndrome is an autosomal dominant condition affecting 1:40,000 to 1:100,000 live births. It involves lower lip pits or mounds, and cleft lip and/or palate. Fifteen percent do not have the associated pits, so genetic associations are useful in diagnosis and counseling. IRF6 is the mutation associated with van der Woude syndrome as well as popliteal pterygium syndrome (webbing behind the knee, lower lip pits, cleft lip and/or palate, and genital hypoplasia). PTCH1 is found in Gorlin syndrome, also known as basal cell nevus syndrome. FGFR1 is associated with Kallmann syndrome (hypogonadotrophic hypogonadism with anosmia, cleft lip and/or palate, renal aplasia/agenesis, dental defects). The majority of orofacial clefts are nonsyndromic (70% of all cleft lip/palate; 50% of all cleft palate only). Cleft lip/palate is more common in males (2:1) and cleft palate only is more common in females (1:2). 22q11.2 deletion is associated with DiGeorge sequence or velocardiofacial syndrome (cardiac anomalies, abnormal facies, thymic aplasia, cleft palate, hypocalcemia/hypoparathyroidism—CATCH). Trisomy 13 is associated with Patau syndrome (cleft palate, CNS disorders, microcephaly, polydactyly, rocker bottom foot, urogenital defects, and cardiac anomalies.

Answer 83

Correct answer is option D. 1. Fara M. The musculature of cleft lip and palate. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co; 1990;4:2612. 2. Hobar PC, Johns DF, Flood J, et al. Cleft palate repair and velopharyngeal insufficiency. In: Aston SJ, Beasley RW, Thorne CH, eds. Grabb & Smith's Plastic Surgery. 5th ed. Philadelphia, Pa: Lippincott-Raven; 1997:263.

Answer 84

The correct response is Option A. Informed consent in the pediatric surgical patient can present a pediatric plastic surgeon with complex ethical issues. This is especially true when minor children and their legal guardians disagree about surgical decisions. In this common scenario described, the parents are interested in cleft nasal revision, whereas the patient is not. In such a scenario, it is important to understand both stakeholders’ viewpoints. Is the patient worried about missing vacation or a sporting activity over the summer or does his deformity not bother him? Do the parents have perspective they have not shared or do they have an unreasonable expectation for their child? These are all important considerations. Of the answer choices, only open discussion starts to bring the competing parties closer together.

Answer 85

The correct response is Option D. Velocardiofacial syndrome (VCF syndrome), also termed 22q11.2 deletion syndrome, is characterized by overt or submucous clefting of the palate, hypotonia, cardiac anomalies, hypocalcemia due to hypoparathyroidism, immune deficiencies, and variable learning disabilities. There should be strong clinical suspicion in any child with cardiac anomalies and a cleft palate, and most patients have a distinct facial appearance (ie, elongated face with a wide nose, small ears, and lower facial muscle tone). The diagnosis can be confirmed in 95% of patients by testing a blood sample using fluorescence in situ hybridization (FISH) for a deletion in chromosome 22q11.2. Repeated studies have demonstrated worse speech outcomes in this patient population following any cleft repair compared with nonsyndromic cleft patient and most other cleft syndromes. This difference has been largely attributed to decreased oropharyngeal tone and/or muscle coordination, but other influences, such as learning differences, may have a role. The risk for persistent velopharyngeal insufficiency following repair of cleft palate is considerably higher in this group than non-VCF syndrome patients undergoing the same repair. The risk for palatal fistula and wound dehiscence is not higher in VCFS; postoperative respiratory compromise can be seen in VCFS patients with concurrent Robin sequence, but this is not discussed in the clinical vignette. Lastly, air embolism is a complication that is not associated with cleft repair in any patient population.

Answer 86

The correct response is Option D. Sphincter pharyngoplasty is a secondary (speech) procedure for cleft palate that rotates the posterior tonsillar pillars as superiorly based flaps to line the posterior pharynx, thereby narrowing the velopharyngeal sphincter. The posterior tonsillar pillars contain the palatopharyngeus muscles. The other muscles are not used in sphincter pharyngoplasty: levator veli palatini runs through the palate to elevate the palate; palatoglossus runs through the anterior tonsillar pillar to depress the palate; musculus uvulae is the muscle of the uvula, and tensor veli palatini contributes to eustachian tube pressure modulation.

Answer 87

The correct response is Option D. A higher prevalence of dental anomalies is expected in children with cleft lip and palate. Agenesis is the most prevalent anomaly found typically in greater than 50% of patients. The most commonly affected tooth is the permanent lateral incisor on the cleft side. Supernumerary teeth are the second most frequently occurring dental anomaly. Dysplastic teeth, ectopic teeth, and translocation of the teeth are all less common than tooth agenesis in this patient population.

Answer 88

The correct response is Option B. The child has already had a lip repair and although she may require revision in the future, the primary goal at this age is palate repair. A repair after age 18 months is considered a late palate repair. The literature supports that late palate repairs have worse speech outcomes. Additionally, internationally adopted children with late palate repairs have higher fistula rates, more hypernasality and velopharyngeal insufficiency, and are more likely to require speech surgery.

Answer 89

The correct response is Option A. 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.

Answer 90

The answer is D. The best choice of the listed options for a patient with a prior palatal lengthening procedure and VPI with a sagittal closure pattern is posterior pharyngeal flap. A sphincter pharyngoplasty would be a better choice for a coronal closure pattern in the setting of poor lateral wall motion. Pharyngeal augmentation has been tried with a variety of synthetic and autologous materials, but data about long-term effectiveness and safety is lacking. This patient’s hypernasal speech is unlikely to improve with continued speech therapy alone in the setting of her demonstrated anatomic VPI.

Answer 91

The correct response is Option C. The success of sphincter pharyngoplasty, a secondary technique performed for correction of velopharyngeal insufficiency, is most dependent on elevation of myomucosal flaps of the palatopharyngeus muscle. These flaps can be used to construct a sphincter in the pharyngeal wall. This technique effectively obliterates the lateral ports of the velopharyngeal mechanism and decreases the diameter of the central port. Because the levator veli palatini muscle is important for initial palatal closure and movement, it would not be available in this child who previously underwent cleft palate repair. The musculus uvulae is a small sagittal muscle of the uvula that is not useful in sphincter creation. The salpingopharyngeus muscle arises from the inferior portion of the auditory tube near its orifice. It passes downward and blends with the posterior fasciculus of the palatopharyngeus muscle. The tensor veli palatini follows close to the eustachian tube and is also affected in patients with cleft palate.

Answer 92

The correct response is Option B. 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.

Answer 93

The correct answer is (D). Because only 10% of patients with a submucous cleft palate have poor speech, don’t correct the cleft unless they have poor speech documented when they are approximately 5 years old.

Answer 94

The correct response is Option E. The diagnosis for this patient is Van der Woude syndrome. Van der Woude syndrome is the most common syndrome associated with cleft lip with or without cleft palate. It is inherited in an autosomal dominant manner with incomplete penetrance, thus clinical presentation for patients with Van der Woude syndrome is variable. This patient has a de novo mutation, since no other family members reported clefting. Morphology for these patients can range from lip pits to bilateral complete cleft lip and palate. Van der Woude syndrome is most commonly related to mutations in interferon regulatory factor 6 (IRF6). Since this is an autosomal dominant condition, the best estimate for this woman's next child to be affected is 50%. The other percentages are all reported in the literature and are related to non-syndromic clefting and familial patterns. If there is one affected sibling with an isolated cleft lip, the risk factor is 2.5%. If there is one affected sibling with a unilateral cleft lip and palate, the risk factor is 4.2%. If there are two affected siblings, the risk factor is 10%. If parental cleft and sibling clefting are present, the risk factor has been reported at 17%. Reference(s) 1. McDonald-McGinn DM, DiCairano L, Mennuti MT. Prenatal and Genetic Counseling. In: Losee JE, Kirschner RE eds. Comprehensive Cleft Craft. 2nd ed. Boca Raton, FL: CRC Press; 2016; 1: 285-301. 2. Hixon K, Rhea L, Standley J, et al. Interferon Regulatory Factor 6 Controls Proliferation of Keratinocytes From Children With Van der Woude Syndrome. Cleft Palate Craniofac J. 2017 May; 54(3):281-286. 3. Greives MR, Camison L, Losee JE. Evidence-based medicine: unilateral cleft lip and nose repair. Plast Reconstr Surg. 2014 Dec;134(6):1372-80.

Answer 95

The correct response is Option D. Reconstruction of the cleft alveolus seeks to close any remaining oronasal fistulae, stabilize the maxillary segments into a single dental arch, and provide bone for eruption of teeth adjacent to the cleft with optimal periodontal support. Primary bone grafting involves placement of a bone graft at the time of the initial cleft lip repair in the neonatal period. It is different from performing a gingivoperiosteoplasty (GPP), in which soft tissue repair of the alveolus is performed to close the anterior palatal fistula and encourage bone formation in the gap. Primary bone grafting has fallen out of favor in most American cleft centers, as several studies have shown diminished maxillary growth, poor arch relationships, and greater need for orthognathic surgery following this procedure. Secondary bone grafting can be performed at various times during dental development. Many children with cleft lip and palate have a poorly formed or missing lateral incisor. When a normal lateral incisor is present, a compelling argument can be made for earlier secondary bone grafting prior to its eruption to optimize the periodontal health and longevity of this tooth. The child in the clinical vignette is missing a lateral incisor on the side of the alveolar cleft. Bone graft material will only heal successfully to adjacent bone. Exposed periodontal ligament, dentin, and enamel from erupted teeth do not adhere/heal to bone graft, and graft material adjacent to these structures will resorb. Growth following secondary bone grafting between 8 and 12 years of age has been shown to be equivalent to growth seen in patients with similar clefts that remained ungrafted. Additional studies have shown decreased success rates for bone grafts performed after the eruption of the secondary canine. Optimal bone graft success seems to result when grafting is performed when the secondary canine root is one-half to two-thirds developed, which allows the canine to erupt through the graft. The maxillary canine erupts between 11 to 12 years of age. Due to the need to graft before canine eruption and the variability in dental eruption in children with clefts, the optimal timing for alveolar bone grafting is often between 8 and 12 years of age, but treatment must be tailored to the patient’s chronological and dental age. References

Answer 96

Correct answer is option C. The Mulliken technique of bilateral cleft lip repair is a commonly used single-staged technique that concurrently corrects the nasal deformity. Primary rhinoplasty techniques addressing the bilateral cleft lip nasal deformity have been challenged by the risk of disrupting the blood supply to the prolabium. Therefore, only a small number of techniques have been characterized. McComb described a two-staged technique in which an open rhinoplasty style incision is made on the nasal tip in the first stage. Primary rhinoplasty is performed at that time (as well as a lip adhesion) and the rhinoplasty incision is closed in a V to Y fashion. In the second stage, the bilateral cleft lip repair is performed. Trott described a single stage technique in which an extended open rhinoplasty is performed that includes the philtrum with the columellar flap. The blood supply to the philtrum is tenuous with the Trott technique which has led to a limited use of this approach. Cutting uses a complete transfixion incision which continues inferiorly under the medial footplates and prolabium to create a composite flap of philtrum, columella and medial crura. This flap is rotated superiorly and posteriorly and the lower lateral cartilages are manipulated from posterior and inferior. Mulliken approaches the lower lateral cartilages through bilateral nostril rim incisions. In his original description of this technique Mulliken described the use of an additional midline nasal incision, however this is not felt to be an optional incision.

Answer 97

The correct response is Option B. Classification of a cleft palate is based on the degree of anatomical disruption of the primary and secondary palates. The Veau classification divides the cleft palate into four categories and is a widely utilized scheme for cleft palate classification. The Veau II cleft involves the hard and soft palate and is limited to the secondary palate; therefore, it is the most appropriate answer. Veau Class I is an incomplete cleft involving the soft palate. A Veau III is a complete unilateral cleft lip and palate, where the nasal septum fails to fuse with one palatal shelf, whereas a Veau IV is a complete bilateral cleft lip and palate in which both palatal shelves fail to fuse with the nasal septum. Other classification systems, such as the Kernahan ?Y? classification, are also widely used.

Answer 98

The correct response is Option D. The posterior pharyngeal flap for velopharyngeal insufficiency (VPI) is generally elevated with a superior base to keep the flap high (at the junction of the naso- and oropharynx) rather than low in the oropharynx. The mucosa and superior constrictor muscle and buccopharyngeal fascia (visceral pretracheal fascia) are elevated, exposing the loose areolar tissue of the retropharyngeal space anterior to the prevertebral fascia. The buccopharyngeal fascia is thin and closely invests the constrictor muscles. Some surgeons elevate the flap superficial to the buccopharyngeal fascia. Some fibers of the middle constrictor may be included in longer pharyngeal flaps for large gaps, but the inferior constrictor is too caudad to be included. The palatopharyngeus muscle is included with sphincter pharyngoplasty, not pharyngeal flaps. The prevertebral fascia is thick and is attached to the buccopharyngeal fascia by loose areolar tissue. This is an avascular plane that is a potential space called the retropharyngeal space. The prevertebral fascia is the deepest layer of cervical fascia and surrounds the cervical column and associated musculature. The investing layer of the deep cervical fascia is the most superficial layer and contains the platysma. The pretracheal layer or buccopharyngeal layer invests the constrictors and esophagus musculature.

Answer 99

The correct response is Option C. The photograph demonstrates through transillumination an absent normal levator muscle sling found in an occult submucous cleft palate. Velopharyngeal insufficiency (VPI) after tonsillectomy is a rare but known complication but can occur as frequently as 1:3000 to 1:10,000 tonsillectomies. Most of these complications occur in patients with either an occult or overt submucous cleft palate. With this anatomic variant, the principle of repair is either to restore normal anatomy with a two-flap palatoplasty or to pair the levator muscles into a functional sling. Techniques to lengthen the palate, such as a Furlow repair, help to obturate the nasal escape and also to improve VPI speech. As such, 8 weeks after VPI onset, the speech pathologist will have difficulty correcting the anatomic deficiency of lacking levator sling. The tensor tenopexy stabilizes the tendon of the tensor tympany to the hamulus during cleft palate repair, and stents open the eustachian tube but will not improve VPI speech

Answer 100

The answer is A: By definition, a submucous cleft palate involves the anomalous insertion of the levator palatine muscles onto the posterior aspect of the hard palate. In normal anatomy, these muscles would be oriented in a transverse fashion. When contracting, the palate then is forced to elevate in a V‑shaped fashion and shorten to some degree. Nevertheless, not all children will develop speech difficulty or velopharyngeal incompetence. The tensor palatine muscles and posterior tonsillar pillars (the palatopharyngeal muscles) are in relatively normal location. Their function is impacted by the incorrectly positioned levator. Children with a submucus cleft have normal palate length.

Answer 101

The correct response is Option E. The use of buccal musculomucosal flaps has gained in popularity for both primary palate repair and secondary speech surgery. The many advantages of adding these flaps to palate surgery include the ability to posteriorize the levator muscles, eliminate dead space, and lengthen the palate. The patient described, who has great palate function but a short palate, is an ideal candidate for buccinator musculomucosal flaps rather than a pharyngoplasty, which may lead to obstructive sleep apnea. Understanding the blood supply is critical to performing this flap in a reliable fashion.

Answer 102

Correct answer is option C. The unilateral cleft lip and nasal deformity is complex and affected multiple structures of the nose. The displacement of the greater and lesser segments combined with the unilateral attachment of the orbicularis oris to the anterior/caudal septum leads to severe septal deviation. The alar base is displaced laterally and posteriorly due to the unopposed pull of the zygomaticus major on the cleft side as well as the posterior collapse of the lesser segment. The columella is foreshortened and the lower lateral cartilage rotated inferiorly on the cleft side due to the displacement of the caudal septum, the greater segment and the alar base.

Answer 103

The correct answer is the incisive foramen, located immediately posterior to the central incisors. The maxillary prominence forms the maxilla; the medial nasal prominence is part of the structure that forms the primary palate. The greater palatine foramen is the exit site for the greater palatine artery.

Answer 104

The correct response is Option C. Secondary alveolar bone grafting is performed in mixed dentition. The goals of alveolar bone grafting are to stabilize the alveolus and allow adequate bone stock to support tooth health for the permanent canine (if that is the tooth erupting into the cleft). Generally, the timing of bone grafting is done before the canine has fully erupted. If it has fully erupted, the root may be exposed in the cleft and not well surrounded by bone. It is preferable to perform the bone graft after the deciduous teeth are gone, but before the tooth is fully erupted. An appropriate time to perform the bone graft is when the canine is crowning. Early grafting may be detrimental to midface growth. Orthodontic preparation may be required before performing secondary bone grafting, and there must be enough permanent dentition to support this.

Answer 105

Correct answer is option D. Columellar struts are commonly used grafts in secondary cleft rhinoplasty. The lower lateral cartilages in these patients tend to be floppy, with limiting structural integrity to withstand the postoperative forces of scar contracture. In addition, nasal projection is often lacking. For these reasons, columellar struts are commonly used in isolation or in combination with other grafts in secondary cleft rhinoplasty. As rigid graft material is required, nasal septum and rib cartilages are the preferred donor material for columellar struts in this patient population. The internal nasal valve is formed by the nasal septum, upper lateral cartilages and nasal floor. Placement of a columellar strut will not affect any of these areas.

Answer 106

The correct response is Option A. Buccal fat pad flaps are a technically simple, quick option for closure of mucosal defects in palatal closure. Their use has not been associated with significant donor site morbidity, and they are able to reach across the transverse maxillary arch, even in wide clefts, which makes them useful in closure or reinforcement of mucosal defects in palatoplasty. A superiorly based pharyngeal would not only create more donor site morbidity than using a buccal fat pad flap, but using the pharyngeal flap could also cause obstructive sleep apnea, and therefore is best used as a secondary or tertiary procedure in case of persistent velopharyngeal insufficiency (VPI), not as an adjunct in primary palatoplasty. A hard palate myomucosal flap would not adequately address the problem of nasal mucosal loss, and attempting to use one to cover nasal lining would likely cause significant donor site complications, such as exposure of the hard palate maxillary bone. Sphincter pharyngoplasty is not an option for nasal mucosal closure, since this procedure is designed to augment the posterior pharynx and tighten the oronasal aperture. Vomer flaps are useful in nasal lining closure for the hard palate, but are not useful for closure of the nasal lining of the soft palate.

Answer 107

Correct answer is option C. The best time to perform secondary cleft rhinoplasty is a matter of debate and each decision should be individualized to the needs of the patient. Performing secondary rhinoplasty prior to the time of facial maturity has the psychological benefit of earlier correction. In the growing face, however, many structures are not manipulated for concern of negatively affecting future facial growth. These structure include the nasal septum and the nasal bones. Therefore correction of significant airway obstruction or dorsal deviation may not be possible until after the time of facial maturity. Performing secondary rhinoplasty after a child is fully mature eliminates the variable of future growth into the final result of the reconstruction. In addition, septal and bony manipulation can be safely performed, providing the surgeon access to the full spectrum of rhinoplasty techniques. Although many consider waiting until the time of facial maturity the ideal treatment plan for secondary rhinoplasty, this may not always be a reasonable or caring plan for patients with a significant nasal deformity.

Answer 108

The correct response is Option E. The diagnosis for this patient is Van der Woude syndrome. Van der Woude syndrome is the most common syndrome associated with cleft lip with or without cleft palate. It is inherited in an autosomal dominant manner with incomplete penetrance, thus clinical presentation for patients with Van der Woude syndrome is variable. This patient has a de novo mutation, since no other family members reported clefting. Morphology for these patients can range from lip pits to bilateral complete cleft lip and palate. Van der Woude syndrome is most commonly related to mutations in interferon regulatory factor 6 (IRF6). Since this is an autosomal dominant condition, the best estimate for this woman's next child to be affected is 50%. The other percentages are all reported in the literature and are related to non-syndromic clefting and familial patterns. If there is one affected sibling with an isolated cleft lip, the risk factor is 2.5%. If there is one affected sibling with a unilateral cleft lip and palate, the risk factor is 4.2%. If there are two affected siblings, the risk factor is 10%. If parental cleft and sibling clefting are present, the risk factor has been reported at 17%.

Answer 109

Correct answer is option A. One of the characteristic findings in the unilateral cleft deformity is a shortened columella. The C flap is designed to lengthen the columella. Other findings in the unilateral cleft nose include deviation and distortion of the septum (corrected with presurgical orthodontics), dislocation and slumping of the alar cartilage (corrected by dissecting the medial part of the cartilage and constructing a medial crus by suturing to the normal side), and flaring of the alar base (corrected with the alar cinch procedure).

Answer 110

The answer is B. 22q11.2 Deletion Syndrome. This syndrome is characterized by short, broad foreheads, hooded upper eyelids, protuberant ears, malar flatness, short philtrum and thin upper lip. The 22q deletion is detected by FISH array and can be a component of DiGeorge syndrome, velocardiofacial syndrome, and Opitz G syndrome. 22q11.2 is the most common microdeletion syndrome with a prevalence of 1in 4000 live births and 70% of patients with 22q deletion have palatal abnormalities. Rates of carotid artery variation have been reported as high as 100%. This must be taken in consideration when planning VPI surgery with either a sphincter pharyngoplasty or a posterior pharyngeal flap and many recommend pre-op imaging of the carotid arteries to determine the anomalous course of these vessels.

Answer 111

The correct answer is maxillary growth restriction, which will likely occur in most patients to some degree after palate repair. Fistula rates are generally in the 10- 20 percent range. Velopharyngeal insufficiency is in the range of 20 percent, and sleep apnea occurs in a small group of patients that is ill-defined.

Answer 112

Correct answer is option A. The presentation of cleft lip/palate concomitant with lower lip pitting is pathognomonic for van der Woude syndrome. Present in 80% of patients with van der Woude syndrome, lower lip pits are associated with accessory salivary glands. The Pierre Robin sequence consists of glossoptosis and micrognathia or retrognathia. Although cleft palate defects usually are associated with this sequence of findings, lower lip pitting is not. Stickler syndrome is related to cleft lip/palate, although it is not associated with lower lip pits on physical examination. Velocardiofacial syndrome is often associated with congenital heart disease and medial displacement of the carotid arteries but is unrelated to lower lip pits. Although it has been reported to cause syndromic cleft lip/palate, Waardenburg syndrome is associated with a white forelock of hair and sensorineural hearing loss but not lower lip pitting.

Answer 113

The correct answer is phenytoin use, which increase the likelihood of clefting by 10-fold. The other environmental causes increase the incidence of clefting, but to a lesser degree.

Answer 114

The correct response is Option A. The most likely cause of this child’s persistent hypernasality is hypotonia of the muscles involved in speech. Because this is neurogenic, it will be difficult to correct with surgery, so the best option becomes a palatal elevator used when talking to close off the nose posteriorly by pushing up the posterior soft palate. Posterior augmentation with fat grafting would decrease the size of the posterior gap but without adequate closure would still not correct the problem. The child has been in therapy and has failed to show improvement so additional therapy especially in light of the neurologic problem is unlikely to do anything but frustrate the child. A sphincter pharyngoplasty is not a viable option because a pharyngeal flap has been done and without adequate muscle function would fail.

Answer 115

The correct response is Option C. This patient has severe maxillary retrusion and mid face hypoplasia in the setting of significant velopharyngeal deficiency. These two issues are at odds with one another. Advancing her mid face with a standard Le Fort I will worsen her velopharyngeal dysfunction (VPD), while addressing her VPD with a pharyngeal flap will make advancing her maxilla challenging. Only anterior segmental maxillary distraction will maintain her current velopharyngeal anatomy but allow for improved mid face projection. A pharyngoplasty can be performed relatively easily at a later date or as a secondary procedure. Performing a mandibular setback is not appropriate because she has normal mandibular projection.

Answer 116

The correct response is Option D. Hyponasal speech describes the sound production when not enough air gets through the velopharyngeal sphincter, resulting in a muffled nasal voice, as if someone was holding his or her nose. This is in contrast to hypernasal speech, where the velopharyngeal sphincter is incompetent, resulting in leakage of excess air with an airy nasal voice. In this scenario, the most likely reason for hyponasal speech is an exuberant posterior pharyngeal flap that is blocking too much of the velopharyngeal complex. The two reasons to treat this are for speech issues that prevent normal interaction and obstructive sleep apnea (OSA). Given that the patient has no problems with people understanding her speech, the most reasonable next step among the choices is polysomnography to evaluate for OSA. None of the other choices are appropriate workups for patients with hyponasality. Some centers may proceed with video nasendoscopy to look at the anatomy, but this was not one of the answer choices.

Answer 117

Correct answer is option B. The nasal structures form during the sixth week of gestation. The nasal pits form the nasal placodes. These eventually deepen as a result of the formation of the medial and lateral nasal prominences. The external nasal structures appear as the medial nasal processes (MNP) enlarge and coalesce in the midline. Failure of this normal process can result in nasal deformities, such as those seen in the cleft nasal deformity. The frontonasal process (FNP) forms above the nasal placode, while the lateral nasal processes (LNP) remain outside and lateral to the nasal placode. Fusion of the medial nasal, lateral nasal, and maxillary prominences produces the continuity between the nose, upper lip, and palate. The MNP give rise to the nasal tip, columella, philtrum, and premaxilla. The nasal septum is a downgrowth from the merged medial nasal prominences. The forehead, the bridge of the nose, and the root of the nose come from the FNP. The nasal alae are derived from the LNP.

Answer 118

Correct answer is option C. Nasoalveolar molding is a form of presurgical orthopedics which corrects many deformities affecting cleft lip patients prior to corrective surgery. The alveolar segments are placed in anatomic position, the alar base is medialized, the lower lateral cartilages are placed in anatomic position and shape, the nasal lining is stretched and the columella is elongated. As Nasoalveolar molding is labor intensive, earlier time surgical repair is typically not possible.

Answer 119

The answer is B: All patients with suspected VPI should have a thorough intraoral exam. Unrepaired oronasal fistula after cleft palate repair is an important cause of VPI to rule out prior to pursuing other interventions on the palate or pharynx.

Answer 120

Correct answer is option C. The cartilage paradigm of primary rhinoplasty in the bilateral cleft lip patient supposes that anatomic repositioning of the lower lateral cartilages is the key to successful nasal correction. Previous approaches of primary bilateral cleft nasal reconstruction focused on using local skin flaps to elongate the columella, often resulting in anterior and superior displacement of the medial crura and a scarred columella. Ultimately, the skin paradigm approaches have had limited success because the anatomic derangement of the nasal framework was not addressed by these techniques. Nasoalveolar molding can elongate the columella but is not part of the classic cartilage paradigm techniques of primary bilateral cleft lip nasal reconstruction.

Answer 121

The Veau classification system includes four categories. Veau I clefts are limited to the soft palate, and include submucous clefts. Veau II clefts include the entire soft and hard palate (the entire secondary palate). Unilateral complete clefts are designated Veau III clefts; bilateral complete clefts of the lip and palate are designated Veau IV clefts.

Answer 122

The correct response is Option E. More than 90% of patients have chronic effusion of middle ear prior to repair. Therefore, all patients with cleft palate should be screened for hearing loss and for fluid in the middle ear due to eustachian tube dysfunction. There is emerging evidence and controversy with regard to treatment of the tensor veli tendon and the hamulus. However, this controversy and the tensor tenopexy, tensor transection, or fracture of the hamulus and their effects on the eustachian tube are beyond the scope of the question. What is clear is that the cleft patient population is at risk for complications related to fluid in the middle ear. Unfortunately, if this condition is unrecognized, elements of preventable hearing loss will occur.

Answer 123

The correct response is Option C. If there is inadequate rotation, the rotation-advancement repair can result in a short lip (white upper lip). A Tennison, or triangular, flap repair can result in an elongated lip and a visible scar across the lower philtrum. The advantage of the rotation-advancement flap is that the design places the scar along the philtral ridge. Straight-line repairs have been shown to result in blunting of Cupid’s bow. An overly wide philtrum is a surgical stigma of a repaired bilateral cleft lip.

Answer 124

The correct response is Option D. In this child who is scheduled to undergo sphincter pharyngoplasty, the palatopharyngeus muscle is incorporated within the flap. Two techniques are used primarily for reconstruction of the pharynx in patients with velopharyngeal insufficiency. The sphincter pharyngoplasty elevates and insets the posterior tonsillar pillars, which contain the palatopharyngeus muscles, into the posterior pharyngeal wall to create a sphincter. In contrast, during pharyngeal flap reconstruction, a flap of tissue from the posterior pharyngeal wall is elevated and attached to the palate. This procedure is typically reserved for correction of deficits of the central palate. The levator veli palatini muscle, which is found in the midline of the soft palate, is used only for pharyngeal flap reconstruction, not for sphincter pharyngoplasty. The buccinator muscle lies anterior to the pterygomandibular raphe, the palatoglossus muscles are contained within the anterior tonsillar pillars, and the tensor veli palatini is located anterior to the levator veli palatini. None of these muscles are used in sphincter pharyngoplasty.

Answer 125

Correct answer is option B. 22q11.2 Deletion Syndrome. This syndrome is characterized by short, broad foreheads, hooded upper eyelids, protuberant ears, malar flatness, short philtrum and thin upper lip. The 22q deletion is detected by FISH array and can be a component of DiGeorge syndrome, velocardiofacial syndrome, and Opitz G syndrome. 22q11.2 is the most common microdeletion syndrome with a prevalence of 1in 4000 live births and 70% of patients with 22q deletion have palatal abnormalities. Rates of carotid artery variation have been reported as high as 100%. This must be taken in consideration when planning VPI surgery with either a sphincter pharyngoplasty or a posterior pharyngeal flap and many recommend pre-op imaging of the carotid arteries to determine the anomalous course of these vessels.

Answer 126

Correct answer is option . There are several structures which contribute to the deviation of the nasal septum in the unilateral cleft lip patient. Converse and Hogan proposed that the displacement of the greater and lesser segments ultimately contributed to septal deviation through their “tilted tripod” theory. There are other structures which can directly contribute to septal deviation. The ala and anterior nasal spine are fix structures which are themselves manipulated by other forces at play in the unilateral cleft lip deformity. There is no information suggesting the nasalis significantly contributes to septal deviation. In the area of the anterior nasal spine, there is unilateral attachment of the orbicularis oris leading to deviation of the anterior caudal nasal septum and dislocation of this structure from the anterior nasal spine.

Answer 127

The correct response is Option A. By definition, a submucus cleft palate involves the anomalous insertion of the levator palatine muscles onto the posterior aspect of the hard palate. In normal anatomy, these muscles would be oriented in a transverse fashion. When contracting, the palate then is forced to elevate in a V €‘shaped fashion and shorten to some degree. Nevertheless, not all children will develop speech difficulty or velopharyngeal incompetence. The tensor palatine muscles and posterior tonsillar pillars (the palatopharyngeal muscles) are in relatively normal location. Their function is impacted by the incorrectly positioned levator. Children with a submucus cleft have normal palate length.

Answer 128

The correct response for Item 117 is Option A and for Item 118 is Option B. The A flap is marked on the medial lip element and is the rotation flap. This flap is designed as a gentle curve from the height of Cupid €™s bow on the cleft side of the medial cleft element to the junction of the philtrum and columella. This incision approaches the normal philtral collum as much as needed to derotate and level Cupid €™s bow. If more rotation is needed to level Cupid €™s bow, a back €‘cut is made, but the normal philtral collum is not violated. This incision is used in philtral subunit reconstruction and leveling Cupid €™s bow. The B flap is made on the non-cleft, lateral lip element and is the advancement flap. This flap is the €œwedge € that is placed in the defect created above the rotation flap as the medial lip element is derotated to level Cupid €™s bow. This flap is marked by first finding the point on the white roll that will correspond to the height of Cupid €™s bow on the medial cleft element. This point is found where the fullness of the lateral lip element begins to fade. A line is drawn from this point cephalad and is the same length as the normal philtral collum as well as the marked edge of the A (rotation) flap. The C flap or columellar flap is made from the tissue medial to the A €‘flap incision. This small flap is rotated into the columella, lengthening the cleft side of the columella. It is also sewn to the D flap (alar base flap). The D flap or alar base flap is cut along the alar €‘facial groove, and this incision corresponds to the cephalad portion of the edge of the B flap (advancement flap). This alar groove incision is made as small as possible and long enough to allow differential rotation of the D flap and the B flap. The L flap is the leading edge of the lateral lip that can be used to fill the nasal lining defect. The M flap is the medially based flap from the rotation.

Answer 129

The correct response is Option D. IRF6 mutations that result in loss-of-function have been reported in both syndromic Van der Woude syndrome as well as nonsyndromic orofacial clefting; Van der Woude syndrome can include popliteal pterygium. PIK3CA gain-of-function mutations have been reported with venous malformations and lymphatic malformations. Mutations in TCOF1 are associated with Treacher Collins syndrome; these patients may have cleft palate, but they do not have lip pits. Gain-of-function mutations in FGFR2 have been implicated in syndromic craniosynostosis (Apert syndrome, Crouzon syndrome, and others). Mutations in NF1 result in increased RAS/MAPK signaling and neurofibromatosis type 1. Reference(s) 1. Azoury SC, Reddy S, Shukla V, Deng CX. Fibroblast growth factor receptor 2 (FGFR21) mutation related to syndromic craniosynostosis. Int J Biol Sci. 2017 Nov 2;13(12):1479-1488. 2.Green AK, Goss JA. Vascular Anomalies: From a clinicohistologic to a genetic framework. Plast Reconstr Surg. 2018 May;141(5):709e-717e. 3. Hao S, Jin L, Wang H et al. Mutational analysis of TCOF1, GSC, and HOSA2 in patients with treacher collins syndrome. J Craniofac Surg. 2016 Sep;27(6):e583-e586. 4. Leslie EJ, Koboldt DC, Kang CJ et al. IRF6 mutation screening in non-syndromic orofacial clefting: analysis of 1521 families. Clin Genet. 2016 Jul;90(1):28-34. 5. Wu-Chou YH, Hung TC, Lin YT et al. Genetic diagnosis of neurofibromatosis type 1: targeted next-generation sequencing with multiple ligation-dependent probe amplification analysis. J Biomed Sci. 2018 Oct 5;25(1):72.

Answer 130

The correct response is Option C. 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.

Answer 131

The correct response is Option A. This neonate has the triad of symptoms associated with Pierre Robin sequence: retrogenia, glossoptosis, and respiratory distress. Approximately 50% of neonates with Pierre Robin sequence have a high-arched cleft in the midline of the soft palate; clefting of the hard palate also occurs in some patients. Glossoptosis is associated with airway obstruction, increased expenditure of energy, and impaired feeding resulting from decreased caloric intake. These feeding difficulties and failure to thrive can be fatal if not managed properly. The most appropriate initial management of airway obstruction is to place the neonate in the prone position, which relieves the glossoptosis and opens the airway. In some infants, this position must be maintained constantly, even during feeding, bathing, and diaper changing. Intubation may be necessary if prone positioning does not resolve the respiratory distress. Lip-tongue adhesion, tracheostomy, and mandibular distraction osteogenesis are secondary procedures that should only be considered if prone positioning is unsuccessful.

Answer 132

The correct response is Option E. Flat tympanograms indicate the eustachian tubes are not draining properly. The action of the tensor veli palatini is key in the equalization of pressure between the middle ear and nasopharynx. In a child with a cleft palate, the tensor is abnormally inserted into the levator palatini, and as a result, there is an increased incidence of ear eustachian tube dysfunction. The primary function of the levator veli palatini is velopharyngeal closure by velar elevation and retrodisplacement. The palatopharyngeus is responsible for controlling the velopharyngeal sphincter by controlling velar size, shape, and position. The palatoglossus works to modulate speech by velar depression and tongue elevation. The superior constrictor is important for pharyngeal closure during swallowing and speech.

Answer 133

The correct response is Option C. The presentation of cleft lip/palate concomitant with lower lip pitting is pathognomonic for van der Woude syndrome. Present in 80% of patients with van der Woude syndrome, lower lip pits are associated with accessory salivary glands. The Pierre Robin sequence consists of glossoptosis and micrognathia or retrognathia. Although cleft palate defects usually are associated with this sequence of findings, lower lip pitting is not. Stickler syndrome is related to cleft lip/palate, although it is not associated with lower lip pits on physical examination. Velocardiofacial syndrome is often associated with congenital heart disease and medial displacement of the carotid arteries but is unrelated to lower lip pits. Although it has been reported to cause syndromic cleft lip/palate, Waardenburg syndrome is associated with a white forelock of hair and sensorineural hearing loss but not lower lip pitting.

Answer 134

The correct response is Option B. The nasal structures form during the sixth week of gestation. The nasal pits form the nasal placodes. These eventually deepen as a result of the formation of the medial and lateral nasal prominences. The external nasal structures appear as the medial nasal processes (MNP) enlarge and coalesce in the midline. Failure of this normal process can result in nasal deformities such as those seen in the cleft nasal deformity. The frontonasal process (FNP) forms above the nasal placode, while the lateral nasal processes (LNP) remain outside and lateral to the nasal placode. Fusion of the medial nasal, lateral nasal, and maxillary prominences produces the continuity between the nose, upper lip, and palate. The MNP give rise to the nasal tip, columella, philtrum, and premaxilla. The nasal septum is a downgrowth from the merged medial nasal prominences. The forehead, the bridge of the nose, and the root of the nose come from the FNP. The nasal alae are derived from the LNP.

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