Cleft lip and palate is a congenital birth defect characterized by a complete or partial cleft of the lip and/or palate. It has an incidence of 1 in 700 live births according to WHO. The document discusses the epidemiology, embryology, classification, diagnosis, associated problems, and management of cleft lip and palate. Management involves a multidisciplinary approach across various stages from birth through adulthood and includes surgical and non-surgical treatments such as feeding adaptations, speech therapy, dental treatment, and reconstructive surgeries.

1. CLEFT LIP AND PALATE
Pushpa Lal Bhadel
Department of Pediatric surgery
Kathmandu Model Hospital

2. Introduction
 The dictionary meaning of cleft is a crack, fissure, split or gap.
 Cleft lip and palate is a congenital birth defect which is characterized
by complete or partial cleft of lip and/or palate.
 Cleft lip and palate is the second most common congenital anomaly
after clubfoot.
 Generally divided into cleft lip (CL), cleft palate (CP) and cleft lip with
cleft palate (CLP).

3. Epidemiology
 According to WHO(2001) 1:700 live births have oral clefts of any form.
 International estimates: cleft lip with or without cleft palate range from
7.94 to 9.92 per 10,000 live births
(IPDTOC, 2011; Tanaka, Mahabir, Jupiter, & Menezes, 2012)
 Unilateral clefts accounting for 80% of incidence and bilateral for remaining
20%.
 Among unilateral clefts, clefts involving left side are seen in 70% of cases.

4. Epidemiology
In Nepal:
 Total birth prevalence of cleft of the lip ± cleft of the palate (CL±P)
was 1.64/1000 live births per year 1
 Another study revealed 23.51% CL, 54.85% CLP and 21.64% CP.
There were 58.21% males and 41.79% females 2
 The male to female ratio of CL/P is 2:1, 3
oWhile the male to female ratio of CP is 1:2 3
1 Singh VP, Sagtani R, Sagtani A. Prevalence of cleft lip and cleft palate in a tertiary hospital in Eastern Nepal. Mymensingh Med J. 2012;21(1):151-4.
2 Khatun, Sanzida. (2019). Study of Cleft Lip and Palate in Kathmandu Model Hospital, Nepal. Orthodontic Journal of Nepal. 9. 6-10. 10.3126/ojn.v9i1.25682.
3 Mai CT, Cassell CH, Meyer RE, et al. Birth defects data from population-based birth defects surveillance programs in the United States, 2007 to 2011: highlighting orofacial clefts.
Birth Defects Res Part A Clin Mol Teratol. 2014;100(11):895-904.

5. Embryology
 Development of facial structures starts at the end of 4th
week
 5 facial prominences around stomatodeum
o Unpaired frontonasal process
o Paired maxillary prominences
o Paired mandibular prominences
12
Thronton JB, Nimer S and Howrd P.The incidence, classification, etiology and embryology of oral clefts. Semin Orthod 1996;2:162-168

6.  In following 2 weeks –
o The 2 medial nasal processes fuse in midline – upper lip
o Mandibular processes fuse in midline – lower lip
Thronton JB, Nimer S and Howrd P.The incidence, classification, etiology and embryology of oral clefts. Semin Orthod 1996;2:162-168

7. Formation of clefts
 Failure of fusion of maxillary and medial nasal processes –
anterior to incisive foramen
 Failure of fusion of palatine shelves – posterior to incisive
foramen
 Cleft lip – failure of proliferation of mesodermal cells in midline

8. Etiology
Genetic factors:
oSATB2 (transcription factor), SHH (Sonic hedgehog), IRF6
Environmental factors: inutero exposure of different
medications and environmental agents
oDrugs: antiseizure agents (phenytoin, sodium valproate,
topiramate), folic acid antagonists (methotrexate),
Ondansetron in 1st trimester, Nitrofurantoin
oCigarette smoking
oFolate deficiency

9. Etiology
Maternal obesity
Amniotic band sequence
Others: viral infection, radiation, poorly controlled DM
Chances increases if more than one family member if
affected
Risk decreases in remotely related individuals
Consanguinity increases the rate because of sharing of genes

10. Etiology
 2 unaffected parents with1 child affected
o Risk for future children:
o 4.4% for CL+/-palate
o 2.5% for CP only
 1 parent affected
o Risk for future children
o 3.2% for CL+/-palate
o 6.8% for CP only
 1 parent affected with 1 child affected
o Risk for future children
o 15.8% for CL+/-palate
o 14.9% for CP only

11. Etiology
Associated with syndromes: approx. 30% of cases of
CL/P and 50 % of cases of CP
oDown syndrome
oDi George syndrome
oVan der Woude syndrome
oTreacher Collins syndrome
oOral-facial digital(OFD) syndrome type I
oSATB2 syndrome

12. Classification
 Bixler divided oral clefts into three groups
1. Syndromic / single-gene / chromosomal or environmental
 1% of CLP AND 8% of isolated cleft palate
2. Familial
 25% of CLP and 12% of isolated cleft palate
3. Isolated / non-familial
 75% of CLP and 80% of isolated cleft palate

13. Classification
 Davis and Ritchie’s classification 1992
 Group I: Prealveolar process cleft (clefts affecting the lip)
1.Unilateral (right/left: complete/incomplete)
2.Bilateral (right: complete/incomplete; left: complete/incomplete)
3.Median (complete/incomplete)
 Group II: Postalveolar process cleft (clefts affecting the palate)
1.Soft palate
2.Hard palate

14. Classification
 Group III: Alveolar process cleft (any cleft involving the
alveolar process)
1.Unilateral (right/left: complete/incomplete)
2.Bilateral (right: complete/incomplete; left: complete/incomplete)
3.Median (complete/incomplete)

15. Classification
Victor and Veau’s classification (1931)
A] Cleft lip
 Class I: U/L notching of vermillion border, not extending into the lip.
 Class II: cleft extending into the lip, but not including the floor of the nose.
 Class III: extending into the floor of the nose.
 Class IV: any b/l cleft of the lip, whether incomplete or complete.

16. B] Cleft palate
 Class I: soft palate
 Class II: soft/hard palate extending no further than incisive foramen.
 Class III: complete unilateral cleft, extending from uvula to incisive foramen,
then deviating to one side
 Class IV: two clefts extending forward from the incisive foramen into the alveolus.
Allori AC et al. Classification of Cleft Lip/Palate: Then and Now. Cleft Palate–Craniofacial Journal 2016;53(1)

17. Fogh-Andersen’s 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
Allori AC et al. Classification of Cleft Lip/Palate: Then and Now. Cleft Palate–Craniofacial Journal 2016;53(1)

18. Kernahan and Stark’s classification (1958)
Allori AC et al. Classification of Cleft Lip/Palate: Then and Now. Cleft Palate–Craniofacial Journal 2016;53(1)
1. Clefts of structures anterior to the incisive foramen
2. Clefts of structures posterior to the incisive foramen
3. Clefts affecting structures anterior and posterior to the incisive foramen

19. American Cleft Palate–Craniofacial Association (ACPA)
classification (1962)
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)
Allori AC et al. Classification of Cleft Lip/Palate: Then and Now. Cleft Palate–Craniofacial Journal 2016;53(1)

20. Allori AC et al. Classification of Cleft Lip/Palate: Then and Now. Cleft Palate–Craniofacial Journal 2016;53(1)
3. Clefts of the prepalate and palate (alveolocheilopalatoschisis)
4.Facial clefts other than prepalatal and palatal
a.Cleft of the mandibular process
b.Naso-ocular clefts
c.Oro-ocular clefts
d.Oro-aural clefts

21. Classification
Kernahan and Stark’s stripped Y classification (1971)

22. Spina’s classification (1973)
22
Allori AC et al. Classification of Cleft Lip/Palate: Then and Now. Cleft Palate–Craniofacial Journal 2016;53(1)
Group I: Preincisive foramen clefts
a. Unilateral
b. Bilateral
c. Medial
Group II: Transincisive foramen clefts
a. Unilateral
b. Bilateral
Group III: Post incisive foramen clefts
a. Total
b. Partial
Group IV: Rare facial clefts

23. Kernahan and Stark’s
stripped-Y:
Modification by Ehlsaky
(1973) and Millard
(1976)
44

24. Schuchardt and Pfeifer’s symbolic classification
24
left Right
Lip
Alveolus
Hard palate
Soft palate
Total cleft
Allori AC et al. Classification of Cleft Lip/Palate: Then and Now. Cleft Palate–Craniofacial Journal 2016;53(1)
Partial

25. LAHSHAL classification by Okriens (1987)
25
Allori AC et al. Classification of Cleft Lip/Palate: Then and Now. Cleft Palate–Craniofacial Journal 2016;53(1)
Lahshal is a paraphrase of the anatomic areas affected by the
cleft.
 L – lip
 A – alveolus
 H – hard palate
 S – soft palate
 H – hard palate
 A – alveolus
 L – lip

26. Diagnosis
Prenatal diagnosis
 Benefits:
oIdentify the associated syndromes prenatally
oProvides time to learn and adjust to facial abnormality
oPrepare for the needs of their offspring
oMake arrangements to transfer to center that specializer in such
diseases
oOption of terminating

27. Diagnosis
Prenatal diagnosis
 Disadvantages :
oEmotional disturbance
oHigh maternal anxiety and dysfunction
oTermination of pregnancy

28. Diagnosis
Prenatal Ultrasound
 Clearly visualized sonographically, which is at 13 to 14 weeks of
gestation by transabdominal ultrasound
 2D US:
oDetection rates: 9% to 100% for CL/P, 0% to 22% for CP and 0% to 73% for all
types of cleft 1
 3D US:
oDetection rate: 100% for CL, 86% to 90% for CL/P and 0% to 89% for CP 1
 CL also have palatal involvement: 85 percent of bilateral CL and 70
percent of unilateral CL cases are associated with CP 2
2 Jensen BL, Kreiborg S, Dahl E, Fogh-andersen P. Cleft lip and palate in Denmark, 1976-1981: epidemiology, variability, and early somatic development. Cleft Palate J.
1988;25(3):258-69.
1 Maarse W, Bergé SJ, Pistorius L, et al. Diagnostic accuracy of transabdominal ultrasound in detecting prenatal cleft lip and palate: a systematic review. Ultrasound Obstet Gynecol.
2010;35(4):495-502.

29. Diagnosis
Prenatal US
 Transabdominal US
o Not reliable till gestational age of 15 weeks
o Done at 20 or more weeks of gestation
 Transvaginal USG
o Earlier visualization (12 weeks)
o Better image resolution
o Greater specificity and sensitivity

30. Problems associated
 Feeding
o Cleft lip= makes it more difficult for an infant to suck on a nipple
o Cleft Palate= may cause formula or breast milk to be accidently
taken up into the nasal cavity
o Inability to create negative pressure inside oral cavity
o Frequent regurgitations
o Infant held at 30-450 angle to aid swallowing
o Upper respiratory tract infections

31. Problems associated
 Dental problems
o Congenitally Missing teeth, Hypodontia, Hyperdontia, Oligodontia
o Presence of natal and neonatal teeth
o Anomalies of tooth morphology like microdontia, macrodontiaetc
o Fused teeth
o Enamel Hypoplasia
o Poor periodontal support, early loss of teeth
 Orthodontics Problems:
o Anterior and Posterior Cross bite
o Spacing and crowding

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

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

34. Problems associated
 Speech Difficulties
o Hearing loss hampers proper development of speech
o Velopharyngeal Insufficiency (VPI)
o Poor pronunciation of
• Bilabial,
• Labiodental,
• Linguoalveolar sounds

35. Management
Multidisciplinary approach
o Genetic Scientist
o Pediatrician
o Pedodontist
o Orthodontist
o Oral and Maxillofacial
Surgeon
o Prosthodontist
o ENT Surgeon
o Plastic Surgeon
o Psychiatrist
o Speech Therapist
o Social Worker

36. Non-Surgical Treatment
 Dental Obturator
o For high-risk patients or those that refuse surgery.
o Advantage- High rate of closure
o Disadvantage- Need to wear a prosthesis, and need to
modify prosthesis as child grows.

37. Schedule of management
 Birth:
o InitialAssessment
o Pre-surgical assessment
 3 Month:
o Primary Lip repair
 18 month:
o Palate Repair
 2 Year:
o Speech assessment
 5 Year:
o Lip Revision Surgery
 9 Year:
o Initial interventional Orthodontics
o Preparation for alveolar bone grafting
 10 Year:
o Alveolar Bone Grafts
 14 Year:
o Definite Orthodontics
 16 Year:
o Nasal Revision Surgery
 20 Year:
o Orthognathic Surgery

38. Management of cleft lip and palate can be divided into following stages:
 Stage I- treatment done from birth to 18 month of age
 Stage II- from 18th month to 5th year of life( primary dentition stage)
 Stage III- treatment carried out during mixed dentition stage from 6th to
11th year oflife
 Stage IV - treatment done during permanent dentition stage ( 12-18
years)

39. Feeding
 Cleft lip = makes it more difficult for an infant to suck on a nipple
o 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
o Don’t feed baby without palatal obturator (prosthetic palate)
o Feed in an upright position to keep milk from coming out of the nose

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

41. Management
 Principles of Surgery:
o To restore the normal anatomy of lip, nose, and face
o Normal or near normal anatomy promotes normal
function, thereby encouraging normal growth and
development of lip, nose, palate and facial skeleton

42. Management
Timing of surgery
 Better results are obtained when the child is at least 3 months.
Millard Criteriae for cleft lip:
-10 pounds of weight
-10 weeks old
-10gm% Hb
-WBC < 10,000/uL
Cleft palate :
-10 kgs weight
-10-18mths old
-10gm% Hb

43. Surgical Management
 Complete Cleft Lip
Goal: Symmetric shaped nostrils, nasal seal, and alar bases; well
defined philtral dimple and columns; natural appearing Cupid’s bow;
functional muscle repair
Flap designs:
1)Rotation-advancement (Millard, Mohler)
2)Quadrangular
3)Triangular (Tennison-Randall)

44. Millard Technique
“Cut as you go” technique
Preserves’ cupid’s bow and philtral dimple
Scar placed in more anatomically correct position along philtral column
Tension of closure under the alar base; reduces flair and promotes better molding of
the underlying alveolar processes

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

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

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

48. 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

49. Von Langenbeck Operation:

50. 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.

51. Thank You