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Cleft lip and its Management
Dr. Abirami.K
PG OMFS
Introduction
Cleft lip:
• A congenital defect in lip continuity due to an embryological
malformation
History
The 1st documented CL surgery was from china in 390 BC in a 18 years old soldier-
wey young chi
■ Jehan yeperman - 1st to describe the unilateral and bilateral CL repair
■ Ambrose pare - 1st diagrammatic representation of CP repair in 14th century
■ Le monnier - 1st successful CP repair in paris (1776)
■ Asensio - Novel technique of CL repair in 1950
■ Germanicus mirault - Originator of triangular flap - modified by tennison and randall
■ Ralph millard - classic- rotational advancement technique in mid 1950s
Applied anatomy
Anatomical LandmarksSKIN
WHITE LIP
• Philtral columns - vertical ridges forming the lateral
boundaries of the philtrum
• Philtral dimple - concave central region where no muscles
insert
• Cupid’s bow - the area of the white-red lip margin at the
base of the philtrum
• White roll - the prominent ridge between white-red lip
formed by the anterior projection of the pars marginalis of
the orbicularis
RED LIP
• Vermillion - squamous epithelium, dry due to a lack of
mucous glands: contains glands of fordyce.
• Superficial capillaries gives red colour
• Red line (of noordhoff) - represents the abrupt transition
from squamous epithelium to mucosa
• Mucosa - wet due to mucous glands
• Central tubercle
Musculature of LipsGroup Origin Muscle/motor Origin Insertion Function
Group I
insert
into
modiolus
Obicularis oris
(buccal &
mandibular branch
CNVII)
Modiolus modiolus, dermal
attachments
(philtral columns,
“white roll” = pars
marginalis)
purses lips, oral
Continence.
buccinator (buccal
br. VII)
posterior alveolar
process of
maxilla,
pterygomandibular
raphe, body of
mandible
Modiolus press
lips/cheeks
against teeth
levator anguli oris
(buccal&zyg. br)
canine fossa of maxilla
below
infraorbital foramen
Modiolus elevates
commissure
depressor anguli
oris (mand. br VII)
oblique line on
anterior
mandible below
canine and
Modiolus depress/lateral
shift
commissure
>T. quauratus lnbti superiors* M. uLcmrcis Jabit gup^rLovi*
M. ori'Ictilaris nm*
M. bttceiual*r
M. Cr]&ijj'tLtf) cijt
Nr. mcnralis
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Group Muscle/motor Origin Insertion Function
Zygomaticus major
(Zyg&buccal br.)
Zygoma just lateral to
Zygomaticotemporal
suture
Line, deep to
obicularis
Modiolus Elevate/lateral
Shift
Commissure
Risorius (buccal br) Parotid fascia Modiolus Lateral shift of
Commissure
Group II
insert
into
upper lip
Levator labii
Superioris (buccal)
Infraorbital rim
above
Upper lip dermis
And obicularis
oris
Elevates upper
Lip
Levator labii
Superioris aleque
Nasi (buccal br.)
Infraorbital foramen
Frontal process of
maxilla
Lower lateral
Cartilage, upper
lip
Dilates nostril,
Zygomaticus minor
(Buccal br.)
Zygoma, deep to
orbicularis
Oculi and lateral to
Zygomaticomaxillary
Upper lip Elevates upper
Lip
Nasolabial fold
Group Muscle/motor Origin Insertion Function
Group
III
Insert
Into
Lower lip
Depressor labii
Inferioris (mand.
Br)
Anterolateral
mandible and
Medial to
insertion of
Depressor anguli
oris
Lower lip dermis
And obicularis
oris
Lower lip
Depressor and
Lateral shift
Mentalis (mand.
Br)
Anterior midline
mandible
Dermis of chin Elevate lower
Lip
Platysma
(cervical
Br. Vii)
Deltopectoral
fascia
Inferior border
of
Mandible
Lip depressor
Neurovasular
Innervation:
• Sensory
• Upper lip: V2
• Lower lip: V3
Blood supply:
• superior and inferior
labial arteries
• superior/inferior labial
veins: drain into facial vein
Lymphatic drainage:
• Upper lip and lateral
portions of lower lip:
submandibular basin
• Central lower lip: submental
basin.
DORSAL NASAL
INFRA ORBITAL
MAXILLARY
POST SUP ALVEOLAR
BUCCAL
SUPERIOR LABIAL
INFERIOR LABIAL
INF ALVEOLAR terminal br
FACIAL
BLOOD SUPPLY LIPS
Embryology
• Development of the lip and palate
begins around the 4th week of
embryological development
• Completed by the end of the 12th
week
• By the end of the 4th week
- 5 facial prominences have formed
• frontonasal process
• paired maxillary processes
• paired mandibular processes
Embryology
• During the 5th week
- Nasal placodes invaginate to form the nasal pits
• Lateral and medial nasal prominences
Frontonasal
prominence
Maxillary
processes
Oral cavity
Mandibular
processes —
Medial nasal
processes
- Lateral
nasal
processes
— Nasal pits
Embryology
• By the end of the 6th week
- Paired maxillary processes have grown medially and pushed the paired medial nasal prominences
together
- Fusion of the paired medial nasal prominences form:
• Philtrum
• Middle upper lip
• Nasal tip
• Columella
- Fusion of the paired maxillary prominences with the paired medial nasal prominences forms the
complete upper lip (maxillary prominences form lateral lip)
- The lateral nasal prominences form the bilateral nasal ala
Oral cavity
Frontonasal
prominence
Maxillary
processes
Mandibular
processes
— Medial
nasal processes
— Lateral
nasal
processes
— Nasal pits
Alae
Cleft Lip and Palate Formation
Maxillary
processes
Oral cavity
• Fusion
- Failure of fusion of the maxillary and medial nasal prominences
unilaterally or bilaterally result in unilateral or bilateral cleft lip
with/without primary palate
- Failure of fusion of the palatal shelves result in clefts of the secondary
palate
Frontonasal
prominence
Mandibular
processes
Medial nasal
processes
Lateral nasal
processes
r} Nasal pits
cleft lip
Etiology
■ The exact cause of clefting is unknown
■ Multifactorial
Chemical Exposures
Radiation
Maternal Hypoxia
Teratogenic Drugs
Nutritional Deficiencies
Physical Obstruction
Genetic Influences.
Genetic (non- syndromal)
■ Genetic basis is significant, but not predictable
■ Lack of potential for mesodermal proliferation across the fusion
lines after the component parts are in contact.
■ Error in the transitional shift of the embryonic blood supply.
■ Chromosomal abnormalities causing multiple congential
malformations including cleft
Etiology
Environmental
Intensity, duration and the time of action - more
important than etiological factor
1. Infections (rubella, toxoplasmosis)
2. Growth hormone deficiency
3. Drugs: Steroids, Diazepam, Aminopterin,
Anticonvulsants (incidence10X), Aspirin,
4. Alcohol: fetal alcohol syndrome
5. Smokers (2X incidence)
6. Vitamin B and Folate deficiency
7. Radiation energy
8. Hypoxia and amniotic fluid alteration
• Some of these
genes include the
MSX, LHX,
goosecoid, and DLX
genes.
Group Syndrome Cleft
sporadic Klippel-Feil syndrome
Shprintzen Syndrome
CP
CP
Multifactorial Pierre Robin Sequence CP
major mutant genes Treacher Collins
Van der Woude
Syndrome
Waardenburg syndrome
Stickler Syndrome
Ectodermal Dysplasia
syndromes
CP
CL(P), CP
CL(P)
CP
CP
Chromosomal
aberrations
Down’s Syndrome
Patau Syndrome
CL(P),
CP
Teratogenic Dilantin
Fetal Alcohol syndrome
Accutane dysmorphic
syndrome
Nutritional deficiency
Epidemiology
■ Cleft lip and palate occurs in about 1 to 2 per 1000 births in the developed world. Rates for
cleft lip with or without cleft palate and cleft palate alone varies within different ethnic
groups.
■ The highest prevalence rates for (CL ± P) are reported for Native Americans and Asians .
Africans have the lowest prevalence rates.
■ Native Americans: 3.74/1000
■ Japanese 0.82/1000 to 3.36/1000
■ Chinese 1.45/1000 to 4.04/1000
■ Caucasians 1.43/1000 to 1.86/1000
■ Latin Americans : 1.04/1000
■ Africans: 0.18/1000 to 1.67/1000
■ Watkins, SE; Meyer, RE; Strauss, RP; Aylsworth, AS (April 2014). "Classification,
epidemiology, and genetics of orofacial clefts.". Clinics in plastic surgery. 41 (2): 149-63
■ CLP: M:F = 2:1
■ Isolated palates: F:M = 2:1
■ Site of clefting : Left: Right: Bilateral 6:3:1
CRANIO-FACIAL CLEFT
Tessier(1973):Orbitocentric
classification of facial clefts.
• Facial clefts are numbered 0-7
• Cranial clefts from 8-14.
• Orbit - reference landmark
• Cleft lip is part of clefts 2 and 3.
CLASSIFICATION
1. DAVIS AND RITCHIE CLASSIFICATION:
The following classification was proposed by Davis and Ritchie in 1922.
This system broadly categorized the clefts into three groups according to
position of cleft in relation to alveolar process.
Group I – Pre alveolar clefts:
• Unilateral cleft lip
• Bilateral cleft lip
• Median cleft lip
Group II - Post alveolar clefts:
• Cleft hard palate alone
• Cleft soft palate alone
• Cleft soft palate and hard palate
• Sub mucous cleft
Group III-Alveolar clefts:
• Unilateral alveolar cleft
• Bilateral alveolar cleft
• Median alveolar cleft
Classification
2. VEAU CLASSIFICATION:
• Veau proposed the following classification in
1931.
• Group I (A) - Defects of the soft palate only
• Group II (B) - Defects involving the hard palate and
soft palate extending not further than the incisive
foramen, thus involving the secondary palate
alone.
• Group III (C) – Complete unilateral cleft, extending
from the soft palate to the alveolus, usually
involving the lip
• Group IV (D) - Complete bilateral clefts, resembles
Group III but is bilateral. When cleft is bilateral,
pre-maxilla is suspended from the nasal septum.
Classification
LAHSHAL system of classification:
• Kreins O proposed LAHSHAL system for classification
of cleft lip and palate patients which was modified on
the recommendation of Royal College of Surgeons
Britain in 2005 by omitting one “H” from the
acronym “LAHSHAL”.
• LAHSAL system is a diagrammatic classification of
cleft lip and palate.
• According to this classification, mouth is divided into
six parts.
• Right lip
• Right alveolus
• Hard palate
• Soft palate (LAHSAL)
• Left alveolus
• Left lip
Kernahan striped Y classification
In 1971
Areas 1 and 4 – Lip
Areas 2 and 5 – Alveolus
Areas 3 and 6 – Primary palate
Areas 7 and 8 - Hard palate
Area 9 - Soft palate
The small circle represents incisive papilla.
MODIFIED STRIPED-Y ELSAHY CLASSIFICATION
• Triangle 1 & 5- the fusion line between the
maxillary prominence and medial nasal prominence
at nostril floor
• Square 2&6 - the fusion line between the
maxillary prominence and medial nasal prominence
at upper lip level
• Square 3 & 7 - the alveolus
• Square 4 and 8- primary and secondary hard palate
• Squares 9 and 10 - the fusion line between palatine shelves
of hard palate and lies posterior to the incisive foramen.
• Square 11 - the soft palate.
• Circle 12 - the posterior pharyngeal wall.
• Circle 13 represents the pre-maxilla.
FRIEDMAN’S CLASSIFICATION (1991)
STEP I:
This step includes the depiction of the deformity
of the nasal arches (triangles 1 and 6)and nasal
floors (triangles 2 and 7). In each triangular space,
defect is recorded in the following manner.
0 = No involvement
1 = Cleft microform (e.g; notching)
2 = Mild deformity (i.e; some actual tissue deficiency)
3 = Moderate deformity
4 = Severe deformity
X = Not rated
STEP II:
It includes the depiction of the amount of prolabium
or pre-maxillary protrusion on one or both
sides (semi circles 14 and 15).
0 = No protrusion
1 = Mild protrusion (<45p )
2 = Moderate protrusion (>45p ,<90p )
3 = Severe protrusion(>90p )
X = Not rated
FRIEDMAN’S CLASSIFICATION (1991)• STEP III:
• This step depicts the upper lip deformity.
• 0 = No involvement
• 1 = Cleft microform
• 1a = Congenital scar in usual cleft position
• (subcutaneous cleft)
• 1b = Notch in the vermillion border
• 2 = 1/3 cleft lip (cleft in vertical dimension)
• 3 = 2/3cleft lip (cleft in vertical dimension)
• 4 = Complete cleft lip
• X = Not rated
• STEP IV:
• It shows the defect in the alveolus.
• 0 = No involvement
• 1 = Cleft microform1a = Submucous cleft
• 1b = Notch
• 2 = Partial cleft
• 3 = Complete cleft
• 3a = Absence of maxillary arch collapse
• 3b = Presence of maxillary arch collapse
• X = Not rated
STEP V:
It represents the pre-incisive foramena
trigones.
0 = No involvement
1= Partial cleft
2= Complete cleft
X= Not rated.
STEP VI:
It describes the anatomy of the
hard palate
0 = No involvement
1 = Posterior 1/3 cleft
2 = Posterior 2/3 cleft
3 = Complete cleft
X = Not rated.
FRIEDMAN’S CLASSIFICATION (1991)
STEP VII:
• It indicates a defect in the soft palate or velum
including uvula.
• 0 = No involvement
• 1= Cleft microform of uvula
• 1a = Hypoplasia of musculus uvulae
• 1b = Septate uvula
• 1c = Bifid uvula
• 2 = Submucous cleft of velum
• 2a = Occult
• 2b = Overt
• 3= Posterior 1/3of velum
• 4= Posterior 2/3 of velum
• 5 = Complete cleft of velum
• X = Not rated.
STEP VIII:
It represents velopharyngeal
valve dysfunction
0 = No impairment
1 = Mild impairment
2 = Moderate impairment
3 = Severe impairment
X = Not rated
DEFECTS IN CLEFT LIP AND PALATE PATIENTS
Deformities:
1. Dental Problems:
Number, Shape, Eruption, Mineralization.
2. Skeletal Problems:
Max. deficiency, Mand.prognathism,
Class III malcclusion.
3. Nasal problems:
Alae flared, Columella pulled to non cleft side.
4. Feeding Problems:
Nasal Regurgitation, Weak sucking, Weak
swallowing reflux.
DEFECTS IN CLEFT LIP AND PALATE
PATIENTS
4. Ear Problems:
Recurrent Middle Ear infection, Possible
Deafness
5. Speech Problems:
Retardation of consonants, Hyper nasality,
Articulation Defects, Hearing problem
6. Associated Anomalies:
Congenital heart defects, Mental retardation
CLINICAL FEATURES
UNILATERAL CLEFT LIP
(a) Microform / Forme fruste
• small notch within the borders of the vermillion
• band of fibrous tissue running from edge of red lip to nostril
floor
• deformity of ala on side of notch or band
(b) Partial Cleft Lip
• Up to 2/3 of lip
• Vermillion involved
• Orbicularis may be partially divided
• Nasal deformity more marked (displaced alar base, asymmetric
tip, flat ala)
• Bony hypoplasia more evident
(c) Simonart’s Band
• a narrow bridge of tissue, in an otherwise complete cleft lip
crossing the cleft
• lip to lip or lateral lip to medial alveolar mucosa
• significance: functional - potentially reduces the deformity
(d) Complete Cleft Lip
• ± Simonart’s band
CLINICAL FEATURES
Structure Cleft side Non cleft side
Maxilla 1.Lateral segment retropositioned
2.Piriform margin hypoplastic & retropositioned
Premaxilla outwardly rotated and
projecting
Nasal septum Mid-septal bulge into cleft side Inferior septum dislocated out of
vomer groove
Columella Shortened columella, with tip deviated to cleft side Base deviated to non-cleft side
Lip
Philtrum short
Shortened philtral height
Preserved 2/3 of Cupid’s bow (one
philtral column and dimple
hollow)
Lateral lip White roll deficient as it progresses into cleft
Vermillion decreased on medial side of cleft, normal
to increased laterally
Orbicularis
oris
- deep portion interrupted → no sphincteric function
- superficial portions parallel cleft margins to insert
into columella & ala
- no decussation to form the philtral ridge
Incomplete clefts
-muscle does not cross cleft unless bridge is
≥ 1/3rd the height of the lip
Heaped up to form visible bulge of lateral lip
element
Hypoplastic muscle between philtral
midline and cleft
Muscle may insert into columellar
Base
Clinical FeaturesSkeleton
• Premaxilla projecting ahead of lateral segments
• Lateral segments retropositioned and medially collapsed
• Maxilla hypoplastic at piriform margins and alar bases
Nose
• Septum midline
• Alae dislocated inferolaterally, with recessed bases
• Alae spread wide, with slump along lateral crura
• Short columella
• Flat, wide, tip
Prolabium
• Variable in size; can be hypoplastic
• No muscle (present in incomplete cases); skin appendages may be deficient / absent
• Absent white roll, cupid’s bow, philtral columns, central dimple & tubercle
• Deficient gingival buccal sulcus
• Hypoplastic vermillion
Lateral Lip
• Vertically shortened
• Orbicularis heaped up to form a visible bulge of the lateral lip element
• Orbicularis abnormally inserted into alar bases
Goals of Treatment
• Steffenson (1953) - five criteria for a satisfactory lip repair:
■ 1. Accurate skin, muscle and mucous membrane union with adequate lip lengthening,
■ 2. Symmetrical nostril floor,
■ 3. Symmetrical vermilion border,
■ 4. Slight eversion of the lip and
■ 5. A minimal of scar, which by contraction will not interfere with the accomplishments of the other,
stated requirements
Two additional criteria were added later by Musgrave (1971)
■ 1. Preservation of the cupid’s bow,
■ 2. Production of symmetrical nostrils.
Timing
Procedure Age
1st appointment (± prenatal consult) in utero - 3 wks
Presurgical orthopedics 2 - 4 wks
1° lip & nasal repair 3 - 6 mos
Palate repair & myringotomy tubes 9 - 14 mos
Speech assessment & Tx 6 wk post-palate+
± VPI surgery 5-7 yrs+
Additional orthodontia Pre/post ABG
Alveolar bone grafting (ABG) 9-12 yrs
± 2° nasal repair 5-6 yrs
± Orthognathic surgery 16+ F, 18+ M
± Adult rhinoplasty, genioplasty 18 yrs +
Cleft Lip: Timing of Procedures for the Cleft Patient:
PRESURGICAL ORTHODONTIA
Repositioning of maxillary alveolar arch using an appliance
• Aim to decrease deformity before performing definitive lip surgery
• Used in conjunction with gingivoperiosteoplasty to achieve closure of alveolar cleft to
reduce the need for bone grafting
Techniques
• Active/fixed device - latham appliance fixed to palatal segments with pins
• Passive/loose appliance - customized appliance fit to roof of month; adjusted every few
weeks to guide growth
Indications
• Wide clefts
• Misaligned arches
Timing: age 2-5 weeks until cleft lip repair
Advantages
• Better correction of lip and nasal deformity
• Alignment of alveolar segments
• Possibility of 1 repair of alveolar cleft (gingivoperiosteoplasty)
Disadvantages
• Compliance
• Added cost
• Delay in lip surgery
• Concern regarding long-term growth imbalance
Lip Taping
• Effective in reducing the width of the cleft in a
nonsurgical manner
• Strip of hypoallergenic tape is placed with tension
across the cleft and secured to the patient’s cheek
• Molds bony tissues by applying pressure to protruding
portions of the maxilla
• Must be worn 24 hours per day
Indication:
• Wide cleft lip or premaxilla protrusion
Advantage:
• To narrow the cleft and mold the
premaxilla before proceeding with surgery
LIP ADHESION
• Suturing the medial and lateral lip segments together prior
to definitive lip repair
Indications:
• Wide complete clefts ± poorly aligned maxillary segments
Timing: 3-5wks of age
Advantages
• May facilitate gingivoperiosteoplasty
• Reduces width of cleft and molds maxillae
• Decreases lip tension at time of definitive closure
Disadvantages
• Additional procedure/OR/anaesthetic
• Increased scarring at time of definitive repair
• Final cosmetic outcome may be worsened or not improved
• Delays definitive lip repair
GINGIVOPERIOSTEOPLASTY
• Alveolar gap is bridged with mucoperiosteal flaps prior
to lip repair
• Evidence that some bone fills in the defect
Timing:
After alveolar segments realigned by lip adhesion or
presurgical orthodontia
Advantages
• May allow for normal tooth eruption
• May obviate need for bone graft in some patients
Disadvantages
• Adverse effect on facial & palatal growth
(controversial)
• Bone formed across gap may be insufficient for
osteointegrated implant or tooth eruption
Definitive lip surgery
Timing
Infant should by healthy and of acceptable weight before surgery to decrease Morbidity risk
• Generally around three months of age, when:
• 10 weeks of age
• 10 lbs
• 10 hemoglobin level
Earlier repairs:
Neonatal
• Repair undertaken in first few weeks of life
Advantages
• Less social concern & stigma for parents
Disadvantages
• Higher anaesthetic risk
• Delicate, small tissues → demanding surgery
• ? Any difference in results
Guidelines for safe neonatal surgery
• Babies should be gestationally mature and healthy
• No opioid analgesia perioperatively
• Experienced nurses
• Apnea monitors
General principles
• Tissues must be released enough to obtain a tension-free closure
• Accurate marking is essential
• Each cleft should be repaired with a customized approach
• Muscle should be released from skin and mucosa both medially and
laterally, to achieve functional cleft lip
• Vermilion flap from lateral side to augment the vermilion height on
the deficient medial side
• Primary correction of cleft nose: varies in complexity due to fears of
growth disturbance and scarring for later repairs
• Alveolar cleft: mucosa may be closed, but grafting is done at time of
mixed dentition
• Augmentation of medial lip with lateral lip tissue
TECHNIQUES OF REPAIR
• U/L CLEFT LIP
• Millard’s rotational advancement
• Modifications of millard’s
technique:
- Millard II
- Skoog’s Technique
- Mohler’s Technique
• Tennison-Randall Triangular flap
• Delaire’s Functional lip closure
B/L CLEFT LIP
• Straight line closure
• Columella lengthening
• Millard repair
• Tennison-Randall triangular flap
• Other techniques:
- Bauer Method
- Manchester method
- Skoog method
- Wynn method
- Barsky technique - Modified Veau II
Basic surgical preparation
• GA using oral RAE (Ring - Adair- Elwyn) tube taped to midline
of lower lip - not to produce any lateral distortion
■ The infant is placed in folded towels with the neck slightly
extended.
■ Points - 30 gauge needle - Bonney’s blue (Brilliant green
1/2 g, Crystal violet 1/2 g, Alcohol 96% 46ml, Water for
injection 53ml) or methylene blue.
■ After markings - 0.5% lignocaine with 1:2,00,000
epinephrine is injected into the lip tissue. It also helps to grip
lip firmly between thumb and index finger to avoid bleeding
while incising. Wait for 5 minutes after infiltration. GA using
oral RAE (Ring - Adair- Elwyn) tube taped to midline of lower
lip - not to produce any lateral distortion
A. Straight-line repair
1. Historically, the first cleft lip repairs relied
on freshening the edges of the cleft and
suturing them together. These have been
largely replaced by various Z-plasty-based
techniques.
2. Rose-Thompson repair:
a. Modified straight-line repair that can be
used for minor clefts with lip length nearly
equal on both sides of cleft (e.g., forme
fruste).
b. Fusiform excision with straight-line closure.
Indications:
Still used for mild forms of cleft lip
Lengthening effect achieved by opening of angle
between white-lip and red-lip.
Advantages
Straightforward
Disadvantages
Minimal lengthening is obtained
Destroys important landmarks
Quadrangular flap
• Proposed by LeMesurier and Hagedorn.
• Rectangular flap from lateral side of cleft is inset into a relaxing
incision on the medial side
• Cupid's bow is derived from the lateral lip.
• 90-degree Z-plasty.
• Violates Cupid's bow and philtral dimple.
• Has a tendency to produce a long lip.
Advantages
• Good results could be achieved in experienced hands
Disadvantages
• Cupid’s bow deformities - flat, notched, etc.
• Increased length on cleft side
• Large amount of tissue sacrificed
• Wide nasal floor
• Redundant red lip
Milliard Rotation-Advancement
• The rotation advancement repair
of the unilateral cleft lip deformity
as described by Millard
• Introduced in 1957
• Most widely used procedure for
unilateral cleft lip repair
• 46% of North American
surgeons, 38% modified versions
Millard Rotation-Advancement
Millard DR. Complete Unilateral Clefts of the Lip. Plas Recon Surg 1960 25(6), 595-605.
Markings/Design
■ 1. center/low point cupids bow
* 2. peak of bow on NCS
* 3, Peak of bow on medial side of cleft
* 4, Alar base, NCS
* 5. Columellar base, NCS
* 6, Commissure, NCS
* 7. Commissure, deft side
* 8, Peak cupids bow lateral side of cleft
* 9, Superior extent of advancement flap
* 10, Alar base, cleft side
* Point x: Back-cut point
1 to 2 = 1 to 3 = 2-4 mm
2 to 6 = 8 to 7 = 20 mm
2 to 4 = 8 to 10 = 9 -11mm
3 to 5 + x = 8 to 9
• Non-cleft side had 2/3 cupids bow, tubercle, white
roll , one philtral column & dimple
— release this tissue from abnormal high
attachment in columellar base
— rotation incision -> drop cupids bow, philtrum
& dimple into normal position
— Leaves triangular gap after rotation
— Maintain position by obtaining "filler" flap
from cleft side
— make horizontal subalar relaxing incision to
allow advancement into gap)
Procedure
Rotation advancement: Technique
• Make markings: from x to 5 to 3 (rotation flap)
• From point 8 to point 9
• Dissect skin off orbicularis on either side of cleft
• Bilateral gingival sulcus incisions made to cleft
margin
• Cheek soft tissue elevated off maxilla above
periosteum (caution w/ infraorbital nerve)
• Free orbicularis from attachments to columella/ alar
margin
• Incise along alar margin from 9 to 10
• Elevate c flap
Rotation advancement: Closure
• Close intraoral mucosa
• Close orbicularis serially
• Cleft alar base medialized with stitch to periosteum of nasal
spine
• C- flap rotated into columellar defect
• Close nasal floor
• Inset/sew flaps
• Nasal correction at same time
The main advantage of this technique is its flexibility and application.
The rotation advancement technique relies on a "cut as you go" strategy that allows continuous
modifications during the design and execution of the repair.
It does not adhere to strict geometrical principles or measurements.
Another advantage is that the suture line approximates a new philtral column.
The aesthetic philtral subunit is not violated, and this tends to create a scar that is more camouflaged.
Minimal tissue is discarded during the rotation advancement technique, and this tends to put less
tension on the closure.
Advantages
Furthermore, the rotation advancement technique allows easy access to the alar cartilages for primary
rhinoplasty to be performed at the time of lip repair. This early repair of the nasal deformity can be
successful in achieving a more symmetric nasal appearance and possibly avoiding the intermediate
rhinoplasty step for many of these children.
Disadvantages
• Lip often short due to under rotated lip
• Tension across repair of wide clefts
• May have constricted nostril on side of
repair
• Difficult to learn
• Must compromise lip height for lip width OR must
compromise on lip
width for lip height
Triangular flap
Tennison - Z plasty
■ Tennison (1952) -frustrated by
straight-line scar contraction in
blacks -Z-plasty technique which
preserves the cupid’s bow and
places it in normal position
- He inserted a wedge from the
lateral lip into the lower portion of
the medial lip, and achieved good
results - and called it ‘stencil
method’ - came to be known as the
Tennison triangular flap technique.
This repair technique is conceptually similar to
the rotation advancement repair.
The primary difference is that the rotation backcut in
the noncleft segment is performed
more inferiorly, closer to the vermilion
border.
Similarly the advancement segment
on the cleft side is designed to occur
inferiorly near the vermilion cutaneous
border.
Markings for unilateral cleft lip repair with the triangular flap technique.
• Marked reference points indicate the
• following:
1. Columellar base noncleft side
2. Columellar base cleft side
3. Alar base noncleft side
4. Alar base cleft side
5. Light scroll mark noncleft side
6. Light scroll mark cleft side. This also
becomes the medial base of the
equilateral triangle flap
7. Peak of Cupid’s bow noncleft side.
8. Low point of Cupid’s bow .
9. Apex of the equilateral triangle flap
10. Lateral base of equilateral triangle
flap .
11. Marks the length of back cut in
noncleft side.
Advantages
• First, it is readily used to close wide clefts without having to perform lip adhesion or presurgical
tissue manipulation.
• Second, the operation is done on strictly geometric methods of mathematical principles and
measurements, leaving not much room for errors in judgment when compared to the “cut as you
go” techniques.
• Therefore, many experts consider the triangular flap techniqueto be well suited for less
experienced surgeons.
• A third possible advantage of this technique is that the zigzag scar prevents scar contracture and
lip shortening leading to a vermilion notch that can be sometimes observed in the rotation
advancement technique.
• Minimal soft tissue dissection
• Easier to learn
• Preservation of cupids bow
Disadvantage
• Triangular repair technique is that the philtrum on the cleft side is
violated by the triangular flap.
• Another potential disadvantage is the difficulty in modifying the repair or performing
• secondary revision at a later stage due to the zigzag scars.
• Late vertical lengthening may occur
Skoog repair
a. Consists of two Zplasties.
b. Violates Cupid's bow
and the philtral dimple
Delaire’s functional lip closure
• Does not make use of flaps - accurate reconstruction of the 3 rings of
muscles of the Lip and nose
• Creates a highly symmetric nose and a functional lip: separates
cosmetics from function
• Delaire - 3 rings of muscles
• NASO-LABIAL/UPPER RING: Transverses nasi, levator labii superioris
alequi nasi, levator labii superioris, zygomaticus minor and the levator
anguli oris.
• BILABIAL/MIDDLE RING: Represents oral sphincter, consisting of the
upper and lower lip orbicularis oris muscle.
• LABIO-MENTAL/LOWER RING:Lower ring has an incomplete
circumference and consists of orbicularis inferior, triangularis labii
and quadrates labii inferioris.
• The integrity of the first ring is fundamental for sustaining and allowing normal
functioning of the other two. The cartilaginous nasal septum stimulates the
periosteum of anterior part of maxilla and ensures harmonius growth of the latter.
• Labial motility in addition to modeling directly the underlying dento alveolar structure
by means of median septum acts in positively influencing the growth of premaxilla.
• The lower ring acts by remodeling the dento alveolar complex and chin portion of the
mandible vertically and transversely.
Drawback:
• Straight-line scar and inability to achieve adequate lengthening of the lip, resulting in
a notching. But its proponents claim that this lack of lip symmetry will gradually
reduce by the effect of normal labial muscle function.
Mohler technique
• He extended the rotation into the base of the columella, made a back-cut, and
sutured it to the lateral flap. Muller in 1989 - concept of differential reconstruction of
the orbicularis oris muscle in unilateral cleft lip repair’
• The Mohler technique yielded a more symmetric result
Bilateral cleft lip Repair
• Achieve symmetrical result
• Reconstruction of vermillion & white roll
• Muscle repair - allow upper lip to function as a single unit
• Reconstruction of gingival-labial sulcus
• Form medial tubercle & vermillion-cutaneous ridge from lateral lip tissue (in cases
where the prolabium is deficient)
• Primary improvement of columellar length and nasal tip projection
• Proper philtral size and shape
a) Manchester repair
• For less severe bilateral deformities in which the prolabium has
adequate white roll and vermillion →these structures are preserved in
the repair (in contrast with Millard, Mulliken, etc. which discard this
tissue)
Advantages
• comparatively straightforward
• less tension on repair
• decreased chance of tight upper lip
Disadvantages
• whistle-notch deformity if used in cases with deficient prolabial
vermillion
Millard Technique
• Variations of this technique are widely used (mulliken, noordhoff)
• Repair brought lateral vermillion flaps under the prolabium, avoiding the whistle
notch deformity of some earlier repairs
• Restores orbicularis continuity deep to the prolabium
• Uses forked flaps derived from the prolabial parings to lengthen the columella; these
are banked in the alar bases until they are required
Advantages
• Good result even when prolabial white roll and vermillion are deficient
Disadvantages
• Forked flap columellar reconstruction yields an unusual appearance to the nose:
• Rectangular columella without a normal-appearing “waist”
• Sharp columella-labial angle
• Abnormally elongated nostrils
• Columellar over-elongation
• Downward drift of the columellar base
• Requires multiple procedures
• Can get tight upper lip
Mulliken Technique
• Many similarities with millard repair, but:
• Emphasized importance of how the repaired lip will grow over time
• Example: prolabium tends to grow too wide, ∴ the freshly reconstructed prolabium
should be conspicuously small
• (4-5mm) to compensate for growth
• Advocated lengthening the columella by repositioning the alar cartilages rather than
deriving tissue from prolabial forked
• Flaps (principle: “the columella is in the nose”)
Advantages
• Good result even when prolabial white roll and vermillion are deficient
• Good scar position - hidden in philtral columns
• Addressed nasal deformity with repositioning of cartilage elements rather than with
external flaps → less external scarring
• Avoids characteristic prolabial wideness of bilateral cleft lip repairs
Disadvantages
• Can get tight upper lip
Postoperative care
A. Orders
1. Arm restraints for 3 weeks to prevent disruption of repair.
2. Specialized nipple/bottle to decrease sucking effort when
bottle-feeding.
3. Breast-feeding is controversial; based on surgeon
preference.
B. Leave Steri-Strips in place over the incision for
reinforcement.
C. Follow up in 1 week for suture removal if nonabsorbable
skin sutures were used.
Secondary Deformities
• Its often better to take down the lip and redo it rather than do multiple small procedures.
(Fisher)
• Stress difference between mucosa and vermillion.
• (a) Vermillion
• (i) Vermillion Deficiency / Whistle notch
Synonym: whistle-notch deformity
• Deficiency of vermilion usually caused by inadequate repair or lateral vertical scar contraction
• Deficient tubercle
Treatment
• minor deformity:
• Z-plasty or V-Y advancement
• Submucosal dermal or alloderm graft
• Injectable fat
• severe deficiency:
• redo Sx (convert to Mulliken-type repair)
• deepithelialized, medially based submucosal flaps tunnelled across the midline
• cross-lip flap
Secondary Deformities
(ii) Stepped Vermillion / White roll deformity
Misalignment of the white-red lip border
Treatment:
Z-plasty to realign white roll
(b) Upper Lip
(I) short upper lip
Problem seen with millard repair (under-rotation)
Causes:
Improper repair
Scar contracture
Lack of orbicularis repair during primary surgery
Treatment:
Minor deformity:
Diamond-shaped excision lengthens lip & results in hidden vertical scar (pantographic
expansion – rose-thomson
• Effect) z-plasty lengthens lip but leaves more visible scar
major (>3mm discrepancy):
• redo lip repair
• orbicularis dysfunction:
• need to redo full-thickness repair and reapproximate muscle fibers
Secondary Deformities
(iii) Tight upper lip
Associated with bilateral repairs, wide clefts, lack of presurgical orthopedics
Treatment:
Cross-lip flap
Abbe flap
• (C) philtral defects
(I) lack of philtral columns or definition
Treatment (tend to give unsatisfactory results):
Rotated subcutaneous flaps
Auricular cartilage reconstruction
(Ii) wide philtrum
Almost always associated with bilateral cleft lip deformity
Causes: 1. Philtrum designed too wide;
2. Orbicularis ends approximated to sides of prolabium instead of to each other → forces
enlarge
Prolabium over time
Treatment:
Excision of philtral edges along previous scars, small enough to compensate for further
stretching
Secondary Deformities
• (d) Unilateral lack of Cupid’s bow
• Treatment:
• hemi-Gillies procedure: triangular skin excision just above mucocutaneous junction
(e) Deficient Buccal Sulcus
• Usually with bilateral, but can be with unilateral repairs
• Treatment:
• Z-plasty
• local buccal mucosal advancement flaps
• buccal mucosa graft
References
• Textbook of plastic surgery Grabb and Smith
• Fonseca volume 6: Cleft and craniofacial deformities
• Mc Carthy: PLASTIC SURGERY. Vol.4. Cleft lip & palate and Craniofacial Anomalies
• Peterson’s principles of oral and maxillofacial surgery volume 2
• Atlas of oral and maxillofacial Clinics of NA: 2009
• Cleft-lip - the historic perespetive S.Bhattacharya, V.Khanna,R. Kohli international
journal of plastic surgery oct 2009
• Thank you

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Cleft lip repair

  • 1. Cleft lip and its Management Dr. Abirami.K PG OMFS
  • 2. Introduction Cleft lip: • A congenital defect in lip continuity due to an embryological malformation
  • 3. History The 1st documented CL surgery was from china in 390 BC in a 18 years old soldier- wey young chi ■ Jehan yeperman - 1st to describe the unilateral and bilateral CL repair ■ Ambrose pare - 1st diagrammatic representation of CP repair in 14th century ■ Le monnier - 1st successful CP repair in paris (1776) ■ Asensio - Novel technique of CL repair in 1950 ■ Germanicus mirault - Originator of triangular flap - modified by tennison and randall ■ Ralph millard - classic- rotational advancement technique in mid 1950s
  • 4. Applied anatomy Anatomical LandmarksSKIN WHITE LIP • Philtral columns - vertical ridges forming the lateral boundaries of the philtrum • Philtral dimple - concave central region where no muscles insert • Cupid’s bow - the area of the white-red lip margin at the base of the philtrum • White roll - the prominent ridge between white-red lip formed by the anterior projection of the pars marginalis of the orbicularis RED LIP • Vermillion - squamous epithelium, dry due to a lack of mucous glands: contains glands of fordyce. • Superficial capillaries gives red colour • Red line (of noordhoff) - represents the abrupt transition from squamous epithelium to mucosa • Mucosa - wet due to mucous glands • Central tubercle
  • 5. Musculature of LipsGroup Origin Muscle/motor Origin Insertion Function Group I insert into modiolus Obicularis oris (buccal & mandibular branch CNVII) Modiolus modiolus, dermal attachments (philtral columns, “white roll” = pars marginalis) purses lips, oral Continence. buccinator (buccal br. VII) posterior alveolar process of maxilla, pterygomandibular raphe, body of mandible Modiolus press lips/cheeks against teeth levator anguli oris (buccal&zyg. br) canine fossa of maxilla below infraorbital foramen Modiolus elevates commissure depressor anguli oris (mand. br VII) oblique line on anterior mandible below canine and Modiolus depress/lateral shift commissure
  • 6. >T. quauratus lnbti superiors* M. uLcmrcis Jabit gup^rLovi* M. ori'Ictilaris nm* M. bttceiual*r M. Cr]&ijj'tLtf) cijt Nr. mcnralis >r. >|«ailrjilU5 L:i I id L deifevtorfe ■'!- iiit-isivttfl ]n.l»l [ Infoi-loris
  • 7. Group Muscle/motor Origin Insertion Function Zygomaticus major (Zyg&buccal br.) Zygoma just lateral to Zygomaticotemporal suture Line, deep to obicularis Modiolus Elevate/lateral Shift Commissure Risorius (buccal br) Parotid fascia Modiolus Lateral shift of Commissure Group II insert into upper lip Levator labii Superioris (buccal) Infraorbital rim above Upper lip dermis And obicularis oris Elevates upper Lip Levator labii Superioris aleque Nasi (buccal br.) Infraorbital foramen Frontal process of maxilla Lower lateral Cartilage, upper lip Dilates nostril, Zygomaticus minor (Buccal br.) Zygoma, deep to orbicularis Oculi and lateral to Zygomaticomaxillary Upper lip Elevates upper Lip Nasolabial fold
  • 8. Group Muscle/motor Origin Insertion Function Group III Insert Into Lower lip Depressor labii Inferioris (mand. Br) Anterolateral mandible and Medial to insertion of Depressor anguli oris Lower lip dermis And obicularis oris Lower lip Depressor and Lateral shift Mentalis (mand. Br) Anterior midline mandible Dermis of chin Elevate lower Lip Platysma (cervical Br. Vii) Deltopectoral fascia Inferior border of Mandible Lip depressor
  • 9. Neurovasular Innervation: • Sensory • Upper lip: V2 • Lower lip: V3 Blood supply: • superior and inferior labial arteries • superior/inferior labial veins: drain into facial vein Lymphatic drainage: • Upper lip and lateral portions of lower lip: submandibular basin • Central lower lip: submental basin. DORSAL NASAL INFRA ORBITAL MAXILLARY POST SUP ALVEOLAR BUCCAL SUPERIOR LABIAL INFERIOR LABIAL INF ALVEOLAR terminal br FACIAL BLOOD SUPPLY LIPS
  • 10. Embryology • Development of the lip and palate begins around the 4th week of embryological development • Completed by the end of the 12th week • By the end of the 4th week - 5 facial prominences have formed • frontonasal process • paired maxillary processes • paired mandibular processes
  • 11. Embryology • During the 5th week - Nasal placodes invaginate to form the nasal pits • Lateral and medial nasal prominences Frontonasal prominence Maxillary processes Oral cavity Mandibular processes — Medial nasal processes - Lateral nasal processes — Nasal pits
  • 12. Embryology • By the end of the 6th week - Paired maxillary processes have grown medially and pushed the paired medial nasal prominences together - Fusion of the paired medial nasal prominences form: • Philtrum • Middle upper lip • Nasal tip • Columella - Fusion of the paired maxillary prominences with the paired medial nasal prominences forms the complete upper lip (maxillary prominences form lateral lip) - The lateral nasal prominences form the bilateral nasal ala Oral cavity Frontonasal prominence Maxillary processes Mandibular processes — Medial nasal processes — Lateral nasal processes — Nasal pits Alae
  • 13. Cleft Lip and Palate Formation Maxillary processes Oral cavity • Fusion - Failure of fusion of the maxillary and medial nasal prominences unilaterally or bilaterally result in unilateral or bilateral cleft lip with/without primary palate - Failure of fusion of the palatal shelves result in clefts of the secondary palate Frontonasal prominence Mandibular processes Medial nasal processes Lateral nasal processes r} Nasal pits cleft lip
  • 14. Etiology ■ The exact cause of clefting is unknown ■ Multifactorial Chemical Exposures Radiation Maternal Hypoxia Teratogenic Drugs Nutritional Deficiencies Physical Obstruction Genetic Influences. Genetic (non- syndromal) ■ Genetic basis is significant, but not predictable ■ Lack of potential for mesodermal proliferation across the fusion lines after the component parts are in contact. ■ Error in the transitional shift of the embryonic blood supply. ■ Chromosomal abnormalities causing multiple congential malformations including cleft
  • 15. Etiology Environmental Intensity, duration and the time of action - more important than etiological factor 1. Infections (rubella, toxoplasmosis) 2. Growth hormone deficiency 3. Drugs: Steroids, Diazepam, Aminopterin, Anticonvulsants (incidence10X), Aspirin, 4. Alcohol: fetal alcohol syndrome 5. Smokers (2X incidence) 6. Vitamin B and Folate deficiency 7. Radiation energy 8. Hypoxia and amniotic fluid alteration • Some of these genes include the MSX, LHX, goosecoid, and DLX genes.
  • 16. Group Syndrome Cleft sporadic Klippel-Feil syndrome Shprintzen Syndrome CP CP Multifactorial Pierre Robin Sequence CP major mutant genes Treacher Collins Van der Woude Syndrome Waardenburg syndrome Stickler Syndrome Ectodermal Dysplasia syndromes CP CL(P), CP CL(P) CP CP Chromosomal aberrations Down’s Syndrome Patau Syndrome CL(P), CP Teratogenic Dilantin Fetal Alcohol syndrome Accutane dysmorphic syndrome Nutritional deficiency
  • 17. Epidemiology ■ Cleft lip and palate occurs in about 1 to 2 per 1000 births in the developed world. Rates for cleft lip with or without cleft palate and cleft palate alone varies within different ethnic groups. ■ The highest prevalence rates for (CL ± P) are reported for Native Americans and Asians . Africans have the lowest prevalence rates. ■ Native Americans: 3.74/1000 ■ Japanese 0.82/1000 to 3.36/1000 ■ Chinese 1.45/1000 to 4.04/1000 ■ Caucasians 1.43/1000 to 1.86/1000 ■ Latin Americans : 1.04/1000 ■ Africans: 0.18/1000 to 1.67/1000 ■ Watkins, SE; Meyer, RE; Strauss, RP; Aylsworth, AS (April 2014). "Classification, epidemiology, and genetics of orofacial clefts.". Clinics in plastic surgery. 41 (2): 149-63 ■ CLP: M:F = 2:1 ■ Isolated palates: F:M = 2:1 ■ Site of clefting : Left: Right: Bilateral 6:3:1
  • 18. CRANIO-FACIAL CLEFT Tessier(1973):Orbitocentric classification of facial clefts. • Facial clefts are numbered 0-7 • Cranial clefts from 8-14. • Orbit - reference landmark • Cleft lip is part of clefts 2 and 3.
  • 19. CLASSIFICATION 1. DAVIS AND RITCHIE CLASSIFICATION: The following classification was proposed by Davis and Ritchie in 1922. This system broadly categorized the clefts into three groups according to position of cleft in relation to alveolar process. Group I – Pre alveolar clefts: • Unilateral cleft lip • Bilateral cleft lip • Median cleft lip Group II - Post alveolar clefts: • Cleft hard palate alone • Cleft soft palate alone • Cleft soft palate and hard palate • Sub mucous cleft Group III-Alveolar clefts: • Unilateral alveolar cleft • Bilateral alveolar cleft • Median alveolar cleft
  • 20. Classification 2. VEAU CLASSIFICATION: • Veau proposed the following classification in 1931. • Group I (A) - Defects of the soft palate only • Group II (B) - Defects involving the hard palate and soft palate extending not further than the incisive foramen, thus involving the secondary palate alone. • Group III (C) – Complete unilateral cleft, extending from the soft palate to the alveolus, usually involving the lip • Group IV (D) - Complete bilateral clefts, resembles Group III but is bilateral. When cleft is bilateral, pre-maxilla is suspended from the nasal septum.
  • 21. Classification LAHSHAL system of classification: • Kreins O proposed LAHSHAL system for classification of cleft lip and palate patients which was modified on the recommendation of Royal College of Surgeons Britain in 2005 by omitting one “H” from the acronym “LAHSHAL”. • LAHSAL system is a diagrammatic classification of cleft lip and palate. • According to this classification, mouth is divided into six parts. • Right lip • Right alveolus • Hard palate • Soft palate (LAHSAL) • Left alveolus • Left lip
  • 22. Kernahan striped Y classification In 1971 Areas 1 and 4 – Lip Areas 2 and 5 – Alveolus Areas 3 and 6 – Primary palate Areas 7 and 8 - Hard palate Area 9 - Soft palate The small circle represents incisive papilla.
  • 23. MODIFIED STRIPED-Y ELSAHY CLASSIFICATION • Triangle 1 & 5- the fusion line between the maxillary prominence and medial nasal prominence at nostril floor • Square 2&6 - the fusion line between the maxillary prominence and medial nasal prominence at upper lip level • Square 3 & 7 - the alveolus • Square 4 and 8- primary and secondary hard palate • Squares 9 and 10 - the fusion line between palatine shelves of hard palate and lies posterior to the incisive foramen. • Square 11 - the soft palate. • Circle 12 - the posterior pharyngeal wall. • Circle 13 represents the pre-maxilla.
  • 24. FRIEDMAN’S CLASSIFICATION (1991) STEP I: This step includes the depiction of the deformity of the nasal arches (triangles 1 and 6)and nasal floors (triangles 2 and 7). In each triangular space, defect is recorded in the following manner. 0 = No involvement 1 = Cleft microform (e.g; notching) 2 = Mild deformity (i.e; some actual tissue deficiency) 3 = Moderate deformity 4 = Severe deformity X = Not rated STEP II: It includes the depiction of the amount of prolabium or pre-maxillary protrusion on one or both sides (semi circles 14 and 15). 0 = No protrusion 1 = Mild protrusion (<45p ) 2 = Moderate protrusion (>45p ,<90p ) 3 = Severe protrusion(>90p ) X = Not rated
  • 25. FRIEDMAN’S CLASSIFICATION (1991)• STEP III: • This step depicts the upper lip deformity. • 0 = No involvement • 1 = Cleft microform • 1a = Congenital scar in usual cleft position • (subcutaneous cleft) • 1b = Notch in the vermillion border • 2 = 1/3 cleft lip (cleft in vertical dimension) • 3 = 2/3cleft lip (cleft in vertical dimension) • 4 = Complete cleft lip • X = Not rated • STEP IV: • It shows the defect in the alveolus. • 0 = No involvement • 1 = Cleft microform1a = Submucous cleft • 1b = Notch • 2 = Partial cleft • 3 = Complete cleft • 3a = Absence of maxillary arch collapse • 3b = Presence of maxillary arch collapse • X = Not rated STEP V: It represents the pre-incisive foramena trigones. 0 = No involvement 1= Partial cleft 2= Complete cleft X= Not rated. STEP VI: It describes the anatomy of the hard palate 0 = No involvement 1 = Posterior 1/3 cleft 2 = Posterior 2/3 cleft 3 = Complete cleft X = Not rated.
  • 26. FRIEDMAN’S CLASSIFICATION (1991) STEP VII: • It indicates a defect in the soft palate or velum including uvula. • 0 = No involvement • 1= Cleft microform of uvula • 1a = Hypoplasia of musculus uvulae • 1b = Septate uvula • 1c = Bifid uvula • 2 = Submucous cleft of velum • 2a = Occult • 2b = Overt • 3= Posterior 1/3of velum • 4= Posterior 2/3 of velum • 5 = Complete cleft of velum • X = Not rated. STEP VIII: It represents velopharyngeal valve dysfunction 0 = No impairment 1 = Mild impairment 2 = Moderate impairment 3 = Severe impairment X = Not rated
  • 27. DEFECTS IN CLEFT LIP AND PALATE PATIENTS Deformities: 1. Dental Problems: Number, Shape, Eruption, Mineralization. 2. Skeletal Problems: Max. deficiency, Mand.prognathism, Class III malcclusion. 3. Nasal problems: Alae flared, Columella pulled to non cleft side. 4. Feeding Problems: Nasal Regurgitation, Weak sucking, Weak swallowing reflux.
  • 28. DEFECTS IN CLEFT LIP AND PALATE PATIENTS 4. Ear Problems: Recurrent Middle Ear infection, Possible Deafness 5. Speech Problems: Retardation of consonants, Hyper nasality, Articulation Defects, Hearing problem 6. Associated Anomalies: Congenital heart defects, Mental retardation
  • 29. CLINICAL FEATURES UNILATERAL CLEFT LIP (a) Microform / Forme fruste • small notch within the borders of the vermillion • band of fibrous tissue running from edge of red lip to nostril floor • deformity of ala on side of notch or band (b) Partial Cleft Lip • Up to 2/3 of lip • Vermillion involved • Orbicularis may be partially divided • Nasal deformity more marked (displaced alar base, asymmetric tip, flat ala) • Bony hypoplasia more evident (c) Simonart’s Band • a narrow bridge of tissue, in an otherwise complete cleft lip crossing the cleft • lip to lip or lateral lip to medial alveolar mucosa • significance: functional - potentially reduces the deformity (d) Complete Cleft Lip • ± Simonart’s band
  • 30. CLINICAL FEATURES Structure Cleft side Non cleft side Maxilla 1.Lateral segment retropositioned 2.Piriform margin hypoplastic & retropositioned Premaxilla outwardly rotated and projecting Nasal septum Mid-septal bulge into cleft side Inferior septum dislocated out of vomer groove Columella Shortened columella, with tip deviated to cleft side Base deviated to non-cleft side Lip Philtrum short Shortened philtral height Preserved 2/3 of Cupid’s bow (one philtral column and dimple hollow) Lateral lip White roll deficient as it progresses into cleft Vermillion decreased on medial side of cleft, normal to increased laterally Orbicularis oris - deep portion interrupted → no sphincteric function - superficial portions parallel cleft margins to insert into columella & ala - no decussation to form the philtral ridge Incomplete clefts -muscle does not cross cleft unless bridge is ≥ 1/3rd the height of the lip Heaped up to form visible bulge of lateral lip element Hypoplastic muscle between philtral midline and cleft Muscle may insert into columellar Base
  • 31. Clinical FeaturesSkeleton • Premaxilla projecting ahead of lateral segments • Lateral segments retropositioned and medially collapsed • Maxilla hypoplastic at piriform margins and alar bases Nose • Septum midline • Alae dislocated inferolaterally, with recessed bases • Alae spread wide, with slump along lateral crura • Short columella • Flat, wide, tip Prolabium • Variable in size; can be hypoplastic • No muscle (present in incomplete cases); skin appendages may be deficient / absent • Absent white roll, cupid’s bow, philtral columns, central dimple & tubercle • Deficient gingival buccal sulcus • Hypoplastic vermillion Lateral Lip • Vertically shortened • Orbicularis heaped up to form a visible bulge of the lateral lip element • Orbicularis abnormally inserted into alar bases
  • 32. Goals of Treatment • Steffenson (1953) - five criteria for a satisfactory lip repair: ■ 1. Accurate skin, muscle and mucous membrane union with adequate lip lengthening, ■ 2. Symmetrical nostril floor, ■ 3. Symmetrical vermilion border, ■ 4. Slight eversion of the lip and ■ 5. A minimal of scar, which by contraction will not interfere with the accomplishments of the other, stated requirements Two additional criteria were added later by Musgrave (1971) ■ 1. Preservation of the cupid’s bow, ■ 2. Production of symmetrical nostrils.
  • 33. Timing Procedure Age 1st appointment (± prenatal consult) in utero - 3 wks Presurgical orthopedics 2 - 4 wks 1° lip & nasal repair 3 - 6 mos Palate repair & myringotomy tubes 9 - 14 mos Speech assessment & Tx 6 wk post-palate+ ± VPI surgery 5-7 yrs+ Additional orthodontia Pre/post ABG Alveolar bone grafting (ABG) 9-12 yrs ± 2° nasal repair 5-6 yrs ± Orthognathic surgery 16+ F, 18+ M ± Adult rhinoplasty, genioplasty 18 yrs + Cleft Lip: Timing of Procedures for the Cleft Patient:
  • 34. PRESURGICAL ORTHODONTIA Repositioning of maxillary alveolar arch using an appliance • Aim to decrease deformity before performing definitive lip surgery • Used in conjunction with gingivoperiosteoplasty to achieve closure of alveolar cleft to reduce the need for bone grafting Techniques • Active/fixed device - latham appliance fixed to palatal segments with pins • Passive/loose appliance - customized appliance fit to roof of month; adjusted every few weeks to guide growth Indications • Wide clefts • Misaligned arches Timing: age 2-5 weeks until cleft lip repair Advantages • Better correction of lip and nasal deformity • Alignment of alveolar segments • Possibility of 1 repair of alveolar cleft (gingivoperiosteoplasty) Disadvantages • Compliance • Added cost • Delay in lip surgery • Concern regarding long-term growth imbalance
  • 35. Lip Taping • Effective in reducing the width of the cleft in a nonsurgical manner • Strip of hypoallergenic tape is placed with tension across the cleft and secured to the patient’s cheek • Molds bony tissues by applying pressure to protruding portions of the maxilla • Must be worn 24 hours per day Indication: • Wide cleft lip or premaxilla protrusion Advantage: • To narrow the cleft and mold the premaxilla before proceeding with surgery
  • 36. LIP ADHESION • Suturing the medial and lateral lip segments together prior to definitive lip repair Indications: • Wide complete clefts ± poorly aligned maxillary segments Timing: 3-5wks of age Advantages • May facilitate gingivoperiosteoplasty • Reduces width of cleft and molds maxillae • Decreases lip tension at time of definitive closure Disadvantages • Additional procedure/OR/anaesthetic • Increased scarring at time of definitive repair • Final cosmetic outcome may be worsened or not improved • Delays definitive lip repair
  • 37. GINGIVOPERIOSTEOPLASTY • Alveolar gap is bridged with mucoperiosteal flaps prior to lip repair • Evidence that some bone fills in the defect Timing: After alveolar segments realigned by lip adhesion or presurgical orthodontia Advantages • May allow for normal tooth eruption • May obviate need for bone graft in some patients Disadvantages • Adverse effect on facial & palatal growth (controversial) • Bone formed across gap may be insufficient for osteointegrated implant or tooth eruption
  • 38. Definitive lip surgery Timing Infant should by healthy and of acceptable weight before surgery to decrease Morbidity risk • Generally around three months of age, when: • 10 weeks of age • 10 lbs • 10 hemoglobin level Earlier repairs: Neonatal • Repair undertaken in first few weeks of life Advantages • Less social concern & stigma for parents Disadvantages • Higher anaesthetic risk • Delicate, small tissues → demanding surgery • ? Any difference in results Guidelines for safe neonatal surgery • Babies should be gestationally mature and healthy • No opioid analgesia perioperatively • Experienced nurses • Apnea monitors
  • 39. General principles • Tissues must be released enough to obtain a tension-free closure • Accurate marking is essential • Each cleft should be repaired with a customized approach • Muscle should be released from skin and mucosa both medially and laterally, to achieve functional cleft lip • Vermilion flap from lateral side to augment the vermilion height on the deficient medial side • Primary correction of cleft nose: varies in complexity due to fears of growth disturbance and scarring for later repairs • Alveolar cleft: mucosa may be closed, but grafting is done at time of mixed dentition • Augmentation of medial lip with lateral lip tissue
  • 40. TECHNIQUES OF REPAIR • U/L CLEFT LIP • Millard’s rotational advancement • Modifications of millard’s technique: - Millard II - Skoog’s Technique - Mohler’s Technique • Tennison-Randall Triangular flap • Delaire’s Functional lip closure B/L CLEFT LIP • Straight line closure • Columella lengthening • Millard repair • Tennison-Randall triangular flap • Other techniques: - Bauer Method - Manchester method - Skoog method - Wynn method - Barsky technique - Modified Veau II
  • 41. Basic surgical preparation • GA using oral RAE (Ring - Adair- Elwyn) tube taped to midline of lower lip - not to produce any lateral distortion ■ The infant is placed in folded towels with the neck slightly extended. ■ Points - 30 gauge needle - Bonney’s blue (Brilliant green 1/2 g, Crystal violet 1/2 g, Alcohol 96% 46ml, Water for injection 53ml) or methylene blue. ■ After markings - 0.5% lignocaine with 1:2,00,000 epinephrine is injected into the lip tissue. It also helps to grip lip firmly between thumb and index finger to avoid bleeding while incising. Wait for 5 minutes after infiltration. GA using oral RAE (Ring - Adair- Elwyn) tube taped to midline of lower lip - not to produce any lateral distortion
  • 42. A. Straight-line repair 1. Historically, the first cleft lip repairs relied on freshening the edges of the cleft and suturing them together. These have been largely replaced by various Z-plasty-based techniques. 2. Rose-Thompson repair: a. Modified straight-line repair that can be used for minor clefts with lip length nearly equal on both sides of cleft (e.g., forme fruste). b. Fusiform excision with straight-line closure. Indications: Still used for mild forms of cleft lip Lengthening effect achieved by opening of angle between white-lip and red-lip. Advantages Straightforward Disadvantages Minimal lengthening is obtained Destroys important landmarks
  • 43. Quadrangular flap • Proposed by LeMesurier and Hagedorn. • Rectangular flap from lateral side of cleft is inset into a relaxing incision on the medial side • Cupid's bow is derived from the lateral lip. • 90-degree Z-plasty. • Violates Cupid's bow and philtral dimple. • Has a tendency to produce a long lip. Advantages • Good results could be achieved in experienced hands Disadvantages • Cupid’s bow deformities - flat, notched, etc. • Increased length on cleft side • Large amount of tissue sacrificed • Wide nasal floor • Redundant red lip
  • 44. Milliard Rotation-Advancement • The rotation advancement repair of the unilateral cleft lip deformity as described by Millard • Introduced in 1957 • Most widely used procedure for unilateral cleft lip repair • 46% of North American surgeons, 38% modified versions
  • 45. Millard Rotation-Advancement Millard DR. Complete Unilateral Clefts of the Lip. Plas Recon Surg 1960 25(6), 595-605.
  • 46. Markings/Design ■ 1. center/low point cupids bow * 2. peak of bow on NCS * 3, Peak of bow on medial side of cleft * 4, Alar base, NCS * 5. Columellar base, NCS * 6, Commissure, NCS * 7. Commissure, deft side * 8, Peak cupids bow lateral side of cleft * 9, Superior extent of advancement flap * 10, Alar base, cleft side * Point x: Back-cut point 1 to 2 = 1 to 3 = 2-4 mm 2 to 6 = 8 to 7 = 20 mm 2 to 4 = 8 to 10 = 9 -11mm 3 to 5 + x = 8 to 9
  • 47. • Non-cleft side had 2/3 cupids bow, tubercle, white roll , one philtral column & dimple — release this tissue from abnormal high attachment in columellar base — rotation incision -> drop cupids bow, philtrum & dimple into normal position — Leaves triangular gap after rotation — Maintain position by obtaining "filler" flap from cleft side — make horizontal subalar relaxing incision to allow advancement into gap) Procedure
  • 48. Rotation advancement: Technique • Make markings: from x to 5 to 3 (rotation flap) • From point 8 to point 9 • Dissect skin off orbicularis on either side of cleft • Bilateral gingival sulcus incisions made to cleft margin • Cheek soft tissue elevated off maxilla above periosteum (caution w/ infraorbital nerve) • Free orbicularis from attachments to columella/ alar margin • Incise along alar margin from 9 to 10 • Elevate c flap
  • 49. Rotation advancement: Closure • Close intraoral mucosa • Close orbicularis serially • Cleft alar base medialized with stitch to periosteum of nasal spine • C- flap rotated into columellar defect • Close nasal floor • Inset/sew flaps • Nasal correction at same time
  • 50. The main advantage of this technique is its flexibility and application. The rotation advancement technique relies on a "cut as you go" strategy that allows continuous modifications during the design and execution of the repair. It does not adhere to strict geometrical principles or measurements. Another advantage is that the suture line approximates a new philtral column. The aesthetic philtral subunit is not violated, and this tends to create a scar that is more camouflaged. Minimal tissue is discarded during the rotation advancement technique, and this tends to put less tension on the closure. Advantages Furthermore, the rotation advancement technique allows easy access to the alar cartilages for primary rhinoplasty to be performed at the time of lip repair. This early repair of the nasal deformity can be successful in achieving a more symmetric nasal appearance and possibly avoiding the intermediate rhinoplasty step for many of these children.
  • 51. Disadvantages • Lip often short due to under rotated lip • Tension across repair of wide clefts • May have constricted nostril on side of repair • Difficult to learn • Must compromise lip height for lip width OR must compromise on lip width for lip height
  • 52. Triangular flap Tennison - Z plasty ■ Tennison (1952) -frustrated by straight-line scar contraction in blacks -Z-plasty technique which preserves the cupid’s bow and places it in normal position - He inserted a wedge from the lateral lip into the lower portion of the medial lip, and achieved good results - and called it ‘stencil method’ - came to be known as the Tennison triangular flap technique.
  • 53. This repair technique is conceptually similar to the rotation advancement repair. The primary difference is that the rotation backcut in the noncleft segment is performed more inferiorly, closer to the vermilion border. Similarly the advancement segment on the cleft side is designed to occur inferiorly near the vermilion cutaneous border.
  • 54. Markings for unilateral cleft lip repair with the triangular flap technique. • Marked reference points indicate the • following: 1. Columellar base noncleft side 2. Columellar base cleft side 3. Alar base noncleft side 4. Alar base cleft side 5. Light scroll mark noncleft side 6. Light scroll mark cleft side. This also becomes the medial base of the equilateral triangle flap 7. Peak of Cupid’s bow noncleft side. 8. Low point of Cupid’s bow . 9. Apex of the equilateral triangle flap 10. Lateral base of equilateral triangle flap . 11. Marks the length of back cut in noncleft side.
  • 55. Advantages • First, it is readily used to close wide clefts without having to perform lip adhesion or presurgical tissue manipulation. • Second, the operation is done on strictly geometric methods of mathematical principles and measurements, leaving not much room for errors in judgment when compared to the “cut as you go” techniques. • Therefore, many experts consider the triangular flap techniqueto be well suited for less experienced surgeons. • A third possible advantage of this technique is that the zigzag scar prevents scar contracture and lip shortening leading to a vermilion notch that can be sometimes observed in the rotation advancement technique. • Minimal soft tissue dissection • Easier to learn • Preservation of cupids bow
  • 56. Disadvantage • Triangular repair technique is that the philtrum on the cleft side is violated by the triangular flap. • Another potential disadvantage is the difficulty in modifying the repair or performing • secondary revision at a later stage due to the zigzag scars. • Late vertical lengthening may occur Skoog repair a. Consists of two Zplasties. b. Violates Cupid's bow and the philtral dimple
  • 57. Delaire’s functional lip closure • Does not make use of flaps - accurate reconstruction of the 3 rings of muscles of the Lip and nose • Creates a highly symmetric nose and a functional lip: separates cosmetics from function • Delaire - 3 rings of muscles • NASO-LABIAL/UPPER RING: Transverses nasi, levator labii superioris alequi nasi, levator labii superioris, zygomaticus minor and the levator anguli oris. • BILABIAL/MIDDLE RING: Represents oral sphincter, consisting of the upper and lower lip orbicularis oris muscle. • LABIO-MENTAL/LOWER RING:Lower ring has an incomplete circumference and consists of orbicularis inferior, triangularis labii and quadrates labii inferioris.
  • 58. • The integrity of the first ring is fundamental for sustaining and allowing normal functioning of the other two. The cartilaginous nasal septum stimulates the periosteum of anterior part of maxilla and ensures harmonius growth of the latter. • Labial motility in addition to modeling directly the underlying dento alveolar structure by means of median septum acts in positively influencing the growth of premaxilla. • The lower ring acts by remodeling the dento alveolar complex and chin portion of the mandible vertically and transversely. Drawback: • Straight-line scar and inability to achieve adequate lengthening of the lip, resulting in a notching. But its proponents claim that this lack of lip symmetry will gradually reduce by the effect of normal labial muscle function.
  • 59. Mohler technique • He extended the rotation into the base of the columella, made a back-cut, and sutured it to the lateral flap. Muller in 1989 - concept of differential reconstruction of the orbicularis oris muscle in unilateral cleft lip repair’ • The Mohler technique yielded a more symmetric result
  • 60. Bilateral cleft lip Repair • Achieve symmetrical result • Reconstruction of vermillion & white roll • Muscle repair - allow upper lip to function as a single unit • Reconstruction of gingival-labial sulcus • Form medial tubercle & vermillion-cutaneous ridge from lateral lip tissue (in cases where the prolabium is deficient) • Primary improvement of columellar length and nasal tip projection • Proper philtral size and shape
  • 61. a) Manchester repair • For less severe bilateral deformities in which the prolabium has adequate white roll and vermillion →these structures are preserved in the repair (in contrast with Millard, Mulliken, etc. which discard this tissue) Advantages • comparatively straightforward • less tension on repair • decreased chance of tight upper lip Disadvantages • whistle-notch deformity if used in cases with deficient prolabial vermillion
  • 62. Millard Technique • Variations of this technique are widely used (mulliken, noordhoff) • Repair brought lateral vermillion flaps under the prolabium, avoiding the whistle notch deformity of some earlier repairs • Restores orbicularis continuity deep to the prolabium • Uses forked flaps derived from the prolabial parings to lengthen the columella; these are banked in the alar bases until they are required Advantages • Good result even when prolabial white roll and vermillion are deficient Disadvantages • Forked flap columellar reconstruction yields an unusual appearance to the nose: • Rectangular columella without a normal-appearing “waist” • Sharp columella-labial angle • Abnormally elongated nostrils • Columellar over-elongation • Downward drift of the columellar base • Requires multiple procedures • Can get tight upper lip
  • 63.
  • 64. Mulliken Technique • Many similarities with millard repair, but: • Emphasized importance of how the repaired lip will grow over time • Example: prolabium tends to grow too wide, ∴ the freshly reconstructed prolabium should be conspicuously small • (4-5mm) to compensate for growth • Advocated lengthening the columella by repositioning the alar cartilages rather than deriving tissue from prolabial forked • Flaps (principle: “the columella is in the nose”) Advantages • Good result even when prolabial white roll and vermillion are deficient • Good scar position - hidden in philtral columns • Addressed nasal deformity with repositioning of cartilage elements rather than with external flaps → less external scarring • Avoids characteristic prolabial wideness of bilateral cleft lip repairs Disadvantages • Can get tight upper lip
  • 65. Postoperative care A. Orders 1. Arm restraints for 3 weeks to prevent disruption of repair. 2. Specialized nipple/bottle to decrease sucking effort when bottle-feeding. 3. Breast-feeding is controversial; based on surgeon preference. B. Leave Steri-Strips in place over the incision for reinforcement. C. Follow up in 1 week for suture removal if nonabsorbable skin sutures were used.
  • 66. Secondary Deformities • Its often better to take down the lip and redo it rather than do multiple small procedures. (Fisher) • Stress difference between mucosa and vermillion. • (a) Vermillion • (i) Vermillion Deficiency / Whistle notch Synonym: whistle-notch deformity • Deficiency of vermilion usually caused by inadequate repair or lateral vertical scar contraction • Deficient tubercle Treatment • minor deformity: • Z-plasty or V-Y advancement • Submucosal dermal or alloderm graft • Injectable fat • severe deficiency: • redo Sx (convert to Mulliken-type repair) • deepithelialized, medially based submucosal flaps tunnelled across the midline • cross-lip flap
  • 67. Secondary Deformities (ii) Stepped Vermillion / White roll deformity Misalignment of the white-red lip border Treatment: Z-plasty to realign white roll (b) Upper Lip (I) short upper lip Problem seen with millard repair (under-rotation) Causes: Improper repair Scar contracture Lack of orbicularis repair during primary surgery Treatment: Minor deformity: Diamond-shaped excision lengthens lip & results in hidden vertical scar (pantographic expansion – rose-thomson • Effect) z-plasty lengthens lip but leaves more visible scar major (>3mm discrepancy): • redo lip repair • orbicularis dysfunction: • need to redo full-thickness repair and reapproximate muscle fibers
  • 68. Secondary Deformities (iii) Tight upper lip Associated with bilateral repairs, wide clefts, lack of presurgical orthopedics Treatment: Cross-lip flap Abbe flap • (C) philtral defects (I) lack of philtral columns or definition Treatment (tend to give unsatisfactory results): Rotated subcutaneous flaps Auricular cartilage reconstruction (Ii) wide philtrum Almost always associated with bilateral cleft lip deformity Causes: 1. Philtrum designed too wide; 2. Orbicularis ends approximated to sides of prolabium instead of to each other → forces enlarge Prolabium over time Treatment: Excision of philtral edges along previous scars, small enough to compensate for further stretching
  • 69. Secondary Deformities • (d) Unilateral lack of Cupid’s bow • Treatment: • hemi-Gillies procedure: triangular skin excision just above mucocutaneous junction (e) Deficient Buccal Sulcus • Usually with bilateral, but can be with unilateral repairs • Treatment: • Z-plasty • local buccal mucosal advancement flaps • buccal mucosa graft
  • 70. References • Textbook of plastic surgery Grabb and Smith • Fonseca volume 6: Cleft and craniofacial deformities • Mc Carthy: PLASTIC SURGERY. Vol.4. Cleft lip & palate and Craniofacial Anomalies • Peterson’s principles of oral and maxillofacial surgery volume 2 • Atlas of oral and maxillofacial Clinics of NA: 2009 • Cleft-lip - the historic perespetive S.Bhattacharya, V.Khanna,R. Kohli international journal of plastic surgery oct 2009