Cleft Lip

Dr.Mgs. Roni Saleh,

I. Embryology
A.

B.

C.

The critical developmental period of the lip and

primary palate occurs during weeks 4 to 6 of
gestation.
Failure of complete union of the medial nasal
prominence and the maxillary prominence leads
to a variable extent of clefting of the primary
palate, invoiving the upper lip, alveolus, and
anterior hard palate to the incisive foramen.
Cleft lip alone (CL) and cleft lip and palate (CLP)
are considered to be the same entity along a
morphologic continuum. Clef palate alone (CF),
on the other hand, has different demographics.

II. Epidemiology and Genetics

A.

Incidence of cleft lip and of cleft lip and

palate
1.
2.
3.
4.

B.

The overall incidence is 1 in 1,000 live births

White ancestry: 1 in 750 live births
Asian ancestry: 1 in 500 live births.
African ancestry: 1 in 2,000 lilve births.

Demographics
1.
2.
3.
4.

Male-to-female ratio of 2:!

The ratio of left (L) to right to bilateral (B) clefts
(L:R:B): 6:3:1
The ratio of CLPmto CL is 2:1
Three percent are syndromic.

II. Epidemiology and Genetics

(Contd)
5.

Risk factors
a.
b.
c.
d.

C.

Medications: Phenytoin, methylprednisolone (Solo-Medrol),

steroids, phenobarbital, diazepam, and isotretinoin.
Smoking
Parental age, especially fathers age, or both mother and father
over 30 years old.
Family history (see Genetics)

Genetics
1.

The risk of having a child with CLP

a.
b.
c.
d.

2.

If parents have one child with CLP: 4%

If one parent has CLP: 2% to 4%
If parents have two children with CLP:9%
If one child and one parent have CLP: 14% to 17%.

Most cases are sporadic(and multifactorial), but may be Xlinked, autosomal dominant (Van der Woudes syndrome) of
familial (see Syndromes Associatied with Cleft Lip and
Palate, later in this chapter).

III. Anatomy
A.

Normal lip anatomy

1.

Topographic landmarks
a.
b.
c.
d.
e.
f.
g.
h.

Nasal alae.
Columella
Philtral columns.
White roll: Well-defined mucocutaneus or vermilioncutaneous border.
Vermilion: Red portion of lip.
Tuberle.
Cupids bow
Wet-dry border: The vermilion-mucosa junction is the
border between keratinized and nonkeratinized mucosa.

III. Anatomy (contd)

2.

Musculature
a.

Orbicularis oris
Fibers cross (decussate) in the midline and create the
opposite philtral columns.
2) Functions as a sphincters (deep fibers) and for speech
(superficial fibers).
1)

b.

Levator labli superioris.

Inserts into the dermis at the vermilion vorder and the
lower edge of the philtral columns.
2) Elevates the upper lip.
1)

c.

Nasalis or depressor septi nasi muscle: The fibers

run from the alveolar bone into the medial crural
footplates, skin of the columelia and the tip of the
nose, and into the opposite philtral columns.

III. Anatomy (contd)

3.

Normal measurements.
a.

Vertical length (height) of the upper lip

1)
2)
3)

b.

4.
5.
6.

Newborn: 10 mm.
Age 3 months: 13 mm.
Adult: 17 mm

The distance between the peaks of Cupids bow:

Aprroximately 3 mm at 3 months

Arterial blood supply: The labial artery, bilaterally.

Sensory Innervation: the trigeminal nerve, cranial
nerve (CN) V, maxillary division (V2).
Motor Innervation: the facial nerve, CN VII,
zygomatic and buccal branches.

III. Anatomy (contd)

B.

Cleft llip anatomy

1.

Alterations in the orbicularis oris, levator labii,

and nasalis result in disruption of continuity,
orientation, and quality of the muscless.

a.
b.

c.

Fibers are disoriented and run parallel to the cleft

margin.
Fibers insert into the alar base on the cleft (lateral)
segment and into the columella in the non cleft
(medial)segment, as well as intradermally.
Incomplete clefts.
1)
2)

d.

Simonarts band consist of a skin bridge across the nasal

sill. It does not usually contain any significant muscle mass.
Some fibers may cross the cleft, if the cleft is less than twothirds of lip height.

Bilateral complete clefts: No muscle tissue is present in

the pro-labium.

III. Anatomy (contd)

2.

3.
4.
5.

Vertical lip length is decreased: Cupids

bow and the lip are rotated cephalad on
both the lateral, cleft side as well as the
medial side.
Disrupted Cupids bow.
The alveolus and nostril floor are open
in a complete cleft lip.
The premaxilla is rotated and
protruding, especially in bilateral cleft
lip, often with collapase of the lateral
segment of the cleft side(s).

III. Anatomy (contd)

6.

Associated cleft lip nasal abnormalities

a.
b.
c.
d.
e.
f.
g.

Hypoplastic, flattened alar dome on the affected

side.
Lack of upper lateral cartilage overlap of lower
lateral cartilage.
Subluxed lower lateral cartilage with alar base
displaced cephalad and posteriorly.
Hypoplastic bony foundation (maxilla).
The caudal septum is pulled toward the noncleft
side.
Flattening of the nasal bones.
Shortened columella, especially in bilateral cases.

IV. Classification
A.

Extent of the cleft: Complete versus

incomplete
1.

Complete cleft lip

a.
b.

2.

Complete disruption of the soft tissues to the nasar

floor.
Tends to be wider than incomplete celfts, with greater
nasal deformities.

Incomplete cleft lilp

a.
b.
c.

Disruption of the soft tissues to varying degrees.

The alveolus is usually intact, with less of a tendency
for the premaxilla to protrude.
Forme fruste: A very mild cleft.
May be difficult to detect
2) May appear as vermilion notching or a scarlike line or
depression
1)

IV. Classification (Contd)

B.

Location of the cleft: Unilateral versus

bilateral
1.
2.

Unilateral cleft lip

Bilateral cleft lip
a. May have complete or incomplete cleft on both sides,or a
combination
b. More likely to be complete clefts and are often wide
c. The premaxillary segment may include tooth buds.
d. In bilateral complete clefts, the prolabium lacks muscle
tissue, and therefore lacks philtral columns.

C.

Alveolar segments
1.
2.

Narrow versus wide cleft

Collapse versus no collapse

V.
A.

Syndromes associated with

cleft lip and palate

Van der Woudes syndrome

1.
2.
3.
4.

Autosomal dominant, with variable

penetrance.
Associated with CLP or CP (40%-50%
penetrance).
Associated with lip pits (accessory
salivary glands, 70%-80% penetrance).
May also have absent second molar,
syndactyly, abnormal genitalia, and
popliteal pterygia.

V. Syndromes associated with

cleft lip and palate (Contd)
B.

Waardenburgs syndrome
1.

2.

A group of anomalies arising from

abnormal development and migration
of neural crest cells.
Features may include cleft lip, cleft
plate.

Down syndrome (trysomy 21)

D. Trisome 13
C.

V. Syndromes associated with

cleft lip and palate (Contd)
E.

Sticklers syndrome
A group of anomalies caused by
connective tissue dysplasia.
2. Typical features: Cleft palate, progressive
joint degeneration, and various ocular
abnormalities that may lead to blindness.
3. Autosomal dominant inheritance.
4. Other anomalies: Cardiac, sensorineural,
and learning disorders or mental
retardation.
1.

V. Syndromes associated with

cleft lip and palate (Contd)
F.

Pierre Robin sequence (Note: A sequence is a group of

anomalies that result from a single disrupted event)
1.

2.
3.

Micrognathia or retrognathia prevents normal descent of the

tongue. The tongue then interferes with fusionof the palatal
shelves. As a result, typical features include micrognathia or
retrognathia, glossoptosis (tongue falls back into the pharynx,
causing airway obstruction), and a U-shaped cleft palate.
May be a part of multiple different syndromes or may be an
isolated finding.
Treatment.

a.
b.
c.
d.
e.

Prone positioning to help move the tongue out of the aierway, the
most conservative approach.
Supplemental oxygen.
Tongue-lip adhesion.
Mandibular distraction osteogeneisi
Intubation/tracheostomy

V. Syndromes associated with

cleft lip and palate (Contd)
4.

5.

Patients may show catch-up

mandibular growth, depending on their
syndromic association.
Palysomnogram: Necessary to evaluate
for desaturations as well as apneic
events

V. Syndromes associated with

cleft lip and palate (Contd)
G.

Velocardiofacial syndrome
1.

2.

Autosomal dominant inheritance: Fluorescent in situ

hybridization (FISH) may show an abnormality in
chromosome 22.
Characteristic feature include the following.

a.
b.
c.
d.
e.
f.

Cleft palate.
Congenital heart disease.
Broad nasal dorsum and elongated face.
Narrow, down-slanting palpebral fissures.
Velopharyngeal insufficiency is common, even with a
submucous cleft palate.
The carotid arteries may be displaced medially, placing
them at high risk of injury during pharyngeal flap surgery
or dynamic sphincter pharyngoplasty. Always palpate the
posterior pharynx prior to making an incision; consider
obtaining a preoperative angiogram.

V. Syndromes associated with

cleft lip and palate (Contd)
H.

Median cleft lip

1.
2.

3.

4.
5.

Rare
A different entity from the typical cleft lip; more
accureately considered a median craniofacial cleft
(Tessier type zero).
Associated with a group of syndromes (median
cerebral facial dysgenesis) that involve more
severe deformities of midline CNS and facial
structures.
Further workup is needed, including a formal CNS
evaluation.
May be associated with holoprosencephaly,
pituitary problems, and a limited lifespan.

VI. Staging of intervention

A.

Initial evaluation
1.
2.
3.
4.

Reassure the parents and family that they are not to blame.
Explain the stages and operations that should be expected
throughout the childs lifetime
Evaluate for associated anomalies.
Consultations

a.
b.
c.

Genetics, for evaluation and possible counseling

Social works
Feeding/nutrition
1)
2)

d.

The child may need special nipples or bottles (e.g., cross-cut nipple)
Monitor for appropriate weight gain

Otolaryngology: Children with cleft lip and palate have a high

incidence of eustachian tube dysfunction, and therefore otitis
media, requiring close follow-up.
1)
2)

The child may need myringotomy tubes.

If unreated, repeat otitis may affect hearing and speech development.

VI. Staging of intervention

(Contd)
B. Wide clefts (> 1 cm)
1.

Goal : Bring the segments closer together

to facilitate a tension-free repair.
a.
b.

2.

Has not been shown to chanbe skeletal

development in the anteroposterior direction.
Does not seem to prevent future crossbite.

Passive: Preoperative taping

a.
b.

Steri-Strip tapes applied across both segments

of the lip.
Requires reliable parents who can reapply the
tape and keep it on at all times

VI. Staging of intervention

(Contd)
3.

Passive: Lip adhesion operation

a.
b.
c.

4.

Suturing the edges of the cleft together is

performed under anesthesia.
The definitive lip repair is performed once the
segments have moved closer together.
Variable success.

Active: Latham-type device

a.
b.
c.

An orthodontic appliance that must be placed onto

the palatal segments under anesthesia.
Parents turn a screw daily, which slowly brings the
palatal segments into better alignment.
Removed at the time of definitive lip repair.

VI. Staging of intervention

(Contd)
C.

Repair
1.

Timing (controversia)

a.
b.

2.

Rule of tens: For increased anesthetic safety,

an infant should
a.
b.
c.

3.
4.

Repair at 3 months is generally accepted.

Some argue for earlier repair in order to produce
better scars.

Be 10 weeks old
Weigh 10 pounds.
Have a hemoglobin level of at least 10 mg/dL.

Cleft palate repair and secondary alveolar

frafting
May also choose to address the cleft, nasal
deformity at time of lip repair.

VII. Intraoperative
considerations
A.

Landmarks
1.

Tattooed with methylene blue, using a

hypodermic needle or a quill pen.
a.
b.
c.
d.

e.

2.

Alar bases.
Columella.
Philtral columns.
D. Peak of Cupids bow midline on the medial segment.
Measure the anticipated distance for the new Cupids
bow (approximately 3-4 mm).
Peak of Cupids bow on the lateral segment.

Account for distortion from the uncountered pull

of the orbicularis on the medial segment. The
philtral columns are usually slightly C-shaped.

VII. Intraoperative considerations

(Contd)
B.
C.

D.

Mark lines for expected repair type.

Only after marks are completed, infiltrate
tissue with local anesthetic to avoid
distortion of anatomy and measurements.
Goals of repair
1.
2.
3.
4.
5.

Reconstitute Cupids bow

Minimize scarring
Produce a slight pout of the tubercle.
Produce functional continuity of the muscles
Recreate symmetry.

VIII. Types or repair

A.

Straight-line repair
1.

2.

Historically, the first cleft lip repairs

relied on freshening the edges of the
cleft and suturing them together. These
have been largely replace by various Zplasty-base techniques.
Rose-Thompson repair
a.

b.

Modified straight-line repair that can be used

for minor clefts with lip length nearly equal
on both sides of cleft (e.g. forme fruste)
Fusiform excision with straight-line closure.

VIII. Types or repair (Contd)

B.

Quadrangular flap
1.
2.
3.
4.
5.

Proposed by LeMesurier and Hagedorn.

Cupids bow is derived from the lateral
lip.
90-degree Z-plasty.
Violates Cupids bow and the philtral
dimple.
May also have problem with a long lip.

VIII. Types or repair (Contd)

C.

Triangular flap
1.

Proposed by Tennison and Randall

a.
b.
c.
d.
e.

2.

The Z-plasty is place at the vermilion border.

Produces an natural appearing Cupids bow
May be used for clefts of all widths.
Violates Cupids bow and the philtral dimple.
Has a tendency to produce a long lip.

Skoog repair
a.
b.

Consists of two Z-plasties

Violates Cupids bow and the philtral dimple.

VIII. Types or repair (Contd)

D.

Rotation advancement
1.

Popularized by Millard

a. Likely the most commonly used repair. Often described as the

cut-as-you-go technique.
b.
The medial lip is rotated downward to fill the cleft defet.
c.
A small pennant-shaped C-flap can either be rotated to create
the nasal sill or used to lengthen the columella.
d.
Does not violate Cupids bow or the philtral dimple____
e.
Difficult for wider clefts.
f.
Common fitfall is inadequate flaprotation leading to nothing and
in-adequate vertical lip length.
1)
2)

2.

Repeat advancement or a small Z-plasty at the vermilion border can

be performed.
Better results are obtained if adequate rotation is permormed at the
time of the original operation.

Poople repair
a.
b.

Preserves the integrity of the aesthetic unit at the columellarlabial junction.

Allows lengthening of the lip woithout extending the
advancement flap up on the ala or encroaching on horizontal lip
length.

VIII. Types or repair (Contd)

E.

Bilateral cleft lip repair

1.
2.
3.

The premaxillary segment is often a greater

problem than in a unilateral cleft lip.
Consider taping, lip adhesion, or presurgical
orthodontics
Most common techniques

a.
b.
c.

d.

Dissect the prolabium to maintain a central skin

flap to resemble the philtrum.
Deepithelialize the remainder of the prolabium
Use the prolabial vermilion to create a labial sulcus,
not for the final lip the lateral lip segments, not
from the prolabium.
Columellar lengthening may be performed at the
time of lip repair or as a secondary procedure.

IX. Postoperative care

A.

Orders
1.
2.

B.
C.

Arm restraints (no-nos) for 3 weeks to

prevent disruption of repair.
Specialized nipple/bottle to decrease
sucking effort when bottle-feeding.

Leave Steri-Strips in place over the

incision for reinforcement.
Follow up in 1 week for suture
removal if nonabsorbable skin
sutures were used.

Pearls
Preoperative
1. Practice lip markings and cuts on
foam first
2. Do not forget to assess for an
adequate bony platform and the
need for orthogmatic surgery when
assessing cleft nasal deformities.

Pearls (Contd)
Intraoperative
1. Mark several times, cut once.
2. Beaver scalpel blades are helpful.
3. Line up the white roll first, placing a
stitch above and below the white roll,
then reapproximate the wet dry border.
4. Bilateral cleft: Do not use the vermilion
of the premaxillary segment in the final
vermilion. It tends to look like an
abnormal, dry patch postoperatively.

Pearls (Contd)
Postoperative
1. Instruct the parents to hold off
feeding prior to the clinic
appointment. In the clinic, the baby
will stay quet during feeding usually
just long enough for suture removal.
2. Keep a Steri-Srip tape in place for 1
to 2 weeks for support.