dr.nikil jain

CLEFT LIP

Cleft: means fissure.

FACIAL CLEFT: a fissure resulting from incomplete merging or fusion of embryonic processes normally uniting in the formation of the face

EPIDEMOLOGY

INDIAN EPIDEMIOLOGICAL DATA STATES

FREQUENCY IN THE RANGE OF 1:700 TO 1:1000 .

FOGH-ANDERSON (1942) FROM DENMARK , WOOLF AND BROADBENT (1963) FROM US AND WILSON (1972) FROM BRITAIN ALL REPORTED THE OVERALL FREQUENCY OF CLEFT LIP AND PALATE AS 1.47 PER 1000 LIVE BIRTHS

There is an excess of males with cleft lip/palate 2 : 1

Female excess has been reported in isolated cleft palate cases.

45% - CLEFT LIP AND PALATE

25% - CLEFT LIP

30% - CLEFT PALATE

FOGH – ANDERSON ET AL

IN ALMOST ALL THE

SERIES, THERE IS A

LEFT SIDED

PREPONDERANCE OF

CLEFT LIP

68 % OF UNILATERAL

CLEFT LIP ARE

ASSOCIATED WITH

CLEFT PALATE -

FRASER ( 1970 )

RACIAL INFLUENCES

Aylsworth (1985)

ORIENTALS

BLACKS

CAUCASIANS

EUROPIANS

PARENTAL AGE

THERE IS SOME EVIDENCE THAT THE RISK

OF PRODUCING AN AFFECTED CHILD IS

DECREASED IN YOUNGER PATIENTS.

FRASER AND CALNAN (1951) CONCLUDED

THAT THE MOST IMPORTANT FACTOR

WAS ELEVATED PARENTAL AGE AND NOT

MATERNAL AGE ALONE.

EMBRYOLOGY AND CLEFT PATHOGENESIS

Neural crest cells plays an important role in facial morphogenisis

Just before neural fold fuse to form neural tube neuro ectodermal cells adjacent to neural plate migrate to facial region

These cells form the skeletal and connective tissue of the face like bone, cartilage, fibrous tissue, dental tissue (except enamel)

Devolopment of face is controlled by 2 organic centres

1. Procencephalic organizer – induces the formation of upper 3rd of the face

2. Rombencephalic organizer – forms the middle and caudal 3rd of face

4th week – stomedium (primitive oral cavity) is covered by maxillary, mandibular and fronto nasal prominence

5TH WEEK – Nasal placods arise as thicknening of either side of the frontal prominence

Horse shoe shaped ridge consisting of medial and nasal swelling sorrounds each nasal placodes

As mesenchyme elevates the ridge the nasal pits are formed

6TH AND 7TH WEEK

TWO MANDIBULAR PROMINENCE EMERGE IN THE MID LINE

THE MAXILLARY PROMINENCE FUSE WITH MEDIAL NASAL PROMINENCE

MEDIAL NASAL PROMINENCE MERGE WITH EACH OTHER FORMING 3 PARTS

1. PHILTRUM

2.DENTAL ARCH COMPONENT FOR MAXILLARY INCISORS

3. PALATAL PART UP TO INCISIVE FORAMEN ANT. PART

A UNILATERAL CLEFT LIP RESULTS FROM FAILUREOF FUSION OF THE MEDIAL NASALPROMINENCE

WITH THE MAXILLARY PROMINENCE.

AETIOLOGY

HEREDITY ( 25 % )

CONGENITAL

ENVIRONMENTAL

Nutritional Deficiency

Radiation Energy

Steroid Injection

Hypoxia

Drugs

Amniotic Fluid Altrns

GENETICS

Genetic factors are assosiated with orofacial clefting

Incidence cleft lip palate is more for monozygotic twins than for dizygotic twins

Some genes assosiated with orofacial clefting

TGF – ALPHATGF – BETAMSX1 (HOMEOBOX GENE)RETINOIC ACID RECEPTOR ALPHA

Some syndromes associated with clefting

VANDER WOUDE SYNDROMETREACHER COLLIN SYNDROMEDEL SYNDROME

EEC SYNDROME – ECTRODACTYLY ECTODERMAL DYSPLASIA, CLEFT LIP

OROFACIAL DIGITAL SYNDROME

VELO CARDIOFACIAL SYNDROME

HUMAN TERATOGENS CAUSING CLEFT

ETHYL ALCHOLDIPHENYL HYDANTOINTRIMETHADIONERETINOIDSMETHOTREXATEHYPERTHERMIASMOKING

THEORIES

THEORY OF DURSY & HIS:

Put forward the hypothesis of failure of fusion of the various facial process.In fact it sounded a very convincing theory

The fusion theory is no longer because it is realized that

importance is for localized prominence.

THEORY OF FAILURE OF MESODERMAL MIGRATION

Fleischmann, a zoology prof in (germany1910) stated that cleft palate isthe arrest of the disappearence of the epithelial membrane,which remains intact,not penetrated by the adjacent mesoderm.This theory was further supported by victoe veau (1935) & by stark

Failure of sufficient mesoderm to migrate into a specific area is responsible for the persistance of a groove. With subsequent breakdown of the epithelium, the persistant groove gives way to an established cleft

IT IS NOW ACCEPTED THAT NO THEORY IT IS NOW ACCEPTED THAT NO THEORY UNIVERSAL ACCEPTANCE.UNIVERSAL ACCEPTANCE.

CLASSIFICATION

DAVID AND RITCHIE (1922)on anatomical basis

GROUP III – ALVEOLAR CLEFTS UNILATERAL BILATERAL OR MEDIAN.

GROUP I – PRE-ALVEOLAR CLEFTS UNILATERAL , BILATERAL OR MEDIAN

GROUP II – POST-ALVEOLAR CLEFTS

• SOFT PALATE ONLY

• SOFT AND HARD PALATES

• SUBMUCOUS CLEFT

VEAU (1931)

1 ) Cleft of soft palate only.2 ) Cleft of hard and soft palate extending no further than incisive foramen, thus involving secondary palate alone.3) Complete unilateral cleft, extending from the uvula to the incisive

foramen in the midline, then deviating to one side and usually extending through alveolus at the position of the future lateral incisor tooth.4) Complete bilateral cleft, resembling Group III with two clefts extending forwards from the incisive foramen through the alveolus

FOG ANDERSONS - 1942

GP -1 – CLEFT LIP – U/B

GP -2 – CLEFT LIP & PALATE – U/B

GP -3 – CLEFT OF PALATE UP TO INCISIVE FORAMEN

INTERNATIONALLY APPROVED

CLASSIFICATION (landmark-incisive foramen) –sanvenelo – roseli 1967

GROUP I: cleft of ant palate a.LIP – R&/L b.Alveolus - R&/L

GROUP II : CLEFT OF ANT & POST PALATE a. LIP – R&/L b. Alveolus – R&/L c. HARD PALATE – – R&/L

GROUP III: CLEFTS OF POST PALATE a. Hard palate : r& / l b. Soft palate

GROUP IV: RARE FACIAL CLEFTSRARE FACIAL CLEFTS

KERNAHAN (1971)

1,4 – LIP2,5 – ALVEOLUS3,6 – PALATE ANT TO INCISIVE FORAMEN7,8 – PALATE POST. TO INCISIVE FORAMEN9 – SOFT PALATE

The LAHSAL code splits the relevant

parts of the mouth into six parts:

Right Lip Right Alveolus Hard Palate Soft Palate Left Alveolus Left Lip

The first character is for the patient's

right lip, and the last character for the

patient's left lip. Example –

. . HS . L – Complete Cleft of Hard &

Soft Palate with Left Complete Cleft Lip

LAHSAL CODE 1987

SCHUCHARDT & PFEIFERS

PFEIFERS MORPHOGENIC CLASSIFICATION

1- generalized malformations

2- malformation in fronto nasal region

3- malformation in diacephalic border

4- malformation in post lat region

5-malformation in the neck

PAUL TESSIER - 1976

ANATOMY OF UNILATERAL CLEFT LIP

MUSCULAR ANATOMY

It is devided into 3 rings

Upper ring Transversus nasi

Levator labi superioris alaeque nasi

Levator labi superioris

Zygomaticus minor

MIDDLE RING

It represent the oral sphincter

Consist of upper and lower lip orbicularis oris muscle

LOWER RING

Orbicularis inferior Triangularis labi

Quadratus labi inferioris

The upper lip orbicularis oris is made up of 3 strata

Horizontal band – internal orbicularis

Oblique band – external orbicularis

Incisal bands

Mytriformis

Internal orbicularis – horizontal fibers from one commisure to anotherMuscles are superficialy inserted into the

mucocutaneous junction giving the prominence “ white roll”

External orbicularis – these are superficial to internal orbicularisThis determines the presence of philtral crest Oblique in natureThis fibers fans out from nasal spine runs to

commisures and intermingles with other fibers

Incisal fibers – They lie deep to the oblique fibers Get inserted into border of mitriform fossa ( apex

of the latral incisors) and then move towards the commisures

Mytriformis – Small fibers which get attached to mitriform

fossa and then with fibers of transverse nasi

Triangularis labi –

Begins at the lower mandibular border latral to chinIt intermingles with the orbicularis oris

Quadratus labi - orginates more medially from lower border of mandible and insert into inferior orbicularis

NORMAL LIP ANATOMY THE ELEMENTS OF THE NORMAL LIP ARE - CENTRAL

PHILTRUM, PHILTRAL COLUMNS ,

CUPID'S BOW

THE MUCOCUTANEOUS RIDGE ABOVE THE JUNCTION

OF THE VERMILION-CUTANEOUS

BORDER IS A FREQUENTLY

REFERRED TO AS THE WHITE ROLL.

WITHIN THE RED VERMILION OF

THE LIP IS A NOTICEABLE JUNCTION DEMARCATING

THE DRY AND WET VERMILION.

LEVATOR LABII SUPERIORIS

ARISING FROM THE MEDIAL ASPECT OF THE

INFRAORBITAL RIM, SWEEP DOWN TO INSERT

NEAR THE VERMILION CUTANEOUS JUNCTION.

ITS MEDIAL-MOST FIBERS SWEEP DOWN TO

INSERT NEAR THE CORNER OF THE IPSILATERAL

PHILTRAL COLUMN AND VERMILION-

CUTANEOUS JUNCTION, HELPING TO DEFINE

THE LOWER PHILTRAL COLUMN AND THE

PEAK OF THE CUPID'S BOW.

LEVATOR SUPERIORIS ALAEQUE

ARISES ALONG THE FRONTAL PROCESS OF THE

MAXILLA AND COURSES INFERIORLY TO INSERT

ON THE MUCOSAL SURFACE OF THE LIP AND

ALA.

TRANSVERSE NASALIS

ARISES ALONG THE NASAL DORSUM

AND SWEEPS AROUND THE ALA TO

INSERT ALONG THE NASAL SILL FROM

LATERAL TO MEDIAL INTO THE

INCISAL CREST AND ANTERIOR NASAL

SPINE.

DEPRESSOR SEPTI ( MYTRIFORMIS )

WHICH ARISES FROM THE ALVEOLUS

BETWEEN THE CENTRAL AND

LATERAL INCISORS TO INSERT INTO

THE SKIN OF THE COLUMELLAR TO

THE NASAL TIP AND THE FOOTPLATES

OF THE MEDIAL CRURA.

ORBICULARIS ORIS DEEP (INTERNAL) FIBERS RUN HORIZONTALLY FROM COMMISSURE (MODIOLUS) TO COMMISSURE AND FUNCTIONS AS THE PRIMARY SPHINCTERIC ACTION FOR ORAL FEEDING.

SUPERFICIAL (EXTERNAL) FIBERS RUN OBLIQUELY, INTERDIGITATING WITH THE OTHER MUSCLES OFFACIAL EXPRESSION TO TERMINATEIN THE DERMIS.

THE SUPERFICIAL FIBERS OF THE ORBICULARIS DECUSSATE IN THE MIDLINE AND INSERT INTO THE SKIN LATERAL TO THE

OPPOSITE PHILTRAL GROOVE FORMING THE PHILTRAL COLUMNS. THE RESULTING PHILTRAL DIMPLE CENTRALLY IS DEPRESSED AS THERE ARE NO MUSCLE FIBERS THAT DIRECTLY INSERT INTO THE

DERMIS IN THE MIDLINE.

Lateral displacement of non-cleft premaxilla and tilts upwards into the cleft.

Nasal septum and columella is bent laterally towards the non-cleft side . The orbicularis oris muscle is inserted laterally into the maxillary bone in

the region of the alar base and also into the lateral crus of the alar cartilage. Medially, it inserts into the bony margin of the cleft and into the nasal

spine. The nose is asymmetrical and there is no nostril sill. On the cleft side, the alar base is displaced posteriorly, inferiorly and

laterally. The alar cartilage is usually unfolded and droops down .

BILATERAL CLEFT

Short columella medial crusMalinsertions of musclesNo nasal silBroad nasal tipDome ofAlar cartilage-down

rotationThe base of the ala are

broadened and withdrawn as a result of back ward position of underlying skeleton and also muscle insertion stops at this level

Effect of 3 muscle rings on skeletal growth

The muscle rings anatomy and function greatly affect the growth of the underlying skeleton

The integrity of 1st ring ( often disrupted in cleft lip) is fundamental for sustaining and allowing the normal function of other 2

PATHOLOGICAL ANATOMY

TOTAL UNILATERAL CLEFTALTERATIONS IN

NASOLABIAL MUSCULATURE -;

Lack of fusion of maxillary and nasal process - prevents the nerve, muscle and blood supply reaching the mid line

All the muscle which attaches to nasal spine ,septum, premaxilla, gets attached to the latral border of the cleft

The absence of central incisor lead to disequilibrium b/w 1st and 2nd ring

Nasal abnormalities

Nasal septum , columella will be deviated to contralatral side of the cleft due to unbalanced traction of muscles

Alar cartilae on affected side is ptotic streched and rotated

Cartilagenous structures are deformed and dislocated but not hypoplastic

Premaxillary contralatral deviation

MUCOCUTANEOUS ABNORMALITIESFrom both external and internal stump of the

cleft the skin of the nasal floor has to be defferentiated from lip

Nasal skin – 1.Fine grained appearance 2. Skin is much flatter

Lip skin – has cutaneous retraction associated with insertion of muscles

SKELETAL ABNORMALITIES

The main stump is rotated out wards due to the pressure from tongue and traction of the muscle of the healthy side, whch is not counter balanced by the cleft side

Hypoplasia of the alveolomaxillary portion of the premaxilla which lies b/w cleft and median suture

Increase in transverse diametre of maxillary tuberosity and pterygoid process because of the non fusion of palatine musculature along the mid line

Both bony and cartilagenous part of the nasal septum is streched towards healthy part

MUSCULOMUCOSAL HARD AND SOFT PALATE ALTERATIONS

Reduction in the fibro mucosa of palatine lamina in the lateral stumps

Where as the maxillary and gingival fibromucosa remain practically normal

BILATERAL CLEFT

Alteration in nasolabial musculature

In lateral stumps similar to unilateral cleft

But in medial tubercle no trace of muscles because muscles orginating from sides and stops at the border of the cleft

NASAL ALTERATIONS

Nasal alterations are similar to unilatral cleft only difference is they are symetrical

The base of the ala are broadaned and withdrawn as a result of back ward position of underlying skeleton and also muscle insertion stops at this level

The domes of the alar cartilage shows downward rotation

SKELETAL ABNORMALITIES

The pre maxilla is protrubrant and rotated forward( with its fulcrum at the level of the nasal spine

Transverse dimension of the pre maxilla are reduced because of the under activity of the median suture which does not under go musculo periosteal traction leading to its traction

MUSCULO MUCOSAL HARD AND SOFT PALATE ALTERATIONS

The posterior part of the nasal septum is vertically under devoloped and does not reach the level of the palatine process

EVOLUTION OF CLEFT LIP SURGERY

390AD in china and document the cutting and suturing of cleft lip edges

Ambroise pare in 1564 did a straight line freshening of cleft edges by introducing long needle through both lip elements wrapped with a thread in fig of 8

Rose (1891) and thompson(1912) described angled excisions of short cleft edges to obtain length with closure

Mirault (1844) described latral inf triangular flap to be aproximated to a medial parring

In 1949 le mesurier – latral quadilatral flap introduced into a releasing incison in the medial element created an artificial cupids bow

Tennison 1952 designed an z - plasty

In 1959 randal modified tennison method with mathematical markings

Skoog 1969 later modified his approach by keeping the inferior latral flap

TIMING OF THE OPERATION

Most surgeons delay lip repair until 10 weeks after birth to get sufficient tissue bulk .

RULE OF TENS states that cleft lip surgery should be delayed until the child is 10 pounds heavy, has a haemoglobin level of 10 gm%

and a WBC count of 10,000/mm3 and is at least 10 weeks old.

TIMING OF REPAIR

MILARD

INITIALLY – 3MONTHS OF AGE

LIP ADHESION – 2-3 WEEKS

DEFINITIVE CLOSURE – 5 – 7 MOTHS LATER

Later Incomplete lip – 3 – 6 months

Complete cleftsLip adhesion – 3weeksDefinitive closure 6 – 8 months Soft and hard palate – 1 ½ - 2 ½ yrs

Delaire

Unilatral cleft lip – end of 6 months

Bilatral cleft lip – 4th month

And during 7th month dento alveolar element of pre maxilla and latral segments are realinged for gingivo perioplasty

Recent concept Talamant

Primary lip nose repair – 6 months in same step as that of closure of soft palate

Hard palate – 18 months of age in 2 planes with a mid line approach with out vomerine flap or denude bone area

Problems???

Dental (Cleft alveolus & teeth)

Congenital absenceSupernumeraryMalpositioned teethDeformed teethHypomneralised

Problems???

MalocclusionOften class III Crowding of teeth Narrow arch as in unilateral cleftNarrow arch in posterior and protruded

anterior segment as in bilateral clefts

Problems???

Nasal deformity

Alar cartilage flared to affected side Columnella pulled to non-cleft side

Feeding

Swallowing normal (hypo pharynx) Ineffective sucking (lack in Negative

pressure + poorly developed musculature)

Problems???

Ear

Ear function disturbedEar infection (due to lack of muscle

function)Otits Media (due to fluid accumulation)Chronic Otitis Media *threat to hearing

Problems???

Speech

Retardation of carsonant sounds (p, b, t, d, k, g)

These are necessary for early development of vocabulary

Hyper nasality (Due to loss of velopharyngeal function)

Articulation suffers (due to dental malformations)

SURGICAL GOAL

Approximation of the cleft edges should be achieved with out loss of natural land mark

There should be little to no discard of tissue

The cupids bow should end in a balanced position

The scar of union should be placed along a natural line

The muscle should be brought together with full bodied alingment resulting eversion of lips free border

Alar base should be balanced and columella equal on both sides

The defenite result should be symetrically functional and esthetically natural

A symetrical red border

STEFFENSON (1953) HAS LISTED FIVE CRITERIA FOR A SATISFACTORY LIP

REPAIR.

Accurate skin, muscle and mucous membrane union with adequate lip lengthening

Symmetrical nostril floor Symmetrical vermilion border and white roll Slight eversion of the lip A minimal of scar which by contraction will not interfere with the other stated requirements.

TWO CRITERIAS WERE ADDED LATER BY MUSGRAVE (1971)

• Preservation of the cupid’s bow• Production of symmetrical nostrils

GENERAL MANAGEMENT

Immediately after birth –

Pediatric consultationCounselingFeeding instructionsEvaluation by genicistDiagnostic tests

Team evaluationHearing testing

After surgery

Speech and language assessmentSpeech therapyFistula repairSoft palate lengtheningPsycho social evaluation

5-6yrs – lip and nose revision if needed

7yrs – orthodontic treatment

9-11yrs – bone grafting of alveolar bony defectImplant placement

UNILATERAL CLEFT LIP REPAIR

DELAIRE TECHNIQUE

A- upper corner of healthy nostril

A1- upper corner of cleft nostril

B- base of the healthy columella

C- mid point of the philtrum at the mucocutaneous junction

D- summit of the cupids bow on the non cleft side

1- base of columella cleft side at equal distance from midline to B

2- continuation from B-1 intersects the mucocutaneous line

3- point in mucocutaneous line whose distance from mid line is little less than distance from C-D ( CD =C3)

4- point in straight line from 3 ,between vermillion and wet mucosa

5- base of the nasal ala on the cleft side

6- point on the mucocutaneous line perpendicular from land mark 5

7- point on greatest vermilion width on the cleft side where the mucocutaneous rim begins to diminish (future lateral peak of cupids bow)

8- Point on line with 7 between vermilion and wet mucosa

Cutaneous incision begins at the inner stump, passes to 2-3-4

From 2 it goes up along the mucocutaneous junction until it reaches the base of the alveolar process

The mucosa and the mucocutaneous border of the free side of the main stump is discarded

in the small stump the incision 5-6

And then if the mucocutaneous line is not pronounced from 6-7-8

If the mucocutaneous line is pronounced then it is necessary to preserve the white roll from 7-E in the form of a triangular flap

Once the cutaneous incision, excision, and preservation of the free border is performed

Finding the muscles

The muscles and there insertions has to be identified

Transverse nasi – can be identified immediately beneath nasal skin ( above line of 5-6)

Levator labi sup. Alaque nasi - prolongation of the line 5-6 to the base of the ala after under mining a few mm of skin muscle can be found

Levator labi sup – mucosal level at the base of the fornix – this fibers should not be detached these fibers will keep the mucosa well raised once the reconstruction is over

SUB PERIOSTEAL UNDERMINING

In order to ensure tension free suturing of the nasolabial musculature, the muscular insertion of the anterior face of maxilla must be widely undermined

Ideal way of doing it is sub periosteally and not supra periosteally – a procedure that does not have any effect on facial growth

Through the incision made in the fornix , the sub periosteal dissection has to be extended to frontal branch of maxilla, orbital rim (going around the infra orbital nerve), to the zygoma as far as the maxillo malar buttress

Sub perichondrial undermining and releasing of alar cartilage –

For a corrected nasal deformity to be

symmetrical it is necessary to free the pathological half of the nose completely from its connection from its healthy half

Through incision 1-2 blunt dissection is done to the 2 medial cura – tip – skin separated from alar cartilage on cleft side

Same under mining done on the dome of the healthy side and dorsum over the triangular cartilage

The freeing of 2 half of nose is completed by sectioning the mid line connective tissue in b/w the intercural tunnel

Base of the columella on cleft side is released by sub mucosal dissection

Superficial and deep levator labi muscle as well as the external orbicularis are sutured to the nasal spine and the corresponding contralateral muscle

Then the internal orbicularis is sutured to vermilion

Then skin is approximated in the most superficial part of nasal floor and the upper half of the lip

Lower half of the lip is sutured subcutaneously

If while assessing the symmetry of the lip if it is too short z- plasty done just above the mucocutaneous border

For optimal continuity of the mucosal layer should be released for a few mm from under lying orbicularis

The innervention concludes with careful reconstruction of vermillion

MILLARDS APPROACH

Surgical techniqueRotationComponents on cleft side –

2/3rd – 3 quarters of cupids bowMedian tubercle on the vermillion One column of philtrum and its associated dimple

All this is rotated down to the normal philtrum

MILLARD’S ROTATION ADVANCEMENT REPAIR

ROTATION ADVANCEMENT MUSCLE DISSECTION ALAR BASE CINCH SUTURING

AdvancementAn advancement flap fills the gap and corrects

the alar flare and wide nostril

Advancement flap is marked generally to fit the rotation

Unilateral cleft lip repair

1- junction of mucocutaneous junction in the middle of cupids bow

2- placed in the height of the bow on the non cleft side

The distance from 1-2 determines the exact distance towards the cleft for point 3

12

37

56

BA

8

9

4

Advisable to mark with dots the normal philtral column to indicate the ideal matching philtral column position of the scar of union during cleft closure

Rotation incision starts at point 3 freshening the cleft with a gentle curve to the base of the columella

This procedure provides 4mm edge towards matching the 10mm of the normal side

At the columella base the rotation incision continues 2/3rd the way across closely hugging the base, which provides another 3mm of edge

The rotation is increased with a acute back cut approximately 90 degree running parallel but medial to the normal philtral column

This provide another 2-3 mm edge on the rotation side

The rotation incision is carried through the muscles to liberate the labial mucosa from maxilla

The skin and the mucosa of the rotation edge is elevated no more than 1-2mm from the muscle

Flap – c – which during incision is cut from lip but is left attached to the side of the columella

Flap- c – is rotated into the back cut, this provides extra length at the base of the short columella

The lateral lip element should be pared to equal the length of rotation side

Release of the lip from alar base by a horizontal incision helps to free the advancement flap

The lateral lip element is detached from attachements to maxilla and advanced into the rotation gap to observe the fit

MUSCLE DESSECTIONLatral lip element

should be freed genourously from skin by careful underminig

Usually tip of advancement flap left undessected so that mucosa and skin advanced together

ALAR BASE CINCH

The alar base is freed from the lip by a circum alar incison

The tip of the alar base flap is denued of epithelium and then threaded under flap c and sutured to the base of the septum to cinch the alar flare

Unilateral cleft lip repair Pros & Cons

Repair Advantages Disadvantages

Millard rotation advancement

•Procedure allows adjustment as operation proceeds•Minimum amount of tissue is discarded•Scar are placed in anatomically correct position, in line of philateral coloumn •Nostril sill is reinforced and built up•Revision is easy

•Most difficult for beginner to master•Approximation of two convex curves leaves the majority of bulk in the centre of the lip and not on the lower free border this may cause pouting appearance in wide clefts•Tendency is to early contracture of long vertical lip scar•Technically difficult in wide clefts•Tendency is toward a constricted nostril on cleft side

triangular flap repairs

•Relatively inexperienced surgeons can obtain reasonable results•Achieves excellent lengthening of shortened cleft side

•Horizontal scars at triangle site transgress normal anatomical features of lip

Tennyson -Randall

•Cupid bow is preserved and well aligned •Procedure is of particular value in wide cleft •Only small amount of tissue is discarded

•Cleft side may end up too long, to avoid this cleft side repair should be designed 1 mm shorter than non cleft side

Nakajima •Straight scar line is easy to revose•Triangular flap is hidden at nostrill sil

•May form vertical contacture

Rose -thompson •Scar orientation good•Uncomplicated by small flaps

•May form vertical contracture•Poor procedure for wide cleft •Too much tissue discarded

REPAIR OF TOTAL BILATERAL CLEFT

If the pre maxilla is properly positioned surgery is recommended – 4 th month

As in unilatral cleft lip the repair begins with repair of soft palate

Nasal layer is not closed with vomerine flap – maxillary growth will be hampered

Correction of lateral stumps are similar to that of unilateral cleft lip

But in the prolabium there is absence of muscle distention

The skin of the columella descent into prolabium

Its considered that, in dimensions running from upper inside angle of the nares to the future top of the cupids bow on the skin of the prolabium the upper half is the columellar skin and the lower half is labial skin

Two symmetrical points 2 are there fore marked on ideal extension of lateral border of columella

And the points at the top of the two peaks of cupids bow is identified

2-3 an incision is made following a curve medially concave

Then incision is continued to the mid line following a curve whose concave side is downward( always remain above the mucocutaneous border)

At the mid line it meets the corresponding contralateral incision

From point 2 another incision is made perpendicular to mucocutaneous border once junction is reached it is prolonged to the level of the bone

The prolabial skin is lifted by cleaving it from the underlying periosteum

Nasal spine and the lower border of the septal cartilage is reached ,these are exposed along with lower border of piriform aperture

Sup periosteal and sub peri chondral undermining of the septum is also done

Nasal layer closed, transverse nasi, orbicularis oris muscle sutured to mid line( nasal spine) and the vermillion border sutured

BILATERAL CLEFT REPAIR

This technique needs large prolabium

Lateral vermilion mucosal flaps with white rolls are brought to the mid line while the prolabial vermillion is turned downward

Muscle to mucosa and mucosa to muscle is done behind philtral strip of prolabium

2nd stage v-y advancement is done to lengthen the columella

This is done in pre school period

Repair of columella

Most procedure – forked flap technique by millard which takes tissue from lateral prolabium and brings it into columella

New concept

States that there is no skin deficiency in columella

The nasolabial angle is probably the only land mark that separates the nose from the philtrum and should be respected in ideal repair

Surgical repositioning of alar cartilage is enough alone to build up a normal columella with out skin plasty to bring tissue into it

ADVANTAGES

Allows adjustments as operation proceeds

Minimal amount of tissue discarded

Scars placed in anatomically correct position Nostril sill is reinforced and built up

Ease of revision

DISADVANTAGES

Difficult technique to master

Two convex curves- pouting appearance

Contracture of long vertical lip scar Difficult in wide scar

Inadequate length

Scar across base of columella

Complications

Wound infection Wound disruption or spreading of scar – due to

excessive tension and infection can complicate the problem

Tilting or retrusion of the premaxillaCan be avoided by preventing excessive

tractionThe vomer should not be resected in the region

of the provomerne – vomeral suture

Whistle deformity – can be prevented by using lateral muscle vermillion flaps to augment the thickness of prolabium

Excessive long lip

RECENT CONCEPT – J C TALAMANT

Pre surgical nasoalveolar molding ,lip adhesion not needed

Precise repositioning of the lower lateral cartilages enough for columellar lengthening

And there is no need for pre surgical naso alveolar molding or bringing tissue from lip

The new concept is “ the columella is inside the

nose”

Primary lip nose repaired at 6 months of age in same step as that of closure of soft palate

The remaining cleft of hard palate is closed at 18 months in 2 planes with a mid line approach with out vomerine flap or denuded bone area

Dissection of nasal cartilage from the overlying skin beginning medially on the septum and laterally of the caudal end of the lateral cura is important

Repositioning secured by custom made appliance is necessary

Complete repositioning of the alar bases of the nose trills both on horizontal and vertical axis must be under taken

Various other methodsTennison method –s: central part of vermillion

protrudes in a normal manner than in straight line closure

: result in zig zag scar

Revision difficult due to scars

Only 1 side repaired at a time

Manchester method:

Disadvantage: Prolabial vermillion is

kept in a exposed position so that appreciable differ in color may occur compared with labial segments

Barsky technique

Not used now

Result in unnatural appearance of lip

Wynn methodCan be used if

prolabium is small

It makes prolabium too long

Does not provide sufficient augmentation for thin prolabial vermillion

PRIMARY ABBE FLAPNOT CONSIDERED AS A PRIMARY

PROCEDURE

RECOGNIZED AS A SECONDARY TREATMENT FOR TIGHT BILATERAL CLEFT LIP

Veau III procedure:

This procedure provides a satisfactory result

Secondary lip repair

Z- plasty is done to correct the notch in the vermillion

Basic idea is to raise vermillion-muscle flaps and transpose them in a way that they will fill the notch and approximate the edges of the vermillion, creating fullness and symmetry

All flap including the vermillion and orbicularis muscles are raised

Vertical scar contracture

After excision of the scar, a triangular flap in the upper portion of the non cleft segment will be transferred into the defect created around the base of the ala on the cleft side.

Excision o f the existing scar to release both lip segments

Incision extended around the base of the ala on the cleft side and bring it to symmetric position with the ala on the opposite side

To prevent secondary secondary vertical scar contracture additional z plasty including skin, muscle, in the lower portion of the lip

Intra uterine cleft lip repair

Fetal surgery is an emerging technology in which the patients are not born and interventions have to be

done in intrauterine life.Indications:1. A defect that if not corrected can cause the fetus

not to survive till delivery.2. A defect that if not corrected before birth can cause

the permanent anatomic or physiological loss of organs.

3. A defect if not intervened before birth can advanced to such a degree that cannot be retrieved

postnatally.

Cleft lip and palate repair is one of the the condition for which fetal surgery has been implicated

Modalities in Fetal Surgery:Open Fetal SurgeryFETENDO (fetoscopic/endoscopic)FIGS (fetal image-guided surgery)

Open Fetal Surgery:This is the most invasive form of fetal surgery. The mother is anaesthetized and an incision is

given in lower abdomen to expose uterus. USG is used to localize the placenta. Fetus is

injected a narcotic analgesia and muscle relaxant.

Amniotic fluid is aspirated and preserved for reperfusion.

Now uterus is opened using a special stapling device to prevent hemostasis in highly vascularised uterus.

Warm saline is continuously infused around the fetus.

Fetus is monitored by pulse oximetry and radio

telemetry. Fetus is intervened and uterine incision is

closed with absorbable sutures and fibrin glue.

It is interesting observation that fetal incisions heal without scars.

This revolutionized the repair for cleft lip and cleft palate in intrauterine life.

EXIT (exutero intrapartum treatment):This is special type of open fetal surgery. Usual open fetal surgery is performed round

about midgestation but this type (EXIT) is performed to

coincide with delivery i.e. fetus is intervened but not

returned in uterine cavity and delivered. In this type of open fetal surgery fetus is delivered

out of uterine cavity but cord is not clamped so that fetus is sustained by mother’s placenta.

EXIT is performed in cases where there is airway obstruction by large neck tumors such as cervical

teratoma and cystic hygroma etc.

EXIT provides time to maintain the airway by resecting the tumor or performing tracheostomy before ligating the umbilical cord.

FETENDO (fetoscopic surgery):This is developed in 1990s to avoid incision in

uterus and minimize preterm labor.

In this technique fetoscopes are inserted through mini-holes in uterus and then in fetus and procedure is performed with less chances of preterm labor

FIGS (fetal image guided surgery):This is the least invasive form of fetal surgery.

The manipulations are done entirely under crosssectional view provided by sonograms.

It can be done under regional anesthesia even under local anesthesia.

FIGS was first used for amniocentesis and fetal blood sampling but now is used for a variety of manipulations including radiofrequency ablation of anomalous vessels in case of TTTS and TRAPS and placement of vesico-amniotic shunts to decompress the urinary tract in case of posterior urethral valves.

Complications:Preterm labor:This risk varies in magnitude depending upon

the invasiveness of the procedure. The risk of preterm

labor is more with open fetal surgery and least with FIGS.

Bleeding:Infection:Puncture of membranes:Anesthesia complications:

Complications of prematurity:Studies has shown there is increased risk of

premature deliveries in mothers underwent fetal surgery causing premature births and prematurity associated problems.

Drugs related harm:Various drugs are used post operatively to

control pain and preterm labor that may itself cause fetal

and maternal complications:Abruption placenta:Fetal death in utero and during procedure

Advantages Fetal repairs may achieve result that more closely

approximate normality

Decresead need for extensive post op care, orthodontia and speech therapy

Alleviate the psychologic trauma associated with the birth of an infant with craniofacial malformations experienced by parents and infant

Sullivan approximation of cleft lip edges without incision underwent refusion and reorganization into lip architecture nearly indistinguishable from noramal

Another advantage of fetal lip and palate repairing is reduction of bony deformation

For example when post natal repair is limited to only cleft lip in complete unilateral clefting of palate ,there is progressive bony deformation and mal alignment of alveolar ridge.

Fetal repair may further limit the extent of bony deformation at a point early during bone development and render a more functional anatomy ,decreasing subsequent surgical reconstruction

Fetal diagnosis

Routine pre natal ultrasonographic examination

Better ultrasonic resolution has improve ability to identify defect

Diagnosis are now being made even at gestation age prior to 20 weeks

Bilateral cleft lip and palate is more elusive than unilateral because premaxilla and primary palate protrude beyond the coronal plane

How and when to intervene

Early intervene in gestation near 20 weeks or less

Skin immaturity would likely to provide better wound healing than during late third trimester repair.

[Skin development and diffrentiation with

increasing dermal complexity and maturation of extracellular matrix may be a limiting feature of scarless wound healing]

This stage uterus more ameanable to manipulation and endoscopic intrusion reducing the risk of inducing pre term labour

Procedure

Procedure

Use of endoscope diameter less than 1 mm allowed to visualization

Limitations – suturing suture placing external knot tying with out

excessive tissue compression

Kirch and Zhu- Microclips

Intra uterine fetal surgery is emerging as the next frontier in advancement of cleft lip and palate repair

Although the prospects for fetal cleft lip and palate repair promising,extensive research comparing the risk and benefits must be employed before this type of surgery.

Rehabilitation of the patient with cleft lip and palate is a challenging task to the

surgical team. The main aim should not be just aesthetics but also anatomy,form

& function for betterment of an individual…

CONCLUSION

REFERENCES

Mccarthy - vol 4Peter Wardbooth – vol 1Principles of oral and maxillofacial surgery –

Peterson vol.2Surgery of mouth and jaw- J.R.MOOREClinics of plastic surgery- prospectives in

cleft lip and palate repair vol 20 no 4 october 1993

FETAL SURGERY -Muhammad Bilal Mirza peadiatric surgery apr 2008

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