Dr Sneha khadkaMScD Orthodontics.

CLEFT LIP AND PALATE

CONTENTS Introduction Incidence Classification Embryological Background Etiology Diagnosis Problems of individuals with clefts Treatment of Cleft Lip and Palate Specialties involved in the treatment of Cleft Lip and Palate Timings of surgical repair Cheilorraphy Palatorrhaphy Alveolar Cleft Grafts Secondary Surgical Procedures Valopharyngeal Insufficiency

INTRODUCTION

What is Cleft Lip and Palate?

Congenital abnormal space or gap in the upper lip, alveolus and palate.

INCIDENCE

More common in south far Asians:1 in 500

Less frequent in Africans:1 in 2000

Prevalence in Europeans and Americans:1 in 750

Boys are more affected than girls by 3:2Cleft Lip and Palate occur twice as often in boys as in girls.Isolated Clefts of Palate are more often in girls.75% of Clefts are Unilateral, rest are Bilateral.Left side is more involved than right side

Head and Neck of 4-Week Old Embryo

Trigeminal nerve

Facial nerve

Glossopharyngealnerve

Vagus nerve

EMBROYOLOGICAL BACKGROUND

Development of the Lip:Unpaired Frontonasal

ProminenceMedial and Lateral Nasal

prominences 2 maxillary prominences2 mandibular prominences

Fusion defects can occur anywhere between these prominencesThe defect in the fusion between the frontonasal and maxillary will

lead to cleft lip

Development of Palate:

We have two parts of two different embryonic origins:

1 ) primary palate : the triangular part of hard palate anterior to incisor foramen which originate from the premaxilla ( frontonasal prominences).

-develop between 4-develop between 4 thth and 8 and 8thth week of gestation week of gestation 2 ) secondary palate : remaining part of the hard palate and all

soft palate posterior to incisor foramen which comes from palatine shelves of the maxillary prominences

-develop between 8-develop between 8 thth and 12 and 12 thth week of gestation week of gestation

Various theories have been given for its development.

1. Alteration in intrinsic palatal shelf force .

2. Failure of tongue to drop down.

3. Non fusion of shelves.

4. Rupture of cyst formed at the site of fusion.

CLASSIFICATION

Early attempts at classification by Davies & Ritchie in 1922.

Group I : prealveolar clefts- unilateral cleft lip. - bilateral cleft lip. - median cleft lip. Group II : post alveolar clefts – cleft hard palate - cleft soft palate - cleft hard and soft palate. - submucosal clefts.

Group III: alveolar clefts- unilateral

- bilateral

- median

Veau in 1931 Group I : clefts of soft palate only. Group II : clefts of hard and soft palate extending upto the incisive foramen. Group III: complete unilateral clefts involving soft palate, hard palate, alveolar ridge and lip on one side. Group IV: complete bilateral clefts of soft and hard palates, alveolar ridge and the lip.

Fogh-Anderson in 1942 studied incidence of CL(P) in Denmark

a. Hare lip including alveolus as far back as incisive foramenb. Hare lip & CPc. Isolated clefts of palate as far forward as incisive foramen

KERNAHAN AND STARK’S CLASSIFICATION OF CLEFTS (1958)

Clefts of primary palate onlyUnilateral (right or left)

Complete Incomplete

Median Complete (premaxilla

absent) Incomplete (premaxilla

rudimentary) Bilateral

Complete Incomplete

Clefts of secondary palate onlyComplete Incomplete Submucous

Clefts of primary and secondary palate

Unilateral (right or left) Complete Incomplete

Median Complete Incomplete

Bilateral CompleteIncomplete

KERNAHAN STRIPED Y CLASSIFICATION ( 1971)

LIP

ALVEOLUS

PRIMARY PALATE

HARD PALATE

SOFT PALATE

INCISIVEFORAMEN

MODIFIED STRIPED Y ELSHAY CLASSIFICATION ( 1973)

13

12

R L

1,5- Fusion line between the maxillary prominence and medial nasal prominence at nostril floor level.

2,6- Fusion line at the level of upper lip.3,7- R/L alveolus . Fusion line between the maxillary prominence

and intermaxillary segments.4,8- Fusion between the primary and secondary hard palate.9,10- Fusion between the palatal shelves of hard palate.11- Represents the soft palate.12- Posterior pharyngeal wall.13- Premaxilla.

MILLARD’S MODIFICATION OF KERNAHAN’S CLASSIFICATION

Kriens “LAHSHALKriens “LAHSHAL ””L = Lip (right)A = Alveolus (right)H = Hard Palate (right)S = Soft Palate (median)H = Hard Palate (left)A = Alveolus (left)L = Lip (left)

Capital letter = complete cleftLowercase letter = incomplete cleft“.” or “-” = normal

ExamplesLA….l = complete right cleft lip and alveolus, incomplete left cleft lipLAHS = complete right unilateral cleft lip, alveolus, hard, and soft palate

Microform Cleft:May look like

-a little dent in the red part of the lip. -a scar from the lip up to the nostril.

Muscle tissue underneath the cleft can be affected and may require surgery

Submucous Cleft Palate:Midline deficiency or lack of muscular

tissueOften a submucous cleft palate is

associated with a bifid or cleft uvulaPosterior nasal spine is almost always

missingSpeech Problems are common

ETIOPATHOGENESIS

Clefts of lip and palate may be isolated deformities or may be part of a syndrome.

Non syndromic clefts are multifactorial in origin.

Multiple factors may be involved, like--Genetics (inherited characteristic) from one or both parents .Environmental factors.-Drugs: corticosteroids (anti-inflammatory), phenytoin

(anticonvulsant), retinoid.-Infections: like rubella during pregnancy,viral infections.-Alcohol consumption, smoking, hypoxia during pregnancy, some of

dietary and vitamins deficiencies (like folic acid and vitamin A deficiency),stress, mechanical disturbances.

Maternal Age.

DrugsPhenytoin

Maternal illnesssmoking Alcohol

Polygenicinheritance

Geneticpredisposition

Non syndromicclefts

Some of the more common syndromes associated with CL/P

ChromosomalTrisomy 13 Trisomy 18 Velocardiofacial syndrome

(22q11 deletion) Non-Mendelian

Pierre Robin syndrome CHARGE association Goldenhar syndrome

TeratogenicFetal alcohol syndrome Fetal phenytoin syndrome Fetal valproate syndrome

Mendelian disorders Ectrodactyly-eetodermal

dysplasia-clefting syndrome (AD)

Gorlin syndrome (AD) Oto-palato-digital syndrome

(XL) Oral-facial-digital syndrome

(XL) Smith-Lemli-Opitz syndrome

(AR) Stickler syndrome (AD) Treacher Collins syndrome

(AD) Van der Woude syndrome

(AD) Unknown

de Lange syndrome Kabuki syndrome

PROBLEMS ASSOCIATED WITH CLEFT LIP AND PALATE

FeedingDental problemsNasal Deformity and Esthetic ProblemsEar ProblemsSpeech DifficultiesAssociated Anomalies

FEEDING DIFFICULTIES

Cleft lip= makes it more difficult for an infant to suck on a nippleCleft Palate= may cause formula or breast milk to be accidently

taken up into the nasal cavityInability to create negative pressure inside oral cavityFrequent regurgitationsUpper respiratory tract infections

DENTAL PROBLEMS

Local Dental Problems:Congenitally Missing teeth, Hypodontia, Hyperdontia,

OligodontiaPresence of natal and neonatal teethAnamalies of tooth morphology like microdontia, macrodontia

etcFused teethEnamel HypoplasiaPoor periodontal support, early loss of teethGemination, Dilacerations

Orthodontics Problems:Class III tendencyAnterior and Posterior Cross biteSpacing and crowding

NASAL DEFORMITY AND ESTHETIC PROBLEMS

Facial Disfigurements.Poor nasal shape.Scar marks of surgeries.Poor lip function during speech.Poor dental alignment and smile.

EAR PROBLEMS

Middle ear disease - 22% to 88% .Conductive hearing loss and chronic suppurative otitis media may

result. Repeated tympanostomy tube placement.

Speech Problems:Hearing loss hampers proper development of speechVelopharyngeal Insufficiency.Poor pronunciation of Bilabial, Labiodental, Linguoalveolar

sounds

PRENATAL/POSTNATAL DIAGNOSIS

Intrauterine - Sonographic diagnosis- Karyotyping

Post partum - Clinical- Radiographic (intra and

extraoral, laminography, pantography)

- Additional aids (speech and sound recording, measurement of nasal and oral pressures, psychiatric examination)

PRENATAL, PERINATAL AND POSTNATAL PARENTAL COUNSELLING

Requisites Accurate information should be given by an expert in the fieldSupport must be available soon after diagnosisParents and members of family given opportunity to express

concern and emotional response

Give a clear view of how the baby is likely to look.Discussion on pregnancy termination based on accurate

information .Promote early adjustment and acceptance of the baby by the

parents and family.Long term dependence on counselor should be avoided and

normalization of family life should be encouraged.