Cleft lip and palate Flashcards
Between what weeks of pregnancy does face formation occur?
between 4th and 8th week
Which prominences will form the face?
frontonasal prominence
2 maxillary prominences
2 mandibular prominences
what will the frontonasal prominence form?
the forehead, nose and central part of the lip
what will the maxillary prominences form?
the upper jaw, the upper half of the cheeks, with its skin, muscles and nerves
what will the mandibular prominences form?
the lower jaw, the lower half of
the cheeks, with its skin, muscles and nerves
while most fusion lines aren’t visible, what ones are?
the philtral columns, which are a normal part of the upper lip.
what has formed by week 11?
what develops first?
what develops next?
the palate
The primary palate develops first (hard palate)
The palatal shelves elevate and fuse in the midline to form the complete palate. This is the secondary palate
why does cleft lip and palate occur?
Whilst it is not known why clefts occur, they are generally caused by a failure of complete fusion of the various prominences that come together to form the face.
___ in every ___ babies born in Ireland has a cleft of the lip and/or palate.
1 in every 625
what factors are thought to contribute to the occurance?
a combination of genetic and environmental factors, since having a sibling or parent with a cleft increases the chance of clefting in a newborn
what are the cleft types?
cleft lip in isolation
unilateral cleft lip and palate
bilateral cleft lip and palate
cleft of the hard and soft palate
cleft of the soft palate
sub mucous cleft palate
what cleft type doesn’t affect speech?
cleft lip in isolation
what type is important to look out for as a SLT?
sub mucous cleft palate
what is a sub mucous cleft palate?
where the oral mucosa is intact but the underlying muscles of the soft palate are abnormally inserted into the posterior margin of the hard palate resulting sometimes in impaired soft palate function.
anomalies of sub mucous cleft palate:
- bifid uvula
- palatal notch: palpable v-shaped notch in posterior border of hard palate
- zona pellucida (not always present): translucent central zone due to muscle diastasis
what is the effect of having syndromes associated with CL/P
Children with syndromes are more likely to have more severe speech sound difficulties (Persson et al. 2005) and require additional surgery
examples of syndromes associated with CP
Treacher Collins, Aperts, Van
der Woude, Prader-Willi, Beckwith-Wiedeman, 22q11 Deletion Syndrome
frequently seen syndrome
22q11.2 Deletion Syndrome
Also known as DiGeorge Syndrome and/or Velocardiofacial Syndrome
22q11.2 Deletion Syndrome: CATCH 22
C - Cardiac defect
A - “Abnormal” facial features
T - Problems with the thymus gland and reduced immunity
C - Cleft palate/Palatal abnormalities
most commonly submucous cleft palate - seen in 69% of children (McDonald-McGinn et
al., 1999)
H - Hypocalcaemia, calcium deficiency
22 - Deletion of chromosomal material on chromosome 22
communication profile of children with 22q11
- VPD -60-70% will require surgery (Spruit et al. 2012)
- Glottal articulation/clicks/pharyngeal fricatives
- Childhood apraxia of speech (CAS)
- Dysarthria
- Language delay (mainly expressive)
- Voice disorders
- Lanryngotracheal abnormalities
other features of 22q11
- Intellectual disability
- Psychiatric diagnosis e.g. schizophrenia
- Anxiety issues
- Autistic Spectrum Disorder
- Attention Deficit Hyperactivity Disorder
implications of cleft
- Feeding
- Speech / Language/ Voice
- Hearing
- Orthodontics / Facial growth
- Dentition
- Psychosocial/appearance
primary surgery to repair cleft lip
3-6 months in 1 or 2 stages
primary repair of the palate
6-12 months in 1 or 2 stages
secondary speech surgery
3 years onwards
Following perceptual and instrumental speech investigations
Palate re-repair (intra-velar veloplasty)
Pharyngeal flap pharyngoplasty
Buccinator flap palatal lengthening.
Pharyngoplasty
lip revision
4-5 years, may try to push out to 7/8 years for better outcomes
alveolar bone graft
10-11 years
orthognathic surgery
17 years
further plastic revision of nose/lip
17 years +
what is oro-nasal fistula?
cause?
- A hole or opening in the palate after cleft palate
repair - Caused by breakdown of tissue during the healing
process post surgery - Can open after maxillary expansion or growth
what are residual clefts?
Unrepaired cleft of hard palate or alveolus which
is deliberately left open to be closed later
Lohmander and Harding Bell (2019)
effect of oronasal fistula depends on:
- Size- larger the more symptomatic
- Location- under the tongue tip will be
most symptomatic - May cause nasal regurgitation
What is the mechanism of normal feeding?
Combination of compression and suction to extract milk from bottle.
Compression helps to push milk out of teat and suction results in milk being extracted from teat.
What is compression in feeding?
positive pressure on teat i.e. when teat is placed between the tongue and palate
What is suction in feeding?
negative pressure created within the oral cavity i.e. lower jaw drops and tongue and nasal cavity is sealed off resulting in an enlarged sealed chamber
What is the effect of cleft lip/palate on feeding?
Babies with a cleft lip or small cleft of the soft palate may feed without difficulty.
Babies with larger clefts may have difficulty feeding due to not being able to create suction.
Milk may also spill into nose and come out of nostril during feeding.
Common Feeding difficulties with cleft lip and palate
Poor oral suction
Weak suck
Difficulty holding soother in mouth
Poor intake with lengthy feeding times
Nasal regurgitation
Intake of excessive air due to cleft
Fatigue (expend excess energy)
Poor weight gain
Stressful feeding interactions
Reid et.al (2007) found 73% of infants with a cleft palate had feeding difficulties
characterised by an inefficient suck and inability to develop adequate intraoral
negative pressure from a breast or regular nipple (Arvedson & Brodsky, 2002)
Why do children have increased risk of middle ear disease compared to adults?
flatter angle of the Eusatchian tube= ineffective drainage and aeration of middle ear
Why do people with CP/L have increased risk of ear infections?
tensor veli palatini (CN V) opens eustachian tube for middle ear aeration and drainage, as well as being part of soft palate muscles
high incidence of otitis media with effusion in children with CP
*92% in children 2-20 mths with cleft (Robinson et al (1992) versus
*20% in 24 mths olds without a cleft (Zielhuis et al, 1990)
what is OME?
A type of conductive hearing loss
Variable and unpredictable hearing impairment e.g. as little as 10dB or as much as 40dB (in which case the child may experience difficulty with consonant discrimination.
how can OME be treated?
grommet insertion or hearing aids
how can CP affect dentition?
Cleft palate can produce variations in number, enamel formation and eruption of teeth in the affected area, with the lateral incisor being most affected.
Alterations of tooth development in other areas can also occur:
- missing, fused or peg-shaped teeth
- delay in tooth formation and eruption of secondary teeth
- teeth are generally smaller than those in the non-cleft population
do dental anomalies have an effect on speech?
minimal effect
what is an alveolar bone graft?
The alveolar bone graft is designed to fill the gap in the bone to allow teeth to erupt normally. Filling the gap also closes the connection (fistula) to the nose and improves the shape of the upper jaw for future alignment of the teeth.
orthodontic alignment
aligning jaw correctly
osteotomy
the surgical cutting of a bone, especially to allow realignment.
malocclusion
misalignment of teeth
lateral occlusion
Malposition of tooth or entire dental arch in a direction away from midline.
classification of the bite/occlusion
class I, II, III
Class I
normal relationship between teeth and jaw
Class II
occurs when the lower dental arch is posterior (more towards the back of the mouth) than the upper one.
Class III occlusion
when the lower molars are very forward and do not fit their corresponding upper molars
impact of class III occlusion on speech production
There is a lack of
maxillary space and so the tongue may have insufficient space to function. Therefore
anterior tongue tip sounds can be produced by the blade of the tongue, causing
lateralisation and palatalisation of consonants.
* In severe Class III malocclusions it may be difficult to achieve labiodental placement
for /f/ and /v/, resulting in bilabial fricatives or dentolabial fricatives
how the palate works for speech
soft palate closes to create pressure needed for the production of certain sounds
normal velopharyngeal function: nasal and oral
nasal: velopharyngeal port remains open
oral: velopharyngeal port closes
weak pressure consonants
/w, l, j, ɹ,h/
cleft velopharyngeal dysfunction (VPD)
structural VP insufficiency
- unrepaired CP (overt/submucous)
- postsurgical insufficiencies (e.g. palatal length too short post palate repair: VPI postadenoidectomy
impact of palate being too short?
can’t reach base of nose
who should never have adenoids out?
children with CP
non-cleft VPD includes:
VP insufficiency: structural
VP Incompetency: neurogenic
VP mislearning
VP insufficiency (structural)
- mechanical interference (e.g. excessive tonsils or posterior pillar webbing
- palatopharyngeal disproportion (deep pharynx)
- ablative palatal lesions (e.g. cancer, traumatic injury)
VP incompetency (neurogenic)
- congenital or acquired primary motor/neuromotor control (dysarthria)
- motor association/motor programming (apraxia)
VP mislearning
- phoneme-specific nasal emission
- persistent postoperative nasal emission (with adequate closure ability)
- compensatory misarticulations
- deafness/hearing impairment
Effects of VP insufficiency/incompetence on speech
- Resonance
Hypernasality - Nasal Airflow
Nasal Emission
Nasal Turbulence - Articulation:
Passive cleft speech characteristics (CSC’s)
how is hypernasality judged?
Temple Street Scale of Nasality and Nasal Airflow (Sweeney, 2000 & 2011)
a)mild: evident but acceptable.
b)mild-moderate: unacceptable distortion , evident on close vowels
c)moderate: evident on close and open vowels.
d)moderate – severe: evident on all vowels and some consonants.
e)Severe: evident on all vowels and most voiced consonants
how is hyponasality judged?
Temple Street Scale of Nasality and Nasal Airflow (Sweeney, 2000 & 2011)
a)mild: evident, but acceptable.
b)moderate: all vowels reduced nasality
c)severe: total denasal production of nasal consonants
nasal airflow errors occur:
when there is an
inappropriate escape of air through the nose during
speech.
nasal emission:
- Audible escape of air through the nose on production
of oral pressure sounds - Perceived as frictional sound
- Accompanies a phoneme
nasal turbulence:
- Audible escape of air through the nose on production
of oral pressure sounds - Perceived as turbulent or snorting sound
- Accompanies a phoneme
active cleft speech characteristics
- adaptive/mislearning
- compensatory articulation is altered in response to previous or present abnormal
structure - responds to therapy
passive cleft speech characteristics
- Structurally/ physiologically related
- Obligatory: articulation is normal but the abnormal structure results in distortion of the speech sound
- needs surgery
