TIMING & SEQUENCE

OF TREATMENT FOR
THE
CLEFT
PATIENT

Constantinos Laskarides DMD, DDS, PharmD, FICD

Tufts University Tufts Medical Center
ORAL & MAXILLOFACIAL SURGERY
Assistant Professor, Course Director
Diplomate, American Board of Oral & Maxillofacial Surgery

Types of cleft deformities

Cleft lip (unilateral or bilateral)
Cleft lip and alveolus (unilateral or bilateral) never
alveolus only
Unilateral cleft lip and palate
Bilateral cleft lip and palate
Cleft palate only
Submucosal clefts
Other facial clefts

Types of cleft deformities

Types of cleft deformities

Types of cleft deformities

The Millard modification of the Kernahan classification system

The Paul Tessier

classification system

Incidence
Cleft lip or Cleft lip and palate
Caucasian: 1 in 1000 births
African-Americans: 1 in 2000 births
Asians: 1 in 500 births

Incidence
Cleft palate only:
1 in 2000 births
isolated cleft palate not racially
influenced

Incidence
U.S.A.
1 in 750 births the overall
rate for all
clefts

Sex Differences
Cleft lip

Cleft lip/palate

males

20%

48%

females

12%

20%

Female to male 2:1 with cleft palate only

Frequency of oral clefts in relatives

based on the proband's
phenotype
Relative

CL
(percent
)

CL/P
(percent)

CP
(percent)

Sibling

2.5

3.9

3.3

Half sibling

1.0

0.5

1.0

Parent

2.5

2.5

2.1

Offspring

3.5

4.1

4.2

Niece/nephew

0.9

0.8

1.1

Aunt/uncle

0.6

1.1

0.6

First cousin

0.3

0.5

0.4

CL: cleft lip, CP: cleft palate, CL/P: cleft lip with or without cleft palate

Embryology
Fourth Week
paired mandibular
processes fuse
the tongue develops from
the first branchial arch
(anterior 1/3) and the third
branchial arch (posterior
1/3)
Mandible formed from the
mandibular processes

Embryology
Fifth Week
medial and lateral nasal processes arise
from the nasal placode and the nasal pits
(nostrils)
lateral nasal processes and maxillary
processes fuse (epithelial remnant becomes
the nasolacrimal duct)
eyes develop

Embryology
Sixth Week
paired medial nasal processes fuse to form
the intermaxillary segment (philtrum,
primary palate, alveolus and incisor teeth)
medial nasal processes fuses with the
maxillary process to form the upper lip
lateral nasal process moves superiorly and
becomes alar of the nose

Embryology
Sixth Week
maxillary and mandibular processes fuse to
form the corners of the mouth
lateral palatine processes develop from the
maxillary process
the face broadens by 2/3
ears develop

Embryology
Seventh Week
The external face
acquires a more
normal appearance
The lateral palatine
processes grow
medially and then
down along the
tongue
The tongue is high
and narrow in the
midline

Embryology
Eight to Twelve Weeks
the tongue descends and broadens, and the
lateral palatine shelves elevate toward the
midline from posterior to anterior
the secondary, then primary palates fuse
later the nasal septum fuses with the palate
Palate formed by the median nasal process
(primary palate) and the lateral palatine
processes (secondary palate)

Embryology
Eight to Twelve Weeks
Primary palate- mass of tissue arising from the
median nasal process, contains the four incisor teeth
Secondary palate- mass of tissue that forms from the
fusion of the bilateral lateral palatine shelves

Embryology
Eight to Twelve Weeks
Maxilla formed from the frontal
prominence, medial nasal processes, lateral
nasal processes and maxillary processes
during this period the entire process is
complete (in terms of the formation of the
cleft lip and alveolus deformity)

Embryology
Cleft Palate
In the fetus, the tongue lies
between the two palatine
shelves. As the head of the
fetus begins to straighten, at
about ten weeks, the tongue
drops down. This action
allows the palatal processes to
meet in the midline with the
vomer. This then fuses with
the primary palate. At eleven
weeks the fusion is usually
complete.

Embryology
Cleft Lip and/or alveolus
Median nasal process gives rise
to lip, alveolus and primary palate
Primary palate is that portion
existing anterior to the
nasopalatine foramen
Cleft lip and alveolus form at
junction of median nasal process
and maxillary process

Etiology
Genetic defects

Proliferation defects, eg, sonic hedgehog gene

Extracellular matrix defects, eg, TGF-alpha variant
Differentiation defects, eg, TGF-beta gene
Interferon regulatory factors, eg, IRF-6

Environmental agents
Medications (phenytoin, sodium valproate, methotrexate, diazepam, other)
Cigarette smoking (hypoxia, cadmiun)
Alcohol (alterations in cell membrane fluidity or reduced activity of specific
enzymes such as superoxide dismutase )
Folate deficiency (essential component in the process of DNA methylation)
Other (maternal obesity)

The interdisciplinary cleft team

Audiologist
Geneticist /
dysmorphologist
Nurse
Oral & maxillofacial
Surgeon
Orthodontist
Otolaryngologist
Pediatric dentist

Pediatrician
Plastic surgeon
Prosthodontist
Psychologist
Social worker
Speech/language
pathologist

Fetal stage
MRI & Ultrasonographic identification
of cleft and associated anomalies
detection rate was 80% when the ultrasound was
done after 20 weeks of gestation.

Counceling
Selected intauterine surgical repair of
cleft (under investigation)

unilateral complete cleft

lip. late second trimester

T2 weighted MR imaging of fetus at 20

weeks. micrognathia and tongue is
high in the oropharynx, no soft palate

3D image, early second trimester.

unilateral complete cleft lip

Primary Care of the

Newborn Cleft Child

FEEDING:
aspiration is a common problem
Semi upright position (45-60)
Soft lambs nipple
Enlarge nipple opening with an X
Syringe NG tube (rare)
Hyper alimentation (rare)

Primary Care
of the Cleft Child

AIRWAY :
usually only a problem in Pierre-Robin patients
(obstructive sleep apnea
sleep study)
Face down position
Tongue / lip adhesion
Nasogastric tube as an airway
Tracheostomy

Primary Care
of the Cleft Child
APPLIANCES (palatal)
Feeding obturator (passive)
Expansion appliances (to keep lateral
processes apart). Active appliance (i.e.
Latham) may be also used to decrease the
distance between the alveolar segments or
retroposition the projecting premaxilla (BL
cleft lip) prior to surgical repair.

Primary Care
of the Cleft Child

Primary Care
of the Cleft Child

Primary Care
of the Cleft Child

Primary Care
of the Cleft Child

Approaches to Surgical
Management

Anatomy

Premaxilla outwardly rotated & projecting.

Lateral maxillary element retropositioned.
Inf. edge of septum dislocated
Unilateral shortness of columella
Lower lateral cartilage attenuated, its
medial crus lower in the columella. Lateral
segment flattened and spread across the cleft
at an obtuse angle.
Alar base rotated ourwardly in a flare.
Vestibular lining deficient on cleft side.
Orbicularis oris in lateral element ends
upward inserting into the alar wing
Philtrum is short
White roll alterations

Anatomy

Cleft Lip

10 pounds
Hbg 10 gms
10 weeks of age
WBC: 10,000

Cleft Lip
Surgical Goals
Narrow the prolabium
Reconstruct the orbicularis oris (introduce
muscle under the prolabium)
Utilize lateral lip vermilion for the central lip
Establish good lip form & function
Restore nasal form
The rotation-advancement Millard repair is the most commonly
employed technique. Other: triangular flap (Tennison),
quadrangular (Skoog)

Cleft Lip

Identify landmarks
Mark important points
(tattoo)
Draw flaps
Inject L.A. (wait 7 min.)

Cleft Lip
Differences of the bilateral cleft
no muscle in prolabium
shortened philtrum
needs advancement but no
rotation flap
poor nasal result

Cleft Lip
Lip adhesion procedure
Can be used for wide unilateral or bilateral clefts
Narrows the alveolar cleft
Lessens the need for underlying lip elements
Results in less tension on repaired lip
Delay definitive lip repair until 3 months of age
Short operation
Especially effective where no appliance therapy is available

The Cleft Nasal Airway

nasal valve: smallest cross-sectional area in non-cleft population.
In cleft patients airspace may be reduced in other areas as
well
difference in airway size between cleft and non-cleft nose: 30%
high prevalence of mouth breathing (impairment of nasal airway)
the possitive effect after surgical intervention may be short-lived.
Nasal airway does not seem to change in size with age.
(Warren et al, J. Dent. Res. 71:746, 1992)

adults had the same prevalence of mouth breathing as did children.

Improvement with growth not sufficient to induce a change in
breathing mode.
(Hairfield et al, Cleft Palate J. 25:135, 1988)

The Cleft Nasal Airway

once the pattern of mouth breathing is established, change does
not readily occur even when the airway improves. Obligatory
mouth breathing is replaced by habitual.
a pharyngeal flap may not necessarily alter breathing mode
a pharyngeal flap may result in sleep apnea especially in children
with hypertrophied adenoid tissue or enlarged tonsils
speech aid prostheses (for VPI) reduce the nasopharyngeal airway
by 30%
orthodontic or surgical maxillary expansion has a beneficial effect
on nasal respiration

Otolaryngology
Cleft patients are at risk for middle ear disease and hearing loss
Hearing assessment by auditory brain stem response indicated
Other causes of conductive hearing loss may be present (infants
with syndromes, like Treacher-Collins)
Routine audiologic and otologic evaluations
Most infants with CP require placement of ventilation tubes

Cleft Palate
Surgical Goals
Establish competent velopharyngeal mechanism
Separate oral from nasal cavities (improve speech
and deglutition)
Improve eustachian tube function (preserve hearing)
Preservation of facial growth (esthetics)
Allow for a functional occlusion and esthetic
dentition

Cleft Palate

Trend towards single stage closure of palate at 6

to 12 months of age.
Push back palatoplasty most frequently used
technique
Furlow Z-plasty gaining in popularity

Cleft Palate
Additional procedures
surgical closure of palate

for

tongue flaps
temporalis muscle flap
FAN flap (facial artery and buccinator muscle)

Submucous Cleft
Musculature does not meet at midline
VPI may be present (more apparent after T & A)
Bifid uvula
V-notch in hard palate
Prominent median raphe
Surgery reserved for symptomatic children
Around 3 years of age

Submucous Cleft

Velopharyngeal Incopetence
Factors
1. Short soft palate
2. Large (deep) pharynx
3. Scarred immobile soft palate
4. Hypoplastic musculature (tensor, levator and
muscularis uvulae)
5. Soft palate sensory or neuromuscular deficits
VPI can be of structural, neurogenic, and mislearning
or functional origins

Velopharyngeal Incopetence
Evaluation
1 3 years of age screening and Tx by speech-language
pathologist.
nasopharyngoscopy (3 4 years)
videofluoroscopy

Velopharyngeal Incopetence
Treatment options
speech therapy
prosthetics: - obturator with speech bulb
- palatal lift device
They require adjustments. Removal at night to prevent OSA
posterior wall augmentation: fat, adjacent soft tissue, paraffin,
(Silastic, Teflon, Proplast restricted by FDA)
autologous cartilage appears to have an advantage
(less than 5mm of VPI, less than 4mm passavants ridge formation,
implant must equal twice the amount of VPI)
pharyngeal flap (most popular method)
sphincter pharyngoplasty
palatal lengthening (V-Y pushback, Furlow Z-plasty)

Around
Around
55
years
years

Velopharyngeal Incopetence
Superiorly based pharyngeal flap

Velopharyngeal Incopetence
Superiorly based pharyngeal flap

Velopharyngeal Incopetence
Furlow Z-plasty

Creates empty space between flaps and bony hard palatal vault

Velopharyngeal Incopetence
Sphincter pharyngoplasty

Velopharyngeal Incopetence
V-Y pushback procedure (Wardill-Kilner)

(The gain in palate length has not been demonstrated to improve VP

function. Also higher incidence of fistula formation)

Interceptive orthopedics
transverse expansion and protraction
(Facial Mask), 6-7 years
GOALS
Eliminate anterior crossbite
Eliminate posterior crossbite
Create optimal space to permit
spontaneous eruption of the incisors
Improve nasal respiration
Improve tongue placement

Alveolar bone grafting

Surgical goals
stabilize dental osseous segments
provide for nasal alar base support
prevent tooth loss
oronasal fistula clossure
improve alveolar ridge form
Presurgical orthodontic treatment may be indicated
(includes alignment of maxillary incisors)

Alveolar bone grafting

Timing
Primary bone grafting - less than 2 years
Early secondary grafting - 2 to 5 years old
Secondary grafting - 7 to 12 years old (prior to
cuspid eruption). Supernumerary teeth
extracted at least 6-8 weeks prior.
Late secondary grafting - adult

Alveolar bone grafting

Donor Site Options

Tibia
Mandible
Rib
Cranium
Iliac Crest (anterior or posterior)

Alveolar bone grafting

Orthognathic surgery

Stal et. al., Clinics in Plastic Surgery

1998, 25:4 493-507

Fistulas
technically difficult due to scar tissue
may require mobilization of large flaps
avoid closure until arch expansion is completed