Cleft lip primary & secondary deformities /certified fixed orthodontic courses by Indian dental academy

Cleft Lip: Primary and
Secondary Deformities
INDIAN DENTAL ACADEMY
Leader in continuing dental education
www.indiandentalacademy.com

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

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

Embryology basics
Primary germ layers
– Ectoderm
• Cutaneous,

Embryology basics

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

Embryology basics
Facial development
– 6th
week
• Medial nasal processes migrate and fuse

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

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

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

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

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

Classification
Standardized
methods
– Millard modification

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

Epidemiology
Relative incidence
– Fraser and Calnan
• 21% cleft lip
• 46% cleft lip and palate
• 33% cleft palate
• Left > right > bilateral
– 6:3:1

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

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

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

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

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

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

Genetics
Associated syndromes
– Blepharo-cheilo-dontic syndrome
• Eyelids
– Euryblepharon
– Ectropion
– Lagophthalmia
• Teeth
– Oligodontia
– Conical crown form

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

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

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

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

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

Genetic counseling

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

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

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

Anatomy
Millard
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

Anatomy
Millard
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

Anatomy
Millard
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

Anatomy
Muscular deformity
– Muscular bulge
• Haphazard arrangement of muscle fibers
– Transverse/oblique/anteroposterior
– Orbicularis oris
• Two well defined components
– Deep orbicularis

Anatomy

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

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

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

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

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

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

– 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

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

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

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

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

– Lip adhesion
• Highly selected unilateral and bilateral wide
clefts
• Temporarily brings lip margins together

Options for presurgical molding

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

– 2nd
principle:
• Using lateral lip tissue in deficient medial
segment
– 3rd
principle:
• Retaining normal anatomic Cupid’s bow
– Hagerdon, LeMesurier
• Quadrangular flap
– Tennison Randall
• Triangular flap

– 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)

– 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

– 6th
principle:
• Restoration of the bony platform
• Presurgical orthopedics
– Passive
– Active
• Latham appliance
• Bone grafting
• Gingivoperiosteoplasty
– controversial

– 7th
principle:
• Restoration of normal nasal anatomy
– Complex
– Topic unto itself (stay tuned for next week!)

Unilateral
cleft lip
Millard repair

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

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

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

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

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

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

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

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

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

Bilateral cleft lip

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

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

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

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

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

– 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

– Vermilion deficiency
and irregularities
• Notch “whistle
deformity”
– Inadequate
approximation of
orbicularis

– Vermilion deficiency
and irregularities
• Mucosal deficiency
– Z plasty
– V-Y advancement

– Vermilion deficiency and
irregularities
• Loss of Cupid’s bow
– Unilateral Gillies operation
– Triangular skin excision
above mucocutaneous line
– Close horizontally
– Modified Abbe flap

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

irregularities
• Lateral vermilion
deficiencies
– Lower lip vermilion flap
– Centrally based cross lip
flap

– 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

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

– 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

– 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

– 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

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

– 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

• 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

• 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

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

– 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

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

– Scars
• Same approach as unilateral
• Millard
– Revise scars on side at a time
• Avoid excessive tension
– Bank excessive lip scar
• Useful for columellar lengthening

– Tight lip
• Often associated with severe clefts
– Innate shortage of lip tissue
– Overresection of tissue at primary repair

– 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

– 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

– Short lip
• More common in bilateral clefts
– Greater tissue deficiency
• Z plasty
– Lengthens by reducing horizontal dimension
• Can need Abbe flap

– Missing or misplaced landmarks
• Missing philtral landmarks
– Absent on prolabium of bilateral clefts
• Same as with unilateral secondary deformity
repair

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

irregularities
– Lateral vermilion flaps
• V-Y advancement

irregularities
– Lateral vermilion flaps
• Double pendulum flaps

– 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

– Orbicularis oris deformities
• Proper muscle reconstruction
– Perioral and perinasal
– Minimizes secondary skeletal deformities

– Lower lip changes

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

Thank you
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Cleft lip primary & secondary deformities /certified fixed orthodontic courses by Indian dental academy

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