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Cleft Palate

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  • Interventi di chirurgia ortognatica: http://www.giuseppespinelli.it/chirurgia-estetica-maxillo-facciale/chirurgia-ortognatica.html
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  • 1. The Cleft Palate
  • 2. Case Study
    • Full term infant, NSVD
    • Respiratory Distress at birth
    • Numerous O2 desaturations
    • Unable to feed
  • 3. Case Study
    • Cleft palate
  • 4. Incidence
    • Cleft lip
    • 1/1000 live births
    • Cleft palate
    • 1/2000 live births
  • 5. Classification - Veau
    • Clefts of soft palate only
    • Clefts of soft and hard palate reaching anteriorly to incisive foramen
    • Complete unilateral alveolar clefts - generally involve the lip as well
    • Bilateral alveolar clefts - associated with bilateral clefts of the lip
  • 6. Classification
    • A - Incomplete cleft of the secondary palate
    • B - Complete cleft of the secondary palate
    • C- Incomplete cleft of the primary and secondary palates
    • D - Unilateral complete cleft of the primary and secondary palates
    • E - Bilateral complete cleft of the primary and secondary palates
  • 7. Case Study
    • Micrognathia
  • 8. Case Study
    • Glossoptosis
    • Increase in frequency of O2 desaturations that improve when neonate is placed in prone position
  • 9. Timeline of Care
    • Neonatal to one month of age
    • Refer to cleft palate team
    • Medical diagnosis
    • Genetic counseling
    • Address psychosocial issues
    • Airway management
    • Provide feeding instructions
    • Monitor growth
    • Hearing screening
  • 10. The NICU team consults ENT…
  • 11. What might your flexible fiberoptic laryngoscopy show?
  • 12. Doctor, what is in your differential diagnosis?
    • Environmental
    • Alcohol
    • Tobacco smoke
    • Phenytoin
    • Retinoic acid
    • Folate Deficiency
  • 13. Doctor, what is in your differential diagnosis? Syndromes
    • van der Woude syndrome - Autosomal dominant disorder is on chromosome
  • 14. Syndromes
    • Robin Sequence
    • Micrognathia
    • Glossoptosis
    • Cleft palate
    • Associated abnormalities - 52% to 83%
  • 15. Syndromes
    • Velocardiofacial syndrome
    • Overlaps with DiGeorge syndrome and conotruncal anomaly face syndrome
    • CATCH 22: cardiac anomalies (usually conotruncal), a bnormal facies, t hymic disorders, c left palate, and h ypocalcemia caused a deletion of chromosome 22 at the q11.2 region.
  • 16. Syndromes
    • Stickler syndrome
    • Most common syndrome found in children with Robin Sequence - 14% to 34% of cases
    • Sensorineural hearing loss
    • Myopia - Risk of retinal detachment and early cataracts
    • Hypermobilityof joints
    • Stiffness and premature osteoarthritis
  • 17. Airway management
    • Prone positioning, which allows the tongue to fall away from the posterior pharyngeal wall.
    • Gavage feeding may be needed in such cases
    • Glossopexy or lip-tongue adhesion in infants
    • Tracheotomy
    • Mandibular distraction osteogenesis
  • 18. Seibert’s Technique Lip Adhesion
    • Medially and laterally based rectangular flaps from the margin of the cleft
    • Tension-bearing suture through the membranous septum.
    • Unilateral lip adhesion: A, landmarks and incisions; B, flaps elevated and undermined; C, placement of 3-0 nylon retention suture; D, final suturing.
  • 19. Airway Management
    • One hundred seven patients with Pierre Robin Sequence
    • 74 (69.2%) - positioning alone
    • 29 - tongue lip adhesion
      • Airway obstruction relieved in 20 (83.3%)
    • 10 - Tracheostomy
      • Including six (20.7%) who initially underwent a TLA
  • 20. Timeline One to Four months
    • Monitor feeding and growth
    • Monitor ears and hearing
    • Repair cleft lip with possible PE tube placement
  • 21. Incidence of Eustachian Tube Dysfunction
    • Middle ear disease - 22% to 88%
    • Conductive hearing loss and chronic suppurative otitis media may result
    • Repeated tympanostomy tube placement
  • 22. Why do cleft kids have eustachian tube dysfunction?
    • Paratubal cartilage abnormalities
    • Hypoplasia of the lateral cartilage relative to the medial cartilage
    • Abnormal curvature of the eustachian tube lumen
    • Cephalometric data - width and angulation of the skull base with respect to the eustachian tube are different
    • Abnormal insertions of the tensor and levator veli palatini muscles into the cartilages and skull base
    • Palatal muscle dysplasia
  • 23. How do you manage ear disease?
    • 96% of children with cleft palate required tympanostomy tube placement
    • 50% of these children required repeat tympanostomy tube placement.
    • Frequency of otitis media decreases as the child with CP ages
    • Audiology and tympanometry as well as exams / clinical history
    • First set are frequently placed during cleft lip or cleft palate repair
  • 24. Latham Appliance
  • 25. Cleft Lip Repair Rule of 10’s
    • Surgery > 10 weeks old
    • Weight > 10 pounds
    • Hemoglobin level > 10 g/dL
  • 26. Cleft Lip Repair Goals
    • Bridge the cleft
    • Create a complete muscular sling around the entire circumference of the oral cavity
    • Approximate cleft edges
    • Maintain Cupid's bow and philtral dimple
    • Align vermillion border
    • Create an intact nasal floor and sill
    • Produce symmetry of the alar base and columella
    • Reconstituting the circumferential integrity of the orbicularis oris muscle
  • 27. Cleft Lip Repair - Types
    • Millard Repair - Rotation advancement technique
    • Randall - Graham - Triangular flap interposition
    • Rose - Thompson - Straight line repair, Risk of vertical contracture
  • 28. Millard Repair
    • Downward and lateral rotation of the medial segment of the cleft lip combined with the medial advancement of the lateral cleft segment into the defect
    • Bridges the gap
    • Maintains the rotation flap in position
    • Maintains the amount of vertical height gained by the rotation flap and back cut
  • 29. Timeline Five to Fifteen Months
    • Monitor feeding, growth, and development
    • Repair cleft palate
    • Monitor ears and hearing
    • Instructions in oral hygiene
  • 30. Cleft Palate Repair - Timing
    • Dorf and Curtin
      • 10% occurrence of articulation errors when palatoplasty was completed by 1 year
      • 86% incidence of articulation errors when repair was complete after 1 year
    • Haapanen and Rantala - Significantly fewer children in the groups repaired before 18 months had hypernasal speech, articulation errors, or required secondary surgery to correct speech
  • 31. Cleft Palate Repair
    • V-Y Pushback
    • Two Flap Palatoplasty
    • Four Flap Palatoplasty
    • Schweckendick’s Primary Veloplasty
    • Furlow Palatoplasty
  • 32. Cleft Palate Repair V-Y Pushback
    • Two uni-pedicled flaps (greater palatine artery) and one or two anteriorly based pedicled flaps
    • Posterior flaps rotated in a V-Y advancement technique - increasing the length of the palate
    • Nasal mucosa not closed
    • Improved speech results compared with bipedicled techniques
    • Indicated for incomplete clefts
  • 33. Cleft Palate Repair Schweckendick’s Primary Veloplasty
    • Incisions made in soft palate
    • Muscle bundles released from the posterior hard palate and rotated
    • Reconstruction of levator sling
    • Closure of mucosal layers separately
  • 34. Cleft Palate Repair Furlow Palatoplasty
    • Lengthens the soft palate
    • Reconstructs the muscle sling.
    • Also commonly used to correct velopharyngeal insufficiency in patients with submucous cleft palate
    • Speech outcomes are improved compared with other palatoplasty techniques.
  • 35. Cleft Palate Repair Complications
    • Oronasal fistula - 8.7% to 23%
      • Sites of fistulization are typically the anterior hard palate and the junction of the hard and soft palate.
    • Velopharyngeal insufficiency
  • 36. Timeline Two to Five years old
    • Monitor speech and language development
    • Manage velopharyngeal insufficiency
    • Monitor ears and hearing; ear tubes if indicated
    • Assess development and psychosocial needs
    • Consider lip/nose revision before school
  • 37. Timeline Six to Eleven years old
    • Monitor speech and language; manage velopharyngeal insufficiency
    • Orthodontic evaluation and treatment
    • Alveolar bone graft
    • Monitor school and psychosocial needs
  • 38. Timeline Twelve to Twenty One years old
    • Monitor school and psychosocial needs
    • Orthodontics and restorative dentistry
    • Genetic counseling
    • Rhinoplasty (if needed)
    • Orthognathic surgery (if needed)

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