1. 6/9/2015Prof. Sameer Bafaqeeh1 Clefts of the Lip, Alveolus and Palate Professor Sameer Bafaqeeh Otolaryngology Department KSU

2. 6/9/2015Prof. Sameer Bafaqeeh2 Overview yIntroduction yBasic Science yTimetable of Events neonatal toddler gradeschool teenage ySurgical Procedures yConclusion/Future Directions

3. 6/9/2015Prof. Sameer Bafaqeeh3 Introduction zA TEAM APPROACH IS REQUIRED pediatrician surgeon OMFS dentist ENT psychiatrist speech nurse coordinator

4. 6/9/2015Prof. Sameer Bafaqeeh4 Introduction zMost common congenital malformation of H and N (1:1000 in US; 1:600 in UK) zSecond most common overall (behind club foot)

5. 6/9/2015Prof. Sameer Bafaqeeh5 Epidemiology zSyndromic CLAP yassociated with more than 300 malformations xPierre Robin Sequence; Treacher-Collins, Trisomies 13,18,21, Apert’s, Stickler’s, Waardenburg’s zNonsyndromic CLAP ydiagnosis of exclusion

6. 6/9/2015Prof. Sameer Bafaqeeh6 Syndromic CLAP zSingle Gene Transmission ytrisomies 21, 13, 18 zTeratogenesis yfetal alcohol syndrome yThalidomide zEnvironmental factors ymateral diabetes yamniotic band syndrome

7. 6/9/2015Prof. Sameer Bafaqeeh7 Epidemiology: continued zIsolated cleft palate genetically distinct from isolated cleft lip or CLAP ysame among all ethnic groups (1:2000, M:F 1:2) zIsolated CL or CLAP ydifferent among ethnic groups xAmerican Indians: 3.6:1000 (m:f 2:1) xAsians 3:1000 (m:f 2:1) xAfrican American 0.3:1000 (m:f 2:1)

8. 6/9/2015Prof. Sameer Bafaqeeh8 Embryology zPrimary versus secondary palate ydivided by incisive foramen xprimary palate develops 4-5 wks xsecondary palate develops 8-9 wks zPrimary palate ymesodermal proliferation of frontonasal and maxillary processes ynever a cleft in normal development

9. 6/9/2015Prof. Sameer Bafaqeeh9 Embryology: continued zSecondary palate ymedial ingrowth of lateral maxillae with midline fusion yalways a cleft in normal development xmacroglossia, micrognathia may provide anatomical barriers to fusion

10. 6/9/2015Prof. Sameer Bafaqeeh10 Classification zVeau Classification - 1931 yVeau Class I: isolated soft palate cleft yVeau Class II: isolated hard and soft palate yVeau Class III: unilateral CLAP yVeau Class IV: bilateral CLAP zIowa Classification - a variation of Veau Classification

11. 6/9/2015Prof. Sameer Bafaqeeh11 Classification; continued zComplete Clefts yabsence of any connection with extension into nose yvomer exposed zIncomplete Clefts ymidline attachment (may be only mucosal) xex: submucous cleft (midline diasthasis, hard palatal notch, bifid uvula)

12. 6/9/2015Prof. Sameer Bafaqeeh12 Anatomy - Normal zLip: “Cupid’s Bow” zMaxilla yprimary/secondary palates ysoft palate yalveolus ymaxillary tuberosity yhamulus

13. 6/9/2015Prof. Sameer Bafaqeeh13 Anatomy: palatal muscles xSuperior constrictor –primary sphincter xTensor veli palatini –tenses palate xLevator Veli palatini –elevates palate –dilates ET xSalpingopharyngeus, palatopharyngeous, palatoglossus: minor contribution

14. 6/9/2015Prof. Sameer Bafaqeeh14 Cleft Anatomy zUnilateral Cleft Lip and alveolus xlack of mesodermal proliferation cleft of orbicularis –medial portion to columella –lateral portion to nasal ala cleft of alveolus –alveolar bone graft

15. 6/9/2015Prof. Sameer Bafaqeeh15 Cleft Anatomy - The Nose zIpsilateral LLC yflattened yrotated downward zShort columella zBifid tip

16. 6/9/2015Prof. Sameer Bafaqeeh16 Cleft Antatomy: continued zBilateral Cleft Lip/Alveolus/nose yduplication of unilateral defect xpremaxilla xorbicularis to alar cartilages bilaterally xbifid tip xextremely short columella

17. 6/9/2015Prof. Sameer Bafaqeeh17 Cleft Anatomy: continued zClefts of the primary hard palate/alveolus ycleft alveolus always associated with cleft lip ycleft lip not necessarily associated with cleft alveolus yby definition there is opening into nose

18. 6/9/2015Prof. Sameer Bafaqeeh18 Cleft Anatomy: continued zClefts of secondary palate xFailure of medial growth maxillae fusion at incisive foramen macroglossia x Submucous vs. complete xVomer

19. 6/9/2015Prof. Sameer Bafaqeeh19 Multidisciplinary Approach zThese are not merely surgical problems yRequires team approach throughout life xneonatal period xtoddler xgrade school xadolescence xyoung adulthood

20. 6/9/2015Prof. Sameer Bafaqeeh20 The Neonatal Period zPediatrician: ydirects care yestablishes feeding xcomplete clefts preclude feeding breast feeding not possible a soft, large bottle with large hole is required a palatal prosthesis may be required S.B.

21. 6/9/2015Prof. Sameer Bafaqeeh21 The Neonatal Period zPresurgical Orthodontics (Baby Plates) Molds palate into more anatomically correct position decreases tension may improve facial growth Grayson, presurgical nasal alveolar molding (PSNAM) S.B.

22. 6/9/2015Prof. Sameer Bafaqeeh22 The Neonatal Period zSurgical Repair yCleft Lip xIn US - “the rule of tens” - 10 wks, 10 lbs, Hgb 10 xLip adhesion vs baby plates yCleft Palate xVaries from 6-18 months - most around 10 mo xEarly repair may lead to midface retrusion xEarly repair improves speech

23. 6/9/2015Prof. Sameer Bafaqeeh23 The Toddler Years zPriority: Speech y“Cleft errors of speech” in 30% xprimary defects - due to VPI (hypernasality) consonants are most difficult sounds (plosives) xsecondary defects - due to attempted correction glottic stops, nasal grimace yVelopharyngeal insufficiency xdiagnosed by fiberoptic laryngoscopy or BaSw xsurgical repair after failed speech therapy - usually around age 4

24. 6/9/2015Prof. Sameer Bafaqeeh24 The Toddler Years zGrowth hormone deficiency y40 times more common in CLAP ysuspects when below 5% on growth chart

25. 6/9/2015Prof. Sameer Bafaqeeh25 The Grade School Years zThree primary issues yOrthodontics xpoor occlusion xcongenitally absent teeth yalveolar bone grafting xfills alveolar defect - around age 12 ypsychological growth xconsidered standard of care

26. 6/9/2015Prof. Sameer Bafaqeeh26 The Teenage Years yMidface retrusion xetiology - ?early palatal repair xsurgical correction around age 18 yPsychological development xcounseling standard of care yRhinoplasty xusually last procedure performed, around age 20

27. 6/9/2015Prof. Sameer Bafaqeeh27 Surgical Techniques zCleft Lip Repair yunilateral xrotation-advancement flap developed by Millard xcomplications dehiscence –infection thin white roll –excess tension

28. 6/9/2015Prof. Sameer Bafaqeeh28 Surgical Techniques zCleft Lip Repair ybilateral xbilateral rotation advancement with attachment to premaxilla mucosa xcomplications dehiscence thin white roll

29. 6/9/2015Prof. Sameer Bafaqeeh29 Surgical Techniques zVelopharyngeal Incompetnece ysuperior based pharyngeal flap ysphincter pharyngoplasty palatopharyngeus ycomplications continued VPI stenotic side ports

30. 6/9/2015Prof. Sameer Bafaqeeh30 Surgical Techniques zAlveolar Bone Grafting yiliac crest bone graft ycomplications xinfected donor site hematoma xfailed graft dehiscence palatal prosthesis

31. 6/9/2015Prof. Sameer Bafaqeeh31 Surgical Techniques zMidfacial Advancement yLeForte osteotomies xleave vascular pedicle attached in back of maxilla - prevents necrosis xcomplications malocclusion infection necrosis

32. 6/9/2015Prof. Sameer Bafaqeeh32 Surgical Techniques zRhinoplasty ystandard techniques xtip projection xalar rotation xcolumellar length ycomplications xalar stenosis

33. 6/9/2015Prof. Sameer Bafaqeeh33 Controversies: Otologic Disease z>90% have COME xRobinson, et al prospective, 150 patients - 92% x Muntz, et al. retrospective, 96% zPathology: ETD (controversial) xabnormal muscular attachment xHuang, et al. - Cadaveric study palatal repair restores ET function. ?Midface growth?

34. 6/9/2015Prof. Sameer Bafaqeeh34 Controversies: Timing of Repair zEarly repair xAdvantage: improved speech Rohrich, et. al; retrospective study. The earlier the repair, the better speech. xDisadvantage: worsening midface retrusion Rohrich, et. al; people with unrepaired palates have less midface retrusion

35. 6/9/2015Prof. Sameer Bafaqeeh35 Controversies: VPI zSurgical Repair yReserved for failure of speech pathology yPharyngeal Flap - superiorly based xAdvantage: time tested, severe cases xDisadvantage: passive obturator ySphincter Pharyngoplasty (palatopharyngeus rotation flap) xAdvantage: active sphincter xDisadvantage: new technique

36. 6/9/2015Prof. Sameer Bafaqeeh36 Controversies zPresurgical Nasal Alveolar Molding ymolds palate, alveolus and nose xAdvantage: excellent early results xDisadvantage: no long term results yGrayson, et al.

37. 6/9/2015Prof. Sameer Bafaqeeh37 Conclusion and Future Directions zMultidisciplinary approach zNot merely a “surgical problem” zAlveolar bone grafting zPSNAM zPharyngoplasty vs. pharyngeal flap

38. Tuesday, June 09, 2015 Professor Sameer Ali Bafaqeeh38

39. Tuesday, June 09, 2015 Professor Sameer Ali Bafaqeeh39