Cleft Lip and Palate

Cleft Lip and Palate - Presentation.
Cleft Lip and Palate is the 2nd most common Congenital Anomaly after Clubfoot. This presentation goes in depth about the Presentation, eitiology, Genetics, Medical management, Nasoalveolar Moulding, Surgical management of Cleft Lip & Palate

1. Cleft Lip And Palate
Chairpersons :
Dr. Mohan G
Dr. Vijay Kumar
Dr. Sunil Kumar
Dr. Yogitha
Presenter : Dr. Vikas V

2. Introduction

Cleft lip and palate is the 2
nd most common congenital
anamoly after clubfoot

Clefts of the lip, alveolus and hard and soft palate are the MC
Congenital Abnormalities among 15 of the orofacial structure
anomalies.

Frequently occur as isolated deformities but can be a/w other
medical conditions, particularly CHDs.

Associated feature in over 300 recognised syndromes.

3. History

Hippocrates (400 BC) and Galen (150 AD) mention cleft
lip, but not cleft palate in their writings

Cleft palate – Fanco (1556)

Repair of cleft lip – as early as 255-206 BC in China

The first successful closure of a soft palate defect was
reported in 1764 by LeMonnier, a French dentist.

4. Incidence

Cleft Lip + Palate – 1:600 LB

Isolated Cleft Palate – 1:1000 LB

Typical distribution of cleft type:
cleft lip alone: 15%
cleft lip and palate: 45%
isolated cleft palate: 40%.

CL/P – Males ;CP – Females
A child is born with a cleft somewhere in the world every 2 minutes
according to a WHO study published in 2001

In India alone the number of infants born every year with CLP is
28,600
78 affected infants are born every day, or 3 infants with clefts born
every hour

5. Anatomy of Lip

6. Embryology

Development of facial
structures starts at
end of 4th week

5 facial prominences around stomatodeum
1. Unpaired frontonasal process

Nasal Placodes – Invagination – Nasal Pits
Medial Nasal Process
Lateral Nasal Process
2. Paired maxillary prominences
3. Paired mandibular prominences

7. Development of Lips

In following 2 weeks –
2 medial nasal processes fuse in midline – upper lip
Mandibular processes fuse in midline – lower lip

8. Development of Lip and Nose

The maxillary and lateral nasal process separated by nasolacrimal
groove/duct

Frontonasal process – bridge of the nose

Medial nasal process – tip of nose and philtrum of upper lip

Lateral nasal process – ala of the nose

9. Development Of Palate

Primary palate –
maxillary and medial nasal process merge
containing the premaxilla, alveolus,and lip, is anterior to
the incisive foramen

Formation of intermaxillary segment from merged medial
nasal prominences

10. Development Of Palate (Contd)

Secondary palate –
formed from 2 outgrowths from maxillary prominences i.e
palatine shelves
containing the uvula, palatal musculature, maxilla, palatine
bones, and pterygoid plates

Fuse in midline at 7
th week

Incisive foramen –
midline landmark between primary and secondary palate
contains the nasopalatine vessels and nerve

12. Formation of clefts

13. Formation of clefts (Contd)

14. Aetiology --> 1] Genetic

Family history : First-degree relative affected increases
the risk to 1:25 live births.

Genetic influence ---> more significant in Cleft lip/Palate ;
Environmental factors ---> Isolated cleft palate
Role of TGF-β
3 in Palatal Fusion : Expressed in MEE
Expressed by Medial Edge Epithelial cells
Homozygous null TGF-β
3 --> Cleft Secondary Palate

Defect in Wnt9b Signaling
Insufficient Growth of Maxillary prominces

Mutation in FOXE1 gene
Expressed at point of Fusion b/w Maxillary & Nasal Process

15. 2] Environmental factors

Maternal smoking or Tobacco exposure

Viral infections • Poor nutrition

Maternal Epilepsy

Teratogens like:
Rubella virus, Cortisone/ steroids, Mercaptopurine,
Methotrexate, Valium, Dilantin, diazepam, phenytoin
3] Predisposing Factors
• High maternal age • Diabetes • Toxemia
• Reduced blood supply • Folic acid deficiency
• Racial – mongoloids • Radiations

16. Classification of Cleft Lip
Cleft Lip can be classified by two main characteristics:

Unilateral v/s Bilateral deformity &

Complete v/s Incomplete deformity

Through-and-through /Partial Penetrance involvement of the lip,
alveolus, and nasal sill.

Incomplete cleft lips may be further characterized as

Minor form (defect extending 3 mm or more above the normal
Cupid’s bow peak),

Microform (notch less than 3 mm above the normal peak),

Minimicroform (disrupted vermilion cutaneous junction without
elevation of the bow peak),

17. Classification of Cleft Lip

18. Incomplete Cleft lip types

Minor Form Microform Microminiform

19. Classification of Cleft Palate

Cleft palate may be defined as:
(1) Unilateral or Bilateral

depending on laterality of involvement of the primary palate
(2) Complete or Incomplete

Complete:
extends from the Alveolar ridge to the uvula
Involves primary and secondary palates.

Incomplete :
involves the secondary palate only,

Submucous cleft palate

Bifid Uvula

20. Classification Of Cleft Palate

21. Veau’s classification (1931)
CLEFT LIP

Class I : U/L notching of vermillion border, not extending
into the lip.

Class II : U/L cleft extending into the lip, but not including
the floor of the nose.

Class III: U/L extending into the floor of the nose.

Class IV: Any B/L cleft of the lip, whether incomplete or
complete.

22. Veau’s classification (1931) Cleft Lip

23. Veau’s classification (1931)

CLEFT PALATE

Group 1 – Cleft involving soft palate only

Group 2 – Cleft of Hard and Soft palate extending upto
incisive foramen

Group 3 – Complete Unilateral clefts involving soft
palate, hard palate, lips and alveolar ridge

Group 4 – Complete Bilateral clefts affecting the soft
palate, hard palate, lips and alveolar ridge

25. 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

26. Kernahan and Stark’s classification (1958)

Pictorial Representation of Cleft Lip & Palate

Upper Limbs –
Represent – Rt & Lt sides of Primary palate
Lip, alveolus, hard palate anterior to Incisive Foramen

Lower Limb -
Represent – Secondary Palate
Hard & Soft Palate post to Incisive Foramen

Limitation:
Clefts of Secondary Palate cannot be classified to Left or
Right

27. Kernahan and Stark’s classification

28. 
Kernahan and
Stark’s stripped-Y:
Modification by
Ehlsaky (1973) and
Millard (1976)

29. Facial Growth in Cleft Lip & Palate

Prenatal Growth

Various forces which influence the facial growth in utero are:

A] Over maxillary segment on non-cleft side:
Pull of lip and cheek muscles
Tongue pressure
Relatively unstrained nasal septum growth

B] Over maxillary segment on cleft side:
Instrinsic Deficiency
Pressure from alar base due to stretching of the nostrils.

30. Facial Growth in Cleft Lip & Palate

Due to above mentioned forces, deficiency produced in cleft lip and
palate babies are:

A] Incomplete unilateral cleft lip and palate:
Severe deviation of midline away from cleft.
Smaller maxillary segment shows retro-positioning or growth
inhibition and collapse.
Nose is deviated towards normal side.

B] In bilateral cleft lip and palate cases
Premaxilla tilts forward and/or shifts to one side due to tongue
pressure.

31. Prenatal Diagnsois

Prenatal ultrasound – 2D or 3D

Usually made after 16-20 weeks of
Gestation

Prenatal counselling

Prenatal Detection Rates:
22% to 33% rates for detecting facial clefts

73% - fetal cleft lip

1.4% - isolated cleft palate

Color Doppler ultrasonography can also be used

32. Babcock and McGahan (1997)

Starts with coronal plane

Assessment continues in axial view

Bilateral clefts: sagittal view • Isolated clefts: axial view

33. Problems Associated With Cleft Lip & Palate

34. DENTAL

Tooth agenesis, hypodontia (MC)

Supernumerary teeth (2nd MC)

Enamel hypoplasia (CI)

Crossbites

Ectopic eruption, transposition

Taurodontism, dilacerations

35. Skeletal

Maxillary deficiency

Mandibular prognathism

Class III malocclusion

Concave profile

36. FEEDING PROBLEMS

Oronasal fistulas

Draining of oral fluids in nasal cavity and vice versa

Bottle, cup and spoon, tube feeding

Infant held at 30-45 0 angle to aid swallowing

38. Syndromes A/w CLP

Around 400 syndromes

Chromosomal anomalies
Trisomy 13 (Patau)
Trisomy 18 (Edward)
Trisomy 21 (Down’s)
Velocardiofacial syndrome (22q11 deletion)

Teratogenic
Fetal alcohol syndrome
Fetal phenytoin syndrome
Fetal valproate syndrome


39. 
Inherited syndrome
Sticklers (Autosomal dominant)
Treacher Collins (AD)
Van der Woude (AD)

Non-inherited syndrome
Pierre Robin Syndrome –

Triad of
cleft palate (60%-90%),
glossoptosis,
retrognathia
Goldenhar syndrome
Syndromes A/w CLP

40. MULTIDISCIPLINARY MANAGEMENT

41. Overall Cleft Treatment Plan

TIMING AND SEQUENCING OF
CONSERVATIVE SURGICAL-
ORTHODONTIC TREATMENT

42. Stages in Management
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 of life

Stage IV- treatment done during permanent dentition stage
( 12-18 years )

43. Stages of Management

Stage I treatment Includes:

1] Fabrication of a passive obturator

2] Pre surgical orthopedics

3] Surgical management of cleft lip

4] Surgical management of cleft palate

Passive maxillary obturator:

Intraoral prosthetic device

Fills the palatal clefts

Provides false roofing against which child can suckle

Reduces the feeding difficulties like insufficient suction, choking,
excessive air intake

44. •Obturator is fabricated
using cold cure acrylic
after selective blocking
of all the undesirable
undercuts
•Clasp aid in retention

45. Pre Surgical Orthopaedics
Nasoalveolar Molding
1.Creation of good functioning palate.
2.Normalize tongue position.
3.Help in speech development.
4.Improve symmetry of nose and cleft of maxilla.
5.Psychologically boost patient and parents as the patient get
continued supervision.

46. Presurgical alveolar and nasoalveolar molding

Alveolar molding :
External taping with or without dental plate
Strip of Micropore tape is placed across the cleft to
approximate the upper lips.
Objective :

simulate effects of an adhesion cheiloplasty

reposition the maxillary segments into proper alignment
Dental plate

47. Forces That Exert an Influence on Cleft Alveolus

Central tongue force, pushing the cleft laterally,

Tongue in the cleft alveolus, preventing approximation.

Anteriorly, the Muscle pulls laterally

Cheek pressure pushes posteriorly on the palate.

48. Naso-Alveolar molding

Liou’s method
Molding Bulb attached to a Dental Plate
Mold the nose along with External Taping of the Lip
The force from taping and counterforce from the molding bulb
provide the combined force necessary to bring the Alveolus into
proper position
Nasal & alveolar molding --> done at the same time
Time frame : approximately 3 months
Overstretching of the nasal cartilage on the cleft side
Nasal projection & dental plate adjusted every 1–2 weeks

49. Liou’s Method

50. Grayson’s Method -

Nasal molding After alveolar approximation to avoid overstretching the nasal
cartilage

Appliance consists of
An acrylic or Resin plate which fits over Maxillary Dental arch
An acrylic retention arm or button
A Nasal stent

Micropore tape applied to the lips and connected with orthodontic elastics hold the
dental plate to the palate

Gradually adding soft resin on the inner surface of the buccal flange approximates
the alveolar shelves.

Nasal projection is added and adjusted underneath the deformed cartilage

Started within the first 2 weeks after birth

Monitoring is required every 1–2 weeks, for a period of 3–6 months to complete it

51. Grayson’s Method

54. Surgical Cleft Lip Repair

Primary closure of lip - Age of 3 months or 10 weeks when
child is fit to undergo General Anaesthesia

Millard has suggested “RULE OF TEN”.

10 weeks of age

10 pounds (4.54 kg)

10 gram% - Blood haemoglobin not less than 10g%

Four techniques have been proposed:
a)Straight Line Repair
b)Quadrangular Flap
c)Triangular Flap
d)Rotation Advancement Flap – Millard’s Repair

55. A. Straight-line repair
●1. Historically, the first cleft lip repairs relied
on freshening the edges of the cleft and
suturing them together. These have been
largely replaced by various Z-plasty-based
techniques.
●2. Rose-Thompson repair
–a. Modified straight-line repair
used for minor clefts with lip length nearly
equal on both sides of cleft (e.g., forme fruste).
–b. Fusiform excision with straight-line closure

56. B. Quadrangular flap
●B. Quadrangular flap
–Proposed by LeMesurier
and Hagedorn.
–Cupid's bow is derived
from the lateral lip.
–90-degree Z-plasty.
–Violates Cupid's bow
and philtral dimple.
–Has a tendency to
produce a long lip

57. C. Triangular flap
●Initially described in 1952 by Tennison
●In 1959, Randall described a
mathematical approach
●Conceptually similar to the rotation
advancement repair.
●Difference - rotation back- cut in the
noncleft segment is performed more
inferiorly, closer to the vermilion border

58. D. Rotation Advancement- Millard’s Repair
●The rotation advancement repair
of the unilateral cleft lip deformity as
described by Millard is the most commonly
used method of repair

59. Markings for Millard’s Technique
1 to 2 = 1 to 3 = 2-4 mm
2 to 6 = 8 to 7 = 20 mm
2 to 4 = 8 to 10 = 9 -11mm
3 to 5 + x = 8 to 9
1) Center/Low Point of Cupid’s Bow
2) Peak of Cupid’s Bow on NCS
3) Peak of bow on medial Cleft
4) Alar Base, NCS
5) Columellar Base, NCS
6) Commisure, NCS
7) Commisure , Cleft Side
8) Peak cupid’s bow lateral side of Cleft
9) Superior extent of advancement flap
10) Alar base, Cleft side
Point X – Back Cut Point

60. Important Markings and Incision Plan

61. Rotation advancement: Technique
●Make markings: from x to 5 to 3 (rotation flap)
–From point 8 to point 9
–Dissect skin off orbicularis on either side
of cleft
–Bilateral gingival sulcus incisions made to
cleft margin
–Cheek soft tissue elevated off maxilla
above periosteum (caution w/ infraorbital N)
–Free orbicularis from attachments to
columella/ alar margin
–Incise along alar margin from 9 to 10
–Elevate c flap

62. Rotation advancement: Closure
●Close intraoral mucosa
●Close orbicularis serially
●Cleft alar base medialized with stitch to
periosteum of nasal spine
●C- flap rotated into columellar defect
●Close nasal floor
●Inset/sew flaps
●Nasal correction at same time

64. Outline of Surgical Plan

65. Advantages
●Main Advantage : Flexibility and application of Surgery
●Relies on a “cut as you go” strategy that allows continuous
modifications during the design and execution of the repair.
●It does not adhere to strict geometrical principles or
measurements.
●The suture line approximates a new philtral column.
●The aesthetic philtral subunit is not violated, and this tends to
create a scar that is more camouflaged.
●Minimal tissue is discarded during the rotation advancement
technique, and this tends to put less tension on the closure.

66. Postoperative care
●A. Orders
–1. Arm restraints for 3 weeks to prevent disruption of repair.
–2. Specialized nipple/bottle to decrease sucking effort
when bottle-feeding.
–3. Breast-feeding is controversial; based on surgeon
preference.
●B. Leave Steri-Strips in place over the incision for
reinforcement.
●C. Follow up in 1 week for suture removal if nonabsorbable
skin sutures were used.

67. Complications of cleft lip repair
1) Failure to Reconstitute Competent Oral Sphincter
•Inadequate reapproximation of the orbicularis oris
•Visible muscle bulge - readily apparent under the skin of
the repaired lip on dynamic motion.
2) Inaccurate alignment of Vermilion-Cutaneous Junc
n
•Small step-off deformity Visible to the untrained eye
3) Vertical scar contracture or inadequate rotation.
•shortening of the lip segment
•notch in vermilion – Whistle Deformity
4) Scar contracture - narrow nostril sill – Vestibular Stenosis
5) Wound dehiscence & Scar widening

68. B/L Cleft Lip Repair
●Repair of bilateral cleft lip is essentially the same, except
that the premaxilla which is projecting has to be pushed
back.
●Steps:
–Labial dissection -Alveolar closure - Labial closure
–Nasal dissection & positioning Lower Lateral cartilages

70. Alveolar Closure

71. Completed bilateral complete cleft lip/nasal repair.

72. Alveolar Cleft repair
●There are two main ways in which this can be achieved.
–Multi Staged Approach
●Secondary Alveolar bone grafting @ Age 9-11 yrs
●Preceded by primary soft-tissue closure with a
Vomer flap at the time of primary lip repair,
●Although the alveolar cleft may be left unrepaired at
the time of primary surgery.
–Primary gingivo-periosteoplasty (GPP) at either the
time of palate closure or delayed until the age of 3 to 5

73. BONE GRAFTING FOR ALVEOLAR CLEFT DEFECTS

74. Alveolar Cleft Repair

75. Alveolar Cleft Repair

76. MANAGEMENT OF CLEFT PALATE
●Primary goals
–Separate the nasal cavity from the oral cavity
–Creation of velopharyngeal valve for swallowing and speech
–Preservation of midface growth
●Timing (Controversial )
–Speech outcomes improved with early closure
–Midface growth maybe hindered by early closure (2 stage palate
repair)
–Most repaired between 8-12 months of age to minimize speech
abnormalities ( Nasal Speech)
–Closure of the hard palate defect can be deferred till the time of
secondary dentition.

77. LANGENBECK’S OPERATION
●Margins of the cleft are pared, the nasal septum is defined
and separated off the upper surface of the cleft palate.
●Mucoperiosteal flaps lifted up from the hard palate
●Two release incisions are made one on each side just
medial to the alveolar margins.
●Mucoperiosteal flaps mobilized till release incisions reached
●Cleft repaired in midline by bringing mucoperiosteal flaps &
Nasal mucosa medially.
●Repair is done in Three layers –
–Nasal mucosa and Soft palate muscles with
interrupted catgut sutures
–Mucoperiosteal flaps with interrupted silk.

78. Von Langenbeck Repair.
A) Relaxing incisions
are made behind the
alveolar ridge,
creating bilateral
bipedicle flaps for
midline closure. The
greater palatine
vessels must be
preserved.
B)The cleft margins are
incised in a manner
to leave adequate
nasal mucosa for
complete closure.
C)Closure of the nasal
mucosa and muscle
repair.
D)Final appearance.
A
B
C D

79. WARDILL’S OPERATION ( Four flap operation)
●This operation differs from the previous one in three aspects.
–i) The two flaps of the cleft palate are divided obliquely up to the releasing
incisions.
●Four flaps are available.
●
Anterior two flaps brought to the midline and sutured.
●Posterior flaps brought to the midline, Also pulled posteriorly to lengthen
Palate.
●Lengthens the palate, Space between Oropharynx & Nasopharynx is
diminished.
–ii) Tension at the suture line is reduced further by
●breaking Hamulus process of pterygoid bone on each side
●thus relaxing the tensor palati muscle.
–iii) Pharyngoplasty is added.
●Aimed at reduction of the diameter of the nasopharynx
●Makes Ridge of Passavant prominent.

80. V-Y pushback (Veau–Wardill–Kilner)
A) Design of anterior “W”
incision.
(B) Elevation of bilateral
mucoperiosteal flaps
based on palatine
vessels. The levator veli
palatini muscles are freed
from the posterior border
of the hard palate.
(C) The muscles are
repaired across the
midline of the soft
palate.
(D) The “Y” closure
creates additional length
but also leaves large raw
areas bilaterally.

81. Pharyngoplasty
●Children with repaired cleft palate may
have a resulting condition referred to as
“VPI” (Velopharyngeal Incompletence).
●This means that too much air escapes through the nose
during speech,resulting in nasal speech.
●This occurs because the repaired soft palate is too short or
does not move adequately.
●In this operations,some of the tissue from palate and back
of throat are repositioned to help close off the escape of air
through the nose

82. RECENT ADVANCES
●Fetal surgery – done in intrauterine life (prior to 20 weeks)
●Non-life threatening defects like cleft lip, cleft palate, Pierre
Robin syndrome, Treacher-Collins syndrome, craniofacial
microsomia
●Open fetal surgery
●Feto-endoscopic approach

83. CONCLUSION
●Oral clefts are the second most common congenital anamoly, having
multifactorial origin.
●A considerable knowledge about the etiology and embryology is
required for proper diagnosis and treatment planning of such patients
●Treatment begins soon after birth and continues till adulthood
requiring a team approach.
●Multiple problems and syndromes with clefts
●Multidisciplinary approach for management, patient-centered
●Variations in treatment sequence
●Role of orthodontists.

84. Thank You