Transcript Congenital malformations

Congenital
malformations
Be not the first whom the new is tried Nor
yet the last whom the old is cast aside.
The secret of success is constant to
purpose.
Common congenital
lesions of the oral cavity
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Cleft lip,
Cleft palate, and
Ankyloglosia (Tongue tie)
Ankylosis (not true congenital problem)
Topics to be covered
 Cleft lip and palate
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Embryology
Etiologic factors
Classification
Problems of cleft
Treatment of clefts
Dental needs of cleft afflicted patients
 Prosthetic speech aid appliances
Topics
 Problems of the cleft
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Dental problems
Nasal deformity
Feeding
Ear problems
Speech difficulties
Associated anomalies
Topics
 Treatment of clefts
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Timing of cleft repair
Cheilorrhaphy
Palatorrhaphy
Alveolar cleft grafts
Correction of maxillomandibular
disharmonies
 Secondary surgical procedures
Introduction
 A cleft is a congenital space or gap in the
upper lip, alveolus or palate.
 Cleft lip also known as “hare lip”.
 Cleft can be on
 Lip only
 Unilateral or bilateral
 Palate only
 Complete or incomplete
 Unilateral or bilateral
 Or both lip and palate
Classification
 Cleft lip alone
 Unilateral (one side only)
 Bilateral (both sides)
 Cleft palate alone
 Complete
 Incomplete
 Cleft lip and palate
 Complete
 Incomplete
Classification cont…
 Useful classification divides palate into;
 Primary palate
 Structure anterior to the incisive foramen.
 Pre-maxilla (lip and alveolus)
 Secondary palate
 Those structure posterior to the incisive foramen
 Hard and soft palate
 Cleft of the soft palate alone- bifid uvula.
Epidemiology
 Occurrence of clefts in US is 1 in every
600 to 1000 live births.
 Clefts exhibit racial predilections
 Occur less frequently in blacks, more in
orientals.
 Boys affected more often than females In
ratio of 3:2.
 Cleft of lip more frequently in boys
Epidemiology cont…
 Whereas isolated cleft palates are more
common in in girls.
 Oral clefts commonly affects lip, alveolar,
hard and soft palate.
 Three fourth are unilateral clefts.
 One fourth are bilateral clefts.
Embryology
 Face develop around shallow ectodermal
depression (stomatodeum).
 At first stomatodeum closed by the
bucopharyngeal membrane which ruptures the
3rd week of embryonic life.
 Stomatodeum is surrounded on all sides by
mesodermal swellings
 Unpaired frontal prominence (process)
 Paired maxillary prominence
 Paired mandibular prominence.
Embryology cont…
 Bilateral nasal placodes develop in the
ectoderm covering the frontal process above
the stomodeum.
 Frontal process divided into medial and lateral
nasal processes as nasal placodes appear.
 5th week the nasal placodes become flanked
by these medial and lateral nasal processes.
Embryology cont…
 Nasal pit formed when the swelling unite above
and below the placode.
 Medial processes meet each other and fuse
forming intermaxillaty segment.
 Intermaxillary segment will for;
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Middle portion of the nose
The philtrum of the upper lip
Premaxilla (for 4 incisors)
Primary palate
Embryology cont…
 Lateral processes will form the alae of the nose.
 Nasal pit are temporary continuous ventrally with the
stomodeum.
 But are closed off again as the medial frontonasal and
maxillary processes unite beneath them.
 Furrow between the lateral frontonasal swellings and
maxillary swellings is continuous laterally with that
maxillary and frontomedial swellings and run from
upper lip to the region of the developing eye.
 This is the nasolacrimal groove.
 It is eventually bridged by the processes on each side
to form part of the nasolacrimal (tear) duct.
Embryology cont…
 Primary palate
 Form from the intermaxillary segment.
 Secondary palate
 Originates from as shelf like outgrowths
which appear 6th week from deep surface of
each maxillary processes.
 When first formed these palatine processes
are directed medially and downwards on
either sides of the developing tongue.
Embryology cont…
 The processes reorientate themselves
horizontally starting posteriorly so that they
approach each other above the tongue.
 Eventually fusion occurs;
 Between the free edges of the palatine processes
 Between each process and the free edge of the
primary palate anteriorly
 Between the newly formed secondary palate and
the inferior margin of the nasal septum.
Embryology cont…
 Palate is usually completed by 60 days.
 The point of union of the apex of the
primary palate and the two processes of
the secondary palate is marked by the
incisive foramen.
Clinical aspects of
embryology
 Failure of fusion of maxillary processes –
cleft lip.
 Failure in fusion of secondary palatal
processes gives cleft palate.
 Cleft of lip and premaxilla gives “hare lip”
Etiological factors
 Hereditary is contributary factor
 Environmental factors play contributory role at
critical time of embryologic development when
the lip and palatal halves are fusing.
 Nutritional deficiencies
 Radiation
 Several drugs
 Hypoxia
 Vitamin excess and defiencies.
Problems of the cleft
 Cleft of alveolus affect development the
primary and secondary dentition and jaw itself.
 Most common problem is absence of teeth.
 Usually between lateral incisor and canine.
 Malocclusion
 Skeletal discrepancy between size, shape and
position of teeth.
 Class III malocclusion in most cases.
 Mandibular prognathism most common finding,
caused by maxilla retrusion than mandibular
protrusion (Pseudoprognathism).
Problems of the cleft
cont…
 Nasal deformity
 If clefts extend from into the floor of the nose, alar
cartilage on that is flared,
 columella of the nose is pulled towards the non cleft
side
 There is lack of underlying bony support to the base
of the nose.
 Feeding
 Difficulty of producing negative pressure during
sucking either breast or bottle milk.
 But baby can swallow normally once milk fed
reaches the hypopharynx.
Problems of the cleft
cont…
 Ear problems
 Middle ear infection is common in children with cleft
of the palate.
 Review anatomy of soft palate musculature (tensor
veli palatini and levator veli palatini) are inserted into
same muscles opposite side,
 And have origins directly or near auditory tube.
 The muscles allow opening of the ostium of this
tube.
 Without action of this muscles the ostium is closed –
no drainage mechanism, serous fluid accumulate.
Problems of the cleft
cont…
 Speech difficulties
 Created by cleft of lip and palate.
 Retardation of consonant sounds (p,b,t,d,k and g).
 Hypernasality is usual in cleft of nasal palate.
 Associated anomalies
 Associated congenital problems can be clubfoot to
neurological disturbances (30%).
 Congenital heart disease (10%)
 Mental retardation (10%), etc.
Treatment
 Cleft lip and palate
 Aim of repair is to correct the cleft and
associated problems, thus remove the
anomally so that patient can live normal life.
 Surgical correction of deformity
 Vocal apparatus that permit intelligible speech
 Dentition that allows optimal function and
esthetics
Treatment cont…
 Timing of surgical repair
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Cleft lip (rule of 10).
Cleft lip repaired earlier than palate.
Exact time still debatable.
Rule of 10- health baby is fit for surgery.
 10 weeks of age
 10 pounds in body weight
 At least 10 g/dl of Hb.
 Soft palate cleft closed between 18 and 24 moths of
age.
 Hard palate cleft is closed between in preschool age
that is between 4 and 5 years.
Treatment cont…
 Cheilorrhaphy is the surgical correction of the cleft lip
deformity.
 Objectives;
 Functional
 Aesthetics
 Palatorrhaphy is the surgical correction of palate.
 May be performed in one or two operation
 In two operation staphylorrhaphy (soft palate closure) is
usually performed first and
 Hard palate closure (Uronorrhaphy) is performed second.
Treatment cont…
 Objective; to create mechanism capable
of speech and deglutition without
significantly interfering with subsequent
maxillary growth.
 Alveolar cleft grafts
 Alveolar cleft defects not corrected in the
original surgical correction.
 Alveolar cleft bone graft provide several
advantages
Treatment cont…
 Advantages of alveolar cleft graft
 Unite alveolar segments and help prevent
collapse and constriction of the dental arch.
 Provide bone support for teeth adjacent to
the cleft and for those that will erupt into
area of cleft.
 Closure of the oroantral fistulla
Correction of maxillomandibular
disharmony
 People with cleft exhibit maxillary
retrusion and transverse maxillary
constriction resulting from the cicatricial
contraction of previous surgeries.
 Secondary surgical procedures are
performed after the initial repair of the
clefts defects in an effort to improve the
speech or correct residual defects.
Dental needs of cleft
affected patients
 Placing a denture to replacing
congenitally missing lateral incisor before
alveolar ridge graft.
 Frequent prophylaxis and special oral
hygiene instructions with carefully
reinforcement, because of the malformed
or absent teeth, sometimes are in the
cleft, predisposing them to periodontitis.
Dental needs of cleft
affected patients cont…
 Prosthetic speech appliances
 Teeth missing should be replaced.
 Patients who have failed to obtain
velopharyngeal competence need speech
appliance to decrease hypernasal speech.
 Treating clefts is as
playing chase, think,
plan, see and do as
you see.
 Remember when you
hear you forget,
when you see you
remember, and when
you do you
understand.