1. Cleft Lip and Palate Christian El Amm, MD Plastic and Reconstructive Surgery

2. Normal Anatomy Columella Philtral columns Cupid’s bow Vermillion roll Wet vermillion Dry vermillion

3. Classification Unilateral / Bilateral Complete / Incomplete Cleft palate / complete / incomplete / Isolated / syndromic Cleft lip and palate

4. Cleft Lip CompleteIncomplete Simonart’s band

5. Complete Cleft Lip Complete cleft lip Cleft alveolus and gingiva Cleft anterior palate = “primary palate up to incisor foramen Severe nasal deformity

6. Incomplete cleft lip Simonart’s band May have a notch in the alveolus Milder nasal deformity

7. Cleft Palate

8. Cleft palate Primary palate Secondary palate RL Hard Soft Uvula Incisor Foramen

9. Cleft Palate VOMER

10. Bilateral Cleft Lip Prolabium Premaxilla

11. Bilateral Cleft Lip and Palate Vomer Palatal Shelf Premaxilla Prolabium Absent columella

12. Collapse of the lateral segment due to cheek pressure

13. Cleft palate Collapse of both lateral segments of the palate due to cheek pressure: The premaxilla is unable to move into its right position: “Premaxillary lockout” Incomplete cleft palateUnilateral complete cleft lip and palate Complete Cleft PalateBilateral complete CLP

14. Embryology 6 weeks gestation (human) ~ 13 somite stage (mouse)

15. Embryology Sperber: Clefting occurs because of failure of fusion of MNP (medial nasal process) and maxillary process (MxP)

16. Embryology Carstens and Walters: Clefting occurs because of failure of Rhombomere r2’ to migrate. This better explains the clinical observation that the most severe deficiency is in the lateral nasal area

17. Embryology Proposed migration path of r2’: the rhombomere process migrates towards the free margin of the lip and gingiva before continuing cephalad towards the lateral nose

18. Embryology Gene activation during differentiation and migration

19. Embryology and Genetics

20. Embryology and genetics

21. Treatment Priority=restore the Levator Veli Palatini muscle sling

23. Furlow double opposing Z- plasty Z-plasty results in longer palate

24. Treatment Treatment sequence of complete cleft lip and palate: First, get the segments in alignment by pre-surgical orthodontics, then perform lip and gingiva repair. Typically this occurs during the first months of life (3 months)

25. Palate repair is a separate stage: Typically before the age of one year. The levator muscle should be ready and mobile (free of scarring) for the phase of speech acquisition: 15-18 months

26. Surgical aims in cleft lip repair Reposition ala Restore nasal floor Lengthen columella on cleft side Lengthen medial lip segment (typically, lateral lip segment has enough length) Reconstitute symmetrical vermillion roll Restore dry vermillion medially (typically, lateral segment has enough dry vermillion) Align wet vermillion to dry vermillion line (“wet to dry line” Realign and correct abnormal insertion of orbicularis oris muscle Reconstitute philtral column (typical by placing the scar at the philtral column site)

28. Randall-Tennysson Millard

29. Techniques of cleft palate repair Von-Langenbeck with Intravelar Veloplasty: linear scar with muscle alignement (see previous slides) Two-Flap palatoplasty with IVV Furlow: double opposing Z-plasty (previous slides)

30. Secondary deformities Velopharyngeal incompetence

32. VPI: pharyngeal flap

33. Dental eruption Lateral incisor absent in 70% cases Canine tooth absent or abnormal in 15% (?) of cases Canine tooth can be successfully erupted through cleft once the cleft alveolus is bone-grafted Implants-Orthodontics

34. Growth: Maxilla and mandible Higher prevalence of class III occlusion (maxillary retrognathia): The maxilla is underdeveloped, due to surgical and/or congenital etiology Growth restriction highly correlated with surgical technique: scarring, incisions and denuded bone. Can be corrected by maxillary advancement (lefort I or lefort III)

36. Immediate results: scar will fade Technique used at OU

37. Long term

38. Craniofacial Malformations Craniosynostosis Craniosynostosis syndromes Mandibulo-Facial Dysostosis Hemifacial Microsomia and Oculo- Auriculo-vertebral Syndrome

39. Craniosynostosis

40. Sagittal: Scaphocephlay (boat) Unicoronal: Plagiocephaly (twisted) Bicoronal: brachycephaly (short) Metopic: trigonocephaly (keel) Most common=sagital Biggest differential: “deformational plagiocephaly” or “positional plagiocephaly”: non-synostotic

41. Trigonocephaly

42. Scaphocephaly

44. Craniosynostosis syndromes Associated with midface abnormalities: Base of skull synchondrosis Associated with finger/toe malformation: often diagnostic Other associated malformations

45. Major Craniosynostosis syndromes Crouzon: normal hand Apert’s: Major syndactyly Pfeifer: broad thumbs Muenke’s syndrome: First genetic- based diagnosis: FGFr-3 mutation predictably causing bicoronal (or unicoronal) synostosis

46. Crouzon

47. Apert’s

48. Pfeiffer’s

49. Madibulo-facial dysostosis Treacher-collins syndrome Nager’s

50. Treacher Collins Autosomal dominant Variable expression Zygomatic arch, masseter, mandible, side of mouth variably affected