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By

Mo`men Mohmed
Bakr
History of the Procedure

Chinese physicians were the first to describe the technique of


repairing cleft lip. The early techniques involved simply excising
the cleft margins and suturing the segments together. The
evolution of surgical techniques during the mid-17th century
resulted in the use of local flaps for cleft lip repair. These early
descriptions of local flaps for the treatment of cleft lip form the
foundation of surgical principles used today
Tennison introduced the triangular flap technique of unilateral cleft lip

repair, which preserved the Cupid's bow in 1952. The geometry of the

triangular flap was described by Randall, who popularized this method of lip

repair. Millard described the technique of rotating the medial segment and

advancing the lateral flap; thus, preserving the Cupid's bow with the

philtrum. This technique has resulted in improved outcomes in cleft lip

repair
Epidemiology and genetics
A. Incidence of cleft lip and of cleft lip and palate

1. The overall incidence is 1 in 1,000 live births.

2. White ancestry: 1 in 750 live births.

3. Asian ancestry: 1 in 500 live births.

4. African ancestry: 1 in 2,000 live births.


B. Demographics

1. Male-to-female ratio of 2:1.

2. The ratio of left (L) to right (R) to bilateral (B) clefts (L:R:B):
6:3:1.

3. The ratio of CLP to CL is 2:1.

4. Three percent are syndromic.


5. Risk factors

a. Medications:

a.Phenytoin, methylprednisolone (Solu-


Medrol), steroids, phenobarbital, diazepam, and isotretinoin.
b. Smoking.
c. Parental age, especially father's age, or both mother and father over
30 years old.
d. Family history.

If parents have one child with CLP: 4%.

If parents have two children with CLP: 9%.

If one child and one parent have CLP: 14% to


17%.

N.B. Most cases are sporadic (and multifactorial), but may be X-linked,
autosomal

dominant (Van der Woude's syndrome) or familial


Anatomy
A. Normal lip anatomy
1.Topographic landmarks

a. Nasal alae.

b. Columella.

c. Philtral columns.

d. White roll: Well-defined mucocutaneous or vermilion-cutaneous border.

e. Vermilion: Red portion of lip.

f. Tubercle.

g. Cupid's bow.

h. Wet-dry border: The vermilion-mucosa junction is the border between keratinized and
nonkeratinized mucosa.
2. Musculature.

a. Orbicularis oris.

(1)Fibers cross (decussate) in the midline and create the opposite


philtral columns.

(2) Functions as a sphincter (deep fibers) and for speech (superficial


fibers).

b. Levator labii superioris.

(3)Inserts into the dermis at the vermilion border and the lower edge of
the
philtral columns.

(2) Elevates the upper lip.

c. Nasalis or depressor septi nasi muscle: The fibers run from the alveolar
bone into the medial crural footplates, skin of the columella and the tip of
3. Normal measurements.

a. Vertical length (height) of the upper lip.

(1) Newborn: 10 mm.

(2) Age 3 months: 13 mm.

(3) Adult: 17 mm.

b. The distance between the peaks of Cupid's bow: Approximately 3 mm at 3 months.

4. Arterial blood supply: The labial artery, bilaterally.

5. Sensory innervation: The trigeminal nerve, cranial nerve (CN) V, maxillary division (V2).

6. Motor innervation: The facial nerve, CN VII, zygomatic and buccal branches.
B. Cleft lip anatomy.

1. Alterations in the orbicularis oris, levator labii, and nasalis result in


disruption of continuity, orientation, and quality of the muscles.

a. Fibers are disoriented and run parallel to the cleft margin.

b. Fibers insert into the alar base on the cleft (lateral) segment and into
the columella in the noncleft (medial) segment, as well as
intradermally.

c. Incomplete clefts.

(1)Simonart's band consists of a skin bridge across the nasal sill. It does not
usually contain any significant muscle mass.

(2) Some fibers may cross the cleft, if the cleft is less than two-thirds of lip
height.
d. Bilateral complete clefts: No muscle tissue is present in the prolabium.

2. Vertical lip length is decreased: Cupid's bow and the lip are rotated on both the

lateral, cleft side as well as the medial side.

3. Disrupted Cupid's bow.

4. The alveolus and nostril floor are open in a complete cleft lip.

5. The premaxilla is rotated and protruding, especially in bilateral cleft lip, often
with

collapse of the lateral segment of the cleft side(s).


6. Associated cleft lip nasal abnormalities .

a. Hypoplastic, flattened alar dome on the affected side.

b. Lack of upper lateral cartilage overlap of lower lateral cartilage.

c. Subluxed lower lateral cartilage with alar base displaced


posteriorly.

d. Hypoplastic bony foundation (maxilla).

e . Flattening of the nasal bones.

f . Shortened columella, especially in bilateral cases.


Classification
A. Extent of the cleft: Complete versus incomplete

Complete cleft lip .1

a. Complete disruption of the soft tissues to the nasal


floor.

b.Tends to be wider than incomplete clefts, with greater


nasal deformities.
1. Incomplete cleft lip

a. Disruption of the soft tissues to varying degrees.


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

1. Unilateral cleft lip

2. Bilateral cleft lip

a. May have a complete or incomplete cleft on both sides, or a


combination.

b. More likely to be complete clefts and are often wide.

c. In bilateral complete clefts, the prolabium lacks muscle tissue, and


therefore lacks philtral columns.

C. Alveolar segments

3.Narrow versus wide cleft


Staging of intervention
A. Initial evaluation
1. Reassure the parents and family that they are not to
blame.
2. Explain the stages and operations that should be expected
throughout the child's lifetime.
3. Evaluate for associated anomalies.
4. Consultations
a. Genetics, for evaluation and possible counseling
b. Social work
c. Feeding/nutrition

(1) The child may need special nipples or bottles (e.g., cross-cut nipple).

(2) Monitor for appropriate weight gain.

d. Otolaryngology: Children with cleft lip and palate have a high incidence

of eustachian tube dysfunction, and otitis media, requiring close follow-up.

(3)The child may need myringotomy tubes.

(4) If untreated, repeat otitis may affect hearing and speech development.
B. Wide clefts (>1 cm)

1. Goal: Bring the segments closer together to facilitate a tension-free repair.


a. Has not been shown to change skeletal development in the anteroposterior
direction.

2. Passive: Preoperative taping


a. Steri-Strip tapes applied across both segments of the lip.
b. Requires reliable parents who can reapply the tape and keep it on .
3. Passive: Lip adhesion operation
a.Suturing the edges of the cleft together is performed under
anesthesia.
b.The lip repair is performed once the segments have moved closer
together.
c. Variable success.
C. Repair
1. Timing (controversial)
a. Repair at 3 months is generally accepted.
b. Some argue for earlier repair in order to produce better
scars.

2. Rule of tens: For increased anesthetic safety, an infant


should
a. Be 10 weeks old.
b. Weigh 10 pounds.
3. Cleft palate repair and secondary alveolar
grafting .

4. May also choose to address the cleft nasal deformity at time of


lip repair
Goals of repair

The basic goal of primary cleft lip repair is to


reconstitute oral competence and a dynamic muscular
sphincter with the orbicularis oris muscle. Equally
important is the achievement of cosmetic
reconstruction of the lip appearance. The focus is on
1)correct alignment of Cupid’s bow.
2)symmetric reconstruction of the vermilion.
3)and accurate construction of the philtral
column.
Additionally, the goals of primary cleft rhinoplasty
performed at the time of initial lip repair are important to
achieve.
1)nasal function is optimized by closing the nasal floor
and nostril sill. Establishing a correct position for the alar
base is beneficial in overcoming the nasal asymmetry.
2)improve the position and contour of the lower lateral
cartilages. This enhances nasal aesthetics in the short term
and sets the stage for future nasal reconstruction.
Ideally these goals are achieved without causing excessive
scar tissue formation, wound breakdown, or restriction of
maxillofacial growth.
Types of repair
A. Straight-line repair
1.Historically, the first cleft lip repairs relied
on freshening the edges of the cleft and
suturing them together. These have been
largely replaced by various Z-plasty-based
techniques.
2. Rose-Thompson repair
a. 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).
b. Fusiform excision with straight-line
closure.
B. Quadrangular flap
1.Proposed by
LeMesurier
and Hagedorn.
2.Cupid's bow is derived
from the lateral lip.
3. 90-degree Z-plasty.
4.Violates Cupid's bow
and philtral dimple.
5.Has a tendency to
produce a long lip.
C. Triangular flap

The triangular flap repair was initially


described in 1952 by In
1959, Randall described
Tennison, a mathematical
approach to the triangular flap that was on
the basis of precise measurements.

This repair technique is conceptually similar to


the rotation advancement repair. The
primary difference is that the rotation back-
cut in the noncleft segment is performed
more inferiorly, closer to the vermilion
border. Similarly the advancement segment
on the cleft side is designed to occur
inferiorly near the vermilion cutaneous
border.
Markings for unilateral cleft lip repair
with the triangular flap technique.
Marked reference points indicate the
following:
1. Columellar base noncleft side
2. Columellar base cleft side
3. Alar base noncleft side
4. Alar base cleft side
5. Light scroll mark noncleft side
6. Light scroll mark cleft side. This also
beco m es the m edial base of the
equilateral triangle flap
7. Peak of Cupid’s bow noncleft side.
8. Low point of Cupid’s bow .
9. Apex of the equilateral triangle flap
10.Lateral base of equilateral
triangle flap .
11.Marks the length of back cut
in noncleft side.
There are 3 main advantages of the triangular flap
repair technique.

First, it is readily used to close wide clefts without having to perform


lip adhesion or presurgical tissue manipulation.

Second, the operation is done on strictly geometric methods of


mathematical principles and measurements, leaving not much room for
errors in judgment when compared to the “cut as you go” techniques.
Therefore, many experts consider the triangular flap
technique to be well suited for less experienced surgeons.

A third possible advantage of this technique is that the zigzag scar


prevents scar contracture and lip shortening leading to a vermilion
notch that can be sometimes observed in the rotation advancement
technique.
The main disadvantage of the
triangular flap

repair technique is that the


philtrum on the cleft side is
violated by the triangular
flap. Some authors believe
this leaves a more noticeable
scar.

Another potential disadvantage


is the difficulty in modifying
the repair or performing
secondary revision at a later
stage due to the zigzag scars.
2. Skoog repair
a. Consists of two
Z- plasties.
b.Violates Cupid's bow
and the philtral
dimple.
D. Rotation advancement.
The rotation advancement repair of the unilateral cleft
lip deformity as described by Millard is the most
commonly used method of repair at present in the USA.

The main advantage of this technique is its flexibility and


application. The rotation advancement technique relies on a
“cut as you go” strategy that allows continuous modifications
during the design and execution of the repair. It does not
adhere to strict geometrical principles or measurements.

Another advantage is that the suture line approximates a new


philtral column. The aesthetic philtral is
violated, and this tends to create a scar that
subunit not
camouflaged. is
more
Minimal tissue is discarded during the rotation advancement
technique, and this tends to put less tension on the
Furthermore, the rotation advancement technique allows easy access to the alar
cartilages for primary rhinoplasty to be performed at the time of lip repair. This
early repair of the nasal deformity can be successful in achieving a more
symmetric nasal appearance and possibly avoiding the intermediate rhinoplasty
step for many of these children.

The primary disadvantage of the rotation advancement technique is that


experience and artistry are required to achieve optimal results. The operation
relies on the surgeon’s spatial awareness and judgment.

Additionally, the vertical scar that occupies the philtral column can be subject to
wound contracture. Such contracture can lead to shortening of the lip on the cleft
side with resultant vermilion notching and whistle deformity.

Finally the surgeon needs to be cautious when using the rotation advancement
technique to avoid excessive narrowing of the nostril sill on the cleft side. This can
lead to nasal vestibular stenosis as the wound matures.
Markings for unilateral cleft lip
repair with the rotation
advancement technique.
1. Center (low point) of Cupid’s bow
2. Peak of Cupid’s bow lateral,
noncleft side
3. Peak of Cupid’s bow, medial,
noncleft side
4. Alar base, noncleft side.
5. Columellar base noncleft side; X. Back
cut point, noncleft side.
6. Oral c ommissure noncleft
side.
7. Oral comm issure cleft side; 8.
Light scroll, cleft side.
9. Medial tip of advancement flap,
cleft side.
10.Midpoint of alar base cleft side.
11.Lateral alar base, cleft side.
12.Lateral alar base, extent of
E. Bilateral cleft lip repair
1.The premaxillary segment is often a
greater problem than in a unilateral
cleft lip.
2. Consider taping, lip adhesion, or
presurgical orthodontics (see above).
3. Most common techniques
a.Dissect the prolabium to maintain a
central skin flap to resemble the
philtrum.
b.Deepithelialize the remainder of the
prolabium.
 c. Use the prolabial vermilion to

create a labial sulcus, not for the


final lip vermilion. The final lip
vermilion is composed only of
vermilion from the lateral lip
segments, not from the prolabium.

 d. Columellar lengthening may be

performed at the time of lip


repair or as a secondary
procedure.
Postoperative care
A. Orders
1. Arm restraints for 3 weeks to prevent disruption of
repair.
2.Specialized nipple/bottle to decrease sucking effort
when bottle-feeding.
3. Breast-feeding is controversial; based on surgeon
preference.

B.Leave Steri-Strips in place over the incision


for reinforcement.

C.Follow up in 1 week for suture removal if nonabsorbable


skin sutures were used.
Complications of cleft lip repair
1) inadequate reapproximation of the orbicularis oris muscle
with a failure to reconstitute a competent oral
sphincter. This can result in a visible muscle bulge that
is readily apparent under the skin of the repaired lip on
dynamic motion.
2) inaccurate alignment of the vermilion-cutaneous
junction leaving a small step-off deformity that is readily
noticeable even to the untrained eye.
3) vertical scar contracture or inadequate rotation
can cause shortening of the lip segment leading to
a notch in the vermilion and a whistle deformity.
4) scar contracture causing a narrow nostril sill
with vestibular stenosis.
5) wound healing complications such as dehiscence
and scar widening

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