Cleft Lip and Palate 01-22, 24 Flashcards

Question

An 8-year-old girl with a history of complete cleft lip and palate repaired as an infant presents for management of her alveolar cleft. During the preoperative visit, options for management of the bony defect are discussed. Which of the following is the primary disadvantage of secondary bone grafting using iliac crest cancellous bone rather than using demineralized bone matrix? A) Cost B) Donor site morbidity C) Failure rate D) Impact on mid face growth E) Surgical procedure duration

Answer 1

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 2

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 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 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 5

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 6

The correct response is Option E. Median cleft lip results from failed fusion of the medial nasal prominence during embryologic development. A unilateral cleft lip results from failed fusion of the medial nasal prominence and the maxillary prominence. An oblique facial cleft results from failure of fusion of the lateral nasal prominence and the maxillary prominence. A lateral oral commissure cleft is produced by the failed fusion of the mandibular and maxillary prominences. A cleft of the primary palate is produced by failure of fusion of the medial and lateral palatine processes.

Answer 7

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 8

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 9

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 10

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 11

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 12

The correct response is Option E. The primary goal of cleft palate repair is normal speech. Velopharyngeal competence, the ability to completely close the velopharyngeal sphincter, is required for the normal production of all but the nasal consonants (in English: /m/, /n/, and /ng/). Velopharyngeal insufficiency is defined as the inability to completely close the velopharyngeal sphincter. The primary effects of velopharyngeal insufficiency are nasal air escape and hypernasality. Video fluoroscopy and nasal endoscopy can detect the sagittal deficiency closure pattern occurring in patients with velopharyngeal insufficiency after cleft palate surgery. Speech articulation errors (i.e., distortions, substitutions, and omissions) are secondary effects of velopharyngeal insufficiency. The result is decreased intelligibility of speech. The velopharyngeal port is bordered anteriorly by the velum, bilaterally by the lateral pharyngeal walls, and posteriorly by the posterior pharyngeal wall. Velopharyngeal insufficiency can be diagnosed by both subjective and objective means. The speech evaluation by a trained pathologist with perceptual evaluation of speech by an experienced speech language pathologist is the standard. Multiview videofluoroscopy and nasendoscopy both provide visual information (i.e., closure pattern and closure rating) that is valuable for surgical planning. However, the need for radiation has caused most cleft centers to migrate to direct nasal endoscopy. The MRI is emerging technology but would not be the first line choice in the diagnostic workup. Rhinometry is an objective measurement of nasal air emission during speech and is not routinely used for surgical decision-making. Overall, the exam under anesthesia would likely not be required but instead a complete exam and nasal endoscopy would likely be able to be performed comfortably in the office. Experience with nasal endoscopy has grown in most comprehensive cleft centers and has become an invaluable tool for surgical planning.

Answer 13

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 14

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 15

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 16

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 17

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 18

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 19

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 20

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 21

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 22

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 23

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 24

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 25

The correct response is Option E. The primary goal of cleft palate repair is normal speech and swallowing. Velopharyngeal competence, the ability to completely close the velopharyngeal sphincter, is required for the normal production of all but the nasal consonants (in English: /m/, /n/, and /ng/). Velopharyngeal insufficiency (VPI) is defined as the inability to completely close the velopharyngeal sphincter. The primary effects of VPI are nasal air escape and hypernasality. Video fluoroscopy and nasopharyngoscopy can detect the sagittal deficiency closure pattern occurring in patients with VPI after cleft palate surgery. Speech articulation errors (i.e., distortions, substitutions, and omissions) are secondary effects of VPI. The result is decreased intelligibility of speech. The velopharyngeal port is bordered anteriorly by the velum, bilaterally by the lateral pharyngeal walls, and posteriorly by the posterior pharyngeal wall. VPI can be diagnosed by both subjective and objective means. Perceptual evaluation of speech by an experienced speech language pathologist is the standard. Multiview video fluoroscopy and nasopharyngoscopy both provide visual information (i.e., closure pattern and closure rating) that is valuable for surgical planning. However, the need to avoid radiation if centers are migrating away from fluoroscopy has caused most cleft centers to migrate to direct nasopharyngoscopy. MRI is emerging as a technology for evaluating VPI, but it would not be the first choice for diagnostic workup. Overall, examination during anesthesia in the operating room would likely not be required, but instead a complete examination and nasopharyngoscopy would likely be performed comfortably in the office. Experience with nasopharyngoscopy has grown in most comprehensive cleft centers and has become an invaluable tool for surgical planning. Lateral cephalogram does not provide a dynamic evaluation of the pharynx.

Answer 26

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 27

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 28

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

Answer 29

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 30

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 31

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 32

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 33

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 34

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 35

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 36

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 37

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 38

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 39

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 40

The correct response is Option D. Serial cephalometric x-ray study is a reliable method to determine completion of maxillofacial growth in adolescents. Chronologic age cannot also be used in the examination of adolescent growth because there is wide variation among individuals in the timing of the pubertal growth spurt. Minimal or no change in the velocity of maxillofacial growth at this time is a good indicator of skeletal maturity. Ossification of bones of the wrist and hand is normally the standard for assessing skeletal development. However, its validity in the examination of craniofacial growth has recently come into question. Dental development indicators are not reliable predictors of an individual’s stage of skeletal development.

Answer 41

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.

Answer 42

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 43

The correct response is Option D. Serial cephalometric x-ray study is a reliable method to determine completion of maxillofacial growth in adolescents. Chronologic age cannot also be used in the examination of adolescent growth because there is wide variation among individuals in the timing of the pubertal growth spurt. Minimal or no change in the velocity of maxillofacial growth at this time is a good indicator of skeletal maturity. Ossification of bones of the wrist and hand is normally the standard for assessing skeletal development. However, its validity in the examination of craniofacial growth has recently come into question. Dental development indicators are not reliable predictors of an individual’s stage of skeletal development.

Answer 44

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 45

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 46

The correct response is Option C. Routinely, postoperative velopharyngeal insufficiency is treated with either a posterior pharyngeal flap or a sphincter pharyngoplasty. A sphincter pharyngoplasty is performed by taking the posterior tonsillar pillar, containing the palatopharyngeus muscle, and elevating it inferiorly to superiorly. The elevated posterior tonsillar pillar, pedicled superiorly, is rotated 90 degrees medially, positioned side by side, and sewn into an incision made horizontally on the posterior pharyngeal wall at the level of the adenoid pad. The levator veli palatini muscle, in the normal palate, rests horizontally (coronally) within the middle third of the velum (soft palate) and functions as the motor of the velum. This muscle pulls the soft palate posteriorly and superiorly, allowing for apposition of the free edge of the velum against the posterior pharyngeal wall (velopharyngeal competence). The musculi uvulae arise as paired slips from the palatine aponeurosis and course sagittally in the velar midline, ending in the uvula. The musculi uvulae act as a flexible beam, providing a stiffness-modifying mechanism for the velum. The superior pharyngeal constrictor arises from the hamulus and course sagittally along the lateral pharyngeal walls to decussate in the midline of the posterior pharyngeal wall. The superior pharyngeal constrictor is the muscle from the posterior pharyngeal wall that is utilized in the posterior pharyngeal flap. The tensor veli palatini muscle arises from the skull base and courses inferiorly and medially around the hook of the hamulus and into the palate as the tensor aponeurosis. It joins the velum (soft palate) to the hard palate.

Answer 47

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 48

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 49

The correct response is Option A. In cleft palate reconstruction, repair of the levator veli palatini can improve eustachian tube function. Individuals with unrepaired cleft palate suffer chronic otitis media, which can lead to permanent hearing loss. This is thought to be caused by the dysfunction of the eustachian tube. There are several paratubal muscles that are responsible for the opening and closing of the eustachian tube: the tensor veli palatini, the levator veli palatini, and the salpingopharyngeus. Contraction of the levator veli palatini with superior and posterior displacement of the levator sling opens the eustachian tube. In an unrepaired cleft palate, the ability of the levator veli palatini to open the tube is lost because of its abnormal insertion on the posterior hard palate. Repositioning of this muscle during cleft palate repair restores the levator sling, allowing dilation of the eustachian tube. The tensor veli palatini opens the eustachian tube and may have €œpumping action € that milks the contents of the tube. This muscle €™s function is likely unaffected by clefting. However, its ability to open the eustachian tube may be iatrogenically reduced by complete hamular fracture or division of its tendon during cleft palate repair. The salpingopharyngeus also opens and closes the eustachian tube. However, because of its small size, it is the least important of the paratubal muscles and has minimal functional significance. The palatopharyngeus muscle optimizes velopharyngeal closure. Along with the superior constrictor, it causes medial displacement of the lateral pharyngeal wall. The superior constrictor is the main component of the Passavant ridge and functions to bring about medial displacement of the lateral pharyngeal wall through a sphincteric mechanism.

Answer 50

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 51

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% (as in the scenario described). Normal parents, two children with a CL/P: the risk for the next child is 9%. Affected parent with a CL/P, no affected children: the risk for the next child is 4%. Affected parent with a CL/P, one child with a CL/P: the risk for the next child is 17%. Affected parent with a CL/P and lip pits who has van der Woude syndrome: the risk for an affected child would be 50%, as this is an autosomal dominant condition.

Answer 52

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 53

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 54

The correct response is Option C. The patient shown below has maxillary retrusion and mandibular prognathism, which is most appropriately treated with a maxillary (Le Fort I) advancement and a sagittal split osteotomy (mandibular setback). The Angle class III malocclusion is demonstrated in photograph, and cephalometric analysis shows overprojection of the mandible and underprojection of the maxilla. Le Fort I advancement also could be attempted but would be difficult for such a discrepancy in occlusion; bimaxillary prognathism would result.

Answer 55

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 56

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 57

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 58

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 59

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 60

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 61

The correct response is Option 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 velocardiofacial 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 62

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 63

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 64

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 65

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 66

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

Answer 67

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 68

The correct response is Option C. The levator veli palatini originates from the petrous portion of the temporal bone and the eustachian tube. It travels alongside the eustachian tube to enter the soft palate. This muscle elevates the velum toward the posterior pharyngeal wall to close the velopharyngeal mechanism and pull the eustachian tube open. The palatoglossus muscle arises from the lateral margin of the tongue. It travels in the anterior tonsillar pillar to enter into the soft palate and functions to pull the soft palate downward. The posterior tonsillar pillar is created by the palatopharyngeus. The palatopharyngeus muscle arises from fibers that commingle with the superior pharyngeal constrictor. It enters the soft palate, where its fibers intermingle with those of the levator. The palatopharyngeus functions to depress the soft palate and provide inward motion of the lateral pharyngeal wall. In a sphincter pharyngoplasty, superiorly based flaps are elevated from the posterior tonsillar pillar, sutured together, and inset into the posterior pharyngeal wall. These flaps contain the palatopharyngeus muscle. The superior pharyngeal constrictor originates from the posterior pharyngeal raphe and courses downward and forward to insert into the medial pterygoid plate, the pterygomandibular raphe, and the posterior part of the hyoid. The stylopharyngeal muscle arises from the styloid process. It inserts between the fibers of the superior and middle pharyngeal muscles in the pharyngeal wall. These muscles play a role in swallowing.

Answer 69

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 70

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 71

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 72

The correct response is Option D. The tensor veli palatini muscle descends from the base of the skull at a point adjacent to the eustachian tube, courses around the hamulus of the pterygoid, and then forms a broad aponeurosis with the contralateral muscle within the anterior soft palate. The levator veli palatini passes posterior to the pterygoid hamulus and creates a muscular sling that is critical to palatal function. The palatoglossus and palatopharyngeus muscles are components of the palate but do not pass around the hamulus. The musculus uvula is confined within the soft palate and acts to alter the shape of the uvula.

Answer 73

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 74

The correct response is Option D. The nasal structures form during the sixth week of gestation as the medial nasal processes enlarge and coalesce in the midline. Any abnormalities that occur during formation of the nasal structures may result in deformity, such as the cleft nasal deformity. The nasal alae arise from the lateral nasal processes. The medial nasal processes give rise to the columella, nasal tip, philtrum, and premaxilla. The bridge and root of the nose arise from the frontonasal processes.

Answer 75

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 76

The correct response is Option B. During embryologic development, the nasal structures form during the sixth week of gestation as the frontonasal and medial nasal processes enlarge and coalesce in the midline. Any abnormalities occurring during this gestational stage are likely to lead to the development of a cleft nasal deformity or other nasal deformities.

Answer 77

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, while cleft lip results from fusion of the medial nasal prominence and maxillary prominence. 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 78

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 79

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 80

The correct response is Option B. In patients who have unilateral cleft lip and associated nasal deformities, the premaxilla is rotated and projected outward, and the lateral maxillary element is collapsed and retropositioned. The inferior edge of the septum lies outside of the vomer groove, while the nasal spine is located in the floor of the normal nostril. The affected columella is 25% to 50% shorter than the unaffected side. The lower lateral cartilage is attenuated, and the nasal dome lies separate, below the opposite cartilage. The alar base is flared and rotated outward, and the vestibular lining is deficient on the side of the cleft. With regard to the lip deformities, the philtrum termination of the orbicularis oris muscle in the lateral lip is shortened at the margin of the cleft; at this point, the muscle inserts into the alar wing. The muscles between the philtral midline and the cleft are hypoplastic. Two thirds of the cupid's bow is preserved, as well as one philtral column and a dimple hollow.

Answer 81

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 82

The correct response is Option D. Patients with complete cleft lip that includes the alveolus have a higher risk for lateral incisor tooth agenesis. Both congenital absence and orthodontic canine substitution are independently associated with an increased risk for maxillary hypoplasia, Angle class III malocclusion, and the subsequent need for orthognathic surgery.

Answer 83

The correct response is Option B. Le Fort I distraction is a powerful tool to address Angle class III malocclusion in patients with cleft lip and palate. Numerous studies have shown that distraction yields improved speech outcomes when controlling for the degree of advancement, while also decreasing the rate of relapse, as compared with conventional distraction. However, final occlusion with internal maxillary distraction is less predictable than external distraction or conventional maxillary advancement surgery. Complication rates and psychosocial outcomes have not been shown to differ between these techniques.

Answer 84

The correct response is Option D. Patients with submucous cleft palate may have delayed presentation to cleft centers due to undiagnosed disease. They might be considered developmentally behind and have behavioral issues given their inability to express themselves and be understood. It is not uncommon to miss their abnormal soft palate anatomy, which can be subtly V-shaped and have the midline bluish line (pellucid zone). Patients with arterial venous malformation of this area do not necessarily produce hypernasal speech. Chronic tonsillitis can be associated with hyponasality, enlarged tonsils, and bad breath. Palatal neoplasm is usually slow-growing and not associated with a pellucid zone or long-term speech issues. Torus palatini does not produce hypernasal speech.

Answer 85

The correct response is Option D. Dental anomalies are frequent in patients with cleft lip and palate. The maxillary lateral incisor is the most frequently congenitally absent secondary tooth, missing in around 60% of patients. The other teeth listed are missing at a much lower incidence.

Answer 86

The correct response is Option A. Secondary bone grafting is widely accepted in cleft care; however, there is limited consensus on the technique, timing, and perioperative management of the cases. In general, the age of the patient at secondary bone grafting is typically at the time of mixed dentition and ranges from 8 to 12 years of age. Several studies have demonstrated that later age at time of bone grafting is associated with higher rates of graft failure, especially when the canine has already erupted. Lack of orthodontic preparation and postoperative antibiotic regimen were not predictors of graft failure. Time to postoperative oral intake was also not predictive of graft failure.

Answer 87

The correct response is Option C. The marginal mandibular nerve is a branch of the facial nerve that innervates the depressor labii inferioris muscle, depressor anguli oris muscle, and mentalis muscle. A patient with injury to the marginal mandibular branch of the facial nerve presents with a deformity opening the mouth, smiling or grimacing. Injury to the marginal mandibular nerve causes paralysis of muscles of the lower lip of that side. The other options presented would not produce weakness of the depressor labii inferioris muscle.

Answer 88

The correct response is Option B. In a traditional Furlow palatoplasty, the flap labeled "A" includes the levator veli palatini muscle as a musculomucosal flap, and it is transposed posteriorly in the final Z-plasty.

Answer 89

The correct response is Option C. The timing of cleft palate repair affects speech outcome. This child is already older than optimal timing, so repair of the cleft palate to allow for generation of normal speech is the correct next step. There is no reason for speech therapy or video fluoroscopy since the palate is not repaired yet. Primary nasal repair is done at the time of original lip repair, and revisions depend on the degree of deformity, but the definitive rhinoplasty is deferred until skeletal maturity. A complete lip revision for this patient's hypertrophic scar can improve the appearance of this child but should wait until after palate repair.

Answer 90

The correct response is Option D. In patients with 22q11.2 deletion syndrome (DiGeorge syndrome) with velopharyngeal insufficiency that persists after palatoplasty, posterior pharyngeal flap surgery is an option, but it must be performed carefully because these patients tend to have medialized internal carotid arteries, and these can be injured during dissection of the posterior pharyngeal flap. The other options are incorrect, since these are not features of the anatomy in patients who have 22q11.2 deletions.

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Cleft Lip and Palate 01-22, 24 Flashcards

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