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Cleft Lip & PalateCleft Lip & Palate
Aetilogy,Pathogenesis and Classification
NEETHU SALAMFINAL YR(PART 1)
EMBRYOLOGYDuring the fifth week, two fast-growing
ridges, the lateral and medial nasal swellings, surround the nasal vestige.
The lateral swellings will form the alae of the nose; the medial swellings will give rise to four areas:
(1) the middle portion of the nose, (2) the middle portion of the upper lip,
(3) the middle portion of the maxilla, (4) the entire primary palate.
Simultaneously the maxillary swellings will approach the medial and lateral nasal swellings but remain separated from them by well marked grooves.
During the next 2 weeks, the appearance of the face changes considerably. The maxillary swellings continue to grow in a medial direction and compress the medial nasal swellings toward the midline. Subsequently these swellings simultaneously merge with each other and with the maxillary swellings laterally. Hence the upper lip is formed by the two medial nasal swellings and the two maxillary swellings.
The two medial swellings merge not only at the surface but also at the deeper level. The structures formed by the two merged swellings are known together as the intermaxillary segament which is comprised of three components:
(1) a labial component, which forms the philtrum of the upper lip;
(2) an upper jaw component, which carries the- four incisor teeth; and
(3) a palatal component, which forms the triangular primary palate.
Two shelflike outgrowths from the maxillary swellings form the secondary palate. These palatine shelves appear in the sixth week of development and are directed obliquely downward on either side of the tongue. In the seventh week, however, the palatine shelves ascend to attain a horizontal position above the tongue and fuse with each other, thereby forming the secondary palate. Anteriorly the shelves fuse with the triangular primary palate, and the incisive foramen is formed at this junction.
PATHOGENESIS
Clefts of the primary palate result from a failure of mesoderm to penetrate into the grooves between the medial nasal and maxillary processes, which prohibits their merging with one another.
Clefts of the secondary palate are caused by a failure of the palatine shelves to fuse with one another. The causes for this are Speculative and include failure of the tongue to descend into the oral cavity.
Clefts of the lip, alveolus, hard and soft palate are the most common congenital abnormalities of the orofacial structures.
They frequently occur as isolated deformities but can be associated with other medical conditions particularly congenital heart disease.
They are also an associated features in over 300 recognized syndromes.
All children born with a cleft lip and palate need careful pediatric assessment to exclude other congenital abnormalities.
IncidenceIncidence
Incidence of cleft lip/palate is 1:600 live births.
1:1000 live births for isolated cleft palateThe highest incidence occurs in native
Americans and occurs less frequently in African Americans.
Typical distribution of cleft types is: • cleft lip alone 15 % • cleft lip & palate 45 % • isolated cleft palate 40 % Cleft lip/palate predominate in males Cleft palate alone appears more
common in females
EtiologyEtiology
Cleft lip/palate and isolated cleft palate have a genetic predisposition and environmental component
Studies have been able to implicate genetics in only 20% to 30% of cleft lip or palate patients
The majority of nonsyndromic clefts appear to be caused by an interaction between the individual's genes (i.e., genetic predisposition) and certain factors In the environment that may or may not be specifically identified
AETIOLOGY
1.Maternal infection and toxicity Infection-rubella,influenza
Toxicity-hypoxia,teratogenic drugs like valium,phenytoin,corticosteroids,cytotoxic drugs like nitrogen mustard.
2. Maternal dietary imbalace Folic acid deficiency diet Hypervitaminosis A
3.Maternal hormone imbalance at the time of conception
HEREDITYMultifactorial inheritance plays a major role.Familial
inheritance occurs ,but classic Mendelian inheritance is rare.
Unaffected parents who have a child with a cleft lip and palate have a 4.4% chance that their second child will also have a cleft of the lip or palate and a 2.5%chance that their second child will have an isolated cleft palate.
If one parent has a cleft deformity,there is a 3.2%chance that their first born will have a cleft lip or palate.
SYNDROMES ASSOCIATED WITH CLEFT PALATE
PIERRE POBIN SYNDROME APERT’S SYNDROME PATAU’S SYNDROME TREACHER COLLIN’S SYNDROME EDWARDS SYNDROME
The symptoms of these abnormalities are visible during the first examination after birth.
prenatal ultrasounds can detect a cleft lip or palate prior a child's birth.
By detecting the cleft abnormality during a pregnancy, the expecting parents can have a prenatal consultation with a plastic surgeon.
CLINICAL PROBLEMS ASSOCIATED WITH CLEFTS
DENTAL ABERRATIONS
PRESENCE OF NATAL OR NEONATAL NEONATAL TEETH SUPERNUMERARY OR MISSING TEETH ECTOPICALLY ERUPTING TEETH MICRODONTIA,MACRODONTIA,FUSED TEETH MOBILE TEETH WITH POOR PERIODONTAL SUPPORT POSTERIOR AND ANTERIOR CROSSBITES MALALIGNMENT OF ALVEOLAR ARCHES
Aesthetic and growth problems
Concave profile with midface deficiencyHypoplastic maxilla on the cleft sideShortened columella on the cleft sideFloor of nose communicates freely with
the oral cavityWidened and flattened nasal tip
feeding difficultiesFeeding difficulties occur more with cleft palate abnormalities. The infant may be unable to suck properly because the roof of the mouth is not formed completely.
ear infections and hearing lossEar infections are often due to a dysfunction of the tube that connects the middle ear and the throat. Recurrent infections can then lead to hearing loss.
speech and language delayDue to the opening of the roof of the mouth and the lip, muscle function may be decreased, which can lead to a delay in speech or abnormal speech. (hypernasal speech) due to Velopharyngeal incompetence.
Airway Problems • Hypoxic episodes during sleep and feeding are
life threatening Airway problems may arise in children with cleft
palates.For eg:Pierre Robin sequence is the combination of micrognathia,cleft palate,and glossoptosis.Affected patients may develop airway distress from their tongue becoming lodged in the palatal defect.
Psychological problems Due to poor social image due to poor
facial aesthetics.Psychological counselling may be
necessary.
MORPHOLOGIC CLASSIFICATIONMORPHOLOGIC CLASSIFICATION
1.CLEFT LIP2.CLEFT LIP AND PALATEISOLATED CLEFT PALATE
KERNOHAN AND STARK’S KERNOHAN AND STARK’S CLASSIFICATIONCLASSIFICATION
I.CLEFTS OF PRIMARY PALATE ONLY UNILATERAL COMPLETE INCOMPLETE MEDIAN BILATERAL II.CLEFTS OF SECONDARY PALATE ONLY COMPLETE INCOMPLETE SUBMUCOUSIII.CLEFTS OF PRIMARY AND SECONDARY PALATE UNILATERAL COMPLETE INCOMPLETE BILATERAL MEDIAN
VEAU’S CLASSIFICATION(1931)VEAU’S CLASSIFICATION(1931)
Of cleft palateGroup I-cleft of the soft palate only.Group II-cleft of soft and hard palate not
involving the lips.Group III-Unilateral complete cleft lip and
palate involving soft and hard palate,alveolar ridge and lip.
Group IV-Bilateral complete cleft lip and palate.
Of cleft lip Class I-A unilateral notching of the vermilion not extending into
the lip. Class II-A unilateral notching of the vermilion border,with the cleft
extending into the lip but not including the floor of the nose. Class III-A unilateral clefting of the vermilion border of the lip
extending into the floor of the nose. Class IV-Any bilateral clefting of the lip,whether it be incomplete
notching or complete clefting.
Davis and Ritchie classification(1922)Davis and Ritchie classification(1922)
Group I- Pre alveolar clefts(clefts involving lip only)
Group II-Post alveolar clefts(submucous cleft)
Group III-Alveolar clefts(complete clefts) Unilateral,Medaian,Bilateral
Blocks 1 and 4-LipBlocks 2 and 5-AlveolusBlocks 3 and 6-Hard Palate anterior to the
incisive foramen-premaxillaBlocks 7 and 8-Hard palate behind incisive
foramenBlock 9-Soft palate The blocks were clefts are present are
shaded.
TREATMENT OF CLEFT LIP AND TREATMENT OF CLEFT LIP AND PALATEPALATE
Cleft team•Plastic surgeon•Paediatrician•Paedodontist•Orthodontist•Speech pathologist•Otologist•Audiologist•Geneticist•Nurse•Psychiatrist•Social worker•Prosthodontist
I.Neonatal intervention and I.Neonatal intervention and nursing carenursing care
•Early contact and counselling of parents•First contact of the cleft team with the patient and parents•Instructions on breast feeding should be given
II.3-6 weeksII.3-6 weeks
Infant orthopedics Light elastic strapsacross
premaxillary segment Orthodontic appliances pinned to the
segmentsFeeding Plate can be left in plates for 1yr.
III.2-3 MONTHSIII.2-3 MONTHS
Cleft lip repair is commonly performed between 3-6 months of age (rule of ten 10g/dl, 10 pounds, 10 weeks )
Various methods Lip adhesion procedure Rose-Thompson procedure Millard rotation advancement flap
Hearing: all children should undergo assessment before 12 months for sensorineural and conductive hearing loss
12-18 MONTHS12-18 MONTHS
Pedodontic review Assessment by speech pathologist The advantages for early closure(prior to 18 months) of palatal defects
are (1) better palatal and pharyngeal muscle development once repaired, (2) ease of feeding, (3) better development of phonation skills (4) better auditory tube function, (5) better hygiene when the oral and nasal partition is competent, and (6) improved psychologic state for parents and baby
The disadvantages of closing palatal clefts early in life are also several: The two most important are
(1) surgical correction is more difficult in younger children with small structures, and
(2) scar formation resulting from the surgery causes maxillary growth restriction.
Methods of cleft surgeryMethods of cleft surgery
Bernard von Langenbeck’s palatal closure by elevation of mucoperiosteal flaps.
V-Y pushback palatoplasty.
V.2-6yrsV.2-6yrs
Pedodontic review to monitor growth and development
Oral hygiene instructionReview appointments with plastic
surgeon,audiologist and speech pathologist.
VII.6-8yrsVII.6-8yrs
Fissure sealing of primary molars on eruption.
Removal of deciduous or supernumeraru teeth
Restoration of hypoplastic incisors adjacent to the cleft
Palatal expansion proceduresFcemask maxillary protraction
VIII.12-13yrsVIII.12-13yrs
Comprehensive orthodontic treatmentPreadjusted edgewise appliances are
preferred.
Use of chin cup to redirect maxillary growth.
IX.14-16yrsIX.14-16yrs
Maxillary protraction by orthognathic surgery
Maxillary distraction osteogenesis