Cleft Lip and Palate 01-22 Flashcards
A 6-year-old girl with bilateral cleft lip and palate presents to the office with velopharyngeal dysfunction. Secondary speech surgery is planned. Which of the following procedures carries the highest risk for developing postoperative obstructive sleep apnea?
A) Conversion Furlow palatoplasty
B) Palatal lengthening with bilateral buccal flaps
C) Repeat palate repair with intravelar veloplasty
D) Sphincter pharyngoplasty
E) Velopharyngeal augmentation with structural fat grafting
The correct response is Option D.
Multiple techniques have been described for management of velopharyngeal dysfunction (VPD) following primary palatoplasty in patients with cleft palate and cleft lip/palate. These include pharyngoplasty techniques, such as posterior pharyngeal flaps and sphincter pharyngoplasties. These procedures add tissue to the velopharyngeal port and partially occlude airflow. These procedures are the most effective in correcting VPD but carry with them the highest risk for postoperative obstructive sleep apnea (OSA). The incidence of postobstructive OSA following sphincter pharyngoplasty varies between 50 to 69% of patients. Other procedures described include palatoplasty techniques such as intravelar veloplasties, conversion Furlow palatoplasties, and palatal lengthening procedures. These procedures have been shown to correct VPD in 40 to 80% of cases and have varying risks for postoperative OSA, varying from 10 to 54%. Finally, augmentation of the velopharyngeal port with structural fat grafting is successful in correcting mild cases of VPD, but it was recently shown to carry a small (11%) risk for postoperative OSA. Treatment of VPD is nuanced, and all techniques should be considered. Patient factors such as the history of prior palate surgery, physical examination, dynamic assessment of velopharyngeal port function, and patient comorbidities must be considered to select the appropriate procedure in every case.
A 12-week-old infant undergoes repair of a complete bilateral cleft lip. Which of the following is a principle of both bilateral and unilateral cleft lip repair?
A) Centralizing the columella
B) Creating a rotation advancement flap
C) Discarding the prolabial vermillion
D) Establishing muscular continuity
E) Preparing the projecting premaxilla
The correct response is Option D.
Establishing orbicularis oris continuity is a goal in both unilateral and bilateral cleft lip repair. Centralizing the columella is performed only in unilateral cleft lip repair. The prolabium is only apparent in bilateral cleft lips. Preparing the projecting premaxilla is done only in bilateral cleft lip repairs. Creating a rotation advancement flap is performed only in unilateral cleft lip repair.
A 2-week-old male newborn with a wide unilateral cleft lip and palate presents for evaluation and surgical treatment planning. To align the alveolar segments, decrease the cleft width, and improve the nasal contour, nasoalveolar molding versus lip adhesion is discussed with his parents. The primary advantage of lip adhesion over nasoalveolar molding is which of the following?
A) Decreased cost
B) Decreased scar tissue
C) Fewer treatment complications
D) Improved clinical outcome
E) Increased compliance
The correct response is Option E.
Lip adhesion is the surgical version of nasoalveolar molding (NAM) in terms of accomplishing decreased cleft width and alveolar alignment; however, it does not do much for the nasal contour. The primary advantage of lip adhesion surgery is that it minimizes the burden on the family. It costs more, potentially can create more scar, and has a higher complication rate in the form of either scar or wound dehiscence. There is no proven clinical outcome advantage, and some would argue that because NAM also addresses the nose, the outcome is better, but that will take additional studies to validate.
Which of the following is the normal anatomic position of the levator veli palatini muscle?
A) It originates from the floor of the mouth and runs anterior to the palatine tonsils
B) It originates from the lateral aspect of the Eustachian tube and inserts onto an aponeurosis in the anterior 25% of the velum
C) It originates from the medial aspect of the Eustachian tube and inserts onto the posterior aspect of the hard palate and tensor aponeurosis
D) It originates from the medial aspect of the Eustachian tube and runs transversely in the middle 50% of the velum
E) It originates from within the posterior palate and runs posterior to the tonsilar fossa
The correct response is Option D.
Velopharyngeal port (VP) function results from the coordinated effort of the musculature of the velum and pharyngeal wall. The levator veli palatini muscle is the key muscle responsible for elevation of the soft palate, which is necessary for VP closure. In patients with a cleft palate or submucous cleft palate, clefting of the muscle and its abnormal insertions result in loss of the normal levator sling that elevated the palate. Reconstruction of this levator sling through an intravelar veloplasty (IVVP) is a critical component of palatoplasty procedures to ensure optimal VP function and speech development.
In its normal anatomic relationship, the levator veli palatini muscle has a muscular origin from the petrous temporal bone, the carotid canal, and cartilaginous Eustachian tube. The muscle forms a muscular sling by joining with its paired contralateral muscle running in a transverse orientation in the middle 50% of the velum.
The pathologic orientation of the levator muscle is seen in a cleft palate. The muscles are not transversely oriented. Instead of joining with the contralateral levator muscle in the midline, the muscles are instead oriented more sagittally and insert into the posterior edge of the hard palate and tensor aponeurosis laterally.
The tensor veli palatini muscle originates from the greater wing of the sphenoid and lateral aspect of the cartilaginous and membranous Eustachian tube. The majority of its fibers pass around the pterygoid hamulus as a tendon and insert into the tensor aponeurosis, which occupies the anterior 20 to 30% of the velum. This muscle acts as a shock absorber for the velum and primary dilator of the Eustachian tube.
The palatoglossus muscle is also a paired muscle of the velum that descends in the anterior faucial pilar anterior the palatine tonsil and inserts into the dorsolateral aspect of the posterior tongue. It contributes to velar depression and glossal elevation.
The palatopharyngeus muscle originates from the posterior palate and runs posterior to the tonsillar fossa. These muscles are often used for sphincter pharyngoplasty.
A plastic surgeon is on a global surgery mission for primary cleft repairs in a developing country. It is most appropriate to perform a single-stage primary cleft lip and palate repair in which of the following patients?
A) Developmentally normal 1-year-old, 4 kg (8.8 lb), hemoglobin concentration of 8 g/dL
B) Developmentally normal 2-year-old, 8 kg (17.6 lb), hemoglobin concentration of 7 g/dL
C) Developmentally normal 4-year-old, 17 kg (37.5 lb), hemoglobin concentration of 11 g/dL
D) Syndromic, globally-delayed 2-year-old, 10 kg (22 lb), hemoglobin concentration of 10 g/dL
E) 67-Year-old woman, 55 kg (121.2 lb), hemoglobin concentration of 11 g/dL
The correct response is Option C.
In the United States, most patients undergo cleft lip repair at age 3 to 6 months and cleft palate repair between age 9 and 18 months. Some children may undergo repairs earlier or later because of surgeon or patient factors. Most children in the United States undergo staged repairs, but it may be indicated to perform combination surgery of primary cleft palate and cleft lip surgery at the same setting. Combination surgery may occur in cases of older children adopted from developing countries who need both speech improvement and aesthetic reconstruction, and are healthy enough for the combined surgeries.
In global surgery, there is increasingly more information about patient outcomes and more data collection to guide decision making. Still, the role of the surgeon’s clinical decision-making is important and patient factors are relevant. If access to care is limited for a patient who is very healthy, then combination cleft lip and palate surgery may be indicated. Complications are more likely when the two surgeries are combined. Palate bleeding and surgical blood loss may be increased in combination surgery, and blood transfusions are more often necessary than in single-staged surgery.
Many global surgery programs in developing countries recommend that patients have a hemoglobin concentration of 10 g/dL or greater in order to safely undergo cleft palate surgery. Cleft lip surgery generally involves less blood loss than cleft palate surgery, and some surgeons/programs may safely proceed with lip surgery alone when a patient’s hemoglobin concentration is less than 10 g/dL.
Combination surgery requires longer surgical times than single-staged surgery. The longer anesthetic time may be a factor for patients with other medical conditions or for syndromic patients. Underweight, young children with a hemoglobin concentration less than 10 g/dL are not good candidates for combined cleft lip and palate surgery. Staged surgery would be safer for those children. Administration of blood transfusion before surgery to increase the hemoglobin is not recommended.
Cleft palate repair in elderly patients is unlikely to lead to a meaningful improvement in quality of life regarding speech, and these patients may have bleeding and healing difficulties. Therefore, palate repair in elderly patients is not recommended in combination surgery. Aesthetic reconstruction of the lip can often be safely performed under local anesthesia if the patient is otherwise healthy.
A 9-year-old boy with a history of cleft lip and palate is brought for evaluation by his parents because they have a strong desire for their son to undergo nasal revision this summer. The patient is otherwise healthy, communicative, and developmentally appropriate. Physical examination shows that he has mild nasal tip asymmetry; his nasal airway is patent; his midface is well-projected; Cupid’s bow is balanced and symmetric, as is his vermillion. The patient states he does not want to have surgery. Which of the following is the most appropriate initial course of action?
A) Openly discuss the disparity between the parents’ and patient’s goals to better understand their respective motivations
B) Refer the parents and child to family counseling to address their competing interests
C) Schedule the patient for surgery this summer; the parents have medical decision-making authority
D) Set aside the parents’ desires; the child does not want surgery
E) Surgery is not indicated; refer the case to child protective services
The correct response is Option A.
Informed consent in the pediatric surgical patient can present a pediatric plastic surgeon with complex ethical issues. This is especially true when minor children and their legal guardians disagree about surgical decisions. In this common scenario described, the parents are interested in cleft nasal revision, whereas the patient is not. In such a scenario, it is important to understand both stakeholders’ viewpoints. Is the patient worried about missing vacation or a sporting activity over the summer or does his deformity not bother him? Do the parents have perspective they have not shared or do they have an unreasonable expectation for their child? These are all important considerations. Of the answer choices, only open discussion starts to bring the competing parties closer together.
A 16-year-old boy presents with an unrepaired alveolar cleft. Because he is an athlete, he refuses iliac crest bone grafting. To reduce donor morbidity, a decision is made to proceed with repair using bone morphogenetic protein-2 (BMP-2) in an absorbable collagen sponge carrier. Compared with autologous bone grafting, repair with BMP-2 is more likely to place the patient at increased risk for which of the following postoperative complications?
A) Heterotopic ossification
B) Infection
C) Malignant transformation
D) Mucosal atrophy
E) Severe edema
The correct response is Option E.
Bone morphogenetic protein-2 (BMP-2) works via osteoinduction to stimulate osteoprogenitor cells to differentiate into osteoblasts that begin new bone formation. Randomized controlled trials comparing BMP-2 to bone graft controls in alveolar cleft reconstruction have shown variable results, with most showing equivalence. An analysis of 17 randomized controlled trials of BMP-2 showed that severe prolonged edema was the most common complication, in some cases requiring steroid treatment or reoperation or leading to wound dehiscence. The use of recombinant human bone morphogenetic protein-2 (rhBMP-2) in patients younger than 18 years of age remains off-label.
Concerns have been raised about a potential increase in malignancy, infection, and heterotopic ossification (HO) with the use of BMP-2 in spinal surgery. However, in these randomized controlled trials, HO and malignant transformation were not observed and rates of infection were no different. There is no difference in the rate of mucosal atrophy between these techniques.
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.
A 17-year-old boy with a history of unrepaired submucous cleft palate comes to the office for follow-up evaluation 3 months after undergoing a Le Fort I procedure. The parents report difficulty understanding his speech since the surgery. Nasometry discloses hypernasal speech. Which of the following would necessitate a pharyngeal flap compared to a Furlow palatoplasty or a sphincter pharyngoplasty?
A) Le Fort I advancement of 10 mm
B) Poor lateral wall motion on nasopharyngoscopy
C) Postoperative nasopharyngeal depth of 25 mm
D) Preoperative soft palatal length of 32 mm
E) Velopharyngeal gap of 12 mm on maximum phonation
The correct response is Option E.
A Le Fort I procedure is needed in 25 to 40% of patients with cleft lip and palate, and velopharyngeal insufficiency (VPI) is often seen postoperatively. Preoperative soft palatal length and postoperative pharyngeal depth have been associated with the need for a VPI procedure. In a 17-year-old, the normal soft palatal length is 32 mm and the normal nasopharyngeal depth is 24 mm. A significant velopharyngeal gap of greater than 5 mm cannot be adequately corrected with a Furlow palatoplasty or sphincter pharyngoplasty. Poor lateral wall motion alone would be a finding that supports a sphincter pharyngoplasty. The Le Fort I advancement distance has not been directly related to the need for a specific type of procedure.
References
During dissection of the nasal mucosal flap in a Furlow palatoplasty procedure for correction of velopharyngeal insufficiency, the surgeon inadvertently makes a large defect in the nasal mucosal flap, leading to the inability to transpose the nasal lining flap for closure. Which of the following closure options will provide the least donor site morbidity for this patient?
A) Buccal fat pad flaps
B) Hard palate myomucosal flap
C) Sphincter pharyngoplasty
D) Superiorly based pharyngeal flap
E) Vomer mucosal flap
The correct response is Option A.
Buccal fat pad flaps are a technically simple, quick option for closure of mucosal defects in palatal closure. Their use has not been associated with significant donor site morbidity, and they are able to reach across the transverse maxillary arch, even in wide clefts, which makes them useful in closure or reinforcement of mucosal defects in palatoplasty.
A superiorly based pharyngeal would not only create more donor site morbidity than using a buccal fat pad flap, but using the pharyngeal flap could also cause obstructive sleep apnea, and therefore is best used as a secondary or tertiary procedure in case of persistent velopharyngeal insufficiency (VPI), not as an adjunct in primary palatoplasty.
A hard palate myomucosal flap would not adequately address the problem of nasal mucosal loss, and attempting to use one to cover nasal lining would likely cause significant donor site complications, such as exposure of the hard palate maxillary bone.
Sphincter pharyngoplasty is not an option for nasal mucosal closure, since this procedure is designed to augment the posterior pharynx and tighten the oronasal aperture.
Vomer flaps are useful in nasal lining closure for the hard palate, but are not useful for closure of the nasal lining of the soft palate.
A 6-month-old female infant presents with a bifid uvula, notch of the hard palate, and zona pellucida. The infant is feeding well with no signs of airway obstruction. Which of the following is the most appropriate next step in management?
A) Initiation of speech therapy
B) Nasoendoscopy
C) Palatal elevator
D) Palate repair at 1 year of age
E) Re-evaluation with cleft team in 1 year
The correct response is Option E.
The patient has the three classic clinical features of submucous cleft palate. The overall incidence of overt submucous cleft palate is exceedingly rare (0.02 to 0.08%). Although there is some ongoing debate regarding the timing of surgical management, it is agreed that only a small number of patients will need surgical intervention for good speech outcomes.
Velopharyngeal insufficiency (VPI) refractory to speech therapy is the most widely accepted indication for surgical correction. Speech cannot be adequately assessed until age 2.5 years at the earliest, and speech therapy cannot be initiated before this age.
A typical course of management is diagnosis of VPI and initiation of speech therapy for 6 to 12 months. When therapy is found to be refractory, surgical intervention is planned. Surgical options are: excision with primary closure of the submucous cleft, pharyngeal flap, palatal pushback, palate repair with intravelar veloplasty, Furlow Z-plasty, or combined techniques. The approach is often tailored to the patient.
In this case of a 6-month-old patient, it is too early to consider surgical intervention. The patient should be followed by a cleft palate team. When VPI is diagnosed by the speech therapist, therapy should be initiated, and if improvement is refractory to therapy, surgery should be planned. Nasoendoscopy can be used as a preoperative assessment to plan for surgery. There is no role for palatal elevators in the management of the submucous cleft palate.
Speech outcomes are best if surgery is performed before age 7 years. Before age 7, 84% of patients go on to develop normal speech or occasional hypernasality versus only 64% of patients have an equivalent outcome when the operation is performed after age 7 years.
A 3-week-old newborn with unilateral complete cleft lip and palate is brought to the clinic for initial evaluation. The parents are very concerned about dental development. Which of the following permanent teeth is most likely to be congenitally absent as a result of the clefting?
A) Canine
B) Central incisor
C) First premolar
D) Lateral incisor
E) Second premolar
The correct response is Option D.
Although all of the teeth listed have been described as being affected in patients born with cleft lip and palate, studies demonstrate that the permanent lateral incisor is most prone to be affected in the area of the cleft. It is frequently congenitally missing. It may also be hypoplastic or demonstrate a delay in root development and eruption. The other teeth can all be affected by alveolar clefting, but not to the degree to which the lateral incisor is affected. The lateral incisor is congenitally absent in up to 56% of patients with unilateral cleft lip and palate.
A patient with unilateral complete cleft lip and palate presents for discussion of alveolar bone grafting. The permanent lateral maxillary incisor is absent. Which of the following is the most accepted time period to perform an alveolar bone graft in this patient?
A) Before eruption of the central incisors
B) Following eruption of the permanent canine
C) In infancy, at the time of primary lip repair
D) In mixed dentition, before eruption of the permanent canine
The correct response is Option D.
Reconstruction of the cleft alveolus seeks to close any remaining oronasal fistulae, stabilize the maxillary segments into a single dental arch, and provide bone for eruption of teeth adjacent to the cleft with optimal periodontal support.
Primary bone grafting involves placement of a bone graft at the time of the initial cleft lip repair in the neonatal period. It is different from performing a gingivoperiosteoplasty (GPP), in which soft tissue repair of the alveolus is performed to close the anterior palatal fistula and encourage bone formation in the gap. Primary bone grafting has fallen out of favor in most American cleft centers, as several studies have shown diminished maxillary growth, poor arch relationships, and greater need for orthognathic surgery following this procedure. Secondary bone grafting can be performed at various times during dental development. Many children with cleft lip and palate have a poorly formed or missing lateral incisor. When a normal lateral incisor is present, a compelling argument can be made for earlier secondary bone grafting prior to its eruption to optimize the periodontal health and longevity of this tooth. The child in the clinical vignette is missing a lateral incisor on the side of the alveolar cleft.
Bone graft material will only heal successfully to adjacent bone. Exposed periodontal ligament, dentin, and enamel from erupted teeth do not adhere/heal to bone graft, and graft material adjacent to these structures will resorb. Growth following secondary bone grafting between 8 and 12 years of age has been shown to be equivalent to growth seen in patients with similar clefts that remained ungrafted. Additional studies have shown decreased success rates for bone grafts performed after the eruption of the secondary canine. Optimal bone graft success seems to result when grafting is performed when the secondary canine root is one-half to two-thirds developed, which allows the canine to erupt through the graft. The maxillary canine erupts between 11 to 12 years of age. Due to the need to graft before canine eruption and the variability in dental eruption in children with clefts, the optimal timing for alveolar bone grafting is often between 8 and 12 years of age, but treatment must be tailored to the patient’s chronological and dental age.
References
For a cleft palate and craniofacial team to be credentialed and approved, the American Cleft Palate-Craniofacial Association requires a minimum core of providers that includes a surgeon, speech and language pathologist, and which of the following providers?
A) Audiologist
B) Geneticist
C) Orthodontist
D) Psychologist
E) Social worker
The correct response is Option C.
A cleft palate and craniofacial team accredited by the American Cleft Palate-Craniofacial Association (ACPA) must have, as a minimum core, health care providers from the speech-language pathology, surgery, and orthodontics specialties. These providers must participate in team meetings as appropriate for specific patient needs. The participation of these individuals should be documented in each patient’s team reports.
The ACPA team must also have access to professionals in the disciplines of psychology, social work, audiology, genetics, general and pediatric dentistry, otolaryngology, and pediatrics/primary care. However, these providers are not considered core providers.
A 15-year-old girl with a history of a bilateral cleft lip and palate is evaluated because she is concerned about her nasal-sounding speech. On examination, she has a wide and poorly projected nasal tip, a bilateral cleft lip scar with a whistle deformity, mid face hypoplasia, persistent alveolar clefts with a protrusive premaxilla, a large fistula at the incisive foramen, and a straight line scar on the palate. Which of the following is the most appropriate first step in addressing her multiple cleft-related problems?
A) Alveolar bone graft
B) Fistula closure with premaxillary setback
C) Lip revision
D) Maxillary advancement
E) Rhinoplasty
The correct response is Option B.
Patients with cleft palate who are late-presenting and have undergone previous procedures that were performed out of the preferred sequence can be especially challenging cases. This patient, with a bilateral cleft lip and palate, has only had her lip and palate repaired. Yet she has significant speech, skeletal, and soft tissue sequelae of her cleft including unrepaired anterior hard palate fistulae and alveolar fistulae, inadequate central lip fullness (whistle deformity), a wide and poorly projected nose, mid face hypoplasia, and velopharyngeal insufficiency.
Sequencing procedures to address these issues is crucial to having favorable surgical outcomes. Addressing speech is the patient’s main concern and should be performed first. This includes closing of the oronasal fistula along with a premaxillary setback followed by a pharyngoplasty, if necessary. Alveolar bone grafts should be performed to stabilize the maxillary dental arch and minimize tooth injury. This should be followed by a lip revision to establish the lip-tooth relations prior to undergoing Le Fort I advancement. Finally, a rhinoplasty can be performed once the maxilla has been advanced.
A 7-year-old patient with a history of submucous cleft palate and persistent severe velopharyngeal insufficiency after Furlow palatoplasty undergoes video nasoendoscopy. An abnormal closure pattern is observed, with excellent movement of the velum and Passavant’s ridge but poor lateral wall motion. On the basis of these findings, which of the following is the most appropriate treatment for this patient’s velopharyngeal insufficiency?
A) Inferiorly based pharyngeal flap
B) Palatal lift appliance
C) Speech therapy
D) Sphincter pharyngoplasty
E) Superiorly based pharyngeal flap
The correct response is Option D.
With a “bow tie” pattern seen on the nasoendoscopy, the patient is an ideal candidate for sphincter pharyngoplasty, which will bring the lateral walls in more centrally, so the velum and pharynx can close off the velopharyngeal port.
Pharyngeal flap surgery is ideal for patients who have a large central gap or sagittal closure pattern caused by palatal hypotonia or shortened palatal length. Palatal soft tissue augmentation is ideal for patients with a very small central gap following adenoidectomy. A palatal lift appliance is typically used in patients with adequate palatal length, but inadequate motor function. The patient described in this scenario has excellent motor function of the velum, but poor lateral wall motion.
Speech therapy does not correct the anatomic deficiency in this patient population.
A 17-year-old girl with a history of bilateral cleft lip/palate presents for evaluation. She underwent lip and palate repair as a child, and alveolar bone grafting in mixed dentition. She has hypernasal speech. Physical examination shows severe mid face hypoplasia with 12 mm of negative overjet. On cephalometric analysis, SNA angle is 73 degrees (N 80–82), and SNB angle is 79 degrees (N 79–81). She is concerned about her appearance and her speech. Which of the following is the best initial option to address the patient’s concerns?
A) Bilateral sagittal split osteotomy with mandibular setback
B) Combined Le Fort I advancement and mandibular advancement
C) Maxillary distraction
D) Pharyngoplasty
E) Speech therapy
The correct response is Option C.
This patient has severe maxillary retrusion and mid face hypoplasia in the setting of significant velopharyngeal deficiency. These two issues are at odds with one another. Advancing her mid face with a standard Le Fort I will worsen her velopharyngeal dysfunction (VPD), while addressing her VPD with a pharyngeal flap will make advancing her maxilla challenging. Only anterior segmental maxillary distraction will maintain her current velopharyngeal anatomy but allow for improved mid face projection. A pharyngoplasty can be performed relatively easily at a later date or as a secondary procedure. Performing a mandibular setback is not appropriate because she has normal mandibular projection.
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.
A 16-year-old girl, who was born with a complete unilateral cleft of the lip, alveolus, and palate, is missing the lateral incisor within the cleft. After secondary bone grafting of the alveolar cleft, which of the following prosthetic treatments is the best option for dental restoration in this patient?
A) Nasoalveolar molding
B) Osseointegrated implant and crown
C) Palatal obturator
D) Removable partial denture
E) Three-unit fixed partial denture
The correct response is Option B.
Patients with cleft lip and palate frequently have absence of teeth in the alveolar cleft or teeth that may be grossly abnormal or that erupt at an inappropriate angle and require removal. The lateral incisors are most commonly affected, although central incisors and canines may also be affected. Alveolar bone grafting during the mixed dentition phase restores adequate bone support for subsequent placement of an endosseous titanium implant, to which a permanent crown may then be attached. Many studies have reported the efficacy and safety of this approach. This has become the dentofacial prosthetic treatment of choice for the replacement of a single tooth due to its appearance, functionality, and longevity. A removable partial denture is one that rests on the surrounding soft tissues of the alveolar ridge and palate. Although aesthetics may be reasonable, it may cause irritation of the surrounding soft tissues and may produce movement during function. It is often a temporary solution at best.
A three-unit fixed partial denture is a prosthesis which spans the gap produced by the missing tooth by anchoring to the adjacent two teeth. However, the abutment teeth often require reduction to permit fixation of the prosthesis. While certainly longer lasting than a removable partial denture, a fixed partial denture will need to be replaced periodically, and therefore would not be the best choice for this young patient.
A palatal obturator is a prosthesis used to treat a residual oronasal fistula by physically blocking air escape during speech. This decreases hypernasality. It rests on the soft tissues of the palate and may anchor to the alveolar ridge or teeth. It does not, however, play a role in dental restoration. Nasoalveolar molding is a prosthesis-based treatment used early in life, typically for wide clefts, prior to repair of the lip and palate, but it is not used for dental restoration.
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%.
A 4-year-old boy is referred for evaluation after failing a hearing test. His mother reports that she has difficulty understanding his speech, and that liquids come out of his nose when he drinks beverages. Medical history also includes obstructive sleep apnea that was confirmed via sleep study. There is no history of previous surgery. Physical examination shows a bifid uvula. Which of the following indications is the primary reason to recommend a velopharyngeal insufficiency (VPI) procedure for this patient?
A) Conductive hearing loss
B) Hypernasal speech
C) Presence of a submucous cleft palate
D) Reflux of liquids through the nose
E) Snoring
The correct response is Option B.
The bifid uvula is a marker for a submucous cleft palate, but neither the bifid uvula or the submucous cleft palate are an indication for surgery in isolation. Children with a cleft palate will often have hearing loss, but this is not addressed with VPI procedures. Snoring will likely be made worse by the VPI procedure and is not an indication for this procedure.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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 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
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.
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.
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.
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.
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.
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.
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.
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.
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).
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.
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.
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.
A 31-year-old woman comes to the office with her husband for consultation about the risk that their child will have a cleft lip deformity. Neither of them have a cleft lip deformity, but they have a 2 €‘year €‘old child with an isolated unilateral cleft lip deformity. Which of the following percentages best represents the risk that this couple will have another child with a cleft lip deformity?
(A) 2%
(B) 4%
(C) 9%
(D) 15%
(E) 22%
The correct response is Option B.
The genetics of the cleft lip (CL) deformity, with or without a cleft palate (CL/P) or an isolated cleft palate deformity (CP), are complicated and do not follow a typical mendelian inheritance pattern. The cause is thought to be multifactorial, involving both genetic and environmental factors. Most researchers agree that there is a genetic component to the development of the CL/P or CP deformities. This is supported by studies in cleft monozygotic twins, in whom the concordance rate is 30% to 60% compared with 1% to 4.7% in dizygotic twins. Families with a member with a CL/P or CP deformity have a higher risk than the general population of having another child with a similar deformity. The increased risk is outlined in the table below.
A 16-year-old girl who has had repair of bilateral cleft lip, nose, and palate, and a pharyngeal flap has the occlusion shown in the photograph and radiograph. She has 12 mm of negative overjet. Cephalometric evaluation shows no mandibular abnormalities. Which of the following is the most appropriate procedure for aesthetic correction of the malocclusion?
(A) Le Fort I maxillary advancement
(B) Le Fort I maxillary distraction osteogenesis
(C) Mandibular setback with advancement genioplasty
(D) Segmental maxillary advancement
(E) Two-jaw maxillary advancement and mandibular setback
The correct response is Option B.
For children with orofacial clefting and severe Class III malocclusion, maxillary advancement is the procedure of choice. When there is a large maxillary move to be performed (greater than 6 to 8 mm), and there has been prior surgery resulting in scarring in the region (cleft lip, cleft palate, alveolar bone graft, pharyngeal flap), large movements of the maxilla are challenging and result in significant relapse. In addition, when there is normal shape and position of the mandible, surgery on the mandible is not indicated. Historically, because of the difficulty in performing the large required movements of the maxilla, the €œdifference € of movement would be shared between the maxilla and mandible. For a 12-mm movement, 6 mm of anterior movement of the maxilla would be performed, and the mandible would be set back 6 mm. Today, with the option of mid-face distraction, the appropriate aesthetic large movements of the maxilla can be performed to spare unnecessary surgery on the mandible.
For the preoperatively marked rotation-advancement repair shown, select the corresponding labeled flap (A €“D).
(A) A
(B) B
(C) C
(D) D
(Item 117) Rotation
(Item 118) Advancement
The correct response for Item 117 is Option A and for Item 118 is Option B.
The A flap is marked on the medial lip element and is the rotation flap. This flap is designed as a gentle curve from the height of Cupid €™s bow on the cleft side of the medial cleft element to the junction of the philtrum and columella. This incision approaches the normal philtral collum as much as needed to derotate and level Cupid €™s bow. If more rotation is needed to level Cupid €™s bow, a back €‘cut is made, but the normal philtral collum is not violated. This incision is used in philtral subunit reconstruction and leveling Cupid €™s bow.
The B flap is made on the non-cleft, lateral lip element and is the advancement flap. This flap is the €œwedge € that is placed in the defect created above the rotation flap as the medial lip element is derotated to level Cupid €™s bow. This flap is marked by first finding the point on the white roll that will correspond to the height of Cupid €™s bow on the medial cleft element. This point is found where the fullness of the lateral lip element begins to fade. A line is drawn from this point cephalad and is the same length as the normal philtral collum as well as the marked edge of the A (rotation) flap.
The C flap or columellar flap is made from the tissue medial to the A €‘flap incision. This small flap is rotated into the columella, lengthening the cleft side of the columella. It is also sewn to the D flap (alar base flap).
The D flap or alar base flap is cut along the alar €‘facial groove, and this incision corresponds to the cephalad portion of the edge of the B flap (advancement flap). This alar groove incision is made as small as possible and long enough to allow differential rotation of the D flap and the B flap.
The L flap is the leading edge of the lateral lip that can be used to fill the nasal lining defect. The M flap is the medially based flap from the rotation.
A 10-year-old boy who underwent bilateral cleft lip repair during infancy has tightness of the upper lip. A photograph is shown above. On physical examination, the philtrum is excessively wide and hypoplastic with absence of muscle competence. Redundancy of the lower lip is also noted. Which of the following is the most appropriate method of flap reconstruction?
(A) Abbe flap
(B) Banked fork flaps
(C) Gilles fan flap
(D) Karapandzic flap
(E) Nasolabial flap
The correct response is Option A.
The most appropriate management is reconstruction with an Abbe flap. This lip-switch flap has been designed specifically to create a functional philtrum in patients who have tightness of the upper lip following cleft lip repair. After the abnormal philtrum is excised, redundant tissue is harvested from the lower lip to replace the resultant defect as an aesthetic subunit. The Abbe flap is pedicled on the submucosal labial artery of the lower lip at the superior edge of the inner free border of the lip. The pedicle is subsequently divided during a delayed second procedure, which is performed a minimum of 10 days to four weeks after flap transfer.
In adults, the reconstructed philtrum should be no wider than 10 mm and no longer than 15 mm; however some surgeons advocate using a smaller flap that has a width of 7 to 9 mm at the cupid’s bow and 4 to 5 mm at the labial-columellar junction.
Banked fork flaps were used previously for bilateral lip reconstruction. At the time of primary lip repair, extra tissue on either side of the new philtrum from the prolabium was “banked” within the creases of the alar base to be used as forked flaps for columellar reconstruction at a later date. However, it was subsequently determined that the columella in fact lies within the nasal region and that primary nasoplasty should be performed instead at the time of lip repair to create the columella. In addition to primary nasoplasty, adequate preoperative nasoalveolar molding has been determined to be more appropriate, and banked fork flaps are no longer used.
The Gilles fan and Karapandzic flaps are oral circumference advancement flaps that were originally designed for lower lip reconstruction, but are also used for upper lip defects. These flaps rotate around the commissure and narrow the oral aperture. They are not designed for reconstruction of the philtrum as an aesthetic subunit.
In patients with nasal deformities associated with complete unilateral cleft lip, which of the following best describes the displacement of the ala?
(A) Lateral, inferior, and anterior
(B) Lateral, inferior, and posterior
(C) Medial, inferior, and posterior
(D) Medial, superior, and anterior
(E) Medial, superior, and posterior
The correct response is Option B.
In patients who have nasal deformities associated with complete unilateral cleft lip, the ala is displaced laterally, inferiorly, and posteriorly. The orbicularis oris inserts onto the alar base abnormally, on the lateral cleft side, and pulls the ala laterally and inferiorly. The medial orbicularis inserts on the nasal spine and caudal septum and pulls these structures to the contralateral side. The piriform rim, which normally supports the ala, is also deficient, allowing the ala to be displaced posteriorly on the side of the cleft.
