Cleft lip & palate.ppt

3,959 views 36 slides Apr 29, 2023
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About This Presentation

Cleft lip & palate for MBBS sudents


Slide Content

Cleft Lip
&
Cleft Palate
Dr Sushil Dhungel
Ast. Professor
KIST MCTH

Def:
Congenital abnormal space or gap in the
upper lip, alveolus and palate
Cleft lip and palate is result from failure of
embryonic processes

ETIOLOGY (Multifactorial)
•Genetic factors (% 25-40)
Otozomal ressesive
•Environmental factors(% 60-75)

Genetic Factors
(autosomal Ressesive)
•First child has anomaly
•Probability of second child % 4
•Probability of third child % 10
•Two childern have anomaly
•Probability of third child % 20
•Mother or Father has anomaly
•Probability of first child % 5
•Both mother and father have anomaly
•Probability of first child % 25

Environmental factors(% 60-75)
•Mothers sickness during first trimester (viral infections)
•Cronic diseases ( Diabetes Mellitus etc.)
•Drugs (tranquilizer, hipnotics, sedatives, cortizon, etc)
•Smoking
•Alcohol
•Aspirin
•X Rays

Associated Syndromes
•identified in >300 syndromes,
•most of which are rare.
•Strickler syndrome (ophthalmic and
musculoskeletal abnormalities)
•Down’s syndrome
•Apert’s syndrome
•Pierre Robin’s Syndrome

Head and Neck of 4-Week Old Embryo
Trigeminal nerve
Facial nerve
Glossopharyngeal
nerve
Vagus nerve
Embryological Background

Embryological Background
•Development of the Lip:
–Unpaired Frontonasal Prominence
–Medial and Lateral Nasal
prominences
–2 maxillary prominences
–2 mandibular prominences

Embryological Background
•Fusion defects can occur anywhere between these
prominences
•The defect in the fusion between the frontonasal and maxillary
will lead to cleft lip

Embryological Background
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( frontonasalprominences).
develop between 4
th
and 8
th
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
th
and 12
th
week of gestation

Cleft lip:
◦Caused by incomplete fusion of the nasomedial or intermaxillary process during
the 2
nd
month of embryonic development
◦Cleft causes structures of mouth and face to develop without the normal restraints
of encircling lip muscles
◦May affect external nose, nasal cartilages, nasal septum, and alveolar processes
also
◦Usually just beneath the center of one nostril
◦Can occur bilaterally, symmetrically or asymmetrically
◦More complete the cleft lip, the greater the chance that teeth in the line of the cleft
will be missing or malformed
Complete cleft= entire thickness of the lip
Incomplete cleft= only a portion of the lip is involved

Cleft Palate:
◦Often associated with cleft lip, but may occur without it
◦Fissure may affect only the uvula and soft palate (secondary palate, formed ~ 9 weeks), or
may extend forward to the nostril and involve the hard palate and the maxillary alveolar ridge
(primary palate, formed ~ 4-5 weeks )
◦Complete= involves the primary and secondary palate
◦Incomplete= involves the secondary palate only
◦Unilateral= on one side the palatal process of the maxilla is fused with the nasal septum
◦Bilateral= not attached to the nasal septum, and the septum is visible through the cleft
◦Cleft occupies the midline posteriorly and can go as far forward as the alveolar process. Clefts
involving the palate only are usually midline
◦Some cases, the vomer and nasal septum are partly or completely undeveloped
When these facial bones are involved, the nasal cavity and the oral cavity are open to each other

Classification
•Based on alveolar arcus
(Davies-Ritchie 1922)
•Prealveolar (cleft lip)
•Postalveolar (cleft palate)
•Transalveolar (cleft lip and palate)
•Based on embryologic development
(Kernehan-Stark 1958)
•Primary cleft palate (anterior to incisive
foramen)
•Secondary cleft palate (posterior to incisive
foramen)

•A‐incomplete cleft of the
secondary palate
•B‐Complete cleft of the
secondary palate
•C‐Incomplete cleft of the
primary and secondary palate
•D‐Unilateral complete cleft of
the 1 and 2 palates
•E‐bilateral complete cleft of the
1 and 2 palates
Classification

Classification of cleft lip
WWW.SMSO.CC

NORMAL LIP
MUSCULAR
ANATOMY
CLEFT LIP
ANATOMY

Prenatal Diagnosis
•Cleft lipcan be easily diagnosed
by performing ultrasonography in
the second trimester
•Diagnosing a cleft palate with
ultrasonography is very difficult
•Three-dimensional imaging has
been introduced to prenatal
ultrasonography diagnostics of
cleft anomalies

Diagnosis
•Advantages of Prenatal
Diagnosis:
1.Time for parental education
2.Time for parental
psychological preparation
3.Opportunity to investigate
other associated anomalies
4.Gives parents the choice of
continuing the pregnancy

Problems in Cleft Lip and Cleft Palate
•Feeding difficulty
•Nasal regurtation of food
•Frequent upper respiratory tract
infection(Aspiration pneumenia)
•Frequent gas regurtation
•Otitis media
•Dental problems
•Speech Difficulties
•Associated Anomalies
•Growing retardation
•Psycological problems (family)

Feeding
•Cleft lip makes it more difficult for an infant to suck on a nipple
–use special nipples to allow the baby to latch properly
(either pump or use formula)
•Cleft Palate may cause formula or breast milk to be accidentally
taken up into the nasal cavity
–don’t feed baby without palatal obturator (prosthetic
palate)
–feed in an upright position to keep milk from coming out of
the nose

Feeding Rules
•Swallowing is not impaired, oral feeding is
possible
•Bottle feed with additional cross cut in the end
•Elastic plastic bottle
•Bulb syringe with a nipple
•Feeding with a spoon
•The child should be held in a head-up position at
about 45 º during and after feeding
•Lateral position during sleeping

Mead Johnson/Enfamil Cleft Feeder Special Needs Feeder /
Haberman Feeder
Pigeon Feeder Dr. Brown’s Natural
Flowto relieve gas

When to Operate
Generally (Rules of 10’s)
•Weight > 10 pound (4500 gr)
•Hb > 10 gm/dl
•Age > 10 weeks
Cleft lips between 3-6 months
Cleft palate between 12-18 months (preferred before
speech develops)

Cleft lip and palate treatment team
•Pediatric Surgeon experienced in cleft
management
•Pediatrician
•Orthodontist
•Pediatric Otorhinolaryngologist
•Pediatric dentist
•Geneticist
•Speech Terapist
•Social Worker
•Nurse experienced in cleft problems

Schedule of Treatment
Birth:
Initial Assessment
Pre-surgical assessment
3 Month:
Primary Lip repair
9-18month:
Palate Repair
2 Year:
Speech assessment
3-5 Year:
Lip Revision Surgery
8-9 Year:
Initial interventional Orthodontics
Preparation for alveolar bone
grafting
10 Year:
Alveolar Bone Grafts
12-14 Year:
Definite Orthodontics
16 Year:
Nasal Revision Surgery
17-20 Year:
OrthognathicSurgery

Advantages for early closure of
palatal defect
(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.
WWW.SMSO.CC

Disadvantages
of closing palatal clefts in early life
(1)surgicalcorrectionismoredifficultinyoungerchildren
withsmallstructures,and
(2)scarformationresultingfromthesurgerycauses
maxillarygrowthrestriction.
WWW.SMSO.CC

Complications
•Acute Period
•bleeding,
•Airway obstruction
•Infection
•Wound seperatiom
•Late
•maxillary hypoplasia, dental occlusion problems)
•Hearing problems
•velopharyngeal insufficency
•Fistula formation

Other Operations
•Fistula Repair
•Velopharyngeal Insufficency correction (5 yeras)
•Secondary Onarımlar (preschool age)
•Alveolar bone grafting (before canine theth eruption)
•Orthodontic Surgery (12-14 years)
•(Le-Fort I Maksillary osteotomy, Mandibular split ramus osteotomy)
•Rhinoplasty (16-18 years)

Thank you
for your presence

South Africa:
◦2006 study: participants included 35 cleft patients and their families
Beliefs about the cause:
God
Witchcraft
Ancestors punishing the mother
Fate
Genetics and family history of the condition
◦2007 study: looked at the beliefs and practices of religious healers
Most common belief for both Muslim and Hindu healers= it was God’s will or because of an
eclipse
Both also identified evil spirits, witchcraft and genetics as causes
Hindu group identified karma as being a possible cause
Both groups reported that their patients came to traditional healers because they were looking for
comfort, or to be relieved of guilt because of their culture and what their ancestors did
Beliefs of African Americans in US are similar (punishment for something the
mother has done, evil spirits, or displeasing a god)

Nigeria
◦2007 study regarding the beliefs of mothers inNigeriawho had a
child with a cleft lip or palate
◦Identified as Hausu/Fulani (Muslim)= believed cleft was an act of
God
Other beliefs included the mother going outside during an eclipse while
pregnant or laughing at someone with a cleft lip
Because of their belief that the cleft is an act of God, may feel less
shame related to the condition and also less likely to have it repaired
◦Identified as Yoruba (Christian)= believed cleft was due to evil
spirits or punishment from ancestors
Other beliefs included curses, not having enough food during
pregnancy or hereditary/environment factors

Asia:
◦1990 paper discussing the beliefs of people of Asian/Pacific Island
descent living in the United States:
Some view a cleft as a gift from God, while others view it as a curse
Many beliefs exist about the role different foods play in causing or healing
physical problems
Ex/ eating hare while pregnant could cause a cleft lip
Members of some religions, such as Buddhism, may view a cleft as part of a
person's karma and not treat it at all
Beliefs regarding the cause include spirits, negative forces, punishment for the
actions of ancestors and eating certain foods
Depending on the belief regarding the cause of the cleft, patient may feel
shame

Latino
◦Article focused on Latino people (from Mexico, Central and South
America, and the Caribbean) who are living in the United States
Eiology of a cleft lip or palate may be accounted for by folklore or
mythology
Some beliefs include that cleft is due to natural events (eclipse),
actions of the parent (drinking alcohol or having a STD), or emotional
factors (stress during pregnancy)
Witchcraft or evil spirits also believed to be a cause
◦Health care underutilized due to lack of insurance and/or fear of
not being a legal citizen of the US