Cleft lip and palate

Cleft lip and palate

1. CLEFT LIP AND PALATE Dr Sumer yadav MCh -Plastic and reconstructive surgery

2. Introduction Cleft lip and palate are the most common congenital craniofacial anomalies treated by plastic surgeons . Cleft care requires that the plastic surgeon be a member of a collaborative multidisciplinary team . Successful treatment of these children requires technical skill, in-depth knowledge of the abnormal anatomy, and appreciation of three-dimensional facial aesthetics .

3. EPIDEMIOLOGY the most common diagnosis is cleft lip and palate at 46 %, Isolated cleft palate at 33 % isolated cleft lip at 21 %. Majority of bilateral cleft lips (86%) and unilateral cleft lips (68%) are associated with a cleft palate . In the white population , cleft lip with or without cleft palate occurs in approximately 1 in 1,000 live births . twice as common in the Asian population half as common in African Americans

4. ETIOPATHOGENESIS Both environmental teratogens and genetic factors are implicated in the genesis of cleft lip and palate Intrauterine exposure to the anticonvulsant phenytoin is associated with a 10-fold increase in the incidence of cleft lip . Maternal smoking during pregnancy doubles the incidence of cleft lip . Genetic abnormalities can result in syndromes that include clefts of the primary or secondary palates among the developmental fields affected . The most common syndrome associated with cleft lip and palate is van derWoude syndrome DiGeorge , or conotruncal anomaly syndromes are the most common diagnoses associated with isolated cleft palate.

5. MULTIDISCIPLINARY CLEFT CARE cleft lip and or palate require coordinated care from multiple specialties to optimize treatment outcome . Typical members of a cleft team : audiologist , dentist , geneticist , nurse , nutritionist / dietitian , oral surgeon , orthodontist , otolaryngologist , pediatrician, plastic surgeon, psychologist, social worker, and speech pathologist . The goal of cleft care is to eliminate as many steps in the treatment plan as possible by optimizing the outcome and benefit of each essential intervention . a child with a cleft requires a complex lengthy surgical treatment plan

6. Head and Neck of 4-Week Old Embryo Trigeminal nerve Facial nerve Glossopharyngeal nerve Vagus nerve Embryological Background

7. Embryological Background Various theories have been given for its development. Alteration in intrinsic palatal shelf force Failure of tongue to drop down Non fusion of shelves Rupture of cyst formed at the site of fusion

8. Classification We classify as the follows: its combined ( cl+cp ) or isolated cleft(cl or cp )? is it unilateral or bilateral? is it complete (if it cross the nasal philtrum ) or incomplete ( if it doesn’t cross the nasal philtrum .

9. Nagpur classification Group I - Cleft of lip Group I(A) - Cleft of lip & alveolus Group II - Cleft of palate Group II(S) - Submucous cleft of palate Group III - Cleft of lip & palate

10. Karnahan’s Classification Millard’s Modification of Karnahan’s Classifcation Lip Alveolus Primary Palate Hard Palate (Maxillary) Hard Palate (Palatine) Soft Palate

11. Kriens “LAHSHAL” L = Lip (right) A = Alveolus (right) H = Hard Palate (right) S = Soft Palate (median) H = Hard Palate (left) A = Alveolus (left) L = Lip (left) Capital letter = complete cleft Lowercase letter = incomplete cleft “.” or “-” = normal Examples LA….l = complete right cleft lip and alveolus, incomplete left cleft lip LAHS = complete right unilateral cleft lip, alveolus, hard, and soft palate

12. Other Types of Clefts Microform Cleft: May look like a little dent in the red part of the lip a scar from the lip up to the nostril. Muscle tissue underneath the cleft can be affected and may require surgery Submucous Cleft Palate: Midline deficiency or lack of muscular tissue Often a submucous cleft palate is associated with a bifid or cleft uvula Posterior nasal spine is almost always missing Speech Problems are common

13. Prenatal Diagnosis Cleft lip can be easily diagnosed by performing ultrasonography in the second trimester Diagnosing a cleft palate with ultrasonography is very difficult Three-dimensional imaging has been introduced to prenatal ultrasonography diagnostics of cleft anomalies

14. Diagnosis Advantages of Prenatal Diagnosis: Time for parental education Time for parental psychological preparation Opportunity to investigate other associated anomalies Gives parents the choice of continuing the pregnancy Opportunity for fetal surgery

15. Etiology “Actually no one knows exactly what causes clefts” Multiple factors may be involved, like: Genetics (inherited characteristic) from one or both parents . Environmental factors Drugs: corticosteroids (anti-inflammatory), phenytoin (anticonvulsant), retinoid. Infections: like rubella during pregnancy. Alcohol consumption, smoking, hypoxia during pregnancy, some of dietary and vitamins deficiencies (like folic acid and vitamin A deficiency) Maternal Age

16. GENETICS CL/P Normal parents, one child with CL/P  4% risk CL/P in next child Normal parents, two kids with CL/P  9% risk CL/P in next child One parent CL/P, no affected kids  4% risk CL/P in next child One parent CL/P, one child CL/P  17% risk CL/P in next child Risk of CL/P in siblings increases with severity of deformity - child with unilateral CL  risk CL/P next child 2.5% - child with bilateral CL/P  risk CL/P next child 5.7% CP Normal parents, one child with CP  2% risk of CP in next child Normal parents, 2 children with CP  7% risk of CP in next child Parent with CP, no affected children  6% risk for next child Parent with CP, one child with CP  15% risk for next child All infants with clefs must be evaluated for presence of other anomalies Associated nomalies are more common in infants with isolated CP

17. Nonsyndromic Cleft Pierre Robin Sequence is the most common associated nonsyndromic anomaly is a relative term describing the small size of the lower jaw ) and Glossoptosis ( is a medical condition and abnormality which refers to the downward displacement or retraction of the tongue)

18. Syndromic Cleft Common Syndromes Stickler (25% of syndromic CP) – autosomal dominant type 2 collagen gene Pierre Robin, ocular/hearing/joint malformations Velocardiofacial ( Shprintzen’s ) – 15% of syndromic CP Autosomal dominant, variable expressivity, CATCH 22 – Deletions at 22q11, Facial, cardiovascular, immunologic, developmental anomalies Van der Woude’s (19% of syndromic CL/P and CP) Autosomal dominant CL/P or CP with bilateral lower lip pits Shprintzen ’

19. Problems Associated With Cleft Lip and Palate Feeding Dental problems Nasal Deformity and Esthetic Problems Ear Problems Speech Difficulties Associated Anomalies

20. Feeding Difficulties Cleft lip= makes it more difficult for an infant to suck on a nipple Cleft Palate= may cause formula or breast milk to be accidently taken up into the nasal cavity Inability to create negative pressure inside oral cavity Frequent regurgitations Upper respiratory tract infections

21. Dental Problems Local Dental Problems: Congenitally Missing teeth, Hypodontia, Hyperdontia, Oligodontia Presence of natal and neonatal teeth Anamalies of tooth morphology like microdontia, macrodontia etc Fused teeth Enamel Hypoplasia Poor periodontal support, early loss of teeth Gemination, Dilacerations Orthodontics Problems: Class III tendency Anterior and Posterior Cross bite Spacing and crowding

22. Nasal Deformity and Esthetic Problems Facial Disfigurements Poor nasal shape Scar marks of surgeries Poor lip function during speech Poor dental alignment and smile

23. Ear Problems Middle ear disease - 22% to 88% Conductive hearing loss and chronic suppurative otitis media may result Repeated tympanostomy tube placement

24. Why do cleft kids have eustachian tube dysfunction? Abnormal curvature of the eustachian tube lumen Cephalometric data - width and angulation of the skull base with respect to the eustachian tube are different Abnormal insertions of the tensor and levator veli palatini muscles into the cartilages and skull base Palatal muscle dysfunction

25. Speech Problems: Hearing loss hampers proper development of speech Velopharyngeal Insufficiency (VPI) Abnormal air Poor pronunciation of Bilabial , Labiodental , Linguoalveolar sounds

26. MANAGEMENT OF CLEFT LIP AND PALATE

27. Schedule of Treatment Birth: Initial Assessment Pre-surgical assessment 3 Month: Primary Lip repair 9-18 month: Palate Repair 2 Year: Speech assessment 3-5 Year: Lip Revision Surgery 8-9 Year: Initial interventional Orthodontics Preparation for alveolar bone grafting 10 Year: Alveolar Bone Grafts 12-14 Year: Definite Orthodontics 16 Year: Nasal Revision Surgery 17-20 Year: Orthognathic Surgery

28. Multidisciplinary Cleft Lip And Palate Team Genetic Scientist Pediatrician Pedodontist Orthodontist Oral and Maxillofacial Surgeon Prosthodontist ENT Surgeon Plastic Surgeon Psychiatrist Speech Therapist Social Worker

29. Feeding Cleft lip = makes it more difficult for an infant to suck on a nipple use special nipples to allow the baby to latch properly (either pump or use formula) Cleft Palate = may cause formula or breast milk to be accidentally taken up into the nasal cavity don’t feed baby without palatal obturator (prosthetic palate) feed in an upright position to keep milk from coming out of the nose

30. Mead Johnson/Enfamil Cleft Feeder Special Needs Feeder / Haberman Feeder Pigeon Feeder Dr. Brown’s Natural Flow to relieve gas

31. Presurgical Orthopeadics : Reduces the size of cleft; Aids in Surgery Partial obturation aids in feeding Parental Reassurance at a crucial time Maxillary Strapping Nasoalveolar Moulding Appliances (NAM)

32. Require orthopedic repositioning of the nasal cartilages, columella , nasal tip, and lateral wall of the vestibule Presurgical infant nasal remodelling  nasal molding Nasal molding by post surgical nasal stenting Facial taping  2 to 3 months

33. BILATERAL CLEFT LIP The most challenging condition The premaxilla is extremely protrusive The premaxilla and prolabium can be of variable size The columella is deficient/almost nonexistent The palatal shelves are collapsed

34. Protrusive maxilla  imperative to be repositioned Premaxillary orthopedics with inraoral aplliance Denture adhesive Elastic strap

35. Latham Appliance

36. Rule of Ten Primary repair- repaired at approximately 10 weeks The surgeon usually uses the “Rule of Ten” The child weighs 10 pounds The child has a hemoglobin of at least 10 grams The child has a white count of no higher than 10,000 The child is at least 10 weeks of age

37. Surgical Techniques Cleft Lip Repair unilateral rotation-advancement flap developed by Millard complications dehiscence infection excess tension

38. Surgical Techniques Cleft Lip Repair bilateral bilateral rotation advancement with attachment to premaxilla mucosa

39. Cleft Palate Repair - Timing Dorf and Curtin 10% occurrence of articulation errors when palatoplasty was completed by 1 year 86% incidence of articulation errors when repair was complete after 1 year Haapanen and Rantala – Significantly fewer children in the groups repaired before 18 months had hypernasal speech, articulation errors, or required secondary surgery to correct speech

40. Cleft Palate Repair Schweckendick’s Primary Veloplasty V-Y Pushback Von Langenbeck Palatal Repair Furlow Palatoplasty

41. Cleft Palate Repair Schweckendick’s Primary Veloplasty Incisions made in soft palate Muscle bundles released from the posterior hard palate and rotated Reconstruction of levator sling Closure of mucosal layers separately

42. Cleft Palate Repair V-Y Pushback (WARDILL OPERATION) Two uni-pedicled flaps (greater palatine artery) and one or two anteriorly based pedicled flaps Posterior flaps rotated in a V-Y advancement technique - increasing the length of the palate Nasal mucosa not closed Improved speech results compared with bipedicled techniques Indicated for incomplete clefts

43. Von Langenbeck Operation:

44. Cleft Palate Repair Furlow Palatoplasty Lengthens the soft palate Reconstructs the muscle sling. Also commonly used to correct velopharyngeal insufficiency in patients with submucous cleft palate Speech outcomes are improved compared with other palatoplasty techniques.

45. How do you manage ear disease? 96% of children with cleft palate required tympanostomy tube placement 50% of these children required repeat tympanostomy tube placement. Frequency of otitis media decreases as the child with CP ages Audiology and tympanometry as well as exams / clinical history

46. Orthodontic Treatment of Transitional Dentition The purpose  the dentition adjacent to cleft has to be orthodontically repositioned to prepare the cleft side for the secondary alveolar bone graft Preparing the maxillary arch for a bone graft (6-12 months) : Bonded edgewise appliance Supported with a maxillary expander quad helix expander

47. Alveolar Bone Grafting Primary Bone Grafting Bone graft done at the time of primary cheiloplasty Bone graft done during the first 2 years of life Bone graft done prior to the eruption of the primary canine

48. Secondary Bone Grafting Done before eruption of the permanent canine Usually when the root of the canine is 1/3 to 2/3 formed Usually between ages 8-10 In CLP dental age is usually behind chronological age

49. Quadhelix to expand prior to ABG

50. angle brackets to keep roots away from cleft

51. Alveolar Bone Grafting Provide bone for the eruption and/or orthodontic repositioning of teeth Closure of oro-nasal fistulas Support and elevation of the alar base Stabilization of the pre-maxilla in bilateral cases Provide continuity of the alveolar ridge

52. Types of bone grafts Autogenous Cancellous- iliac crest Cortical- calvarium, mandible Cortico-cancellous- iliac, rib, tibia, mandible *Allogeneic Graft resorbs, remodels, may contribute to osteoinduction and osteoconduction **Alloplast Bone grows into, around alloplast No active osteoinduction but some osteoconduction Teeth do not erupt through alloplast *Nique T, Fonseca RJ, et al: Particulate allogeneic bone grafts into maxillary alveolar clefts in humans- A preliminary report. J Oral Maxillofac Surg 45: 386-392, 1987 **Horswell BB, El Deeb M: Nonporous HA in the repair of alveolar cleft defect in a primate model. J Oral Maxiilofac Surg 47:946-952, 1989

53. Alveolar Bone Grafting

54. Alveolar Bone Grafting Preoperative Cleft Defect Postoperative Bone Graft

55. Correction of anterior crossbite Arch expansion Quad Helix Expansion screws Orthodontic Treatment For Permanent Dentition

56. Orthognathic Surgery Midfacial Advancement LeForte osteotomies leave vascular pedicle attached in back of maxilla - prevents necrosis

57. Rhinoplasty Rhinoplasty standard techniques tip projection alar rotation columellar length Age =17-20

58. Surgical Management of Velopharyngeal Insufficiency Major Goals of Surgery Close the gap or hole between the roof of the mouth and the nose. Reconnect the muscles that normally make the palate work. Make the repaired palate long enough so that when the muscles are working, the palate can perform its function properly.

59. Velopharyngeal Insufficiency VPI

60. Wardill Operation: WY push back

61. Dorrance and Brown’s – U shaped push back palatoplasty

62. Prosthetic Management of VPI

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