13.cleft lip

cleft lip

1. CLEFT LIP PRESENTED BY : BHANUPRIYA U MODERATED BY : DR AKSHAY SHETTY

2. CONTENTS INTRODUCTION EMBRYOLOGY ETIOLOGY EPIDEMIOLOGY CLASSIFICATION ANATOMY PRENATAL DIAGNOSIS TIMING OF REPAIR MANAGEMENT CONCLUSION REFERENCES

3. INTRODUCTION The enigma of cleft lip and the historic journey to repair the defect has been well described in the narrative book by Millard The documentation of the first cleft lip repair in the year 390 AD where an unidentified Chinese physician cut the edges of the cleft and stitched it together with a postoperative order of limited lip movements for the next 100 days. The need to identify an ideal technique was derived on the basis of the anatomical outcome of the repair and also the postoperative scarring. The concepts of cleft lip repair have evolved from straight line repairs to a variety of techniques using various cutbacks, triangles, z- plasties and other flaps.

4. EMBRYOLOGY

5. Components of face

6. Theories of cleft 1.CLASSICAL THEORY Dursy (1869) and His (1874) postulated that there were five processes of the face , namely the frontonasal, the paired maxillary and mandibular processes . These processes grew towards and fused with each other, to form the face. Failure to fuse was thought to lead to cleft formation. This theory was too pat and did not serve to explain the formation of median clefts.

7. 2. MESODERMAL REINFORCEMENT Stark (1954 ) managed to six cleft embryos, three of which had bilateral clefts, and after serial coronal section and planimetric measurements , he found of deficiency of mesoderm on the cleft side in every case and postulated his theory of mesodermal penetration .

8. His theory states that there are three mesodermal masses within that epithelial wall which constitutes the primitive palate. If mesoderm is deficient on one side, then the epithelial wall rupture on that is lacking, the cleft is either complete or incomplete .

9. ETIOLOGY The embryological event occurs leading to cleft lip with or without cleft palate during 3–7 weeks and leading to cleft palate occurs during 5–12 weeks.

10. Syndromic Cleft Lip with or without Cleft Palate 1 . Trisomy 13 and trisomy 21 2. Waardenburg’s syndrome 3. Van der Woude syndrome 4 . Velocardiofacial syndrome 5. Stickler syndrome 6. Treacher-Collins syndrome 7. Downs syndrome 8. Goldenhar syndrome 9. Fetal alcohol syndrome

11. Van der Woude syndrome Syndactyly: Right unilateral cleft lip

12. Bilateral cleft lip and palate with chest wall deformity Congenital deformity of right upper limb with bilateral cleft lip

13. Environmental Factors 1. Vitamin B deficiency 2. Vitamin A deficiency and vitamin A excess 3. Viral infection during first trimester 4 . Influence of drugs a . Cortisone b. Deoxyguanosine c. Insulin d. Alcohol e. Cyclopamine 5. Exposure to radiation 6 . Anoxia 7. Stress 8. Smoking.

14. EPIDEMIOLOGY CL/P affects about one in 700 births, with wide variability across geographic origin Asian or American Indian populations have the highest birth prevalence, often as high as 1: 500, with European-derived populations intermediate at about 1:1000 and African-derived populations the lowest at 1:2500. There is a 2:1 male: female ratio and a similar 2:1 left-side: right-side ratio for unilateral clefts.

15. CLASSIFICATION

16. Davis and Ritchie ( 1922)

17. VEAU (1931) Group 1: Cleft of soft palate only. Group 2: Cleft of hard and soft palate extending no further than the incisive Foramen Group 3: Complete unilateral cleft Group 4: Complete bilateral cleft

18. KERNAHAN STRIPED Y (1971)

19. MILLARD MODIFIED STRIPPED Y

20. Right microform cleft lip Right incomplete cleft lip

21. Right complete cleft lip with normal alveolus Left complete cleft lip with cleft alveolus

22. Left complete cleft lip with palate Bilateral left lip

23. Prenatal Diagnosis in Cleft Lip usually established during the second and third trimester

24. ANATOMY

25. Normal upper lip anatomy 1 . Skin, mucous membranes and vermillion with labial mucosal glands. 2. Muscles: orbicularis oris insertion is displaced in unilateral and bilateral cleft patients. 3. Vascular supply: superior labial artery, inferior labial artery and septal arteries are the major blood supplies of the lips and the nose.

26. ORBICULARIS ORIS

27. BLOOD SUPPLY OF CLEFT LIP

28. Surface Anatomy

29. Timing of intervention based on chronological age

30. TIMING OF INTERVENTION BASED ON STATUS OF DENTITION

31. BASIC TREATMENT ALGORITHM FOR UNILATERAL CLEFT LIP

32. Presurgical Nasoalveolar Moulding presurgical maxillofacial orthopaedic appliances mould the nasolabial structures through specifically directed forces, thus reducing the deformity before surgery for an easy repair based on the theory that increased hyaluronic acid content is present in the infant cartilage, which makes ,the cartilaginous structure more pliable and plastic.

33. PNAM is based on the principles of negative sculpting and passive moulding of the alveolus, lip and nasal tissues . An initial impression is obtained, and custom-made plates are fabricated. A series of modifications are made to the surface of the appliances with the addition and deletion of materials in certain areas. The purpose of this is achieving approximation and symmetry of the two maxillary alveolar segments and addressing the nasal deformity by moulding the cartilage in anatomical position

34. Grayson’s technique The protruding premaxilla is molded first into proper position with passive type of orthopedic appliances and tapping of the lip in bilateral cleft lip and palate and alveolar approximation in unilateral cleft lip with palate A nasal molding device is added to orthopedic appliances to increase the columellar length , reshape alar dome. This two-stage procedure avoid overstretching of nasal cartilage.

35. Figuera’s technique Alveolar molding and nasal molding are performed simultaneously with an acrylic plate and rigid acrylic nasal extension. A soft resin ball attached to acrylic plate across the prolabium maintains nasolabial angle. Rubber bands help gentle retraction of premaxilla backwards .

36. Liou’s technique The nasoalveolar molding device is composed of a dental plate, nasal component for nasal molding and micropore taps for premaxillary retraction. Dental plates are kept on lateral maxillary segments with dental adhesive. It increases columellar height and supports nasal tip and cartilages.

37. Protocols

38. Principles of Cleft Lip Repair Steffensen , 1953 ••Achieving accurate skin, muscle, and mucous membrane union. ••Reconstruction of symmetrical nostril floor. ••Symmetrical vermilion border. ••Slight eversion of the lip. ••Minimal scar whose contractions will not jeopardize the remaining objectives mentioned.

39. Surgical Method of Red Lip Reparation Z PLASTY

40. Unilateral Cleft Lip Cleft Severity Index

41. Unilateral Cleft Lip Cleft Severity Index

42. Surgical Method of Unilateral Cleft Lip Straight Line Suture ( Rose-Thompson principle)

43. Millard’s Rotation-Advancement Flap . (1957)

44. Reference points for Millard rotation-advancement technique Point 1: Center or low point of Cupid’s bow Point 2: Peak of Cupid’s bow on noncleft side Point 3: Peak of Cupid’s bow, medial side of cleft Point 4: Alar base, noncleft side Point 5: Columellar base, noncleft side Point 6: Commissure, noncleft side Point 7: Commissure, cleft side Point 8: Peak of Cupid’s bow, lateral side of cleft Point 9: Superior extent of advancement flap Point 10: Alar base, cleft side Point x: Back-cut point Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg 1993;9:169

45. Once the markings are made, the rotation and advancement flaps are designed. The basic aim is to achieve symmetry of the philtral columns. 1 . Curve the incision on the rotation side; this helps in gaining length on the cleft side philtral column. 2. Back cut: The role of the back cut comes into force when the rotation is not adequate to provide enough length to match the normal philtral column. It can be made to cross the midline or made parallel to the normal side philtral column. 3. Role of “C” flap: The “C” flap can be designed to accomplish either of the following goals: closure of nasal sill, columellar lengthening or back into the defect created by rotation of the flap

46. TENNISON Triangular Flap Method

47. Modifications to Millard’s Technique Mohler (1987) The medial lip rotation incision was modified to extend onto the columella. The back cut was added which was confined to the columella. The C flap is used to fill the defect created by rotating the medial lip element at the base of the columella. Thus it is used for lengthening the shortened columella

48. curvilinear incision that extends up and parallels the medial cleft margin but stops near the medial edge of the base of the columella. The lateral lip element advancement incision curves outward and then medially as it extends superiorly before it again extends to the alar base Delaire repair.

49. Pfeifer described a wavy-line repair that allowed downward rotation as the curves were approximated into a straight line.The two curves are brought together such that the highest and lowest points of one curve are approximated with the corresponding highest and lowest points of the other, thus creating a straight line . Pfeiffer repair.

50. Afroze repair. The Afroze incision is a combination of two incisions—the Millard incision on the medial non cleft side and the Pfeiffer incision on the cleft side

51. Fisher repair. The repair allows for a repair line that ascends the lip at the seams of anatomical subunits

52. Noordhoff -Chen Technique Noordhoff addressed the vermillion mismatch

53. Bilateral Cleft Lip Repair Straight Line Repair

54. Millard’s Repair Millard’s repair allowed rotation of the Cupid’s bow with the gap filled from skin advanced from the lateral element . It allowed complete elevation of the prolabium and suturing of the orbicularis across the premaxilla. In addition, Millard created lateral segments of the prolabium as “forked flaps”. These flaps were banked to add columellar height at a later stage , thus addressing the vertical height deficiency, and it also corrected the wide alar bases

55. Mulliken’s Repair Mulliken designed a narrow prolabial flap with slightly concave sides ; 2; 2.5 mm at base and 3.5–4 mm between Cupids bow peak. The surgical stratagem is symmetrical labial repair and synchronous anatomic positioning of the alar cartilages with sculpturing/draping of the nasal soft tissues.

56. • Suture of the orbicularis oris in the midline. • Fixation to anterior nasal spine. • Positioning dislocated lower lateral cartilages: interdomal suture. • Suspension over ipsilateral upper lateral cartilage with Inter cartilaginous suture.

57. Veau -III repair

58. Microform Cleft Lip Mulliken has classified microform clefts into: •• Mini microform , where the cleft is confined to a vermillion notch with the Cupid’s bow points at the same level. •• Microform, where the cleft involves the vermillion and less than 3 mm of the lip above the Cupid’s bow •• Minor form, where more than 3 mm of the body of the lip above the Cupid’s bow is cleft. These are to be treated like partial cleft lips.

59. Primary Chielorhinoplasty A variety of options exists to address the nasal deformity with cleft lip. These include preoperative nasoalveolar moulding , overcorrection of nostril width and alar cartilage at the time of lip repair and postoperative use of nasal conformers. Primary treatment of the nose at the time of lip repair has become popular, in order to gain early restoration of the symmetry by repositioning the alar cartilage and lengthening the columella.

60. Postoperative Wound Care and Outcome Assessment 1. Suture area should be cleaned daily with saline and baby soap twice; topical application of antibiotics is recommended for 10 days. 2. 3 weeks post-op, we recommend to massage the scar towards the mucosa to prevent scar contracture. 3. Massage can be done with vitamin E cream/silicone gel/ oil. It is recommended to massage for at least 6 months.

61. Outcomes Mucosal notching mucosal dissection should always be in the plane between the muscles and the mucosa plus minor salivary glands. Failure to maintain that results in a thin mucosa that inverts after suturing . This leads to mucosal notching. In case of very mild mucosal notching presenting at 3 weeks, it is advised to continue the massage. If notching persists after 6 months of lip repair, it can be treated by surgically improving placement of muscle.

62. Scarring A scar can present in various forms . A scar can be linear but hypertrophic in appearance. Such scars can be avoided if proper massaging is done in the postoperative phase . If the scar has keloid tendency, triamcinolone injections are to be given at an interval of 15 days followed by vigorous massage

63. Contracture A scar can also present as a gross or mild vertical contracture. This usually results due to inadequate rotation , thus pulling the lip towards the nose resulting in vertical shortening of cleft side philtral column. Improper muscle closure or overzealous separation of the skin from the muscle can lead to scarring of the skin.

64. Inadequate philtral column matching This again results due to lack of a proper design and improper mobilization of the rotation flap . Complete breakdown of the surgical wound may be the most severe adverse outcome in the immediate post-operative phase .

65. The basic indications for a lip revision surgery are : 1 . Unilateral. • The scar is not in line with the natural philtral column . • The lip length is short. • There is a mucosal notching. • There is a white roll to Cupid’s bow mismatch. • There is a nasolabial fistula . 2. Bilateral. • There is a wide philtral column . • Mucosal notching. • Whistling defect. • No sulcus depth.

66. Intrauterine Cleft Repair Fetal surgery is an emerging technology in which the patients are not born and interventions have to be done in intrauterine life. Indications : 1 . A defect that if not corrected can cause the fetus not to survive till delivery. 2 . A defect that if not corrected before birth can cause the permanent anatomic or physiological loss of organs. 3 . A defect if not intervened before birth can advanced to such a degree that cannot be retrieved postnatally

67. Milestones of fetal cleft lip/palate Because of fetal scarless wound healing, fetal surgery had become a promising option for CLP . Similar to the other indications of fetal surgery, animal studies on CLP repair started in the mid 1980s . There is only one case of human fetal CLP in literature reported by Ortiz Monasterio in 1997. After a premature birth and 2-month intensive care the infant had been discharged well. Unfortunately , the infant died two months after the discharge

68. De Boutray M, Et Al., Median Cleft Of The Upper Lip: A Newclassification To Guide Treatment Decisions, Journal Of Cranio- maxillo -facial Surgery (2016), extremely rare group of facial clefts defined by a cleft involving the upper lip, situated on the median line . MCL have different clinical expressions, from a small notch of the vermilion to a cleft of the entire length of the philtrum with nasal and maxillary extension

69. Complete Midline Cleft of Lower Lip, Mandible, Tongue , Floor of Mouth with Neck Contracture: A Case Report and Review of Literature. Anantheswar Y. N. The median cleft of the lower jaw was first described by Couronne in 1819. Since then, only 80 cases have been reported so far

70. REFERENCES Peter Wardbooth – Vol 1 Principles Of Oral And Maxillofacial Surgery – Peterson Vol.2 Fonseca –Oral And Maxillofacial Surgery Volume 3 Art And Science Of Cleft Lip And Cleft Palate Repair- Girish N Amlani Atlas Of Cleft Lip And Palate & Facial Deformity Surgery- Jian -min Yao • Jing- hong Xu Cleft Lip Surgery A Practical Guide- Bart Van De Ve Sayler And Bardachs Atlas Of Craniofacial Surgery Cleft Lip and Palate Primary Repair – brian c sommerald

71. THANK YOU