CME

Primary Repair of Bilateral Cleft Lip and

Nasal Deformity
John B. Mulliken, M.D.
Boston, Mass.

Learning Objectives: After studying this article, the participant should be able to: 1. List five principles that guide
synchronous repair of bilateral complete cleft lip and nasal deformity. 2. Explain how different growth rates for the
principal nasolabial features are applied during primary repair. 3. Describe two approaches for positioning the alar
cartilages to form the columella. 4. Discuss the influences on referral patterns for a newborn with bilateral cleft lip.
Traditional repair of bilateral cleft lip focused on labial
closure but accentuated the nasal deformities, which were
addressed later. By the end of the past century, singlestaged labial closure had replaced the old multistaged
procedures and the technical emphasis had begun to shift
from secondary to primary nasal correction. Now, presurgical maxillary orthopedics sets the bony foundation for
synchronous nasolabial repair and for closure of the alveolar clefts. The study of normal nasolabial growth and
the typical stigmata of the conventional methods provides
the necessary foreknowledge to guide surgical sculpture in
three dimensions and to anticipate the fourth dimension.
The convergence of several forces are changing referral
lines for children born with bilateral cleft lip. These include affirmation of centers of excellence, surgeons selfregulation, prenatal diagnosis, economics of health-care
delivery, and increasing parental sophistication. These
pressures are not necessarily in conflict. Care by a subspecialized plastic surgeon and experienced team is in the
best interests of the child and the third-party
payer. (Plast. Reconstr. Surg. 108: 181, 2001.)

These branded children beckoned surgeons to

change their traditional operative strategies.
Over the past decade, two important advances have been made in the repair of bilateral cleft lip and nasal deformity: (1) evolution
to single-stage nasolabial closure with positioning of the alar cartilages and sculpting of the
soft tissues to shape the columella and nasal
lobule, and (2) improved techniques for presurgical maxillary alignment to permit closure
of the alveolar clefts and facilitate primary nasolabial repair. Although the principles for single-stage repair are established, craftsmanship
continues to evolve. Now, over a half century
after Barrett Browns discomfortable observation, it can be said that the outcome for the
infant born with bilateral cleft lip is equal to
and can surpass that of its unilateral counterpart.2 Furthermore, given the preoperative advantage of nasolabial symmetry, these children
require very few revisions.
Before undertaking primary repair of double cleft lip, there are some lessons to review.

James Barrett Brown and colleagues introduced their 1947 article with the pithy statement that a bilateral cleft lip is twice as difficult
to repair as a unilateral cleft and the results are
only half as good.1 Indeed, techniques for correction of bilateral cleft lip have lagged behind
those for unilateral cleft lip. The infant with
bilateral cleft lip has been subjected to multiple procedures only to endure sundry revisions
throughout childhood. Despite the surgeons
best efforts, the childs diagnosis was painfully
obvious to all even at a distanceand these
stigmata were not easily erased by revisions.

LESSONS

FROM

SURGICAL HISTORY

Many complex malformations, once only

reparable by staged operations, can be corrected, usually more successfully, in a single
procedure. This lesson is illustrated in the annals of the bilateral cleft lip and nasal deformity. Emphasis had focused on labial closure
and ignored nasal distortion. Surgical text rec-

From the Division of Plastic Surgery and Craniofacial Centre, Childrens Hospital, Harvard Medical School. Received for publication August
4, 2000; revised October 17, 2000.

181

182

ommended staged repair, one side of the cleft

and then the other. There was a misconception
that the diminutive prolabium lacks the potential for growth. Techniques for repairing the
double-labial cleft were adapted from those
used for the more common unilateral form,
and they typically involved the introduction of
a rectangular or triangular flap from the lateral
labial elements to augment prolabial height.
These procedures left geometric (often asymmetric) labial scars and usually resulted in a
long-lip deformity and a tight upper lip.
Straight-line repair minimized vertical elongation of the central lip but produced an abnormally wide and shield-shaped philtrum. There
was longstanding controversy over whether or
not to preserve the prolabial vermilion, leave it
as a tiny strip, or excise it completely. Apposition of the orbicularis oris muscle was usually
not mentioned. Some surgeons thought muscular closure would inhibit premaxillary
growth.3 Nevertheless, with increasing attention to muscular closure in the unilateral deformity, reports began to underscore the importance of orbicular repair in bilateral
clefts.4 8
Surgeons conceded to the complexity of the
bilateral cleft nasal deformity and deferred correction.9 11 The conventional teaching was that
the columella is inadequate, and numerous
secondary procedures were devised to elongate the short columella. There are two major strategies.12 The first, popularized by Cronin, involves rotating bipedicled straps of tissue
from the nostril sills.13 This method gives modest columellar length. A second method, the
forked-flap procedure of Millard, involves recruiting labial tissue to the columella.5,9,14
There are two permutations of this method. In
infants with a wide prolabium, the tines of the
forked flap are banked at the time of labial
closure and transposed to the columella in
early childhood. More often, Millard prefers
three-stage columellar lengthening: (1) bilateral labial adhesions to stretch the prolabium;
(2) elevation, rotation, and banking the tines
while narrowing the philtrum (at age 18
months); and, finally, (3) retrieval of the prolabial prongs and elevation, along with the medial crura, to augment the columella (at age 2
years).15
The forked-flap method, like all secondary
procedures, causes peculiar tertiary distortions.16 Most techniques introduce a nexus of
scars across the columellar-labial junction. This

PLASTIC AND RECONSTRUCTIVE SURGERY,

July 2001

produces a midline nasolabial crease that

deepens with smiling. The circumferential
philtral scar produces a bulge rather than a
dimple. The best prolabial scars follow repair
in infancy, done under minimal tension. Recruiting tines of a forked flap from the central
lip in a child can cause thickened and permanently wide philtral scars. Even in the best of
hands, the staged forked-flap procedure results
in an unusual appearance: (1) a rectangular
columella (with a broad base and without a
waist); (2) a sharp columellar-labial angle; 3)
abnormally elongated/enlarged nostrils; (4) a
tendency to columellar over-elongation with a
disproportionate ratio of nostril length-to-nasal
tip; and, sometimes, (5) a downward drift of
the columellar base.16 18 Furthermore, the medial crura become unnaturally positioned in
the tip, resulting in a break at the columellarlobular connection. Because of these problems, some surgeons began to wonder whether
labial skin belongs in the columella or if
more tissue is needed at all.
Whereas delayed nasal repair was customary
for the repair of bilateral cleft lip, the simultaneous correction of the unilateral left lip and
nasal deformity became accepted practice. Furthermore, there was no evidence that early
manipulation of the alar cartilage impairs the
development of the nasal tip.
LESSONS

FROM

BILATERAL CLEFT STIGMATA

Every child with a repaired bilateral cleft lip

has a characteristic appearance whose origins
are both intrinsic to the malformation and iatrogenic. The philtrum is bowed, wide, undimpled, overly long, often asymmetric, and
lacking a white ridge. If the prolabial vermilion-mucosa is preserved, the free margins of
the lateral labial elements hang like swags,
flanking a thin median tubercle that is covered
by insufficient vermilion and chapped mucosa
(whistling lip deformity).19 In profile, the upper lip is flat or convex, whereas the lower lip
everts (cleft lip lower lip deformity).20 The
child struggles to obtain bilabial closure over a
protrusive, retroclined, and vertically elongated premaxilla. The accompanying nasal deformities are primarily deformational but are
also postsurgical. The tip is broad, the medial
crura are pulled inferior-posteriorly, the nostrils are slumped, the alar domes are buckled
and splayed, and the alae nasi are flared, sometimes likened to cats knees.19 Often the alar
lobules are hypoplastic (a primary deformity).

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CLEFT LIP AND NASAL DEFORMITY

The caudal margin of the alar cartilage protrudes into the lateral vestibule, producing an
oblique ridge or web.21 Without amends for the
vertically long lateral labial elements and normal muscular attachment of the alar bases, an
unnatural elevation of the alae nasi will occur
and become more pronounced whenever the
child smiles.22 But of all the nasolabial distortions, the short columella is most obvious.
Principles that guide the surgical repair of
bilateral cleft lip and nasal deformity have
been induced from the stigmata of conventional techniques and by study of the literature19: (1) Symmetry. This is foremost. Staged
repair portends asymmetry. Even the smallest
nasolabial difference on the two sides will magnify with growth. (2) Primary muscular continuity. Orbicularis oris muscular bundles must be
completely mobilized from the lateral labial
elements and apposed throughout the vertical
extent of the upper lip. (3) Proper philtral size
and shape. The constructed philtrum widens
remarkably (in the upper portion more so than
inferiorly) and displays considerable vertical
growth. (4) Formation of median tubercle from lateral labial elements. There is no prolabial white
roll in the complete deformity, and both central vermilion and mucosa are deficient. (5)
Primary positioning of alar cartilages to construct the
nasal tip and columella. Techniques based on
this principle have dramatically changed the
faces of children born with bilateral cleft lip.
THE KEY: PRIMARY REPAIR
DEFORMITY

OF THE

NASAL

Nasal dissection of stillborn infants with bilateral labial clefting reveals that the alar
domes and middle crura are splayed, caudally
rotated like a bucket handle,23 and subluxed
from their normal anatomic position overlying
the upper lateral cartilages.19,23 Broadbent and
Woolf24 described a case of primary medial
advancement of the alar domes combined with
excision of skin from the broad tip. However, it
was McComb who led the vanguard for primary
columellar elongation. He initially tried primary elevation of a forked flap21 and published
his follow-up analysis 10 years later, including
measurements of columellar growth.23 By then
he had become disenchanted with this strategy
and presented a revised technique for primary
nasal repair in 1990 without a forked flap.19
In the first stage, McComb used an external
incision (flying bird) to open the nasal tip
and allow the apposition and suspension of the

splayed alar cartilages. The nasal tip was narrowed by V-Y plasty. Bilateral adhesions were
done, followed by definitive labial repair at a
second stage. The columellae looked quite
normal in McCombs assessment at 4 years.25
Mulliken also focused on early positioning of
the alar cartilages. This was initially done at a
second stage in conjunction with intranasal
transposition of the banked tines of a forked
flap.19 By l987, banking had been abandoned,
and primary columellar lengthening and nasaltip projection were achieved solely by apposition and elevation of the alar domes and by
sculpting the nasal soft tissues.22 Other surgeons were on the same track to primary nasal
correction. In 1991, Trott and Mohan were
working in Malaysia, where socioeconomic factors made multistaged repairs impractical.
They devised a single-stage nasolabial repair,
based on open rhinoplastic exposure of the
dislocated alars.26 Cutting and associates described another variation on the open-tip approach and added presurgical stretching or
elongation of the columella.27
The columella is in the nose became the
shibboleth of surgeons who advocated primary
nasal repair.16,25 The old, non-anatomic techniques that involved secondary recruitment of
prolabial skin into the columella were wrong.
Instead, the alar cartilages should be placed in
the proper position at the time of labial repair,
followed by trimming and redraping the soft
tissues of the nasal tip. No longer would the
columella include labial (often hair-bearing)
skin, the nasolabial junction be transgressed by
scars, or the philtrum be encircled by scar
tissue.
LESSONS

FROM THE

FOURTH DIMENSION

Unlike the sculptor who works in stone, the

surgeon must work with a patient who grows
and whose nasolabial proportions change.
Thus, the surgeon must conceptualize the
childs appearance as a young adult. To do so,
the surgeon must have a thorough understanding of three-dimensional form and fourthdimensional alterations that occur with normal
growth. Thankfully, Farkas and colleagues documented the changes in the important nasolabial features, between 1 and 18 years, in 1593
North American Caucasians.28 Nasal height (nsn) and width (al-al) develop early, reaching a
mean of 76.9 and 87 percent of adult size,
respectively, by age 5 years. In contrast, nasaltip protrusion (sn-prn) and columellar length

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PLASTIC AND RECONSTRUCTIVE SURGERY,

(sn-c') develop slowly, attaining a mean of only

two-thirds of adult size by 5 years of age. All the
labial landmarks grow rapidly, reaching approximately 90 percent of adult proportions by
age 5 years. The cutaneous upper lip attains
adult height by 3 years in girls and by 6 years in
boys.30
A working fourth-dimensional hypothesis for
the repair of bilateral cleft lip is to craft on a
small scale those features that are programmed
for rapid growth, compared with normal, agematched infants. The corollary premise is that
slow-growing features can be constructed of
normal size or slightly larger than normal
size.16 This accounting for temporal changes
must also include the nasolabial distortions
that are particular to children with repaired
bilateral cleft lip. Such knowledge can be
gained only by observation of older patients,
ones own and those of predecessors and colleagues. The fast-growing features grow even
faster in the child with bilateral cleft lip, with
the exception of the median tubercle. Conversely, the slow-growing features, specifically
nasal-tip projection and columella, seem to
grow more slowly in the child with bilateral
cleft lip.29
PRESURGICAL PREMAXILLARYMAXILLARY
MANIPULATION

Synchronous nasolabial repair can be accomplished only after proper alignment of the
three maxillary segments. Most bilateral cleft
lips are complete, but sometimes there is a tiny
band on one side that causes rotation of the
premaxilla. The protrusive premaxilla must be
retracted and centralized, whereas the lateral
maxillary segments usually require expansion.
There are two basic strategies for presurgical
premaxillary orthopedic manipulation, active
and passive. The latter method is favored by
those who are concerned about the potential
deleterious effects of forcing the segments into
position. The passive molding plate is retained
by undercuts; this maintains the transverse
width of the maxillary segments. Because there
is no expansion, space is often inadequate for
the premaxilla. External force is needed to
retract the premaxilla, using either adhesive
tape, an elastic band attached to a headcap, or
bilateral labial adhesion. Because there is no
muscle in the prolabial element of a bilateral
complete cleft lip, preliminary labial adhesions
are prone to dehisce. Furthermore, external
traction techniques tend to focus pressure in-

July 2001

ferior to the basilar premaxilla, causing it to

lingually incline and causing the vomer to bow.
The most commonly used active presurgical
device is based on the prototypic design of
Georgiade and associates,30,31 refined and popularized by Latham while working in collaboration with Millard (Fig. 1).32,33 The acrylic
plates of the custom-made appliance are
pinned to the maxillary shelves. A looped wire
is passed transversely through the neck of the
premaxilla, just behind the premaxillary alveolus and well anterior to the premaxillaryvomerine suture. The maxillary segments are
expanded by a ratcheted screw in the midplane
of the device, which is turned daily by the
childs parents. Elastic chains on each side are
connected to the trans-vomerine wire, looped
around a pulley in the posterior section of the
appliance, and attached to a cleat on the most
anterior point of the maxillary acrylic plates.
Tension on the elastic chain retracts the premaxilla; tension may be periodically adjusted.
Typically it takes about 6 weeks to align the
premaxilla with the expanded palatal segments
to effect closure of the alveolar clefts (gingivoperiosteoplasty). Lathams device is most successful in correcting the premaxillary anteroposterior position; however, the movement is
more retroclination than retroposition. The
device is somewhat less successful in amending
rotation and is least successful in preventing
vertical elongation.

FIG. 1. The Latham pin-retained presurgical orthopedic

appliance. Tightening the elastic chain retrudes the premaxilla; turning the screw expands the palatal shelves.

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185

CLEFT LIP AND NASAL DEFORMITY

Some orthodontists think there are no advantages to premaxillary orthopedics in the management of bilateral cleft lip.34 However, proponents
of active versus passive techniques constitute the
two major sides of the ongoing debate. Early
longitudinal studies of children managed with a
Latham device show no serious deleterious effects on occlusion and growth.33,35 However, critics of active premaxillary orthopedics document
long-term evidence for minor midfacial retrusion.36,37 Whatever the outcome in terms of midfacial position, three advantages of presurgical
alignment of the maxillary segments must be
underscored: (1) it permits philtral design of
proper proportions, (2) it facilitates primary nasal correction, and (3) it allows closure of the
alveolar gaps, thus preventing fistulas and (possibly) permitting bony ingrowth and stabilization
of the arch. Furthermore, if there is a nearnormal maxillary foundation, there could be less
postoperative prolabial distortion and interalar
widening. If the child exhibits midfacial retrusion, maxillary advancement is a predictably successful procedure when done after completion of
growth. In the near future, it is likely that maxillary distraction with an entirely internal device
will be available for patients who might benefit in
childhood.
PRIMARY REPAIR

OF THE

PRIMARY PALATE

Markings

The infant is typically 4 to 5 months old at

the time of synchronous repair. The philtral
flap is designed with slightly biconcave sides
and a dart-shaped tip. The size of the flap
depends on the race and age of the infant and
on the appearance of the parents. For a Caucasian infant 4 to 6 months of age, suggested
dimensions are 6 to 8 mm for philtral flap
length: 3 to 4 mm wide between the peaks of
Cupids bow and 2 mm wide at the columellarlabial junction. A strip of skin is drawn on each
side of the philtral flap; these will be deepithelialized to simulate the philtral ridges. The proposed Cupids bow peak-points are sited on the
lateral labial elements so there will be sufficient
central white roll for the handle of the Cupids
bow and enough vermilion to construct the
median tubercle. The alar base flaps are drawn
at their junction with the lateral labial elements. The vermilion-mucosal line is tattooed,
as are the other important anatomic points to
be preserved during repair (Fig. 2, above, left).

Dissection

The philtral flap is incised, the flanking tabs

are deepithelialized, and the remaining prolabial skin is discarded. The lateral white linevermilion-mucosal flaps are incised, and the
alar base flaps are elevated. Orbicular muscular
bundles are dissected from the lateral labial
elements (Fig. 2, above, center). The splayed alar
cartilages are exposed through the rim incisions; it is helpful to support the cartilages with
a cotton-tipped swab (Fig. 2, above, right).
Labial Closure

Mucosal flaps are elevated from the lateral

and medial sides of the cleft defects to construct the nasal floors. Gingivomucoperiosteal
flaps are apposed, closing the alveolar clefts.
The premaxillary vermilion-mucosa is trimmed
to shorten the anterior wall of the gingivolabial
sulcus (Fig. 2, second row, left). The remaining
premaxillary mucosa is sutured to the periosteum to form the posterior wall of the anterior
gingivolabial sulcus. The lateral labial elements
are advanced medially as the buccal sulci are
closed. The lateral mucosal flaps form the anterior wall of the central sulcus. The orbicular
muscles are apposed, inferiorly-to-superiorly,
throughout the vertical height of the lip. The
uppermost suture suspends pars peripheralis
to the periosteum of the anterior nasal spine
(Fig. 2, second row, right, left panel).
The redundant tips of the lateral labial flaps
are trimmed to form the median tubercle (Fig.
2, second row, right, right panel). The distal end
of the philtral flap is inset. The philtral flap is
secured to the muscular layer. This helps to
depress the philtral flap and to raise the lateral
labial flaps in an effort to simulate philtral
ridges. Yet, a realistic philtral dimple and flanking columns seem just beyond the surgeons
craft. The cephalic margin of the cutaneous
flaps must be trimmed to correct for lateral
labial height; rarely is adjustment necessary at
the medial edges. These final steps in cutaneous closure should be done after nasal
correction.
Nasal Repair

A midline nasal-tip incision is not necessary,

for with experience, it is possible to fully visualize the dislocated alar cartilages through bilateral rim incisions.29 An interdomal mattress
suture is placed to appose the middle crura
and genua. One or two mattress sutures sus-

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PLASTIC AND RECONSTRUCTIVE SURGERY,

July 2001

FIG. 2. The Mulliken method of single-stage nasolabial repair: the semi-open approach to nasal-tip cartilages
through bilateral rim incisions.

pend each lateral genu (and lateral crus) to

the ipsilateral upper lateral cartilage (Fig. 2,
third row, left). A cinch suture is placed through
each alar base and is tightened until the interalar distance is less than 25 mm (Fig. 2, third
row, right). The tips of the alar base flaps are
trimmed to form the nasal sills. A suture placed
through the dermis of each alar base to the
underlying muscle serves to (1) prevent alar
elevation with smiling and normal action of the
depressor alae nasi muscles, and (2) form the

normal cymal shape of the lateral sill (Fig. 2,

third row, right panel).
Once the alar cartilages are in proper position, extra skin in the soft triangles becomes
obvious and should be excised, including the
skin of the lateral columella (Fig. 2, below, left,
left panel). This resection narrows the tip, defines the columellar-lobular junction, elongates the nostrils, and narrows the columellar
waist. There is also redundancy in the vestibular lining that becomes apparent after position-

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187

CLEFT LIP AND NASAL DEFORMITY

FIG. 3. (Above, left) Newborn with bilateral complete cleft lip/palate. The cutaneous band on the left is incised to allow
placement of the transverse pin of the Latham device. (Above, center) Intraoperative view of patient at age 4.5 months, after
maxillary orthopedics and premaxillary centralization. (Above, right) Intraoperative view after single-stage nasolabioalveolar
repair. (Below, left, center, right) Appearance of patient at age 1 year (6 months postoperatively).

ing the alar cartilages. Lenticular excision of

this extra mucosa helps to obliterate the lateral
vestibular web and supports the lateral crura
(Fig. 2, below, left, inset). The completed repair
is illustrated in Figure 2, below, right. Clinical
examples are show in Figures 3 and 4.
TECHNICAL MODIFICATIONS
ANATOMIC VARIANTS

FOR

Although most bilateral cleft lips are complete, there are various incomplete forms, symmetric and asymmetric, with a spectrum of
clefting of the alveolar ridges and secondary
palate.38

Symmetric Bilateral Incomplete

Certainly, the symmetric bilateral incomplete variant is the easiest to repair. Usually,
the alveolar ridge is intact or there is minor
notching. The steps are the same as described
for the bilateral complete deformity, with some
technical considerations. The most important
decision is whether or not to build the median
tubercle from the lateral labial elements
(aforesaid principle 4). If there is sufficient
prolabial muscle, white roll, and vermilion,
these can be preserved and apposed to the
corresponding lateral elements in a bilateral
butt joint. However, more often than not, the
central lip should be constructed as in the

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PLASTIC AND RECONSTRUCTIVE SURGERY,

July 2001

FIG. 4. (Above, left) Newborn with bilateral cleft lip, right cutaneous band, and intact right
alveolus and secondary palate. (Above, right; below) Appearance of the patient at age 5.5 years.

complete deformity. Careful attention should

be given to the width of the philtral flap because it has the same tendency to overgrow
transversely, as in the complete deformity.
Positioning the alar cartilages may not be
necessary unless the columella measures short
and the genua are slumped. Another caveat is
to sufficiently narrow the interalar dimension,
for it will widen with time.
Asymmetric (Complete/Incomplete)

There is a range of severity in the asymmetric

complete/incomplete bilateral cleft lip, de-

pending on the extent of soft-tissue bridging

and underlying alveolopalatal disjunction. If
a tiny cutaneomucosal band on the incomplete side pulls over the premaxilla, it is usually best to divide it to allow presurgical centralization of the premaxilla and symmetric
repair.
If one side is only partially cleft, the complete side should be addressed first. Unilateral
dentofacial orthopedics, followed by a lipnasal adhesion on the complete side, levels
the surgical field before simultaneous bilateral nasolabial (and unilateral alveolar) clo-

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CLEFT LIP AND NASAL DEFORMITY

sure. During the second stage, the surgeon

should emphasize (overcorrect) repair on
the more severely involved side. Furthermore, it is easier to match the good side to
the bad side than vice versa.
Complete Bilateral with Intact Secondary Palate

The rare complete bilateral cleft lip with an

intact secondary palate is a particular challenge. Neither external elastic traction nor
dentofacial orthopedics is possible because the
premaxilla is rigidly procumbent. There are
two alternatives: (1) try to accomplish bilateral
nasolabial repair over the protruding premaxilla; or (2) perform a premaxillary-vomerine
ostectomy and positioning and bilateral gingivoperiosteoplasty, in conjunction with nasolabial repair. A word of caution: the risk of premaxillary necrosis is real. The incisions for
premaxillary ostectomy/positioning and alveolar closure impair the premaxillary blood supply and limit venous drainage to the septal
mucosa and preserved vomerine mucosa. Primary premaxillary positioning in an infant with
bilateral complete cleft lip can cause midfacial
retrusion.39,40 However, this is unlikely in a
child with an intact secondary palate.
Other Technical Variations on the Theme of SingleStage Repair

Open rhinoplasty is another way to access

and primarily position the subluxed alar cartilages, as first described by Trott and Mohan in
1993.26 Their dissection plane is anterior to the
medial crura. The prolabial-columellar unit is
pedicled on the dorsal nasal skin and based on
the paired columellar arteries.41 43 Their philtral flap is designed to correspond to the width
of the columellar base. Because the lateral philtral incisions extend across the columellar
base, this tissue cannot be used to construct the
medial sills; the sills are formed almost entirely
from the alar flaps. Redundant skin is removed
from the lateral labial elements. The interdomal fat is elevated, the middle crura are
apposed and secured to the septum, and the
soft tissues are gathered to enhance tip projection (Fig. 5). Their postoperative photographs
show normally proportioned columellar length
and nasal-tip protrusion. The philtra appear to
be wide. Perhaps this is acceptable in Asian
children, who have a slightly broader Cupids
bow than Caucasian children.44 Distal ischemia
is a potential problem if the philtral flap were
to be designed smaller using Trotts method.

FIG. 5. The Trott method of open approach to nasal-tip

cartilages.26 The philtral-columellar flap (with rim extensions) is turned upward to expose the anterior surface of the
dislocated middle crura and genua.

Furthermore, this technique requires sutural

reconstitution of the columellar labial angle,
and this could also impede philtral circulation.
Nakajima and coworkers introduced presurgical and postsurgical molding to minimize the
bilateral cleft nasal deformity.45,46 Cutting and
associates extended this strategy to preoperative stretching of the columella. They fabricated an acrylic, double outrigger and prolabial band, attached it to a passive palatal
molding plate, and secured it to the cheeks
with tape.27,47 Their labial markings are similar
to those of Trott and Mohan.26 However, their
open-tip approach differs in that the prolabialcolumellar flap is elevated at a deeper plane
(membranous septum). This dissection is less
likely to compromise the vascularity of the distal prolabium. Their placement of interdomal
sutures is done from the underside of the alar
cartilages (Fig. 6). Cutting and associates underscore that preoperative expansion of nasal
lining is as important as columellar elongation
because it lessens tension on the interdomal
apposition and mimimizes widening of the nasal tip.48 They emphasize that the molding
prongs must push anteriorly because of the
tendency to produce a turned up nasal tip.
Neither the Trott nor the Cutting methods
include cutaneous excision of the domalcolumellar rims or resection of vestibular
lining.
REVISIONS

Symmetry is the major preoperative advantage of a bilateral cleft lip over a unilateral
cleft. In part for this reason, the number of
revisions for a double cleft lip should be less
than for a single cleft lip. The most common
secondary problem is extra mucosa (festooning) in the lateral labial elements. Often this is
associated with minor vertical deficiency of the
median tubercle. It is best to wait until after the

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PLASTIC AND RECONSTRUCTIVE SURGERY,

July 2001

FIG. 6. The Cutting method29 of open approach to nasal-tip cartilages. The philtral-medial
crural-columella complex (incised through the membranous septum with extensions into the
intercartilaginous ridges) is reflected by retrograde dissection to display the underside of the
splayed middle crura and genua.

permanent central incisors have erupted and,

if possible, after the premaxilla is in the correct
sagittal position and angulation before adjusting the free labial margin to give proper dental
show. There is a litany of procedures for the
whistling lip deformity (e.g., V-Y advancements, double/single Z-plasty, mucosal grafting). Particularly effective is the technique of
deepithelialized, medially based submucosal
flaps, tunnelled across the midline, to augment
the central red lip.49 If there is insufficient
lateral submucosa, a thick dermal graft serves
to plump the median tubercle. Any excess mucosa can be trimmed to give a normal contour
of the free margin in relation to the central
incisors.
An uncommon, annoying problem is prolapse of the posterior wall of the anterior gingivolabial sulcus. Resuspension of the sulcal
mucosa to the premaxillary periosteum is easily
accomplished.
Nasal revisions in childhood are rarely
needed. The most common is correction of an

abnormally wide nose, requiring readvancement of the alar bases. Sometimes domal divergence must be addressed. The alar cartilages
can be readjusted through rim incisions without the need for open rhinoplastic exposure.
Final nasal adjustments are done after completion of growth and maxillary advancement (if
necessary) (e.g., tip reduction, nasomaxillary
narrowing, and septal resection).
OBLIGATION

TO

PERIODIC ASSESSMENT

Not only must surgeons prefigure the rate of

growth of nasolabial features, compounded by
the distortion of the deformity, but they also
are obligated to periodically assess these
changes to learn whether or not the predictions are accurate. Photography is the minimal
documentation needed. Frontal and lateral
views (with the head in a neutral position) are
not enough; there must also be a basal view.
For the latter, Pigott recommends that the nasal dome be placed well above a line drawn
between the medial canthi.50 Lehman suggests

Vol. 108, No. 1 /

CLEFT LIP AND NASAL DEFORMITY

that McCombs rule be followed in publishing

photographs in support of a new method of
cleft lip repair, i.e., show 10 consecutive patients with follow-up of 10 or more years.51
Although admirable, this rule is unrealistic,
particularly if applied to the repair of bilateral
cleft lip. Less than 10 percent of cleft lips are
bilateral, so even a high-volume surgeon might
see only a few such new patients each year.
Thus, in an average professional career of 25 to
30 years, a surgeon will follow a relatively small
number of these children to adulthood.
What is needed is a convenient, objective,
and rapid way to evaluate nasolabial symmetry
and proportions throughout the growing years.
Although clumsy, a panel can be convened to
assess the photographs, using a rating scale.52
Other methodologies are direct anthropometry and computer-aided photogrammetry (indirect anthropometry). There is great potential for laser scanning or a similar advanced
technology to assess results.53,54 This technology will likely incorporate the soft-tissue landmarks used in medical anthropometry, so until
it becomes available, anthropometry can be
done the old-fashioned way, by handheld vernier caliper.16,55,56
Intraoperative anthropometry provides baseline values for subsequent documentation of
changes in the nasolabial dimensions. This was
done for 45 consecutive infants undergoing
single-stage repair of bilateral complete cleft
lip and nasal deformity.29 Fast-growing features, and nasal length and nasal width, were
set 88 percent and 96 percent, respectively,
shorter than those of age-matched control infants. Slow-growing features, nasal protrusion
and columellar length, were constructed
longer than normal (130 percent and 167 percent, respectively). Because all labial features
grow rapidly, these were downsized, with the
exception of central vermilion-mucosal height
that was deliberately made full.
Follow-up anthropometry required first measuring a cohort of normal children; this
showed no differences16 from the results of the
larger samples determined by Farkas and colleagues.28 Thirty-two children with repaired bilateral complete cleft lip were assessed from
age 1 to 12 years.16 Nasal-tip protrusion and
columellar height were at the mean or longer.
Interalar distance was about 2 SD above the
mean. Cutaneous upper labial height remained short in children younger than age 5
years but tended to scatter around the mean in

191

the older children. A short cutaneous upper lip

is attractive, provided there is sufficient height
of the central red lip. Although vermilionmucosal height was made full, in one-half of
the older children this dimension was just below the mean. Nevertheless, the total upper
labial height was either normal or slightly more
than normal. Admittedly, there are problems
with this study. First, the authors technique
evolved through three technical phases in the
study period, from two-stage to single-stage repair but the principles did not change. Second, the study was serial and retrospective, so
the measurements in the three phases cannot
be compared at the same point in time. Third,
only 12 of 32 children had undergone the
one-stage repair described herein, and their
mean age at evaluation was 2.5 years.
A proviso for any anthropometric study is
that normal scalar measurements do not necessarily equate with normal appearance. Nevertheless, it is the authors strong impression
that a child with measurements within 1 SD of
normal looks better than a child with abnormal
values. Furthermore, inclination of the upper
lip influences appearance; i.e., a vertical upper
lip looks longer than a protrusive lip. Thus, if
the premaxilla is lingually inclined, which
many are, this causes the upper lip to appear
longer than it may be by mensuration.
It is difficult to compare one surgical
method with another without a standard way to
assess anatomic outcome. Kohout and colleagues used photogrammetric analysis of two
surgeons results; one used the Mulliken
method (group I) and the other used the Trott
method (group II).57 The authors attempted to
distinguish results attributable to methodologic design from those produced by execution of the particular method. Nasal-tip projection was above normal with both methods but
more so in group I. Interalar dimension was
abnormally wide in both groups. Columellar
length, as a proportion of tip projection, approached normal in group I but was short in
group II. Philtral width (in proportion to nasal
width) was normal in group I but abnormally
high in group II. An overly wide Cupids bow in
group II was attributed to design.
Photogrammetry also permits the determination of nasolabial angle and nasal-tip angle,
provided the head is not rotated. Kohout and
associates found that the nasolabial angle was
obtuse in early childhood but narrowed in late
childhood and adolescence, presumably be-

192

PLASTIC AND RECONSTRUCTIVE SURGERY,

cause of an increase in upper labial and columellar obliquity. Nasal-tip angle was blunted
after both the Mulliken and Trott methods;
there were no long-term measures of possible
change in the slow-growing lobule.57
EPILOGUE

Older children with bilateral cleft lip continue to walk through the doors of cleft centers
having undergone many procedures and requiring more. They are unhappy because of
their appearance and functional problems, but
the patterns of cleft care are changing. Fewer
general plastic surgeons take on primary cleft
lip repair. Two possible reasons for this trend
are (1) established postgraduate fellowship
training in pediatric plastic surgery, and (2)
increased number of craniofacial teams and
opportunities for pediatric plastic surgeons.
Remarkably, this increased focus on care by a
few is occurring despite diminished reimbursement for cleft work.
Outcome analyses in other pediatric surgical
specialities underscore the value of subspecialization and high-volume operators. For example, there are lower inpatient costs and fewer
complications if ureteroneocystostomy is done
by fellowship-trained pediatric urologists as
compared with general urologists.58 For pediatric cardiac surgeons, there is an inverse relationship between the annual number of procedures (or a surgeons case load) and inpatient
mortality.59,60 In these examples, the determinant of outcome is indisputable; the results of
cleft treatment are more difficult to assess.
Surely every infant born with cleft lip/palate,
particularly a complete deformity, deserves the
care of an experienced team. Scandinavia has
the longest tradition of centralized cleft care,
beginning in 1933. There is only one center in
Denmark, two in Finland, two in Norway, and
six in Sweden. The six-center Euro Cleft Study
showed that standardization, centralization,
and participation of high-volume operators
were associated with good outcomes (and
fewer revisions).61 In the United Kingdom, the
threshold volume for primary cleft repair has
recently been mandated, the result of an outcome assessment of the countrys cleft centers
under the auspices of the National Health Service. The investigating committee concluded
that the number of cleft units should be reduced drastically and that children should be
sent only to teams composed of two surgeons,
each seeing 40 to 50 new patients annually.62

July 2001

Boorman goes further by raising the question

of whether the rarity of the bilateral deformity
and its attendant problems might argue for
further control of referral.62
Such sweeping regulations would be unlikely
in Americas decentralized health-care system,
where change occurs slowly and, usually, voluntarily. Parameters for cleft care have been
established by the American Cleft PalateCraniofacial Association (ACPA).63 This organization also surveyed North American teams
and determined standards-of-care delivery.64
Of 220 responding teams, the mean number of
new cleft lip cases seen per year was 17. However, 37 percent of U.S. and Canadian centers
reported performing fewer than 10 primary
labial repairs each year. These numbers would
be even lower with more than one plastic surgeon on a team. The ACPA evaluation did not
address the level of activity necessary to maintain competency or provide optimal care.
These issues are on the agenda of the Craniofacial Outcomes Registry, which is supported
by a grant from The National Institute of Dental and Craniofacial Research. This program is
being conducted with the cooperation of
ACPA and its members. Prospective data, collected, and centralized by the Craniofacial Outcomes Registry, will permit inter-team comparisons of outcome, the purpose being to
promote higher-quality care in the future.
In the meantime, the traditional referral
lines for a newborn with cleft lip/palate are
changing at the grassroots level. Rather than a
pediatrician, the prenatal ultrasonographer or
perinatologist is increasingly the first to guide
parents to a plastic surgeon. Savvy parents often seek information (albeit nonpeer-reviewed) in medical cyberspace, and parental
networks advise these Internauts where to
search. Having completed a cram course in
cleft care, parents are likely to demand to take
their infant to a cleft specialist outside their
insurance network. Countercurrent to this rising tide of parental self-referral stand the
health maintenance organizations that insist
that all children stay within the network and
accept assignment to their general plastic
surgeon.
Rather than take up arms against third-party
payers, why not join them? Consider collaborative outcome studies. One hypothesis would be
that primary repair of a cleft lip in a restrictive
insurance setting is penny-wise and poundfoolish. Perhaps if initial closure were done by

Vol. 108, No. 1 /

CLEFT LIP AND NASAL DEFORMITY

subspecialists, there would be fewer procedures and revisions, and decreased costs to all
parties. It is easy to document efficacy in terms
of speech, dentition, and facial growth. Standardized evaluation of aesthetic results should
soon be possible using advanced technology,
such as laser scanning. A childs happiness and
parental acceptance could be assessed by a
quality-of-life outcome study.65
Who should care for an infant with cleft lip?
At a societal level, this question exemplifies the
larger issue of what we want our health-care
system to beand for whom. But the same
question also could be asked by the surgeon
called to see a newborn with cleft lip. Remember the golden rule of pediatric care: Do for the
child what you would want done for your own.
John B. Mulliken, M.D.
Division of Plastic Surgery
Childrens Hospital
300 Longwood Avenue
Boston, Mass. 02115
mulliken@a1.tch.harvard.edu
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28. Farkas, L. G., Posnick, J. C., Hreczko, T. M., and Pron,
G. E. Growth patterns of the nasolabial region: A morphometric study. Cleft Palate Craniofac. J. 29: 318, 1992.
29. Mulliken, J. B., Burvin, R., and Farkas, L. G. Repair of
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anthropometry. Plast. Reconstr. Surg. 107: 307, 2001.
30. Georgiade, N. G., and Latham, R. A. Maxillary arch
alignment in bilateral cleft lip and palate infant, using
pinned coaxial screw appliance. Plast. Reconstr. Surg.
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31. Georgiade, N. G., Mason, R., Riefkohl, R., et al. Preoperative positioning of the protruding premaxilla in the
bilateral cleft lip patient. Plast. Reconstr. Surg. 83: 32, 1989.
32. Millard, D. R., Jr., and Latham, R. A. Improved primary
surgical and dental treatment of clefts. Plast. Reconstr.
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33. Millard, D. R., Latham, R., Huifen, X., et al. Cleft lip
and palate treated by presurgical orthopedics, gingivoperiosteoplasty, and lip adhesions (POPLA) compared with previous lip adhesion method: A preliminary study of serial dental casts. Plast. Reconstr. Surg.
103: 1630, l999.
34. Ross, R. B., and MacNamera, M. C. Effect of presurgical
infant orthopedics on facial esthetics in complete bilateral cleft lip and palate. Cleft Palate Craniofac. J. 31:
68, 1994.
35. Bitter, K. Lathams appliance for presurgical repositioning of the protruded premaxilla in bilateral cleft
lip and palate. J. Craniomaxillofac. Surg. 20: 99, 1992.
36. Berkowitz, S. A comparison of treatment results in com-

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Henkel, K.-O., and Gundlach, K. K. H. Analysis of primary
gingivoperiosteoplasty in alveolar cleft repair. Part I: Facial growth. J. Craniomaxillofac. Surg. 25: 266, 1997.
Mulliken, J. B. Primary repair of bilateral cleft lip and
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Friede, H., and Pruzansky, S. Long-term effects of premaxillary setback on facial skeletal profile in complete
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Slaughter, W. B., Henry, J. W., and Berger, J. C.
Changes in blood vessel pattern in bilateral cleft lip.
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Fra, M. Anatomy and arteriography of cleft lips in
stillborn children. Plast. Reconstr. Surg. 42: 29, 1968.
Bennun, R. D., and Dogliotti, P. L. Anatomical basis for
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Farkas, L. G. (Ed.). Anthropometry of the Head and Face,
2nd Ed. New York: Raven Press, 1994. P. 344.
Nakajima, T., Yoshimura, Y., and Sakakibara, A. Augmentation of the nostril splint for retaining the corrected contour of the cleft lip nose. Plast. Reconstr.
Surg. 85: 182, 1990.
Nakajima, T., Yoshimura, Y., Nakanishi, Y., Kuwahara, M.,
and Oka, T. Comprehensive treatment of bilateral
cleft lip by multidisciplinary team approach. Br. J.
Plast. Surg. 44: 486, 1991.
Grayson, B. H., Santiago, P. E., Brecht, L. E., and Cutting,
C. B. Presurgical nasoalveolar molding in infants
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Cutting, C., Grayson, B., and Brecht, L. Columellar
elongation in bilateral cleft lip (Letter). Plast. Reconstr.
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Pigott, R. W. Alar leapfrog: A technique for repositioning the total alar cartilage at primary cleft lip
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Aung, S. C., Ngim, R. C. K., and Lee, S. T. Evaluation
of the laser scanner as a surface measuring tool and its
accuracy compared with direct facial anthropometric
measurements. Br. J. Plast. Surg. 48: 551, 1995.
Bush, K., and Antonyshyn, O. Three-dimensional facial
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Farkas, L. G., and Lindsay, W. K. Morphology of the
adult face following repair of bilateral cleft lip and
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Farkas, L. G., Hajnis, K., and Posnick, J. C. Anthropometric and anthroposcopic findings of the nasal and facial
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Kohout, M. P., Aljaro, L. M., Farkas, L. G., and Mulliken,
J. B. Photogrammetric comparison of two methods
for synchronous repair of bilateral cleft lip and nasal
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surgical subspecialization in pediatrics provide highquality, cost-effective care? Pediatrics 97: 14, 1996.
Jenkins, K. J., Newburger, J. W., Lock, J. E., et al. Inhospital mortality for surgical repair of congenital
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1187, 1999.

Self-Assessment Examination follows on

page 195.

Self-Assessment Examination
Primary Repair of Bilateral Cleft Lip and Nasal Deformity
by John B. Mulliken, M.D.
1. ALL THE FOLLOWING ARE COMMON STIGMATA AFTER TRADITIONAL REPAIR OF BILATERAL CLEFT
LIP, EXCEPT:
A) Slumped genu
B) Elevated alae nasi
C) Bowed philtrum
D) Proclined premaxilla
E) Thin median tubercle
2. THE FOLLOWING ARE CRITICISMS OF THE FORKED FLAP PROCEDURE FOR COLUMELLAR
ELONGATION, EXCEPT:
A) Scars at columellar-labial angle
B) Inadequate columellar length
C) Wide philtral scars
D) Abnormal columellar shape
E) Medial crura at nostril-lobular junction
3. PRINCIPLES FOR PRIMARY REPAIR OF BILATERAL COMPLETE CLEFT LIP AND NOSE INCLUDE THE
FOLLOWING, EXCEPT:
A) Maintain symmetry
B) Secure primary muscular continuity
C) Design proper prolabial size and configuration
D) Construct median tubercle from prolabial vermilion-mucosa
E) Form columella and nasal tip by positioning alar cartilages
4. ALL OF THE FOLLOWING ARE POTENTIAL ADVANTAGES OF PRESURGICAL ORTHOPEDICS, EXCEPT:
A) Permit gingivoperiosteoplasty
B) Correct protrusive basal premaxilla
C) Facilitate primary nasal correction
D) Stabilize dental arch
E) Allow proper design of philtrum
5. ALL OF THE FOLLOWING ARE FAST-GROWING NASOLABIAL FEATURES, EXCEPT:
A) Interalar width
B) Columellar length
C) Cutaneous upper lip height
D) Cupids bow width
E) Median tubercle
6. WHICH OF THE FOLLOWING NASOLABIAL FEATURES IN A CHILD WITH REPAIRED BILATERAL
COMPLETE CLEFT LIP IS LEAST LIKELY TO APPROXIMATE THE AGE-MATCHED NORMAL
MEASUREMENT?
A) Interalar width
B) Nasal protrusion
C) Columellar length
D) Width of Cupids bow
E) Total upper lip height

7. WHICH OF THESE NASOLABIAL FEATURES SHOULD BE CONSTRUCTED CONTRARY TO ITS PREDICTED

GROWTH RATE?
A) Nasal-tip protrusion
B) Columellar length
C) Interalar width
D) Cupids bow
E) Median tubercle
8. WHICH IS THE EASIEST AND MOST AVAILABLE METHODOLOGY TO ASSESS NASOLABIAL FEATURES
AFTER REPAIR OF BILATERAL CLEFT LIP?
A) Anthropometry
B) Computer-aided photogrammetry (indirect anthropometry)
C) Laser surface scanning
D) Panel evaluation
E) Digital three-dimensional photography
9. WHICH (OR WHO) IS LEAST LIKELY TO GUIDE PARENTS OF AN INFANT WITH A BILATERAL COMPLETE
CLEFT LIP TO A SUBSPECIALIST PLASTIC SURGEON?
A) American Cleft Palate-Craniofacial Association
B) Health maintenance organization
C) General plastic surgeon
D) Internet
E) Another parent

To complete the examination for CME credit, turn to page 283 for instructions and the response form.