Cleft Lip & 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 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.
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.
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-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).
A newborn presents with an asymmetric bilateral cleft lip-nose-palate; the right side is incomplete and the left side is complete. A photograph is shown. Which of the following sequences of repair is most likely to provide the optimal outcome?
A) One-stage repair of the bilateral cleft lip
B) Repair cleft palate at the initial surgery
C) Repair the complete side cleft lip first, then stage incomplete side cleft lip at a later surgery
D) Repair the incomplete side cleft lip first, then stage complete side cleft lip at a later surgery
E) Simultaneous repair of cleft lip and cleft palate
The correct response is Option A.
Bilateral cleft lip repair is more commonly symmetric and is usually repaired as a one-stage repair around 3 to 6 months of age.
Asymmetrical bilateral cleft lip repair can be done in one stage or two stages, and the sequencing has been controversial. However, two recent studies show convincing evidence that even for asymmetrical bilateral cleft lip repairs, a one-stage repair leads to overall better symmetry and cleft lip repair outcomes. Therefore, a two-stage bilateral cleft lip repair is incorrect.
Cleft palate repair is typically performed closer to 9 to 12 months of age, therefore, cleft palate repairs at 6 to 9 months of age are less typical and irrelevant, as the two-stage repair is considered not optimal for this asymmetric bilateral cleft lip scenario.
2020
A 9-year-old boy with a history of a Veau class II cleft palate presents with hypernasality and stigmatizing speech. His surgical history includes a double opposing Z-plasty palatoplasty at 12 months of age. His palate is short, but it elevates appropriately. A palatal lengthening procedure with posteriorly based bilateral buccinator musculomucosal flaps is planned. This flap’s blood supply is based on branches from which of the following arteries?
A) Anterior ethmoid
B) Facial
C) Greater palatine
D) Internal carotid
E) Internal maxillary
The correct response is Option E.
The use of buccal musculomucosal flaps has gained in popularity for both primary palate repair and secondary speech surgery. The many advantages of adding these flaps to palate surgery include the ability to posteriorize the levator muscles, eliminate dead space, and lengthen the palate. The patient described, who has great palate function but a short palate, is an ideal candidate for buccinator musculomucosal flaps rather than a pharyngoplasty, which may lead to obstructive sleep apnea. Understanding the blood supply is critical to performing this flap in a reliable fashion.
2021
A 9-month-old infant undergoes repair of the palatal defect shown in the photograph using a Furlow palatoplasty. Which of the following best represents this patient’s risk for developing a palatal fistula postoperatively?
A) 5%
B) 10%
C) 15%
D) 20%
E) 25%
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.
2021
A 6-month-old female infant with holoprosencephaly is brought by her parents to the clinic for follow-up. The patient has no history of seizures, and she has demonstrated successful weight gain and good oral intake. A photograph is shown. The patient’s parents desire closure of the lip defect when appropriate. Which of the following treatments is most likely to result in the best outcome with minimal risk?
A) Closure with an Abbe flap
B) Closure with an Estlander flap
C) Closure with a Karapandzic flap
D) Primary lip adhesion
E) Single-stage local tissue arrangement
The correct response is Option E.
Treatment of children with a midline facial cleft is challenging because the plastic surgeon must balance the safety of the procedure with the family’s desire for treatment. Many of the children have severe brain deformities; due to seizures and metabolic issues, they are never surgical candidates and expire within the first year of life. A subset, now larger than previously recognized, do reasonably well and by 12 months of age are suitable surgical candidates. Because of their comorbidities, the plastic surgeon’s planning must optimize seizure control, airway protection, and nutrition. The goal is to achieve three-dimensional closure of the lip and nasal defect while minimizing the number of procedures and anesthesia risk.
Of the options listed, lip adhesion and Karapandzic flap only address the lip and not the columella. The Abbe flap addresses the columella but necessitates two operations and, due to the decreased intraoral opening between the two procedures, increases airway and feeding risk. The three-in-one procedure as described by Zwahlen et al gets everything adequately closed in a single stage. An Abbe flap can always be performed later if warranted and if the child survives long enough to need it. While these deformities are rare, the need for a plastic surgeon to reconstruct these three-dimensional defects in this area is not. An Estlander flap is incorrect, as it is similar to a Karapandzic flap in that it does not address the nasal (columellar) defect.
2023
