3. Introduction
• Orofacial clefts are the most common craniofacial birth defects,
second only to clubfoot in frequency of major birth anomalies .
• Patients who have cleft lip or palate face significant lifelong
communicative and aesthetic challenges, and difficulties with
deglutition
• The overall incidence of orofacial clefting is typically quoted as 1 in
700 live births
4. Introduction
• Cleft lip is associated with cleft palate in 68% to 86% of cases.
• The incidence of CL(P) varies significantly by racial group and with
socioeconomic status, with an incidence of 1 in 1,000 births in
whites, 1 in 500 births in Asians and Native Americans
• Gender
• – 2:1 – M:F ratio – cleft lip +/- palate
• – 1:2 – M:F ratio – cleft palate only (late closure of palatine shelves)
5. Etiology
Early Chinese
• Eating rabbit – “hare lip”
• Bad karma or wrongdoings
Philippines
• Force to the foetal face
Familial or “In the blood”
6. Familial
2 unaffected parents with 1 child affected
– Risk for future children:
• 4.4% for CL+/- palate
• 2.5% for CP only
1 parent affected
- Risk for future children
• 3.2% for CL+/- palate
• 6.8% for CP only
1 parent affected with 1 child affected
• – Risk for future children
• 15.8% for CL+/- palate
• 14.9% for CP only
7. Etiology
Majority of orofacial clefts are nonsyndromic
• 70% of CL +/- palate
• 50% of CP only
Nonsyndromic clefts
• multifactorial
• Clusters in families but not mendelian
• Palate development complex process with several proteins, growth
factors, and transcription factors
• Any disturbance in the process can result in clefting
8. Etiology
Syndromic clefts:
• Associated with over 300 syndromes
• Van der Woude syndrome – the most common
– Autosomal dominant
– Lower lip pits
Teratogen exposure
• Ethanol, thalidomide, phenytoin
Environmental factors
• maternal diabetes, maternal folate deficiency
9. Embryology
Development of the lip and palate begins around the 4th week of
embryological development
Completed by the end of the 12th week
By the end of the 4th week
– 5 facial prominences have formed
• Frontonasal process
• paired maxillary processes
• paired mandibular processes
10. Embryology
• During the 5th week
• – Nasal placodes invaginate to form the nasal pits
• - Lateral and medial nasal prominences
11. Embryology
• By the end of the 6th week
– Paired maxillary processes have grown medially and pushed the paired
medial nasal prominences together
– Fusion of the paired medial nasal prominences form:
• Philtrum
• Middle upper lip
• Nasal tip
• Columella
12. Embryology
– Fusion of the paired maxillary prominences with the paired medial
nasal prominences forms the complete upper lip (maxillary
prominences form lateral lip)
– The lateral nasal prominences form the bilateral nasal ala
13. Cleft Lip Formation
• Fusion
– Failure of fusion of the maxillary and medial nasal prominences
unilaterally or bilaterally result in unilateral or bilateral cleft lip with/without
primary palate
– Failure of fusion of the palatal shelves result in clefts of the secondary
palate
14. Normal anatomy of Lip
• Laterally,Two vertical lines at the corners of lip
• Superficially by lower portion of the nose
• Below ,upper half of oral apertures encloses the upper lip.
• Cupid’s bow
15. Normal anatomy of Lip
• Lip has two surfaces
• Superficial surface further divided into cutaneous and mucous
membrane
• Two rounded vertical elevations of variable prominence called philtrum
columns
• Philtral depression deepens
• Vermilion –upper zone(pinkish)
-lower zone (red)
• Below cupids bow,vermilion in
the midline forms protrusion
16. Normal anatomy of Lip
Orbicularis oris –two units-outer/superficial group
-inner/deep
Extrinsic fibers affixed to the skin, denser into central portion of lip,insert
into ANS,upper maxilla,lower end of the nasal septum
Intrinsic fibers-incisive labii superioris and inferioris
Inner /deep bundle of orbicularis oris –lower half of upper lip. Arranged to
form scissor like closing mechanism.
17. Classification
Clefts
– Unilateral or bilateral
– Complete or incomplete
Veau classification
– Class I – incomplete cleft involving only the soft palate
– Class II – cleft involving the hard and soft palate
– Class III – complete unilateral cleft involving the lip and palate
– Class IV – complete bilateral cleft
Modified versions
18. Kernahan and Stark symbolic classification
• Areas 1 and 4 – Lip
• Areas 2 and 5 –Alveolus
• Areas 3 and 6 –Palate between the
alveolus and the incisive foramen
• Areas 7 and 8 –Hard palate
• Area 9 – Soft palate
19. International Confederation of Plastic and
Reconstructive Surgery classification
• Classification system uses an embryonic framework to divide clefts into
3groups, with further subdivisions to denote unilateral or bilateral cases,
as follows:
• Group I – Defects of the lip or alveolus
• Group II – Clefts of the secondary palate (hard palate, soft palate, or
both)
• Group III – Any combination of clefts involving the
primary and secondary palates
20. David and Ritchie classification
GROUP I- Prealveolar clefts:
• Unilateral cleft lip
• Bilateral cleft lip
• Median cleft lip
GROUP II-Post alveolar clefts
• Cleft hard palate alone
• Cleft hard and soft palate alone
• Submucous cleft
GROUP III-Alveolar clefts
• Unilateral alveolar cleft
• Bilateral alveolar cleft
• Median alveolar cleft
21. Unilateral Cleft Lip
Incomplete
– Muscle fibers of the orbicularis oris are often intact but hypoplastic
– Varying degrees of cleft noted.
23. Complete
– Orbicularis oris inserts at the columella medially and ala laterally on
the cleft side
– Columella is displaced to the normal side
– Nasal ala on the side of the cleft is displaced laterally, inferiorly, and
posteriorly
– Nasal tip is deflected towards the noncleft side
Alveolus may or may not be involved
24. Bilateral Cleft Lip
• Orbicularis oris attaches at the lateral cleft margins bilaterally at the nasal
ala
• Premaxilla protrusion
• Symmetrical nasal deformities
• – Laterally displaced ala – widely flared
• – Extremely short columella
25. Cleft Lip Management
• Multidisciplinary approach
– Cleft care team
• Plastic surgery
• Audiology
• Speech pathology
• Otolaryngology
• Orthodontist
• Oral maxillofacial surgery
• Psychologist
26. Surgical Correction
• Age 1-3 months – Lip taping and nasoalveolar molding
• Age 3 months - Repair of cleft lip
• Age 9-12 months -Repair of cleft palate
• Age 1-7 years - Orthodontic treatment
• Age 7-8 years - Alveolar bone graft
• 18 years old or skeletal maturity– Midface advancement and continued
orthodontic treatment
27. Presurgical
Wide cleft lip or premaxilla protrusion
– Advantageous to narrow the cleft and mold the premaxilla before
proceeding with surgery
• Taping
– Effective in reducing the width of the cleft in a nonsurgical manner
– Strip of hypoallergenic tape is placed with
tension across the cleft and secured to the
patient’s cheek
– Molds bony tissues by applying pressure to
protruding portions of the maxilla
– Must be worn 24 hours per day
28. Presurgical
• Nasoalveolar molding devices
• – Custom made devices which utilize
wiring and nasal stenting to mold
the nasal cartilage, premaxilla, and
alveolar ridge
29. Presurgical
• – Nasal stenting can be elongated and adjusted to lengthen the columella
and mold the nasal cartilage
• – Takes advantage of the malleability of nasal cartilage
30. Presurgical
• Lip adhesion
– Surgically convert a complete cleft to
an incomplete cleft
– Performed at 2-4 weeks with definitive
repair at5-6 months
– Indications
• Wide unilateral cleft where conventional repair
might produce excessive tension
• Bilateral cleft – premaxilla protrusion
– Disadvantages – scar tissue
31. Cleft Lip Repair
• Typically performed at 3 months of age
• • “Rule of Tens”
• – 10 weeks old, 10 lbs, and hemoglobin of 10
• • Wide clefts or clefts with premaxilla protrusion that require lip adhesions
will have definitive lip repair at 5-6 months of age
33. Surgical technique
• The skin is elevated off of the orbicularis oris muscle for approximately 1
cm on both sides of the cleft.
• The soft tissues of the lip and cheek are elevated off of the maxilla in a
supraperiosteal plane
• This elevation may continue as superiorly as the level of the nasal bones to
allow maximal flap advancement and rotation, and tensionless closure if
the cleft is wide
• The orbicularis is freed from its abnormal attachments to the columellar
base and alar margin on the lateral side of the cleft.
• The alar margin on the cleft side is released from its attachment to the
piriform aperture
34. Surgical technique
• The advancement flap elevation is completed by incising along the
nasal sill
• The c-flap is elevated after incising along the vermilion-cutaneous
junction from the height of Cupid’s bow medially on the medial
margin of the cleft
35. Closure
• The orbicularis oris is
reconstituted across the cleft
with semipermanent suture
• The alar base on the cleft side is
medialized by placement of a
subcutaneous stitch from the
alar base to the periosteum of
the nasal spine.
• The c-flap may be rotated into
the nasal floor to prevent
stenosis of the nostril on the
cleft side, or it may be
discarded.
36. Closure
• The skin closure in the nasal floor is performed with 6-0
monofilament fast-absorbing suture.
• The lip skin closure is performed with 5-0 monofilament subcuticular
sutures .
• The skin closure is reinforced with surgical skin tape
37. Bilateral cleft lip
• In the bilateral cleft lip, the orbicularis oris muscle inserts on both alar
margins, and no muscle fibers invade the prolabium.
• Unrestrained growth of the vomer and nasal septum result in protrusion
of the premaxilla
• The prolabial skin is flat, lacking philtral ridges, a philtral dimple, and
Cupid’s bow.
• The columella is very short, and both lateral crura are flattened, resulting
in alar flaring.
• The advantage of the bilateral cleft lip is symmetry
38. Cheiloplasty techniques for the bilateral
cleft lip
• Early bilateral cleft lip repair techniques involved excision of the
premaxilla and prolabium, resulting in an unnatural appearance to the
upper lip and deleterious effects on midfacial growth .
• Later, premaxillary setback with vomerine osteotomies was popularized
in the 1800s to manage premaxillary protrusion. This technique was also
associated with significant midfacial growth hindrance,
• However, current bilateral cleft cheiloplasty techniques are modifications
of Millard’s bilateral straight-line repair.
39. Principles
• Symmetry
• Primary muscular continuity
• Proper philtral size and shape
• Formation of the median tubercle from lateral lip elements
• Primary positioning of alar cartilages to construct the nasal tip and
columella
40. Surgical technique
• The prolabial vermilion-cutaneous junction is incised.
• The mucosa of the prolabium is dissected from the premaxilla in a
supraperiosteal plane, and is turned down to line the premaxillary
gingivolabial sulcus
41. • The philtral flap is designed based on Mulliken and colleagues
• The prolabial skin is elevated in a supraperiosteal plane to the level of the
nasal spine
• The advancement flaps are elevated by incising along the vermilion
cutaneous junction from the height of Cupid’s bow medially on the lateral
lip elements
• The mucosal flaps created by these incisions along the cleft margins are
dissected supraperiosteally, and sutured to the prolabial mucosa with
absorbable suture, thus effecting bilateral gingivoperiosteoplasty.
42. • The skin is elevated off of the orbicularis oris muscle for approximately 1
cm on the lateral elements of the cleft. Bilateral gingivolabial sulcus
incisions are made, which extend to the cleft margins
• Elevation of the advancement flaps is completed by incising along the
nasal sills
43. Closure
• The orbicularis oris is reconstituted across the cleft.
• The alar bases are medialized by placement of subcutaneous stitches from
the alar bases to the periosteum of the nasal spine.
• The dermis of each alar base is sutured to the underlying muscle to
prevent alar elevation with smiling.
• The skin closure in the bilateral nasal floor is performed with 6-0
monofilament fast-absorbing suture.
• The lip skin closure is performed with 5-0 monofilament subcuticular
sutures.
• The skin closures are reinforced with surgical skin tape.
44. Complications and their management
Notch in the vermilion.
• This complication indicates incomplete muscular repair
or dehiscence of the inferior portion of the orbicularis oris repair.
It is corrected by reapproximation of the lowest portion of the lip
muscle.
The overlying mucosa may be excised, or a V-Y advancement performed
45. Complications and their management
Malalignment of Cupid’s bow or whistle deformity
• This condition is frequently caused by contracture of the lip scar,
• It can be prevented by placement of a Z-plasty at the vermilion-
cutaneous junction during primary cheiloplasty.
• The scar may be excised secondarily, and the vermilion correctly
repositioned with a Z-plasty to prevent recurrence of the deformity
46. Complications and their management
Absence of the median tubercle and part of Cupid’s bow.
• Commonly seen after bilateral cleft lip repair, this problem is difficult to
correct.
• Paired vermilionorbicularis flaps may be used to correct this deformity,
or a cross lip flap may be necessary
