Cleft lip and palate

cleft lip and palate

1. Management of cleft lip and palate Role of pedodontist in the management of cleft lip and palate

2. Contents Introduction History Definition Classification Prevalence T heories Etiology Development of palate Clinical features Parental attitude Management Treatment plan

3. INTRODUCTION Congenital abnormal space or gap in the upper lip, alveolus and palate  Most common congenital deformity at birth Earlier numerous misbelieves associated with cleft Problems are cosmetic, dental, speech, swallowing, hearing, facial growth, emotional HISTORY Believed due to solar eclipse ,bad omen. Pare – obturators in 1561 Le monnier-1 st surgical repair of cleft palate 1764 American cleft palate association-1943

4. Definition Cleft lip : congenital deformity of the upper lip that varies from a notching to a complete division of lip,any degree of clefting can occur. Cleft palate : a congentital split of palate that may extend to uvula,soft palate and into hard palate,the lip may or maynot involve in the cleft of the palate Obturator : a plastic ,acrylic appliance usually removable used to cover a cleft or a fistula in the hard palate or help to achieve velopharyngeal closure in order to promote clear speech

5. Prevalance Overall-0.3 to 6.5 /1000 live birth More common in south far Asians:1 in 500 Less frequent in Africans: 1 in 2000 Prevalence in Europeans and Americans: 1 in 750 Prevalence in Pakistan: 1 in 523 Boys are more affected than girls by 3:2 Cleft Lip and Palate occur twice as often in boys as in girls Isolated Clefts of Palate are more often in girls75 % of Clefts are Unilateral, rest are Bilateral Left side is more involved than right side 400+ syndromes associated

6. Etiology Hereditary enviornment : rubella virus etc Mutant gene: mendelian inheritance Chromosomal: trisomy 21 Increased maternal age Decreased blood supply to nasomax region Decreased folic acid and vit A Multifactorial

7. Cleft palate Insufficient suckling Escape of food in to nose Fatigue Inadequate milk intake Excessive air swallowing Aspiration During feeding   Nasal regurgitation Irritability Hunger Poor weight gain Vomiting Coughing and choking Problems associated with cleft lip & palate  

8. Development of the Palate: 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

9. Development of 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

10. Theories Dursy his hypothesis: failure of fusion b/n median nasal and maxillary process Veau’s hypothesis: failure of ingrowth of mesoderm b/n 2 palatal shelf Non-fusion of shelves Alterations in palatal forces Fusion of shelves with subsequent break down Excessive tongue resistance excessive head width Failure of tongue to drop down Inclusion of cyst pathology

11. Classification Davis and Ritchie Veau’s classification Foghanderson Kernahans and starks American cleft palate association Karnahan’s y striped Classification Millard’s Modification of Karnahan’s Classifcation Pfiefer classification Tessier classification Based on embryology Graphic method of recording

12. Davis and Ritchie Group 1- prealveolar cleft Group 2-Post alveolar cleft Group 3-alveolar cleft Veaus classification Group 1-cleft of soft palate Group 2-cleft of hard and soft palate to incisive foramen Group 3-complete unilateral cleft of soft,hard palate,lip,alveolar ridge Group 4-complete bilateral cleft of soft,hard palate,lip,alveolar ridge Anderson classification Hare lip-alveolus and back as incisive foramen Hare lip and cleft palate Isolated cleft palate as forward as incisive foramen

13. Kernahans classification Cleft of primary palate : Unilateral Complete incomplete Median Complete incomplete Bilateral Complete incomplete Cleft of secondary palate Complete incomplete submucous Cleft of secondary and primary palate Unilateral Complete incomplete Median Complete incomplete Bilateral Complete incomplete

14. Pfeiffer classification

15. Kernahans striped y classification milliards modification of striped y

16. American cleft palate association Cleft of prepalate Cleft lip Unilateral Bilateral Median Prolabium Congenital scar Cleft of alveolar process : Unilateral Bilateral Median Cleft of prepalate : Prepalate protrusion Prepalate rotation Prepalate arrest Cleft of palate: Cleft of soft palate:extent palatal shortness Submucous cleft Cleft of hard palate: Extent:vomer attachment Submucous cleft

17. Syndromes associated Autosominal dominant: Vander woude syndrome EEC syndrome Larsen syndrome Autosomal recessive : Conradi syndrome Mekels syndrome Orofacial digital syndrome type 2 Fryns syndrome X linked Orofacial digital syndrome type 1 Isolated x linked cleft palate with ankyloglossia chromosomal Trisomy 13 Trisomy 18 Non mendelian Pierre robin sequence Clefting with congenital heart disease

18. Clinical features Common finding-natal neonatal teeth (cleft palate) absent lateral incisor missing premolar supernumerary teeth ectopic lateral incisor Palatal eruption of per. Canine Anomalies like enamel hypoplasia, microdontia,macrodontia,fused teeth over bite Lateral facial profile protruberance and mobile premaxilla in infants Bilateral cleft Posterior cross bite in u/b cleft palate Rotated per.incisor Premature loss deficiency of alveolar bone

19. Hearing loss : Hearing loss and middle ear disease Otitis media- early , in 1 st month of life Prolonged middle ear effusion →permanent damage to the middle ear Speech difficulties : Retardation of consonant sounds (p, b , t, d, k, g) Hindrance to early vocabulary development. Hyper nasality – cleft of SP Hearing problems contribute Nasal deformities : Cleft extending to floor of nose- alar cartilage flared , Columella pulled to non cleft side Lack of underlying bone support - ↑ problem Surgical corrections- deferred until all other procedures are completed - as correction of alv clefts, max skeletal retrusion - alter osseous foundation of nose.

20. Parental attitudes Weatcher 1959 Child appearance Request for immediate surgery Speech development feeding Reaction of the spouse Action of siblings Reaction and family and friends Intellectual development Financial problems Recurrence of the defect in other unborn children

21. DIET Milk , cooled boiled water- suitably safe drinks Training beaker, cup – at 6 months Weaning foods/drinks – sugar free, avoidance of pacifiers TOOTH BRUSHING Potential area for plaque around teeth, cleft region. After upper lip surgery- how to lift it, stretching lip carefully sliding an index finger along labial gingiva without any damage to scar Small size head ideal – lack of sulcus depth, awkward tooth position Interspaced tooth brush- crowding, bilateral clefts with upper anterior retroclined low fluoridated toothpaste(600ppm) - Below 6 yrs High risk caries pts - 1000ppm Twice daily brushing USE OF FLUORIDE Fluoride supplements 1. fluoride content local water supply 2. likelihood of compliance 3. caries experience of pts and other family members Fluoride varnish, 5% NaF USE OF PERSONAL CHILD HEALTH RECORD information on child health growth development from birth

22. MANAGEMENT OF NEONATE 2 kinds of problems a) hazards affecting the child - neonatal respiratory obstruction b) feeding difficulties NEONATAL RESPIRATORY OBSTRUCTION Most babies- no problems Infants born - very small & post displaced mandible , tongue falling backwards →severe potentially lethal obstrucn to airway. Ex : Pierre robin syndrome. MANAGEMENT : Bottle feeding- 3 quarter prone position with head turned to the side. Nurse thumb – behind angle of mandible holding it forward. Haber man feeder is recommended. std bottle ,soft variable flow teat, pump action valve – no active suction. If signs of aspiration- avoid feeding Use of Nasogastric tube ( Mc Evitt 1973 ) Lamb’s nipple – regular bottle , using base as washer

23. Feeding Breast feeding should be encouraged Feeding by bottle rather than spoon – encouraged, biting action of lower lip , jaw function , development Nostrils – cleaned. Lips- well lubricated with Vaseline If orthopedic splint worn – remove before feeding, clean it , place it , then feed Tapes – checked for dry, cleaned firmly attached to elasto plast base on cheeks Splints- foundation plaster removed only when it is soiled, with olive oil POSITION FOR FEEDING : A semi-upright position so that the milk will flow downward into the baby's stomach Feeding the baby in a reclining position - contamination of the middle ear and ear infections. Always the baby’s head – higher than his stomach.

24. Traditional methods of alternative methods of feeding Ex: Spoon feeding, traditional devices Earlier-choice of feeding in cleft infants now it is not recommended, it is laborious and time consuming. Modified cup feeding devices : Ex: Baby cup feeder, soft feeder Special feeding teats : MAM vented teat Pigeon cleft palate nipple new born teat Nuck cleft palate teat MAM soft teat tapered teat teat with air vent normal teat with cross cut or Y-cut.

25. MOIDIFIED FEEDING BOTTLES Mead Johnson bottle: soft bottle, any std teat. Haber man feeder – perrie robin syndrome. std bottle, soft variable flow teat, pump action valve – no active suction Lact -aide device- Used in Cleft lip palate Used after lip closure. Delivers the milk through a small tube while the infant is placed at mothers breast. Regular breast feeding or bottle feeding do not work well Unable to seal their lips, velvopharynx Cannot develop sufficient suction on bottle nipple.

26. Isolated cleft palate : a ) breast feeding b) bottle feeding using soft nipple with enlarged outlet. c) commercially used cross cut (juice) nipples . Cleft of the soft palate Correctly shaped, regular nipple . Cleft lip only a ) breast feeding b )large nipple size.

27. MANAGEMENT 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

28. PEDIATRICAN: family physician – responsible for pt overall health assess pt physiological status, developmental milestone PEDODONTIST : Routine prophylaxis, fluoride treatment mandatory Fluoride supplements rinses, dentifrices Close communication b/ n other team members . ORTHODONTIST : obtains records OPG study models diagnostic photographs . Provides comprehensive ortho care ORAL & MAXILLOFACIAL SURGEON : Surgically alter skeletal relationship of maxillo Mandibular complex and repair cleft lip and palate MAXILLOFACIAL PROSTHODONTIST : replaces, restores, rehabilitates– congenitally missing or malformed orofacial structures

29. GENETIC COUNSELLOR : examines- syndromes associated with CLP assess risk factor for future offspring surgeon – first member to counsel parents Printed hand outs given to parents Pre &post operative Photos of similarly affected children- helpful SOCIAL WORKER : Acts as pt ` s advocate Aids in psychological assessment PLASTIC SURGEON : Timing of surgery responsible for obtaining alv bone grafts Nasopharyngeal examination of speech pharyngoplasty - improve velvopharngeal function correct internal nasal deformities

30. PSYCHATRIST & PSYCHOLOGIST : Evaluate pt – strength, weakness in cognitive interpersonal, emotional behavioral & social development . Emphasis – cope with emotional, physical stress SPEECH PATHOLOGIST : monitor speech output Offers therapeutic option – maturation of speech AUDIOLOGIST : performs test- hearing difficulties perform - middle ear surgery if needed ROLE OF NURSE : Advisor , support family during time of anxiety Daily care of infant Teaches mother- care of nose, facial skin, cleaning of splints at each feeding time Actively communicate with team members . Emphasis total family involvement not just treatment of pt

31. Treatment plan Stage 1:Maxillary orthopedic stage Birth to 18 months Management of CLP- immediate attention of new born. Feeding problems- difficult to maintain adequate nutrition . Numerous prosthetic devices. Mc Neil (1950) devised , Intra oral maxillary obturator Plates pumps and nipples

32. INTRA ORAL MAXILLARY OBTURATOR : Provides a false palate, reducing feeding difficulties in infants preventing arch collapse Mold the cleft segments into approximation before primary alveolar bone grafting. create sufficient – ve pressure, which allow adequate sucking of milk. Instructions to parents: Recall the pt after 2 days. Appliance – approximate 3 month until the time of initial lip closure

33. Clinical management of initial obturator therapy (birth to 3 months). appliance positioned in the infant’s mouth excessive pressure areas identified by acrylic resin are identified Donot impinge on resin Parents instructed on placement and removal of the appliance and its daily cleaning . infants are usually seen for adjustments 2 days after initial appliance delivery Monthly observations scheduled. In most cases, this appliance will serve until the time of initial lip closure at approximately 3 months of age. The major advantage of the obturator during this stage of treatment is to enhance the child’s ability to obtain nourishment.

34. Initial obturator therapy :: birth to 3 months obturators used appliance cleaned before and after feed Presurgical orthopedics : birth to 5 months Aims achieve upper arch that conforms to lower arch Premaxillary retraction tape required in case of anteriorly placed premaxilla Surgical lip closure : 3 to 9 month Best time for lip closure Rule of 10 types of lip repair are- milliard repair tennison randal reapir veau repair rose Thompson repair Le Mesuirier technique Surgical plate repair : 10-18 months time of palatal repair Repair done early-good esthetics Repair latre -facial growth better,poor esthetics Rule of Ten Primary repair- appx 10 weeks weighs 10 pounds hemoglobin of at least 10 grams white count of no higher than 10,000 at least 10 weeks of age

35. Le Mesuirier technique tennison randal reapir

36. milliard repair

37. There are 2 types of palatal repair: Single stage: Von langenbeck repair V-y pushback palatoplasty Carried out of 1hf year Two stage repair : 18 months hard palate at 4yrs Schweckendiek procedure Performed In 2 Steps first soft palate-closure ( staphylorrhaphy ) Closure- 3 layer step a ) nasal mucosa b )muscle c) oral mucosa - second hard palate closure ( uranorrhaphy ) Often done after deciduous dentition is completely erupted

38. VON LANGENBECK OPERATION : Uses- relaxing incision close to dentition Soft tissue –sutured in water tight manner over cleft defect & allowed to heal Bone areas exposed by lateral relaxing incision- heal by secondary intention Mucosa from margin of cleft - Removed Mucoperiosteal flaps- lateral releasing incisions Sutures placed into nasal mucosa Nasal mucosa has been closed

39. Frontal section – Repair of nasal mucosa Closure of oral mucoperiosteum

40. Stage 2 :primary denition stage 18 months- 5 years) Adjustments to obturators Restoration of decayed teeth Maintaince of oral hygiene Evaluationg erupting dentition Stage 3:mixed dentition stage ( 6 -10 \ 11 years) main problem:ectopic eruption of teeth and malalignment Correction of crossbite Maxillary expansion 2ndary grafting Stage 4 permanent dentition stage: 12 - 18 years) Fixed orthodontic treatments Cosmetic repair Cleft with severely retrusive maxilla - LeFort I maxillary advancement .

41. Reference Textbook of pedodontics Nikhil marwah macdonalds shoba tandon