Cleft Lip and Palate

Question 1

What percentage of cleft lip/palate pts have both or only one d/o?

Answer 1

Both - 50%
Palate only - 30%
Lip only - 20%

Question 2

What side for cleft lip is more common?

Answer 2

Left: right: bilateral = 6:3:1

Right sided clefts are more commonly a/w syndromes

Question 3

Gender predilection?

Answer 3

Cleft lip - male

Cleft palate - female

Question 4

Primary palate

Answer 4

Lip and pre-maxilla

Question 5

Secondary palate

Answer 5

Extends from incisive canal back

Question 6

Embryology of fusion

Answer 6

The lip and alveolus are formed by fusion of frontonasal process and lateral maxillary processes
This fusion is reinforced by migration of mesenchymal tissue derived from neuroectoderm (stabalized by folate)

Question 7

Role of orbicularis oris in cleft lip

Answer 7

Complete u/l: the muscle is interrupted and remnants flow upwards to base of columella medially and alar base laterally
Incomplete: variable amnt of muscle intact across upper lip portion
Complete b/l: no muscle in central portion (prolabium)

Question 8

Role of levator palatini in cleft palate

Answer 8

Normally forms a sling to elevate soft palate
In cleft palate, fibers are oriented longitudinally (parallel to cleft)
Repair includes reorientation of the fibers

Question 9

Why do cleft palates get PE tubes?

Answer 9

Tensor palatini muscle is also abnlly oriented, more longitudinally causing inadequate opening of eustachian tube (and high incidence of serous OM)

Question 10

Complete vs incomplete cleft lip (classification)

Answer 10

Complete: separation of the lip that extends through nasal sill and alveolus into the palate
Incomplete: variable width w/ intact bridge of skin below nasal sill known as Simonart’s band

Question 11

Simonart’s band

Answer 11

Intact bridge of skin below the nasal sill in an incomplete cleft lip

Question 12

Forme fruste (or microform cleft lip)

Answer 12

Small cleft

May be as little as a small notch in the vermillion

Question 13

What is a problem in a child with b/l complete cleft

Answer 13

The central portion of the alveolus, the premaxilla, is attached only to nasal septum and the central lip (prolabium) is attached only to the premaxilla and the columella
Problem: Premaxilla migrates anteriorly and can be virtually horizontal in orientation. The premaxilla must be brought down into a closer relationship with the lateral segments to achieve a b/l cleft lip repair

Question 14

Complete vs incomplete cleft palate

Answer 14

Complete: occurs in a/w complete cleft lip
Incomplete: cleft of secondary palate only

Question 15

Submucous cleft palate

Answer 15

Special subset of cleft palate
Separation of the levator palatini muscles but intact mucosa
Dx by classic triad: bifid uvula, central thinning of soft palate, palpable notch in posterior border of hard palate
Tx: observe as speech develops (most nl speech)
If nasal air loss occurs, consider surg
Furlow double-opposing Z-plasty is good option for repair

Question 16

Syndromes a/w clefts

Answer 16

Velocardialfacial Synd
Van der Woude Synd
Stickler Synd
Pierre Robin Sequence

Question 17

Velocardialfacial synd (or Shprintzen synd)

Answer 17

22q del
Cleft palate (may have VPI w/o cleft)
Cardiac anomalies
Ch facial appearance
Developmental delay affecting speech
Dx: FISH

Question 18

Van der Woude syndrome

Answer 18

Cleft palate (+/- lip)
Lip pits (lower lip sinus tracts of minor salivary glands)
Auto Dom, variable penetration/presentation

Question 19

Stickler Synd

Answer 19

Clefts
Ocular (severe myopia, retina)
Thus, all kids w/ clefts need pedi ophtho exam to r/o the Dx in 1st yr of life

Question 20

Why should kids w/ clefts get a pedi ophtho exam w/in year of life 1?

Answer 20

R/o ocular abnlities like in Stickler synd

Question 21

Pierre Robin Sequence

Answer 21

Triad: retrogenia, retrodisplacement of the tongue, and resp insuff
Clefts of the secondary palate (u-shaped, wide)
Breathing difficulties from post tongue position and upper post pharyngeal obst
Turn infant prone after birth to alleviate obst
Over time, mandible grows forward and problem improves
If conservative measures fail –> surg (goal is to avoid trach)
Bronch to r/o laryngomalacia or tracheomalacia
Tongue-lip plication (glossopexy): suture lower lip to tongue
Mandibular distraction (preferred): used in infants to elongate ramus and bring tongue forward

Question 22

Pre-op considerations

Answer 22

Multidisciplinary team approach & evaluation
Cleft nurses, assess weight gain
Pre-op manipulation of the alveolar segments in complete cleft lip & palate often used to reduce the width of a cleft facilitating tension-free closure (orthodontic molding plates)

Question 23

Nasoalveolar molding (NAM)

Answer 23

When extensions of molding plates are used for stretching the nasal ala
Labor-intensive

Question 24

Lip adhesion

Answer 24

Procedure where the cleft segments are surgically united via small flaps creating an incomplete cleft lip
This molds the alveolar segment
Secondary operation is needed after an interval to convert the adhesion to a formal lip repair
However, this creates scar tissue that can impede final repair

Question 25

When should a cleft lip be repaired?

Answer 25

Rule of 10’s: 10 lbs, 10 wks, 10 Hgb
Wait longer if presurgical manipulation of alveolus or premaxilla required
Less urgent for incomplete cleft bc alveolar segments held in place by the intact Simonart’s band

Question 26

Goals of cleft lip repair

Answer 26

Create a symmetrical Cupid’s bow and lip fullness w/o losing nl contour of lip and philtrum
To create length on the cleft side, some tissue from the lateral lip element must be inserted into the medial segment

Question 27

Z-plasty location in cleft lip repair

Answer 27

Triangular lip repair: above the vermillion border
Other repairs: central portion of the lip
Rotation advancement repair: below nasal sill

Question 28

Red line

Answer 28

Junction of the wet and dry vermilion

Question 29

Rotation advancement cleft lip repair

Millard repair

Answer 29

M/c technique
Almost no tissue discarded
Medial lip element is rotated downward, even w/ a back cut if necessary, and the lateral lip is advanced into the defect under the nasal sill
Mucosal flaps are used to line the nose and the vestibule of the lip
“cut-as-you-go” technique
Recruits length for the lateral advancement flap by following the vermillion border
Adv: Good lip projection (“pout”) by creating tension under the nasal sill rather than along the vermillion border
Disadv: Lip may be short after healing (placement of a tiny z-plasty may improve this)

Question 30

Triangular cleft lip repair

Tennison-Randall repair

Answer 30

Evolved from earlier quadrilateral repairs
Nearly horizontal incision made in lower half of the medial cleft segment
A triangular piece is fashioned in the lateral flap to fit in the resulting defect
Closure is a modified z-plasty placed low on the lip
Like all z-plasties, length is borrowed at the expense of width
Excellent lip length
Disadv: flat repair when viewed from the side

Question 31

Challenges a/w bilateral cleft lip repair

Answer 31

Greater complexity

1. Premaxilla
2. Nasal deformity
3. Blood supply maintenance
4. Symmetrical deformities

Question 32

Premaxila challenge w/ b/l cleft lip repair

Answer 32

Premaxilla is usu quite protrusive and must be controlled preop (alveolar molding); severe protrusion can be approached with osteotomy of the vomer to allow premaxilla setback (last resort bc a/w maxillary hypoplasia)

Question 33

Nasal deformity in b/l cleft lip repair

Answer 33

Columella is extremely short and nasal tip is flat w/ b/l alar base widening
Alveolar molding may be combined w/ nasal molding by adding small prongs anteriorly to lengthen the columella
Post-op nasal stents can be useful
Can try V-Y advancement flaps for columella lengthening