Cleft Palate.pptx

Seminar on Cleft palate management

1. Cleft Palate Presented by: Dr. Neha Umakant Chodankar PG OMFS

2. Contents Introduction History Embryology Epidemiology Genetics and Etiology Associated Syndromes Anatomy Classification Clinical Features and Evaluation Treatment Planning and Timing Cleft palate surgery Complications Secondary deformities of cleft palate Conclusion References

3. Introduction The comprehensive treatment of cleft lip and palate deformities requires thoughtful consideration of the anatomic complexities of the deformity and the delicate balance between intervention and growth. Goals of surgical care: Normalized esthetic appearance of the lip and nose Intact primary and secondary palate Normal speech, language, and hearing Nasal airway patency Class I occlusion with normal masticatory function Good dental and periodontal health Normal psychosocial development

4. History 1 st recorded operative treatment of only cleft lip -- Chin ( Tsin ) Dynasty ( c 390 AD ) The first diagrammatic representation -- Ambrose Pare in the 14th century The first cleft palate repair --by a dentist, Le Monnier , in 1766 in Paris John Stephenson (1797–1842), a physician --wrote the earliest recorded description of Palatoplasty performed by Philibert Roux ( 1780–1854 Paris)-- the primary repair of velum at age of 22 Roux later published his account of the procedure in 1825, --“ staphyloraphie ” Carl Ferdinand von Graefe (1787–1840), proclaimed that he , was the first surgeon to perform velar closure in 1819. Von Graefe eventually reported a successful repair in 1820.

5. The first successful closure of the soft palate in America --John Collins Warren in 1820 in Boston . Johann Friedrich Dieffenbach ( 1792–1847 Berlin ) -expanded the technique of soft palate repair to include closure of the hard palate. Bernhard von Langenbeck (1810–1887) --first to describe the mucoperiosteal plane of dissection and to use its advantage in mobility to cleft palate closure. In the mid 19th century, Hullihen , recognized as the father of American oral and maxillofacial surgery, published a treatise on comprehensive care of cleft lip and palate deformities.

6. von Langenbeck (1859, 1861) extended these concepts, and described a Bipedicled flap procedure Veau (1931) converted the bipedicled flaps of von Langenbeck to single pedicled flaps based on greater palatine vessels, and emphasized the need for palatal lengthening Wardill (1937) and Kilner (1937) modified Veau's procedure Dorrance and Barnsfield (1946) described the use of a skin graft in the raw palatal surface after the procedure .

7. Embryology The failure of fusion of the frontonasal and maxillary processes at 6 weeks IUL, gives rise to the cleft of the primary palate, which includes the lip, alveolar process, and the hard palate anterior to the incisive foramen.

8. The lateral palatal shelves fuse later than the primary palate, around 7–8 weeks gestation They rotate from vertical to horizontal orientation. This fusion proceeds from anterior to posterior, which helps to understand the spectrum of clefts of the secondary palate.

9. Epidemiology Clefts of the upper lip and palate are the most common major congenital craniofacial abnormality ---1 in 700 live births Bilateral clefts of the lip are most often associated primary and secondary cleft palates Cleft palate alone is seen in approximately 1 in 2,000 live births and this incidence is similar in all racial groups In the majority of cases unilateral cleft lip and palate is an isolated nonsyndromic birth defect that is not associated with any other major anomalies

10. Genetics and Etiology Cleft lip and palate is not considered a single gene disease, although inheritance may play a role--- multifactorial These factors may include chemical exposures, radiation, maternal hypoxia, teratogenic drugs, nutritional deficiencies, physical obstruction, or genetic influences. Recently multiple genes have been implicated in the etiology of clefting --- MSX , LHX , goosecoid , and DLX genes Disturbances in growth factors or receptors—FGF, TGF, PDGF and EGF

11. Associated Syndromes Stickler’s syndrome Van der Woude’s syndrome DiGeorge syndromes Treacher Collins Syndrome 22q deletion Syndrome Pierre Robin Sequence

12. Anatomy

13. Classification Based on morphology I Davis & Ritchie's classification II Veau ' s classification Based on embryology 1. Fogh - Anderson classification 2. Kernahan's & Starks classification 3. American cleft palate association Graphic methods of recording clefts 1. Pfiefer classification 2. Kernahan's striped Y classification 3. Millard's modification of striped Y classification

14. Davis & Ritchie's classification (1922) Group I: Prealveolar process cleft 1 . Unilateral (right/left: complete/incomplete) 2. Bilateral (right: complete/incomplete; left: complete/incomplete ) 3. Median (complete/incomplete) Group II: Postalveolar process cleft 1 . Soft palate 2. Hard palate Group III: Alveolar process cleft 1 . Unilateral 2. Bilateral 3. Median

15. Veau ' s classification (1931) Group 1- cleft of the soft palate only Group 2-cleft of the hard and soft palate to the incisive foramen Group 3-complete unilateral cleft of the soft hard palate & lip & alveolar ridge on one side Group 4-complete bilateral cleft of the soft hard palate & lip & alveolar ridge on both sides

16. Fogh –Anderson Classification (1942) 1. Harelip (single or double ) 2. Harelip with cleft palate 3. Isolated cleft palate 4. Rare atypical clefts, e.g., median cleft lip

17. Kernahan & Stark’s Classification (1958 ) Clefts of primary palate 1 . Unilateral complete/ incomplete 2. Median complete (premaxilla absent) incomplete (premaxilla rudimentary ) 3. Bilateral complete/incomplete B) Clefts of secondary palate only Complete/ Incomplete/ Submucous C) Clefts of primary and secondary palate 1. unilateral complete/incomplete 2. median complete/ incomplete 3. bilateral complete/ incomplete

18. American Cleft Palate Association Classification 1. Clefts of the prepalate (cleft of lip and embryologic primary palate ) a. Cleft lip ( cheiloschisis ) b. Cleft alveolus ( alveoloschisis ) c. Cleft lip, alveolus, and primary palate ( cheiloalveoloschisis ) 2. Clefts of the palate (cleft of the embryologic secondary palate ) a. Cleft of the hard palate ( uranoschisis ) b. Cleft of the soft palate ( staphyloschisis or veloschisis ) c. Cleft of the hard and soft palate ( uranostaphyloschisis ) 3. Clefts of the prepalate and palate ( alveolocheilopalatoschisis )

19. 4 . Facial clefts other than prepalatal and palatal a. Cleft of the mandibular process b. Naso -ocular clefts c. Oro-ocular clefts d. Oroaural clefts

20. Spina classification (1974) Group I – Pre-incisive foramen clefts Unilateral Bilateral Median (CL with or without alveolar), total or partial Group II – Trans incisive foramen clefts Unilateral Bilateral Group III – Post incisive foramen clefts Total Partial Group IV – Rare facial clefts

21. Pfiefer Classification

22. Lahshals Classification

23. Kernahan’s Stripped Y Classification 1971

24. Millard’s Modification Of Stripped Y 1976

25. CLAP notation

26. Clinical Features Cleft palate with cleft lip and alveolus Asymmetry Alveolar cleft between the maxillary lateral incisor and canine tooth roots Malposition of the maxillary lateral incisor and cuspid in both the deciduous and permanent dentition Unilateral complete cleft palate is characterized by direct communication between the entire length of the nasal passage and oropharynx

27. The nasal septum is deviated and buckled toward the cleft side The absence of a portion of the inferior piriform aperture and the hypoplasia of the lateral nasal bony platform at the maxillary wall contribute to the cleft nasal deformity the nasal base is depressed, the ala is collapsed, and the floor is wide

28. In the bilateral complete cleft lip and palate, the pre-maxillary segment containing the central and lateral incisor teeth is discontinuous from the alveolar arch The lateral segments often collapse inward and lingually , resulting in “locking out” of the premaxilla Speech problems and nasal regurgitation of fluid

29. Clefts of the secondary palate a cleft of the secondary palate may be variable, from an opening in the posterior soft palate to a cleft extending up to the incisive foramen There is almost always a separation of the bony shelves of the hard palate Most commonly, dentition is normal and symmetric.

30. Ear Pathology Chronic obstruction of drainage leads to serous otitis media, and long-standing effusion can result in hearing loss Untreated children with clefts and severe effusions may have total deafness

31. Speech The ability to partition the oropharynx and nasopharynx is crucial for normal speech production The palate elevates during production of any sounds requiring positive pressure in the oropharynx; the levator palatini is primarily responsible for this movement.

32. Speech The ability to partition the oropharynx and nasopharynx is crucial for normal speech production The palate elevates during production of any sounds requiring positive pressure in the oropharynx; the levator palatini is primarily responsible for this movement.

33. If palate function is not corrected, velopharyngeal insufficiency results When complete closure cannot be anatomically or functionally obtained, compensatory mechanisms for sound production are learned Eliminating these learned compensatory articulations is difficult, even with the best of speech and language therapy Compensatory articulations may persist even in the face of a functional palate repair, especially in later repairs or secondary correction of velopharyngeal insufficiency

34. Growth C left infants exhibit poor weight gain in early infancy Initial growth retardation by the time they undergo surgical lip repair When the same children reach the age for palatoplasty , they have significantly lagged on the growth curve Causes: --feeding difficulties early in life before palate repair intrinsic growth disturbances increased frequency of ear and airway infections multiple operative procedures growth hormone levels may be diminished

35. Growth C left infants exhibit poor weight gain in early infancy Initial growth retardation by the time they undergo surgical lip repair When the same children reach the age for palatoplasty , they have significantly lagged on the growth curve Causes: --feeding difficulties early in life before palate repair intrinsic growth disturbances increased frequency of ear and airway infections multiple operative procedures growth hormone levels may be diminished

36. Feeding and swallowing Most infants with clefts are unable to breast-feed Infants with clefting limited to the posterior velum can often use posterior tongue position to generate a partial negative seal The exception to this is the child with Pierre Robin sequence and isolated velar cleft, who can develop respiratory distress or ineffective suction from glossoptosis

37. Feeding and swallowing Infants who are unable to breast-feed because of cleft palate have a number of options for feeding: -specialty nipples such as lamb’s nipples - crosscutting of standard nipples - long soft nipples that place the liquid at the posterior tongue - Special flow bottles such as gravity flow and squeeze bottles allow the caregiver to carefully control the flow rate Children with clefts generally do not have difficulty with swallowing and aspiration unless intrinsic neuromuscular abnormality of the tongue or pharynx is present.

38. Aspiration with swallowing --diagnostic evaluation, including - thin barium swallow studies - bronchoscopy - gastroscopy Children may cough or sputter with reflux of the ingested material into the nose, particularly if volume or rate of feeding is excessive When the palate has an open cleft, food may reflux into the nasal passage Nasal reflux is irritating to the nasal mucosa and can predispose to sinusitis and ulceration regurgitation of food through the nose

39. Submucous cleft palate Calnan’s classic triad of - a midline clear zone (zona pellucida ) -a bifid uvula - a palpable notch in the posterior hard palate With contraction of velar musculature, a distinct midline muscle diastasis may be seen symptomatic with regard to speech, serous otitis media, or hearing loss

40. Patients with velopharyngeal insufficiency and submucous cleft palate on examination require full evaluation, including speech evaluation and endoscopy Even in the absence of obvious findings on clinical examination, anatomic abnormalities are found (> 90%) at the time of surgery---- occult submucous cleft palate Corrective surgical technique ---anatomic correction of the velar muscle diastasis Primary means of treatment-- pharyngeal flaps and sphincter pharyngoplasty The Furlow double opposing Z- plasty is an ideal procedure

41. 2 schools of thought Only soft palate closed 12 – 18 months Hard palate 5 -- 10 years Soft palate + hard palate -- 18 months Most severe deformity , when palate repaired between 8 – 12 years ( rapid maxilla growth) Maxillary under- development To allow maxillary growth Because speech is critical Management

42. Timing of palate repair Speech The driving force for palatoplasty is the development of normal speech Two crucial aspects of palatoplasty are important in optimal speech outcome: (1) surgical technique and (2) timing of palate repair Victor Veau first made the observation of a correlation between age at repair and speech outcome in 1931 Variables of technique, surgeon’s skill, lack of standardization of speech evaluations, and therapies preclude exact determination of optimal age at repair

43. Maxillary growth Transverse growth of the maxillary arch is narrowed---- resulting in typical malocclusion traits of crowding, lateral cross-bite, and open bite due to surgical scarring or intrinsic maxillary underdevelopment There may be a sagittal growth deficiency It is more difficult to establish normal speech in older children after cleft repair than to correct occlusion with a combination of orthodontic treatment and orthognathic surgery

44. Procedure Timing Cleft lip repair After 10 weeks Cleft palate repair 9–18 months Pharyngeal flap or pharyngoplasty 3–5 years or later based on speech development Maxillary/alveolar reconstruction with bone grafting 6–9 years based on dental development Cleft orthognathic surgery 14–16 years in girls, 16–18 years in boys Cleft rhinoplasty After age 5 years but preferably at skeletal maturity; after orthognathic surgery when possible Cleft lip revision Anytime once initial remodeling and scar maturation is complete but best performed after age 5 years

45. The primary goals of cleft palate surgery are Construction of an air- and water-tight velopharyngeal valve The three procedures used today for constructing a tight valve are ( i ) Closure of palate with reconstruction of the levator muscle sling (ii) V-Y retropositioning of the palate (iii) Simultaneous closure of palate and primary pharyngeal flap preservation of hearing preservation of facial growth functional occlusion and aesthetic dentition

46. S urgical technique P erioperative considerations -general health and the developmental status of the child - Audiology evaluation The use of a RAE endotracheal tube placement of the Dingman gag without kinking the tube Constant assessment of the airway LA infiltrated into the palate 7–10 min before incision; (maximum of 1 mL/kg is used)

47. The important aspect of surgical anatomy is the location of the greater palatine neurovascular bundle Incisions on each side are best made with the surgeon’s contralateral hand to bevel the incision away from the vascular pedicle Circumferential freeing of the palatal attachments around the pedicle and gentle stretching of the pedicle out of the foramen are essential to obtain a tension-free closure of the oral flap The goal is to obtain complete nasal and oral closure The most difficult area for closure, around the junction of the hard and soft palate, is the most common location for fistulas

48. Objectives of Palatoplasty To produce anatomical closure of the defect To create an apparatus for development and production of normal speech To minimize the maxillary growth disturbances and dentolveolar deformities Agrawal--Cleft palate repair variations S103 Indian J Plast Surg Supplement 1 2009 Vol 42

49. Principles of Palatoplasty 9-12 months of age 8-10 kgs weight Dissection under magnification Closure of the defect in 3 layers Correction of abnormal position of the muscles of the soft palate Reconstruction of the muscle sling Retro-positioning of the soft palate

50. Schweckendiek’s P rimary veloplasty Medial margin incision: the uvula Medial and lateral incisions.

51. Schweckendiek’s P rimary veloplasty Medial margin incision: the uvula Medial and lateral incisions.

52. Mucosal nasal surface Sutures through mucosa and muscle Closure of soft palate(9 mths). Suture tips of uvula together and clip suture onto head towels Evert uvula. Complete sutures on nasal surface and return on oral surface including muscle in both layers Closure of soft palate Pack with Lyostypt if there is a large deficit Undermining of mucosa at site of previous“incisions or incise and rele Incision around margin of residual defect, eversion of mucosa and closure Bipedicled flaps raised and advanced medially to close oral surface Closure of hard palate (14 mths)

53. von Langenbeck Simple approximation of the cleft margins with a relaxing incision that began posterior to the maxillary tuberosity and followed the posterior portion of the alveolar ridge

54. von Langenbeck Simple approximation of the cleft margins with a relaxing incision that began posterior to the maxillary tuberosity and followed the posterior portion of the alveolar ridge

55. The essence of the pushback repair is the central V incision on the hard palate that is then closed in a straight line, creating length on the oral side of the closure V-Y pushback ( Veau – Wardill – Kilner )

56. The essence of the pushback repair is the central V incision on the hard palate that is then closed in a straight line, creating length on the oral side of the closure V-Y pushback ( Veau – Wardill – Kilner )

57. The essence of the pushback repair is the central V incision on the hard palate that is then closed in a straight line, creating length on the oral side of the closure V-Y pushback ( Veau – Wardill – Kilner )

58. Advantages provides increased length for the palate places the levator muscle in a more favorable position Disadvantage: Large open areas are left anteriorly and on the nasal surface Heal by contraction and decrease in length Contraction of the oral mucosal defects results in loss of maxillary width anteriorly The arch may also be flattened anteriorly The closure anteriorly in a complete cleft is a single layer of nasal mucosa only, which gives rise to a higher fistula rate

59. Two-flap Palatoplasty A technique of freeing mucoperiosteal flaps from the cleft margins only

60. Two-flap Palatoplasty A technique of freeing mucoperiosteal flaps from the cleft margins only

61. Buccal mucosal flap

62. Vomer flaps one-layer (nasal) closure Incisions- through nasal mucosa on underside of nasal septum (vomer) and mucosa of cleft margins . Mucosa of nasal septum is dissected and inserted under palatal mucosa at margins of cleft and sutured . Undersurface of nasal mucosa will epithelialize

63. Vomer flaps one-layer (nasal) closure Incisions- through nasal mucosa on underside of nasal septum (vomer) and mucosa of cleft margins . Mucosa of nasal septum is dissected and inserted under palatal mucosa at margins of cleft and sutured . Undersurface of nasal mucosa will epithelialize

64. Intravelar veloplasty Victor Veau first advocated midline reapproximation of the levator palatini muscle Braithwaite was the first to perform more extensive muscle dissection and freeing of the muscle for posterior repositioning and tension-free approximation Cutting has described a technique of veloplasty that includes division of the tensor palatini tendon and repositioning of the muscle at the Hamulus This method, known as radical levator transposition, requires an extensive dissection of the levator muscle, freeing it from both nasal and oral mucosa

65. Double opposing Z- plasty

66. Postoperative care In the immediate postoperative period, breathing is the critical concern--use of a traction suture Monitoring with continuous pulse oximetry and minimizing narcotic use Adequate pain relief Observe for at least 48h for tongue edema Bleeding is not uncommon after palate repair (12–24 h) Light pressure on the hard palate repair at the end of the procedure Application of ice packs to the posterior neck Postoperative feeding is generally limited to liquids for 10 – 14 days Arm splints

67. Complications SURGICAL COMPLICATIONS ANESTHETIC COMPLICATIONS INTRAOPERATIVE COMPLICATIONS IMMEDIATE POSTOPERATIVE COMPLICATIONS LATE POSTOPERATIVE COMPLICATIONS COMPLICATIONS OF PALATOPLASTY HEMORRHAGE RESPIRATORY OBSTRUCTION HANGING PALATE DEHISCENCE FISTULAE BIFID UVULA VPI MAXILLARY HYPOPLASIA DENTAL MALALIGNMENT OTTIS MEDIA HEMORRHAGE PEDICLE LOSS FLAP TEARING TUBE DISCONNECTION DIFFICULT INTUBATION HYPOTHERMIA

68. Management of Secondary Deformities Timing : -- Symptomatic fistulas usually require surgical repair --asymptomatic fistulas can be addressed during other procedures

69. Technique: Nasal layer closure can be accomplished with T urnover flaps from the mucosa lining the fistula Mucoperiosteal vomer flaps, or from mucosa of the posterior pharyngeal wall Oral layer closure can be accomplished using local palatal mucoperiosteum Flaps may be designed unilaterally or bilaterally, elevated in either unipedicle or bipedicle fashion

70. W hen closing the oral layer with local or adjacent tissue Design of the flaps: larger than the defect there should be minimal tension on these flaps at the time of closure osteotomize the greater palatine foramen if needed islandization of hemipalatal flaps can be performed to release additional tension avoid overlapping the nasal and oral layer suture lines

71. Closure using biomaterial: - Kirschner et al. used acellular dermis ( Alloderm ) to augment palatal fistula repair by sandwiching a thin sheet between the recreated nasal and oral mucosa layers There are still situations in which a tension-free repair is not possible with palatal tissue-----regional axial pattern flaps

72. 3 intraoral tissue options for palatal fistula closure: -buccal mucosa - the tongue - the posterior pharyngeal wall The buccal tissue can also be harvested based on the facial artery This axial flap can be based either superiorly (relying on retrograde flow) or inferiorly (relying on antegrade flow ) The superiorly based flaps are primarily used to close defects involving the hard palate and alveolus, while inferiorly based flaps are used to close defects in the posterior hard palate, soft palate, and posterior portions of the alveolus

73. Advantages: - its design prevents damage to the facial nerve and Stensen’s duct its robust blood supply the flap can be designed to be long ( length-to-width ratio of 5 : 1) Flap can be folded over on to itself to form a two-layered structure

74. For particularly recalcitrant defects--the tongue flap - Initially , the flap was raised as a thick and bulky flap - Assuncao ---thin (5 mm at base, and 3 mm distally) flaps could be based anteriorly Disadvantages— the tongue must be tethered to the palate for 2–3 weeks the texture and color are a poor match changes in articulation postoperatively Second surgery needed

75. T he temporoparietal flap When local and regional flaps have failed, microvascular tissue transfer - t he radial forearm flap is the flap of choice - first dorsal metatarsal artery dorsalis pedis flaps - the osseous angular scapular flap the lateral upper arm fasciocutaneous flap Prosthetic obturation dento -osseous transport distraction osteogenesis in persistent nasoalveolar fistulas

76. Velopharyngeal incompetence The main characteristic of VPI is the hyper nasal quality of speech The procedures available for the correction of VPI are Palatal lengthening procedures Pharyngeal flaps Augmentation of the posterior pharyngeal wall Reconstruction of velopharyngeal sphincter

77. Conclusion Overall , results of cleft palate repair have improved as far as speech outcomes and is probably due to the growth of centers for cleft care as well as to refinement of techniques. The team approach has decreased the number of operations needed to obtain better outcomes as the surgeon has gained knowledge from the other specialists involved in cleft care. The increased application of methods that incorporate reconstruction of the levator palatini muscle has produced much more predictable speech results.

78. References Neligan Plastic Surgery vol 3 Petersons principles Oral and Maxillofacial Surgery 3 rd ed Text book of plastic surgery - Joseph McCarthy; VOL 4. Text book of oral and maxillofacial surgery - Peter W ard B ooth, vol 2. Textbook of Plastic Surgery Mc MCarthy AOMSI clinicians manual Slideshare