Introduction to clefts- Embryology, anatomy

Introduction to clefts- Embryology, anatomy

1. Introduction to clefts

2. Student learning objectives:- At the end of this lecture the students should able to explain What is cleft Types of clefts Clinical features of clefts Management of these patients

3. Introduction Most common congenital malformations.. Incidence – 1 : 700 live births Cleft palate  more in females. Cleft lip & palate  more in males. Unilateral cleft (75%) more common than bilateral cleft (25%). Left side affected in 60%.

4. Isolated cleft palate genetically distinct from isolated cleft lip or CLAP Same among all ethnic groups (1:2000 live births, M:F 1:2) Isolated CL or CLAP Different among ethnic groups American Indians: 3.6:1000 live births (M:F 2:1) Asians: 3:1000 live births (M:F 2:1) African American: 0.3:1000 births (M:F 2:1) Otolaryngology Grand Round Presentation 2000

5. Embryology of cleft palate

6. Golden terminology

7. Palate surgical anatomy & A pplied anatomy of cleft palate

8. Etiology Genetic factors. Environmental factors (smoking , alcohol, anticonvulsants, steroids, radiation, viral infections and mechanical factors ) Folic acid deficiency Syndromic clefts .

9. Classification Davis and Ritchie According to anatomical basis - GROUP 1- Prealveolar clefts ( unilateral, bilateral and median) GROUP 2- Postalveolar clefts GROUP 3- Complete alveolar cleft (unilateral, bilateral and median)

10. Classification Veau ( 1931) GROUP 1- Clefts of soft palate only GROUP 2- Clefts of hard & soft palate GROUP 3- Complete unilateral cleft, extending from uvula to incisive foramen and then deviates to one side extending through the alveolus GROUP 4- Complete bilateral alveolar cleft

11. Kernahan Classification Represent in the form of Y The anterior portion of Y depict the lip (1-4) The middle alveolus (2-5) Incisive foramina and the posterior portion (the area of hard palate from the alveolus back to incisive foramen)( 3-6) Posterior to to the incisive foramen, the hard palate (7-8) The soft palate (9)

12. Timing of surgical repair Repair has been attempted as early as second day after delivery. Most surgeons prefer early repair of the deformity. Rule of tens -10 weeks, 10 gm% Hb and weight of 10 pounds.

13. Sr. No Procedure Timing mentioned in the literature Timing followed at our institute 1. CL repair After 10 weeks 16 weeks 2. CP repair 9-18 months 12 months 3. Pharyngeal flap/ Pharyngoplasty 3-5 yrs or later based on speech development - 4. Alveolar recon with bone graft 6-9 yrs based on dental development. 6-9 years 5. Cleft Orthognathic Surgery 14-16 yrs in girls, 16-18 yrs in boys. same 6. Cleft Rhinoplasty After age 5 yrs but preferably at skeletal maturity; after orthognathic surgery when possible. same 7. Cleft lip revision Anytime once initial remodeling & scar maturation is complete. Best done after 5 yrs. 2 years after first surgery Staged Reconstruction Of Cleft Lip Cleft Palate Deformities

14. Lip Repair Tennison and Randall triangular flap repair. Millard rotation and advancement repair. Delaire philosophy and technique. LeMesurier technique. Skoog technique. Manchester method. Barsky ( Veau II) method. Techniques

15. It is not a commonly practiced technique for the unilateral cleft lip deformities as the scar contractures invariably results in the notching of the lip. Practiced in case of bilateral lip deformity where prolabium segment has inadequate tissue and the mobilization or advancement/ rotation is not possible STRAIGHT LINE REPAIR

16. STRAIGHT LINE REPAIR

17. TENNISON AND RANDALL TECHNIQUE ADVANTAGES : Lengthens both the lip segments of cleft as well as non cleft sides. It results in final symmetric, balanced lip with a well defined cupid’s bow. Only a small amount of tissue is discarded. It also contributes to correction of nasal deformity. This technique is comparatively easier for inexperienced surgeons.

18. TENNISON AND RANDALL TECHNIQUE DISADVANTAGES It results in a final horizontal scar on the lip. Precise matching of the incisions on both the sides is essential for both muscle and mucosa. All layers to be sutured on the basis of triangular flap. If any mistake done in marking or incision it can’t be recorrected . Procedure is difficult to revise in secondary surgical procedures.

19. TENNISON AND RANDALL TECHNIQUE

20. Millards Rotational Advancement

21. MILLARDS ROTATIONAL ADVANCEMENT 1 to 2 = 1 to 3 = 2-4 mm 2 to 6 = 8 to 7 = 20 mm 2 to 4 = 8 to 10 = 9 -11mm 3 to 5 + x = 8 to 9

22. MILLARDS ROTATIONAL ADVANCMENT

23. Advantages This procedure allows adjustments at the time of surgery. Minimal amount of tissue is discarded. Places scar in anatomically correct position i.e. scar line forms philtral ridge on cleft side. Nostril sill is reinforced and built up with this procedure. Revision is easy at time of secondary surgeries.

24. Disadvantages This technique is difficult to master, especially in wide clefts. Major bulk of lip segments is in centre and not on lower free border giving pouting appearance in wide clefts. Difficulty in obtaining adequate lip length. It has got a tendency toward vertical scar contracture. It forms constricted nostril toward the cleft side.

25. MILLARDS ROTATIONAL ADVANCEMENT

26. Delaires technique Incision design (Delaire) when there is little retraction of cleft margins when the white line of lip is not too accentuated.

27. Rationale for Palate repair Growth Feeding and swallowing Speech Eustachian tube Timing of palatoplasty Speech out come Maxillary growth Syndromic child with cleft palate

28. Nerve and blood supply

29. Single stage palatal repair Von Langenbeck Pushback ( Veau - Wardill - Kilner ) Two flap palatoplasty ( Baradach & Salyers ) Vomer flaps Double opposing Z- plasty ( Furlow ) Intravelar Veloplasty ( sommerlad )

30. It consists of two Z plasty . One in the oral mucosa of the soft palate, and the other in the reverse orientation on the nasal mucosa of the soft palate. The hard palate cleft is closed by a vomer flap in one or two layers. Double reversing “z” plasty ( furlow 1980, 1986)

31. It lengthens the soft palate within the substance of soft palate, making it unnecessary to raise large mucoperiosteal flaps from the hard palate.(with possible retardation of midfacial growth.) This technique reorients the malposition of the levator muscles. If the closure of the mucomuscular flaps seems to be too tight lateral relaxing incisions can be given to reduce the tension. Advantages

32. DOUBLE REVERSING “Z” PLASTY (FURLOW 1980, 1986

33. DOUBLE REVERSING “Z” PLASTY (FURLOW 1980, 1986

34. DOUBLE REVERSING “Z” PLASTY (FURLOW 1980, 1986

35. DOUBLE REVERSING “Z” PLASTY (FURLOW 1980, 1986

36. INTRVELAR VELOPLASTY (KREINS 1970, 1975) Kreins emphasized clearly on the abnormal orientation of the levator palitini muscles and the need to detach them from there insertions on the posterior edge of the palatal bone and to reorient them in the transverse direction. In the authors series additional improvement in speech was achieved by reorienting the muscle in the midline.

37. VON LANGENBACK OPERATION (1859, 1861) It is an established procedure that includes the elevation of large mucoperiosteal flaps from the hard palate. It does not include a lengthening maneuver. The incision is carried through the periosteum and the mucoperiosteum is lifted widely of the bone with a blunt elevator. Lateral relaxing incision is open widely in both the soft and hard palate areas.

38. VON LANGENBACK OPERATION (1859, 1861)

39. WARDILL KILNER VEAU OPERATION This surgery was done to increase the anteroposterior length of the palate at the time of primary palatoplasty . A “V-Y” lengthening operation is done in the tissues of the mucoperiosteum of the hard palate.

40. WARDILL KILNER VEAU OPERATION

41. WARDILL KILNER VEAU OPERATION

42. …….Thank you