SLHS Unit 2: Cleft Lip/Palate

Know Anatomical Landmarks

Know Oral Landmarks

Maxilla (Upper Jaw)

Fuses medially during embryogenesis

Anterior 2/3 of hard palate = palatine process of maxilla

Horizontal Plate of Palatine Bone

Posterior 1/3 of hard palate

Hard Palate:

Provides stable platform for mobile muscular valve located posteriorly

Facial & Palatal Development: 4 weeks gestation

Mandible fuses

Facial & Palatal Development: 7 weeks gestation

Lip fuses

Facial & Palatal Development: 9 weeks gestation

Hard palate fuses

Facial & Palatal Development: 12 weeks gestation

Soft palate fuses

Clefting caused by a…

Failure of the structures to fuse during early embryological development

4-7 weeks after conception

Face and anterior parts of mouth are formed

Normal development of face: result of complex fusion of 5 main embryonic processes

Mandibular processes (R and L)

Frontonasal process

Maxillary processes (R and L)

Fusion of R and L mandibular processes

Occurs 4-5 week of gestation

Fuse at the midline of the face

Frontonasal and Maxillary process

Frontonasal process grows downward

Lateral nasal and nasomedian processes fuse with maxillary process

Tissue from the nasomedian process will form the…

Anterior portion of the upper lip

Front of the alveolus

Front of the hard palate (premaxilla)

Week 7 gestation

8-12 weeks after conception

Hard and soft palates are formed

Fusion of palatal shelves

The palatal plates descend from…

…the inner side of the maxillary process

The palatal plates fuse…

from the front to the back

Nasal septum grows downward and meets the palatal plates in the midline

Soft palate starts forming around ___ weeks and is complete by ___ weeks

10

12

Velopharyngeal Port

Opening between oral and nasal cavities

Velopharyngeal Port is important for: (motor functions)

Speech, blowing, whistling, sucking, swallowing, vomiting

Velopharyngeal Port is important for: (speech sounds)

All vowels, most consonants (plosives, fricatives, affricates), pressure and nasal consonants

Velopharyngeal dysfunction

Velopharyngeal value does not close consistently and completely

Results in abnormal resonance, airflow and articulation

Velopharyngeal Valving

Closure of nasal cavity from oral cavity accomplished by coordinated action of:

• Velum

• Lateral pharyngeal walls

• Posterior pharyngeal wall

  • Levator veli palatini (LVP)

  • Superior constrictor (SC)

  • Tensor veli palatini

  • Musculus uvulae

What is the Levator veli palatini (LVP)

Main velar elevator

Pulls velum up & back like a sling

What is the Superior constrictor (SC)

Primary sphincter

moves lateral pharyngeal walls medially and posterior pharyngeal wall anteriorly

What is the Tensor veli palatini

Tenses the soft palate

What is the Musculus uvulae

Increases velar mass during speech, making it larger target for pharyngeal contact

LVP + SC + increased mass of velum =

VP closure

Craniofacial Anomalies

Malformations that affect the head and face, and often cause speech problems

A Cleft:

Abnormal opening in an anatomical structure

Caused by failure of the structures to fuse during early embryological development

Cleft Lip and Palate Epidemiology

2nd most frequently occurring congenital deformity

Most common diagnosis: unilateral cleft lip and palate, followed by isolated cleft palate

Cleft lips show racial variability

What causes clefts?

• Genetic Disorders

• Chromosomal Aberrations

• Teratogenically induced abnormalities

• Mechanically induced abnormalities

Genetic Predisposition + Environmental Factors

Types of Cleft Lip and Palate

• Cleft lip only

  • Complete

  • Incomplete

  • Microform

• Cleft palate only

  • Complete (hard and soft palate)

  • Incomplete (soft palate only)

• Cleft lip and palate

  • Unilateral

  • Bilateral

• Submucous cleft palate

  • Overt

  • Occult

Incomplete (partial) Cleft Lip

Minor “V” shaped notch in vermilion border

Complete Cleft Lip

Separation of upper lip tissue into the nostril floor

Identify:

Microform, incomplete, complete

Identify:

Bilateral and mixed one

Unilateral Cleft Lip and Palate

Extends from the upper lip through the alveolus and hard and soft palate

Nasal septum attaches to the larger of the two palatal segments

Bilateral Cleft Lip and Palate

Most severe due to tissue loss

Lip and alveolus are cleft under both nostrils

Lip and premaxilla are abnormally positioned (free-floating premaxilla)

Palatal shelves completely separated

Overt (Classic) Submucous Cleft

Muscular cleft is covered by thin layer of mucosal membrane

(Can see the blue line)

Some signs that a submucous cleft is present are:

• Bifid uvula

• Bluish color/furrow in the middle of the soft palate zona pellucida

  • Notch that can be felt in hard palate

Occult Submucous Cleft

Palate appears to be normal

• Lack the triad (bifid uvula, hard palate bony notch, furrow in midline of soft palate)

Muscle fibers are abnormally oriented

Leads to velar dysfunction

Velar Dysfunction

Insertion of the palate muscles onto the hard palate rather than onto the midline soft palate

Related Issues of CLP: Feeding

Can’t build up negative pressure to extract milk

Related Issues of CLP: Otitis media

Middle ear inflammation w/ accumulation of thick, mucus-like fluid

• Eustachian tube often doesn’t function well

• May lead to hearing loss (tubes inserted early to equalize pressure)

Related Issues of CLP: Dental Problems

Cleft usually involves the alveolus (gums)

Related Issues of CLP: Parental concerns

Can be traumatic for parents

Requires immediate professional intervention

28% of pregnancies electively terminated

Related Issues of CLP: Later Social Consequences

Teasing

Self-concept

Challenges Throughout Life: At Birth

Obstructive breathing problems, food intake problems

Can be life-threatening

Challenges Throughout Life: Early Years

Surgical closure, middle ear infections, VPD evaluations, dental construction, speech therapy

Challenges Throughout Life: Adolescence

Orthodontic treatment, secondary surgeries, psychological counseling, continuing speech therapy

Challenges Throughout Life: Adulthood

Genetic counseling

Causes of Speech Disorders Secondary to Clefts

1. Nasal Obstruction

2. Short Upper Lip

3. Dental/Occlusal Abnormalities

4. Palatal Fistula

5. Hearing Loss

6. Tonsils and Adenoids

7. Velopharyngeal Dysfunction

1. Nasal Obstruction

Anomalies of the nose and septum

Narrowing of the nares post-surgery

Midface retrusion with growth

Nasal Obstructions can cause:

Hyponasality

Cul-de-sac resonance

Airway problems

Sleep apnea

2. Short Upper Lip

Due to scar contraction

Possible bilabial incompetence

3. Dental/Occlusal Abnormalities

Quite common

Missing, malpositioned or extra teeth

Malocclusion (Class III - greatest impact)

• Obligatory vs compensatory errors

4. Oronasal Fistula: Classifications

Post-surgical palatal hole

• Most common surgical complication

Recurrence rate = 4-35%

Classified as small, medium, or large

4. Oronasal Fistula: Factors

Preoperative size/type of cleft

Surgical procedure

Skill of surgeon

Postoperative infection

5. Hearing Loss

Muscle that opens the ET during swallowing starts in the velum (tensor veli palatini)

Middle ear effusion & otitis media

Conductive HL

Pressure equalizing tubes often used

Tonsils

Lymphoid tissue

Several different types

• Pharyngeal Tonsil

• Palatine Tonsil

• Lingual Tonsil

General term “tonsils” refers to the palatine tonsils

6. Adenoids (Pharyngeal Tonsils)

Lymphoid tissue

Directly above and behind the velum

Disappear with age and growth (no change in hypernasality)

May be removed for sleep apnea, hyponasal speech, poor olfaction, chronic OM

7. Palatine Tonsils

Do not assist with VP closure

May interfere with medial movement of the lateral pharyngeal walls or movement of the velum

Tonsillectomy: no risk for hypernasality (may improve resonance)

8. Velopharyngeal Dysfunction

Resonance Disorder (hyper/hyponasality)

Articulation Disorder (pharyngeal fricatives)

Communication Disorders

• Abnormal resonance

• Abnormal airflow and pressure

• Abnormal articulation

• Abnormal phonation

Abnormal Resonance

• Hypernasality

• Hyponasality

• Cul-de-sac resonance

• Mixed nasality

Hypernasality

Too much sound resonating through the nose

Most perceptible on vowels and stops (/s/, /b/, /k/)

Almost always requires surgical or prosthetic management

Hyponasality

Results from too little air escaping through the nose

Most perceptible on nasal consonants, but also vowels

Abnormal Airflow and Pressure

• Nasal Emission

• Nasal rustle/nasal turbulence

• Nasal grimace

Nasal emission

Air emission from nose during speech due to leak in VP value

Occurs in pressure-sensitive consonants (plosives, fricatives, affricates)

Articulation problems in children with clefts may be due to:

• VPD

• Structural deviations

• Missing/misaligned teeth

• Faulty learning

Most are compensatory

Persist post surgery until the correct ways to produce phonemes are taught

Abnormal Articulation

• Obligatory distortions

• Compensatory articulations

Obligatory Disortions

Nasalized phonemes

Nasal emission on all pressure sounds

Compensatory Articulations

Posterior nasal fricative

Pharyngeal plosive

Pharyngeal fricative/affricate

Glottal stop

Abnormal Phonation

Increased risk for dysphonia: hoarseness, breathiness, abnormal pitch

• Glottal stops

• Laryngeal hyperfunction to achieve VP closure

• Other congenital abnormalities of vocal cords/larynx

  • Breathiness may be strategy to mask hypernasality/nasal emission

Oronasal Fistula

Articulation errors and nasal emission

Midface hypoplasia and dental maloocculsion

Obligatory articulation errors

Velopharyngeal dysfunction

Hypernasality, nasal emission, weak intraoral pressure, oral for nasal sound substitutions

Vocal fold nodules

Can occur with velopharyngeal dysfunction

Can be caused by glottal stops

Nasal occlusion

Hyponasality or mixed resonance

Bilabial incompetence

Articulation errors

Effect of Cleft Lip on Feeding

May have initial difficulty latching

Usually able to breastfeed

Effect of Cleft Palate on Feeding

Babies with small SP cleft may breastfeed

Babies with complete cleft palate have feeding problems

• Difficulty generating negative pressure for sucking

• Problems with nasal regurgitation of milk, choking, swallowing air

• Successfully bottle-fed with modifications

Assessment for Resonance Problems

• Indirect procedures: nasal emission (mirror, straw), Seescape, nasometer

• Perceptual Ratings

• Direct Procedures: videofluoroscopy, nasoendoscopy

Assessment for Articulation Problems

• Standard articulation tests

• IPAT

• Articulation inventories

• Phonological process analysis

  • Oral mechanism examination

Speech Sound and Resonance Evaluation

• Perceptual evaluation

• Intraoral evaluation

• Instrumental evaluation

Perceptual Evaluation

Use connected speech if possible

Evaluate articulation, nasal emission, resonance, voice

Intraoral evaluation

Can evaluate oral structure and function, not VP function

Instrumental evaluation

Nasometry, nasopharyngoscopy, some centers use pressure flow studies

Oral Exam

Dentition and occlusion

Oral cavity size

Presence of fistulae

Signs of submucous cleft

Movement and symmetry of uvula

Size of tonsils

Signs of upper airway obstruction

Signs of oral motor dysfunction

Mouth opens bigger

Speech Sample

Prolongation of speech

Repetition of syllables

Counting

Repetition of sentences with pressure sensitive consonants

Connected speech

Nasometry

Measure acoustic energy from oral and nasal cavities

Give objective nasalance score: ratio of oral/total (oral+nasal) energy

SeeScape

Detects nasal emissions

Indirect Assessment of Resonance

Straw and Mirror Tests

Nonvoiced, high pressure sounds

Nasopharyngoscopy

Allows direct observation of structures and function of the VP mechanism

Can see location and size of the opening

Avoids “false negatives” seen in videofluoroscopy studies

Perceptual Rating of Resonance

Best way to assess is during connected speech

Ask clients to produce sentence full of oral sounds with the nose unoccluded then occluded

Management of Resonance Problems

Persons with mild resonance problems can try behavioral therapy

Most of the time, prosthetic and surgical management is necessary and most effective

Management of Articulation Problems

Surgery/prosthetics may indirectly improve articulation

Most children will require some behavioral intervention

Typical Surgical Timelines: Lip Repair

2 ½ to 6 months