CL & CP, CL Nose, VPI Flashcards
A 25-year-old woman who is pregnant with her second child comes to the office for consultation regarding the risk that the child will have a cleft lip and palate. She has a history of a cleft lip and palate, and her first child also has a cleft lip and palate. The father has no history of cleft lip or palate. Which of the following best represents the likelihood that the patient’s second child will be born with a cleft lip and palate?
A) 14%
B) 2%
C) 4%
D) 5%
E) 10%
Correct answer is option A.
An affected parent with one affected child has a 14% risk for future offspring to have a cleft lip and palate. If both parents are not affected, and their first child has a unilateral defect, the risk would be 2.7% for the next child and 5.4% if the first child had a bilateral defect. If both parents were unaffected and had two affected children, then the risk for the subsequent pregnancy to result in a cleft lip and palate would be 10%.
What structure separates the primary and secondary palates?
A) Maxillary prominence
B) Incisive foramen
C) Medial nasal prominence
D) Greater palatine foramen
The correct answer is the incisive foramen, located immediately posterior to the central incisors. The maxillary prominence forms the maxilla; the medial nasal prominence is part of the structure that forms the primary palate. The greater palatine foramen is the exit site for the greater palatine artery.
During embryologic development, which of the following structures arises from the lateral nasal processes?
(A) Columella
(B) Nasal bridge
(C) Nasal tip
(D) Nasal ala
(E) Nasal septum
The correct response is Option D.
The nasal structures form during the sixth week of gestation as the medial nasal processes enlarge and coalesce in the midline. Any abnormalities that occur during formation of the nasal structures may result in deformity, such as the cleft nasal deformity.
The nasal alae arise from the lateral nasal processes. The medial nasal processes give rise to the columella, nasal tip, philtrum, and premaxilla. The bridge and root of the nose arise from the frontonasal processes.
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 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 25 year old man who underwent repair of unilateral cleft lip and palate comes to the office for consultation regarding a dentofacial deformity. On cephalometric analysis, the SNA angle is 70 degrees (N 81.2 degrees), the SNB angle is 80 degrees (N 77.3 degrees), and 18-mm negative overjet is noted. Which of the following is the most appropriate management for correction of this deformity?
(A) Le Fort I advancement and jumping genioplasty
(B) Le Fort I advancement and sagittal split osteotomy advancement
(C) Le Fort I advancement and sagittal split osteotomy setback
(D) Le Fort II advancement
(E) Le Fort III advancement and mandibular setback
The correct response is Option C.
The patient shown below has maxillary retrusion and mandibular prognathism, which is most appropriately treated with a maxillary (Le Fort I) advancement and a sagittal split osteotomy (mandibular setback). The Angle class III malocclusion is demonstrated in photograph, and cephalometric analysis shows overprojection of the mandible and underprojection of the maxilla. Le Fort I advancement also could be attempted but would be difficult for such a discrepancy in occlusion; bimaxillary prognathism would result.
For the preoperatively marked rotation-advancement repair shown, select the corresponding labeled flap (A through D) that denotes the Advancement Flap.
A) A
B) B
C) C
D) D
The A flap is marked on the medial lip element and is the rotation flap. This flap is designed as a gentle curve from the height of Cupid’s bow on the cleft side of the medial cleft element to the junction of the philtrum and columella. This incision approaches the normal philtral column as much as needed to de-rotate and level Cupid’s bow. If more rotation is needed to level Cupid’s bow, a back-cut is made, but the normal philtral column is not violated. This incision is used in philtral subunit reconstruction and leveling Cupid’s bow. The B flap is made on the non-cleft, lateral lip element and is the advancement flap. This flap is the “wedge” that is placed in the defect created above the rotation flap as the medial lip element is de-rotated to level Cupid’s bow. This flap is marked by first finding the point on the white roll that will correspond to the height of Cupid’s bow on the medial cleft element. This point is found where the fullness of the lateral lip element begins to fade. A line is drawn from this point cephalad and is the same length as the normal philtral column as well as the marked edge of the A (rotation) flap. The C flap or columellar flap is made from the tissue medial to the A-flap incision. This small flap is rotated into the columella, lengthening the cleft side of the columella. It is also sewn to the D flap (alar base flap). The D flap or alar base flap is cut along the alar-facial groove, and this incision corresponds to the cephalad portion of the edge of the B flap (advancement flap). This alar groove incision is made as small as possible and long enough to allow differential rotation of the D flap and the B flap. The L flap is the leading edge of the lateral lip that can be used to fill the nasal lining defect. The M flap is the medially based flap from the rotation.
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 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.
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.
A 5-year-old child who underwent cleft palate repair in infancy has velopharyngeal insufficiency. Nasoendoscopy shows closure of the central velopharyngeal mechanism with residual openings on the left and right. The patient is scheduled to undergo sphincter pharyngoplasty for reconstruction of the pharynx.
This procedure involves transposition of which of the following muscles?
(A) Buccinator
(B) Levator veli palatini
(C) Palatoglossus
(D) Palatopharyngeus
(E) Tensor veli palatini
The correct response is Option D.
In this child who is scheduled to undergo sphincter pharyngoplasty, the palatopharyngeus muscle is incorporated within the flap. Two techniques are used primarily for reconstruction of the pharynx in patients with velopharyngeal insufficiency. The sphincter pharyngoplasty elevates and insets the posterior tonsillar pillars, which contain the palatopharyngeus muscles, into the posterior pharyngeal wall to create a sphincter. In contrast, during pharyngeal flap reconstruction, a flap of tissue from the posterior pharyngeal wall is elevated and attached to the palate. This procedure is typically reserved for correction of deficits of the central palate.
The levator veli palatini muscle, which is found in the midline of the soft palate, is used only for pharyngeal flap reconstruction, not for sphincter pharyngoplasty. The buccinator muscle lies anterior to the pterygomandibular raphe, the palatoglossus muscles are contained within the anterior tonsillar pillars, and the tensor veli palatini is located anterior to the levator veli palatini. None of these muscles are used in sphincter pharyngoplasty.
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.
The C flap in the Millard rotation advancement repair of unilateral cleft lip is used to achieve which of the following?
A) Lengthening of the columella
B) Lengthening of the lip
C) Reduction of alar flare
D) Shortening of the lip
E) Symmetry in the philtral column
Correct answer is option A.
One of the characteristic findings in the unilateral cleft deformity is a shortened columella. The C flap is designed to lengthen the columella. Other findings in the unilateral cleft nose include deviation and distortion of the septum (corrected with presurgical orthodontics), dislocation and slumping of the alar cartilage (corrected by dissecting the medial part of the cartilage and constructing a medial crus by suturing to the normal side), and flaring of the alar base (corrected with the alar cinch procedure).
The parents of the 3-month-old male infant shown come to the office for consultation. The parents have another child, who also has a cleft lip and palate (CL/P). Neither parent has CL/P. Which of the following best represents the likelihood that their third child will have CL/P?
A ) 2%
B ) 4%
C ) 9%
D ) 17%
E ) 50%
The correct response is Option C.
The risk of having a child with a cleft lip and palate (CL/P) is dependent on multiple factors. Familial cases have a risk that is dependent on family history, parental involvement, and other sibling involvement. Risks can be categorized as follows:
-Normal parents, one child with a CL/P: the risk for the next child is 4%.
-Normal parents, two children with a CL/P: the risk for the next child is 9% (as in the scenario described.)
-Affected parent with a CL/P, no affected children: the risk for the next child is 4%.
-Affected parent with a CL/P, one child with a CL/P: the risk for the next child is 17%.
-Affected parent with a CL/P and lip pits who has van der Woude syndrome: the risk for an affected child would be 50%, as this is an autosomal dominant condition.
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
A 4-year-old girl who has velocardiofacial syndrome is evaluated because of severe velopharyngeal insufficiency. Reconstruction using a superiorly based pharyngeal flap is planned. Which of the following is the most important factor in determining the width of the flap?
A) Ability to close the pharyngeal donor defect
B) Lateral pharyngeal wall motion
C) Length of the soft palate
D) Palatal movement (coronal closure)
E) Position of the carotid arteries in the posterior pharynx
The correct response is Option B.
Reconstruction with a pharyngeal flap is a highly effective method of treating velopharyngeal insufficiency. This procedure involves elevating a rectangular flap, based superiorly or inferiorly, from the posterior pharynx and insetting it into the soft palate. The posterior raw surface of the flap is typically lined with trapezoidal flaps raised from the nasal side of the soft palate to limit contraction. The flap serves to obstruct air leakage into the nasal passage during speech. Extremely wide flaps can, consequently, also result in obstructive sleep apnea. In such situations, sphincter pharyngoplasties (Hynes) are sometimes used to avoid this potential complication.
The width of the flap is based on the degree of lateral pharyngeal wall motion. Since this creates a static bridge between the soft palate and the nasopharynx, the length of the palate and the degree of palatal movement have no influence on the choice of flap width. Although closing the donor defect expedites healing and decreases pain, this step is not necessary. Medial displacement of the internal carotid arteries as they pass along the posterior pharynx has been described in patients with velocardiofacial syndrome and should be considered when raising the flap. However, this should not impact the ability to raise a flap of sufficient width.
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.
A 6-year-old boy with velopharyngeal incompetence is scheduled to undergo sphincter pharyngoplasty. He underwent cleft palate repair in infancy. In the sphincter pharyngoplasty procedure, which of the following muscles is elevated in the posterior tonsillar pillar?
(A) Levator veli palatini
(B) Palatoglossus
(C) Palatopharyngeus
(D) Stylopharyngeus
(E) Superior pharyngeal constrictor
The correct response is Option C.
The levator veli palatini originates from the petrous portion of the temporal bone and the eustachian tube. It travels alongside the eustachian tube to enter the soft palate. This muscle elevates the velum toward the posterior pharyngeal wall to close the velopharyngeal mechanism and pull the eustachian tube open.
The palatoglossus muscle arises from the lateral margin of the tongue. It travels in the anterior tonsillar pillar to enter into the soft palate and functions to pull the soft palate downward.
The posterior tonsillar pillar is created by the palatopharyngeus. The palatopharyngeus muscle arises from fibers that commingle with the superior pharyngeal constrictor. It enters the soft palate, where its fibers intermingle with those of the levator. The palatopharyngeus functions to depress the soft palate and provide inward motion of the lateral pharyngeal wall. In a sphincter pharyngoplasty, superiorly based flaps are elevated from the posterior tonsillar pillar, sutured together, and inset into the posterior pharyngeal wall. These flaps contain the palatopharyngeus muscle.
The superior pharyngeal constrictor originates from the posterior pharyngeal raphe and courses downward and forward to insert into the medial pterygoid plate, the pterygomandibular raphe, and the posterior part of the hyoid. The stylopharyngeal muscle arises from the styloid process. It inserts between the fibers of the superior and middle pharyngeal muscles in the pharyngeal wall. These muscles play a role in swallowing.
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 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.
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 10‐year‐old boy who underwent bilateral cleft lip repair during infancy has tightness of the upper lip. A photograph is shown. 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
Correct answer is option A.
The most appropriate management is reconstruction with an Abbe flap. This lip‐switch flap has been designed specifically to create a functional philtrum in patients who have tightness of the upper lip following cleft lip repair. After the abnormal philtrum is excised, redundant tissue is harvested from the lower lip to replace the resultant defect as an aesthetic subunit. The Abbe flap is pedicled on the submucosal labial artery of the lower lip at the superior edge of the inner free border of the lip. The pedicle is subsequently divided during a delayed second procedure, which is performed a minimum of 10 days to four weeks after flap transfer. In adults, the reconstructed philtrum should be no wider than 10 mm and no longer than 15 mm; however some surgeons advocate using a smaller flap that has a width of 7 to 9 mm at the cupid’s bow and 4 to 5 mm at the labialcolumellar junction. Banked fork flaps were used previously for bilateral lip reconstruction. At the time of primary lip repair, extra tissue on either side of the new philtrum from the prolabium was “banked” within the creases of the alar base to be used as forked flaps for columellar reconstruction at a later date. However, it was subsequently determined that the columella in fact lies within the nasal region and that primary nasoplasty should be performed instead at the time of lip repair to create the columella. In addition to primary nasoplasty, adequate preoperative nasoalveolar molding has been determined to be more appropriate, and banked fork flaps are no longer used. The Gilles fan and Karapandzic flaps are oral circumference advancement flaps that were originally designed for lower lip reconstruction, but are also used for upper lip defects. These flaps rotate around the commissure and narrow the oral aperture. They are not designed for reconstruction of the philtrum as an aesthetic subunit. The nasolabial flap is a transposition flap that is derived from the nasolabial crease. It is most often used for reconstruction of the nasal alar subunit and has limited indications for lip reconstruction. It is not typically used for aesthetic subunit reconstruction of the philtrum.
A 2-year-old boy with a cleft palate and recurrent episodes of serous otitis media caused by impaired eustachian tube function is scheduled to undergo cleft repair. In reconstructing the palate, repair of which of the following muscles is most likely to improve eustachian tube function?
A ) Levator veli palatini
B ) Palatopharyngeus
C ) Salpingopharyngeus
D ) Superior constrictor
E ) Tensor veli palatini
The correct response is Option A.
In cleft palate reconstruction, repair of the levator veli palatini can improve eustachian tube function.
Individuals with unrepaired cleft palate suffer chronic otitis media, which can lead to permanent hearing loss. This is thought to be caused by the dysfunction of the eustachian tube. There are several paratubal muscles that are responsible for the opening and closing of the eustachian tube: the tensor veli palatini, the levator veli palatini, and the salpingopharyngeus.
Contraction of the levator veli palatini with superior and posterior displacement of the levator sling opens the eustachian tube. In an unrepaired cleft palate, the ability of the levator veli palatini to open the tube is lost because of its abnormal insertion on the posterior hard palate. Repositioning of this muscle during cleft palate repair restores the levator sling, allowing dilation of the eustachian tube.
The tensor veli palatini opens the eustachian tube and may have a pumping action that milks the contents of the tube. This muscle function is likely unaffected by clefting. However, its ability to open the eustachian tube may be iatrogenically reduced by complete hamular fracture or division of its tendon during cleft palate repair.
The salpingopharyngeus also opens and closes the eustachian tube. However, because of its small size, it is the least important of the paratubal muscles and has minimal functional significance.
The palatopharyngeus muscle optimizes velopharyngeal closure. Along with the superior constrictor, it causes medial displacement of the lateral pharyngeal wall.
The superior constrictor is the main component of the Passavant ridge and functions to bring about medial displacement of the lateral pharyngeal wall through a sphincteric mechanism.
The C flap in the Millard rotation advancement repair of unilateral cleft lip is used to achieve which of the following?
(A) Lengthening of the columella
(B) Lengthening of the lip
(C) Reduction of alar flare
(D) Shortening of the lip
(E) Symmetry in the philtral column
The correct response is Option A.
One of the characteristic findings in the unilateral cleft deformity is a shortened columella. The C flap is designed to lengthen the columella. Other findings in the unilateral cleft nose include deviation and distortion of the septum (corrected with presurgical orthodontics), dislocation and slumping of the alar cartilage (corrected by dissecting the medial part of the cartilage and constructing a medial crus by suturing to the normal side), and flaring of the alar base (corrected with the alar cinch procedure).
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.
The firstborn child of a Caucasian couple with no abnormalities has bilateral cleft lip and palate. Which of the following percentages best represents the possibility that this couple’s next child will have cleft lip, with or without cleft palate?
A) 2%
B) 4%
C) 10%
D) 16%
E) 32%
Correct answer is option B.
The correct answer for the sibling of a child with bilateral cleft lip and palate is approximately 4%, depending on the reference. Regardless, the risk decreases if there is no cleft palate in the proband and/or if the condition is unilateral. The incidence of cleft lip with or without cleft palate for the sibling of a child with bilateral cleft lip but no cleft palate is 6.7%, for the sibling of a child with unilateral cleft lip and palate is 4.9%, and for the sibling of a child with unilateral cleft lip but no cleft palate is 4.0%. If there are two affected children with cleft lip with or without cleft palate, the risk for a third child would be at least 9%. The overall risk of cleft lip with or without cleft palate in a Caucasian population is 1:1000. In a first‐degree relative, that risk is multiplied by 40; in a second‐degree relative, the risk is multiplied by 7; and in a third‐degree relative, the risk is multiplied by 3. However, the risk is increased when more than one relative is affected, if the family member has a severe form of the disorder, if the family member is of the sex least likely to be affected, or if the parents are consanguineous. For one affected parent, the risk of having one child with cleft lip with or without cleft palate is 4%, and the risk for a second child increases to 17%.
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.
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.
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.
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.
What contributes to formation of the secondary palate?
A) Medial and lateral nasal prominences
B) Lateral shelves of the maxillary prominence
C) Frontonasal prominence
D) Mandibular prominence
Correct answer is option B.
The lateral maxillary prominences elevate, and then approach the midline, with fusion of the palatal shelves from anterior to posterior at about day 50 of embryonic development. The medial and lateral nasal prominences, frontonasal prominence and the mandibular prominence contribute to development of facial structures.
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 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.
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.
A 10-month-old infant with cleft palate is scheduled to undergo repair via the Veau-Wardill-Kilner V-Y technique. Which of the following structures will NOT be directly manipulated during this repair procedure?
(A) Levator veli palatini muscles
(B) Musculus uvulae
(C) Nasal lining
(D) Palatopharyngeus muscles
(E) Tensor veli palatini muscles
The correct response is Option D.
Because cleft palate does not disrupt the palatopharyngeus muscles, they do not need to be repaired. Cleft palate repair aims to eliminate the oronasal fistula and optimize the function of the soft palate. The fistula is closed by creating a lining for the nasal and oral sides of the fistula. The abnormal attachments of the levator veli palatini and tensor veli palatini muscles to the hard palate are released, and the muscles are repaired in the midline, giving them a more functional transverse orientation. Finally, the musculus uvulae is repaired in the midline. This muscle provides bulk on the upper surface of the soft palate during velopharyngeal closure. Although the palatopharyngeus muscles are involved in velopharyngeal closure, they are not disrupted by cleft palate.
A neonate is undergoing evaluation because of airway obstruction. Physical examination shows retrogenia and glossoptosis. Which of the following is the most appropriate initial management of the airway obstruction?
(A) Prone positioning
(B) Orotracheal intubation
(C) Lip-tongue adhesion
(D) Tracheostomy
(E) Mandibular distraction osteogenesis
The correct response is Option A.
This neonate has the triad of symptoms associated with Pierre Robin sequence: retrogenia, glossoptosis, and respiratory distress. Approximately 50% of neonates with Pierre Robin sequence have a high-arched cleft in the midline of the soft palate; clefting of the hard palate also occurs in some patients. Glossoptosis is associated with airway obstruction, increased expenditure of energy, and impaired feeding resulting from decreased caloric intake. These feeding difficulties and failure to thrive can be fatal if not managed properly.
The most appropriate initial management of airway obstruction is to place the neonate in the prone position, which relieves the glossoptosis and opens the airway. In some infants, this position must be maintained constantly, even during feeding, bathing, and diaper changing. Intubation may be necessary if prone positioning does not resolve the respiratory distress.
Lip-tongue adhesion, tracheostomy, and mandibular distraction osteogenesis are secondary procedures that should only be considered if prone positioning is unsuccessful.
A 2-year-old girl is evaluated because of bifid uvula, notching of the posterior hard palate, and midline thinning of the posterior palate. She feeds well and has normal hearing. Which of the following is the most appropriate management at this time?
A)Furlow double-opposing Z-plasty
B)Nasendoscopy
C)Speech evaluation
D)Straight-line veloplasty
E)Veau-Wardill-Kilner push-back palatoplasty
The correct response is Option C.
This patient has physical findings suggestive of a submucous cleft palate: bifid uvula, notching of the posterior hard palate, and a thin midline region in the soft and/or hard palate termed the zona pellucida. The presence of any or all of these features is not required to secure the diagnosis and some patients will have none of them (occult submucous cleft palate). This anomaly is characterized by abnormal attachment of the palatal muscles (usually to the posterior edge of the hard palate) with intact nasal and oral mucosa.
Most patients with submucous cleft palate are asymptomatic; however, about 10 to 15% of affected individuals will demonstrate some degree of velopharyngeal insufficiency (VPI) and hypernasal speech. Other symptoms may include early feeding difficulties and recurrent middle ear effusion/infection. Borderline VPI may improve with speech therapy alone and early operative treatment should be deferred. Patients with severe or frank VPI, or those with less severe VPI who do not respond to speech therapy, will likely require operative intervention to improve speech.
The child in the scenario described is too young to reliably determine the presence of VPI by speech evaluation or diagnostic tests and, therefore, operative treatment of the submucous cleft using Furlow Z-plasty, straight-line veloplasty, or Veau-Wardill-Kilner push-back palatoplasty would be premature and potentially unnecessary. While the diagnosis of VPI can be made by a careful speech evaluation, it is difficult to obtain a reliable evaluation before 2 years of age. Nasendoscopy and videofluoroscopy are very useful to confirm the presence of VPI and to characterize the nature and degree of the deficiency. However, these important diagnostic tests are most informative in a cooperative patient and are rarely used in children younger than 3 years of age. Nasendoscopy could help confirm the presence of a submucous cleft palate, but such information will not change management unless the child develops hypernasal speech. Early evaluation and follow-up with a speech therapist would be appropriate to facilitate and monitor speech development.
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.
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.
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.
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.
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 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 16-month-old boy who underwent correction of a complete unilateral cleft lip 2 months ago is brought to the office because his mother is concerned about the appearance of the scar on his lip. Physical examination shows a corrected upper lip with a good pout and contour. The scar is flat, slightly widened, and moderately erythematous. Which of the following is the most appropriate management at this time?
A ) Continued optimal scar management
B ) Immediate revision of the scar
C ) Laser resurfacing of the scar
D ) Revision of the scar in 4 months
The correct response is Option A.
The patient described displays a good result after unilateral cleft lip repair. However, the mother is overly concerned about the appearance of the scar, and she needs to be reassured about the result. She should be reeducated concerning good scar care, which includes use of a strong sunblock, and massage of the scar. Even if the scar were a bad one, the best option at this early stage would be optimal scar care. Revision of scars in children earlier than 12 months is generally not advisable, as they typically continue to improve during this time. Laser resurfacing has not been shown to be an effective early modifier of scar outcomes.
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 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 part of the Furlow double opposing Z plasty and the intravelar veloplasty operations occurs in both operations?
A) Closure of the nasal layer
B) Dissection of hard palate flaps
C) Posterior repositioning of the muscle
D) Mucoperiosteal pushback
The correct answer is posterior repositioning of the muscles of the soft palate. Both techniques attempt to place the muscle in an anatomically correct position.
What is the most likely complication following palatoplasty?
A) Fistula formation
B) Velopharyngeal insufficiency
C) Sleep apnea
D) Maxillary growth restriction
The correct answer is maxillary growth restriction, which will likely occur in most patients to some degree after palate repair. Fistula rates are generally in the 10- 20 percent range. Velopharyngeal insufficiency is in the range of 20 percent, and sleep apnea occurs in a small group of patients that is ill-defined.
A 5-year-old boy who underwent repair of cleft palate via double opposing Z-plasty four years ago has hypernasality indicative of velopharyngeal insufficiency. Direct nasendoscopy shows a coronal closure pattern of the velopharyngeal port and little or no motion of the lateral pharyngeal wall. Which of the following surgical procedures is most appropriate for correction of the velopharyngeal insufficiency?
(A) Augmentation of the posterior pharynx
(B) Inferiorly based posterior pharyngeal flap
(C) Sphincter pharyngoplasty
(D) Superiorly based posterior pharyngeal flap
(E) V-Y pushback palatoplasty
The correct response is Option C.
Velopharyngeal competence results from sufficient apposition of the velar mucosa against the posterior pharyngeal wall and from motion of the lateral pharyngeal wall that causes sphincteric closure of the velopharyngeal port. Many patients with velopharyngeal insufficiency after cleft palate repair have a shortened, scarred velum, resulting in a deficiency in the anterior-posterior coronal closure pattern. Other patients, such as those with velocardiofacial syndrome, have generalized pharyngeal dysfunction with poor lateral pharyngeal wall motion, which contributes to a large central gap that leads to velopharyngeal incompetence.
To correct velopharyngeal insufficiency in this patient, a sphincter pharyngoplasty is most appropriate. In this procedure, the posterior tonsillar pillars (palatopharyngeus muscles) are bilaterally dissected from the tonsillar fossae and rotated 90 degrees medially. Then they are affixed in an overlapping fashion against the posterior pharyngeal wall. Because the palatopharyngeus muscles are a continuation of the soft palate and lateral pharyngeal walls, this procedure narrows the entire pharyngeal port in a sphincteric fashion and augments the posterior pharyngeal wall.
In a patient with little or no motion of the lateral pharyngeal wall, augmentation of the posterior pharynx is not appropriate. Although it may decrease the anterior-posterior coronal deficiency, it does nothing to treat the poor lateral wall motion. In addition, augmentation of the posterior pharynx has been attempted with multiple materials (including fat, Teflon, and silicone) in the past, without success. Today, the procedure has all but been abandoned.
Likewise, a posterior pharyngeal flap (whether inferiorly based or superiorly based) is not appropriate because it may not completely correct the hypernasality associated with velopharyngeal insufficiency. This is true because it does not allow the lateral pharyngeal walls to move medially and seal off the lateral ports between the pharyngeal flap and lateral pharyngeal walls.
V-Y pushback palatoplasty would not adequately correct the problem.
Which of the following muscles is/are NOT involved in normal velopharyngeal closure?
(A) Levator palatini
(B) Palatopharyngeus
(C) Superior pharyngeal constrictors
(D) Tensor veli palatini
(E) Uvulus
The correct response is Option D.
Muscles involved in velopharyngeal closure include each of those mentioned above except for the tensor veli palatini. Normal velopharyngeal closure is crucial for production of intelligible speech; any abnormalities in this mechanism can result in hypernasality, nasal emissions, imprecise production of consonants, decreased speech volume, and/or shortness of phrases.
The levator palatini muscles are of primary importance in pharyngeal closure. These muscles join in the midline to form a sling in the normal palate, inserting into the palatal aponeurosis. Their mechanism of action is to pull the middle third of the soft palate superiorly and posteriorly, resulting in contact with the pharyngeal walls.
In contrast, patients with cleft palate have abnormal insertion of the levator palatini on the posterior aspect of the hard palate. Cleft palate repair recreates the “normal” muscle sling by reorienting the muscles horizontally through a procedure known as intravelar veloplasty.
If normal pharyngeal closure is not achieved following cleft palate surgery, additional procedures may be considered to allow for a more complete velopharyngeal closure. Sphincter pharyngoplasty or reconstruction with pharyngeal flaps is most frequently performed.
The palatopharyngeus muscles pull the soft palate posteriorly, while the superior pharyngeal constrictors move the lateral and posterior pharyngeal walls. The uvulus muscle enhances velopharyngeal closure by thickening the uvula.
Although the tensor veli palatini attaches to the eustachian tubes, acts to tense the soft palate, and is important for eustachian tube function, it does not provide an important contribution to velopharyngeal closure.
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.
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 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 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.
Which of the following muscles is used to construct the sphincter during a sphincter pharyngoplasty for the treatment of velopharyngeal insufficiency?
A ) Levator veli palatini
B ) Musculus uvulae
C ) Palatopharyngeus
D ) Superior constrictor
E ) Tensor veli palatini
The correct response is Option C.
Routinely, postoperative velopharyngeal insufficiency is treated with either a posterior pharyngeal flap or a sphincter pharyngoplasty. A sphincter pharyngoplasty is performed by taking the posterior tonsillar pillar, containing the palatopharyngeus muscle, and elevating it inferiorly to superiorly. The elevated posterior tonsillar pillar, pedicled superiorly, is rotated 90 degrees medially, positioned side by side, and sewn into an incision made horizontally on the posterior pharyngeal wall at the level of the adenoid pad.
The levator veli palatini muscle, in the normal palate, rests horizontally (coronally) within the middle third of the velum (soft palate) and functions as the motor of the velum. This muscle pulls the soft palate posteriorly and superiorly, allowing for apposition of the free edge of the velum against the posterior pharyngeal wall (velopharyngeal competence).
The musculi uvulae arise as paired slips from the palatine aponeurosis and course sagittally in the velar midline, ending in the uvula. The musculi uvulae act as a flexible beam, providing a stiffness-modifying mechanism for the velum.
The superior pharyngeal constrictor arises from the hamulus and course sagittally along the lateral pharyngeal walls to decussate in the midline of the posterior pharyngeal wall. The superior pharyngeal constrictor is the muscle from the posterior pharyngeal wall that is utilized in the posterior pharyngeal flap.
The tensor veli palatini muscle arises from the skull base and courses inferiorly and medially around the hook of the hamulus and into the palate as the tensor aponeurosis. It joins the velum (soft palate) to the hard palate.
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.
Which of the following is the most appropriate management for a submucous cleft palate?
A) No speech evaluation, repair at age 1 year
B) Speech evaluation, repair at age 1 year
C) No speech evaluation, repair at age 5 years
D) Speech evaluation at age 5 years
E) No repair
The correct answer is (D). Because only 10% of patients with a submucous cleft palate have poor speech, don’t correct the cleft unless they have poor speech documented when they are approximately 5 years old.
Which of the following in cleft palate management is most closely associated with maxillary growth restriction?
A) Earlier age at palate repair
B) Placement of pharyngeal flap
C) Delayed palate repair
D) Sphincterpharyngoplasty
The earlier the palate is repaired, the more likely there will be maxillary growth inhibition. A pharyngeal flap and a sphincterpharyngoplasty are techniques to treat velopharyngeal insufficiency.
In neonates with submucous cleft palate, the zona pellucida results from abnormal morphology of which of the following muscles?
(A) Levator veli palatini
(B) Muscularis uvulae
(C) Palatopharyngeus
(D) Pharyngeal constrictor
(E) Tensor veli palatini
The correct response is Option A.
The zona pellucida is formed by parallel bulges of anterior-posterior muscle on either side of the soft palate in the midline. Between these paired and cleft levator veli palatini muscles is a bluish two-layered mucosal bridge, which is the submucous cleft palate. With abnormal morphology, the levator veli palatini muscles insert into the posterior edge of the hard palate, causing Veau’s cleft muscle as is seen in the typical cleft palate. The levator veli palatini muscles originate from the petrous portion of the temporal bone and the medial surface of the auditory tube and insert in the middle soft palate. Inferiorly, they form a V-shaped sling that suspends the velum from the base of the cranium and pull the soft palate up and back.
The other muscles listed are unrelated to submucous cleft palate. The muscularis uvulae runs longitudinally along the medial palate from the tensor aponeurosis to the uvula. During speech, it functions as a flexible beam, lifting and bending the palate back and modifying the stiffness of the palate.
The palatopharyngeus muscle originates from the palatal aponeurosis and runs to the posterior pharyngeal pillar. It functions to depress the palate and displace it backward. It is used to perform the sphincter pharyngoplasty.
The superior pharyngeal constrictor muscle is a continuation of the posterior buccinator and tongue. This muscle curves back and up, ending in a tendinous median raphe attached to the occipital bone. It serves to pull the lateral and posterior pharyngeal walls medially, narrowing the pharynx. In patients with velopharyngeal insufficiency, a horizontal bend in this muscle elevates the mucosa to form ridge at the junction of the nasopharynx and oropharynx. In patients with cleft palate, this hypertrophied area is called Passavant ridge.
The tensor veli palatini muscle originates from the scaphoid fossa, medial pterygoid plate, and spine of the sphenoid. It courses inferiorly around the hamulus to form the palatal aponeurosis. It acts to tighten the palate so the tongue has a firm surface against which to create a bolus of food. It is primarily involved in swallowing, rather than in speaking.
Which of the following muscles are disrupted to the greatest degree in a cleft palate?
A) Uvulus
B) Stylopharyngeus
C) Middle pharyngeal constrictor
D) Superior pharyngeal constrictor
E) Genioglossus
The correct answer is option A.
Among the muscles listed above, only the uvulus is directly involved in a cleft palate.
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%.
The firstborn child of a Caucasian couple with no abnormalities has bilateral cleft lip and palate. Which of the following percentages best represents the possibility that this couple’s next child will have cleft lip, with or without cleft palate?
(A) 2%
(B) 4%
(C) 10%
(D) 16%
(E) 32%
The correct response is Option B.
The correct answer for the sibling of a child with bilateral cleft lip and palate is approximately 4%, depending on the reference. Regardless, the risk decreases if there is no cleft palate in the proband and/or if the condition is unilateral. The incidence of cleft lip with or without cleft palate for the sibling of a child with bilateral cleft lip but no cleft palate is 6.7%, for the sibling of a child with unilateral cleft lip and palate is 4.9%, and for the sibling of a child with unilateral cleft lip but no cleft palate is 4.0%. If there are two affected children with cleft lip with or without cleft palate, the risk for a third child would be at least 9%.
The overall risk of cleft lip with or without cleft palate in a Caucasian population is 1:1000. In a first-degree relative, that risk is multiplied by 40; in a second-degree relative, the risk is multiplied by 7; and in a third-degree relative, the risk is multiplied by 3. However, the risk is increased when more than one relative is affected, if the family member has a severe form of the disorder, if the family member is of the sex least likely to be affected, or if the parents are consanguineous. For one affected parent, the risk of having one child with cleft lip with or without cleft palate is 4%, and the risk for a second child increases to 17%.
A 6-year-old boy with velopharyngeal insufficiency is brought to the office for sphincter pharyngoplasty. History includes repair of a cleft palate as an infant. Innervation of the muscle used to create the sphincter arises from which of the following?
A) Cranial part of the accessory (XI) nerve
B) Glossopharyngeal (IX) nerve
C) Greater and lesser palatine branches of the pterygopalatine ganglion
D) Hypoglossal (XII) nerve
E) Medial pterygoid nerve
The correct response is Option A.
Sphincter pharyngoplasty for correction of velopharyngeal insufficiency is performed by elevation of myomucosal flaps from the posterior tonsillar pillar, which involves the palatopharyngeus muscle. The palatopharyngeus muscle is supplied by the cranial part of accessory (XI) nerve through the pharyngeal branch of vagus (X) nerve via the pharyngeal plexus. The medial pterygoid nerve innervates the tensor veli palatini. The greater and lesser palatine nerves are branches of the pterygopalatine ganglion which provide sensory innervation to the palate. The hypoglossal (XII) nerve innervates the lingual muscles, with the exception of the palatoglossus, which is supplied by pharyngeal branch of the vagus (X) nerve, via the pharyngeal plexus. A photograph is shown.
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.
For the preoperatively marked rotation-advancement repair shown, which is the rotation flap?
A) A
B) B
C) C
D) D
E) E
The A flap is marked on the medial lip element and is the rotation flap. This flap is designed as a gentle curve from the height of Cupid’s bow on the cleft side of the medial cleft element to the junction of the philtrum and columella. This incision approaches the normal philtral column as much as needed to de-rotate and level Cupid’s bow. If more rotation is needed to level Cupid’s bow, a back-cut is made, but the normal philtral column is not violated. This incision is used in philtral subunit reconstruction and leveling Cupid’s bow. The B flap is made on the non-cleft, lateral lip element and is the advancement flap. This flap is the “wedge” that is placed in the defect created above the rotation flap as the medial lip element is de-rotated to level Cupid’s bow. This flap is marked by first finding the point on the white roll that will correspond to the height of Cupid’s bow on the medial cleft element. This point is found where the fullness of the lateral lip element begins to fade. A line is drawn from this point cephalad and is the same length as the normal philtral column as well as the marked edge of the A (rotation) flap. The C flap or columellar flap is made from the tissue medial to the A-flap incision. This small flap is rotated into the columella, lengthening the cleft side of the columella. It is also sewn to the D flap (alar base flap). The D flap or alar base flap is cut along the alar-facial groove, and this incision corresponds to the cephalad portion of the edge of the B flap (advancement flap). This alar groove incision is made as small as possible and long enough to allow differential rotation of the D flap and the B flap. The L flap is the leading edge of the lateral lip that can be used to fill the nasal lining defect. The M flap is the medially based flap from the rotation.
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 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.
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.
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.
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.
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
cORRECT ANSWER IS OPTION c.
Nasoalveolar molding (NAM) is the fabrication and adjustment of a custom intraoral appliance that narrows the cleft, aligns the cleft segments, shapes existing nasal structures, and expands deficient nasal tissues. Bilateral cleft lip and palate is associated with a nasal deformity that is defined by a short columella. The premaxilla is typically protuberant if the cleft is complete. NAM allows alignment of the premaxilla with the lateral alveolar segments. Once this is accomplished, nasal stents are placed to lengthen the columella. The alveolar clefts are aligned and narrowed. Generally, the septum is in the midline and does not require any lengthening. The upper cleft lip does not require expansion; rather, it requires layered repair with good muscular approximation at the time of the primary lip and nasal repair.
