1. 12 th July 2008 Dr. Christine Underhill Orthodontic Management of Cleft Lip and Palate Patient Dr. Christine Underhill

2. 12 th July 2008 Dr. Christine Underhill AGENDA What is Cleft Lip and Cleft Palate ? Embryology Orthodontic Treatment –Infant –Deciduous dentition –Mixed dentition, prior to ABG –Permanent dentition, with or without jaw surgery. Q&A

3. 12 th July 2008 Dr. Christine Underhill Basic Facts Orofacial clefts are the most common oro- facial anomaly in newborn infants and the second commonest congenital abnormality. Affecting approx one in every 800 births worldwide Non “life-threatening” abnormality, which can have significant effect on maternal bonding. A small proportion of oro-facial clefts may be associated with a genetic syndrome

4. 12 th July 2008 Dr. Christine Underhill Types of Clefts Oro-facial clefts include the cleft of the lip with or without cleft palate (CL[P]) and the isolated cleft palate (CP) CL[P] CP

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6. 12 th July 2008 Dr. Christine Underhill Gender For Clefts affecting the Lip only or Lip and Palate (CL[P]), males are more commonly affected than females (approx 2-1). Clefts palate alone (CP) is found in approx 1 in 2000; females are more often affected than males.

7. 12 th July 2008 Dr. Christine Underhill CL[P] varies between different ethnic populations –Highest rates reported for Native Americans 3.6 per 1000 –Lowest rates for African-Americans 0.4 per 1000 CP only is reported as being generally more stable across different ethnic populations. (0.6-0.8 per 1000 live births) Incidence

8. 12 th July 2008 Dr. Christine Underhill CL[P] and CP are developmentally and genetically different hereditary factors appear to play a more important role in the occurrence of CL[P] and environmental factors in that of CP

9. 12 th July 2008 Dr. Christine Underhill Incidence in Fiji Not seen data for Fiji, but….Epidemiologic survey on oral diseases in Fiji. V. Incidence of the cleft lip and palate. J Osaka Univ Dent Sch. 1986 Dec;26:249-53. Usui MUsui M, Tsunemitsu A, Sobue S, Nakagawa H, Shizukuishi S, Morisaki K, Ohmae H, Pal V.Tsunemitsu ASobue SNakagawa H Shizukuishi SMorisaki KOhmae HPal V

10. 12 th July 2008 Dr. Christine Underhill Orofacial clefts Approx 40% presented with CP only Approx 60% presented with CL[P]

11. 12 th July 2008 Dr. Christine Underhill Of those with CL[P]….. approx 30% involved the lip+/-alveolus, and 70% involved lip and palate

12. 12 th July 2008 Dr. Christine Underhill Of those with CP..... approx 30% involved the soft palate only and 70% involved hard and soft palate

13. 12 th July 2008 Dr. Christine Underhill Risk Factors for Orofacial clefts There is a known risk in taking certain types of drugs during pregnancy e.g. Phenytoin, sodium valproate, benzodiazepines and corticosteroids. There may be a link to maternal smoking whereby the risks for clefts are increased among foetuses lacking enzymes involved in the detoxification of tobacco-derived chemicals. Alcohol use, and specifically type, may also be a factor. There is debate on the role of folic acid; there may be dose dependency.

14. 12 th July 2008 Dr. Christine Underhill Embryology The lip has usually formed by 5-6 weeks of intrauterine life. The palate has formed by 10 weeks The cleft may be picked up by high resolution ultrasound at 20 weeks gestation. Diagnosis is otherwise made after delivery.

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17. 12 th July 2008 Dr. Christine Underhill facial morphogenesis In facial morphogenesis, neural crest cells migrate into the facial region, where they form the skeletal and connective tissue and all dental tissues except the enamel. Vascular endothelium and muscle are of mesodermal origin (Cohen, 2000). The upper lip is derived from medial nasal and maxillary processes. Failure of merging between the medial nasal and maxillary processes at the fifth week of embryonic development, on one or both sides, results in CL.

18. 12 th July 2008 Dr. Christine Underhill CL occurs when an epithelial bridge fails, due to lack of mesodermal delivery and proliferation. CL usually occurs at the junction between the central and lateral parts of the upper lip on either side. The cleft may affect only the upper lip, or it may extend more deeply into the maxilla and the primary palate. (Cleft of the primary palate includes CL and cleft of the alveolus.) If the fusion of palatal shelves is impaired also, the CL is accompanied by CP, forming the CLP abnormality. In general, patients with clefts have a deficiency of tissue and not merely a displacement of normal tissue.

19. 12 th July 2008 Dr. Christine Underhill Clefts of the primary palate occur anterior to the incisive foramen. Clefts of the secondary palate are due to lack of fusion of the palatal shelves, and always occur posterior to the incisive foramen

20. 12 th July 2008 Dr. Christine Underhill Development of the Face (I) 5 facial primordia Frontonasal prominence Paired maxillary prominences Paired mandibular prominences Surround primordial mouth (stomodeum) Neural crest: source for almost all connective tissues in the face Frontonasal prominence forms forehead and nose and a short margin of mouth Lower jaw and lip form first Nasal placodes (and pit): surrounded by medial & lateral nasal prominences Nasal pit remains connected to mouth Maxillary prominences grow toward each other, pushing nasal prominences Medially

21. 12 th July 2008 Dr. Christine Underhill Development of the Face (II) Medial nasal prominences merge with each other and with lateral nasal & maxillary prominences Nasolacrimal groove: between lateral nasal and maxillary prominences Becomes nasolacrimal duct Intermaxillary segment Merger of medial nasal prominences Gives rise to philtrum, premaxillary bones, primary palate From Moore, 1982

22. 12 th July 2008 Dr. Christine Underhill Development of the Palate: 1. Primary Palate Palatal development begins in week 5, but weeks 6-9 are most critical Formation of intermaxillary segment from merged medial nasal prominences Primary palate forms from median palatine process Ossifies as the premaxillary portion of the maxilla

23. 12 th July 2008 Dr. Christine Underhill CP is a partial or total lack of fusion of palatal shelves. It is thought to occur in a number of ways: Defective growth of palatal shelves Failure of the shelves to attain a horizontal position Lack of contact between shelves Rupture after fusion of shelves The secondary palate develops from the right and left palatal processes. Fusion of palatal shelves begins at the 8th week of the foetal period and continues usually until the 12th week. One hypothesis is that a threshold exists beyond which delayed movement of palatal shelves does not allow closure to take place, and this results in a CP.

24. 12 th July 2008 Dr. Christine Underhill Development of the Palate: 2. Secondary Palate Lateral palatine processes Ingrowths from maxillary prominences Eventually project horizontally above the tongue Fuse with each other, primary palate and nasal septum Nasal septum Downgrowth of med. nas. promin. Fusion with lateral palatin processes starts anteriorly, then moves back Hard palate Primary palate: premaxilla Lateral palatine processes: maxilla Soft palate: unossified portion of lateral palatine processes

25. 12 th July 2008 Dr. Christine Underhill Treatment Cleft Lip and Palate patients present a multitude of problems: Functional Impairment Suckling Swallowing Speech Hearing Malocclusion

26. 12 th July 2008 Dr. Christine Underhill Aesthetic Compromise Physiological implications Sociological implications

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28. 12 th July 2008 Dr. Christine Underhill Long list of list of procedures these children undergo Neonatal orthopaedics-orthodontist 3-12 months repair of lip and anterior maxilla- plastic surgeon 9-18 months repair palate-plastic surgeon 5 yrs revision of lip repair-plastic surgeon Grommets placed due to ‘glue ear’- ENT 7-10 yrs orthodontics/ ABG / repair OAF- oral surgeon 12-18 yrs orthodontics/orthognathic surgery

29. 12 th July 2008 Dr. Christine Underhill 11 month old girl cleft lip repair

30. 12 th July 2008 Dr. Christine Underhill Successful treatment requires a multidisciplinary approach No doubt this is required.…however, no consensus in sequence and timing due to: –Lack of randomised clinical trials comparing outcomes, effects of timing etc –Treatment sequence and timing not always problem specific.

31. 12 th July 2008 Dr. Christine Underhill Orthodontic treatment:- performed at different stages of development. Neonatal maxillary orthopaedics as an infant Orthodontic-orthopaedics in deciduous dentition. Orthodontics in the mixed dentition. Orthodontics alone or in conjunction with maxillofacial surgery (+/_distraction osteogenesis) in the permanent dentition. (Patients with cleft of lip only or soft palate only, defect will not effect dentition.)

32. 12 th July 2008 Dr. Christine Underhill Neonatal Orthopaedics Performed on new born before surgical repair of lip. Rationale...realignment of the collapsed segments before surgery Either simple passive, active orthopaedic, extra orally activated, pin retained Considerable debate re true long-term benefits but…… Definitely makes lip and anterior palate surgery easier at the time.

33. 12 th July 2008 Dr. Christine Underhill Pre surgical plates, moulding plates, feeding plates…….

34. 12 th July 2008 Dr. Christine Underhill Feeding plates to assist in early feeding Obturator plate

35. 12 th July 2008 Dr. Christine Underhill NAM Nasoalveolar moulding

36. 12 th July 2008 Dr. Christine Underhill Orthodontic orthopaedic treatment in the deciduous dentition In vicinity of cleft alveolus ….delayed eruption, malformation or absence of deciduous lateral incisor Unilateral or bilateral cross bites often present…often associated with functional shift. soft tissue drape often disguises skeletal defect at this stage. Treat with expansion now.. Together with protraction headgear to ‘develop the maxilla’ Definitely effective at the time…can exhaust patient cooperation No solid data that in the long term benefits

37. 12 th July 2008 Dr. Christine Underhill Mixed dentition…common since event of alveolar bone grafts (ABG) Requires careful assessment of problem, risks and benefits Timing of treatment closely related to timing of planed bone graft.. either before lateral incisor erupts (argued can effect maxillary growth), or before canine erupts When root of canine 1/3 to ½ developed. Orthodontic treatment involves expansion to develop favourable arch form, alignment..care not to move roots into cleft defect.. correct root angulation post grafting

38. 12 th July 2008 Dr. Christine Underhill Repaired cleft palate in 8 year old

39. 12 th July 2008 Dr. Christine Underhill Quadhelix to expand prior to ABG

40. 12 th July 2008 Dr. Christine Underhill angle brackets to keep roots away from cleft

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42. 12 th July 2008 Dr. Christine Underhill Alveolar bone grafting (ABG) Provides continuity of alveolar ridge… Provides bone for canine to erupt Osseous support for adjacent teeth Majority of canines erupt spontaneously…others require surgical exposure often in combination with orthodontics. The erupting teeth often appear to then stimulate the formation of new alveolar bone

43. 12 th July 2008 Dr. Christine Underhill Elimination of the residual alveolar cleft by secondary bone grafting and subsequent orthodontic treatment. Cleft Palate J. 1986 Jul;23(3):175-205 Bergland O, Semb G, Abyholm FE. Bergland OSemb GAbyholm FE Assessed 450 grafted cleft sites. In 292 of the cases, the canine had reached its final position in the arch, which allowed a four-group semi quantitative assessment of the newly obtained interdental septum on dental radiographs. The best results have been achieved in cases where the bone graft was carried out prior to the eruption of the canine. In this group, a normal (category I) interdental septal height was achieved in 64% Slightly lower (category II) interdental septal height in 32% Interdental septal height classified as type I and II were considered to be acceptable (96%) The cleft space was closed in 90 percent of the cases. No significant difference between unilateral and bilateral cases was found. When the same procedure was carried out after eruption of the canine, the results were less favorable

44. 12 th July 2008 Dr. Christine Underhill Long-term results after secondary bone grafting of alveolar clefts J Oral Maxillofac Surg. 1987 Nov;45(11):913-9 Enemark H, Sindet-Pedersen S, Bundgaard M. Enemark HSindet-Pedersen SBundgaard M The aim of this study was longitudinally to evaluate the treatment results after secondary bone grafting in 224 cleft patients with an observation period of more than four years. Group A included 94 patients with a mean age of 10 years, operated before eruption of the canine; group B included 72 patients with a mean age of 13.1 years operated after eruption of the canine; and group C included 58 patients operated after the age of 16 years (mean age, 20.4 years). The evaluation of the treatment results included longitudinal comparison of marginal bone level, periodontal status on cleft-related teeth, dental status in the bone grafted region, aesthetical and functional properties of the reconstructed alveolar process, as well as the influence on growth of the maxilla.

45. 12 th July 2008 Dr. Christine Underhill erupted canine

46. 12 th July 2008 Dr. Christine Underhill Orthodontics in the permanent dentition Orthodontics alone... adolescent. Orthodontics in conjunction with orthognathic/distraction surgery... adults. Long term retention especially important. Long term stability of results???…

47. 12 th July 2008 Dr. Christine Underhill Adolescent treatment

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49. 12 th July 2008 Dr. Christine Underhill Adult treatment- Orthognathic surgery

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56. 12 th July 2008 Dr. Christine Underhill In Summary Oro facial clefts require a multidisciplinary approach Treatment extends over many years and risks exhausting patient cooperation Need to keep the patients best interests in mind.

57. 12 th July 2008 Dr. Christine Underhill Thanks I would like to thank: Dr Kit Chan, Consultant Orthodontist at the Westmead Children's Hospital in Sydney. Dr Peter Fowler, Consultant Orthodontist, Christchurch Hospital New Zealand. for all their help, advise, and photo’s ! My husband and sons for teaching me how to use power point!