Cleft Lip & Palate

Aetilogy,Pathogenesis and Classification

NEETHU SALAM FINAL YR(PART 1)

 EMBRYOLOGY

During 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.

Incidence of cleft lip/palate is 1:600 live births. 1:1000 live births for isolated cleft palate The highest incidence occurs in native Americans and occurs less frequently in African Americans.

Incidence

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

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

Etiology

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

HEREDITY

Multifactorial 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 APERTS SYNDROME PATAUS SYNDROME TREACHER COLLINS 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 deficiency Hypoplastic maxilla on the cleft side Shortened columella on the cleft side Floor of nose communicates freely with the oral cavity Widened and flattened nasal tip

feeding difficulties Feeding 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 loss Ear 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 delay Due 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.

CLASSIFICATION OF CLEFT LIP AND PALATE

1.CLEFT LIP 2.CLEFT LIP AND PALATE ISOLATED CLEFT PALATE

MORPHOLOGIC CLASSIFICATION

KERNOHAN AND STARKS CLASSIFICATION

I.CLEFTS OF PRIMARY PALATE ONLY UNILATERAL COMPLETE INCOMPLETE MEDIAN BILATERAL II.CLEFTS OF SECONDARY PALATE ONLY COMPLETE INCOMPLETE SUBMUCOUS III.CLEFTS OF PRIMARY AND SECONDARY PALATE UNILATERAL COMPLETE INCOMPLETE BILATERAL MEDIAN

Of cleft palate Group 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.

VEAUS CLASSIFICATION(1931)

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.

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

Davis and Ritchie classification(1922)

Kernohans stripped Yclassification

Blocks 1 and 4-Lip Blocks 2 and 5-Alveolus Blocks 3 and 6-Hard Palate anterior to the incisive foramen-premaxilla Blocks 7 and 8-Hard palate behind incisive foramen Block 9-Soft palate The blocks were clefts are present are shaded.

Classification of cleft lip

Cleft team
Plastic surgeon Paediatrician Paedodontist Orthodontist Speech pathologist Otologist Audiologist Geneticist Nurse Psychiatrist Social worker Prosthodontist

TREATMENT OF CLEFT LIP AND PALATE

Early contact and counselling of parents First contact of the cleft team with the patient and parents Instructions on breast feeding should be given

I.Neonatal intervention and nursing care

Infant orthopedics
Light elastic strapsacross premaxillary segment Orthodontic appliances pinned to the segments

Feeding

Plate can be left in plates for 1yr.

II.3-6 weeks

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

III.2-3 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.

12-18 MONTHS

Bernard von Langenbecks palatal closure by elevation of mucoperiosteal flaps. V-Y pushback palatoplasty.

Methods of cleft surgery

Pedodontic review to monitor growth and development Oral hygiene instruction Review appointments with plastic surgeon,audiologist and speech pathologist.

V.2-6yrs

Fissure sealing of primary molars on eruption. Removal of deciduous or supernumeraru teeth Restoration of hypoplastic incisors adjacent to the cleft Palatal expansion procedures Fcemask maxillary protraction

VII.6-8yrs

Alveolar bone grafting

VII.9-11yrs

Comprehensive orthodontic treatment Preadjusted edgewise appliances are preferred.

Use of chin cup to redirect maxillary growth.

VIII.12-13yrs

Maxillary protraction by orthognathic surgery Maxillary distraction osteogenesis

IX.14-16yrs

References
Contemporary orthodontics-William. R .Proffit Gowri Shankar-Text book of orthodontics Sridhar Premkumar-Orthodontics text book

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