39

CHAPTER

Bilateral Cleft Lip

David M Fisher

Summary
INTRODUCTION
1. The correction of premaxillary protrusion prior to definitive lip The patient with complete bilateral cleft lip and palate represents the
repair is described, including presurgical orthodontics and lip severe extreme within the spectrum of clefting. The initial deformity
adhesion. is startling to the ill-prepared and challenging for even the experienced
2. There are two general techniques of presurgical orthodontics, surgeon. The relative rarity of the deformity adds to the challenge by
passive and active. The passive technique of alveolar molding decreasing any one surgeon’s experience. Brown et al.1 in 1947 wrote:
is our preferred method. The rationale is that this gentler ‘The surgical repair of double cleft lips is about twice as difficult as in
method can achieve similar results while reducing the single clefts and the results are about half as good.’ This degree of
deleterious effects of forced movement. pessimism is no longer valid. Surgeons today have the added advantage
of 70 years’ further experience. Techniques have evolved and lessons
3. Simultaneous bilateral repair: Any primary asymmetry of the
have been learned. Much of the stigmata of the primary deformity can
lip and alar bases is best addressed at the primary repair, and
be corrected and many of the iatrogenic secondary deformities can be
is more difficult to accomplish secondarily.
avoided. In contrast to unilateral clefts, the bilateral deformity holds
4. Reduction of the prolabium: The prolabial cutaneous roll is the promise of symmetry and remarkable change for the better. I am
universally of poor quality relative to that of the lateral lip far more optimistic about these cases than I am about patients on the
elements. Prolabial cutaneous roll and vermilion should be other end of the spectrum; the unilateral microform cleft lip where
discarded. the initial deformity is so minor and expectations so high relative to
5. In complete bilateral clefts the upper labial sulcus is deficient. the potential change that can be realized by one operation. That is not
Prolabial mucosa should be preserved, elevated as a caudally to say that our goals and expectations should be any different. Rather,
based flap and then used to drape over the periosteum of the patience, time and numerous well-planned interventions are required
premaxilla to form the posterior wall of the upper labial to approach the same goal.
sulcus. In complete bilateral cleft lip and palate (Fig. 39.1) the prolabium
6. Formation of Cupid’s bow and median tubercle from lateral lip and premaxilla, both derivatives of the frontonasal process, remain
elements: The cutaneous roll forms an anatomic subunit of entirely separated from the lateral lip and maxillary arch elements
undulating non-hair bearing skin above the vermilion– derived from the maxillary processes. The prolabium will vary in
cutaneous junction. The continuity of this roll can be dimensions between affected individuals but there are some universal
reconstructed, if the lateral lip elements donate cutaneous roll findings. The cutaneous portion is void of the median groove and the
flaps to meet in the midline below the cutaneous shield of the philtral ridges which in the non-cleft lip define the shape and dimen-
prolabium. sions of the philtrum. The cutaneous roll of the prolabium is of poor
quality when compared to that of the lateral lip elements. The height
7. Muscle repair: Orbicularis oris muscle should be adequately
of the vermilion is inadequate. The labial sulcus is shallow if at all
separated from the lateral lip skin and alar bases. This will
present. The prolabium, lacking orbicularis muscle, sits flaccid on the
release the muscular bulge of the lateral lip elements and
premaxilla, rotated anteriorly into elevation and in extreme cases even
allow for midline approximation of the muscle throughout the
adopts a horizontal posture overlying an advanced and anteriorly
vertical length of the repair.
rotated premaxilla. The lateral lip elements in the bilateral cleft are
8. Primary rhinoplasty may be carried out – the extent of which similar to the lateral lip element of a unilateral cleft. On each lateral
remains controversial. lip element there will be good quality cutaneous roll and adequate
9. Thoughtful discard of tissue: ‘Little or no discard of tissue’ has vermilion height, however both are lost medially. Noordhoff2 has
been a tenet of cleft surgeons for decades. However, some of described an anatomic point (Noordhoff’s point) on the lateral lip
the cleft marginal tissue is hypoplastic. In efforts to adhere to element where the parallel lines of the vermilion–cutaneous junction
the ‘replace like with like’ principle it is best to discard tissue and the vermilion–mucosal junction (red line) begin to converge medi-
of poor quality. ally. Lateral to this point the cutaneous roll is full and the vermilion
10. With patience, time, and well-planned interventions much of is tall. Medial to this point the cutaneous roll becomes less obvious,
the stigmata of the primary deformity can be corrected and and the vermilion becomes progressively short in vertical height,
many of the iatrogenic secondary deformities can be ultimately becoming non-existent. Muscle bundles of the lateral lip
avoided. elements run along the cleft edge converging toward the alar base.
The caudal derivatives of the frontonasal process and the medial
elements of the maxillary arches are bridged by the nose. As the
premaxilla advances relative to the bilaterally hypoplastic maxillae
the nasal lobule becomes progressively distorted.3 The lower lateral 493

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 4
CRANIOFACIAL SURGERY

A B C D

E F G H

I J K L

M N O P
Fig. 39.1 Clinical case 1: complete bilateral cleft lip and palate. A, age 6 days – anterior view. B, age 6 days – antero-inferior view. C, age 6
days – intraoral view. D, age 6 days – right lateral view. E, age 4 months – with nasoalveolar molding appliance. F, age 4 months and 3
weeks – surgical markings. G, age 4 months and 3 weeks – immediate postoperative; anterior view. H, age 4 months and 3 weeks –
immediate postoperative; antero-inferior view. I, age 5 months – 1 week postoperative; anterior view. J, age 5 months – 1 week
postoperative; antero-inferior view. K, age 61/2 months – 6 weeks postoperative; anterior view. L, age 10 months – anterior view. M, age 16
months – anterior view. N, age 5 years – anterior view. O, age 5 years – antero-inferior view. P, age 5 years – anterolateral view.

494

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 cartilages, the framework of the nasal lobule become progressively
misshapen. The true dome points separate and the infra-tip lobule
becomes broad in the transverse dimension and shortened in the
skeletal maturity when segmental surgery can be performed safely
without risk to the adult dentition.
There are two general techniques of presurgical orthodontics,
39
antero-posterior dimension. The deformation effectively lengthens the passive and active. The passive technique of alveolar molding is our

Bilateral Cleft Lip

lateral crura and shortens the medial crura. The genua of the lower preferred method. The rationale is that this gentler method can achieve
lateral cartilages migrate progressively into the medial crura and the similar results while reducing the deleterious effects of forced move-
columella becomes progressively shorter. The angle between the medial ment. A custom acrylic plate is fashioned from an impression and
and lateral crura increases and the soft triangles become progressively stone model so to allow for initiation of the orthodontic course within
broad, short in the antero-posterior dimension and laterally displaced. the first 2–3 weeks of life. The plate is held in position by elastics
Bilaterally, the lower lateral cartilage, accessory cartilages and their taped to the patient’s cheeks. The plate remains in position at all times
shared investing perichondrium,4 under tension because of increased but may be removed for hygiene. The patient is seen thereafter every
separation of their skeletal base attachments (premaxilla and lateral 2 weeks and the plate modified; acrylic is shaved off areas towards the
piriform margin), raise fold of vestibular lining, the vestibular web. desired direction of alveolar movement and soft acrylic is added to
With the pull of the unopposed lateral lip musculature, the alar inser- areas of alveolar displacement (Fig. 39.2). Over a period of 6–12 weeks,
tions separate. considerable correction can be realized as the patient grows into the
periodically adjusted plate. When the alveolar clefts have been reduced
to 5 mm or less, a nasal stent can be added to the plate, and nasoal-
INDICATIONS AND CONTRAINDICATIONS veolar molding5,6 continues. The nasal stents then add a gentle ante-
riorly directed force to encourage lower lateral cartilage repositioning
and columellar lengthening.
L In the setting of associated congenital anomalies or illness,
The active Latham device7 has two custom fitted acrylic plates
delay is preferable to error.
which are fixed to the palate shelves with pins, and a wire loop, which
L Cleft lip repair should not be considered until the surgery can is placed transversely through the premaxilla behind the alveolus, and
be performed safely. anterior to the vomerine suture. An elastic chain retrudes the premax-
L In an otherwise healthy infant, a cleft lip can be repaired at illa while the turning of a ratcheted screw expands the lesser segments.
any age. The ‘rule of tens’, 10 weeks of age, 10 pounds in The device is placed in the operating room under anesthesia. Daily
weight, and 10 grams of hemoglobin is a widely accepted thereafter, the caregiver turns the intraoral screw to achieve the desired
pediatric dictum. effect.

Bilateral lip adhesion

Bilateral lip adhesion is an option to consider if presurgical orthodon-
Three months of age is the earliest that we would perform bilateral
tia is not available, or if presurgical orthodontia has not been success-
cleft lip repair at our institution. However, this is often delayed further
ful in correcting the premaxillary protrusion. The goal is not to achieve
by other factors. It often takes four to five months for presurgical
aesthetic, or anatomic reconstruction of the lip, but rather to provide
orthodontics to setback the overly protrusive premaxilla and align the
continuity of the lip. The lip will then provide a physiologic restraint
alveolar arches. If a lip adhesion is performed, definitive cleft lip repair
to anterior growth of the premaxilla. The design of the definitive che-
may be carried out at 12–18 months of age. There are some advantages
lioplasty should be marked first, and incisions for the lip adhesion then
to achieving definitive lip repair before 6 months of age. An older child
must be made on the cleft margin of the design. In this way important
just learning to walk may inadvertently disrupt the repair. The older
landmarks and tissues will remain intact, free of scar to allow for
the child the more challenging for the parent will be postoperative scar
subsequent definitive repair. Because the adhesion is often performed
massage.
under tension and there is no muscle within the prolabium bilateral
lip adhesions are prone to dehisce. The adhesion may also encourage
collapse of the lateral maxillary segments.
PREOPERATIVE HISTORY AND
CONSIDERATIONS Surgical premaxillary setback
Surgical repositioning of the protrusive premaxilla can be performed
primarily or secondarily. At primary lip repair primary surgical pre-
Management of the premaxilla maxillary setback is performed to allow for lip repair without tension.
Presurgical orthodontics Because of the great potential for subsequent midface growth restric-
The benefits of presurgical orthodontia have yet to be proven by ortho- tion primary premaxillary setback should be performed conservatively
dontic measure, but for the cleft surgeon faced with the challenge of and as a last resort. Premaxillary setback is probably better performed
a newborn with a severe bilateral cleft lip and palate, the assistance of secondarily either during the mixed dentition concurrent with alveolar
a skilled orthodontist is most welcome. The goal is to control the bone grafting, or at skeletal maturity by segmental Le Fort I osteot-
growth of the premaxilla, while allowing for catch up growth and omy.
expansion of the lateral segments. Gradually, an arch relationship
approaching normal can be established, and the widths of the alveolar
clefts can be reduced. This will allow for simultaneous bilateral cleft OPERATIVE APPROACH
lip repair with minimal tension of closure, and for primary gingivo-
periosteoplasty, if desired. Bilateral cleft lip repair
If the lip is repaired in the setting of uncorrected premaxillary
protrusion, the repaired lip will elongate vertically under tension and Principles
the lateral segments will collapse behind the protruding premaxilla. Simultaneous bilateral repair
The latter configuration will not allow for incorporation of the primary Unlike unilateral clefts, bilateral clefts hold the promise of symmetry.
palate in the subsequent palate repair. The residual bilateral alveolar This will be lost if the repair is staged. The slightest asymmetry will
clefts will communicate posteriorly by way of the residual cleft behind become more apparent with growth. The same patient may scar dif-
the incisive papilla. This is to be avoided if at all possible. Secondary ferently at different ages. Any primary asymmetry of the lip and alar
alveolar bone grafting will be a much greater surgical challenge and bases is best addressed at the primary repair, and is more difficult to
the reconstitution of normal dental arch relationships must await accomplish secondarily. 495

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 4 +++
CRANIOFACIAL SURGERY

–––
– –
– + + –
– + + –
+ +

A B C D

Fig. 39.2 Nasoalveolar molding. A, appliance – superior view. B, appliance – anterior view. C, appliance – antero-lateral view. D, The custom
orthodontic appliance is modified every two weeks. Hard acrylic is removed ( ) from the appliance from areas in the direction of desired
movement. Soft acrylic is added to areas (+++) on the opposiste side to apply gentle pressure. Over time the arch form adopts the shape of
the appliance. Once the arch form is improved the nasal components are added.

A B C D
Fig. 39.3 Creation of the upper labial sulcus. A, mucosal flap outlined. B, mucosal flap elevated. C, mucosal flap inset to create the posterior
wall of the sulcus. D, upper labial sulcus at palatoplasty (age 12 months).

Reduction of the prolabium of this roll can be nicely reconstructed, if the lateral lip elements
The prolabial cutaneous roll is universally of poor quality relative to donate cutaneous roll flaps to meet in the midline below the cutaneous
that of the lateral lip elements. The vermilion height of the prolabium shield of the prolabium. The incised lower margin of the prolabial
is inadequate. Prolabial cutaneous roll and vermilion should be dis- shield will determine the shape and depth of the newly reconstructed
carded. Use of the lateral prolabial skin as forked flaps for primary Cupid’s bow. Vermilion, with some marginal bundles of the orbicu-
or secondary columellar lengthening has been abandoned.8 These laris marginalis, should be carried with these lateral lip element flaps
flaps will not be used to repair the lip nor should it be used to second- to construct the red lip of the median tubercle. The incisions crossing
arily lengthen the columella. The short columella is the result of lower the free borders of the lateral lip elements should be made at Noord-
lateral cartilage malposition. Columellar lengthening procedures do hoff’s point. Accordingly, the cutaneous roll and vermilion flaps will
little to correct the primary deformity and add prolabial (often hair- carry only good quality cutaneous roll and vermilion of adequate
bearing) skin and unsightly scars to the columella and additional scar height to meet below the prolabial shield. If the incisions are made
to the base of the nose. medial to Noordhoff’s point, the cutaneous roll of Cupid’s bow will
be of relatively poor quality. More importantly the vermilion of the
Creation of the upper labial sulcus median tubercle will be of insufficient height, and the patient will be
In complete bilateral clefts the upper labial sulcus is deficient. The left with a whistle notch deformity, or elevation of the red line and
upper limit of the sulcus is low, often at the upper margin of the mucosal exposure. The exposed mucosa is visibly different in color
attached gingiva. Failure to deepen the sulcus will result in a tethered than the adjacent vermilion. It dries, chaps, flakes and peels. The
upper lip, poor aesthetics – particularly with animation, and may patient habitually licks the area or uses lip balm to keep it moist. If
contribute to periodontal disease and compromised dentition. Prola- the incisions are made lateral to Noordhoff’s point, the surgeon will
bial mucosa should be preserved, elevated as a caudally based flap be unnecessarily reducing the transverse length of the lateral lip
and then used to drape over the periosteum of the premaxilla to form elements and the patient will be left with a tight upper lip.
the posterior wall of the upper labial sulcus (Fig. 39.3). Lateral lip
element mucosa will meet in the midline to form the anterior wall of Muscle repair
the sulcus. The prolabial mucosal flap should be raised in a plane just Orbicularis oris muscle should be adequately separated from the
deep to the mucosa and have the thickness of a graft, to avoid bulk lateral lip skin and alar bases. This will release the muscular bulge
and mobility of the tissue.9 of the lateral lip elements and allow for midline approximation of the
muscle throughout the vertical length of the repair.
Formation of the Cupid’s bow and the median tubercle
from the lateral lip elements Primary rhinoplasty
The cutaneous roll forms an anatomic subunit of undulating non-hair Any tip work which requires an external incision and leaves a visible
496 bearing skin above the vermilion–cutaneous junction. The continuity scar on the nose, the central presenting part of the face, should be

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 delayed until access to the tip can be achieved through a transcolumel-
lar and open rhinoplasty approach. There is no excess of skin. Skin
excisions of any kind should be avoided.
and produces a visible scar. Furthermore, while perhaps not appre-
ciable in the infant, the excision produces rotation of the nasal vibrisi
such that they are visible from the anterior view. The most difficult
39

Bilateral Cleft Lip

I am in full agreement with the abandonment of the older problem however becomes apparent at secondary tip rhinoplasty. If
techniques of columellar lengthening that involved the recruitment an attempt is made to further improve lower lateral cartilage position
of skin from the prolabium or from the nostril sill into the columella. secondarily, further anteromedial repositioning of the domes will
I question those who feel that primary repair of the nasal deformity is uncover the skin deficiency produced by the previous rim excisions
paramount and who feel that lower lateral cartilage repositioning and notching will result in the regions of the soft triangles.
cannot wait to be performed secondarily. The most difficult to correct
of the secondary cleft lip nasal deformities are iatrogenic. These
deformities include: alar rim excisions, lateral rhinotomy scars, nasal The achievable goals of bilateral cleft lip primary rhinoplasty
tip scars and columellar lengthening scars. With the exception of the include:
transcolumellar incision at secondary rhinoplasty, cutaneous nasal
incisions are unnecessary and produce visible cutaneous scars which L a midline columellar base;
are permanent, and as such may at best be amenable to scar revision L symmetrical alar bases;
only. L reduced inter-alar distance;
‘The columella is in the nose’. So too are the true dome points and L nares of equal size and shape;
the support of the infratip lobule. The short columella is secondary to
lower lateral cartilage deformation, and not the cause of the deformity. L sufficient release of the lower lateral cartilage – accessory
The lower lateral cartilages need to be repositioned into normal con- cartilage complexes from the lateral piriform rims so to allow
figuration before any lengthening of the columella, and the infra tip anteromedial repositioning of the splayed alar bases without
lobule can be realized. Because the deformation of the lower lateral accentuation of the alar rim buckling and lateral alar flaring;
cartilages is secondary to underlying deformation of the skeletal base, L anteromedial advancement of the lateral crura and domes
the skeletal base deformity should be corrected as best as possible concurrent with:
before any attempt is made to correct the nasal tip, infratip and colu- L creation of the normal overlap of the cranial margin of the
mella. Presurgical orthodontics or lip adhesion can correct the protru- lateral crura over the caudal margins of the upper lateral
sion of the premaxilla, but the bilateral maxillary hypoplasia of the cartilages.
alar bases and lateral piriform margins must await alveolar bone
grafting. Most importantly, the small lower lateral cartilages of an
infant lack the structural integrity required to keep their position and
to effectively support the overlying soft tissues of the tip and infratip Thoughtful discard of tissue
lobule. ‘Little or no discard of tissue’15 has been a tenet of cleft surgeons for
When the child is older, the lower lateral cartilages can be ade- decades. It must be recognized that some of the cleft marginal tissue
quately exposed using a transcolumellar and infra-alar rim incisions is hypoplastic. In efforts to adhere to the ‘replace like with like’ prin-
approach. Lower lateral cartilages can be adequately released from ciple it is best to discard tissue of poor quality. The quality of the lip
their lateral attachments and fully mobilized by V-Y advancement as can only be as good as the component parts. The prolabium is too
chondromucosal flaps.10 The true dome points can be identified, defined wide and will grow further following repair.16
and repositioned anteromedially, increasing the anteroposterior length
of the nose by increasing the anteroposterior lengths of both the Minimal introduction of scar
infratip and of the columella. A columellar strut can be added for Scar should be minimized and placed along the seams of anatomic
support. As the skin envelope is redraped over the now antero-medi- subunits.
ally repositioned dome points, the skin of the slumped alar rims will
be supported and the nares will adopt a circular shape. There will be Author’s preferred technique for complete
no skin excess, and no need for trimming. symmetrical bilateral clefts
Formal attempts at lower lateral cartilage repositioning at primary See Figs. 39.1 and 39.4.
lip repair have their limitations. Access is a problem. McComb11 uti-
lized an external ‘flying bird’ incision. Mulliken12 described an exter- Markings
nal midline tip incision but later changed to a ‘semi-open approach’8 See Fig. 39.5.
through bilateral marginal rim incisions. Trott and Mohan13 described With the patient supine, general anesthesia is induced. An uncuffed
elevation of the prolabial skin in continuity with the skin of the colu- oral Rae tube is placed and taped in the midline to the chin. The tube
mella to gain an open rhinoplasty exposure of the lower lateral car- is further immobilized with a mouth pack marked with a long silk
tilages. Alteration of the lip–columellar junction and viability of the suture or umbilical tape. A head ring and shoulder roll are placed.
prolabium are concerns with this approach. The retrograde nasal Presurgical photographs are taken and anthropometric measurements
approach of Morovic and Cutting14 likely has less disruption of the are obtained with calipers. The face is prepared and draped. Sterile
lip–columellar angle but visualization of the domes is less than with tapes are placed over the closed eyelids. Markings are made with
the open approach and as such they combine with their retrograde gentian violet dye and pen and later tattooed with a 25-gauge needle
approach the bilateral marginal rim incisions of Mulliken. Mulliken and dye.
also describes excision of ‘extra skin in the soft triangles’. This excess The size and shape of the philtral flap will vary depending on the
of skin represents inadequate cartilaginous support of the infratip age and race of the patient, the appearance of the parents, and the
lobule. The cartilages of the infant cannot be mobilized sufficiently available vertical height of the prolabial skin. The prolabial skin is
through this semi-open approach nor do they have the structural reduced to a tie-shaped shield. For in infant of 4–6 months of age, the
integrity to project the overlying soft tissues of the tip sufficiently. As rough dimensions of the flap will be 2.5–3 mm at the lip–columellar
a result, the skin of the soft triangles and anterior nostril margins lack junction (between points 3 and 3`), 3.5–4.5 mm between lower corners
the support offered by a well projected tip and they remain in a (points 4 and 4`), and 6–8 mm in height (between points 1 and 2). The
depressed position. As tempting as it may be to excise this rim skin three lower points (4`, 2 and 4) are positioned above the existing cuta-
and achieve rounded nares at the primary repair, this should be neous roll of the prolabium; the roll and vermilion will be discarded.
avoided. In contrast to the intranasal rim incision used as an access The depth of the ‘V’ made by these three lower points will determine
to the dome and lateral crus, an alar sculpting excision removes tissue the ultimate shape of Cupid’s bow. Race and appearance of the parents 497

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 4
CRANIOFACIAL SURGERY

A B C

D E F
Fig. 39.4 Clinical case 2 – Bilateral complete cleft lip and palate with left Simonart band – age 5 months. A, Bilateral complete cleft lip and
palate with left Simonart band – age 5 months. B, age 5 months – preoperative appearance. C, age 5 months – surgical markings. D, age 5
months – immediate postoperative. E, age 9 months. F, age 15 months.

may influence surgeon preference at this stage. Bilaterally, lateral to overlap. If the lateral lip element is long in vertical height relative to
the upper base of the flap, the markings will pass superolaterally in a the height of the prolabium, more commonly observed in incomplete
curvilinear fashion just outside the lip columellar crease into the clefts, a medially base wedge excision of lateral lip skin can be per-
nostril sill (points 5` and 5) and then will continue posteriorly into formed. This wedge excision should be kept medial to subalare so to
the nose. A rectangular prolabial mucosal flap caudally based on the avoid the unnecessary lateral rhinotomy (circumalar) scar.
attached gingiva is marked.
On each lateral lip element Noordhoff’s point is identified. This Dissection
point (6) will mark the medial limit of the cutaneous roll–vermilion Photographs of the surgical plan are taken. Bilateral infraorbital nerve
flap and will ultimately meet its contralateral counterpart in the blocks are performed with 1% lidocaine with epinephrine (1 : 200,000)
midline. The point is identified on the lateral lip element where the solution. The prolabium, alar bases, piriform margins and inferior
parallel lines of the vermilion–cutaneous junction and the vermil- turbinates are infiltrated with the same. Care is taken to not exceed
ion–mucosal junction (red line) begin to converge medially. Lateral to the toxic dose (7 mg lidocaine [with epinephrine] per kg).
this point the cutaneous roll is full and the vermilion is tall. Medial Release of the lateral lip elements is performed first. This will
to this point the cutaneous roll becomes less obvious and the vermil- ensure the surgeon that the repair can be performed without undue
ion becomes progressively short in vertical height and ultimately non- tension. Otherwise, bilateral lip adhesion should be performed. Mucosal
existent. Point 7 is then placed above Noordhoff’s point just above the incisions start at the anatomic point (12) where the cleft margins of
cutaneous roll. Point 8 is positioned below Noordhoff’s point on the the lip, alveolus and vestibule converge. Laterally from this point an
vermilion–mucosal junction. These three points (7, 6 and 8) should upper buccal sulcus incision is performed, with a back-cut if necessary.
form a straight line perpendicular the vermilion–cutaneous junction. Cephalad from this point the incision will ascend within nasal ves-
The line will be drawn straight across the cutaneous roll (between tibular mucosa along the piriform margin to the level of the inferior
points 6 and 7) and will be drawn slightly convex medially across the turbinate. Soft tissues of the lateral lip element and alar base are
vermilion (between points 6 and 8). Point 9 is marked just above the released from the underlying maxilla in a supraperiosteal plane. Ade-
cutaneous roll lateral to point 7. The length of the resultant cutaneous quate mobilization to allow for repair without tension is accomplished.
roll to the vermilion flap (distance between points 7 and 9) should At this stage it is decided whether a turbinate flap will be used. The
equal the distance between points 2 and 4. Two points (5 and 10) are width of the alveolar cleft will influence the decision. If the gap is too
chosen which will meet at the height of the lip repair at the anterior narrow, access to the turbinate flap will be limited. If the gap is great,
nostril sill. Point 5 is chosen at or just lateral to the lip–columellar the turbinate flap may not be broad enough to be useful. The rectan-
crease. Point 10 is chosen medial to the alar insertion. These two gular flap is based anteriorly. It will be rotated caudally and anteriorly
points do not represent specific anatomic sites and require some deci- to line the raw area produced by the anteromedial repositioning of the
sion making by the surgeon. The two points should meet so to provide alar base. The lower lateral cartilage–accessory cartilage complex is
(1) appropriate positioning of the alar base relative to the columella released from periosteal attachments to the piriform rim. If a turbinate
base and (2) nostril margins of symmetric shape and circumference. flap has been elevated this will necessitate a transition to a subperios-
Point 11 is positioned near the cleft margin at a distance from point teal plane along the piriform rim. If a turbinate flap is not utilized this
9 equal to the distance between points 3 and 4, and at a distance from can be accomplished in the submucosal plane. Adequate release of the
point 10 equaling the distance between points 3 and 5. Incisions of nose from the piriform rim is confirmed when anteromedial advance-
498 the lateral lip element flaps are simplified if lines 7–9 and 11–9 ment of the alar base is accompanied by a rounding of the alar margin

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 Surgical markings for complete bilateral cleft lip repair

A
Surgical markings for complete bilateral cleft lip repair
B
39

Bilateral Cleft Lip

5' 3' 1 3 5
10' 10
11' 11

7' 4' 4 7
2

9'
6'
8' 8 6 9

Surgical markings for complete bilateral cleft lip repair

C

Fig. 39.5 Surgical markings for complete bilateral cleft lip repair (see text for description). 1 – midline of the lip-columellar crease,
2 – midline of the prolabium just above the cutaneous roll, 3 – height of the philtral column incision at the lip-columellar crease, 4 – base of
the philtral column incision above the cutaneous roll of the prolabium, 5 – medial point of closure in the nostril sill, 6 – Noordhoff’s point,
7 – point above the cutaneous roll above Noordhoff’s point, 8 – point on vermillion-mucosal junction below Noordhoff’s point, 9 – base of the
philtral column incision of the lateral lip element, 10 – lateral point of closure in the nostril sill, 11 – height of the philtral column incision of
the lateral lip element.

rather than by a buckling or kinking lateral to the soft triangle. Primary posterior incised margin of the initial vestibular mucosal incision. The
rhinoplasty is performed (see below). Previously marked incisions of distal (now caudal) end of the flap is sutured to the upper margin of
the lateral lip completed with scalpel full thickness through skin and the mucosa of the lateral lip element across the alveolar cleft gap. If a
muscle leaving the mucosa intact. From points 8 and 8`, vertical inci- turbinate flap has not been elevated, the area to be covered by the flap
sions are made through mucosa to points 12 and 12`. Cleft marginal is left to heal secondarily.
tissues are discarded. Dissection at the incised edges produce three The caudally based prolabial mucosal flap is elevated in a submu-
layers (skin, muscle, and mucosa) for closure. The dissection in the cosal plane. The flap should be the thickness of a full thickness mucosal
plane between skin and muscle extends as far laterally as far as neces- graft so to prevent bulkiness and mobility of the tissue. The prolabium
sary so to relieve the muscle bulge of the lateral lip element. The lateral is elevated from the anterior surface of the premaxilla to the base of the
lip element orbicularis is freed from its attachment to the alar base. anterior nasal spine. The flap is tacked (5-0 chromic) to the premaxil-
Dissection must be sufficient to allow free caudal rotation of the cuta- lary periosteum. This will form the posterior wall of the gingivobuccal
neous roll–vermilion flaps. sulcus, anterior to the premaxilla. Mucosa of the lateral lip elements
The turbinate flap (Fig. 39.6) is inset with 5-0 chromic suture. Its meet in the vertical midline to form the anterior wall of the sulcus.
anterior margin (following caudal and anterior rotation) is sutured to An alar base cinch stitch, of 4-0 PDS, is utilized to narrow the
the anterior incised margin of the initial vestibular mucosal incision. interalar base width. Muscle is approximated in the vertical with 5-0
The distal half of the posterior margin of the flap is sutured to the Monocryl. Skin is approximated with deep dermal sutures of 5-0 499

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CRANIOFACIAL SURGERY

A B
Fig. 39.6 Turbinate flap. A, elevated bilaterally for bilateral complete cleft lip repair. B, inset in a unilateral complete cleft lip and palate
repair; the flap covers the raw area created by alar base advancement.

Monocryl and simple skin stitches of 7-0 Proline. Vermilion is approx- The above techniques of primary bilateral rhinoplasty share in
imated with 7-0 Vicryl sutures (Fig. 39.3). common McComb’s vision of lower lateral cartilage repositioning;
approximation of the separated domes and suspension of the lateral
Primary rhinoplasty crura. I am in full agreement with the concept of lower lateral cartilage
McComb11 championed primary bilateral rhinoplasty utilizing an open repositioning but question the extent to which this can be accom-
approach through a ‘flying bird’ incision. This incision leaves a notice- plished at primary lip repair, and at what cost? My opinion is that
able scar but allows sufficient access to the nasal tip to allow for primary rhinoplasty offers an opportunity to incompletely correct the
anteromedial advancement and approximation of the splayed domes. cartilage deformity and offers a greater opportunity to introduce unnec-
The tip is narrowed and projected as the flying bird incision is closed essary iatrogenic deformity to the skin envelope. Unless there has been
in a V-Y fashion. The McComb primary rhinoplasty is classically sufficient correction of the underlying skeletal nasal base deformity
performed in combination with bilateral lip adhesion with definitive (premaxillary protrusion and bilateral lateral maxillary hypoplasia),
lip repair performed at a second stage. lower lateral cartilage repositioning will be incomplete. The surgeon
Mulliken8 initially utilized a vertical midline tip incision, but later will have introduced scar and potentially injury to the cartilages, which
abandoned this approach in favor of bilateral rim incisions. Through will only make definitive rhinoplasty more challenging. If the reposi-
these access incisions, sutures are placed to approximate the medial tioning is incomplete, the support to the overlying skin envelope
crura and domes, and to suspend each lateral crus to the ipsilateral offered by the cartilages will be inadequate. Now there is too much
upper lateral cartilage. The bilateral rim incisions are then converted temptation to alter the skin envelope. Again, a plea is made for con-
to sculpting excisions. ‘Excess skin in the soft triangles’ is excised. The servatism and the avoidance of alteration to the skin envelope. My
excisions then extend medially to the skin of the lateral columella in primary bilateral cleft lip rhinoplasty is relatively conservative at the
order to narrow the nasal tip, define the columellar–lobular junction, nasal tip, but perhaps more aggressive at the nasal base. Full mobiliza-
elongate the nostrils and narrow the columella. tion of the alar bases is accomplished in an extraperiosteal plane. The
Trott and Mohan13 devised a method of primary open tip rhino- dissection continues cephalad to release the attachments of the lower
plasty achieved by elevating the skin of the prolabium and columella, lateral cartilage–accessory cartilage complex from the lateral piriform
in continuity with the skin overlying the domes. The approach does margin. The release must extend above the level of the inferior turbi-
not require external skin incisions but requires undermining of the nate. This will allow for mobilization of the alar base without distor-
skin of the nasolabial crease and places the prolabial skin at the distal tion of the nostril margin. The dome is retracted anteromedially and
extreme of the nasal flap. internal nasal valve plication sutures (Fig. 39.7) are placed. These
Cutting’s6 primary rhinoplasty is performed following a course of sutures maintain some anteromedial advancement of the lateral crura
presurgical columellar stretching achieved by presurgical nasoalveolar and produce the overlap of the scroll area. Laterally, alar transfixion
molding.5,6 A prolabial–columellar flap is elevated utilizing a ‘retro- sutures are placed. If the alveolar cleft gap is sufficient to allow for
grade’ approach through the membranous septum. Bilateral marginal adequate access, and if the gap is not too great to be spanned by the
rim incisions provide additional access to the lower lateral cartilages. flap, a turbinate flap is used. The flap will resurface the raw area above
Interdomal mattress sutures and internal suspension sutures are the lateral lip element alveolus produced by mobilization of the alar
placed and the external skin of the rim incisions are folded to produce base. An alar base cinch stitch is used to narrow the interalar
500 the inner surfaces of the soft triangles as described by Tajima.17 distance.

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 Symmetrical incomplete bilateral clefts
See Fig. 39.8.
Markings are similar to that of the complete cleft. Because the
excisions should be kept medial to subalare to avoid a visible lateral
rhinotomy scar. 39
Mixed bilateral clefts

Bilateral Cleft Lip

lateral lip elements are usually vertically long, medially based wedge
excisions above points 10 and 10` are often necessary. The wedge See Fig. 39.9.
The repair is designed asymmetrically, often requiring an upper lip
wedge excision on the incomplete side. Rhinoplasty should be more
aggressive on the complete side in order to achieve as symmetric a
nose possible.

Asymmetrical bilateral clefts with unilateral forme

fruste cleft lip
See Fig. 39.10.
Forme fruste or microform clefts present an interesting challenge
for the cleft surgeon. As with the unilateral microforme cleft, the
surgeon and family must decide whether to operate on the cleft or
accept the minor deformity. If it is decided that the degree of deformity
on the microform side is not sufficient to warrant intervention, then
the cleft on the contralateral side can be treated as a unilateral cleft.
If on the other hand it is decided that the microform cleft will benefit
from correction, my preference is to treat the lip as an asymmetrical
bilateral cleft lip. Synchronous bilateral cleft lip repair offers the oppor-
tunity to correct the associated central vermilion deficiency and a
single operation may offer the best chance for symmetry of form and
for lip scars of uniform quality.

Secondary bilateral cleft lip rhinoplasty

See Figs 39.11 and 39.12.
My preference is to attempt correction of the nose as a formal
rhinoplasty performed in the teen years. If, however, the deformity is
significant, a rhinoplasty can be performed earlier, perhaps at age six
or seven at the earliest, to reshape the lobule of the nose. Potter’s
Fig. 39.7 Placement of internal nasal valve placation suture. rhinoplasty10 in my opinion is the most rational procedure to address

A B C

D E
Fig. 39.8 Clinical case 3 – Incomplete bilateral cleft lip. A, age 41/2 months – preoperative appearance. B, age 41/2 months – surgical
markings; anterior view. C, age 41/2 months – surgical markings; antero-inferior view. D, age 41/2 months – immediate postoperative;
anterior view. E, age 12 months – anterior view. 501

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 4
CRANIOFACIAL SURGERY

A B C D
Fig. 39.9 Clinical case 4 – Mixed bilateral cleft lip. A, age 5 months – preoperative appearance. B, age 5 months – surgical markings; antero-
inferior view. C, age 5 months – immediate postoperative; anterior view. D, age 12 months – anterior view.

A B C

D E E
Fig. 39.10 Clinical case 5 – Left incomplete unilateral cleft lip with right forme fruste cleft. A, age 4 months – preoperative appearance. B,
age 4 months – surgical markings; anterior view. C, age 4 months – immediate postoperative; anterior view. D, age 5 years – anterior view.
E, age 5 years – antero-inferior view. F, age 5 years – left lateral view.

Secondary bilateral rhinoplasty – Modified after Potter (1954) Fig. 39.11 Secondary bilateral rhinoplasty – modified
after Potter (1954). The tip of the nose is opened
utilizing transcolumellar and lintra-alar rim incisions.
A B C
The latter are extended posterolaterally within
mucosa just below the caudal margins of the lower
lateral cartilages and accessory cartilages as far as
the piriform rim. The incisions continue
anteromedially as back cuts as intercartilagenous
incisions between the upper and lower lateral
cartilages. The lower lateral cartilages are mobilized
anteromedially as chondromucosal flaps and the
advancement closed in a V-Y fashion. Modifying
Potter’s initial description, a columellar strut is used.
Suspension sutures, dome defining sutures, interdomal
502 sutures, and tip grafts are also added as indicated.

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 39

Bilateral Cleft Lip

A B C D

E F G
Fig. 39.12 Clinical case 6 – Repaired right unilateral cleft lip with forme fruste left cleft lip and bilateral cleft lip nasal deformity – Secondary
bilateral rhinoplasty – age 17 years. A, preoperative appearance – anterior view. B, preoperative appearance – anterolateral view.
C, preoperative appearance – antero-inferior view. D, intraoperative appearance – bilateral lower lateral cartilage malposition.
E, postoperative appearance – anterior view. F, postoperative appearance – anterolateral view. G, postoperative appearance – antero-inferior
view.

the deformity of the tip and nostril margins. The procedure involves
an open rhinoplasty through a transcolumellar incision and infra-alar anterior fistula cannot be corrected until segmental Le Fort
rim incisions along the caudal margins of the lateral crura. On each osteotomy at skeletal maturity.
side the infra-alar incision is continued posterolaterally along the L The lip repair as described above will not look pleasing at the
caudal margin of the vestibular web to the piriform margin at the level end of the case. Faith and patience must prevail. The prolabial
of the inferior turbinate. At this level, a submucosal incision releases flap is initially small. The repair will appear tight and geometric.
the attachments of the lower lateral cartilage–accessory cartilage Nostrils will appear misshapen. After a month, the scars may
complex to the piriform margin and a back-cut is made in an intercar- raise, thicken and contract. However, with time the prolabial
tilaginous fashion between the lateral crus and the upper lateral carti- skin flap will grow, scars will mature, the form of the lip will
lage. In this manner, a chondromucosal flap containing the lateral crus soften and the initially angled limbs of the lip roll will adopt
is produced. The flap is advanced anteromedially and resultant defect and undulating and more natural form. Nostrils will round to
is closed in a V-Y fashion. The domes are advanced anteromedially. some extent and will benefit from formal open rhinoplasty.
Modifying Potter’s original description, medial crura and tip projection
L Massage with sufficient pressure, duration, and periodicity,
are supported with a medial crural strut (resorbable fixation plate18 in
over months will optimize the appearance of the repair. Parents
younger patients and septal cartilage in older patients). Dome defining
are instructed to massage the scars with petroleum jelly as
sutures are placed. The normal overlap of the cranial margin of the
lubricant, starting at 4–6 weeks until the scars are mature.
lower lateral cartilage with the caudal margin of the upper lateral car-
tilage at the scroll area is recreated and maintained with suspension
sutures. In the older patient, if dome projection remains inadequate,
a tip graft (septal cartilage) is added. The nasal skin envelope is
redraped. Rounding of the nostril margins and modest columellar COMPLICATIONS AND SIDE EFFECTS
lengthening is achieved with the redraping of the skin.
Airway obstruction and anesthetic complications are rare in the
otherwise healthy infant. Bleeding, infection and wound dehiscence
are rare potential complications and can be minimized by diligent
adherence to the sound principles of surgical technique. Scar contrac-
Optimizing outcomes
tion is very common and entirely treatable with scar massage and time.
L The optimal result will come only from appropriate presurgical True hypertrophic scars are much less common.
orthodontics, thoughtful repair design, good technique and
appropriate postoperative care.
L The protrusive premaxilla must be corrected before cleft lip POSTOPERATIVE CARE
repair. If the lateral segments are allowed to collapse behind
the premaxilla before palatoplasty, the abnormal arch form and Patients are allowed to resume oral intake in the recovery room fol-
lowing surgery. Most of our patients with complete clefts which involve 503

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 4 the secondary palate will use the Haberman FeederTM. Patients with 3. Fisher DM, Mann RJ. A model for the cleft lip nasal deformity. Plast
cleft lip only are often able to breast feed and this is allowed postop- Reconstr Surg 1998; 101(6):1448.
eratively. Intravenous lines are maintained until the patient is meeting 4. Le Pasteur J, Firmin F. Reflexions sur l’auvent cartilageneux nasal. Ann
his/her daily oral requirements. Patients are observed in hospital over- Chir Plast 1977; 22:1.
CRANIOFACIAL SURGERY

night following surgery. Parents are given instructions regarding lip 5. Grayson BH, Santiago PE, Brecht LE, Cutting CBL. Presurgical
nasoalveolar molding in infants with cleft lip and palate. Cleft Palate
care. The suture line is cleansed with a cotton tip applicator and
Craniofac J 1999; 36:486.
saline. Antibiotic ointment is used for 3 days and then the suture line
6. Cutting C, Grayson B, Brecht L, Santiago P, Wood R, Kwon S.
is kept moist with petroleum jelly. Patients wear arm restraints for 2 Presurgical columellar elongation and primary retrograde nasal
weeks. reconstruction in one-stage bilateral cleft lip and nose repair. Plast
The patient is returned to hospital at 5–7 days postoperatively for Reconstr Surg 1998; 101:630.
suture removal. At our hospital, this is done in the operating room 7. Millard DR, Jr, Latham RA. Improved primary surgical and dental
under a brief general anesthetic. This allows for the use of fine non- treatment of clefts. Plast Reconstr Surg 1990; 86:856.
absorbing skin sutures and their clean removal without disruption of 8. Mulliken JB. Primary repair of bilateral cleft lip and nasal deformity.
the repair. Plast Reconstr Surg 2001; 108:181.
Follow up is arranged at 4–5 weeks. At this visit parents are given 9. Marcus JR, Fisher DM, Lenz GJ, Magee WP, Zuker RM. Inadequate
instructions for scar massage. For most babies obligate nasal breathing gingivolabial sulcus remains an avoidable problem after bilateral cleft lip
repair. Plast Reconstr Surg 2005; 115:220.
tends not to be a problem after age 3 months. In patients with mul-
10. Potter J. Some nasal tip deformities due to alar cartilage abnormalities.
tiple medical diagnoses and developmental delay we prefer to postpone
Plast Reconstr Surg 1954; 13:358.
cleft lip repair in order that this risk is minimized.
11. McComb H. Primary repair of the bilateral cleft lip nose: a 15-year
review and a new treatment plan. Plast Reconstr Surg 1990; 86:882.
12. Mulliken JB. Principles and techniques of bilateral complete cleft lip
CONCLUSION repair. Plast Reconstr Surg 1985; 75:477.
13. Trott JA, Mohan N. A preliminary report on one stage open tip
The patient with complete bilateral cleft lip and palate represents the rhinoplasty at the time of lip repair in bilateral cleft lip and palate: The
severe extreme within the spectrum of clefting. The bilateral deformity Alor Setar experience. Br J Plast Surg 1993; 46:215.
holds the promise of symmetry and remarkable change towards normal. 14. Morovic CG, Cutting C. Combining the Cutting and Mulliken methods
With patience, time, and well-planned interventions much of the for primary repair of the bilateral cleft lip nose. Plast Reconstr Surg
2005; 116:1613.
stigmata of the primary deformity can be corrected and many of the
iatrogenic secondary deformities can be avoided. 15. Millard DR, Jr. Unilateral cleft lip deformity. In: Plastic Surgery.
Philadelphia: WB Saunders; 1990:2632.
16. Mulliken JB, Burvin R, Farkas LG. Repair of bilateral complete cleft lip:
intraoperative nasolabial anthropometry. Plast Reconstr Surg 2001;
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Obstet 1947; 85:20. 18. Wong GB, Burvin R, Mulliken JB. Resorbable internal splint: an adjunct
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