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1. Cleft Lip: Primary and
Secondary Deformities
INDIAN DENTAL ACADEMY
Leader in continuing dental education
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2. Historical background
Cleft lip
– 1st
repair
• Unidentified Chinese surgeon
• 390 AD
– 1st
description
∀ ∼1300 AD
• Straight line repair
– Malgaigne
• 1843
• Local flap closure
– Mirault
• Lateral flap to fill medial deficit
• Basis of most modern techniques
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3. Historical background
Cleft lip
– Millard
• 1955
• Concept:
– Lateral flap advancement into upper lip
– Downward rotation of medial segment
– Preserves Cupid’s bow and philtral dimple
– Tension of closure at alar base
• Reduces nasal flare
• Improved alveolar molding
• Most popular method for unilateral lip closure
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6. Embryology basics
Facial development
– 4th
to 10th
week gestation
– Fusion of five processes:
• Unpaired frontonasal
process
– Nose and philtrum
• Paired maxillary swellings
– Cheeks and upper lip
• Paired mandibular swellings
– Lower face
– Lower lip and chin
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11. Embryology of CleftingEmbryology of Clefting
Facial DevelopmentFacial Development
Medial nasal processesMedial nasal processes (green) migrate toward
each other and fuse
Inferior tips of medial nasal processes expand
laterally to form the intermaxillary process
Tips of maxillary swellings (yellow) grow to meet the
intermaxillary process and fuse
66thth
weekweek
7th
week
Failure of maxillary swellings to fuse with intermaxillary process = cleft lipFailure of maxillary swellings to fuse with intermaxillary process = cleft lip
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
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17. Epidemiology
Cleft lip and palate
– Racial heterogeneity
• Asians
– 2.1 in 1000 live births
• Whites
– 1 in 1000 live births
• African Americans
– 0.41 in 1000
Isolated cleft palate
– Constant incidence
• 0.5 in 1000 live births
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18. Epidemiology
Relative incidence
– Fraser and Calnan
• 21% cleft lip
• 46% cleft lip and palate
• 33% cleft palate
• Left > right > bilateral
– 6:3:1
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19. Epidemiology
Associated factors
– Parental age
• Incidence increases with age
• Father’s age more significant
• Risk highest with both parents over 30 years
– Seasonal incidence
• No strong evidence
– Birth order
• No evidence
– Social class
• High incidence in low socio economic status
• Poor nutrition
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20. Epidemiology
Associated factors
– Parental head topography
• Parents:
– Underdeveloped maxillae
– Flattened anterior surfaces
– Trapezoidal/rectangular faces
– Thin upper lips
– Increased interorbital and intercoronoid process
distance
– Wide nasal cavity
– Increased length of anterior cranial base
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21. Epidemiology
Associated defects
– Overall incidence of associated defects
29%
• CNS malformations
• Club foot
• Cardiac abnormalities
– Highest with isolated cleft palate
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22. Etiology
Categorize cleft deformity
– Malformation
• Morphologic defect of organ or body region
– Intrinsic error of morphogenesis
– Disruption
• Morphologic defect
– Extrinsic breakdown of normal developmental process
– Ie. infectious
– Deformation
• Abnormal form, shape or position caused by
mechanical forces
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23. Etiology
Categorize cleft deformity
– Syndromic
• More than one malformation
• More than one developmental field
– Non-syndromic
• One defect
• Multiple anomalies as a result of a single
initiating event or primary malformation
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24. Genetics
Associated syndromes
– Stickler
• Cleft palate alone
• Autosomal dominant
• Type 2 collagen gene mutation
• Myopia, retinal detachment and glaucoma
– Van der Woude’s syndrome
• Autosomal dominant
• Bilateral lower lip pits
• Absence second molars
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29. Environmental agents
Altitude
– Higher relative risk in highlands
• Also microtia
• Preauricular tags
• Branchial arch anomaly complex
• Constriction band
• Anal atresia
– Speculation
• Chronic hypobaric hypoxia during
embryologic and fetal development
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30. Multifactorial model
Non mendelian inheritance
– Concept of genetic susceptibility
• Threshold determined by genetics and enviroment
– Defect clusters in families
– Risk for first degree relatives = √population risk
– Risk for second degree relatives = lower than first
degree
– Greater severity; increased recurrence
– Increased number of affected relatives; increased risk
– Risk of recurrence increased in relatives of less affected
sex
– Consanguinity increases risk
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32. Prenatal diagnosis
Ultrasound
– Late 1st
trimester/early second trimester
• 3.5 MHz scanner
– Cleft lip/nose at 15 weeks
• 6.5 MHz transvaginal scanner
– 12 weeks
– Controversy
• Termination of pregancy
– Northern Israel
– 23/24 abortions
– 1/24 couple would terminate if faced with situation again
• Variation in culture
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33. Timing of surgery
Rule of tens
– 10 weeks of age
• Allow lip tissues to develop
– 10 lbs in weight
– Hgb 10 g/dL (100 in our world!)
– WBC less than 10,000
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34. Anatomy
Millard
– Critical anatomic features of unilateral
cleft lip:
• Cleft side
– Premaxilla outwardly rotated
– Lateral maxillary segment retropositioned
– Inferior edge of septum dislocated out of vomer
groove
• Nasal spine in floor of nostril
– Shortened columella
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35. Anatomy
Millard
– Critical anatomic features of unilateral
cleft lip:
• Cleft side
– Lower lateral cartilage attenuated
• Medial crus lower in columella
• Dome rests below opposite alar cartilage
• Lateral segment flattened and spread across
cleft at obtuse angle
• Alar crease continues through rim of ala
– Alar base rotated outwardly in a flare
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36. Anatomy
Millard
– Critical anatomic features of unilateral
cleft lip:
• Cleft side
– Skin curtain droops over alar rim
• Reduces apparent height of columella
– Deficient vestibular lining
– Orbicularis oris ends upward at margin of cleft and
inserts into alar wing
• Incomplete cases muscle does not cross cleft
– Short philtrum
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37. Anatomy
Millard
– Critical anatomic features of unilateral
cleft lip:
• Non cleft side
– Shortened philtral height
– Shortened columella
– Two thirds of Cupid’s bow, one philtral column and
a dimple hollow preserved
– Hypoplastic muscle between philtral midline and
cleft
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38. Anatomy
Muscular deformity
– Muscular bulge
• Haphazard arrangement of muscle fibers
– Transverse/oblique/anteroposterior
– Orbicularis oris
• Two well defined components
– Deep orbicularis
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48. Unilateral cleft lip
Evaluation and classification
– Three categories of unilateral cleft lip:
• Microform cleft lip
• Incomplete cleft lip
• Complete cleft lip
– Associated nasal deformity:
• Mild
• Moderate
• Severe
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49. Unilateral cleft lip
Microform cleft lip (forme fruste)
– Presentation:
• Furrow or scar
• Transgresses vertical length of lip
• Vermilion notch
• White roll imperfections
• Vertical lip shortness
– Three characteristic elements:
• Vermilion notch
• Band of fibrous tissue from edge of red lip to nostril
floor
• Deformity of ala on notch side
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50. Unilateral cleft lip
Microform cleft lip
(forme fruste)
– Three characteristic
elements:
• Vermilion notch
• Band of fibrous
tissue from edge of
red lip to nostril floor
• Deformity of ala on
notch side
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51. Unilateral cleft lip
Microform cleft lip
– Surgical management
• Usually indicated
• Vertical height equal on affected side and
normal side
– Straight line repair
• Elliptical excision
• 2 layer closure
• Vertical difference greater than 1-2mm
– Rotation advancement repair
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52. Unilateral cleft lip
Unilateral incomplete cleft lip
– Varying degree of vertical separation of
the lip
– Intact nasal sill
• Simonart’s band
– Corrected with rotation advancement
repairs
– Nasal repair carried out with primary
repair
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53. Unilateral cleft lip
Unilateral complete cleft lip
– Presentation:
• Separation of lip, nostril sill and alveolus
– Derivative of primary palate
• Secondary palate often is involved
• Position of alveolar segments critical
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54. Unilateral cleft lip
Unilateral complete cleft lip
– Alveolar (maxillary) segment
• Four positions
– Narrow with no collapse
– Narrow with collapse
– Wide with no collapse
– Wide with collapse
• Wide
– Alveolus position lateral to desired alar base
position
• Collapse
– Lingual position of lateral maxillary segment
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62. Unilateral cleft lip
Evolution of cleft lip repair
– 1st
principle
• Lengthen vertical height of cleft side to match
normal side
• Rose Thompson
– Straight line repair; curvilinear cleft side
– Ideal for microform clefts
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65. Unilateral cleft lip
Evolution of cleft lip repair
– 4th
principle
• Rotation advancement concept
• Millard
– Incision line follows natural anatomic position of
philtral ridge
– Avoid placement of scars across lower philtrum
(different from quadrangular and triangular
repairs)
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66. Unilateral cleft lip
Evolution of cleft lip repair
– 5th
principle:
• Muscle reconstruction and preservation of lip
function
– Extensive dissections
– Nicolau and delineation of layers of muscle
• Deep and superficial orbicularis oris
• Intertwined with paraoral/paranasal muscles
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67. Unilateral cleft lip
Evolution of cleft lip repair
– 6th
principle:
• Restoration of the bony platform
• Presurgical orthopedics
– Passive
– Active
• Latham appliance
• Bone grafting
• Gingivoperiosteoplasty
– controversial
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68. Unilateral cleft lip
Evolution of cleft lip repair
– 7th
principle:
• Restoration of normal nasal anatomy
– Complex
– Topic unto itself (stay tuned for next week!)
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75. Bilateral cleft lip
Complex surgical dilemna
– Multiple techniques described and
utilized
– No one technique clearly superior
– Compared to unilateral clefts:
• Twice as difficult with result ½ as good
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76. Bilateral cleft lip
Deformity
– Protruding premaxilla
• Lack of connection of premaxilla with lateral
palatal shelves during development
– Absent nasal spine
• Retruded area under base of septal cartilage
• Recession of medial crura footplates
• Lower lateral cartilage footplates drawn by
lateral palatal shelves
– Broad flat nasal tip
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77. Bilateral cleft lip
Deformity
– Short columella
• Skin over columella is short
• “absent columella”
– Prolabium
• Anterior inferior extent of frontonasal process
normally contributes skin between philtral
columns
• Wide and short
• Hangs directly from nasal tip skin
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78. Bilateral cleft lip
Incomplete bilateral cleft lip
– Near normal nose
– Normal premaxilla
– Simonart’s bands across nasal floor
– Surgical management
• Rotation advancement
• Triangular flap
• Similar to unilateral
• Single or double stages
• Can also use bilateral straight line technique
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79. Bilateral cleft lip
Protruding premaxilla
– Main obstacle in bilateral clefts
• Multiple approaches described
• Lip repair/adhesion
– Stages attempt at retracting premaxilla
– Unpredictable
– Closed under tension
– Wide scars with repair
– Lip adhesion
• Inflammation
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80. Bilateral cleft lip
Protruding premaxilla
– Alternate techniques:
• Elastic bonnet
– Poor control of premaxilla position relative to lateral
segments
• Premaxilla excision/setback at 1st
operation
– NOT a present day option
– Discards permanent incisors
– Severe midface retrusion
• Pin retained premaxillary retraction devices
– Allows for gingivoperiosteoplasty
– Bone grows across small cleft
• Nasoalveolar molding
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81. Bilateral cleft lip
Construction of central lip vermilion
– Two general methods:
• Buccal mucosa
– Inferior aspect of prolabial skin
– Forms central vermilion
– Bulk
• Strips of muscle across
• Deepithelialized buccal mucosa from lateral lip
– Most often inadequate bulk in central section
• Whistle deformity
– Dry versus wet lip problem
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82. Bilateral cleft lip
Construction of
central lip vermilion
– Two general
methods:
• Lateral vermilion
tissue
– Muscle rotates
with lateral lip
elements
– Single scar at
depth of Cupid’s
bow
– Scar mimics white
roll
– Good bulk
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83. Bilateral cleft lip
Skin paradigm
– How to best use
prolabial skin and to
attempt to lengthen
columella?
• Split prolabium
– Form philtrum and
neocolumella
• Millard fork flap
technique
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93. Secondary deformities
Introduction
– Factors in decreased need for revisionary
surgery:
• Improved primary techniques
• Specialized centers of excellence
• Sophisticated presurgical orthodontics
• Nasal correction simultaneously
• Gingivoplasty
• Nasal molding
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94. Secondary deformities
Introduction
– Lip repair expectations
• Accurate skin, muscle and mucous
membrane union
• Proper rotation of lateral orbicularis into
horizontal position
• Symmetric nostril floor and tip
• Even vermilion border and cupid bow’s
• Eversion of central upper lip
• Minimal scar
– Failure of above needs secondary repair
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95. Secondary deformities
Approach
– Assess following variables:
• Anatomic elements
– Components to be preserved and altered
• Residual deformities
– Uncorrected
– Recurrences
– Iatrogenic
• Realistic surgical goals
– Choose procedure with most predictable results
with fewest interventions
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104. Secondary deformities
Unilateral cleft lip
– Short upper lip
• Measure of distance from Cupid’s bow to
columella
– Failure to lengthen lip at primary repair
• Initial shortening
– 1st
2 months
– Maximal at 6-8 weeks
– Softens and relaxes subsequently
– Resumes immediate post op appearance if
muscle repair adequate
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106. Secondary deformities
Unilateral cleft lip
– Short upper lip
• Most common after straight line repair
– Rotation advancement flap useful
– Indications
• Cleft philtral scar short
• Cupid’s bow pulled up toward nostril
• Wide nostril floor
• Alar displacement laterally and downwards
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107. Secondary deformities
Unilateral cleft lip
– Short upper lip
• Millard repair
– Inadequate rotation
– Inadequate muscle repair
• Consider rerotation and muscle repair
• Triangular repair
– Flattening of Cupid’s bow
– Shift of vermilion tubercle to cleft side
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108. Secondary deformities
Unilateral cleft lip
– Long upper lip
• More common in triangular and quadrangular
repairs
• Unusual to have overrotation of rotation
advancement flap
• Horizontal excision at alar base
– Full thickness
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110. Secondary deformities
Unilateral cleft lip
– Tight upper lip
– Abbe flap
• Brings lower lip pouting tissue
to upper lip
• Most often with bilateral
repairs
– Recreates philtrum
• Rotate on intact labial artery
and vein
• 1/3 of lower lip can be
harvested
– Mental crease should not be
violated
• Division of pedicle after 10-14
days
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111. Secondary deformities
Unilateral cleft lip
– Unfavorable scars
• 1st
scar often the best
– Often restraint between 8 to 18 years best
• Hypertrophic or widened scars
– Present one month post op
– Red, raised and firm
– Taping
• Revision
• Pink scar
– Yellow light laser
• Dermabrasion
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112. Secondary deformities
Unilateral cleft lip
– Unfavorable scars
• Revisional techniques
– Excision and closure
• Straight line
• Wave line
• Z plasty
• W plasty
• Stair step technique
– Philtral column
• Epithelium is resected
• Leave dermis for bulk
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114. Secondary deformities
Unilateral cleft lip
– Orbicularis oris derangement
• Secondary repair of muscle
– Orient fibers transversely across defect
• Muscle layers
– Superficial
– Deep
• Peripheral and marginal slips
– Separate repair of different layers recommended
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115. Secondary deformities
Bilateral cleft lip
– More commonly has secondary deformity
– Issues
• Scars
• Tight lip
• Wide lip
• Short lip
• Missing or misplaced landmarks
• Vermilion deficiencies
• Buccal sulcus abnormalities
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116. Secondary deformities
Bilateral cleft lip
– Scars
• Same approach as unilateral
• Millard
– Revise scars on side at a time
• Avoid excessive tension
– Bank excessive lip scar
• Useful for columellar lengthening
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117. Secondary deformities
Bilateral cleft lip
– Tight lip
• Often associated with severe clefts
– Innate shortage of lip tissue
– Overresection of tissue at primary repair
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118. Secondary deformities
Bilateral cleft lip
– Tight lip
• Lip switch
– Abbe flap
– Midline placement
– Attempt recreation of
philtrum
– Dimensions
• 0.8-1.2 cm wide at
vermilion border
• 0.6-0.9 cm at base of
columella
• 1.7 cm high
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119. Secondary deformities
Bilateral cleft lip
– Wide lip
• Classic
– Failure to reunite orbicularis oris muscle during
primary surgery
– Gradual widening of philtrum
• Correction
– Muscle realigning techniques
– Removal of excess philtral skin
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120. Secondary deformities
Bilateral cleft lip
– Short lip
• More common in bilateral clefts
– Greater tissue deficiency
• Z plasty
– Lengthens by reducing horizontal dimension
• Can need Abbe flap
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121. Secondary deformities
Bilateral cleft lip
– Missing or misplaced landmarks
• Missing philtral landmarks
– Absent on prolabium of bilateral clefts
• Same as with unilateral secondary deformity
repair
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122. Secondary deformities
Bilateral cleft lip
– Vermilion deficiency and
irregularities
• Paucity of central lip
• Whistle deformity
– Thin central lip
– Relative
• Excessive vermilion
laterally
• Transverse wedge
excisions
– Tendency to contract
• Bulky design of flaps
necessary
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