Ent - Kj Lee 10Th Ed - Chapter 13 - Cleft Lip & Palate

Front 1

Upper lip anatomy

What is the primary muscle?

What is it's nerve & blood supply?

Back 1

Orbicular oris muscle

Nerve: CN VII

Blood: superior labial artery (runs deep to the muscle) - branch of facial artery

Front 2

Palate anatomy

What are the structures in the primary & secondary palate?

Back 2

Primary palate - includes alveolar ridge & hard palate anterior to the incisive foramen

Secondary palate - includes hard palate posterior to the incisive foramen & soft palate

Front 3

Palate anatomy

What are the muscles of the palate?

Name the nerves that supply each one & their actions.

Back 3

1) Tensor veli palatini (CN V3) - opens eustachian tube, tenses soft palate.
2) Levator veli palatini (pharyngeal plexus) - elevates palate
3) Uvularis (pharyngeal plexus) - moves uvula upward & forward
4) Palatopharyngeus (pharyngeal plexus) - draws palate downard

Front 4

Palate anatomy

What is the primary arterial supply to the hard & soft palates?

Back 4

Hard palate - greater palatine artery

Soft palate - descending palatine branch of the internal maxillary artery; ascending palatine branch of the facial artery, palatine branch of the ascending pharyngeal artery, lesser palatine artery

Front 5

Embryology of the lip & palate

Critical embryologic window for facial development is the first ___ weeks of gestation.

Back 5

12

Front 6

Embryology of the lip & palate

Critical gestational period for lip development is from __ to __ weeks

How about palate development?

Back 6

Lip - 4 to 6 weeks

Palate - 8 to 12 weeks

Front 7

Embryology of the lip & palate

Does development occur in anterior to posterior fashion or posterior to anterior?

Back 7

anterior to posterior

Front 8

The five facial prominence's appear around the __________ early in the fourth week of gestation.

What are the five facial prominence's?

Back 8

stomodeum (primordial mouth)

A) Frontonasal prominence
B) Paired maxillary prominences
C) Paired mandibular prominences

Front 9

The ____________ is formed by the fusion of the medial nasal prominences.

What does it develop into?

Back 9

Intermaxillary segment

Develops into the nasal tip, columella, central upper lip/philtrum, and (includes central maxillary alveolar ridge, maxillary incisors, and the hard palate anterior to the incisive foramen)

Front 10

The lateral lip is formed when what two things fused?

Back 10

maxillary prominences & the medial nasal prominences

Front 11

How is the secondary palate formed?

Back 11

The maxillary promiences form shelf-like processes that fuse anterior to posterior.

Front 12

What creates a clefting of the primary palate?

Back 12

lack of fusion between the medial nasal promience and the maxillary prominence.

Front 13

What creates a clefting of the secondary palate?

Back 13

lack of fusion between the maxillary prominence to the contralateral maxillary prominence

Front 14

List some environmental factors linking to clefting

Back 14

1) Anticonvulsant meds
2) Retinoic acid derivatives
3) Folic acid antagonists or deficiency
4) Cigarette smoking
5) Fetal alcohol exposure
6) Pregestational maternal diabetes

Front 15

What percentage of cleft lip (with or without cleft palate) is considered nonsyndromic?

Back 15

70%

Front 16

What percentage of cleft palate only is considered nonsyndromic?

Back 16

30%

Front 17

Mutations on which gene may contribute up to 12%of the genetic etiology for cleft lip/palate?

Back 17

IRF6 gene mutation on chromosome 1q32

Front 18

Which syndrome Is classically associated with the triad: micrognathia, glossoptosis and U-shaped cleft palate?

Back 18

Pierre Robin sequence

Front 19

What is the distribution of:

Cleft lip with palate
Cleft palate only
Cleft lip only

Back 19

Cleft lip with palate - 45%
Cleft palate only - 30%
Cleft lip only - 25%

Front 20

What percentage of clefts are unilateral & bilateral?

Back 20

Unilateral - 80%
Bilateral - 20%

Front 21

Cleft lips are more common on the left or right?

Cleft lips are more common in males or females?

Back 21

2/3rd are LEFT sided

2/3rd are MALE

Front 22

Cleft palates are more common in males or females?

Back 22

2/3rd are FEMALE

Front 23

What are the key components of classifying clefts?

Back 23

Detailed description of the following elements: lip, palate, unilateral, bilateral, complete, incomplete

Front 24

Which clefts, lips +/- palates, or isolated cleft palates vary incidences across ethnicites?

Back 24

Cleft lips +/- palates vary incidences across ethnicities

Isolated cleft palates are uniform across ethnicities

Front 25

What are the 3 main degrees of lip clefts?

Back 25

1) Microform cleft

2) Incomplete cleft

3) Complete cleft

Front 26

Which cleft lip does this describe?

Dehiscence of the orbicularis oris muscle without overt clefting of the epidermis, creating a notching at the vermilion

Back 26

Microform cleft

Front 27

Which cleft lip does this describe?

Dehiscence of the epidermis, orbicularis oris muscle, and mucosa. Nasal floor and sill are intact.

Back 27

Incomplete cleft

Front 28

Which cleft lip does this describe?

Dehiscence of the epidermis, orbicularis oris muscle, and mucosa extending into the nasal floor.

Back 28

Complete cleft

Front 29

Describe submucosal clefts

Back 29

A type of palatal clefts, where there is intact mucosa with underlying dehiscence of palatal musculature.

Classical physical findings are:
- bifid uvula
- Zona pellucida (hyperlucent gray line in the midline of the soft palate)
- Notch in the posterior palate at midline
- Risk for velopharyngeal insufficiency

Front 30

Which palatal cleft does this describe?

Dehiscence of the mucosa and palatal musculature posterior but does not fully extend through the entire secondary palate (or primary palate if there is a cleft lip)

Back 30

Incomplete cleft palate

Front 31

Which palatal cleft does this describe?

Dehiscene of the mucosa and palatal musculature and does fully extend through the entire secondary palate (and primary palate if there is a cleft lip)

Back 31

Complete cleft

Front 32

What are Tessier clefts?

Back 32

Clefts may involve the mouth, cheeks, eyes, ears and forehead and may continue into the hairline. These craniofacial clefts are often referred to as Tessier clefts.

They are numbered from 0-14 to indicate the location and extent of the cleft using the mouth, nose and eyesockets as landmarks, with the midline designated 0. These more extensive conditions may also be described anatomically, such as “oro-occular cleft” and “fronto-nasal dysplasia.”

Front 33

What are some presurgical orthopedic techniques that can be performed to narrow the cleft or reshape the nose?

Back 33

1) Nasoalveolar molding (NAM) - very involved and requires weekly visits to the prosthodontist for remolding the appliance. (it's thought that the neonatal cartilage is "moldable" up to the first 6 weeks of life)

2) Taping: requires compliance from the family to properly apply tape across the cleft tightly. Taping will not address the nasal deformity.

Front 34

Unilateral cleft lip

What is the muscle defect?

Back 34

Dehiscence of the orbicularis oris with abnormal attachments to the alar base & columella

Front 35

Unilateral cleft lip

Which way is the displacement of the nasal ala on the cleft side?

Back 35

Nasal ala on the cleft side is displaced inferiorly, laterally, and posteriorly

Front 36

Unilateral cleft lip

What are the differences in sizes of the lateral & medial cruses, & lower lateral cartilages on the cleft & noncleft side

Back 36

Lower lateral cartilages - same on both sides

Medial crus - on the cleft side it is shorter

Lateral crus - on the cleft side it is longer

Front 37

Unilateral cleft lip

Why is the columella and caudal septum displaced to the noncleft side?

Back 37

Caused by the pull of the abnormally inserted orbicularis muscle.

Front 38

In complete cleft lips, what happens to the nasal floor?

Back 38

it is absent

Front 39

What is the timing of surgical repairs of unilateral cleft lips? (rule of 10's..)

Back 39

>10 weeks
>10 Hgb
>10 lbs

Front 40

Describe the surgical repair technique for unilateral cleft lip

Back 40

1) Release of the orbicular oris from its abnormal attachments and recreation of the sphincter.
2) Lengthening the cleft lip to equal the length of the upper lip on the noncleft side
3) Symmetric alignment of the alar base (this can be dificult secondary to deficiency of the skeletal structure on the cleft side)
4) Closure of the nasal floor and sill
5) Meticulous realignment of the vermilion-cutaneous border at the cleft repair site and restablishing the "white roll"
6) Camouflage of the incisional scars
7) Primary rhinoplasty may be utilized to address the nasal deformity.

Front 41

List the three most widely used techniques for unilateral cleft lip repair.

Back 41

1) Millard rotation-advancement repair (46%)
2) Modified rotation advancement (38%)
3) Triangular flap technique (9%)

Front 42

Bilateral cleft lip

What happens to the muscle fibers and vermilion in the prolabial segment?

Back 42

they are both absent

Front 43

Are bilateral cleft lip repairs typically performed as a single stage procedure?

Back 43

Typically yes, but it can be staged at least 3 months apart with repairing the widest cleft first.

Front 44

Cleft palate

What is the defect in the velopharyngeal sling?

Back 44

The levator veli palatini muscle abnormally attach to the posterior margin of the hard palate, instead of interdigitating at midline.

Front 45

Early aggressive palatal surgery may impact _________, however, delayed palatal surgery may result in significant _________

Back 45

midface growth

speech disorders

Front 46

What is the most common site of fistula formation post-palatoplasty?

Back 46

junction of the hard palate-soft palate

Front 47

What are the three main types of cleft palate repair techniques?

Back 47

1) Straight-line closure
2) Double opposing Z-plasty
3) Schweckendiek two-stage palatoplasty repair

Front 48

What are the three types of straight-line closure techniques for cleft palate?

Back 48

1) Two-flap palatoplasty (Bardach)

2) Bipedicled-flap palatoplasty (von Langenbeck).

3) V-Y pushback palatoplasty (Veau-Wardill-Kilner).

Front 49

Describe the two-flap palatoplasty (Bardach) straight-line closure procedure

Back 49

Two posteriorly based mucoperiosteal flaps based off of the greater palatine arteries.

Shortens palatal length
Easy visualization of the arterial pedicles
Used for wide clefts
Single staged

Front 50

Describe the bipedicled-flap palatoplasty (von Langenbeck) straight-line closure procedure

Back 50

Attached anteriorly & posteriorly
Blood supply from both anterior & posterior attachments
Blinded dissection around the greater palatine artery pedicles.
Shortens palatal length
Used for more narrow clefts
Single staged

Front 51

Describe the V-Y pushback palatoplasty (Veau-Wardill-Kilner) straight-line closure procedure

Back 51

Lengthens the palate
Used for wide clefts
Single staged

Front 52

Describe the doule opposing Z-plasty palatoplasty (Furlow)

Back 52

Most commonly used for submucosal and soft palate clefts
For complete palatal clefts, the Furlow technique may be used to close soft palate and combined with straight-line hard palate closure technique.
Best technique for reestablishing the levator veli palatini into its normal horizontal sling orientation
Significantly lengthens palate
When velopharyngeal insufficiency persists after a straight-line closure palatoplasty, a revision Furlow palatoplasty may improve speech prior to pharyngeal surgery.
Single staged

Front 53

Describe the Schweckendiek two-stage palatoplasty repair

Back 53

Closes the soft palate cleft and leaves the hard palate repair until the maxilla or palate is more developed.
A prosthetic obturator is used to obstruct the oronasal communication until time of hard palate repair.
Poor compliance with the children wearing the obturator leads to poor speech development.
Not a frequently used technique
Two-stage repair.

Front 54

What are some otologic associations with cleft patients?

Back 54

- 95% of cleft pt's have chronic otitis media with effusion
- Poorly fnct tensor veli palatini (due to its defective attachment) contributes to poor pressure equalization
- Increased nasopharyngeal reflux from the cleft may exacerbate middle ear dz
- Ventilation tubes are often placed at the time of lip repair.
- Ear dz typically improves with age and after palatal repair

Front 55

What are some swalling associations with cleft patients?

Back 55

- Not able to create suction or create a seal around nipple
- Use modified nipples that ease the flow and create less need for suction
- Popular cleft nipple or bottles include: Pigeon, Haberman, and Mead-Johnson bottles
- Cleft babies need to be burped more frequently

Front 56

What & of cleft pt's have speech difficulties?

Back 56

25%

Front 57

What is the initial treatment for VPI?

Back 57

Intensive speech therapy for at least 6 months.

Failure to improve with 6-12 months of speech therapy may indicate need for surgical intervation.

Front 58

If a pt with VPI has had intensive speech therapy but is not a good candidate for surgical intervantion, what can you do to improve VPI?

Back 58

dental obturator

Front 59

Children with this syndrome often have persistent VPI even after surgical intervention.

Back 59

Velo-cardio-facial syndrome

Front 60

Good anterior-posterior wall motion with poor lateral wall closure classically is repaired via ___________________

Back 60

Orticochea spincteroplasty (aka sphincter pharyngoplasty)

Front 61

Good lateral wall motion with poor anterior-posterior wall motion is classically is repaired via ___________________

Back 61

posterior pharyngeal flap