Cleft Lip and

Palate
Objectives
• Relevant Anatomy and
Pathophysiology
• Classification
• Counseling of parents
• Basic repair
techniques
 Introduction
 Facialclefting is the second most common
congenital deformity (after clubfoot).
 Affects 1 in 1000 births
 Problems are cosmetic, dental, speech,
swallowing, hearing, facial growth,
emotional
Face is formed by fusion of number of embryonic
processes.

Around the 4th week of intrauterine life, five brachial

arches develop at the site of future neck.
Embryological Background
Head and Neck of 4-Week Old Embryo

Glossopharyngeal
nerve
Vagus nerve

Trigeminal nerve

Facial nerve
The first arch, mandibular arch plays a role in
development of nasomaxillary complex.
Mandibular arch gives rise to maxillary process
from the dorsal end.
5-6th week

With the formation of nasal pits, frontonasal

process gets divided into a medial nasal
process and two lateral nasal processes.
Maxillary process fuse with medial and
lateral nasal processes to form upper lip
and primary palate respectively
Development of palate
Development begins in 6th
week

Develops from-
1.Primary palate(from medial nasal process) 2.
Secondary palate(from maxillary process)
 Palate is formed by contibution of
1. Maxillary process
2.Palatal shelves given off by maxillary process
3. Fronto-nasal process

Fronto-nasal process give rise to premaxillary

region while palatal shelves form rest of palate.
Fusion of palatal shelves begin at 8th week which
continues till 12/17th week.

Initially the palatal shelves are covered by an

epithelial lining.
As they join epithelial cells degenerate.

The connective tissue of the palatal shelves

intermingle with each other resulting in their
fusion.
Lower Lip is formed by the fusion of mandibular
process of two sides.

Upper Lip is derived from medial nasal and

maxillary processes.

Failure of merging between the medial nasal

and maxillary processes at 5 weeks’gestation,on
one or both sides,results in cleft lip.
If cleft of lip extends deep into primary palate, it
results in cleft of alveolus too.

Occasionally, cleft of lip and alveolus may have bands

of soft tissue bridging across the two sides called
simonnarts band.
 Cleft of palate occurs in number of ways:
 Defective growth of palatal shelves
 Delayed or total failure of shelves to elevate
and attain a horizontal position
 Lack of contact between shelves
 Post fusion rupture of shelves
 Failure of mesenchyme consolidation.
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
 Non-syndromic inheritance is multifactorial
◦ Cleft Lip, With or Without Cleft Palate:
One Parent-2%
One Sibling- 4% Two Siblings- 9%
One Parent + One Sibling- 15%
◦ Cleft Palate:
One Parent- 7%
One Sibling- 2% Two Siblings- 1%
One Parent + One Sibling- 17%
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 Esthetic Problems

• Facial Disfigurements
• 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 dysfunction?
• Abnormal curvature of the eustachian tube lumen
• 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
Antenatal
diagnosis
 Ultrasound technique

- Non-invasive diagnostic tool

- Confirm fetal viability
- Determine gestational age
- Establish number of fetuses and their growth
- Check placental location
- Examine fetal anatomy for detecting
malformations

27
Transabdominal US
- Not reliable till gestational age of 15
weeks
- Done at 20 or more weeks of gestation

Transvaginal USG
- Earlier visualization (12 weeks)
- Better image resolution
- Greater specificity and sensitivity
 Advantages of prenatal cleft diagnosis

- Psychological preparation for parents to have realistic

expectations

- Parent education for cleft management

- Preparation for neonatal care and feeding

- Opportunity to investigate other abnormalities

- Possibility of fetal surgery

29

Graber Vanarsdall and Vig. Orthodontics: Current Principles and Techniques. Elsevier
 Disadvantages of prenatal cleft diagnosis

- Emotional disturbance

- High maternal anxiety and

dysfunction

- Termination of pregnancy

30

Graber Vanarsdall and Vig. Orthodontics: Current Principles and Techniques. Elsevier
 Epidemiology
 Cleft Lip +/- Palate- M:F 2:1
 Cleft Palate - F:M 2:1
 Cleft Lip +/- Palate- Native Americans
> Oriental and Caucasians > Blacks
 Cleft Palate- Same among ethnic groups
 Environmental: Ethanol, Rubella virus, Thalidomide,
Aminopterin B
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 doesn’t cross the nasal philtrum.
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
Anatomy
• Philtrum
• Philtral Ridges
• Cupid’s Bow
• Philtral Dimple
• Commisure
• White roll
• Tubercle
 History & Examination
 Cosmetic disfigurement (cleft lip)
 Feeding problems in cleft palate
◦ Suckling difficulties
◦ Nasal regurgitation
 Ear disease
 Respiratory infections
 Malnutrition and failure to thrive (wt. for age)
 Other congenital anomalies – digits, cardiac, craniofacial
syndromes etc.
 Pre operative evaluation
 History
 Physicalexamination
 Laboratory data
 Hb%
 Syndromes – X–ray mandible, Cardiac workup
Management
Care of the newborn
 FEEDING PROBLEMS


Oronasal fistulas

 Draining of oral fluids in nasal cavity and vice versa

 Bottle, cup and spoon, tube feeding

 Infant held at 30-450 angle to aid swallowing

5
8
 Mead Johnson/Enfamil Cleft Special Needs
Feeder Feeder /
Haberman Feeder

Pigeon Dr. Brown’s

Feeder Natural
Flow to relieve gas
MULTIDISCIPLINARY MANAGEMENT

Dentis
ENT/ t
Surgeon
audiologist

Speech Nursing
therapis
t
CLEFT
TEAM
Paediatrician Genecists

Psychologi Social
st worke
Ophthalmol r
ogist 64
 Schedule of Treatment
Birth: 8-9 Year:
 Initial Assessment  Initial interventional
 Pre-surgical assessment Orthodontics
 Preparation for alveolar bone
grafting
3 Month:
 Primary Lip repair
10 Year:
 Alveolar Bone Grafts
9-18 month:
 Palate Repair 14 Year:
 Definite Orthodontics
2 Year:
 Speech assessment 16 Year:
 Nasal Revision Surgery
5 Year:
 Lip Revision Surgery 20 Year:
 Orthognathic Surgery
Lip Repair
Timing of Cleft Lip Repair
 At three months of age
◦ Larger anatomical parts
◦ Safer anesthesia

Rule of 10
 10 weeks
 10 grams
 10 pounds
 10k WBC
Millard Lip Reapir
Randall-Tenisson Lip Reapir
Palate Repair
Timing of Cleft Palate
repair
 Early repair (< 24 mts) – speech and
hearing improved
 Delayed closure (>4y) – less
retardation of mid facial growth
 Optimal time
 Soft palate 3 – 6 mts
 Hard palate 15-18 mts
Intervelar Veloplasty
Surgical
Complications
 Early
Airway compromise Bleeding
 Late Fistula
Reduced movement of the soft
palate Persistent VPI
Under developed facial skeleton