Transcript Heat Shock Proteins and the Rat Dorsal Island Flap

Cleft Lip: Primary and
Secondary Deformities
Nadia Afridi MD, BSc (Med)
Kenneth Wilson MD, FRCSC
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:
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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
Embryology basics
Embryology basics
Embryology basics
Facial development
– 6th week
• Medial nasal processes migrate and fuse
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
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
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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:
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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
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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
Genetics
Associated syndromes
– Blepharo-cheilo-dontic syndrome
• Eyelids
– Euryblepharon
– Ectropion
– Lagophthalmia
• Teeth
– Oligodontia
– Conical crown form
• Autosomal dominant
Genetics
Non syndromic presentations
– Cleft lip +/- palate
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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
Environmental agents
 Folic acid
– Beneficial effect
– Reduced incidence of unilateral cleft lip and
palate with at risk mothers
 Isotretinoin
– Accutane dysmorphic syndrome
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Rudimentary external ears
Absent/imperforate auditory canals
Triangular microcephalic skull
Cleft palate
Depressed midface
Brain/jaw/heart anomalies
Environmental agents
Altitude
– Higher relative risk in highlands
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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
– 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
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
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
Anatomy
Muscular deformity
– Muscular bulge
• Haphazard arrangement of muscle fibers
– Transverse/oblique/anteroposterior
– Orbicularis oris
• Two well defined components
– Deep orbicularis
Anatomy
Anatomy
Anatomy
Anatomy
Anatomy
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
Unilateral cleft lip
 Microform cleft lip (forme fruste)
– Presentation:
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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
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
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
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
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
Unilateral cleft lip
Unilateral complete cleft lip
– Alveolar (maxillary) segment
• Four positions
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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 cleft lip
Unilateral cleft lip
 Unilateral complete
cleft lip
– Narrow with no
collapse
• Rotation advancement
lip and nasal repair
• Static molding useful for
maintaining ideal arch
Unilateral cleft lip
 Unilateral complete
cleft lip
– Narrow with collapse
• Ideal with presurgical
palatal orthopedic
expansion
– Start at 2 weeks
– Continue until surgical
intervention
Unilateral cleft lip
 Unilateral complete
cleft lip
– Wide without collapse
• Molding appliance
– Maintain width
– Guide alveolar
segments together
• Lip adhesion
Unilateral cleft lip
 Unilateral complete
cleft lip
– Wide with collapse
• Presurgical appliance
– Expands collapse
– Molds to reduce width
of interalveolar space
Unilateral cleft lip
Unilateral complete cleft lip
– Lip adhesion
• Highly selected unilateral and bilateral wide
clefts
• Temporarily brings lip margins together
Unilateral cleft lip
Options for presurgical molding
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
Unilateral cleft lip
Evolution of cleft lip repair
– 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
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)
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
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
Unilateral cleft lip
Evolution of cleft lip repair
– 7th principle:
• Restoration of normal nasal anatomy
– Complex
– Topic unto itself (stay tuned for next week!)
Unilateral
cleft lip
Millard repair
Unilateral cleft lip
Unilateral cleft lip
Unilateral cleft lip
Unilateral cleft lip
Unilateral cleft lip
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
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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
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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
Bilateral cleft lip
Bilateral cleft lip
Bilateral cleft lip
Bilateral cleft lip
Bilateral cleft lip
Bilateral cleft lip
Bilateral cleft lip
Bilateral cleft lip
Secondary deformities
Introduction
– Factors in decreased need for revisionary
surgery:
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Improved primary techniques
Specialized centers of excellence
Sophisticated presurgical orthodontics
Nasal correction simultaneously
Gingivoplasty
Nasal molding
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
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
Secondary deformities
Timeline
– Complete majority prior to school age
• Facilitate peer interactions
– Final revisions in adolescence
Secondary deformities
Unilateral cleft lip
– Deficient tubercle
– Vermilion deficiency and irregularities
– Short upper lip
– Long upper lip
– Tight upper lip
– Unfavorable scars
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
Secondary deformities
 Unilateral cleft lip
– Vermilion deficiency
and irregularities
• Notch “whistle
deformity”
– Inadequate
approximation of
orbicularis
Secondary deformities
 Unilateral cleft lip
– Vermilion deficiency
and irregularities
• Mucosal deficiency
– Z plasty
– V-Y advancement
Secondary deformities
 Unilateral cleft lip
– Vermilion deficiency and
irregularities
• Loss of Cupid’s bow
– Unilateral Gillies operation
– Triangular skin excision
above mucocutaneous line
– Close horizontally
– Modified Abbe flap
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
Secondary deformities
 Unilateral cleft lip
– Vermilion deficiency and
irregularities
• Lateral vermilion
deficiencies
– Lower lip vermilion flap
– Centrally based cross lip
flap
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
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1st 2 months
Maximal at 6-8 weeks
Softens and relaxes subsequently
Resumes immediate post op appearance if muscle
repair adequate
Secondary deformities
Unilateral cleft lip
– Short upper lip
• Lip lengthening techniques
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Rotation advancement flaps
Z plasties
V-Y forked flaps
Muscle advancements
Abbe flap
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
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
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
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
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
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
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
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
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
Secondary deformities
Bilateral cleft lip
– More commonly has secondary deformity
– Issues
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Scars
Tight lip
Wide lip
Short lip
Missing or misplaced landmarks
Vermilion deficiencies
Buccal sulcus abnormalities
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
Secondary deformities
Bilateral cleft lip
– Tight lip
• Often associated with severe clefts
– Innate shortage of lip tissue
– Overresection of tissue at primary repair
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
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
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
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
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
Secondary deformities
 Bilateral cleft lip
– Vermilion deficiency and
irregularities
– Lateral vermilion flaps
• V-Y advancement
Secondary deformities
 Bilateral cleft lip
– Vermilion deficiency and
irregularities
– Lateral vermilion flaps
• Double pendulum
flaps
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
Secondary deformities
Bilateral cleft lip
– Orbicularis oris deformities
• Proper muscle reconstruction
– Perioral and perinasal
– Minimizes secondary skeletal deformities
Secondary deformities
Bilateral cleft lip
– Lower lip changes
Secondary deformities
Residual skeletal deformities
– Issues
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Maxillary hypoplasia
Alveolar bone grafting
Orthognathic surgery
Palatal fistulas
– anterior