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Clefts of the Lip,  Alveolus and Palate Michael E. Prater, MD Norman R. Friedman, MD
Overview <ul><ul><li>Introduction </li></ul></ul><ul><ul><li>Basic Science </li></ul></ul><ul><ul><li>Timetable of Events ...
Introduction <ul><li>A TEAM APPROACH IS REQUIRED </li></ul><ul><ul><ul><ul><li>pediatrician </li></ul></ul></ul></ul><ul><...
Introduction <ul><li>Most common congenital malformation of H and N (1:1000 in US; 1:600 in UK) </li></ul><ul><li>Second m...
Epidemiology <ul><li>Syndromic CLAP </li></ul><ul><ul><li>associated with more than 300 malformations </li></ul></ul><ul><...
Syndromic CLAP <ul><li>Single Gene Transmission </li></ul><ul><ul><li>trisomies 21, 13, 18 </li></ul></ul><ul><li>Teratoge...
Epidemiology:  continued <ul><li>Isolated cleft palate genetically distinct from isolated cleft lip or CLAP </li></ul><ul>...
Embryology <ul><li>Primary versus secondary palate </li></ul><ul><ul><li>divided by incisive foramen </li></ul></ul><ul><u...
Embryology:  continued <ul><li>Secondary palate </li></ul><ul><ul><li>medial ingrowth of lateral maxillae with midline fus...
Classification <ul><li>Veau Classification - 1931  </li></ul><ul><ul><li>Veau Class I:  isolated soft palate cleft </li></...
Classification; continued <ul><li>Complete Clefts </li></ul><ul><ul><li>absence of any connection with extension into nose...
Anatomy - Normal <ul><li>Lip:  “Cupid’s Bow” </li></ul><ul><li>Maxilla </li></ul><ul><ul><li>primary/secondary palates </l...
Anatomy: palatal muscles <ul><ul><ul><li>Superior constrictor </li></ul></ul></ul><ul><ul><ul><ul><ul><li>primary sphincte...
Cleft Anatomy <ul><li>Unilateral Cleft Lip and alveolus </li></ul><ul><ul><ul><li>lack of mesodermal proliferation  </li><...
Cleft Anatomy - The Nose <ul><li>Ipsilateral LLC  </li></ul><ul><ul><li>flattened </li></ul></ul><ul><ul><li>rotated downw...
Cleft Antatomy:  continued <ul><li>Bilateral Cleft Lip/Alveolus/nose </li></ul><ul><ul><li>duplication of unilateral defec...
Cleft Anatomy:  continued <ul><li>Clefts of the primary hard palate/alveolus </li></ul><ul><ul><li>cleft alveolus always a...
Cleft Anatomy:  continued <ul><li>Clefts of secondary palate </li></ul><ul><ul><ul><li>Failure of medial growth maxillae  ...
Multidisciplinary Approach <ul><li>These are not merely surgical problems </li></ul><ul><ul><li>Requires team approach thr...
The Neonatal Period <ul><li>Pediatrician: </li></ul><ul><ul><li>directs care </li></ul></ul><ul><ul><li>establishes feedin...
The Neonatal Period <ul><li>Presurgical Orthodontics (Baby Plates) </li></ul><ul><ul><ul><ul><li>Molds palate into more an...
The Neonatal Period <ul><li>Surgical Repair </li></ul><ul><ul><li>Cleft Lip </li></ul></ul><ul><ul><ul><li>In US - “the ru...
The Toddler Years <ul><li>Priority:  Speech </li></ul><ul><ul><li>“ Cleft errors of speech” in 30% </li></ul></ul><ul><ul>...
The Toddler Years <ul><li>Growth hormone deficiency  </li></ul><ul><ul><li>40 times more common in CLAP </li></ul></ul><ul...
The Grade School Years <ul><li>Three primary issues </li></ul><ul><ul><li>Orthodontics </li></ul></ul><ul><ul><ul><li>poor...
The Teenage Years <ul><ul><li>Midface retrusion </li></ul></ul><ul><ul><ul><li>etiology - ?early palatal repair </li></ul>...
Surgical Techniques <ul><li>Cleft Lip Repair </li></ul><ul><ul><li>unilateral </li></ul></ul><ul><ul><ul><li>rotation-adva...
Surgical Techniques <ul><li>Cleft Lip Repair </li></ul><ul><ul><li>bilateral </li></ul></ul><ul><ul><ul><li>bilateral rota...
Surgical Techniques <ul><li>Velopharyngeal Incompetnece </li></ul><ul><ul><li>superior based pharyngeal flap </li></ul></u...
Surgical Techniques <ul><li>Alveolar Bone Grafting </li></ul><ul><ul><li>iliac crest bone graft </li></ul></ul><ul><ul><li...
Surgical Techniques <ul><li>Midfacial Advancement </li></ul><ul><ul><li>LeForte osteotomies </li></ul></ul><ul><ul><ul><li...
Surgical Techniques <ul><li>Rhinoplasty </li></ul><ul><ul><li>standard techniques </li></ul></ul><ul><ul><ul><li>tip proje...
Controversies:  Otologic Disease <ul><li>>90% have COME </li></ul><ul><ul><ul><li>Robinson, et al </li></ul></ul></ul><ul>...
Controversies: Timing of Repair <ul><li>Early repair </li></ul><ul><ul><ul><li>Advantage:  improved speech </li></ul></ul>...
Controversies:  VPI <ul><li>Surgical Repair </li></ul><ul><ul><li>Reserved for failure of speech pathology </li></ul></ul>...
Controversies <ul><li>Presurgical Nasal Alveolar Molding </li></ul><ul><ul><li>molds palate, alveolus and nose </li></ul><...
Conclusion and Future Directions <ul><li>Multidisciplinary approach </li></ul><ul><li>Not merely a “surgical problem” </li...
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  1. 1. Clefts of the Lip, Alveolus and Palate Michael E. Prater, MD Norman R. Friedman, MD
  2. 2. Overview <ul><ul><li>Introduction </li></ul></ul><ul><ul><li>Basic Science </li></ul></ul><ul><ul><li>Timetable of Events </li></ul></ul><ul><ul><ul><ul><li>neonatal </li></ul></ul></ul></ul><ul><ul><ul><ul><li>toddler </li></ul></ul></ul></ul><ul><ul><ul><ul><li>gradeschool </li></ul></ul></ul></ul><ul><ul><ul><ul><li>teenage </li></ul></ul></ul></ul><ul><ul><li>Surgical Procedures </li></ul></ul><ul><ul><li>Conclusion/Future Directions </li></ul></ul>
  3. 3. Introduction <ul><li>A TEAM APPROACH IS REQUIRED </li></ul><ul><ul><ul><ul><li>pediatrician </li></ul></ul></ul></ul><ul><ul><ul><ul><li>surgeon </li></ul></ul></ul></ul><ul><ul><ul><ul><li>OMFS </li></ul></ul></ul></ul><ul><ul><ul><ul><li>dentist </li></ul></ul></ul></ul><ul><ul><ul><ul><li>ENT </li></ul></ul></ul></ul><ul><ul><ul><ul><li>psychiatrist </li></ul></ul></ul></ul><ul><ul><ul><ul><li>speech </li></ul></ul></ul></ul><ul><ul><ul><ul><li>nurse coordinator </li></ul></ul></ul></ul>
  4. 4. Introduction <ul><li>Most common congenital malformation of H and N (1:1000 in US; 1:600 in UK) </li></ul><ul><li>Second most common overall (behind club foot) </li></ul>
  5. 5. Epidemiology <ul><li>Syndromic CLAP </li></ul><ul><ul><li>associated with more than 300 malformations </li></ul></ul><ul><ul><ul><li>Pierre Robin Sequence; Treacher-Collins, Trisomies 13,18,21, Apert’s, Stickler’s, Waardenburg’s </li></ul></ul></ul><ul><li>Nonsyndromic CLAP </li></ul><ul><ul><li>diagnosis of exclusion </li></ul></ul>
  6. 6. Syndromic CLAP <ul><li>Single Gene Transmission </li></ul><ul><ul><li>trisomies 21, 13, 18 </li></ul></ul><ul><li>Teratogenesis </li></ul><ul><ul><li>fetal alcohol syndrome </li></ul></ul><ul><ul><li>Thalidomide </li></ul></ul><ul><li>Environmental factors </li></ul><ul><ul><li>materal diabetes </li></ul></ul><ul><ul><li>amniotic band syndrome </li></ul></ul>
  7. 7. Epidemiology: continued <ul><li>Isolated cleft palate genetically distinct from isolated cleft lip or CLAP </li></ul><ul><ul><li>same among all ethnic groups (1:2000, M:F 1:2) </li></ul></ul><ul><li>Isolated CL or CLAP </li></ul><ul><ul><li>different among ethnic groups </li></ul></ul><ul><ul><ul><li>American Indians: 3.6:1000 (m:f 2:1) </li></ul></ul></ul><ul><ul><ul><li>Asians 3:1000 (m:f 2:1) </li></ul></ul></ul><ul><ul><ul><li>African American 0.3:1000 (m:f 2:1) </li></ul></ul></ul>
  8. 8. Embryology <ul><li>Primary versus secondary palate </li></ul><ul><ul><li>divided by incisive foramen </li></ul></ul><ul><ul><ul><li>primary palate develops 4-5 wks </li></ul></ul></ul><ul><ul><ul><li>secondary palate develops 8-9 wks </li></ul></ul></ul><ul><li>Primary palate </li></ul><ul><ul><li>mesodermal proliferation of frontonasal and maxillary processes </li></ul></ul><ul><ul><li>never a cleft in normal development </li></ul></ul>
  9. 9. Embryology: continued <ul><li>Secondary palate </li></ul><ul><ul><li>medial ingrowth of lateral maxillae with midline fusion </li></ul></ul><ul><ul><li>always a cleft in normal development </li></ul></ul><ul><ul><ul><li>macroglossia, micrognathia may provide anatomical barriers to fusion </li></ul></ul></ul>
  10. 10. Classification <ul><li>Veau Classification - 1931 </li></ul><ul><ul><li>Veau Class I: isolated soft palate cleft </li></ul></ul><ul><ul><li>Veau Class II: isolated hard and soft palate </li></ul></ul><ul><ul><li>Veau Class III: unilateral CLAP </li></ul></ul><ul><ul><li>Veau Class IV: bilateral CLAP </li></ul></ul><ul><li>Iowa Classification - a variation of Veau Classification </li></ul>
  11. 11. Classification; continued <ul><li>Complete Clefts </li></ul><ul><ul><li>absence of any connection with extension into nose </li></ul></ul><ul><ul><li>vomer exposed </li></ul></ul><ul><li>Incomplete Clefts </li></ul><ul><ul><li>midline attachment (may be only mucosal) </li></ul></ul><ul><ul><ul><li>ex: submucous cleft (midline diasthasis, hard palatal notch, bifid uvula) </li></ul></ul></ul>
  12. 12. Anatomy - Normal <ul><li>Lip: “Cupid’s Bow” </li></ul><ul><li>Maxilla </li></ul><ul><ul><li>primary/secondary palates </li></ul></ul><ul><ul><li>soft palate </li></ul></ul><ul><ul><li>alveolus </li></ul></ul><ul><ul><li>maxillary tuberosity </li></ul></ul><ul><ul><li>hamulus </li></ul></ul>
  13. 13. Anatomy: palatal muscles <ul><ul><ul><li>Superior constrictor </li></ul></ul></ul><ul><ul><ul><ul><ul><li>primary sphincter </li></ul></ul></ul></ul></ul><ul><ul><ul><li>Tensor veli palatini </li></ul></ul></ul><ul><ul><ul><ul><ul><li>tenses palate </li></ul></ul></ul></ul></ul><ul><ul><ul><li>Levator Veli palatini </li></ul></ul></ul><ul><ul><ul><ul><ul><li>elevates palate </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>dilates ET </li></ul></ul></ul></ul></ul><ul><ul><ul><li>Salpingopharyngeus, palatopharyngeous, palatoglossus: minor contribution </li></ul></ul></ul>
  14. 14. Cleft Anatomy <ul><li>Unilateral Cleft Lip and alveolus </li></ul><ul><ul><ul><li>lack of mesodermal proliferation </li></ul></ul></ul><ul><ul><ul><ul><li>cleft of orbicularis </li></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>medial portion to columella </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>lateral portion to nasal ala </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><li>cleft of alveolus </li></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>alveolar bone graft </li></ul></ul></ul></ul></ul>
  15. 15. Cleft Anatomy - The Nose <ul><li>Ipsilateral LLC </li></ul><ul><ul><li>flattened </li></ul></ul><ul><ul><li>rotated downward </li></ul></ul><ul><li>Short columella </li></ul><ul><li>Bifid tip </li></ul>
  16. 16. Cleft Antatomy: continued <ul><li>Bilateral Cleft Lip/Alveolus/nose </li></ul><ul><ul><li>duplication of unilateral defect </li></ul></ul><ul><ul><ul><li>premaxilla </li></ul></ul></ul><ul><ul><ul><li>orbicularis to alar cartilages bilaterally </li></ul></ul></ul><ul><ul><ul><li>bifid tip </li></ul></ul></ul><ul><ul><ul><li>extremely short columella </li></ul></ul></ul>
  17. 17. Cleft Anatomy: continued <ul><li>Clefts of the primary hard palate/alveolus </li></ul><ul><ul><li>cleft alveolus always associated with cleft lip </li></ul></ul><ul><ul><li>cleft lip not necessarily associated with cleft alveolus </li></ul></ul><ul><ul><li>by definition there is opening into nose </li></ul></ul>
  18. 18. Cleft Anatomy: continued <ul><li>Clefts of secondary palate </li></ul><ul><ul><ul><li>Failure of medial growth maxillae </li></ul></ul></ul><ul><ul><ul><ul><li>fusion at incisive foramen </li></ul></ul></ul></ul><ul><ul><ul><ul><li>macroglossia </li></ul></ul></ul></ul><ul><ul><ul><li>Submucous vs. complete </li></ul></ul></ul><ul><ul><ul><li>Vomer </li></ul></ul></ul>
  19. 19. Multidisciplinary Approach <ul><li>These are not merely surgical problems </li></ul><ul><ul><li>Requires team approach throughout life </li></ul></ul><ul><ul><ul><li>neonatal period </li></ul></ul></ul><ul><ul><ul><li>toddler </li></ul></ul></ul><ul><ul><ul><li>grade school </li></ul></ul></ul><ul><ul><ul><li>adolescence </li></ul></ul></ul><ul><ul><ul><li>young adulthood </li></ul></ul></ul>
  20. 20. The Neonatal Period <ul><li>Pediatrician: </li></ul><ul><ul><li>directs care </li></ul></ul><ul><ul><li>establishes feeding </li></ul></ul><ul><ul><ul><li>complete clefts preclude feeding </li></ul></ul></ul><ul><ul><ul><ul><li>breast feeding not possible </li></ul></ul></ul></ul><ul><ul><ul><ul><li>a soft, large bottle with large hole is required </li></ul></ul></ul></ul><ul><ul><ul><ul><li>a palatal prosthesis may be required </li></ul></ul></ul></ul>
  21. 21. The Neonatal Period <ul><li>Presurgical Orthodontics (Baby Plates) </li></ul><ul><ul><ul><ul><li>Molds palate into more anatomically correct position </li></ul></ul></ul></ul><ul><ul><ul><ul><li>decreases tension </li></ul></ul></ul></ul><ul><ul><ul><ul><li>may improve facial growth </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Grayson, presurgical nasal alveolar molding (PSNAM) </li></ul></ul></ul></ul>
  22. 22. The Neonatal Period <ul><li>Surgical Repair </li></ul><ul><ul><li>Cleft Lip </li></ul></ul><ul><ul><ul><li>In US - “the rule of tens” - 10 wks, 10 lbs, Hgb 10 </li></ul></ul></ul><ul><ul><ul><li>Lip adhesion vs baby plates </li></ul></ul></ul><ul><ul><li>Cleft Palate </li></ul></ul><ul><ul><ul><li>Varies from 6-18 months - most around 10 mo </li></ul></ul></ul><ul><ul><ul><li>Early repair may lead to midface retrusion </li></ul></ul></ul><ul><ul><ul><li>Early repair improves speech </li></ul></ul></ul>
  23. 23. The Toddler Years <ul><li>Priority: Speech </li></ul><ul><ul><li>“ Cleft errors of speech” in 30% </li></ul></ul><ul><ul><ul><li>primary defects - due to VPI (hypernasality) </li></ul></ul></ul><ul><ul><ul><ul><li>consonants are most difficult sounds (plosives) </li></ul></ul></ul></ul><ul><ul><ul><li>secondary defects - due to attempted correction </li></ul></ul></ul><ul><ul><ul><ul><li>glottic stops, nasal grimace </li></ul></ul></ul></ul><ul><ul><li>Velopharyngeal insufficiency </li></ul></ul><ul><ul><ul><li>diagnosed by fiberoptic laryngoscopy or BaSw </li></ul></ul></ul><ul><ul><ul><li>surgical repair after failed speech therapy - usually around age 4 </li></ul></ul></ul>
  24. 24. The Toddler Years <ul><li>Growth hormone deficiency </li></ul><ul><ul><li>40 times more common in CLAP </li></ul></ul><ul><ul><li>suspects when below 5% on growth chart </li></ul></ul>
  25. 25. The Grade School Years <ul><li>Three primary issues </li></ul><ul><ul><li>Orthodontics </li></ul></ul><ul><ul><ul><li>poor occlusion </li></ul></ul></ul><ul><ul><ul><li>congenitally absent teeth </li></ul></ul></ul><ul><ul><li>alveolar bone grafting </li></ul></ul><ul><ul><ul><li>fills alveolar defect - around age 12 </li></ul></ul></ul><ul><ul><li>psychological growth </li></ul></ul><ul><ul><ul><li>considered standard of care </li></ul></ul></ul>
  26. 26. The Teenage Years <ul><ul><li>Midface retrusion </li></ul></ul><ul><ul><ul><li>etiology - ?early palatal repair </li></ul></ul></ul><ul><ul><ul><li>surgical correction around age 18 </li></ul></ul></ul><ul><ul><li>Psychological development </li></ul></ul><ul><ul><ul><li>counseling standard of care </li></ul></ul></ul><ul><ul><li>Rhinoplasty </li></ul></ul><ul><ul><ul><li>usually last procedure performed, around age 20 </li></ul></ul></ul>
  27. 27. Surgical Techniques <ul><li>Cleft Lip Repair </li></ul><ul><ul><li>unilateral </li></ul></ul><ul><ul><ul><li>rotation-advancement flap developed by Millard </li></ul></ul></ul><ul><ul><ul><li>complications </li></ul></ul></ul><ul><ul><ul><ul><li>dehiscence </li></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>infection </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><li>thin white roll </li></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>excess tension </li></ul></ul></ul></ul></ul>
  28. 28. Surgical Techniques <ul><li>Cleft Lip Repair </li></ul><ul><ul><li>bilateral </li></ul></ul><ul><ul><ul><li>bilateral rotation advancement with attachment to premaxilla mucosa </li></ul></ul></ul><ul><ul><ul><li>complications </li></ul></ul></ul><ul><ul><ul><ul><li>dehiscence </li></ul></ul></ul></ul><ul><ul><ul><ul><li>thin white roll </li></ul></ul></ul></ul>
  29. 29. Surgical Techniques <ul><li>Velopharyngeal Incompetnece </li></ul><ul><ul><li>superior based pharyngeal flap </li></ul></ul><ul><ul><li>sphincter pharyngoplasty </li></ul></ul><ul><ul><ul><ul><li>palatopharyngeus </li></ul></ul></ul></ul><ul><ul><li>complications </li></ul></ul><ul><ul><ul><ul><li>continued VPI </li></ul></ul></ul></ul><ul><ul><ul><ul><li>stenotic side ports </li></ul></ul></ul></ul>
  30. 30. Surgical Techniques <ul><li>Alveolar Bone Grafting </li></ul><ul><ul><li>iliac crest bone graft </li></ul></ul><ul><ul><li>complications </li></ul></ul><ul><ul><ul><li>infected donor site </li></ul></ul></ul><ul><ul><ul><ul><li>hematoma </li></ul></ul></ul></ul><ul><ul><ul><li>failed graft </li></ul></ul></ul><ul><ul><ul><ul><li>dehiscence </li></ul></ul></ul></ul><ul><ul><ul><ul><li>palatal prosthesis </li></ul></ul></ul></ul>
  31. 31. Surgical Techniques <ul><li>Midfacial Advancement </li></ul><ul><ul><li>LeForte osteotomies </li></ul></ul><ul><ul><ul><li>leave vascular pedicle attached in back of maxilla - prevents necrosis </li></ul></ul></ul><ul><ul><ul><li>complications </li></ul></ul></ul><ul><ul><ul><ul><li>malocclusion </li></ul></ul></ul></ul><ul><ul><ul><ul><li>infection </li></ul></ul></ul></ul><ul><ul><ul><ul><li>necrosis </li></ul></ul></ul></ul>
  32. 32. Surgical Techniques <ul><li>Rhinoplasty </li></ul><ul><ul><li>standard techniques </li></ul></ul><ul><ul><ul><li>tip projection </li></ul></ul></ul><ul><ul><ul><li>alar rotation </li></ul></ul></ul><ul><ul><ul><li>columellar length </li></ul></ul></ul><ul><ul><li>complications </li></ul></ul><ul><ul><ul><li>alar stenosis </li></ul></ul></ul>
  33. 33. Controversies: Otologic Disease <ul><li>>90% have COME </li></ul><ul><ul><ul><li>Robinson, et al </li></ul></ul></ul><ul><ul><ul><ul><li>prospective, 150 patients - 92% </li></ul></ul></ul></ul><ul><ul><ul><li>Muntz, et al. </li></ul></ul></ul><ul><ul><ul><ul><li>retrospective, 96% </li></ul></ul></ul></ul><ul><li>Pathology: ETD (controversial) </li></ul><ul><ul><ul><li>abnormal muscular attachment </li></ul></ul></ul><ul><ul><ul><li>Huang, et al. - Cadaveric study </li></ul></ul></ul><ul><ul><ul><ul><li>palatal repair restores ET function. ?Midface growth? </li></ul></ul></ul></ul>
  34. 34. Controversies: Timing of Repair <ul><li>Early repair </li></ul><ul><ul><ul><li>Advantage: improved speech </li></ul></ul></ul><ul><ul><ul><ul><li>Rohrich, et. al; retrospective study. The earlier the repair, the better speech. </li></ul></ul></ul></ul><ul><ul><ul><li>Disadvantage: worsening midface retrusion </li></ul></ul></ul><ul><ul><ul><ul><li>Rohrich, et. al; people with unrepaired palates have less midface retrusion </li></ul></ul></ul></ul>
  35. 35. Controversies: VPI <ul><li>Surgical Repair </li></ul><ul><ul><li>Reserved for failure of speech pathology </li></ul></ul><ul><ul><li>Pharyngeal Flap - superiorly based </li></ul></ul><ul><ul><ul><li>Advantage: time tested, severe cases </li></ul></ul></ul><ul><ul><ul><li>Disadvantage: passive obturator </li></ul></ul></ul><ul><ul><li>Sphincter Pharyngoplasty (palatopharyngeus rotation flap) </li></ul></ul><ul><ul><ul><li>Advantage: active sphincter </li></ul></ul></ul><ul><ul><ul><li>Disadvantage: new technique </li></ul></ul></ul>
  36. 36. Controversies <ul><li>Presurgical Nasal Alveolar Molding </li></ul><ul><ul><li>molds palate, alveolus and nose </li></ul></ul><ul><ul><ul><li>Advantage: excellent early results </li></ul></ul></ul><ul><ul><ul><li>Disadvantage: no long term results </li></ul></ul></ul><ul><ul><li>Grayson, et al. </li></ul></ul>
  37. 37. Conclusion and Future Directions <ul><li>Multidisciplinary approach </li></ul><ul><li>Not merely a “surgical problem” </li></ul><ul><li>Alveolar bone grafting </li></ul><ul><li>PSNAM </li></ul><ul><li>Pharyngoplasty vs. pharyngeal flap </li></ul>
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