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Questions and Answers

Which of the following is the best option for improvement of speech in this patient?

  • Posterior pharyngeal flap
  • Tonsillectomy
  • Continued speech therapy
  • Use of a palatal lift device (correct)
  • Furlow palatoplasty

    • Which of the following layers is included with a posterior pharyngeal flap?

  • Prevertebral fascia
  • Investing layer of the deep cervical fascia
  • Palatopharyngeus muscle
  • Superior pharyngeal constrictor muscle (correct)

    • For patients with velopharyngeal incompetence, which of the following muscles is used to perform sphincter pharyngoplasty?

  • Tensor veli palatini
  • Levator veli palatini
  • Palatopharyngeus (correct)
  • Palatoglossus
  • Musculus uvulae

    • A 2-year-old has a midline cleft of the upper lip. This cleft results from failure of fusion of which of the following embryologic structures?

    <p>Medial nasal prominence and maxillary prominence</p> Signup and view all the answers

    Given her diagnosis, which of the following is the most likely finding in a 3-year-old girl with a cleft lip and palate?

    <p>Tooth agenesis</p> Signup and view all the answers

    Which of the following is the best treatment option for a 17-year-old girl with a left unilateral cleft lip and palate and class III malocclusion?

    <p>Two-piece Le Fort I osteotomy with closure of the alveolar gap</p> Signup and view all the answers

    Which of the following represents the percentage of patients with isolated cleft palate who are most likely to have middle ear effusion?

    <p>90%</p> Signup and view all the answers

    Which postoperative risk is higher in a patient with 22q11.2 deletion syndrome undergoing cleft palate repair compared with nonsyndromic patients?

    <p>Velopharyngeal insufficiency</p> Signup and view all the answers

    Which of the following is true about cleft lip with or without palate when compared with cleft palate only?

    <p>Cleft lip/palate is less likely to be associated with a syndrome</p> Signup and view all the answers

    What is the primary disadvantage of iliac crest bone grafting?

    <p>Postoperative pain at the donor site</p> Signup and view all the answers

    The L flap used in the rotation-advancement technique of cleft lip repair is implemented to accomplish which of the following?

    <p>Provide lining in the gingivobuccal sulcus</p> Signup and view all the answers

    Completion of facial growth is best determined by which of the following?

    <p>Serial cephalometric x-ray study</p> Signup and view all the answers

    What is the most appropriate management for a 6-year-old boy with hypernasality after cleft palate repair?

    <p>Pharyngeal flap</p> Signup and view all the answers

    Innervation of the muscle used to create the sphincter for sphincter pharyngoplasty arises from which nerve?

    <p>Cranial part of the accessory (XI) nerve</p> Signup and view all the answers

    Velar competence after treatment of velopharyngeal insufficiency with Furlow double-opposing Z-plasty is most strongly correlated with which factor?

    <p>Preoperative closure gap</p> Signup and view all the answers

    Development of which of the following tooth buds is most likely to be impaired in a patient with complete unilateral cleft of the lip and palate?

    <p>Lateral incisor</p> Signup and view all the answers

    Failure of fusion of which of the following results in the formation of a cleft of the lip?

    <p>Medial nasal and maxillary prominences during the first 6 to 8 weeks of gestation</p> Signup and view all the answers

    Which of the following is the most appropriate management for a 4-year-old girl with persistent nasal air escape following cleft palate repair?

    <p>Sphincter pharyngoplasty</p> Signup and view all the answers

    What is the risk of a 2-year-old boy with a cleft of lip and palate having a child with cleft of lip?

    <p>5%</p> Signup and view all the answers

    Timing for initiation of palatal expansion should be based on which of the following?

    <p>Canine eruption</p> Signup and view all the answers

    In addition to evaluation of speech, which diagnostic tool is the most appropriate for assessing a patient with possible velopharyngeal insufficiency?

    <p>Nasopharyngoscopy</p> Signup and view all the answers

    What is the most appropriate management for a 4-year-old girl with velocardiofacial syndrome and hypernasal speech?

    <p>Reconstruction with a superiorly based pharyngeal flap</p> Signup and view all the answers

    What genetic condition is associated with lower lip pits and cleft lip and/or palate?

    <p>van der Woude syndrome</p> Signup and view all the answers

    Which of the following is the most appropriate management at this time for the patient concerned about the scar after unilateral cleft lip repair?

    <p>Continued optimal scar management</p> Signup and view all the answers

    What is the best representation of the likelihood that the patient's second child will be born with a cleft lip and palate?

    <p>14%</p> Signup and view all the answers

    Which of the following muscles is used to construct the sphincter during a sphincter pharyngoplasty for the treatment of velopharyngeal insufficiency?

    <p>Palatopharyngeus</p> Signup and view all the answers

    Repair of which of the following muscles is most likely to improve eustachian tube function during cleft palate reconstruction?

    <p>Levator veli palatini</p> Signup and view all the answers

    Which of the following structures arises from the lateral nasal processes during embryologic development?

    <p>Nasal ala</p> Signup and view all the answers

    Which of the following is the most appropriate Veau classification of a cleft that extends to the incisive foramen?

    <p>II</p> Signup and view all the answers

    What is a goal of the nasoalveolar molding seen in a 3-month-old infant with a bilateral cleft lip and palate?

    <p>Lengthening of the columella</p> Signup and view all the answers

    Blood supply to the tongue runs primarily in which of the following regions?

    <p>Ventral third</p> Signup and view all the answers

    Completion of facial growth is best determined by which method?

    <p>Serial cephalometric x-ray study</p> Signup and view all the answers

    What is the most important factor in determining the width of the flap in pharyngeal flap reconstruction?

    <p>Lateral pharyngeal wall motion</p> Signup and view all the answers

    Which of the following physical findings suggests a submucous cleft palate?

    <p>All of the above</p> Signup and view all the answers

    What deviation of the bony septum is commonly found in unilateral cleft lip and palate patients?

    <p>Deviation to the cleft side and decreased projection of the pyriform aperture</p> Signup and view all the answers

    What is the L flap used for in the context of cleft lip repair?

    <p>To line the lateral nasal vault</p> Signup and view all the answers

    What is the most likely genetic defect in the patient with cleft lip and lower lip pits?

    <p>22q11.2</p> Signup and view all the answers

    Which muscle is most likely responsible for tympanogram showing bilateral noncompliance in a 3-year-old boy with unilateral cleft lip and palate?

    <p>Tensor veli palatini</p> Signup and view all the answers

    In a patient with velopharyngeal insufficiency after palatoplasty, which muscle should the surgical procedure target?

    <p>Levator veli palatini</p> Signup and view all the answers

    What is the most likely facial change after a Le Fort I advancement procedure?

    <p>Increased alar base</p> Signup and view all the answers

    After age 18 months, what is a potential negative outcome of late palate repair?

    <p>Higher fistula rates</p> Signup and view all the answers

    What is the best dental reference for determining the timing for secondary alveolar bone grafting?

    <p>Crowning of the permanent canine</p> Signup and view all the answers

    What is the most appropriate treatment for a child with persistent velopharyngeal insufficiency?

    <p>Fabrication of a custom palatal elevator</p> Signup and view all the answers

    Which stigmata is most common after a rotation-advancement repair of a unilateral cleft lip?

    <p>Blunting of Cupid's bow</p> Signup and view all the answers

    What is the best treatment option for a child with obstructive sleep apnea and severe midface hypoplasia?

    <p>Continuous positive airway pressure (CPAP)</p> Signup and view all the answers

    What is the most appropriate initial diagnostic tool for a child with velopharyngeal insufficiency?

    <p>Video nasal endoscopy</p> Signup and view all the answers

    What is the primary goal of using the nasoalveolar molding (NAM) appliance?

    <p>Repositioning and approximation of the alveolar segments and nasal cartilage</p> Signup and view all the answers

    What is the most appropriate next step for managing a child with hyponasal speech and a history of cleft lip and palate?

    <p>Polysomnography</p> Signup and view all the answers

    What technique can help improve hypernasal speech in a child with a prior cleft palate repair?

    <p>Conversion Furlow palatoplasty</p> Signup and view all the answers

    Study Notes

    Cleft Lip and Palate Management

    • Unilateral cleft lip repair typically yields good aesthetic results, even if scarring is present.
    • Optimal scar management includes strong sunblock and massage, particularly before revising scars at 12 months of age.
    • Laser resurfacing is not proven effective for early scar modification.

    Genetic Risks for Cleft Lip and Palate

    • A parent with one affected child has a 14% risk of future offspring developing cleft lip and palate.
    • If both parents are unaffected, but the first child has a unilateral defect, the risk for subsequent children is 2.7%.
    • The risk increases to 5.4% if the first child has a bilateral defect, and to 10% if two affected children are born.

    Anatomy and Surgical Techniques

    • Postoperative velopharyngeal insufficiency often treated with posterior pharyngeal flap or sphincter pharyngoplasty.
    • Sphincter pharyngoplasty uses the palatopharyngeus muscle, elevating the posterior tonsillar pillar to improve closure.
    • The levator veli palatini muscle is crucial for velopharyngeal competence and is realigned in cleft palate repairs.

    Embryological Development

    • Nasal structures form around the sixth week of gestation and develop from medial and lateral nasal prominences.
    • Failure in fusion can lead to cleft nasal deformities; lateral nasal processes contribute to the formation of nasal alae.

    Classification of Cleft Palate

    • Veau classification divides cleft palates:
      • Class I: Incomplete soft palate cleft.
      • Class II: Complete soft and hard palate cleft, limited to the secondary palate.
      • Class III: Complete unilateral cleft lip and palate.
      • Class IV: Complete bilateral cleft lip and palate.

    Nasoalveolar Molding (NAM)

    • NAM is a treatment for bilateral cleft lip and palate, aligning and reshaping cleft segments and nasal structures.
    • Goals include narrowing alveolar clefts and lengthening the columella, optimizing nasolabial appearance before surgical repair.

    Important Blood Supply Considerations

    • Major vascular supply to the tongue is predominantly from the lingual arteries located in the ventral third.
    • Repair techniques involving the tongue flap necessitate knowledge of the vascular layout for successful outcomes.

    Skeletal Development Assessments

    • Serial cephalometric x-ray studies are crucial for determining maxillofacial growth completion in adolescents, more reliable than chronological age or dental development.
    • Minimal changes in growth velocity signal skeletal maturity, guiding future surgical interventions.

    Velopharyngeal Insufficiency Reconstruction

    • Pharyngeal flap technique effectively addresses velopharyngeal insufficiency by enhancing closure during speech, with considerations for flap width and donor site management.
    • Conditions like obstructive sleep apnea may arise with overly wide flaps, requiring careful planning.

    Submucous Cleft Palate

    • Features like bifid uvula and hard palate notching indicate submucous cleft palate presence.
    • While many cases remain asymptomatic, management options like speech therapy or surgical intervention depend on the degree of velopharyngeal insufficiency.

    Anatomical Deviations in Unilateral Clefts

    • Typical anomalies include deviation of the anterior nasal spine towards the noncleft side with decreased projection on the cleft side.
    • Completion of craniofacial growth and surgical timing are essential to manage these skeletal issues.### Cleft Palate and Velopharyngeal Insufficiency Management
    • An obturator aids in palatal closure when tissue is insufficient, while intravelar veloplasty addresses gaps in unrepaired clefts or submucous clefts.
    • A 6-year-old boy with hypernasality and a posterior gap of 10 mm requires intravelar veloplasty (option A) as the appropriate intervention.

    Surgical Techniques and Innervation

    • Sphincter pharyngoplasty corrects velopharyngeal insufficiency (VPI) by elevating myomucosal flaps from the posterior tonsillar pillar, utilizing the palatopharyngeus muscle, supplied by the cranial part of accessory (XI) nerve and pharyngeal branch of vagus (X) nerve.
    • The medial pterygoid nerve innervates the tensor veli palatini, while greater and lesser palatine nerves provide sensory innervation to the palate.

    Furlow Double-Opposing Z-Plasty

    • Effective for treating velopharyngeal insufficiency, particularly in patients with submucous cleft palate or post-palatoplasty.
    • Smaller preoperative velopharyngeal gaps correlate better with postoperative competence.
    • The procedure's success is less favorable in older children and those with overt clefts or syndromes such as velocardiofacial syndrome.

    Dental Impacts of Cleft Palate

    • Cleft conditions can lead to dental abnormalities, mainly affecting the upper lateral incisor tooth bud among both deciduous and permanent teeth.

    Genetic Factors and Cleft Risks

    • Cleft lip and palate occur in 1.7 of every 1000 live births, influenced by genetic and environmental factors.
    • Risk of recurrence in families varies; males have a 4.7% and females a 3.6% chance of having a child with a cleft.

    Palatal Expansion Timing

    • Alveolar bone grafting timing is crucial and ideally performed after incisor eruption but before canine eruption.

    Velopharyngeal Insufficiency Diagnosis

    • VPI is characterized by inability to close the velopharyngeal sphincter, leading to speech intelligibility issues due to nasal air escape; evaluation includes subjective speech assessments and nasopharyngoscopy.

    Subsequent Surgical Options for VPI

    • Patients with little velar motion may require pharyngeal flap surgical options; those with better lateral wall motion can benefit from sphincter pharyngoplasty.

    Van der Woude Syndrome

    • An autosomal dominant condition characterized by lower lip pits or mounds and cleft lip/palate, linked to the IRF6 gene mutation, which is vital for clinical diagnosis.

    Eustachian Tube Dysfunction and Muscle Involvement

    • Flat tympanograms in children with cleft palate indicate eustachian tube dysfunction, often due to abnormal tensor veli palatini insertion affecting drainage.

    Palatopharyngeus Muscle

    • The palatopharyngeus muscle plays a pivotal role in velopharyngeal function, aiding in the separation of the oropharynx from the nasopharynx during swallowing.

    Effects of Le Fort I Advancement

    • Procedures can significantly alter facial aesthetics, affecting midfacial projection, lip vermilion fullness, and nasolabial angles, necessitating preoperative counseling to manage patient expectations.### Cleft Lip and Palate Management
    • Repair of cleft lip and palate in infancy is crucial for speech functionality and aesthetic considerations.
    • Typically, palate repair is prioritized over lip revision due to potential complications associated with late palate repair.
    • Late palate repairs (after 18 months) are linked to worse speech outcomes and increased rates of complications like fistulas.

    Surgical Options and Outcomes

    • Assessment of children post-cleft repair often leads to decisions on palate repair vs. lip revision.
    • Secondary alveolar bone grafting is essential during mixed dentition to stabilize the alveolus for permanent tooth health.
    • Optimal timing for bone grafting is ideally before complete eruption of the permanent canine to ensure adequate bone support.

    Speach and Velopharyngeal Insufficiency

    • Persistent hypernasality post-repair can occur due to hypotonia of speech muscles; surgical corrections like sphincter pharyngoplasty may be required.
    • Initial management of velopharyngeal insufficiency may include the use of a palatal elevator to enhance speech outcomes.
    • Video nasal endoscopy is a valuable diagnostic tool for evaluating velopharyngeal closure patterns and planning further surgeries.

    Nasoalveolar Molding (NAM)

    • NAM primarily aims to reposition and approximate alveolar segments; it plays a significant role in improving nasal cartilage shape.
    • Financially advantageous and labor-intensive for families, NAM differs from other interventions like lip adhesion, which may not have the same level of impact.

    Hyponasal Speech

    • Hyponasal speech indicates insufficient air flow through the velopharyngeal sphincter, potentially due to an exuberant posterior pharyngeal flap.
    • Evaluation for obstructive sleep apnea (OSA) is essential in patients with speech anomalies, especially if no understanding difficulties are reported.

    Cleft-associated Dental Anomalies

    • Children with cleft lip and palate exhibit higher prevalence rates of dental anomalies, primarily tooth agenesis.
    • Missing lateral incisors are common on the cleft side, necessitating surgical approaches to address both malocclusion and aesthetic restoration.

    Embryologic Origins of Clefts

    • Median cleft lips arise from the failure of fusion of medial nasal prominence.
    • Understanding the embryologic causes of various clefts can inform surgical approaches and expected outcomes.

    Patient Management and Surgical Challenges

    • Surgical management involves addressing not just the cleft defect but also ensuring proper dental and skeletal alignment.
    • A two-piece Le Fort I advancement is often seen as a promising solution for older patients who present with significant malocclusion and cleft-related deficiencies.

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