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CLEFT LIP

AND
CLEFT PALATE
Jyoti Gupta, M.Sc. Foods and Nutrition

CLEFT PALATE / CLEFT


CLEFT
PALATE
LIP
(cheiloschisis)
is
a
condition in which opening in
the roof of the mouth due to a
failure of the palatal shelves to
come fully together from either
side of the mouth and fuse
during the first months of
development as an embryo.
The opening in the palate
permits
communication
between the nasal passages
and the mouth.

CLEFT LIP (palatoschisis) is a physical


split or separation of the two sides of
the upper lip and appears as a narrow
opening or gap in theskinof the upper
lip. This separation often extends
beyond the base of the nose and
includes the bones of the upper jaw
and/or upper gum.

BACKGROUND
The cleft palate was not recognized as a
congenital disorder until 1556.
For centuries, perforations of the palate were
considered to be secondary to syphilis.
The first successful closure of a soft palate
defect was reported in 1764 by LeMonnier, a
French dentist.
The first closure of the hard palate was
performed in 1834.
Clefts occur more often in kids of Asian, Latino,

OROFACIAL CLEFT (CLEFT |


Cleft lip and palate
are the most common
LIP)
congenital craniofacial anomalies treated by
plastic surgeons.

Between the 6th and 12th weeks of fetal


gestation, the left and right sides of the face
and facial skeleton fuse in the middle. When
they do fail to do so, the result is a
craniofacial cleft.
It is possible that facial clefts are caused by
a disorder in the migration ofneural crest
cells.
Another theory is that facial clefts are

Incidence
More

1 in 500

Less

common in south Asians:


frequent in Africans:

1 in 2000

Prevalence

in Europeans and Americans:

1 in 750

*Prevalence

in Pakistan:

1 in 523
India: 27,000 to 33,000 per year

(Little J and Mossey P; Indian J

Plast Surg.2009 October;42(Suppl): S9S18)

*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
DEVELOPMENT OF
CLEFT LIP

EMBRYOLOGICAL
DEVELOPMENT OF
CLEFT PALATE

Classification
It

can be classified as the follows:


Combined (cl+cp) or isolated
cleft(cl or cp)
Unilateral
Complete

or bilateral

(if it cross the nasal


philtrum) or incomplete ( if it
doesnt cross the nasal philtrum.

CLASSIFICATION
A mild form of a cleft lip is amicroform cleft. A
microform cleft can appear as small as a little
dent in the red part of the lip or look like a scar
from the lip up to the nostril.
Incomplete cleft lips can take on a variety of
appearances. There may be just a small gap or
cleft in the vermillion or it may slightly extend
into the skin above
the lip or extend almost to the nostril.
As with a complete cleft lip deformity, the nose
has some distortion. The distortion is usually to
a lesser degree. The nostril may be widened, but
the floor of the nostril is

In a bilateral incomplete cleft lip


deformity, the nose is affected,
although not to the same degree as
with a bilateral complete cleft lip
deformity.

A bilateral complete cleft lip affects


both the right and left sides of the
lip. There are no philtral columns, no
philtral dimple, and there is no
orbicularis muscle in the central
segment. On both sides, the cleft
extends from the vermillion to the

Classification Systems
Kernahan Striped Y
Millard Modification of
Kernahan Striped Y

Kriens LAHSHAL
Veau classification

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

T
A
P

H
P
O
H

O
I
YS

Y
G
O
L

PATHOGENESIS/CAUSES

Actually no one knows exactly what causes clefts


Multiple factors may be involved, like:
Genetics (inherited characteristic) from one or both parents . A study
has identified that 4 different regions of the human genome contains
genes controlling risk for cleft lip and palate:

IRFG Gene on chromosome 1

MAFB gene on chromosome 20

ABCA4 on chromosome 1

a region on chromosome 8
Environmental factors
Drugs: corticosteroids (anti-inflammatory), phenytoin
(anticonvulsant), retinoid.
Infections: like rubella during pregnancy.
Maternal smoking
Alcohol consumption, smoking, hypoxia during pregnancy, some of
dietary and vitamins deficiencies (like folic acid and vitamin A
deficiency)
Maternal Age
Women with diabetes diagnosed before pregnancy

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 clefts must be evaluated for


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

DIAGNOSTIC TEST/
EXAMS
Orofacial clefts
can beDiagnosis:
detected during
Advantages
of Prenatal

pregnancy by ROUTINE ULTRASOUND.


Cleft lipcan be easily diagnosed by
1.
Time for parental
education in the
performing
ultrasonography
2.
Time
for parental psychological
second
trimester.

preparation
3.Usually,
Opportunity
to investigate
other
orofacial
clefts are diagnosed
anomalies
whenassociated
the baby is
born.
4.
Gives parents the choice of
Minor
clefts might
not be diagnosed
continuing
the pregnancy
until
later in life.for fetal surgery
5.
Opportunity
Diagnosis can also be
PHYSICAL EXAMINATION.

made

by

PROBLEMS &
COMPLICATIONS

Failure to gain weight


Feeding problems
Dental problems
Flow of milk through nasal passage during feeding
Poor growth
Speech difficulties
Hearing loss
Difficulty in swallowing
Nasal Speaking voice
Language Delay
Recurring ear infections
Change in Nose shape
Poorly aligned teeth
Social Development

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
The oral nasal communication diminishes the
ability to create negative pressure necessary for
sucking.
Nasal regurgitations of food
Excessive air intake that requires frequent
burping and choking
Feeding time is significantly longer & fatigues
both baby as well as mother.

Dental Problems
A

child with a cleft palate or a cleft


through the gum line may have missing
or abnormally shaped baby and
permanent teeth.
In addition, the upper jaw may not grow
as fast & forward as the lower jaw,
necessitating corrective surgery later in
life.

Nasal Deformity and Esthetic


Problems
The

nasal alar cartilage may be


displaced or deformed,
producing a noticeable
deformity of the nasal orifice.
Facial Disfigurements
Poor nasal shape
Scar marks of surgeries
Poor lip function during speech
Poor dental alignment and
smile

Hearing and Ear Infections


Any child with a cleft palate is at risk of developing
frequent ear infections.
The Eustachian tube, which drains the middle ear,
malfunctions, causing persistent fluid build up in the
middle ear.
Otitis media
A combination of fluid and repeat ear infections can
cause scaring of the tympanic membrane (ear drum)
and hearing loss.
Pressure equalizing eardrum tubes can be inserted at
the time of lip or palate repair to drain the middle ear
and reduce the risk of ear infection and hearing loss.

Speech Problems:

Children with an unrepaired cleft palate have a nasal quality


to their speech (hypernasality) because air escapes through
gap in the roof of mouth & out of nose. This inability to use
palate muscles to block off nasal cavity from the mouth so
that air flows through mouth is called Velopharyngeal
incompetence
These children may also have difficulty generating enough air
pressure to produce certain sounds.
After cleft palate repair, most children develop near normal
speech.
Hearing loss further hampers proper development of speech.

MANAGEMENT OF CLEFT
LIP AND PALATE

Once the child is born with CLP, the


services of a team of specialists are
needed to care for it until adolescence.

King NM etal, 1996

Multidisciplinary Cleft Lip And


Palate Team

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

Neonatal Period
Parents counselling about immediate & long term
implications of cleft defect & necessary surgery.
The immediate problems of infant with CLP are concerned
with feeding & prevention of aspiration & infection.
Naso-gastric tube feeding
Use of medicine dropper for feeding
Breastfeeding is desirable but may not be successful &
expressing of milk may be most appropriate approach
Bottlefeeding is usually achieved but with some
difficulty due to their inability to generate sufficient
suction.
The nipple of feeding bottle should be positioned on
non-cleft side & moved about or squeezed by parent.
Cutting of a cross in nipple increases the volume of
milk that flows; but only when the nipple is compressed
hence the child doesnt need to create suction.

Feeding plate obturates the cleft &


restores the separation b/w oral & nasal
cavities. It creates a rigid platform towards
which the baby can press the nipple &
extract the milk. It restores basic function
of mastication, deglutition & speech
production until the cleft lip &/or palate can
be surgically corrected.

Here are some tips & tools to help when feeding a baby with a cleft:
Nipples: There are special nipples that can help a baby get the most out
of feeding.
Nipple Shape: There are two main shapesstraight standard nipples
& wide flat NUK or orthodontic nipples.
Nipple Length: The nipple should be long enough for babys tongue to
press against it.
Nipple Pliability: A soft nipple makes milk come out faster, then the
baby will not have to suck as much.
Nipple Hole Size: The size of nipple hole can make milk come out
faster or slower - X shape
Modified Nipples: There are some commercially available bottles
specifically designed for use with infants with CLP. These include Special
Needs feeding (Haberman), cleft palate nurser (Mead Johnson) & Pigeon
bottle. The caregiver provides a gentle squeeze on the bottle as the baby
sucks to get milk.

Mead Johnson/
Enfamil Cleft Feeder

Pigeon Feeder
Natural Flow

Special Needs Feeder /


Haberman Feeder

Dr. Browns

Positioning the Baby: upright or slightly reclined.


Positioning the Nipple: It is important to put nipple under a hard bone in
babys mouth to help them squeeze milk out.
Pacing During Feeding: Carefully control flow of milk by squeezing bottle
when baby is sucking & stopping if baby seems upset or overwhelmed.
Oral Facilitation: Use hand & fingers to help keep babys mouth around
nipple during feeding.
Prevent Swallowing of Air: Burp the baby frequently as babies with a cleft
swallow more air than other babies.
Managing Liquid in the Nose: If milk comes through nose, position the
baby up in an upright position, then squeeze less frequently to make the milk
go down towards the belly.
Consistency of Feeder Method: The baby should be fed in the same
position, with the same nipple and bottle, for each feeding. This allows the
baby to learn what to expect with feeding.

Oral Care: Clean the area around the cleft using a wet washcloth after
each feeding. Wet cloth with hydrogen peroxide or water (as advised
by your doctor).
Cup Feeding: Most doctors recommend changing from a bottle to a
cup before the palate is repaired. It can cause the scar not to heal if
the baby sucks on a bottle after the surgery. Thicker liquids can be
used to make it easier to drink when the baby is first trying a cup.
Begin offering a cup for small amounts of liquid beginning at 6 months
to help the baby with this transition.
Solid Foods: Solid foods can be given to the baby with cleft palate
at about the same time as with any other baby.
Breast Milk: If attempts at breast feeding are not successful, the
mother can use a breast pump to get milk. Then the baby can drink
the breast milk from a bottle

Once the child is feeding well indicated by


progressive weight gain & degree of healthiness,
the child will be able to cope with the primary
surgery to the lip & subsequent surgery to the
palate.
Lip repair aims to restore the continuity of the
orbicularis oris muscle of the lip & with it the
appearance and function of upper lip. The most
common technique for repair utilizes a staggered
suture line to minimize notching of the lip from
retraction of scar tissue. A logan clamp, which is a
wire bow attached by adhesive to the cheeks, is
applied immediately after operation to take tension
off the suture line.
Palate repair aims to reconstruct the abnormally
inserted musculature of the soft palate to normalize
movements of the soft palate & permit the
development of normal speech. Early repair aids

Since clefts of palate vary considerably in size,


shape and degree of deformity, timing for
operation should be individualized.
Criteria - width of cleft, adequacy of palatal parts,
morphology of surrounding areas (such as the
width of the oropharynx) & neuromuscular func of
soft palate & pharyngeal walls should affect
decision.
The surgeon usually uses the Rule of Ten
Primary repair- repaired at approximately 10
weeks
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

PREOPERATIVE AND
POSTOPERATIVE MANAGEMENT

Investigation of any infection prior to operation.


Bleeding & Clotting times should be determined
earlier.
If child is in good nutritional state & in fluid &
electrolyte balance, feeding may be permitted to
within 6 hrs of the operation.
Gentle aspiration of the nasopharynx minimizes the
hazards of the common complications of atelectasis
and/or of pneumonitis.
The primary consideration is maintenance of a clean
suture line & elimination of strain on the sutures.
For these reasons, infant is fed with a medicine
dropper & the arms are restrained with elbow cuffs.
A fluid or semifluid diet is maintained for 3 weeks.
The patients hands as well as toys & other foreign
bodies must be kept away from the palate.

CHILDHOOD
Monitoring of hearing & middle ear function to ensure
appropriate language & speech development.
Causes of speech problems---developmental delay
---hearing problems
---oronasal fistulae
---dental malocclusion
---velopharyngeal incompetence (VPI): detected by
nasendoscopy; corrected by pharyngeal flap surgery
which reduces nasal air flow during speech; should be
performed before age of 6 yrs
Speech therapy
Regular dental care to minimize morbidity due to loss of
teeth. It includes oral hygiene instruction & prophylactic
measures to prevent dental caries & periodontal disease;
repair carious & malformed teeth.
Orthodontics- align the teeth in the arch before bone
grafting of residual alveolar cleft prior to the eruption of

Alveolar bone grafting should be timed to precede the


eruption of the canine tooth on the cleft side, which
usually occurs at approx. 11 yrs of age. This provides a
bony bridge across the cleft so that the canine tooth can
erupt into this new bone & allows the tooth to have a
healthy supporting periodontium.

LATE ADOLESCENCE AND EARLY ADULTHOOD

A Le Fort 1 osteotomy with modification may be


performed to correct the occlusion and facial
appearance.
If necessary, an aesthetic rhinoplasty & cheiloplasty
should be performed as a final surgical stage.

SUPPORT GROUPS
Hong Kong Parents Association of Cleft Lip and Palate Children
Canadian Cleft Lip and Palate Family Association
Operation smile, US based NGO provides free reconstructive
surgery & associated services for children born with facial
deformities as well as children in need of surgery due to injury;
presence in more than 60 countries.
Trinity Care Foundation that provides free facial deformities &
CLP surgeries in reputed hospitals in Bangalore, India.
SmileTrain, NGO - US
Transforming Faces, NGO - Canada
The following organization is a good resource for information
oncleft palate:
Cleft Palate Foundation -www.cleftline.org
March of Dimes -
www.marchofdimes.com/professionals/14332_1210.asp
U.S. Centers for Disease Control and Prevention -http://
www.cdc.gov/ncbddd/birthdefects/CleftLip.html