2. Introduction
• Cleft lip and palate are the most common congenital
craniofacial anomalies treated by plastic surgeons.
• Cleft care requires that the plastic surgeon be a
member of a collaborative multidisciplinary team.
• Successful treatment of these children requires
technical skill, in-depth knowledge of the abnormal
anatomy, and appreciation of three-dimensional facial
aesthetics.
3. EPIDEMIOLOGY
• the most common diagnosis is cleft lip and palate at 46%,
• Isolated cleft palate at 33%
• isolated cleft lip at 21%.
• Majority of bilateral cleft lips (86%) and unilateral cleft lips (68%) are associated with a
cleft palate.
• In the white population, cleft lip with or without cleft palate occurs in approximately 1
in 1,000 live births.
• twice as common in the Asian population
• half as common in African Americans
4. ETIOPATHOGENESIS
• Both environmental teratogens and genetic factors are implicated in the genesis of
cleft lip and palate
• Intrauterine exposure to the anticonvulsant phenytoin is associated with a 10-fold
increase in the incidence of cleft lip.
• Maternal smoking during pregnancy doubles the incidence of cleft lip.
• Genetic abnormalities can result in syndromes that include clefts of the primary or
secondary palates among the developmental fields affected.
• The most common syndrome associated with cleft lip and palate is van derWoude
syndrome
• DiGeorge, or conotruncal anomaly syndromes are the most common diagnoses
associated with isolated cleft palate.
5. MULTIDISCIPLINARY CLEFT CARE
• cleft lip and or palate require coordinated care from multiple
specialties to optimize treatment outcome.
• Typical members of a cleft team:
• audiologist, dentist, geneticist, nurse, nutritionist/dietitian, oral
surgeon, orthodontist, otolaryngologist, pediatrician, plastic
surgeon, psychologist, social worker, and speech pathologist.
• The goal of cleft care is to eliminate as many steps in the treatment
plan as possible by optimizing the outcome and benefit of each
essential intervention.
• a child with a cleft requires a complex lengthy surgical treatment plan
6. Head and Neck of 4-Week Old Embryo
Trigeminal nerve
Facial nerve
Glossopharyngeal
nerve
Vagus nerve
Embryological Background
7. Embryological Background
• Development of the Lip:
• Unpaired Frontonasal Prominence
• Medial and Lateral Nasal
prominences
• 2 maxillary prominences
• 2 mandibular prominences
8. 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
9. 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
10.
11. Embryological Background
•Various theories have been given for its
development.
1. Alteration in intrinsic palatal shelf force
2. Failure of tongue to drop down
3. Non fusion of shelves
4. Rupture of cyst formed at the site of fusion
12. 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.
15. Kriens “LAHSHAL”
L = Lip (right)
A = Alveolus (right)
H = Hard Palate (right)
S = Soft Palate (median)
H = Hard Palate (left)
A = Alveolus (left)
L = Lip (left)
Capital letter = complete cleft
Lowercase letter = incomplete cleft
“.” or “-” = normal
Examples
LA….l = complete right cleft lip and alveolus,
incomplete left cleft lip
LAHS = complete right unilateral cleft lip, alveolus,
hard, and soft palate
16. 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
17. Prenatal Diagnosis
• Cleft lip can 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
18. Diagnosis
• Advantages of Prenatal
Diagnosis:
1. Time for parental education
2. Time for parental
psychological preparation
3. Opportunity to investigate
other associated anomalies
4. Gives parents the choice of
continuing the pregnancy
5. Opportunity for fetal surgery
19. 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
20. 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 2% risk of CP in next child
• Normal parents, 2 children with CP 7% risk of CP in next child
• Parent with CP, no affected children 6% risk for next child
• Parent with CP, one child with CP 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
21. 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)
22. Syndromic Cleft
Common Syndromes
Stickler (25% of syndromic CP) –
• autosomal dominant type 2 collagen gene
• Pierre Robin, ocular/hearing/joint malformations
Velocardiofacial (Shprintzen’s) – 15% of syndromic
CP
• Autosomal dominant, variable expressivity, CATCH
22 – Deletions at 22q11,
• Facial, cardiovascular, immunologic, development
al anomalies
Van der Woude’s (19% of syndromic CL/P and CP)
• Autosomal dominant
• CL/P or CP with bilateral lower lip pits
Shprintzen’
23. Problems Associated With Cleft Lip
and Palate
•Feeding
•Dental problems
•Nasal Deformity and Esthetic Problems
•Ear Problems
•Speech Difficulties
•Associated Anomalies
24. 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
25. 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
26. Nasal Deformity and Esthetic
Problems
• Facial Disfigurements
• Poor nasal shape
• Scar marks of surgeries
• Poor lip function during speech
• Poor dental alignment and smile
27. Ear Problems
• Middle ear disease - 22% to 88%
• Conductive hearing loss and chronic suppurative otitis media may
result
• Repeated tympanostomy tube placement
28. 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
29. Speech Problems:
• Hearing loss hampers proper development of speech
• Velopharyngeal Insufficiency (VPI)
• Abnormal air
• Poor pronunciation of
• Bilabial,
• Labiodental,
• Linguoalveolar sounds
32. 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
33. 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
34. 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
• don’t feed baby without palatal obturator (prosthetic
palate)
• feed in an upright position to keep milk from coming
out of the nose
35. Mead Johnson/Enfamil Cleft Feeder Special Needs Feeder /
Haberman Feeder
Pigeon Feeder Dr. Brown’s Natural
Flow to relieve gas
36. 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)
37. 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
38. 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
39. • Protrusive maxilla
imperative to be
repositioned
• Premaxillary orthopedics
with inraoral aplliance
• Denture adhesive
• Elastic strap
42. 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
46. 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
48. Cleft Palate Repair
Schweckendick’s 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
49. 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
51. 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.
52. How do you manage ear
disease?• 96% of children with cleft 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
53. 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
54. 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
55. 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
58. Alveolar Bone Grafting
1. Provide bone for the eruption and/or orthodontic repositioning of teeth
2. Closure of oro-nasal fistulas
3. Support and elevation of the alar base
4. Stabilization of the pre-maxilla in bilateral cases
5. Provide continuity of the alveolar ridge
59. 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
77. 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.