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Cleft Lip

Dr.Mgs. Roni Saleh,

I. Embryology
A. The critical developmental period of the lip and
primary palate occurs during weeks 4 to 6 of
B. 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.
C. 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. 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 lilve births.
B. Demographics
1. Male-to-female ratio of 2:!
2. The ratio of left (L) to right to bilateral (B) clefts
(L:R:B): 6:3:1
3. The ratio of CLPmto CL is 2:1
4. Three percent are syndromic.

II. Epidemiology and Genetics
5. Risk factors
a. Medications: Phenytoin, methylprednisolone (Solo-Medrol), steroids,
phenobarbital, diazepam, and isotretinoin.
b. Smoking
c. Parental age, especially fathers age, or both mother and father over 30
years old.
d. Family history (see Genetics)

C. Genetics
1. The risk of having a child with CLP
a. If parents have one child with CLP: 4%
b. If one parent has CLP: 2% to 4%
c. If parents have two children with CLP:9%
d. If one child and one parent have CLP: 14% to 17%.

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

III. Anatomy
A. Normal lip anatomy
1. Topographic landmarks
a. Nasal alae.
b. Columella
c. Philtral columns.
d. White roll: Well-defined mucocutaneus or vermilion-
cutaneous border.
e. Vermilion: Red portion of lip.
f. Tuberle.
g. Cupids bow
h. Wet-dry border: The vermilion-mucosa junction is the
border between keratinized and nonkeratinized mucosa.
III. Anatomy (contd)
2. Musculature
a. Orbicularis oris
1) Fibers cross (decussate) in the midline and create the
opposite philtral columns.
2) Functions as a sphincters (deep fibers) and for speech
(superficial fibers).
b. Levator labli superioris.
1) Inserts into the dermis at the vermilion vorder 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 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) Newborn: 10 mm.
2) Age 3 months: 13 mm.
3) Adult: 17 mm
b. The distance between the peaks of Cupids bow:
Aprroximately 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.
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. 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 non cleft
(medial)segment, as well as intradermally.
c. Incomplete clefts.
1) Simonarts band consist 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
III. Anatomy (contd)
2. 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.
3. Disrupted Cupids 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
collapase of the lateral segment of the cleft
III. Anatomy (contd)
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
c. Subluxed lower lateral cartilage with alar base displaced
cephalad and posteriorly.
d. Hypoplastic bony foundation (maxilla).
e. The caudal septum is pulled toward the noncleft side.
f. Flattening of the nasal bones.
g. Shortened columella, especially in bilateral cases.
IV. Classification
A. Extent of the cleft: Complete versus
1. Complete cleft lip
a. Complete disruption of the soft tissues to the nasar floor.
b. Tends to be wider than incomplete celfts, with greater
nasal deformities.
2. Incomplete cleft lilp
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
IV. Classification (Contd)
B. Location of the cleft: Unilateral versus bilateral
1. Unilateral cleft lip
2. Bilateral cleft lip
a. May have complete or incomplete cleft on both sides,or a
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. Narrow versus wide cleft
2. Collapse versus no collapse
V. Syndromes associated with cleft
lip and palate
A. Van der Woudes syndrome
1. Autosomal dominant, with variable
2. Associated with CLP or CP (40%-50%
3. Associated with lip pits (accessory salivary
glands, 70%-80% penetrance).
4. May also have absent second molar,
syndactyly, abnormal genitalia, and popliteal
V. Syndromes associated with cleft
lip and palate (Contd)
B. Waardenburgs syndrome
1. A group of anomalies arising from abnormal
development and migration of neural crest
2. Features may include cleft lip, cleft plate.
C. Down syndrome (trysomy 21)
D. Trisome 13
V. Syndromes associated with cleft
lip and palate (Contd)
E. Sticklers syndrome
1. 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
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. 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.
2. May be a part of multiple different syndromes or may be an
isolated finding.
3. Treatment.
a. Prone positioning to help move the tongue out of the aierway,
the most conservative approach.
b. Supplemental oxygen.
c. Tongue-lip adhesion.
d. Mandibular distraction osteogeneisi
e. Intubation/tracheostomy
V. Syndromes associated with cleft
lip and palate (Contd)
4. Patients may show catch-up mandibular
growth, depending on their syndromic
5. Palysomnogram: Necessary to evaluate for
desaturations as well as apneic events
V. Syndromes associated with cleft
lip and palate (Contd)
G. Velocardiofacial syndrome
1. Autosomal dominant inheritance: Fluorescent in situ
hybridization (FISH) may show an abnormality in chromosome
2. Characteristic feature include the following.
a. Cleft palate.
b. Congenital heart disease.
c. Broad nasal dorsum and elongated face.
d. Narrow, down-slanting palpebral fissures.
e. Velopharyngeal insufficiency is common, even with a submucous
cleft palate.
f. 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
V. Syndromes associated with cleft
lip and palate (Contd)
H. Median cleft lip
1. Rare
2. A different entity from the typical cleft lip; more
accureately considered a median craniofacial cleft
(Tessier type zero).
3. Associated with a group of syndromes (median
cerebral facial dysgenesis) that involve more severe
deformities of midline CNS and facial structures.
4. Further workup is needed, including a formal CNS
5. May be associated with holoprosencephaly, pituitary
problems, and a limited lifespan.
VI. 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 childs lifetime
3. Evaluate for associated anomalies.
4. Consultations
a. Genetics, for evaluation and possible counseling
b. Social works
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 therefore otitis media, requiring close
1) The child may need myringotomy tubes.
2) 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. Has not been shown to chanbe skeletal
development in the anteroposterior direction.
b. Does not seem to prevent future crossbite.
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 at all times
VI. Staging of intervention (Contd)
3. Passive: Lip adhesion operation
a. Suturing the edges of the cleft together is performed under
b. The definitive lip repair is performed once the segments
have moved closer together.
c. Variable success.
4. Active: Latham-type device
a. An orthodontic appliance that must be placed onto the
palatal segments under anesthesia.
b. Parents turn a screw daily, which slowly brings the palatal
segments into better alignment.
c. Removed at the time of definitive lip repair.
VI. Staging of intervention (Contd)
C. Repair
1. Timing (controversia)
a. Repair at 3 months is generally accepted.
b. Some argue for earlier repair in order to produce better
2. Rule of tens: For increased anesthetic safety, an
infant should
a. Be 10 weeks old
b. Weigh 10 pounds.
c. Have a hemoglobin level of at least 10 mg/dL.
3. Cleft palate repair and secondary alveolar frafting
4. 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. Alar bases.
b. Columella.
c. Philtral columns.
d. D. Peak of Cupids bow midline on the medial segment.
Measure the anticipated distance for the new Cupids bow
(approximately 3-4 mm).
e. Peak of Cupids bow on the lateral segment.
2. 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
B. Mark lines for expected repair type.
C. Only after marks are completed, infiltrate
tissue with local anesthetic to avoid distortion
of anatomy and measurements.
D. Goals of repair
1. Reconstitute Cupids bow
2. Minimize scarring
3. Produce a slight pout of the tubercle.
4. Produce functional continuity of the muscles
5. Recreate symmetry.
VIII. Types or 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 replace by various Z-plasty-base
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.
VIII. Types or repair (Contd)
B. Quadrangular flap
1. Proposed by LeMesurier and Hagedorn.
2. Cupids bow is derived from the lateral lip.
3. 90-degree Z-plasty.
4. Violates Cupids bow and the philtral dimple.
5. May also have problem with a long lip.
VIII. Types or repair (Contd)
C. Triangular flap
1. Proposed by Tennison and Randall
a. The Z-plasty is place at the vermilion border.
b. Produces an natural appearing Cupids bow
c. May be used for clefts of all widths.
d. Violates Cupids bow and the philtral dimple.
e. Has a tendency to produce a long lip.
2. Skoog repair
a. Consists of two Z-plasties
b. 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) Repeat advancement or a small Z-plasty at the vermilion border can be
2) Better results are obtained if adequate rotation is permormed at the time of the
original operation.
2. Poople repair
a. Preserves the integrity of the aesthetic unit at the columellar-labial
b. 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. The premaxillary segment is often a greater
problem than in a unilateral cleft lip.
2. Consider taping, lip adhesion, or presurgical
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 the lateral lip segments, not from the
d. Columellar lengthening may be performed at the time of lip
repair or as a secondary procedure.
IX. Postoperative care
A. Orders
1. Arm restraints (no-nos) for 3 weeks to
prevent disruption of repair.
2. Specialized nipple/bottle to decrease sucking
effort when bottle-feeding.
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.
1. Practice lip markings and cuts on foam
2. Do not forget to assess for an adequate
bony platform and the need for
orthogmatic surgery when assessing cleft
nasal deformities.
Pearls (Contd)
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
Pearls (Contd)
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.