CLEFT

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Cleft Lip Primary and Secondary Deformities

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• Nadia Afridi MD, BSc (Med)
• Kenneth Wilson MD, FRCSC

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

3
Historical background

• Cleft lip
• Millard
• 1955
• Concept
• Lateral flap advancement into upper lip
• Downward rotation of medial segment
• Preserves Cupids bow and philtral dimple
• Tension of closure at alar base
• Reduces nasal flare
• Improved alveolar molding
• Most popular method for unilateral lip closure

4
Embryology basics

• Primary germ layers
• Ectoderm
• Cutaneous,

5
Embryology basics
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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

7
Embryology basics
8
Embryology basics
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Embryology basics
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Embryology basics

• Facial development
• 6th week
• Medial nasal processes migrate and fuse

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Embryology of Clefting
Facial Development
6th week
Medial nasal processes (green) migrate toward
each other and fuse
7th week
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
Failure of maxillary swellings to fuse with
intermaxillary process cleft lip
Clinical Aspects of Cleft Lip/Palate
Reconstruction
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Classification

• Standardized methods
• Key anatomic structure
• Incisive foramen
• Primary
• Lip
• Premaxilla
• Alveolus
• Secondary
• Soft palate
• Hard palate

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Classification

• Cleft of primary palate (cleft lip)
• Unilateral
• Incomplete
• Lip only
• Complete
• Primary palate
• Lip, nasal floor, alveolus

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Classification

• Cleft of primary palate (cleft lip)
• Bilateral
• Incomplete
• Lip only
• Complete
• Primary palate
• Lip, nasal floor, alveolus

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Classification

• Standardized methods
• Kernahan
• 1971
• Striped Y
• Incisive foramen as focal point
• Position 7
• Hard palate
• Position 8
• Soft palate
• Position 9
• Submucous cleft

16
Classification

• Standardized methods
• Millard modification

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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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Epidemiology

• Relative incidence
• Fraser and Calnan
• 21 cleft lip
• 46 cleft lip and palate
• 33 cleft palate
• Left gt right gt bilateral
• 631

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Epidemiology

• Associated factors
• Parental age
• Incidence increases with age
• Fathers 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

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

21
Epidemiology

• Associated defects
• Overall incidence of associated defects 29
• CNS malformations
• Club foot
• Cardiac abnormalities
• Highest with isolated cleft palate

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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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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Genetics

• Associated syndromes
• Stickler
• Cleft palate alone
• Autosomal dominant
• Type 2 collagen gene mutation
• Myopia, retinal detachment and glaucoma
• Van der Woudes syndrome
• Autosomal dominant
• Bilateral lower lip pits
• Absence second molars

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Genetics

• Associated syndromes
• Blepharo-cheilo-dontic syndrome
• Eyelids
• Euryblepharon
• Ectropion
• Lagophthalmia
• Teeth
• Oligodontia
• Conical crown form
• Autosomal dominant

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Genetics

• Non syndromic presentations
• Cleft lip /- palate
• Different chromosome regions
• 6p23
• 2q13
• 19q13.2
• Cleft palate alone
• Recessive single major gene
• ? 2q13/TGFA

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Environmental agents

• Chemical agents
• Animal model
• Alcohol
• No increased risk of cleft with low quantities of
  EtOH
• Increased risk of clefting with higher quantities
  of EtOH
• Dilantin
• 10X higher risk of cleft lip
• Smoking
• Dose response relationship
• Increased risk of clefting

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Environmental agents

• Folic acid
• Beneficial effect
• Reduced incidence of unilateral cleft lip and
  palate with at risk mothers
• Isotretinoin
• Accutane dysmorphic syndrome
• Rudimentary external ears
• Absent/imperforate auditory canals
• Triangular microcephalic skull
• Cleft palate
• Depressed midface
• Brain/jaw/heart anomalies

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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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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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Genetic counseling
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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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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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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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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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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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Anatomy

• Millard
• Critical anatomic features of unilateral cleft
  lip
• Non cleft side
• Shortened philtral height
• Shortened columella
• Two thirds of Cupids bow, one philtral column
  and a dimple hollow preserved
• Hypoplastic muscle between philtral midline and
  cleft

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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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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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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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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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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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Unilateral cleft lip

• Unilateral incomplete cleft lip
• Varying degree of vertical separation of the lip
• Intact nasal sill
• Simonarts band
• Corrected with rotation advancement repairs
• Nasal repair carried out with primary repair

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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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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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Unilateral cleft lip
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Unilateral cleft lip

• Unilateral complete cleft lip
• Narrow with no collapse
• Rotation advancement lip and nasal repair
• Static molding useful for maintaining ideal arch

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Unilateral cleft lip

• Unilateral complete cleft lip
• Narrow with collapse
• Ideal with presurgical palatal orthopedic
  expansion
• Start at 2 weeks
• Continue until surgical intervention

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Unilateral cleft lip

• Unilateral complete cleft lip
• Wide without collapse
• Molding appliance
• Maintain width
• Guide alveolar segments together
• Lip adhesion

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Unilateral cleft lip

• Unilateral complete cleft lip
• Wide with collapse
• Presurgical appliance
• Expands collapse
• Molds to reduce width of interalveolar space

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Unilateral cleft lip

• Unilateral complete cleft lip
• Lip adhesion
• Highly selected unilateral and bilateral wide
  clefts
• Temporarily brings lip margins together

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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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Unilateral cleft lip

• Evolution of cleft lip repair
• 2nd principle
• Using lateral lip tissue in deficient medial
  segment
• 3rd principle
• Retaining normal anatomic Cupids bow
• Hagerdon, LeMesurier
• Quadrangular flap
• Tennison Randall
• Triangular flap

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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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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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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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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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Unilateral cleft lip

• Millard repair

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Unilateral cleft lip
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Unilateral cleft lip
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Unilateral cleft lip
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Unilateral cleft lip
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Unilateral cleft lip
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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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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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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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Bilateral cleft lip

• Incomplete bilateral cleft lip
• Near normal nose
• Normal premaxilla
• Simonarts 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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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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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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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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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 Cupids bow
• Scar mimics white roll
• Good bulk

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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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Bilateral cleft lip
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Bilateral cleft lip
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Bilateral cleft lip
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Bilateral cleft lip
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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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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 bows
• Eversion of central upper lip
• Minimal scar
• Failure of above needs secondary repair

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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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Secondary deformities

• Timeline
• Complete majority prior to school age
• Facilitate peer interactions
• Final revisions in adolescence

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Secondary deformities

• Unilateral cleft lip
• Deficient tubercle
• Vermilion deficiency and irregularities
• Short upper lip
• Long upper lip
• Tight upper lip
• Unfavorable scars

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Secondary deformities

• Unilateral cleft lip
• Deficient tubercle
• V-Y advancement
• Dermal graft
• Create tunnel along horizontal length with
  orbicularis
• Rotate medial edges of vermilion mucosal flaps
  inferiorly
• Temporoparietal fascia flap

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Secondary deformities

• Unilateral cleft lip
• Vermilion deficiency and irregularities
• Notch whistle deformity
• Inadequate approximation of orbicularis

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Secondary deformities

• Unilateral cleft lip
• Vermilion deficiency and irregularities
• Mucosal deficiency
• Z plasty
• V-Y advancement

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Secondary deformities

• Unilateral cleft lip
• Vermilion deficiency and irregularities
• Loss of Cupids bow
• Unilateral Gillies operation
• Triangular skin excision above mucocutaneous line
• Close horizontally
• Modified Abbe flap

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Secondary deformities

• Unilateral cleft lip
• Vermilion deficiency and irregularities
• Loss of philtral column
• Limited surgical useful techniques
• Subcutaneous rotation flap
• Rollover muscle flap
• Chondrocutaneous composite flap
• Auricular cartilage graft
• Muscle splitting technique
• Vest over pants closure

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Secondary deformities

• Unilateral cleft lip
• Vermilion deficiency and irregularities
• Lateral vermilion deficiencies
• Lower lip vermilion flap
• Centrally based cross lip flap

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Secondary deformities

• Unilateral cleft lip
• Short upper lip
• Measure of distance from Cupids 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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Secondary deformities

• Unilateral cleft lip
• Short upper lip
• Lip lengthening techniques
• Rotation advancement flaps
• Z plasties
• V-Y forked flaps
• Muscle advancements
• Abbe flap

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Secondary deformities

• Unilateral cleft lip
• Short upper lip
• Most common after straight line repair
• Rotation advancement flap useful
• Indications
• Cleft philtral scar short
• Cupids bow pulled up toward nostril
• Wide nostril floor
• Alar displacement laterally and downwards

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Secondary deformities

• Unilateral cleft lip
• Short upper lip
• Millard repair
• Inadequate rotation
• Inadequate muscle repair
• Consider rerotation and muscle repair
• Triangular repair
• Flattening of Cupids bow
• Shift of vermilion tubercle to cleft side

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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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Secondary deformities

• Unilateral cleft lip
• Tight upper lip
• Horizontal tightness across upper lip
• Z-plasty
• Restricts anteroposterior facial growth
• Relative pouting lower lip
• Correction with Abbe flap

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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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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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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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Secondary deformities

• Unilateral cleft lip
• Buccal sulcus abnormalities
• Adhesions/scars
• Z plasty
• Z-Y technique
• Deepening of sulcus
• STSG
• Oral mucosal free grafts
• Local mucosal flaps

99
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

100
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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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

102
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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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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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

105
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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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

107
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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Secondary deformities

• Bilateral cleft lip
• Vermilion deficiency and irregularities
• Lateral vermilion flaps
• V-Y advancement

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Secondary deformities

• Bilateral cleft lip
• Vermilion deficiency and irregularities
• Lateral vermilion flaps
• Double pendulum flaps

110
Secondary deformities

• Bilateral cleft lip
• Buccal sulcus abnormalities
• Local flaps
• Skin grafts
• Needs splinting
• Second choice with children
• V-Y advancement of entire labial sulcus
• Combination
• Z plasty
• VY advancement

111
Secondary deformities

• Bilateral cleft lip
• Orbicularis oris deformities
• Proper muscle reconstruction
• Perioral and perinasal
• Minimizes secondary skeletal deformities

112
Secondary deformities

• Bilateral cleft lip
• Lower lip changes

113
Secondary deformities

• Residual skeletal deformities
• Issues
• Maxillary hypoplasia
• Alveolar bone grafting
• Orthognathic surgery
• Palatal fistulas
• anterior

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