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.