By:

Dr. Waqar Jeelani

Layout
Introduction
Incidence
Classification
Embryological Background
Etiology
Diagnosis
Problems of individuals with clefts
Treatment of Cleft Lip and Palate
Specialties involved in the treatment of Cleft Lip and Palate
Timings of surgical repair
Cheilorraphy
Palatorrhaphy
Alveolar Cleft Grafts
Secondary Surgical Procedures
Valopharyngeal Insufficiency

Introduction
What is Cleft Lip and Palate?
Congenital abnormal space or gap in the

upper lip, alveolus and palate

Incidence

More common in south far Asians:

1 in 500
Less frequent in Africans:
1 in 2000
Prevalence in Europeans and Americans:
1 in 750
*Prevalence in Pakistan:
1 in 523
*Elahi MM, Jackson IT, Elahi O, Khan AH, Mubarak F, Tariq GB, Mitra A.
Epidemiology of cleft lip and cleft palate in Pakistan. Otolaryngol Clin
North Am. 2007 Feb; 40(1):27-60

Incidence
Boys are more affected than girls by 3:2
Cleft Lip and Palate occur twice as often in

boys as in girls
Isolated Clefts of Palate are more often in girls
75% of Clefts are Unilateral, rest are Bilateral
Left side is more involved than right side

Embryological
Background

Head and Neck of 4-Week Old Embryo

Glossopharyngeal
nerve
Vagus nerve
Trigeminal nerve

Facial nerve

Embryological
Background
Development of the

Lip:
Unpaired Frontonasal

Prominence
Medial and Lateral
Nasal prominences
2 maxillary
prominences
2 mandibular
prominences

Embryological
Background
Fusion defects can occur anywhere

between these prominences

The defect in the fusion between the
frontonasal and maxillary will lead to
cleft lip

Embryological
Background
Development of Palate:
We have two parts of two different embryonic origins:
1 ) primary palate : the triangular part of hard palate anterior to
incisor foramen which originate from the premaxilla ( frontonasal
prominences).
develop between 4th and 8th week of gestation
2 ) secondary palate : remaining part of the hard palate and all soft
palate posterior to incisor foramen which comes from palatine shelves
of the maxillary prominences
develop between 8th and 12th week of gestation

Embryological
Background
Various theories have been given for its
1.
2.
3.
4.

development.
Alteration in intrinsic palatal shelf force
Failure of tongue to drop down
Non fusion of shelves
Rupture of cyst formed at the site of fusion

Classification
We classify as the follows:
its combined (cl+cp) or isolated cleft(cl

or cp)?

is it unilateral or bilateral?
is it complete (if it cross the nasal

philtrum) or incomplete ( if it doesnt

cross the nasal philtrum.

Classification Systems

Lip
Alveolus
Primary
Palate
Hard Palate
(Maxillary)
Hard Palate
(Palatine)
Soft Palate

Karnahans Classification
Millards Modification of
Karnahans Classifcation

Kriens LAHSHAL

L = Lip (right)
A = Alveolus (right)
H = Hard Palate
(right)
S = Soft Palate
(median)
H = Hard Palate
(left)
A = Alveolus
Capital
letter(left)
= complete
L = Lip (left)
cleft

Lowercase letter =
incomplete cleft
Examples
.
or - = normal
LA.l = complete right cleft lip and
alveolus, incomplete left cleft lip
LAHS = complete right unilateral cleft
lip, alveolus, hard, and soft palate

Other Types of Clefts

Microform Cleft:
May look like

a little dent in the red part of the lip

a scar from the lip up to the nostril.

Muscle tissue underneath the cleft

can be affected and may require

surgery

Submucous Cleft Palate:

Midline deficiency or lack of
muscular tissue
Often a submucous cleft palate is
associated with a bifid or cleft
uvula
Posterior nasal spine is almost
always missing
Speech Problems are common

Prenatal
Diagnosis
Cleft lipcan be easily

diagnosed by performing
ultrasonography in the
second trimester
Diagnosing a cleft palate
with ultrasonography is very
difficult
Three-dimensional imaging
has been introduced to
prenatal ultrasonography
diagnostics of cleft
anomalies

Diagnosis

Advantages of Prenatal

Diagnosis:

1.
2.
3.
4.
5.

Time for parental education

Time for parental
psychological preparation
Opportunity to investigate
other associated anomalies
Gives parents the choice of
continuing the pregnancy
Opportunity for fetal surgery

Etiology
Actually no one knows exactly what causes
clefts
Multiple factors may be involved, like:
Genetics (inherited characteristic) from one or both

parents .
Environmental factors
Drugs: corticosteroids (anti-inflammatory), phenytoin
(anticonvulsant), retinoid.
Infections: like rubella during pregnancy.
Alcohol consumption, smoking, hypoxia during
pregnancy, some of dietary and vitamins deficiencies
(like folic acid and vitamin A deficiency)
Maternal Age

GENETICS
CL/P

Normal parents, one child with CL/P

4% risk CL/P in next child
Normal parents, two kids with CL/P
9% risk CL/P in next child
One parent CL/P, no affected kids
4% risk CL/P in next child
One parent CL/P, one child CL/P
17% risk CL/P in next child
Risk of CL/P in siblings increases with severity of deformity
- child with unilateral CL risk CL/P next child 2.5%
- child with bilateral CL/P
risk CL/P next child 5.7%

CP

Normal parents, one child with CP

Normal parents, 2 children with CP
Parent with CP, no affected children
Parent with CP, one child with CP

2% risk of CP in next child

7% risk of CP in next child
6% risk for next child
15% risk for next child

All infants with clefs must be evaluated for

presence of other anomalies
Associated nomalies are more common in infants
with isolated CP

Nonsyndromic Cleft
Pierre Robin Sequence is the
most common associated
nonsyndromic anomaly
is a relative term describing the
small size of the lower jaw ) and
Glossoptosis (is a medical
condition and abnormality which
refers to the downward
displacement or retraction of the
tongue)

Syndromic Cleft
Common Syndromes
Stickler (25% of syndromic CP)
autosomal dominant type 2 collagen gene
Pierre Robin, ocular/hearing/joint

malformations

Velocardiofacial (Shprintzens) 15% of

syndromic CP
Autosomal dominant, variable expressivity,
CATCH 22 Deletions at 22q11,
Facial, cardiovascular, immunologic,
developmental anomalies

Van der Woudes (19% of syndromic CL/P

and CP)
Autosomal dominant
CL/P or CP with bilateral lower lip pits

Problems Associated With

Cleft
Lip
and
Palate
Feeding
Dental problems
Nasal Deformity and Esthetic Problems
Ear Problems
Speech Difficulties
Associated Anomalies

Feeding Difficulties
Cleft lip= makes it more difficult for an

infant to suck on a nipple

Cleft Palate= may cause formula or breast
milk to be accidently taken up into the
nasal cavity
Inability to create negative pressure inside
oral cavity
Frequent regurgitations
Upper respiratory tract infections

Dental Problems
Local Dental Problems:
Congenitally Missing teeth, Hypodontia, Hyperdontia,
Oligodontia
Presence of natal and neonatal teeth
Anamalies of tooth morphology like microdontia, macrodontia
etc
Fused teeth
Enamel Hypoplasia
Poor periodontal support, early loss of teeth
Gemination, Dilacerations
Orthodontics Problems:
Class III tendency
Anterior and Posterior Cross bite
Spacing and crowding

Nasal Deformity and

Facial Disfigurements
Esthetic
Problems
Poor nasal shape
Scar marks of surgeries
Poor lip function during

speech
Poor dental alignment and
smile

Ear Problems
Middle ear disease - 22% to 88%
Conductive hearing loss and chronic

suppurative otitis media may result

Repeated tympanostomy tube placement

Why do cleft kids have

eustachian
tube
Abnormal curvature of the eustachian tube
lumen
dysfunction?

Cephalometric data - width and angulation of

the skull base with respect to the eustachian

tube are different
Abnormal insertions of the tensor and levator
veli palatini muscles into the cartilages and
skull base
Palatal muscle dysfunction

Speech Problems:
Hearing loss hampers proper development of

speech
Velopharyngeal Insufficiency (VPI)
Abnormal air
Poor pronunciation of Bilabial, Labiodental,
Linguoalveolar sounds

Schedule of Treatment
Birth:

Initial Assessment
Pre-surgical assessment

3 Month:

Primary Lip repair

9-18 month:

Palate Repair

2 Year:

Speech assessment

3-5 Year:

Lip Revision Surgery

8-9 Year:

Initial interventional

Orthodontics
Preparation for alveolar bone
grafting

10 Year:

Alveolar Bone Grafts

12-14 Year:

Definite Orthodontics

16 Year:

Nasal Revision Surgery

17-20 Year:

Orthognathic Surgery

Multidisciplinary Cleft Lip

And
Palate
Team
Genetic Scientist
Pediatrician
Pedodontist
Orthodontist
Oral and Maxillofacial Surgeon
Prosthodontist
ENT Surgeon
Plastic Surgeon
Psychiatrist
Speech Therapist
Social Worker

Feeding
Cleft lip= makes it more difficult for an infant to suck on a

nipple
use special nipples to allow the baby to latch
properly (either pump or use formula)

Cleft Palate= may cause formula or breast milk to be

accidentally taken up into the nasal cavity

dont feed baby without palatal obturator
(prosthetic palate)
feed in an upright position to keep milk from
coming out of the nose

Mead Johnson/Enfamil Cleft Feeder

Needs Feeder / Haberman Feeder

Pigeon Feeder
Dr. Browns Natural Flow to relieve gas

Special

Presurgical Orthopeadics:
1. Reduces the size of cleft; Aids in Surgery
2. Partial obturation aids in feeding
3. Parental Reassurance at a crucial time
Maxillary Strapping
Nasoalveolar Moulding Appliances (NAM)

Require orthopedic
repositioning of the nasal
cartilages, columella, nasal tip,
and lateral wall of the vestibule

Presurgical infant nasal

remodelling nasal molding

Nasal molding by post surgical

nasal stenting

Facial taping 2 to 3 months

BILATERAL CLEFT LIP

The most challenging
condition
1. The premaxilla is
extremely protrusive
2. The premaxilla and

prolabium can be of
variable size

3. The columella is

deficient/almost
nonexistent

4. The palatal shelves are

collapsed

Protrusive maxilla

imperative to be
repositioned
Premaxillary orthopedics

with inraoral aplliance

Denture adhesive
Elastic strap

Latham Appliance

Rule of Ten
Primary repair- repaired at approximately 10

weeks
The surgeon usually uses the Rule of Ten
The child weighs 10 pounds
The child has a hemoglobin of at least 10
grams
The child has a white count of no higher than
10,000
The child is at least 10 weeks of age

Surgical Techniques
Cleft Lip Repair
unilateral

rotationadvancement flap
developed by Millard

complications
dehiscence

infection
excess tension

Surgical Techniques
Cleft Lip Repair
bilateral

bilateral rotation
advancement with
attachment to
premaxilla mucosa

Cleft Palate Repair Timing

Dorf and Curtin
10% occurrence of articulation errors when

palatoplasty was completed by 1 year

86% incidence of articulation errors when repair
was complete after 1 year

Haapanen and Rantala

Significantly fewer children in the groups
repaired before 18 months had hypernasal
speech, articulation errors, or required
secondary surgery to correct speech

Cleft Palate Repair

Schweckendicks Primary Veloplasty
V-Y Pushback
Von Langenbeck Palatal Repair
Furlow Palatoplasty

Cleft Palate Repair

Schweckendicks Primary Veloplasty

Incisions made in soft palate

Muscle bundles released from the posterior

hard palate and rotated

Reconstruction of levator sling
Closure of mucosal layers separately

Cleft Palate Repair

V-Y Pushback (WARDILL OPERATION)

Two uni-pedicled flaps (greater palatine artery) and one or

two anteriorly based pedicled flaps

Posterior flaps rotated in a V-Y advancement technique increasing the length of the palate
Nasal mucosa not closed
Improved speech results compared with bipedicled
techniques
Indicated for incomplete clefts

Von Langenbeck Operation:

Cleft Palate Repair

Furlow Palatoplasty

Lengthens the soft palate

Reconstructs the muscle sling.
Also commonly used to correct velopharyngeal

insufficiency in patients with submucous cleft palate

Speech outcomes are improved compared with other
palatoplasty techniques.

How do you manage ear

96% of children with cleft
disease?
palate required
tympanostomy tube
placement
50% of these children required
repeat tympanostomy tube
placement.
Frequency of otitis media
decreases as the child with CP
ages
Audiology and tympanometry
as well as exams / clinical
history

Orthodontic Treatment of
Transitional
Dentition

The purpose the dentition adjacent to cleft

has to be orthodontically repositioned to
prepare the cleft side for the secondary
alveolar bone graft
Preparing the maxillary arch for a bone graft
(6-12 months) :
1. Bonded edgewise appliance
2. Supported with a maxillary expander quad
helix expander

Alveolar Bone Grafting

Primary Bone Grafting
Bone graft done at the time of primary
cheiloplasty
Bone graft done during the first 2 years of life
Bone graft done prior to the eruption of the
primary canine

Secondary Bone
Grafting
Done before eruption of the permanent

canine
Usually when the root of the canine is 1/3 to
2/3 formed
Usually between ages 8-10
In CLP dental age is usually behind
chronological age

Quadhelix to expand prior

to ABG

angle brackets to keep roots

away from cleft

Alveolar Bone Grafting

1. Provide bone for the eruption and/or
2.
3.
4.
5.

orthodontic repositioning of teeth

Closure of oro-nasal fistulas
Support and elevation of the alar base
Stabilization of the pre-maxilla in bilateral
cases
Provide continuity of the alveolar ridge

Types of bone grafts

Autogenous
Cancellous- iliac crest
Cortical- calvarium, mandible
Cortico-cancellous- iliac, rib, tibia, mandible

*Allogeneic
Graft resorbs, remodels, may contribute to osteoinduction and

osteoconduction

**Alloplast
Bone grows into, around alloplast
No active osteoinduction but some osteoconduction
Teeth do not erupt through alloplast
*Nique T, Fonseca RJ, et al: Particulate allogeneic bone grafts into maxillary alveolar clefts in
humans- A preliminary report. J Oral Maxillofac Surg 45: 386-392, 1987
**Horswell BB, El Deeb M: Nonporous HA in the repair of alveolar cleft defect in a primate
model. J Oral Maxiilofac Surg 47:946-952, 1989

Alveolar Bone Grafting

Alveolar Bone Grafting

Preoperative Cleft Defect

Postoperative Bone Graft

Orthodontic Treatment For Permanent Dentition

Correction of anterior
crossbite
Arch expansion
Quad Helix
Expansion screws

Orthognathic Surgery
Midfacial

Advancement
LeForte osteotomies

leave vascular
pedicle attached in
back of maxilla prevents necrosis

Rhinoplasty
Rhinoplasty
standard techniques

tip projection
alar rotation
columellar length

Age =17-20

Surgical Management of
Velopharyngeal Insufficiency
Major Goals of Surgery
Close the gap or hole between the roof of
the mouth and the nose.
Reconnect the muscles that normally make
the palate work.
Make the repaired palate long enough so
that when the muscles are working, the
palate can perform its function properly.

Velopharyngeal Insufficiency
VPI

Wardill Operation: WY push back

Dorrance and Browns U shaped push back

palatoplasty

Prosthetic Management
of VPI

Thank You!