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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 )
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.
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.
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.
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
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
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
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
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
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
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
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
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
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
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
Use of endoscope diameter less than 1 mm allowed to visualization
Limitations – suturing suture placing external knot tying with out
excessive tissue compression
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