Cleft lip and palate

CLEFT LIP AND PALATE
BHARAT POKHREL, MD

CLEFT LIP
• A child born with cleft lip/palate or other
craniofacial anomalies has multiple and
complex problems, including early feeding and
nutritional concerns, middle ear disease,
hearing deficiencies, deviations in speech and
resonance, dentofacial and orthodontic
abnormalities, and psychosocial adjustment
problems.
(American Cleft Palate-Craniofacial Association ACPA)

Cleft Lip and Palate
• Clefts of the lip, alveolus and palate are the
most common congenital malformations of
the head and neck
• CL/P are considered intrinsic disorders of
morphogenesis
• Heterogenous
• 30- 65% are assoc. w/ a syndrome

CLEFT LIP
 The philtrum of the upper
lip develops from the fusion
of the medial nasal process
and the lateral portions of
the maxillary process.
 Generally unilateral
(80%).
 Most common in males on
left side.

UNILATERAL CLEFT
• Lateral displacement of the
premaxilla on the non-cleft
side; frequent upward tilting
of the premaxilla into the
cleft defect
• Underdevelopment of the
maxilla on the side of the
cleft
• Marked malalignment of the
alveolar arches
• Shortened columella,
attenuated lower lat.
cartilage w/ a flared alar base
on cleft side
• Vertical shortness of lip on
cleft side

CLEFT LIP WITH CLEFT PALATE
Clinical – The primary palate (pre-maxilla) also
develops from the medial nasal process. It
starts out as vertical shelves, which must
descend in order to fuse with one another.
Their fusion occurs in a unidirectional direction
(anterior to posterior only). The secondary
palate then fuses with the pre-maxilla.
Tx/Prognosis – Good prognosis with surgical
intervention.

CLEFT PALATE DEFORMITY
• Clefts of the 2ndary palate have
varying deficiencies of bone &
muscle
• Mucosal deficiency is always
present except in a submucus
cleft palate
• Palatal muscles are directed
anteriorly & have an abnormal
insertion into posterior margin
of the bony palate and are
usually hypoplastic

BILATERAL CLEFT LIP & PALATE
• Premaxilla is freq. protruding &
often deficient in bone
• Overlying midportion of the lip
commonly attaches directly to
the nasal tip w/ a nearly total
absence of the columella
• Variably-sized prolabium
containing no muscular
elements; no Cupid’s bow or
philtrum
• Maxillary alveolar arches are
often underdeveloped & are
freq. collapsed in an upward
inward direction

SUBMUCOUS CLEFT
• char. by midline mesodermal
deficiency between the oral
&
nasal mucus membranes inv.
the soft palate & posterior
edge of the hard palate
• there is notching of the
posterior edge of the hard
palate
• Loss of posterior nasal spine
• muscular deficiency of the
soft palate w/ a bifid uvula
• “zona pellucida”

BIFID UVULA
Etiology – Hereditary and
environmental factors.
Clinical – A failure of fusion at
the last phase of development of
the soft palate making the uvula
appear split into two.
T/P – No treatment required.
- Prognosis excellent.

Embryologic Development
of the Face and Palate
Development of the face
– Formed between the 5th and 8th weeks of gestation
– Results from the fusion of
 Two mandibular processes
 One frontonasal process
 Two maxillary processes
– Cleft lip occurs when the fusion process between the
frontnasal masses and the maxillary processes is
interrupted
CD-ROM Ch.11.08 morphing sequence depicting
embryologic development of the human face

Etiologies
1. Genetic disorders
n Factor in over 400 different genetic syndromes
2. Chromosomal aberrations
3. Teratogenically induced disorders
n Environmental teratogens are agents that interfere with or
interrupt normal fetal development
4. Mechanically induced abnormalities
Amniotic rupture, intrauterine crowding, uterine tumors,
irregularly-shaped uterus

Causes
• Oral clefts have been linked to genes located
on more than several chromosomes including
1, 2, 4, 6, and 19, among others

Causes
• Maternal smoking (especially more than
20/day) and exposure to passive smoke
• Drugs: Isotretenoin, phenytoin, warfarin,
ethanol
• Maternal folic acid deficiency
• Ingestion of large quantities of Vitamin A

Method Of Transmission
• Depends on the specific cause of the clefting.
• Multifactorial clefts can exhibit evidence of
autosomal dominant, autosomal recessive,
and sex-linked inheritance patterns
• Spontaneous mutation or mutations in one or
more genes.

Method Of Transmission
• If one of the parent has a cleft lip, his/her
child face a risk of 20%
• If their is one child with cleft lip, the following
child faces risk of 14%
• A non cleft parent with a cleft lip faces a risk
of 4% for the following child

Risk Factors and Percent
Predicted Cleft Recurrence
Affected
relatives
Cleft lip and
Palate
Cleft
palate
General
Population
0.1 0.04
1 sibling 4.4 2.5
1 parent 2.2 6.8
1 sibling,
1 parent
15.9 14.9
2 siblings 9.0 1.0

Pathogenesis
• The face and facial structures are formed out
of three plates, each migrating toward a
meeting point in the middle area of the face.
• The facial structures of the orbicularis muscle
form the lip. They are joined at the philitrum
lines.
• Join by 4th week of pregnancy

Pathogenesis
• The palate is then formed out of the structure
that begins as the tongue and palate.
• Between the fourth and the eighth weeks of
gestation, the tongue drops down and the
palatal segments then move from the sides
and toward the middle, fusing in the centre.

Pathogenesis
• A cleft, therefore, is not something that is
formed, so much as it is something that does
not form.

Normal Process
6 weeks
• Maxillary process
• Lateral nasal process
• Median nasal process
• These three processes join and fuse to form
the primary palate

7 weeks
• Median nasal process and maxillary process
have fused creating upper lip and anterior
maxillary alveolus

8 weeks
• Complex totally fused and mesodermal
migration completed Tongue, which has been
postured superiorly between lateral palatal
shelves of maxilla,
• Moves inferiorly allowing palatal processes to
grow toward midline and fuse, form
nasopalatine foramen to uvula

11 weeks
• Total palatal closure

CLASSIFICATION
I. Cleft lip
A. Unilateral or bilateral
B. Complete or incomplete
II. Cleft palate
A. Location in reference to incisive foramen
1. Primary- anterior
2. Secondary- posterior
B. Unilateral or bilateral
c. Complete or incomplete
III. Submucous cleft palate

Thallwitz Classification
• Also known as the LAHSHAL
• Descriptive classification of
each individual case of cleft
lip and palate.
• simple, concise, flexible,
exact but graphic.
• It describe the site, size,
extent as well as type of
cleft.

• Each area is divided into thirds, and cleft
defects are graded as to how much of each
are affected. Grading is done for both sides as
shown:
(right side) (midline) (left side)
L-lip A-alveolus H-hard palate S-soft palate H-hard
palate A-alveolus L-lip

L = Lip - 1/3 or 2/3 or 3/3
A = Alveolar cleft - 1/3 or 2/3 or
3/3
H = Hard palate cleft - 1/3 or 2/3
or 3/3
S = Soft palate cleft - 1/3 or 2/3
or 3/3

Management of Clefts
• Team approach
Surgical Management
• Lip and primary palate is usually repaired at
around 5 months of age.
• Soft palate repair is usually around nine
months of age.
(Newborn services clinical guidelines , Nov. 2011)

Surgical Management
Primary correction
– Lip surgery by 3 months
– Palatal cleft surgery by 6-18 months
Secondary correction
– Pharyngeal flap
 25% of cases
 Improves velopharyngeal competence
 Done between 6-12 years of age

Cleft lip and palate

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