cleft lip and palate

1. CLEFT LIP AND PALATE
Dr Sneha khadka
MScD Orthodontics.

2. CONTENTS
 Introduction
 Incidence
 Classification
 Embryological Background
 Etiology
 Diagnosis
 Problems of individuals with clefts
 Treatment of Cleft Lip and Palate
 Specialties involved in the treatment of Cleft Lip and Palate
 Timings of surgical repair
 Cheilorraphy
 Palatorrhaphy
 Alveolar Cleft Grafts
 Secondary Surgical Procedures
 Valopharyngeal Insufficiency

3. INTRODUCTION
What is Cleft Lip and Palate?
Congenital abnormal space or gap in the upper lip, alveolus and palate.

4. INCIDENCE
More common in south far Asians:
1 in 500
Less frequent in Africans:
1 in 2000
Prevalence in Europeans and Americans:
1 in 750

5. 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 girls.
75% of Clefts are Unilateral, rest are Bilateral.
Left side is more involved than right side

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

7. Development of the Lip:
Unpaired Frontonasal
Prominence
Medial and Lateral Nasal
prominences
2 maxillary prominences
2 mandibular prominences

8. Fusion defects can occur anywhere between these prominences
The defect in the fusion between the frontonasal and maxillary will
lead to cleft lip

9. Development of Palate:
We have two parts of two different embryonic origins:
1 ) primary palate : the triangular part of hard palate anterior
to incisor foramen which originate from the premaxilla
( frontonasal prominences).
--ddeevveelloopp bbeettwweeeenn 44tthh aanndd 88tthh wweeeekk ooff ggeessttaattiioonn
2 ) secondary palate : remaining part of the hard palate and all
soft palate posterior to incisor foramen which comes from
palatine shelves of the maxillary prominences
--ddeevveelloopp bbeettwweeeenn 88tthh aanndd 1122tthh wweeeekk ooff ggeessttaattiioonn

11.  Various theories have been given for its development.
1. Alteration in intrinsic palatal shelf force .
2. Failure of tongue to drop down.
3. Non fusion of shelves.
4. Rupture of cyst formed at the site of fusion.

12. CLASSIFICATION
 Early attempts at classification by
Davies & Ritchie in 1922.
 Group I : prealveolar clefts- unilateral cleft lip.
- bilateral cleft lip.
- median cleft lip.
 Group II : post alveolar clefts – cleft hard palate
- cleft soft palate
- cleft hard and soft palate.
- submucosal clefts.

13.  Group III: alveolar clefts- unilateral
- bilateral
- median

14. Veau in 1931
 Group I : clefts of soft palate only.
 Group II : clefts of hard and soft palate extending upto the
incisive foramen.
 Group III: complete unilateral clefts involving soft palate,
hard palate, alveolar ridge and lip on one side.
 Group IV: complete bilateral clefts of soft and hard palates,
alveolar ridge and the lip.

15.  Fogh-Anderson in 1942 studied incidence of CL(P) in
Denmark
a. Hare lip including alveolus as far back as incisive foramen
b. Hare lip & CP
c. Isolated clefts of palate as far forward as incisive foramen

16. KERNAHAN AND STARK’S CLASSIFICATION OF
CLEFTS (1958)
Clefts of primary palate only
Unilateral (right or left)
Complete
Incomplete
Median
Complete (premaxilla
absent)
Incomplete (premaxilla
rudimentary)
Bilateral
Complete
Incomplete
Clefts of secondary palate
only
Complete
Incomplete
Submucous
Clefts of primary and
secondary palate
Unilateral (right or left)
Complete
Incomplete
Median
Complete
Incomplete
Bilateral
Complete
Incomplete

17. KERNAHAN STRIPED Y CLASSIFICATION ( 1971)
LIP
ALVEOLUS
PRIMARY
PALATE
HARD PALATE
SOFT PALATE
INCISIVE
FORAMEN

18. MODIFIED STRIPED Y ELSHAY CLASSIFICATION
( 1973)
13
R L
12

19. 1,5- Fusion line between the maxillary prominence and medial
nasal prominence at nostril floor level.
2,6- Fusion line at the level of upper lip.
3,7- R/L alveolus . Fusion line between the maxillary prominence
and intermaxillary segments.
4,8- Fusion between the primary and secondary hard palate.
9,10- Fusion between the palatal shelves of hard palate.
11- Represents the soft palate.
12- Posterior pharyngeal wall.
13- Premaxilla.

20. MILLARD’S MODIFICATION OF KERNAHAN’S
CLASSIFICATION

21. KKrriieennss ““LLAAHHSSHHAALL””
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

22.  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

23. ETIOPATHOGENESIS
Clefts of lip and palate may be isolated deformities or may be part
of a syndrome.
Non syndromic clefts are multifactorial in origin.

24. 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,viral infections.
-Alcohol consumption, smoking, hypoxia during pregnancy, some of
dietary and vitamins deficiencies (like folic acid and vitamin A
deficiency),stress, mechanical disturbances.
Maternal Age.

25. Drugs
Phenytoin
Genetic
predisposition
Non syndromic
Maternal illness
Polygenic
inheritance
clefts
smoking Alcohol

26. Some of the more common syndromes associated
with CL/P
 Chromosomal
Trisomy 13
Trisomy 18
Velocardiofacial syndrome
(22q11 deletion)
 Non-Mendelian
Pierre Robin syndrome
CHARGE association
Goldenhar syndrome
 Teratogenic
Fetal alcohol syndrome
Fetal phenytoin syndrome
Fetal valproate syndrome
 Mendelian disorders
Ectrodactyly-eetodermal
dysplasia-clefting syndrome
(AD)
Gorlin syndrome (AD)
Oto-palato-digital syndrome
(XL)
Oral-facial-digital syndrome
(XL)
Smith-Lemli-Opitz syndrome
(AR)
Stickler syndrome (AD)
Treacher Collins syndrome
(AD)
Van der Woude syndrome
(AD)
 Unknown
de Lange syndrome
Kabuki syndrome

27. PROBLEMS ASSOCIATED WITH CLEFT LIP AND
PALATE
Feeding
Dental problems
Nasal Deformity and Esthetic Problems
Ear Problems
Speech Difficulties
Associated Anomalies

28. 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

29. 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

30. NASAL DEFORMITY AND ESTHETIC PROBLEMS
Facial Disfigurements.
Poor nasal shape.
Scar marks of surgeries.
Poor lip function during speech.
Poor dental alignment and smile.

31. EAR PROBLEMS
Middle ear disease - 22% to 88% .
Conductive hearing loss and chronic suppurative otitis media may
result.
Repeated tympanostomy tube placement.

32. Speech Problems:
Hearing loss hampers proper development of speech
Velopharyngeal Insufficiency.
Poor pronunciation of Bilabial, Labiodental, Linguoalveolar
sounds

33. PRENATAL/POSTNATAL DIAGNOSIS
Intrauterine
- Sonographic diagnosis
- Karyotyping
Post partum
- Clinical
- Radiographic (intra and
extraoral, laminography,
pantography)
- Additional aids (speech and
sound recording, measurement
of nasal and oral pressures,
psychiatric examination)

34. PRENATAL, PERINATAL AND POSTNATAL PARENTAL
COUNSELLING
Requisites
Accurate information should be given by an expert in the field
Support must be available soon after diagnosis
Parents and members of family given opportunity to express
concern and emotional response

35. Give a clear view of how the baby is likely to look.
Discussion on pregnancy termination based on accurate
information .
Promote early adjustment and acceptance of the baby by the
parents and family.
Long term dependence on counselor should be avoided and
normalization of family life should be encouraged.