BILATERAL CLEFT LIP
 BCL usually associated with complete CP, sometimes only involves primary palate.
Pathophysiology
 essential same as unilateral but the prolabium is unique in that there is absence of
philtral remnants and muscular elements
 premaxilla is unrestrained by attachment to either segment of maxilla & is projected
forward by growth of cartilaginous septum, which act as force for downward &
forward growth of maxilla transmitted by the septopremaxillary ligament, coursing
through caudal border of nasal septum in a post-inferior direction to blend with
premaxillary periosteum & interpremaxillary suture.
 Maxillary growth, however, is due to deposition of bone on the maxillary tuberosities
 bilateral cleft thus disrupts the normal termination of the muscle fibers that cross the
embryologic fault line of the maxillary and nasal processes, resulting in symmetric but
abnormal muscular forces between the normal equilibrium that exists with the
nasolabial and oral groups of muscles.
1. unrestrained downward and forward growth of premaxilla
2. due to pull of orbicularis, zygomaticus major and risorius, the alar cartilages are
rotated caudally; and pulled inferiorly and laterally. Consequently, the nasal tip
broadens, the columellar is foreshortened, and the alar bases rotate outwardly
cephalad.
 in its complete form, the premaxilla is unrestrained by the lateral maxillary segments,
and the vomer grows anterior to the lateral segments, distorting the alar cartilages apart
from the nasal tip and stretching the alae across a widening cleft.
 The columellar is significantly deficient, virtually nonexistent, and without a distinct
demarcation from the prolabium, which also is vertically deficient.
 No prolabial muscle, philtrum columns, dimples, or Cupid's bow is present. No nasal
floor is present as the cleft continues through the palatal shelf along either side of the
vomer through the soft palate.
CLINICAL
1) Is cleft complete or incomplete?
a. wide range of clinical presentation of the bilateral cleft lip and palate is
possible, from the simple microform cleft to the complete cleft bilaterally
involving the lip, alveolus, palate, and nose.
b. the cleft may present with varying degrees of asymmetric involvement of the
lip and palate. Any degree of combination of involvement of the lip, primary
palate, and secondary palate can exist.
2) Size/Position of premaxilla and prolabium
3) Length of columella
4) In complete clefts, interalveolar space sufficient to accommodate premaxilla?
5) Presence of lip pits or other anomalies?
6) Is child thriving?
7) If CP, middle ear disease?
Anatomic components
 Bilateral cleft lip/palate
 Widened alar bases
 Laterally flared internal nasal valves
 Appearance of a shortened columella with malpositioned alar cartilages
 Excessively obtuse columellalabial angle
 Vertically short upper lip, particularly centrally at the prolabium
 Abberent insertion of lateral lip orbicularis oris into alar-facial bases
 Premaxillary alveolar segment with variable degree of protrusion
o Hypoplastic prolabial lip segment
o Absence of normal gingival sulcus in premaxillary segment
o Absence of orbicularis oris in prolabial segment
o Absence of central philtral dimple, columns or philtral tubercle
o Absence of cupids bow
o Absence of white roll in the prolabial lip segment
o Aberrant dry-moist vermillion on prolabial segment
The projecting premaxilla
 paired premaxillary bones joined in midline at interpremaxillary suture (a component
of midpalatal suture)
 each premaxilla articulates posteriorly with prevomerine bone and extends a short
distance around it, so that premaxillary vomeral suture forms a tongue-in-groove joint
 premaxilla may vary in site and size, development & position. It may contain 4
incisors or only 2
 moulding effect of intact orbicularis oris mm, ie primary repair is far more effective
than lip adhesion except for “diminutive” prolabium where preliminary adhesion
stretches prolabium to a more manageable size, creates a philtrum of normal
dimension, and excess tissue may be used in columellar reconstruction
Control of protruding premaxilla: Options
1) traction by external elastics with head cap
2) lip adhesion - efficient, avoids possibility of a poor definitive repair due to excessive
tension
3) Intraoral devices - rarely, if premaxilla 'locked out' of arch in an anterior position one
needs to reexpand lateral elements and set premaxilla back at the same time,
controlled fixed-pin external traction device (Georgiade, Latham)
4) closure of cleft one side at a time : 2nd side 3 months later. Do wider side first or do
narrow side first with lip adhesion of wider side. Difficult due to distortion of
prolabium by first operation.
5) surgical setback
 involves subperiosteal section of the vomer


leads to long term midface hypoplasia
in patients with bilateral CL/P, a protrusive premaxilla confers an advantage
during most of the growth period because the premaxilla grows at a far
slower rate than the mandible.
6) Surgical excision – abandoned now
Summary of management
1) If the premaxilla is well in the arch, mucoperiosteal flaps can be turned at the time of
lip closure to effect fibrous union of the arches.
2) If the premaxilla is projecting, extraoral traction beginning at the time of birth is
indicated. In the event of poor response to this form of therapy, the controlled fixedpin external traction device of Georgiade’s may be needed.
3) goal of orthodontics is to achieve retrodisplacement of the premaxilla in line with the
maxillary arches, sufficient to allow lip closure with approximation of the lateral
muscle segments across the premaxilla. Undercorrection of the premaxilla is certainly
acceptable.
4) In rare cases, setback may be necessary
5) In older patients the degree of premaxillary protrusion can justify repositioning with
osteotomy. A protrusive premaxilla can be repositioned after age 6 to 8 years without
deleterious effect on midfacial growth.
Prolabium
1) Complete bilateral cleft lip - Functionally sterile. Contains primarily immature
fibroblastic tissue & fine collagen, but no mature muscles
2) Incomplete bilateral cleft lip - has near-normal muscles penetration into prolabium;
muscles bundles are cylindrically bunched in remaining lip bridge, then fan out into
prolabium
Use of Prolabium
mistakes of early procedures :
1) excise or assumed prolabium as displaced columella > lip grossly deficient
horizontally excision of premaxilla invariably results in a tight lip, dish-face
deformity of mid-third of fac and relative mandibular prognathia
2) deceived by deficient appearance & used flaps from lateral lip segment to
supplement vertical height > lip too long & too narrow with prolabial skin
trapped in the centre pin-cushioned - fail to recognise potential of prolabium to
grow in width and height when attached to dynamic lateral lip elements
Applications 1. provide full vertical dimension of central lip
2. lateral prolabial parings can be used for future columellar lengthening
3. an advantage to establish an oral sphincter that is in-continuity early in repair
sequence, by direct suture of lateral muscle elements behind prolabium
4. modified Manchester - if white roll & vermilion of prolabium of good quality, the
lateral prolabial parings may be banked for future columellar lengthening, &
orbicularis oris muscle should be reconstituted behind prolabium
5. Millard - if white roll & vermilion of prolabium of poor quality, borrow these
parts from lat lip
TREATMENT
Objectives
1. complete correction of lip & nasal deformity
2. control of relationship of premaxilla & maxillary segments
3. closure, or provision for closure, of anterior palate & subsequently posterior palate
Principles.
1) prolabium is to form full vertical length of lip
2) prolabial vermilion turned down for lining
3) central vermilion built from vermilion/muscle flaps from lateral lip segments
4) vermilion ridge from lateral lip segment
5) no lateral lip skin below prolabium
6) reposition severely protruding premaxilla, surgically or nonsurgically, permits
earlier & better repair of lip by relieving undue tension & also makes 1 stage
repair possible
7) collapse of maxillary processes behind protruding premaxilla requires prevention
or expansion with maxillary orthopaedics
8) bone grafting - secondary
Anterior palate
may be repaired with lip surgery or after it. If after, should be performed before
premaxilla & maxillary segments become opposed by action of repaired lip
Timing of repair
 In general, most centers prefer to perform the lip reconstruction when the patient is
aged 2-4 months; anesthesia risks are lower, the child is better able to withstand the
stress of surgery, and lip elements are larger and allow for a meticulous reconstruction.
 At PMH
o Presurgical orthopaedics at birth
 presurgical, fabricated, passive, intraoral orthodontic palatal appliance to
maintain the arch width to prevent the nearly inevitable collapse that occurs
with lip repair.
 appliance also assists in the child's oral feeding, helping to decrease nasal
regurgitation and assisting oral suction.
 external pressure with soft elastic tape to help maintain the premaxillary
component within the arch alignment.
Techniques of repair
1. straight line closures - Veau, Manchester
2. Z-plasty
lower - Berkeley, Bauer
upper - Millard, Wynn
both – Skoog
International trends
 75% perform rotation-advancement flaps and 75% perform lip repair at 3-6 months
 9*
Veau III (Straight line closure)
 straight-line closure without elevation of the prolabial skin
 no attempt at restoring the continuity of the orbicularis oris.
 The central cupid's bow and tubercle is constructed from the vermilion of the lateral
lip elements.
 prolabium reduced to width of 1cm
 central lip skin for lip repair and columella lengthening if wide
 alveolar cleft closed
 If prolabium is wide, fork flaps can be developed and banked in the nasal floor
 Essentially technique brings lateral lip into, but not beneath (ie muscle), the prolabium
Manchester
 Single stage lip and palate repair.
 Emphasise the construction of a tubercle by deepithelised prolabial wings.
 keeps prolabial white roll and vermilion
 discards lateral prolabial paring
 does not involve repairing the orbicularis as he felt this would create an overly tight lip
and maxillary retursion - long term results of extensive mm closure (Mulliken,
Millard) have yet to be evaluated .
Problems
1)
2)
3)
4)
Creates a broad philtrum
inadequately addresses the sulcus.
No provision for columella lengthening
using prolabial vermilion in an exposed position where there is a colour
difference compared with that of the lateral segments. Occasionally, the white
roll and vermilion of the prolabium are of good quality,
Post op Care
 Steristrips - not used at PMH
 Logan's bow (wire arch taped across cheek to take tension off lip repair) – not used at
PMH
 Arms splints
 Liquids for three weeks (Mr Baker uses spoon feed for 3 weeks, Mr Gillet allows
squeeze bottle)
Millard/Mulliken Repair
 involved complete elevation of the prolabium and reconstitution of the orbicularis
across the premaxilla.
 lateral segments of the prolabium - "forked flaps" are banked to add columellar height
at a later stage.
 As with Veau, the central vermilion is recreated from the lateral lip elements.
 lateral segments closure design like rotation advancement repair in unilateral case=brings in new white roll from lateral lip elements
 prolabial vermilion is advanced toward sulcus to form a tubercle, & prolabial parings
are banked for future columellar lengthening
best = Millard & modified Manchester (Broadbent & Woolf)
both have a reduction of prolabium to a smaller philtral dimension at time of closure
Excessively long lip may result from collapse of maxillary segments behind premaxilla
Lengthening the columella
Usage of banked forked flaps.
3 unfavourable long term features with adolescent growth spurt (McComb)
1. columella may grow too long & nostrils too large
2. nasal tip often remains too broad
3. columellar base tends to drift & a scar courses through lip-columellar angle
 Mulliken concluded - columella is concealed in the nose of an infant with bilateral
complete cleft lip. There is no deficiency of columellar skin and no need to recruit
tissue from the lip or sills.
 McComb's experience led him to stage the repair. The initial stage involves
approximating and repositioning the splayed alar cartilages through a V-Y nasal tip
"gull-wing" incision that allows redraping the overlying skin with a simultaneous
bilateral lip adhesion. A definitive lip repair follows at a second stage.
Two-stage repair (McComb 1990)
 1st step in primary reconstruction of bilateral CLN is the use of preop orthopedics to
narrow soft-tissue clefts & realign the bony platform
 nasal tip dissection does jeopardise blood supply to prolabium, therefore 2 stages prolabium left attached to premaxilla while nose is repaired at 1st stage :
Stage 1 (3 weeks)
 VY gull wing incision enables
1) medial alar cartilage mobilisation and suturing,
2) columella reconstruction
3) alar width reduction
 tension is taken out of nasal tip by repairing nostril floor & creating long lip adhesions
 columella is reconstructed from tissues within nasal tip
 skin flaps are sutured together to reconstitute a columella that is 5mm in length
Stage 2
 At 3 months, prolabium is lifted away from premaxilla & mucomuscular flaps
advanced to complete lip repair
Disadvantage of gullwing is the scar which may complicate placement of scar for future
rhinoplasties
Primary correction of cleft nasal deformity
 88% of units reposition the lower laterals at the time of lip repair
McComb, Salyer
 correction of nasal deformity at time of lip repair endures, does not disturb growth, &
is beneficial to nasal morphology
 mobilise lower lateral cartilage from overlying nasal skin, simultaneously releasing it
on cleft side from piriform aperture & involving medial crus in septal area, creating a
flap of lining mucosa & cartilage, with lateral portion of lower lateral cartilage
advanced medially & cephalad, & medial crus advanced toward the tip. Suture-fixation
+/or bolster suturing to hold the cartilage in position
Other
1. Tajima repair : reverse-U incision along alar margin with a back-cut along vestibular
lining at junction of piriform aperture to release plica vestibularis
2. Non-surgical moulding in early neonatal periods (Matsuo et al)
3. Post-surgical splinting for 3-4mths (Nakajima)
COMPLICATIONS
Complications
1. Wound infection
2. wound dehiscence
a. most likely due to wound tension
3. Premaxillary malposition
a. Tilting or retrusion – important to prevent excessive tension
b. Prevent with use of splints
4. whistle deformity
a. refers to vermillion deficiency
b. prevented by using lateral muscle vermilion flaps to augment thickness of
prolabium
5. Lip length asymmetry
a. Inaccurate measurements
b. Lack of tissue approximation
6. Lateral maxillary segment collapse medially and posterior to the premaxilla
7. Scarring