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Functional primary closure of cleft lip

A. F. Markus, J. Delaire
Poole liospitul, Pook, Dorset; 8 rue Horuce Vernet, Nantes, France

SCM MA R Y. A technique of primary closure of the cleft lip is described. It is based on a complete understanding
of the anatomy of the entire facial musculature such that it can be restored to normality and so encourage normal
function and development.

IIVTRODUCTION

The outcome of primary surgery for cleft lip is judged


by its effects on the quality of oro-facial function and
,n :n
development. Veau (1938) observed that all normal
anatomical elements exist either side of the cleft and
that surgery should aim to return them to their
correct position. To this end, a technique of functional
closure of the lip and nose was developed (Delaire,
1975) based on extensive research of the deformity.
(Delaire, 1971, 1974; Dclairc et al., 1972; Chateau,
1977; Delaire et ul., 1977; Dclaire & Chateau, 1977;
Delaire, 1978; Delaire & Precious, 1986).
Except in exceptional circumstances, such as holop-
rosencephaly, there is no true hypoplasia in the tissues
either side of the cleft. There is, however, displace-
ment. deformation and under-development of the
muscles and skeletal tissues. As a direct consequence,
the overlying muco-cutaneous tissues are also affected I
and this is of great significance when designing the
Fig. 1 - The anterior facial muscle chains in complete unilateral
incision. In taking these factors into account and
cleft lip. (a) transverse nasalis; (b) Icvator labii supcrioris alacquc
restoring the anatomy and, therefore, function of nasi: (c) levator labii superioris: (d) orbicularis oris.
these tissues one adheres to the principle of ‘embryo-
logical surgery’ as conceived by Veau (1938) in which
the situation which should have existed at birth is cleft side and for under-development of the premaxilla
recreated. on both sides of the median inter-incisive suture
(Fig. 2).
These abnormalities influcncc the muco-cutaneous
tissues and produce well-defined characteristics which
UNII,ATERAL COMPIAETE CLEFT LIP
include the displacement of the skin of the nostril on
to the upper part of the lip, retraction of labial skin
Anatomy
and abnormalities of the tissue immediately either
The functional abnormalities arising from lack of side of the muco-cutaneous junction-the white roll
attachment of all the naso-labial muscles are a prime and mucosa.
cause of underlying skeletal malformation. Compared Due to inadequate muscular support, the skin of
with the non-cleft patient, the three groups of super- the nasal floor, the nasal sill, the vestibule and the
ficial facial muscles, (the naso-labial, bi-labial and base of the columclla drifts inferiorly and lies in the
labio-mental) are all displaced inferiorly (Fig. 1). region normally occupied by the upper part of the
The absence of correct insertion on the medial side upper lip (Fig. 3). The difference between skin of
of the cleft of both the transverse muscles of the nose nasal origin and the lip is clearly demonstrated. The
and the orbicularis of the upper lip on to the tissues skin of the nostril is finely stippled and that of the
around the anterior nasal spine and the nasal septum lip, finely striated and covered with hair. The differ-
(Markus et ul.. 1992a) and to the muscles on the ence bctwcen these two arcas of skin is further
opposite side is directly responsible for deviation of highlighted by the surface anatomy of the lip and in
the anterior border of the nasal septum to the non- practice, a line drawn at a perpendicular from the
281
282 British Journal of Oral and Maxillofacial Surgery

Fig. 4 - Skin retraction either side of a unilateral cleft lip

well-defined zones (Fig. 5). There is an external


cutaneous area: an internal area of moist mucosa and
an intermediate area of mucosa, the vermilion. The
mucosa is dry and attached to the underlying com-
pressor muscle of the lips. In clefts, this latter zone
is not prcscnt, being replaced by dry and somewhat
Fig. 2 - Skeletal and cartilagenous anomalies in unilateral cleft lip thinner mucosa without cithcr underlying glandular
and alveolus.
tissue or muscle. Veau described this area as ‘sterile’.
The white roll assumes an increasingly diminuitive
appearance. On the lateral side it dccreascs over a
length of about 2-3 mm before disappearing com-
plctcly. On the medial side the limits are even less
well-defined.
The cartilages of the nose take on a characteristic
appearance with twisting of the alar on the cleft side
(Fig. 6). The base is more inferiorly positioned and
the cartilage in general is flattened and distended.
The median cartilaginous septum is deviated to the
non-cleft side. The deformities are due to the abnor-
mal muscle attachments or lack of them, but do not
represent cartilaginous hypoplasia.

Fig. 3 - The nasal and labial areas of skin in unilateral cleft lip and
nose.

alar base on the lateral aspects to the muco-cutaneous


junction of the lip and on the medial aspect from the
base of the columella to the mucocutaneous junction,
delineates the two regions.
The skin of the lip on both sides of the cleft has a
dome-like appearance due to the lack of normal
insertion and, therefore. activity of the underlying
muscles. The skin height appears reduced, typically
considered representative of hypoplasia (Fig. 4). The
skin is not only retracted, but also slightly thicker
and following operation resumes normal dimensions
becoming distended and thinner. Fig. 5 - The muco-cutaneous areas of the lip. v: vermilion: mh:
The normal mucosa of the lip is made up of three moist mucosa: mse: excess mucosa; g: gingival mucosa.
Functional primary closure of cleft lip 283

slightly shorter than C-D, so that once muscle repair


has been carried out and the overlying skin is dis-
tended it becomes the same length. The line l--2-3 is
gently curved and not dissimilar to that advocated
by Millard. However, it does not extend more medial
than point I and so avoids scar at the base of the
columella or more medially. in the middle of the
philtrum. This relatively short incision enables clos-
ure, provided that a local subcutaneous undermining
is carried out, dissecting off the muscle and periosteal
attachments. The line 3-4 is parallel to a line drawn
from C to the median fraenum of the upper lip. It
marks the medial limit of that area of the vermilion
that needs to be resected.
On the lateral aspect, the skin of the nose which
has drifted on to the labial arca must be returned to
the correct arca. The line 5 to 6 distinguishes the two
Fig. 6 - The nasal cartilages in unilateral cleft lip (S: septal; A: areas from each other. Point 5 which is situated at
alar). the most inferior and lateral extremity of the alar
base is connected by a line perpendicular to the
mucocutaneous junction, at point 6. Point 7 is situated
The underlying maxilla is also affected by the cleft where the white roll begins to disappear and point E,
(Fig. 2). The greater segment is displaced towards the 2-3 mm beyond where the white roll has completely
non-cleft side, does not have the usual curve and disappeared such that 7-E is equal to C-D on the
maybe under-developed due to insufficient stimula- non-cleft side. Point 8 is situated at the junction of
tion in the region of the inter-incisive suture. Likewise: the vermilion and the moist mucosa below point 7
on the cleft side the lesser segment is similarly dis- and this line, 7-& is perpendicular to the white roll.
placed and deformed. To ensure normal form and continuity of the white
roll of the lip it is important to make reference marks
on either side of the cleft that will allow precise
Surgical technique
alignment of the muco-cutaneous junction at the end
The design of the incision in the skin and mucosa of the operation (Fig. 9A). This is generally easier on
must aim to ensure that the skin of the nose and the the lateral side of the cleft where the white roll
skin of the lip remain in their respective areas and gradually disappears over 2-3 mm. The point where
retain their normal dimensions, the correct height of it begins to disappear should be marked. On the
the skin on the cleft side, that the mucp-cutaneous medial side it is a little more difficult to establish this
junction and white roll have perfect continuity and point. The marking should be just medial to point 3.
that the vermilion of the cleft side is identical to that Where the skin margins arc significantly retracted
of the non-cleft side. and the white roll is not pronounced, a small triangu-
Many procedures have been described, such as the lar ITap may be used (Fig. 9B). A Vcau double needle
quadrangular flap of Le Mesurier (1955) the triangu- (Fig. 10) is used to tatoo on either side of the muco-
lar flap described by Tennison (1952), Trauner (I 950) cutaneous junction.
and Skoog (1970) and Malek (1983) the arciform Starting with the incisions on the lateral side, the
flaps of Millard (1960) and the undulating flap of excess mucosa is removed. It is possible, to use this
Pfeifer (1966) (Fig. 7). It can bc seen that one way or as a superiorly based flap where the cleft is wide and
another these techniques do not respect the origin where it might help in reconstituting the nasal Roor.
and form of the cutaneous and mucosal areas of cleft Access to the transverse muscles of the nose is
lips. Our technique (Fig. 8) taking into account the gained by dissection with scissors, starting laterally
already described characteristics of the mucosal and at the most lateral and inferior point of the alar
cutaneous tissues either side of the cleft demands a cartilage through the incision joining the points 5 and
very careful and exact reconstruction of the naso- 6 (Fig. I 1A). Through this incision it is possible to
labial muscles such that within a few weeks after identify the transverse muscle of the nose, the levator
surgery without any form of flap procedure, the labii superioris and levator labii superioris alaequae
underlying function of the muscles results in progress- nasi muscles, the upper and internal end of the
ive distention of the overlying skin. On the medial oblique head of the orbicularis muscle, the horizontal
aspect of the cleft, a line which extends B-l (which is orbicularis muscle and the vermilion to which it is
parallel to a line from A-A’ representing the supero- adherent (Fig. 11 B).
internal angles of the nostrils) is extended until it In the naso-labial region, the deep muscle layer
meets the mucocutaneous junction. This defines the comprised of transverse nasalis and myrtiformis
two cutaneous areas. Along the muco-cutaneous junc- (depressor nasi septi) (Fig. 12). It is reasonable to
tion the Cupid’s bow must be restored. Point D consider them as two heads of one muscle (Talmant,
represents the peak of the Cupid’s bow on the non- 1991; Precious, 1992). The upper part, the transverse
cleft side and point C, the base. The distance C-3 is nasalis, arises from the crest of the nasal bone and
284 British Journal of Oral and Maxillofacial Surgery

Fig. 7 - Skin incismns for unilateral cleft lip (Lc .Mcsurier, Tennison, Trauncr. Skoog. Malek, Millard: PfeiKcr)

passing inferiorly and around the lateral alar cartilage the dissection extending up to the vomer and the
continues into the nasal sill where it is joined by anterior aspect of the nasal bones.
fibres of myrtiformis. Inferiorly, myrtiformis arises This complete freeing of the muscles and their deep
from the premaxilla above the apices of the lateral insertions on to the maxilla allows medial displace-
incisor and canine teeth and occasionally the first ment and their reattachment to the inferior part of
premolar. In the deep layer, medially below the nasal the anterior border of the nasal septum, the pcrios-
sill: fibres are found which, via fascial attachments teum of the anterior nasal spine and to the muscles
between the medial crus and the columclla, depress of the opposite side.
the tip of the nose. These are fibres of the myrtiformis Commonly, some medial displaccmcnt is achieved
muscle. Both heads of the muscle play a significant by supra-periosteal dissection in the belief that sub-
part in the form of the nasal sill, with their fibres periostcal dissection may hinder maxillary develop-
terminating in stages along its length and ultimately. ment. In reality, the deep layer of the periosteum
in the septo-premaxillary ligament of Latham (1970). only elaborates bone from osteoblasts which have
The muscle, therefore, has an important role in both been generated by the superficial layer. It is this latter
the vertical and transverse dimensions of the nose. layer that is truly gcncrative and which is so depen-
The deeper parts of the orbicularis and the myrti- dent on its vascularisation derived from the muscles
formis are separated by dissection from the glandular inserted into it. Supra-periosteal undermining, there-
tissue of the adjacent mucosa. The deep attachments fore, deprives this layer of its vascularisation and is
of the levator muscles must be conserved. On the more damaging than sub-periosteal dissection. We.
medial side of the cleft the fibres of the horizontal therefore, advise undermining the periosteum over
head of the obicularis are adherent to the vermilion. the entire surface of the maxilla on the cleft side,
Its deep part is separated from the glandular tissue extending posteriorly to the tubcrosity. superiorly to
of the adjacent moist mucosa without damaging the the infra-orbital rim around the infra-orbital foramen,
median fracnum and cellular septum of the upper lip. up to the frontal process of the maxilla, the nasal
whose integrity must remain intact. The anterior bone and the pyriform aperture (Fig. 14).
nasal spine and the anterior border of the scptal It is necessary to make an incision in the buccal
cartilage are then well exposed (Fig. 13). This expo- sulcus usually as far back as the zygomatic buttress
sure is extended to the non-cleft side. ultimately with a small vertical relieving incision posteriorly.
exposing the base of the nasal septum. The perichon- Care must be taken not to damage any superficial
drium of the nasal septum is then elevated widely, teeth buds (Delairc et ul.. 1988).
Functional primary closure of cleft lip 2x5

Fig. 8 - Landmarks used in the design of the incision for primary


closure of a cleft lip.
A: supcro-internal angle of nostril on non-cleft side
A’: supcro-internal angle of nostril on cleft side
9: base of the columclla on the non-cleft side
C: base of the Cupid’s bow
D: peak of the Cupid’s bow on the non-cleft side Fig. 9 -The outline for the incision in the unilateral cleft lip. (A)
E: the extremity of the white roll on the cleft side the inctsion commonly used: (9) where the skin is considerably
I: point on line extended from B such that B-I is parallel to A-A’ retracted.
and A-B = A’- I
2: point on line extended from R-l to mucocutaneous junction
3: point on the muco-cutaneous junction at a distance from C
slightly less than the distance D-C
4: point at the junction of vermilion and moist mucosa at a
distance from the median fraenum equal to C-3
5: point at the.junction of alar cartilage and lip
6: point on the muco-cutaneous junction. the line 5-6 being
perpendicular to the junction
7: point where the white roll begins to disappear
8: point at the junction between moist mucosa and vermilion at a
perpendicular to point 7 and this junction.

To obtain the best functional and aesthetic result,


ensuring patency of the nasal airway, Berkely (1959)
Skoog (1970) and Millard (I 97 1) proposed techniques
of primary rhinoplasty. We achieve these by careful
repositioning of the alar cartilage by dissection with
scissors. from its attachments to the overlying skin
right up to the alar dome and a complete undermining
of the perichondrium of the nasal septum on the side
of the cleft, from the inferior border up to the nasal
bones. It is then necessary to dissect from the base
of the columella, between the crura so that the medial
crura can be freed from the cartilage of the opposite
side, right up to the dome. A band of tissue remains
Fig. 10- The Veau Sccdlc.
between the two arcas that have been undermined at
the base of the columella and this must be divided.
As with wide sub-pcriosteal undermining this very Functional closure can now be started, commenc-
wide dissection, described by Vcau (1938). does not ing with closure of the nasal floor from behind
adversely affect secondary development of the carti- forwards with the exception of the most anterior part
lage. It should now be possible to completely rc- which is closed when the underlying muscles have
position all the nasal cartilages on the cleft side and been sutured. The pcriosteum, transverse nasalis and
these will ultimately bc maintained in the correct myrtiformis muscles arc now identified and sutured
position, not only by restoration of normal muscle to the mid-line. The deep descending muscle layer is
anatomy but also by transfixation sutures. These crossed by the ascending tibrcs of the oblique head
latter sutures, which arc usually resorbable. also of the orbicularis oris. These will have been freed
prevent haematoma formation. from the overlying skin, but must remain adherent
286 British Journal of Oral and Maxillofacial Surgery

Fig. I IA & B - Access to the lateral cartilages and muscles.

Fig. 14 - Sub-periosteal undermining.

stages. The first is to restore the deep oblique part


Fig. 12 - Transverse muscles of the nose. (T) transverse nasalis: which, because it has not been dissected from its
(M) myrtifonnis. attached mucosa, takes that mucosa with it and
ensures satisfactory depth of the sulcus. It is sutured
just above and behind the labial fraenum. The incision
in the oral mucosa is sutured along its entire length
in the buccal sulcus and on the deep surface of the
lip. The second stage is reconstruction of the hori-
zontal head of the orbicularis muscle. firstly on its
deep surface, under the vermilion and then on its
more superficial surface. This suturing must be done
especially carefully to ensure that there is no second-
ary contraction due to scar formation. It gives form
to the lip and in particular, to the philtrum and naso-
labial angle. It is advisable not to knot any of these
Fig. 13 - Exposure of the nasal septum and anterior nasal spine.
sutures until the accuracy of muscle repositioning has
been assessed. The sutures should be held by artery
to the oral mucosa. In the immediate sub-nasal forceps and tied in the same sequence as they were
region, the superficial levator muscles are individual- inserted, in other words, starting with the deeper
ised and separated from the oblique head of the layers first. When these sutures have been tied, it will
obicularis oris. A monofilament suture (Prolene 3/O) be seen that not only will the shape of the nostril be
is used to anchor these tissues to the base of the nasal satisfactory, but the nasal sill will have been restored
septum, behind the anterior nasal spine. Following and the nasal and labial areas of skin will be in their
this, the orbicularis muscle is reconstructed in two correct areas. Finally, when muscle suturing has been
Functional primary closure of cleft lip 287

completed skin suturing is carried out with Prolene results, essentially the same techniques as have been
or Nylon. described for the repair of complete clefts must be
At the end of this procedure, which will have been used. It is necessary to render the more extensive of
performed at the age of 6 months, the lip on the these clefts, complete (Fig. 15). Like complete clefts,
operated side may appear to be shorter than on the primary closure will be performed at the age of 6
non-cleft side and the nostril will appear to be higher. months, or slightly later for the smaller clefts.
Before the operation the muscles and the skin on the
cleft side were retracted. Within 8-l 0 days, this lack
of symmetry will reduce under the cffcct of normal BILATERAL CO.MPLETE CLEFT LIP
muscle function. After 6-12 months complete sym-
metry will develop. Anatomy
The skin and mucosa overlying the lateral segments
have a similar appearance to the lateral aspect of
UNILATERAL INCOMPLETE CLEFT LIP
unilateral clefts. Overlying the prcmaxilla there is, of
course, no muscle. The skin of the prolabium is
Anatomy
retracted and raised by the underlying cellular tissue.
Incomplete clefts of the lip vary greatly from a small The columella is also retracted and partially incorpor-
notch to an almost complete cleft. Similarly there is ated into the prolabium. There is no lack of soft
a great variation in the abnormalities found in the tissue in this region cxccpt in rare cases such as
skin, mucosa and underlying muscles. The choice of holoprosenccphaly. However, no true vermilion
technique must be adapted to suit each situation. exists, it being replaced by ‘excess? mucosa. Likewise,
there is no white roll. (Fig. 16).
As in unilateral clefts, lateral muscle pull distorts
Surgical technique
the alar cartilages but the abnormality is bilateral
In the smallest clefts involving the vermilion or and, therefore, symmetrical. The nasal septum and in
extending just on to the skin a simple suturing of the particular: its anterior border is not subject to abnor-
orbicularis muscle is sufficient to restore normal mal traction and is, thcrcforc, correctly aligned. The
function. It should be remembered, however, that medial crura have, on the whole, normal dimensions
even in these relatively minor clefts: there are always though their inferior extremities are lower than usual.
slight abnormalities of the muscles of the floor of the The premaxilla is very anterior with respect to the
nose and nasal sill. In the long term, this deformity rest of the maxilla (Fig. 17). This is due to lack of
will tend to be exaggerated with mid-facial develop- control by the upper lip on its anterior surface,
ment and may require correction at a later date. In abnormal pressure from behind from the lower lip,
the larger clefts: including those which extend to the excessive dcvclopmcnt at the premaxillary-vomerine
nasal sill or the immediate vicinity, the anomalies arc suture and under-development of the lateral segments
more marked and progressively more comparable of the maxilla. Additionally, transverse development
with those seen in complete clefts. To achieve the best of the premaxilla is greatly reduced, partially due to

Fig. 15 - Unilateral incomplete cleft lip: (a) muco-cutaneous characteristics; (b) the incision; (c) the incision when there is a prominent
white roll.
288 British Journal of Oral and Maxillofacial Surgery

Fig. 16- Bilateral cleft lip: muco-cutaneous characteristics. (I)


nasal skin; (2) r&action of labial skin; (3) disappearing or absent
white roll; (4) excess mucosa.

Fig. 17- The prcmaxilla

the absence of development at the median inter-


incisive suture and partly to collapse of the segments Fig. 18 - Bilateral cleft lip
due to failure of normal functional stimulus. (a) the incisions-
A: at the base of the lateral alar cartilage
B: on the muco-cutaneous junction
Surgical technique of primary closure (Fig. 18) C: where the white roll begins to disappear
D: most superior part of the lip mucosa
Two incisions are made over the lateral segment and (b) raising the flap over the premaxilla:
it is important to remember that the muco-cutaneous (c) completion of flap and exposure ofthc anterior nasal spine.
junction and the excess mucosa should be preserved (d) closure.

in their entirety. An incision is made at the junction


between the skin of nasal origin and the skin of labial to disappear (point C) extending to the most superior
origin, commencing at the most inferior and lateral part of the lip mucosa (point D).
part of the alar base (point A) and extending to the Overlying the prcmaxilla, the skin which originates
muco-cutaneous junction which it meets at point B from the columella and that which originates from
and to which it is perpendicular. A second incision is the lip is sometimes difficult to distinguish. It is:
made along the muco-cutaneous junction starting therefore, reasonable to consider that each of these
laterally at the point where the white roll just begins areas represents half the distance from the supero-
Functional primary closure of cleft lip 289

internal angle of the nostril to the muco-cutaneous age of about 4.5 months as this brings the premaxilla
junction. At a point half way along this line a further under control.
horizontal lint is drawn reprcscnting the junction
between skin originating from the columella and skin
of the lip. A second line joining points 2 and 3 is BILATERAL INCOMPLETE CIXFT LIP
convex laterally, following the muco-cutaneous junc-
tion between these two points. From point 3 on either There are significant variations in this form of cleft
side, the curve of the incision is reversed until they as there arc in their unilateral counterparts. They
both meet in the mid-point, in line with the labial may vary in size from a pit in the lip to more extensive
fracnum. clefts extending up to the nasal sills. In the more
This tracing is not dissimilar to that used by extensive ones the variation in deformity necessitates
Millard for closure of incomplete bilateral clefts of adaptation of the surgical technique. When the cleft
the lip, but does not cross the base of the columella is limited to the lower part of the lip. the median part
and allows for better secondary distention of the of the vermilion is conserved using the tcchniquc
prolabium and lengthening of the columella. Any described by Manchester (1965) (Fig. 20).
excess mucosa that lies between the muco-cutaneous Where the cleft is more extensive involving almost
junction and normal moist mucosa is excised. the entire height of the lip, it is better to USC a
The skin of the prolabium is then raised, along technique which is a slight modification of that
with roughly half the soft tissue which lies bctwcen described by Millard (1971). It differs from Millard’s
it and the periosteum of the premaxilla. The dissection in that less skin is resected and the shield shaped
is continued in this plane up to the nasal spine and incision in the mid-line is smaller (Fig. 21). Primary
the inferior border of the nasal septum. This is repair is usually performed at the age of about
essential to achieve the correct height of the columella 6 months.
and to enable re-insertion of the naso-labial muscles.
As in unilateral clefts, wide sub-periosteal undermin-
ing must be carried out over the maxilla to allow the CLEFT LIP WITH CLEFT PAI.ATE
periosteum and muscles to be moved medially without
any degree of tension. After muscle reconstruction When there is a cleft lip and palate, whether unilateral
and before final suturing of the skin, a deep suture is or bilateral, there is always an effect on the bony
inserted between the skin of the prolabium and the
underlying soft tissues to ensure the correct position
of the columella and philtrum in relation to the
anterior nasal spine.
It is usually possible with this technique to close a
bilateral cleft in one stage. In extremely wide clefts,
achieving complete closure may be dificult. It is
better to render them incomplete clefts by initially
restoring the anatomy of the nasogenal muscles and
the oblique head of orbicularis oris (Fig. 19). The
efTect of function at that level reduces the size of the
cleft and allows complete closure a few months later.
Primary closure of a complete bilateral cleft is
performed not later than 6 months and usually at the

000
&

Fig. 19 - Two stage closure for extremely


2 ;,

wide bilateral clefts


Fig. 20 - Closure
(b) closure.
b
of small incomplete bilateral cleft lip (a) incision:
290 British Journal of Oral and lMaxillofacia1 Surgery

Veau said, possible to repair a cleft lip on the basis


of cutting flaps of different sizes according to the
/ \ I
ingenuity, needs and ultimate aim of the individual
surgeon.

Acknowledgements
The Authors would like to express their thanks to Maureen Pctcrs
for preparation of the manuscript and to Mr Simon Rutherford,
Medical Photographer at Poole IIospital for the illustrations.

References
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h
Delairc, J. (1974). Considerations sur I’accroisscmcnt du
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