Cleft Lip and Palate Flashcards
Types of orofacial clefts
Cleft lip (incomplete)
Unilateral (complete) cleft lip and alveolus
Unilateral (complete) cleft lip and palate
Bilateral cleft lip and palate
Cleft palate only
Submucous cleft
Oblique facial cleft
Categorization of clefts
Syndromic
Non syndromic
What is a non syndromic cleft
The majority of orofacial clefts
A cleft in a normal child with no other congenital deformity
What is a syndromic cleft
Monogenic syndrome or chromosomal aberration
Multiple congenital anomalies
E.g.
Extra/missing fingers/toes
Syndactyly
Rotation of the heart
Mental retardation
Embryogenesis of the face (5th week in utero)
Face develops from the end of 4th week to the 8th week
Formation of the nasal placodes
Maxillary processes havent grown out yet
Formation of frontonasal, lateral nasal, medial nasal, maxillary and mandibular processes
Embryogenesis of the face (7th week in utero)
Maxillary processes advance medially below the lateral nasal process, fusing with the medial nasal processes
Medial nasal processes fuse with each other
This fusion forms the primary palate
Embryogenesis of the face (10th week in utero)
Face and palate are both fully formed
Frontonasal process proliferates downwards, forming the philtrum
Maxillary processes fuse with the frontonasal process
Formation of the palate
6th week: 2 shelf-like swellings project medially from the maxillary processes
7th week: palatal shelves elevate to a horizontal position, tongue moves down to avoid getting caught and causing a cleft palate
Shelves contact in midline and fuse, medial edge epithelium breaks down and mesenchyme flows across the palate
Fusion occurs from anterior to posterior, completing by the 10th week
Muscles of the soft palate are formed via migration of mesoderm from the pharyngeal walls
Embryological etiology of clefts
Caused by one or a combination of:
> hypoplasia of the processes
> abnormal direction of growth of the processes
> failure of fusion of processes
> breakdown of fusion of processes
Pierre Robin syndrome: mandible didn’t grow and is too small, tongue was too large and couldn’t drop down when the palatal shelves extended mesially, causing a cleft palate as the shelves could not fuse
Incidence of cleft deformity
About 1 in 700 births on average
Varies somewhat for different races for cleft lip / cleft lip + palate, but NOT for isolated cleft palate
About 1 in 600 births for Chinese
Demographic of clefts
Cleft lip most often unilateral, accompanied with cleft palate in most cases
More common on left side
20% bilateral cleft lip, 85% of which also has cleft palate
CL +/- palate is approx 2:1 for males:females
- Higher chance if family has
Isolated CP is approx 1:2 for males:females
- No higher chance if family has
Risk of recurrence of clefts
Depends on sex of individual and severity of cleft
Females higher, more severe higher
Highest risk is females with bilateral CL/CP
Lowest risk is males with unilateral CL/CP
How to detect cleft lips in fetuses?
Ultrasound
Ultrasound 3-D reconstruction
Cleft etiology
Multifactorial
Genetic susceptibility
Genetic and environmental factors
> steroids, thalidomide, alcohol, smoking, anticonvulsants
What can reduce cleft incidence?
Taking folic acid supplements - helps neural tube to fuse better and reduces incidence of all clefts
Problems faced by children with clefts
Feeding difficulties during infancy
Facial deformity
Speech deficiencies (air escapes into nose when speaking)
Hearing deficiencies, middle ear infections
Other congenital deformities (like ventricular septal defect, hydrocephalus and syndactyly
Discrepancy in growth causing retruded maxilla
Dental problems, malocclusion
Pediatrician do what
Helps in feeding neonates with cleft lip and palate
> risk of aspiration due to communication between nasal and oral cavities
> airway obstruction in neonates with cleft palate and micrognathia
> difficulties in feeding infants with cleft and nasal regurgitation
> dentists can also help make a feeding plate!
Soft feeding bottle with hole and soft flange, X cut on soft nipple or just squeeze intermittently
OR just hold infant more vertically for breastfeeding
Surgeon do what
Correction of facial deformities
> repair cleft lip at 3 months
> 10 pounds (5 kilos), 10mg Hb, 10 weeks old (abt 3 months)
> repair cleft palate at 9-18 months
> palatal lengthening/pharyngoplasty
> alveolar bone grafting at 7-9yo
> possible osteotomy at puberty
> nose revision at puberty
How to repair palate?
1) Repair soft palate first, joining all the muscles together
2) Make an incision on the sides of the hard palate, moving the sections containing neurovascular bundles medially
3) Stitch everything together
If adult already then only repair they don’t get much improvement in speech, need to go for speech therapy and may require palatal lengthening (as soft palate should touch the posterior pharyngeal wall to aid speech)
Why do cleft palate px get hearing deficiencies and middle ear infections?
The Eustachian tube connects the middle ear to the nasopharynx. Normal opening equalizes atmospheric pressure in the middle ear. Normal mucociliary clearance drains mucus from middle ear to nasopharynx to prevent infections.
Eustachian tube opening and closing is impaired: its normally controlled by the tensor veli and levator veli palatini in the soft palate
When you have a cleft palate these 2 muscles don’t work
Means that eustachian tube cannot equalize air pressure in the middle ear
Also harder to drain mucous from the middle ear into the nasopharynx, get middle ear infection (otitis media) which can lead to hearing loss
Tx of otitis media due to cleft palate
Myrinogstomy (tympanostomy), where you make an incision in the tympanic membrane an insert a ventilation tube (grommet) for drainage and ventilation
What does the speech pathologist do?
Identify difficulties in speech and reasons why
> velopharyngeal mechanisms may not be adequate for normal speech
> may not hear so well
> dental condition may be poor
What do the dentist, pedodontist and orthodontist do?
What dental problems does the child have?
> congenitally missing teeth
> supernumeraries
> irregularities of tooth size
> delayed eruption of teeth in cleft
> collapsed dental arches
> caries from inability to form lip seal and normal movement of cheeks and tongue
Orthodontist at the mixed dentition stage reduces anterior crossbites and expands arches to correct buccal alignment (e.g. via Quad Helix expansion appliance)
Which groups of people deal with the problems faced by cleft children?
Feeding difficulties during infancy (pediatrician, nurse)
Facial deformity (surgeon)
Speech deficiencies (air escapes into nose when speaking) (speech therapist)
Hearing deficiencies, middle ear infections (ENT specialist)
Other congenital deformities like ventricular septal defect, hydrocephalus and syndactyly (cardiologist, neurosurgeon, geneticist)
Discrepancy in growth causing retruded maxilla (surgeon)
Dental problems, malocclusion (dentist, orthodontist)
