cleft lip and cleft palate embryology, features, and management
TRANSCRIPT
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