Cleft Lip and Palate Flashcards
A 7-year-old girl with a history of cleft lip and palate is brought to the office because of hyponasal speech, which is confirmed by the speech language pathologist. The patient and her family report that people have no trouble understanding her speech. Medical history includes primary cheiloplasty, primary rhinoplasty, primary palatoplasty, and posterior pharyngeal flap. Which of the following is the most appropriate next step in management?
A) CT scan of the face B) Genetics referral C) MRI of the palate D) Polysomnography E) Swallow study
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.
2018
A 10-year-old boy with a history of obstructive sleep apnea and prior straight-line cleft palate repair is brought for evaluation of stigmatizing, hypernasal speech. There is no oronasal fistula present, and the velum appears to vault, but the palate elevates well with phonation. Which of the following is the best option for improvement of speech in this patient?
A) Continued speech therapy B) Furlow palatoplasty C) Posterior pharyngeal flap D) Tonsillectomy E) Use of a palatal lift device
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.
2018
Which of the following layers is included with a posterior pharyngeal flap?
A) Investing layer of the deep cervical fascia
B) Palatopharyngeus muscle
C) Prevertebral fascia
D) Superior pharyngeal constrictor muscle
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 caudal 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.
2018
For patients with velopharyngeal incompetence, which of the following muscles is used to perform sphincter pharyngoplasty?
A) Levator veli palatini B) Musculus uvulae C) Palatoglossus D) Palatopharyngeus E) Tensor veli palatini
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.
2018
A 2-year-old has a midline cleft of the upper lip. This cleft results from failure of fusion of which of the following embryologic structures?
A) Lateral nasal prominence and maxillary prominence
B) Maxillary and mandibular prominences
C) Medial and lateral palatine processes
D) Medial nasal prominence and maxillary prominence
E) Medial nasal prominences only
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.
2018
A 3-year-old girl with a cleft lip and palate is brought to the clinic because her parents are concerned that her teeth are not coming in properly. Given her diagnosis, which of the following is the most likely finding?
A) Dysplastic teeth B) Ectopic teeth C) Supernumerary teeth D) Tooth agenesis E) Tooth translocation
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.
2018
A 17-year-old girl with a left unilateral cleft lip and palate comes to the office because she is dissatisfied with her occlusion and facial appearance. Physical examination shows midface hypoplasia and class III malocclusion. Intraoral examination demonstrates 6 mm of negative overjet and a missing left lateral maxillary incisor with retained space. Occlusal x-ray studies demonstrate 5 mm of alveolar bone height at the cleft margin. Which of the following is the best treatment option for this patient?
A) Alveolar transport distraction
B) Observation until skeletal maturity
C) Prosthodontic rehabilitation with a fixed bridge
D) Sagittal split of the mandible with osteointegrated implants
E) Two-piece Le Fort I osteotomy with closure of the alveolar gap
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.
2018
Which of the following represents the percentage of patients with isolated cleft palate who are most likely to have middle ear effusion?
A) 10% B) 30% C) 50% D) 70% E) 90%
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.
2018
A 33-year-old woman is evaluated for hypernasal speech and nasal escape with phonation. She underwent removal of the tonsils and adenoids 10 weeks ago. Transillumination of the palate is shown. Which of the following is the most appropriate approach to restore normal speech in this patient?
A) Fat grafting to tonsil pillars B) Orticochea pharyngoplasty C) Palatoplasty D) Tensor tenopexy E) Observation and speech pathology
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.
2018
A 15-year-old girl presents with a cleft lip nasal deformity. In infancy, she was treated in a cleft center with appropriate management and surgical correction of unilateral cleft lip and palate. Which of the following current findings is most consistent with the deformity?
A) Cleft-side alar base is more anterior than the noncleft-side alar base
B) Cleft-side piriform margin is farther anterior than the noncleft-side piriform margin
C) Columellar base is deviated to the cleft side
D) Medial to lateral crural angle is more acute on the cleft side
E) Noncleft-side alar base is farther from the midline than the cleft-side alar base
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
2018
A 7-year-old boy with a history of bilateral cleft lip and palate has undergone multiple procedures including lip and nose repair, palate repair, and closure of an oronasal fistula. His parents note that during the past 6 months he has had nighttime snoring, frequent pauses in his breathing, and daytime somnolence. Physical examination shows mixed dentition with severe midface hypoplasia and Angle class III malocclusion with 12 mm of negative overjet. A polysomnogram demonstrates an obstructive apnea-hypopnea index (AHI) of 12.5 per hour. The patient is otherwise healthy. Which of the following is the best treatment option for this patient?
A) Continuous positive airway pressure (CPAP)
B) Le Fort I advancement and bilateral sagittal split setback
C) Le Fort I osteotomy and application of bilateral internal maxillary distractors
D) Le Fort III osteotomy and application of external halo distractor
E) Tracheostomy
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.
2017
A 7-year-old girl with a history of cleft palate repair is brought to the office for evaluation of velopharyngeal insufficiency. After speech evaluation, which of the following is the most appropriate initial tool for diagnosis and management of this patient’s condition?
A) Cine MRI B) CT scan C) Physical examination under anesthesia D) Rhinometry E) Video nasal endoscopy
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.
2017
Which of the following best describes the principal goal of using the nasoalveolar molding (NAM) appliance?
A) Decrease of the financial and care burden on the family
B) Improvement in feeding
C) Improvement in midface growth in the sagittal and vertical planes
D) Palatal expansion
E) Repositioning and approximation of the alveolar segments and nasal cartilage
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.
2017
A 2-month-old male infant is evaluated for cleft lip and lower lip pits. Medical history shows his father also had a cleft lip at birth. Which of the following is the most likely genetic defect in this patient?
A) FGFR1 B) IRF6 C) PTCH1 D) Trisomy 13 E) 22q11.2
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.
2016
A 3-year-old boy is evaluated for unilateral cleft lip and palate. Tympanogram shows bilateral noncompliance. Which of the following muscles is most likely responsible for this finding?
A) Levator veli palatini B) Palatoglossus C) Palatopharyngeus D) Superior constrictor E) Tensor veli palatini
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.
2016
A 10-year-old boy who previously underwent palatoplasty for cleft palate is evaluated because of velopharyngeal insufficiency associated with minimal palatal elevation and coronal closure pattern. The most appropriate procedure to treat this patient targets which of the following muscles?
A) Hyoglossus B) Levator veli palatini C) Palatopharyngeus D) Pharyngeal constrictor E) Stylohyoid
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.
2016
A 17-year-old boy is evaluated for Le Fort I advancement and lengthening procedure. Medical history shows repair of bilateral cleft lip and palate in infancy. Postoperatively, which of the following facial changes is most likely in this patient?
A) Decreased nasolabial angle B) Decreased tooth show in repose C) Increased alar base D) Increased depth of nasolabial folds E) Increased length of upper lip
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.
2016
A 30-month-old female infant is evaluated for unilateral complete cleft lip and palate. She was adopted from China, where her lip was previously repaired. Her parents are concerned about her palate, which has not been repaired, and the appearance of her lip and nose. Which of the following is the most appropriate next step in management?
A) Repair the palate and perform a rhinoplasty
B) Repair the palate first and revise the lip at a later time
C) Revise the lip and repair the palate simultaneously with a gingivoperiosteoplasty
D) Revise the lip now and repair the palate at a later time
E) Wait one year until more English vocabulary is acquired, and then undergo speech evaluation before palate repair
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.
2016
Which of the following is the best dental reference to use for determining the timing for secondary alveolar bone grafting in a patient with a unilateral complete cleft lip/palate?
A) Complete eruption of the central incisor next to the cleft
B) Complete eruption of the permanent canine into the cleft
C) Crowning of the permanent canine
D) Loss of the primary canine adjacent to the cleft
E) Loss of the primary ipsilateral central incisor
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.
2016
An 8-year-old boy with a history of submucous cleft palate presents with persistent velopharyngeal insufficiency. Surgical history includes a Furlow palatoplasty 3 years ago, with subsequent revision to a superiorly based pharyngeal flap for persistent hypernasality 1 year ago. He had no improvement after the second surgery and speech therapy. Physical examination shows an intact, high and wide pharyngeal flap. Nasoendoscopy shows patent lateral oronasal ports and poor palatal and lateral pharyngeal wall motion. Which of the following treatments is most appropriate in this patient?
A) Fabrication of a custom palatal elevator
B) Pharyngeal flap port revision
C) Posterior wall augmentation with fat grafting
D) Speech therapy with retraining
E) Sphincter pharyngoplasty
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.
2016
Which of the following stigmata is most common after a rotation-advancement repair of a unilateral cleft lip?
A) Blunting of Cupid's bow B) Elongated lip C) Short lip D) Triangular scar across the philtrum E) Widened philtrum
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.
2016
A 6-year-old boy with a repaired unilateral complete cleft lip and palate presents for an annual cleft team clinic visit. Initiation of palatal expansion is discussed with the child’s parents. Timing for initiation of palatal expansion should be based upon which of the following?
A) Alveolar cleft width B) Canine eruption C) Occlusal status D) Patient age E) Severity of alveolar collapse
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.
2015
A 7-year-old girl with a history of repair of cleft palate is evaluated because of possible velopharyngeal insufficiency. In addition to evaluation of the patient’s speech by trained speech pathologists, which of the following is the most appropriate diagnostic tool?
A) Cine MRI B) CT scan C) Examination during anesthesia D) Lateral cephalogram E) Nasopharyngoscopy
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.
2015
A 4-year-old girl with velocardiofacial syndrome is evaluated for hypernasal speech. She underwent protracted speech therapy after repair of an isolated cleft of the soft palate at age 9 months. Physical examination shows a well-healed palate and trace elevation of the soft palate with phonation. Videofluoroscopy shows poor motion of the velum; adequate lateral pharyngeal wall motion is noted. To improve this patient’s speech, which of the following is the most appropriate management of her velopharyngeal insufficiency?
A) Fat augmentation of the posterior pharyngeal wall
B) Furlow palatoplasty
C) Intravelar veloplasty
D) Reconstruction with a superiorly based pharyngeal flap
E) Sphincter pharyngoplasty
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.
2015
Velar competence after treatment of velopharyngeal insufficiency with Furlow double-opposing Z-plasty is most strongly correlated with which of the following?
A) Age at the time of procedure B) Compliance with speech therapy C) Patient gender D) Preoperative closure gap E) Type of cleft
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.
2014
A 2-month-old male infant is evaluated for complete unilateral cleft of the lip and palate. Development of which of the following tooth buds is most likely to be impaired in this patient?
A) Central incisor B) First molar C) Lateral incisor D) Premolar E) Third molar
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.
2014
Failure of fusion of which of the following results in the formation of a cleft of the lip?
A) Frontonasal and maxillary prominences during the first 4 to 5 weeks of gestation
B) Frontonasal and maxillary prominences during the first 9 to 11 weeks of gestation
C) Lateral nasal and maxillary prominences during the first 2 to 4 weeks of gestation
D) Medial nasal and maxillary prominences during the first 9 to 11 weeks of gestation
E) Medial nasal and maxillary prominences during the first 6 to 8 weeks of gestation
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.
2014
A 4-year-old girl is referred by her speech therapist because she has persistent nasal air escape with phonation. She underwent isolated repair of the cleft palate in infancy. Physical examination shows a long, mobile palate. No fistula is noted. Nasendoscopy shows good coronal closure with poor lateral pharyngeal wall movement. Which of the following is the most appropriate management?
A) Augmentation of Passavant ridge B) Continued speech therapy C) Implantation of a palatal lift prosthesis D) Posterior pharyngeal flap E) Sphincter pharyngoplasty
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).
2014
A 2-year-old boy who was recently adopted is brought to the office for evaluation and treatment of cleft of the lip and palate. Physical examination shows involvement of the lip, alveolus, and entire palate. A photograph is shown. He is otherwise healthy with no other congenital anomalies. Which of the following is this patient’s risk of having a child with cleft of the lip?
A) 1% B) 5% C) 10% D) 15% E) 50%
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).
2014
A 6-year-old boy who underwent repair of cleft palate 5 years ago is brought to the clinic for follow-up. The patient is hypernasal. Nasoendoscopy shows good lateral pharyngeal wall motion and a sagittal closure pattern. Cephalometric analysis shows a posterior gap of 10 mm. Which of the following is the most appropriate management?
A) Intravelar veloplasty B) Obturator C) Palatal lift D) Pharyngeal flap E) Sphincter pharyngoplasty
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.
2013
A 6-year-old boy with velopharyngeal insufficiency is brought to the office for sphincter pharyngoplasty. History includes repair of a cleft palate as an infant. Innervation of the muscle used to create the sphincter arises from which of the following?
A) Cranial part of the accessory (XI) nerve
B) Glossopharyngeal (IX) nerve
C) Greater and lesser palatine branches of the pterygopalatine ganglion
D) Hypoglossal (XII) nerve
E) Medial pterygoid nerve
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.
2013
A 4-year-old girl who has velocardiofacial syndrome is evaluated because of severe velopharyngeal insufficiency. Reconstruction using a superiorly based pharyngeal flap is planned. Which of the following is the most important factor in determining the width of the flap?
A) Ability to close the pharyngeal donor defect
B) Lateral pharyngeal wall motion
C) Length of the soft palate
D) Palatal movement (coronal closure)
E) Position of the carotid arteries in the posterior pharynx
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.
2012
A 2-year-old girl is evaluated because of bifid uvula, notching of the posterior hard palate, and midline thinning of the posterior palate. She feeds well and has normal hearing. Which of the following is the most appropriate management at this time?
A) Furlow double-opposing Z-plasty B) Nasendoscopy C) Speech evaluation D) Straight-line veloplasty E) Veau-Wardill-Kilner push-back palatoplasty
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.
2012
Which of the following skeletal anomalies of the anterior nasal spine are most commonly found in unilateral cleft lip and palate patients?
A) Deviation to the cleft side and decreased projection of the pyriform aperture and dentoalveolar arch on the cleft side
B) Deviation to the cleft side and increased projection of the pyriform aperture and dentoalveolar arch on the cleft side
C) Deviation to the noncleft side and decreased projection of the pyriform aperture and dentoalveolar arch on the cleft side
D) Deviation to the noncleft side and increased projection of the pyriform aperture and dentoalveolar arch on the cleft side
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.
2012
The L flap used in the rotation-advancement technique of cleft lip repair is implemented to accomplish which of the following?
A) Add bulk to the vermilion
B) Allow for sagittal expansion of the nasal sidewall
C) Lengthen the columella
D) Provide lining in the gingivobuccal sulcus
E) Restore symmetry along the philtral column
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.
2012
A 16-year-old boy is referred for consultation regarding treatment of maxillary retrusion and an Angle class III malocclusion. History includes repair of a complete unilateral cleft lip and palate in infancy. Maxillary advancement is planned after completion of facial growth. Completion of facial growth is best determined by which of the following?
A) Chronologic age of the patient
B) Complete eruption of the second molars
C) Hand-wrist x-ray study
D) Serial cephalometric x-ray study
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.
2012
Which of the following structures arises from the lateral nasal processes during embryologic development?
A) Columella B) Nasal ala C) Nasal septum D) Nasal tip E) Premaxilla
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.
2011
A newborn is evaluated because of a cleft of the soft and hard palates that extends to the incisive foramen. Which of the following is the most appropriate Veau classification of this cleft?
A) I
B) II
C) III
D) IV
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.
2011
The 3-month-old infant shown has a bilateral cleft lip and palate. Which of the following is a goal of the nasoalveolar molding seen in the photograph?
A) Expansion of the alveolar cleft
B) Expansion of the soft tissue of the lip
C) Lengthening of the columella
D) Lengthening of the septum
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.
2011
A 21-year-old man comes to the office for evaluation of a secondary palate fistula following palatoplasty 20 years ago. History includes three unsuccessful attempts at fistula repair using local tissue. Repair using a tongue flap to provide soft tissue to the palate is planned. Blood supply runs primarily in which of the following regions of the tongue?
A) Ventral third
B) Middle third
C) Dorsal third
D) Dispersed throughout
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.
2011
A 16-year-old boy is referred for consultation regarding treatment of maxillary retrusion and an Angle class III malocclusion. History includes repair of a complete unilateral cleft lip and palate in infancy. Maxillary advancement is planned after completion of facial growth. Completion of facial growth is best determined by which of the following?
A) Chronologic age of the patient
B) Complete eruption of the second molars
C) Hand-wrist x-ray study
D) Serial cephalometric x-ray study
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.
2011
A 16-month-old boy who underwent correction of a complete unilateral cleft lip 2 months ago is brought to the office because his mother is concerned about the appearance of the scar on his lip. Physical examination shows a corrected upper lip with a good pout and contour. The scar is flat, slightly widened, and moderately erythematous. Which of the following is the most appropriate management at this time?
A ) Continued optimal scar management
B ) Immediate revision of the scar
C ) Laser resurfacing of the scar
D ) Revision of the scar in 4 months
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.
2010
A 25-year-old woman who is pregnant with her second child comes to the office for consultation regarding the risk that the child will have a cleft lip and palate. She has a history of a cleft lip and palate, and her first child also has a cleft lip and palate. The father has no history of cleft lip or palate. Which of the following best represents the likelihood that the patient’s second child will be born with a cleft lip and palate?
A ) 2% B ) 4% C ) 5% D ) 10% E ) 14%
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%.
2010
Which of the following muscles is used to construct the sphincter during a sphincter pharyngoplasty for the treatment of velopharyngeal insufficiency?
A ) Levator veli palatini B ) Musculus uvulae C ) Palatopharyngeus D ) Superior constrictor E ) Tensor veli palatini
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.
2010
A 2-year-old boy with a cleft palate and recurrent episodes of serous otitis media caused by impaired eustachian tube function is scheduled to undergo cleft repair. In reconstructing the palate, repair of which of the following muscles is most likely to improve eustachian tube function?
A ) Levator veli palatini B ) Palatopharyngeus C ) Salpingopharyngeus D ) Superior constrictor E ) Tensor veli palatini
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 “pumping 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.
2010
A 35-year-old woman, gravida 1, para 2, with a history of bilateral cleft lip and palate comes to the office to discuss her risk for having a child with clefting. She reports that one of her twin children had a unilateral cleft lip and palate, and the other child did not have clefting but did have indentations of the lower lip. She reports no other history of clefting in her family, her husband, or her husband’s family. The likelihood that her next child will have a cleft lip and/or palate is closest to which of the following percentages?
A) 2.5% B) 4% C) 10% D) 17% E) 50%
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%.
2019
Which of the following is the prevalence of middle ear effusion in patients with isolated cleft palate?
A) 10% B) 30% C) 50% D) 70% E) 90%
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.
2019
A 3-month-old infant with 22q11.2 deletion syndrome is evaluated for an isolated cleft of the soft palate (Veau I). A Furlow palatoplasty is planned at age 9 months. Compared with nonsyndromic patients with the same cleft type, this patient will have a higher postoperative risk for which of the following?
A) Air embolus B) Palatal fistula C) Respiratory failure D) Velopharyngeal insufficiency E) Wound dehiscence
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.
2019
Which of the following is true about cleft lip with or without palate when compared with cleft palate only?
A) Cleft lip/palate does not have a nasal deformity
B) Cleft lip/palate has a lower overall birth incidence than cleft palate only
C) Cleft lip/palate has a slight female predominance
D) Cleft lip/palate incidence is similar among different ethnicities
E) Cleft lip/palate is less likely to be associated with a syndrome
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.
2019
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
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.
2019
