Craniofacial anomalies, VPI, Clefts

Speech Language Pathology 551 with Estis at University of South Alabama

About this deck

By: Ashley James
Created: 2011-12-11
Size: 96 flashcards
Views: 48

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1. Ala Base

2. Ala Rim

3. Cupid's Bow

4. Nasal Tip

5. Columella

6. Philtral Ridges

7. Philtrum

8. Tubercle

1. Hard Palate

2. Velum (Soft Palate)

3. Uvula

4. Palatine Tonsil

5. Alveolar Ridge

6. Anterior Faucial Pillar

7. Posterior Faucial Pillar

8. Dorsum of Tongue

9. Ventral Surface (Under Tongue)

1. Premaxilla

2. Incisive Suture Line

3. Median Palatine Suture

4. Transverse Palatine Suture

Velopharynx

•Valve that separates the oropharynx and the nasopharynx

•Opens and closes as a valve

•Closure varies across individuals

1. Coronal

2. Sagittal

3. Circular

4. Circula with Passavant's Ridge

Cleft

•an abnormal opening or a fissure in an anatomical structure that is normally closed

Cleft Lip

•the frontal process and maxillary process fail to fuse at approximately 35 days gestation

•Types

–Complete

–Incomplete

–Bilateral

•Most are unilateral, with left much more common than right

•Fewer than 5% occur without clefts of the palate

Cleft Palate

•roof of mouth does not completely close, leaving an opening that can extend to the nasal cavity

Craniofacial Anomalies

•Craniofacial anomalies: deformities in the growth of the head and facial bones, congenital

Causes of Craniofacial Anomalies

–Combination of genes

–Environmental

–Folic Acid Deficiency

Embryological Development

•6-7 wks- lip and alveolus begin to develop

•8-9 wks- palate begins to develop

12 wks- structures come together to form hard palate

Types of Cleft Lips

–Complete

–Incomplete

–Bilateral

Most Common Cleft Lips

Most unilateral,

Left much more common than right

•Fewer than 5% occur without clefts palate

Cleft Palate Types

–Soft

–Hard

–Complete

–Bilateral

Cleft Palate Classifications

–Kernahan (Striped Y)

–Veau

DNA-

• deoxyriboneucleic acid, encode genetic information

Gene

most basic unit of heredity, one segment of the DNA molecule

Chromosome

single, linear double strand of DNA with associated proteins that function to organize and compact the DNA and/or function in regulating gene activity

Single Gene Disorders

•Autosomal recessive

•Autosomal dominant

•X-linked

Multifactorial Inheritance

•Some human disorders result from an interaction of multiple genes with environmental influences.

–Continuous Variation

–Threshold Disorders

Multi-Anomaly Disorders

•350 syndromes that involve orofacial clefting

•Problems:

–Agreement on distinguishing characteristics

–Varying expression of physical signs

–Overlapping signs

•One of the most common

causes of Cleft palate

and/or lip

•1:35,000

•Autosomal dominant (1q32)

•Clinical Features: cleft lip; cleft palate, pits or mounds of the lower lip or on the buccal surface of the lower lip, neonatal teeth and missing teeth

•Isolated or associated with an underlying syndrome

•Retracted and elevated tongue (glossoptosis)

•Small manidble (micrognathia)

•Cleft palate- typically U shaped

•Autosomal dominant disorder with variable expressivity

•1:10,000

•Cleft palate, micrognathia, PR Sequence, myopia (nearsightedness), sensorineural hearing loss, flat facial profile, epicanthal folds, midface hypoplasia, flat nasal bridge

•1:4,000

•AKA Shprintzen Syndrome

•Autosomal dominant condition (22)

•Abnormal skull base

•VPI, Cleft Palate, congenital heart defects, microcephaly, narrow palperbral fissures, wide nasal root, bulbous nasal tip, vertical maxillary excess, thin upper lip, long face, micrognathia

•Autosomal Dominant, chromosome 5

•Downward slanting palpebral fissures, microtia, large mouth, micrognathia, cleft lip for some

•Premature fusion of one or more cranial sutures

•1:2,000-2,500 births

•Cause the skull to grow abnormally, resulting in an abnormally shaped head

•Craniofacial Dysostosis

•Malformations of the skull and face

•Mutation of gene on chromosome 10

•1: 25,000

•Hypoplasia, retrusion of the midface, shallow orbits,

exophthalmus (ocular proptosis),

atrophy of the optic nerve

•Autosomal Dominant, mutuation on 10

•Similar to Crouzan, except have serious hand and foot anomalies

•Narrow palate, sometimes cleft palate, narrow nasal and pharyngeal airway, syndactyly (webbing)

Chromosomal Syndromes

•Monosomy

•Trisomy

•Mosaicism

Deletion

Monosomy

loss of one copy of a chromosome

Trisomy

gain of one extra copy of a chromosome

Mosaicism

presence of cells with two or more different genetic contents in a single individual

Deletion

–part of one chromosome is missing

Left- unilateral complete cleft of lip

Bifid Uvula

Submucous Cleft

Soft Palate Cleft

Hard and Soft Palate Cleft

1. Normal Palate

2. Soft Palate Cleft

3. Hard Palate Cleft

4. Complete Palate Cleft

5. Bilateral Palate Cleft

1. Normal

2. Complete (into Nostril)

3. Incomplete

4. Bilateral

Bilateral Cleft Lip

Unilateral Cleft Lip

Cleft Lip Repair

Goal: create an upper lip that looks good with good muscular continuity across the cleft

Rule of 10s

At least 10 weeks old
Weigh at least 10 pounds
Hemoglobin of 10gm

Presurgical Management

Narrow the width of the gap prior to surgery
Unilateral
Simple tape across the cleft
Orthopedic bonnet with stretchable material across the lip or prolabium

Lip adhesion- surgical procedure that is used to create two raw surfaces that are then allowed to adhere

Primary

Millard Technique
Randall-Tennison Technique

Secondary

Reduce scar tissue on the lip
Improve symmetry
Improve nasal airway

Millard Technique

White roll come from the white roll of the lateral elements

Modified Manchester Repair

White roll from the prolabium is preserved

Cleft Palate Repair

•Palatoplasty

•Goal: establish intact division between oral and nasal cavities and obtain fully functioning VP system

•Timing

•Early- 6-15 months old

Late- 15-24 months old

Techniques for Palatoplasty

•Von Langenbeck

•60%

•Wardill-Kilner “V to Y” or “pushback”

•Furlow

Secondary Procedures to Overcome VPI

•Pharyngeal flaps

•Posterior pharyngeal wall augmentation

•Pharyngeal flaps

•Passive, soft tissue obturator formed by tissue raised from the posterior pharyngeal wall, and then sutured into the velum with lateral ports on each side to allow airflow

•Posterior pharyngeal wall augmentation

•Implant surgically placed or injected in posterior pharyngeal wall

•Implants include: paraffin, cartilage, fat, silicone

Dental Management Team

•Orthodontist: deals with how teeth fit into the craniofacial complex

•Family dentist: provides with routine tooth care

•Oral Surgeon: performs surgical procedures (bone grafts, extractions, implants)

•Prosthodontist: develops prosthetic devices (palatal obturators or lifts)

Normal Dental Development

•Primary Dentition (deciduous)=20 teeth

•8 incisors, 4 cuspids, 4 first molars, 4 second molars

•usually in place by 2 ½ years of age

•Mixed Dentition= deciduous teeth fall out and are replaced by permanent teeth

•Usually begins around 6 years of age

•Permanent Dentition= 32 teeth

•8 incisors, 4 cuspids, 4 first bicuspids, 4 second bicuspids, 4 first molars, 4 second molars, 4 third molars (wisdom)

Dental issues for children with cleft palate

•Delayed dental eruption

•Absent lateral incisor on the side of the cleft

•Supernumerary teeth

•Malocclusion

Hearing Disorders

•90% of children with cleft palate born with middle ear fluid

•High risk of middle ear infection throughout childhood

•Abnormal tensor veli palatini

•Often need PE tube placement

•Sometimes also see sensorineural and mixed losses

•Frequently screen hearing and middle ear (tymps)

Language Problems

•Research presents mixed results

•Factors:

•Syndromes that affect overall development and cognition

•Compromised overall health, hospitalization, surgeries

•Parent/child interaction and stimulation

•Decreased intelligibility may have a negative impact on communicative interactions

Feeding Problems

•Not always present

•May include:

•Poor oral suction

•Poor intake, lengthy feeding times

•Nasal regurgitation

•Choking and gagging

•Excessive air intake

•Solutions:

•Modified positioning for breast feeding

•Modified nipples

•Upright position

•Increase frequency of burping

•Feeding obturators

Resonance

•Hypernasality- sound resonates from the oral and nasal cavities during production of vowels and vocalic consonants

•Hyponasality- reduced nasal resonance due to blockage of the VP

•Mixed- pts with VPI who demonstrate both hyper and hyponasality

Assessing Resonance

•Need samples of nasal and non-nasal sounds

•SNAP (MacKay and Kummer, 1994)

•Conversational sample

•Instrumentation

•Seescape

•Nasometer

•Nasendoscopy

Articulation

Developmental Errors

Phonological Errors

Obligatory Errors

Compensatory Errors

Developmental Errors

produced in a way that is normal at an earlier stage of development

Phonological Errors

phonetic errors may be incorporate into child’s rule system

Obligatory Errors

•direct consequence of the anatomic or physiological defect

•Ex. Nasal emissions, nasal turbulence, malocclusions

Compensatory Errors

•errors that result from trying to make up for the deficiency

•Ex. Glottal stop, laryngeal fricative, pharyngeal fricative, posterior nasal fricative, posterior nasal affricate, laryngeal stop, pharyngeal stop

Treatment for Cleft Palate Speech

•Direct Motor Approach

•Move articulations forward

•Encourage strong contacts without excess force

Avoid...When Treating Cleft Palate Speech

• (according to Golding-Kushner):

•Oral-motor exercises

•Palatal and velopharyngeal exercises

•Blowing exercises

•Apraxia programs

•Sign language

Resonance

selective amplification and filtering of the complex overtone structure by the cavities of the vocal tract after the tone has been produced by the vibration of the vocal folds

Vocal resonance

perceptual increase in loudness of the laryngeal tone due to the concentration and reflection of soundwaves by the oral, pharyngeal, and nasal cavities during voice production

Velopharyngeal inadequacy

a generic term covering any type of abnormal velopharyngeal functioning, whether organically or structurally based

Velopharyngeal insufficiency

Any structurally based malfunctioning that results in imperfect closure of the velopharyngeal mechanism

Velopharyngeal incompetency

Imperfect closure of the velopharyngeal apparatus that is caused by a defect in neuromuscular functioning rather than a deficit of tissue

Velopharyngeal mislearning

Functionally based abnormal velopharyngeal closure, such as in the case of phonological errors, poor modeling influence, deafness or hear

Nasal Resonace Problems

Hypernasality

Hyponasality

Assimilative Nasality

Hypernasality

excessive resonance in the nasal cavity during voice production because of coupling of the oral and nasal cavities via the velopharyngeal port

Hyponasality

(aka denasality) reduced nasal resonance for the three nasalized phonemes

Assimilative Nasality

vowels or voiced consonants appear nasal when adjacent to the three nasal consonants

Evaluation

•Case history

•Analysis of voice in speech

•Stimulability testing

•Articulation testing

•Oral examination

•Instrumental evaluation

Instrumental Evaluation

•Nasometer

•Nasopharyngoscopy

•SeeScape

•Nasal Listening Tube/Stethoscope

Treatment of Hypernasality

•Surgical treatment

•Dental treatment

•Voice therapy

Voice Therapy for Hypernasality Consists of

Altering tongue position

•Change of loudness

•Auditory feedback

•Establishing new pitch

•Counseling

•Feedback

•Open mouth

•Focus

•Respiration

Treatment of Hyponasality

•Auditory feedback

•Counseling

•Feedback

•Nasal-glide stimulation

•Focus

About this deck

By: Ashley James
Created: 2011-12-11
Size: 96 flashcards
Views: 48

About StudyBlue

“I have used this website for three exams, and I see a huge difference in my test results.”
Naj