Cleft Lip and Palate

Question 1

Types of orofacial clefts

Answer 1

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

Question 2

Categorization of clefts

Answer 2

Syndromic
Non syndromic

Question 3

What is a non syndromic cleft

Answer 3

The majority of orofacial clefts

A cleft in a normal child with no other congenital deformity

Question 4

What is a syndromic cleft

Answer 4

Monogenic syndrome or chromosomal aberration
Multiple congenital anomalies

E.g.
Extra/missing fingers/toes
Syndactyly
Rotation of the heart
Mental retardation

Question 5

Embryogenesis of the face (5th week in utero)

Answer 5

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

Question 6

Embryogenesis of the face (7th week in utero)

Answer 6

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

Question 7

Embryogenesis of the face (10th week in utero)

Answer 7

Face and palate are both fully formed

Frontonasal process proliferates downwards, forming the philtrum
Maxillary processes fuse with the frontonasal process

Question 8

Formation of the palate

Answer 8

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

Question 9

Embryological etiology of clefts

Answer 9

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

Question 10

Incidence of cleft deformity

Answer 10

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

Question 11

Demographic of clefts

Answer 11

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

Question 12

Risk of recurrence of clefts

Answer 12

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

Question 13

How to detect cleft lips in fetuses?

Answer 13

Ultrasound
Ultrasound 3-D reconstruction

Question 14

Cleft etiology

Answer 14

Multifactorial
Genetic susceptibility
Genetic and environmental factors
> steroids, thalidomide, alcohol, smoking, anticonvulsants

Question 15

What can reduce cleft incidence?

Answer 15

Taking folic acid supplements - helps neural tube to fuse better and reduces incidence of all clefts

Question 16

Problems faced by children with clefts

Answer 16

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

Question 17

Pediatrician do what

Answer 17

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

Question 18

Surgeon do what

Answer 18

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)

Question 19

Why do cleft palate px get hearing deficiencies and middle ear infections?

Answer 19

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

Question 20

Tx of otitis media due to cleft palate

Answer 20

Myrinogstomy (tympanostomy), where you make an incision in the tympanic membrane an insert a ventilation tube (grommet) for drainage and ventilation

Question 21

What does the speech pathologist do?

Answer 21

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

Question 22

What do the dentist, pedodontist and orthodontist do?

Answer 22

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)

Question 23

Which groups of people deal with the problems faced by cleft children?

Answer 23

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)