CLEFT LIP AND CLEFT PALATE

Dr. Ch. B. Prathyusha,PG ENT

Narayana Medical CollegeNellore

IntroductionPrevalence EtiologyEmbryology Classification ManagementDefects and Reconstruction

INTRODUCTION

History of cleft lip is based on a combination of

religion, superstition, invention and charlatanism.

Aztecs of Central Mexico

Chinese belief

Eating hare causes “hare lip”

All these beliefs came to an end

 Fabricus ab Aquapendente (1537–

1619)

Anatomist and surgeon 

“The Father of Embryology”

William Harvey was his student

Introductionnon syndromic clefts

Majority of orofacial clefts are nonsyndromic 70% of Cleft lip +/- palate 50% of CP only Multifactorial Clusters in families but not Mendelian Palate development complex process with Several proteins, growth factors, and

transcription factors involved IRF-6, TGF –B2, TGF-alpha Any disturbance in the process can result in

clefting

Syndromic clefts

Associated with over 300 syndromes

Van der Woude syndrome – the most common Autosomal dominant Lower lip pits

Teratogen exposure Ethanol, thalidomide,

phenytoin Environmental factors Amniotic banding, maternal

diabetes, maternal folate deficiency

Anatomy of lip and palate

Anatomy of lip

Embryology

Face appears to develop at the end of the fourth week

is completed by the eighth week

The palate is formed by the end of the tenth week.

4th week A series of swellings appear around the

stomodeum

the frontonasal process,

the bilateral maxillary process

the mandibular process.

The developing nasal placodes arise as thickenings on either side of the frontonasal process.

5th week

nasal placode is surrounded by a horseshoe-shaped ridge consisting of lateral and medial nasal processes with nasal pit in the middle.

The nasal pits deepen and rupture, forming communications between the nasal and oral cavities, called primary choanae.

7th week

maxillary processes have advanced medially underneath the lateral nasal swellings and fused with the medial nasal swellings.

This complex comprising the fused medial

nasal swellings and maxillary swellings forms the primary palate

complete by the end of the seventh week

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

Primary palate

secondary palate

Primary palate

Medial nasal prominences fuse to form the primary palate

Consists of maxillary alveolar arch with 4 incisors and the hard palate anterior to the incisive foramen

Primary palate forms before the secondary palate begins formation

Secondary palate During the 6th week Shelf-like outgrowths from the

bilateral maxillary processes, grow vertically down on both sides of the tongue

During the 7th weekThe tongue moves inferiorly and the palatal shelves migrate to a horizontal position above the tongue

Palatal fusion occurs in an anterior to posterior direction and completes with uvular fusion (1 week later in females)

Cleft lip and palate

Disruptions at any stage of the developmental process can result in clefts

Timing

Positioning

Cleft lip and palate formation

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

Classification

Clefts

Unilateral or bilateral

Complete or incomplete

Davis and Ritchie Classification

Veau’s Classification

Davis and Ritchie Classification divides cleft lip and palate

into 2 groups, which subdivided into the extent of the cleft (eg, 1/3, 1/2), as follows:

Group I - Clefts anterior to the alveolus (unilateral, median, or bilateral cleft lip)

Group II - Postalveolar clefts (cleft palate alone, soft palate alone, soft palate and hard palate, or submucous cleft)

Veau classification Class I – incomplete cleft

involving only the soft palate

Class II – cleft involving the hard and soft palate

Class III – complete unilateral cleft involving the lip and palate

Class IV – complete bilateral cleft

Unilateral cleft lip Incomplete

Muscle fibers of the orbicularis oris are often intact but hypoplastic

Varying degrees of clefting

CompleteOrbicularis oris inserts at the columella medially and ala laterally on the cleft side

Columella is displaced to the normal side

Nasal ala on the side of the cleft is displaced laterally, inferiorly, and posteriorly

Nasal tip is deflected towards the noncleft side

Alveolus may or may not be involved

Bilateral cleft lip

Orbicularis oris attaches at the lateral cleft margins bilaterally at the nasal ala

Premaxilla protrusion

Symmetrical nasal deformities

Laterally displaced ala – widely flared

Extremely short columella

Incomplete to complete hard palate

Primary palateClefts anterior to the incisive foramen

Secondary palatePosterior to the incisive foramenDevelops due to failure of the palatal shelves to fuseAbnormal insertion of the muscles into the medial edges of the cleftVomer attachment is variable

Management Multidisciplinary approach

Cleft care team Plastic surgery Audiology Speech pathology Otolaryngology Orthodontist Oral maxillofacial surgery Psychologist Geneticist Pediatrician

Management Birth

Airway concerns

Feeding problems

Otological disease

Speech and language problems

Surgical Repair

Initial management

Airway ManagementIsolated cleft palate rarely results in airway issues usually coexisting structural abnormalities

Pierre Robin sequence (most documented)Micrognathia, glossoptosis, and cleft palateAssociated with several syndromes (stickler, velocardiofacial syndrome, etc.)

Management – prone positioning (severe cases sometimes require tracheostomyMandibular distraction

Feeding difficulties Critical aspect in management

Nutrition and feeding

Cleft palate limits the ability to suck due to the common cavity

Cleft lip alone does not cause much problem

Special bottles

Frequent burping

Premaxillary orthopedics

Otological issues

Abnormal insertion of tensor veli palitini

Persistent OME has been estimated to be between 80-95% in children with cleft palate

Speech development Unrepaired cleft palate – speech abnormality

Primary goal of palate repair is to restore function of the velopharyngeal valve

normal speech 10-20% will manifest VPI following surgical closure of the

palate

Hypernasality and articulation errors (glottal stops and pharyngeal fricatives)

Speech pathologist – important role

Managed surgically (pharyngoplasty or pharyngeal flap) or with dental prosthesis

Surgical correction Age 1-3 months – Lip taping and nasoalveolar molding

Age 3 months - Repair of cleft lip (and placement of ventilation tubes)

Age 9-12 months - Repair of cleft palate

Age 1-7 years - Orthodontic treatment

Age 7-8 years - Alveolar bone graft

18 years old or skeletal maturity– Midface advancement and continued orthodontic treatment

Pre surgical Wide cleft lip or premaxilla

protrusion

Advantageous to narrow the cleft and mold thepremaxilla before proceeding with surgery

Taping

Effective in reducing the width of the cleft it is placed with tension across the cleft

Moulds bony tissues by applying pressure to protruding portions of the maxilla

Must be worn 24 hours per day

Pre surgical Nasoalveolar molding devices

Custom made devices which utilize wiring and nasal stenting to mold the nasal cartilage, premaxilla, and alveolar ridge

Nasal stenting can be elongated and adjusted to lengthen the columella and mold the nasal cartilage

Takes advantage of the malleability of nasal cartilage

Pre surgical Lip adhesion Considered when adhesive

tapes and moulds are ineffective

Surgically convert a complete cleft to an incomplete cleft

Performed at 2-4 weeks with definitive repair at5-6 months

Indications

Wide unilateral cleft where conventional repairmight produce excessive tension

Bilateral cleft – premaxilla protrusion

Disadvantages – scar tissue

Cleft lip repair

Typically performed at 3 months of age

“Rule of Tens”

10 weeks old, 10 lbs, and hemoglobin of 10

Wide clefts or clefts with premaxilla protrusion that require lip adhesions will have definitive lip repair at 5-6 months of age

Cleft lip repair Milliard rotation-advancement

technique widely used Advantages Places scar along philtral

borders Allows complete muscular repair

and primary cleft rhinoplasty Minimizes wasting of normal

tissue

DisadvantageExtensive undermining

Millard rotation and advancement

9 landmarks are marked using blue dye

Rotation flaps cut first(medially), followed by advancement flaps(laterally)

Downward and lateral rotation of medial segment and medial advancement of the lateral segment

Closed in 3 layers

Millards rotation and advancement Goals

Tensionless closureReapproximation of orbicularis orisFormation of Cupid’s bowCreation of a philtrumRepositioning of nasal ala

ComplicationsHypertrophic scarring

Aggressive massageNostril stenosis on cleft side

Modified delaire procedure Avoids crossing aesthetically

sensitive areas, such as the columella base and alar rim unlike Millards procedure

Incorporation of wavy lines and small triangular flaps above the vermillion allow some degree of lengthening.

important contribution that the restoration of labiomaxillary muscle fnction makes towards achieving satisfactory lip length

Bilateral cleft lip repair Technically challenging

GoalsSymmetryOrbicularis oris closureProper philtral size and shapeTubercle formationPositioning of alar cartilages to construct the nasal tipand columella

Bilateral cleft lip more severe deformity than unilateral.

Signifcant degree of hypoplasia in the midline prolabium

characteristic feature of complete bilateral clefs is the marked projection of the prolabium and premaxilla,

Alveolar cleft repair There are two main ways in which this can be

achieved. Secondary alveolar bone grafting at age 9 to 11 years preceded by primary soft-

tissue closure with a vomer flap at the time of primary lip repair, although the alveolar cleft may be left unrepaired at the time of primary surgery.

Primary gingivo-periosteoplasty (GPP) at either the time of palate closure or delayed until the age of three to five years.

Incision design for alveolar bone grafting

Cleft palate repair Primary goals

Separate the nasal cavity from the oral cavityCreation of velopharyngeal valve for swallowing and speechPreservation of midface growth

Timing (Controversial ) Speech outcomes improved with early closure

Midface growth maybe hindered by early closure (2 stage palate repair)

Most repaired between 8-12 months of age to minimize speech abnormalities

Earlier the detrimental effect that palatal surgery has on maxillary growth.

Old Traditional methods Langenbeck flap Veau flap, Veau-Wardill-Kilner Not practised now Scars with exposed bone in the hard palate,

have the potential to inhibit both anterior and transverse growth of the maxilla.

Modifcations of the Langenbeck flap have been introduced whereby the flap is designed inside the greater palatine pedicle in order to move the area of denuded palatal bone

Cleft palate repair

Surgical techniques

Bardach two flap palatoplasty

Furlow double opposing z-plasty

Bardach Palatoplasty Margins of the cleft are cut

separatingthe nasal and oral mucosa

Lateral incisions are made to the boneand carried posteriorly

Mucoperiosteal flaps are elevated Care must be taken to avoid

damage tothe neurovascular bundle

The abnormal muscular attachmentsare raised from the hard palate

Bardach Palatoplasty

Closure occurs in 3 layers

Nasal mucosal

Muscular layer

Oral Mucosal

Bardach Palatoplasty outcomes Keys to optimizing outcomes

Aggressive repositioning of the soft palate musclesAdequate mobilization of flaps to create a tensionless closureComplete palatal closure in one setting

Complications

Fistula3.9 – 29% (anterior hard palate and the soft/hard palate junction)Maxillary growth disturbanceVPI

Furlow Double Opposing Z-plasty

Oral mucosa with muscle is raised on the left from the nasal mucosa and elevated to the posterior

Oral mucosal flap is raised on the right (oral mucosa and submucosa is raised from the muscle) and elevated anteriorly

Nasal mucosa/muscle flap is raised on the right and elevated posteriorly

Nasal mucosal flap is raised on the left and raised anteriorly

4 flaps (2 anterior mucosal flaps and 2 posterior mucomuscular flaps)

Furlow Double Opposing Z-plasty Lower flaps with

nasal mucosa are transposed

Upper flaps with oral mucosa are transposed

Modified for clefts involving the hard palate

Furlow Double Opposing Z-plasty Closure of soft palate

and reconstruction of levator sling

Advantages

Repositions musculature into a more anatomical locationLengthens the palate with potential for improved speech outcomes

DisadvantagesPostoperative airway obstructionFistula formation – wide cleftsTechnically challenging

Take home message

One of the commonest congenital treatable anomaly

Current operative techniques offer excellent outcome

Multi disciplinary approach required

Joaquin Cannes (1974-American Actor

Thank you