Pediatric Plastic and Reconstructive Surgery

provided the first description of the procedure. In his repair,
Yperman recommended a straight-line closure reinforced by
passing a long needle through each side of the lip and fixing the
needle shaft with a figure-of-eight suture. This was refined by
French surgeon Ambroise Pare, who, in 1575, provided the first
illustration of the lip correction with a similar technique. Early
techniques in cleft lip repair mostly involved a straight-line clo-
sure. Later, these evolved to include other methods using geo-
metric advancement flaps and Z-plasties. By the mid-20th
century, the two most widely used repairs were those of LeMe-
surier and Tennison.
While stationed as a Navy surgeon during the Korean War,
Dr. Ralph Millard grew dissatisfied with the results he obtained
from the LeMesurier technique. He noted that although most of
the Cupid’s bow, one philtral column, and the philtral dimple
were all intact on the medial side of the cleft, they were all
rotated upward. Rather than disturbing these structures as in
previous repairs, he believed that these only need to be released
from their attachments and rotated downward to achieve nor-
mal position. These observations led Millard to develop the
rotation–advancement technique, which to this day remains
the basic foundation for unilateral cleft lip repair in most cleft
centers throughout the world.
13.3 Normal and Cleft Lip
Anatomy
Successful correction of a cleft lip is directly related to the abil-
ity to restore the appearance and functionality of the upper lip,
Fig. 13.2 Case example: Note the presence of a
“Simonart’s band.” In such cases, the alveolus is
often well aligned and so is the alar base.
Therefore, active dentofacial orthopedics and lip
adhesion were avoided, and she underwent a
one-stage lip and nasal correction. At 3 years of
age, the girl’s nasal correction is held and will not
require any further revisions.
Cleft Lip and Palate
134

through proper mobilization and accurate repair of the orbicu-
laris oris muscle. The orbicularis oris is a fanlike structure with
a superficial component and a deep component. The former, in
association with extrinsic facial muscles, serves as a retractor of
the upper lip. The deep component of the orbicularis oris, sub-
divided into pars marginalis and pars peripheralis, is responsi-
ble for the sphincteric action of the mouth. The pars marginalis
forms the innermost layer of the oral fissure and is surrounded
by the pars peripheralis in a concentric manner.
Abnormalities in muscular anatomy vary, depending on the
severity of the cleft lip. The deficiency of the orbicularis is more
marked on the medial side than on the lateral side. In incom-
plete clefts, even though some muscular fibers remain in the
upper portion of the lip, these seem to be attenuated and lack-
ing sphincteric action. In the complete cleft lip, the general
direction of muscle fibers parallels the cleft margins. The pars
marginalis is interrupted on either side of the cleft at the point
where the vermilion–cutaneous junction becomes thin. The
peripheralis on the lateral side has abnorm al insertions to the
alar base and the periosteum of the piriform aperture. Similarly,
on the medial side, it has insertions to the columella, nasal sep-
tum, and anterior nasal spine (Fig. 13.3).
Mulliken noted that in the normal Cupid’s bow, the anterior
projection of the pars marginalis gives rise to the white roll or
vermilion–cutaneous junction. However, in the a ected lip, the
pars marginalis was found to be both hypoplastic and disori-
ented adjacent to the cleft. Furthermore, the vermilion width,
though normal on the lateral side, was significantly deficient on
the medial side of the cleft lip.
Emphasizing on the importance of the natural-appearing
philtral column during cleft lip correction, Mohler (1987) cre-
ated a classification of philtral configuration. Type 1 is most
common, with some degree of divergence of the philtral col-
umn at the base of the columella. Type 2 has a degree of conver-
gence at the base of the columella. Lastly, type 3 has a
convergence below the base of the columella.
13.4 Anthropometry of the Lip
Before any attempt to correct a deformity, the norm has to be
correctly understood. Farkas has documented the normal
anthropometry of the face in detail. Such measurements o er
valuable insight into the structural grow th of the nasolabial
complex. By using this guide, a lip may be constructed to
achieve optimal results. It is crucial to maintain photographic
and numeric records, so that an accurate assessment can be
done on the results of a surgeon’s protocol. Newer three-
dimensional anthropometry systems are particularly conven-
ient, as they can record accurate measurements in a split
second.
Farkas et al (1992) found that cutaneous upper lip height
reached approximately 80% of its grow th potential by 1 year of
age and 94% by 5 years of age. Likewise, upper lip height and
nasal and prolabial width show rapid growth early in life. This
is in contrast to the columellar length and nasal tip projection,
which show slow growth. Measurements should be made to
intentionally overcorrect the slow-growing structures, as
opposed to the fast-growing structures. Such distinction
between fast- and slow-growing nasolabial elements is the key
to planning a correction in the fourth dimension. Using anthro-
pometric measurements, Thomas et al (2013) recently reported
on normal nasolabial anatomy in infants younger than 1 year
(Fig. 13.4).
13.5 Anatomy of the Cleft Nasal
Deformity
Pathogenesis of the cleft nasal deformity can be attributed to
three major factors: (1) muscle imbalance, (2) septal deformity,
and (3) asymmetry of the skeletal base. The extrinsic muscles of
facial expression, attached to the orbicularis oris muscle on the
cleft side, are responsible for the lateral pull of the alar base.
Contraction of the unopposed orbicularis muscle insertion on
the medial side results in pulling of both the septum and the
columella to the noncleft side. Further contributing to the septal
deformity is the septospinal ligament, which is also unopposed
on the normal side, fanning from the caudal border of the sep-
tum to the anterior nasal spine. This causes the caudal septum
to be hypertrophied and dislocated o the spine, away from the
cleft.
Hypoplasia (or posterior location) of the lesser maxillary seg-
ment and an asymmetric skeletal base contribute to a general-
ized displacement of the nasal pyramid toward the cleft side
and to the resultant stretching and flattening of the dome of
the alar cartilage. The lower lateral cartilage is inferiorly dis-
tracted, and its normal overlapped relationship with the upper
lateral cartilage is distorted. As a result, a buckling deformity of
this cartilage is usually noted. The orientation of the medial
and lateral crura of the alar cartilage on the cleft side is also
a ected. The medial crus is inferiorly displaced on the cleft side,
Fig. 13.3 Muscle bundles are redirected due to the cleft lip. The medial
portion of the orbicularis oris attaches to the columella, septum, and
anterior nasal spine. The lateral orbicularis oris attaches to the alar base
and piriform rim. If not completely detached and reconstructed,
contraction of the side-to-side repaired muscle may present as bulges
on either side of the lip closure.
Unilateral Cleft Lip
135

whereas the lateral crus is usually rotated inferiorly
(Fig. 13.5). The nasal vestibular mucosa is also stretched sec-
ondary to the width of the cleft. A combination of excess
mucosa and displacement of the lateral crus of the lower lateral
cartilage can result in a vestibular web after correction of the
cleft lip.
13.6 Preoperative Management
13.6.1 Dentofacial Orthopedics
There are a variety of options for presurgical treatment of the
alveolus. They vary depending on expertise, accessibility, finan-
ces, and individual protocols. There are three basic methods of
Fig. 13.4 Representation of normal nasolabial
anthropometry in an infant.
Fig. 13.5 The tripod theory suggests that the
cleft nasal deformity is a result of skeletal
imbalance, septal dislocation, and width of the
cleft and does not self-resolve if uncorrected.
Cleft Lip and Palate
136

presurgical molding: taping, passive molding, and active ortho-
pedic devices. Taping is the simplest method, with external
force placed by gradual approximation of the cleft edges of the
lip with adhesive tape. A dental plate may be used in conjunc-
tion with taping to prevent collapse of the lesser segment.
However, accurate alignment is challenging, and dermatitis and
epidermolysis from frequent tape removal can be annoying.
Alveolar approximation is also possible by progressive molding
of a dental plate. The dental plate is held in place with an adhe-
sive and gradually shaved on the palatal surface. External force
by taping assists in moving the arches along this molded sur-
face. Since infant cartilage can be molded nonsurgically, a nasal
phalange is added when the interalveolar distance is less than
6 mm. This nasal molding bulb is progressively straightened to
achieve an excellent preoperative position of the a ected lower
lateral cartilage. Usually, this process takes 10 to 12 weeks. A
simultaneous correction of the lip, nose, and alveolus (gingivo-
periosteoplasty) is possible after nasoalveolar molding (NAM).
An active dentomaxillary or Latham appliance accomplishes
alveolar alignment much more quickly (4–6 weeks). A custom-
made prosthesis is secured to the palate with pins (under a
brief anesthetic). A screw is turned weekly to activate an arm
that moves the lesser segment anteriorly along the radius of the
arm. An auxiliary elastic chain can be engaged to assist in
decreasing the interalveolar gap. A stronger force is applied on
the maxillary segments for proper positioning. Presurgical
orthopedics e ectively narrows the cleft, facilitating primary
repair and decreasing the need for secondary repair. No long-
term e ects have been noted with use of dentofacial orthope-
dics ( Fig. 13.6). Proponents of active dentofacial orthopedics
tout the ability to move the lesser segment anteriorly in infants
with significant anteroposterior (AP) discrepancy (Fig. 13.7).
13.7 Operative Treatment
13.7.1 Lip Adhesion
A lip adhesion can be a useful adjunct in the treatment of a com-
plete cleft lip. An adhesion may be used instead of or in conjunc-
tion with dentofacial orthopedics. If access to presurgical
orthopedics is unavailable, a lip adhesion can be performed as a
staged procedure to narrow the cleft and approximate the dental
arches, allowing for staged correction of the nasal deformity
(Fig. 13.8). This may decrease the number of secondary proce-
dures necessary. However, many centers have abandoned a pre-
liminary adhesion, given that it may lead to exposure to
anesthesia complications and scarring, and may add to cost.
Markings for a lip adhesion are made beyond any landmarks
that are important for the eventual cleft lip correction. On the
medial side, a linear incision is marked 2 mm beyond the vermi-
lion–cutaneous junction that extends along the base of the
caudal septum. On the lateral side, a similar, corresponding
incision is marked in the lip (beyond the vermilion–cutaneous
junction) to the level of the piriform. A perpendicular incision is
marked from the vestibular–piriform margin along the lateral
buccal sulcus. The lip incisions are made to the level of the sub-
mucosa, until the underlying muscle is visualized. However, the
muscle is not dissected. The cheek is elevated along the supra-
periosteal plane, so it may be advanced across the cleft for a
tension-free closure. A back cut in the mucosa greatly assists in
advancing the flap along the buccal sulcus, and the posterior
mucosa of the adhesion is closed. The nasal floor is constructed
by apposing the septal mucoperichondrium and the lateral ves-
tibular mucosa. Interrupted sutures are placed in the muscular
layer, and the anterior mucosa is carefully approximated. Thin
adhesive skin-closure strips (Steri-Strips) reduce the tension
across the adhesion in the immediate postoperative period.
Typically, the final correction of the cleft lip is delayed for 2 to 3
months after the adhesion (Fig. 13.9).
13.7.2 Gingivoperiosteoplasty
E orts to close the cleft alveolus primarily (with bone grafts)
may produce detrimental e ects on maxillary growth, especially
if dissection is aggressive. Primary bone grafting has been aban-
doned at most institutions. However, an unrepaired alveolar
cleft can result in fluid and air leakage. One option to close the
cleft alveolus is a gingivoplasty or gingivoperiosteoplasty. After
approximation of the arches with dentofacial orthopedics, local
gingival tissue can be used with limited undermining to close
the alveolar cleft (Fig. 13.9d). A gingivoperiosteoplasty e ec-
tively closes the alveolar cleft and completes the dental arch to
provide a stable platform for nasal correction and tooth eruption.
More commonly, the alveolar cleft is left untreated until sec-
ondary bone grafting. Typically, secondary alveolar bone graft-
ing is performed at a stage of mixed dentition, when the canine
root has not fully formed (age 8–10 years). A bone graft is per-
formed to provide bony support for the teeth adjacent to the
cleft and a dental implant (for the missing lateral incisor) or to
allow for movement of the permanent canine into the lateral
incisor position. Other advantages of secondary bone grafting
include stabilization of the arches, provision of gingival contour,
Fig. 13.6 One example of preoperative dentofacial orthopedics is a
Latham appliance. It is a pin-retained palatal appliance, which is used to
correct the AP discrepancy with the turning of the screw; the width of
the cleft is narrowed with tightening of an auxiliary elastic chain.
Unilateral Cleft Lip
137

and support of the alar base. Secondary grafting is successful by
using cancellous bone from the iliac crest.
13.7.3 Controversy of Adjunctive Treat-
ment in Cleft Lip and Nasal Deformity
Anecdotally, the incomplete cleft is the easiest to correct. The
dental arches are well aligned, the alar base is in a good
symmetric position, and there is greater orbicularis bulk secon-
dary to tension from partial completion of the oral sphincter. In
fact, just the presence of a skin bridge across the cleft (Simo-
nart’s band) greatly minimizes the severity of the cleft. Based
on these observations, Millard introduced a protocol to convert
a complete cleft into an incomplete cleft. A dentofacial appli-
ance is first used to approximate the dental arches. Then, lip
adhesion and gingivoperiosteoplasty are performed to set up
the foundation for correction of a complete cleft. This protocol,
also popularized by Mulliken, has the advantages of arch align-
ment, preferred alar base position, and increased muscle bulk,
as well as a slight lengthening of the medial element of the lip.
This can reduce the length of the back cut necessary for the
rotational flap, so that it does not encumber the lip or philtrum
on the normal side. Staged correction of the nasal deformity
also yields better and more consistent results (Fig. 13.10 and
Fig. 13.11).
Alignment of the arches may be performed by either active or
passive dentofacial orthopedics. Controversy surrounds the use
of dentofacial appliance and the e ect of gingivoperiosteo-
plasty. Supporters refer to the advantages of not having an
alveolar fistula and obviation of a secondary bone graft, without
significant e ect on maxillary grow th. Detractors point to its
Fig. 13.7 Case example: In cases of significant AP
discrepancy in the alveolar arches, the use of
dentofacial orthopedics is invaluable. An active
appliance promotes anterior movement of the
lesser arch. After aligning the arches, a one-stage
lip and nasal correction was performed. At 3
years of age, the boy has a slight recurrence of
the vestibular web, which may be corrected
electively.
Cleft Lip and Palate
138

Fig. 13.8 Converting a complete cleft into an incomplete cleft, using dentofacial orthopedics and lip adhesion, results in less tension across the
eventual correction, increased medial lip height, a better foundation for lip and nasal correction, improved alar base symmetry, and more muscle bulk
at the site of repair. It also allows for staged correction of the nasal deformity.
Fig. 13.9 (a) Markings for a lip adhesion. (b) Medial
dissection involves raising a septal mucoperichondrial
flap, as well as separating the abnormal intercrural
attachments in the columella. A limited dissection of the
lip, only to visualize the muscle, is performed. (c) Lateral
dissection involves a buccal sulcus incision, supraperios-
teal dissection over the maxilla, as well as raising the
lateral vestibular mucosa off the piriform rim. (d) Closure
begins with a back cut in the buccal sulcus incision and
closure of the sulcus. Finger pressure, along with a medial
incision in the alveolus, can assist in closure of the
gingival gap. (e) The nasal floor is constructed by
approximating the septal mucosa to the lateral vestibular
mucosa. The buccal mucosa of the upper lip is also
approximated. (f) Finally, the orbicularis is apposed in a
side-to-side fashion with three interrupted sutures, and
the mucosa lining the cleft is closed. All key landmarks
required for the eventual lip and nasal correction are
untouched during this operation.
Fig. 13.10 Staged correction of the cleft nasal
deformity yields more consistent symmetry.
Unilateral Cleft Lip
139

e ect on restricting maxillary growth and an abnormal bite
relationship. This is most likely secondary to the closure of the
lateral incisor space and restriction of the sagittal grow th of the
maxilla. However, recent studies have documented that a cleft
of the primary palate, rather than operative strategy, is the
inciting cause of maxillary restriction. In the same vein, inher-
ent di erences in health care systems, treatment protocols, and
ethnic expectations, as well as cultural acceptance of concavity
of the facial profile, have led to inadequate elucidation of treat-
ment outcomes.
13.7.4 Surgical Repair
Markings
Important landmarks of the lip and nose should be marked,
such as the subnasale (sn), columella (c), subalar point (sbal),
and Cupid’s bow peak (cphi). On the lateral side, sbal is marked
on the a ected side (Fig. 13.12). Note that the subalar point
will be splayed laterally secondary to the under-rotated alar
base of the cleft nose ( Fig. 13.13d). In order to obtain a desir-
able result, an equal vertical length of the cutaneous upper lip
must be achieved. The most important point to mark is the
Cupid’s bow peak (cphi) on the lateral side. Landmarks unaf-
fected by the cleft are the sbal and the cphi on the una ected
side. Therefore, this length should serve as a template for the
a ected side. Calipers are useful to first determine the length of
the upper lip on the una ected side (sbal to cphi) and then to
mark the cphi on the a ected side by marking the point where
the arc of the length of the lip meets the vermilion–cutaneous
(white roll) junction (Fig. 13.14). The horizontal length
between the cphi and its ipsilateral commissure or chelion is
recorded. However, it has been shown that a short preoperative
Fig. 13.11 Case example: Example of a boy with
a left unilateral complete cleft lip and palate, who
underwent a two-stage lip and nasal correction,
followed by a palate closure. At 6 years of age, he
is starting school, with no other revisions
necessary.
Cleft Lip and Palate
140

horizontal distance lengthens after correction of the complete
oral sphincter. The distance between cphi and labiale superius
(ls) is used as a guide to mark the proposed cphi on the medial
side.
Markings can then be made for the rotation and advance-
ment flaps (Fig. 13.12 and Fig. 13.13c). The rotation flap is
marked along the medial margin of the cleft, mimicking the
una ected philtrum and curving toward the columella. A back
cut is designed at the top of the rotation flap ( Fig. 13.13d).
Based on the convergence or divergence of the opposite phil-
trum, the back cut may be trimmed for symmetry during clo-
sure. The advancement flap is marked on the lateral lip element
from the proposed cphi to the nasal sill along the cleft margin
and may be extended to the alar base, as in the case of a com-
plete cleft ( Fig. 13.15). The columellar flap (C-flap) is marked
from the superior edge of the rotation flap to the membranous
septum. This flap will be used to augment the hemicolumella
and reconstruct the nasal sill (Fig. 13.13e).
Attention then turns to the vermilion and mucosa of the lip.
The vermilion is deficient on the medial edge of the cleft; there-
fore, a triangular flap of vermilion is designed at the vermilion–
mucosal junction (“red line”). The labiobuccal mucosa is also
deficient; a triangular flap of mucosa is planned from the lateral
lip to elongate it. A gingivobuccal incision is planned from the
cleft edge to raise the cheek flap for advancement across the
cleft ( Fig. 13.13g). A third triangular flap carrying the white
roll will be designed just before closure of the philtral incision.
The late use of the triangular flap allows for correction of
submillimeter di erences that may be present after the rota-
tion–advancement repair has been completed. It also breaks up a
linear scar vertically across the lip and prevents scar contracture,
especially in the vermillion–cutaneous junction (Fig. 13.16).
Dissection
Incisions are made along the markings, and the mucosa lining
the cleft is discarded. The buccal sulcus incision is made; how-
ever, it does not extend to the level of the parotid duct. A supra-
periosteal dissection is performed on the maxilla up to the level
of the infraorbital foramen. In case of an incomplete cleft lip or
if a lip adhesion has been performed previously, extensive max-
illary dissection is not necessary. Attention is then turned to
the lip. The orbicularis is first separated from its anomalous
attachment to the alar base and then dissected away from the
skin and mucosa of the lip ( Fig. 13.13h). A mucosal back cut
in the buccal sulcus assists in advancing the lateral lip elements
across the cleft ( Fig. 13.13g). Scissors are placed between the
medial crura (under the columellar flap), and blunt dissection
is used to separate it from the opposite medial crura and dome.
A sharp back cut at the top of the rotation flap (on the colum-
ella, toward the opposite philtral column) is then created while
the a ected nostril is held at an optimal level with a retractor.
The back cut is performed until the Cupid’s bow peaks are level
(Fig. 13.13e). If not addressed during a lip adhesion, the septal
mucoperichondrium and the lateral vestibular mucosa are ele-
vated for nasal floor reconstruction.
Fig. 13.12 Markings for a cleft lip correction. Key landmarks are first noted. Based on this, a rotation flap that mirrors the opposite philtrum is marked.
It extends into the columella, where a Mohler-type back cut is marked. This allows for the hemicolumella to be retrogressed to a more anatomic
position. The incision is prevented from entering into the lip, which would be visible as well as cause an effacement in the superior aspect of the
philtrum. The advancement flap is marked from the proposed Cupid’s bow peak into the nasal floor. Although the perialar incision is unnecessary in
incomplete clefts, it is marked in case greater cutaneous length of the upper lip is necessary for symmetry. In cases of a long upper lip, a perialar
incision would obviate the need for the Cupid’s bow peak to be very lateral on the upper lip, which may create asymmetry from the commissure to the
philtrum.
Unilateral Cleft Lip
141

Closure
The nasal floor is closed by approximating the septal mucoper-
ichondrium and the lateral vestibular mucosa. While retracting
the nostril to a level symmetric to the contralateral side, the
columellar flap is retrogressed into the hemicolumella. The alar
base flap is rotated endonasally and is approximated (end to
side) to the columellar flap to form the nasal sill. The buccal sul-
cus is closed after advancing the back cut. A triangular mucosal
flap at the leading edge of this flap is interdigitated into the
labial frenum to augment the mucosa on the cleft side
(Fig. 13.10i). The buccal side of the lip mucosa is then closed.
The orbicularis oris is then apposed from an inferior to a supe-
rior accession. The most superior portion of the orbicularis is
Fig. 13.13 (a,b) A right unilateral complete cleft lip and nasal deformity after alveolar alignment with active dentofacial orthopedics. (c) Markings for a
rotation–advancement repair. (d) Medialization of the lateral element will assist in accurately siting the subalar point (sbal). (e) The mucosal lining of
the cleft is excised. The back cut above the rotation flap is restricted to the columella. (f) Note the dislocation of septum to the noncleft side of the
spine. It is relocated and secured to the cleft side of the anterior nasal spine. The septal mucoperichondrium will be used to construct the nasal floor.
(g) A mucosal back cut in the buccal sulcus assists in advancing the cheek and lip across the cleft, without any tension on the eventual closure. The
maxillary dissection is in the supraperiosteal plane. The lateral vestibular mucosa is raised off the piriform rim to construct the nasal floor. (h) The
orbicularis muscle has been separated from its cutaneous and mucosal attachments. Abnormal attachments of the muscle to alar base and columella
are separated. (i) The buccal sulcus incision is advanced medially and closed, as the leading edge is sutured to a releasing incision near the frenum of
the medial upper lip. A linear incision along the cleft margin of the alveolar gingiva is made, and the lingual and buccal portions of the alveolus are
approximated. This is possible only if the alveolar segments are adjacent to one another from presurgical orthopedics. (j) A guy suture from the
maxillary periosteum is placed into the alar base, to be tightened later. The medial crura of the lower lateral cartilages are approximated under the C-
flap. This will allow retrogression of the C-flap into the back cut in the columella. The laterally based vermilion flap is inserted into a releasing incision
along the vermilion–mucosal junction medially (which is deficient in all cases). (k) The orbicularis oris muscle is apposed from an inferior to superior
fashion, with increasing tension superiorly. The superior-most portion of the orbicularis is tacked down to the anterior nasal spine in order to recreate
the pout. The alar base is sutured to the maxillary periosteum, as well as to an appropriate level of the underlying orbicularis muscle, so as to
accurately secure the alar base in a symmetric position. (l) The skin of the upper lip is draped over the muscle closure. The rotation flap is trimmed to
obtain a symmetric philtral column. Along the inferior border of the rotation flap, a releasing incision lowers the Cupid’s bow to a symmetric position.
A small triangular flap from the advancement side, carrying the white roll, is inserted into this defect, and the rest of the philtral incision is closed. The
superior edge of the advancement flap is tucked into the nostril to recreate the morphology of a normal nasal sill.
Fig. 13.14 Sighting the proposed point for the Cupid’s bow peak.
Calipers are used to measure the length of the cutaneous upper lip on
the unaffected side (sbal–cphi), and this distance is used to mark the
proposed peak along the vermilion–cutaneous junction.
Cleft Lip and Palate
142

a xed to the anterior nasal spine in order to re-create the
appearance of a pout (Fig. 13.13k).
The alar base is secured to the underlying periosteum of the
medial maxilla deeply and to orbicularis muscle inferiorly, to a
point where the interalar distance is symmetric in all axes
(Fig. 13.13j). A 1-mm overcorrection is prescribed, as post-
operative widening is generally noted. The cutaneous closure of
the lip starts at the vermilion–cutaneous junction. A small tri-
angular flap (the white-roll flap) is inserted into a releasing
incision on the medial side to complete the white roll and dic-
tate the balance of the Cupid’s bow (Fig. 13.13I). The triangu-
lar flap of vermilion is inserted into a releasing incision along
the vermilion–mucosal line (wet–dry junction or red line) to
complete closure of the mucosa of the upper lip. The advance-
ment flap is then trimmed to a convex shape and is closed to
the rotation flap in an inferior-to-superior direction
(Fig. 13.16). The subnasal portion of the rotation flap is
trimmed according to the shape of the philtrum and closed
along the nasal sill. The distal edge of the advancement flap is
trimmed and inserted into the superior portion of the philtral
incision, without any tension.
Cleft Nasal Correction
In spite of lip correction, the nose usually demonstrates a
“recurvatum” deformity with buckling of the lower lateral carti-
lage ( Fig. 13.17a). Primary correction of the nasal deformity is
prescribed in all clefts. Internal resorbable sutures are recom-
mended. The use of percutaneous sutures yields a nonanatomic
correction, and the use of bolsters is inadequate, since they are
usually removed in a week (cartilage healing takes considerably
longer).
For adequate correction, an infracartilaginous incision is per-
formed, exposing the lower lateral cartilage (Fig. 13.17b).
Using a cotton-tipped applicator, the contralateral dome is
delivered through the marginal incision; an interdomal suture
is placed between this spot and the proposed dome of the
a ected cartilage. The lateral crus of the lower lateral cartilage
is suspended from the ipsilateral upper lateral cartilage to
reconstruct the scroll (which is usually separated due to the
stretch across a complete cleft; however, it may be intact in an
incomplete cleft and therefore unnecessary) (Fig. 13.17c). Lat-
erally, soft tissue attachments to the piriform displace the
Fig. 13.15 Case example: In cases of a long upper
lip on the unaffected side, it may be necessary to
perform a perialar incision in order to obtain a
longer cutaneous length for the affected side.
This prevents the need to mark the Cupid’s bow
peak in a more lateral position that may result in
greater asymmetry. It also allows for independent
positioning of the alar base. Satisfactory lip and
nasal correction is seen at 5 years of age. The
perialar incision has healed well.
Unilateral Cleft Lip
143

lateral crus of the lower lateral cartilage. Division of these
attachments allows the cartilage to assume a normal position.
Correction of the lower lateral cartilage back to the normal
position usually results in redundancy or “webbing” of the ves-
tibular mucosa and warrants a lenticular excision and suspen-
sion to open up the nostril. Overcorrection is recommended to
overcome the perverse tendency of the lower lateral cartilage to
relapse (Fig. 13.17d).
Owing to incisions along the columella, nasal floor, alar base,
and nostril roof, a circumferential contraction of scar can lead
to nostril stenosis. To avoid nostril stenosis, a postsurgical
silicone conformer can be used during the healing phase. How-
ever, an external splint may not be tolerated by infants and may
also cause frictional abrasions and pressure-related ulcerations.
13.8 Microform Cleft Lip
Correction
A microform cleft lip, as described by Onizuka et al, includes (1)
minimal nasal deformity, (2) philtral groove, (3) thin free mar-
gin of the lip, (4) notch at the vermilion–cutaneous junction
(white roll), and rarely (5) notch in the alveolus. It is important
to note that the philtral groove is an indication of an incomplete
oral sphincter and should be corrected during the operation.
Correction of the microform cleft lip can be done either by a
rotation–advancement repair (for severe defects) or by a “dou-
ble unilimb Z-plasty.” Triangular flaps at the vermilion–cutane-
ous junction and the vermilion–mucosal junction are used
to augment the medial element by insetting the flaps into
corresponding releasing incisions. The orbicularis is carefully
apposed, and the alar base is endonasally rotated in a Y–V
advancement. Lastly, correction of the slumped lower lateral
cartilage is performed as previously described ( Fig. 13.18).
13.9 Postoperative Care
There is no evidence to support the use of arm restraints during
the postoperative period in children undergoing cleft lip or pal-
ate surgery. Although the use of a syringe for feeding has been
proposed, in our practice, both bottle- and breastfeeding are
allowed. Postoperative taping is done for 3 to 6 weeks, and gen-
tle scar massage is recommended thereafter. Scar creams can
be prescribed, but there is no conclusive evidence for any aes-
thetic benefit.
13.10 Technical Di erences in
Unilateral Cleft Lip Correction
After its original description by Dr. Ralph Millard in 1955, the
rotation–advancement technique has remained a basic founda-
tion for unilateral cleft lip correction in cleft centers throughout
the world. Several modifications have been described based on
Fig. 13.16 Demonstration of a Tennison’s triangular flap at the Cupid’s
bow. (a) When the back cut is limited from encumbering into the lip,
there is usually a deficiency in the Cupid’s bow peak at rest. (b) The
difference is measured and an equilateral triangle is marked in the
inferior edge of the advancement flap. The rest of the flap is trimmed
(usually the least vascular portion of the flap). (c) A releasing incision at
the vermilion–cutaneous junction on the medial side lowers the
Cupid’s bow peak to a symmetric level. (d) The triangular flap (carrying
the white roll) is then inset, and the rest of the philtral incision is
closed. Note that a second triangular flap is used to augment the
vermilion deficiency that is usually present on the medial element of
the cleft lip. These triangular flaps break up a linear vertical scar and
prevent contracture, especially at the level of the vermilion–cutaneous
junction.
Fig. 13.17 Primary correction of the cleft nasal deformity. (a) A “recurvatum” deformity of the lower lateral cartilage is noted, in spite of the
correction of the alar base, septum, and medial crura (via the back cut). (b) Exposure of the upper and lower lateral cartilages via a closed approach.
The lateral soft tissue attachments of the lower lateral cartilage are released. (c) Interdomal and lateral crural suspension sutures are placed. (d) Slight
overcorrection is prescribed due to the perverse tendency for the deformity to recur.
Cleft Lip and Palate
144

the same original principle. Millard himself proposed the first
modification by adding a back cut at the end of the rotation
incision, allowing further rotation of the medial element and
less tension on the repair ( Fig. 13.19b). In 1987, Mohler fur-
ther modified this technique by reducing bowing of the incision
toward the cleft and extending it up on to the columella, result-
ing in a wider C-flap to fill the entire medial defect
(Fig. 13.19c). Other modifications have been described for
gaining length in the medial lip element, with Mulliken bowing
the incision toward the cleft in a higher curve and Stal using an
S-shaped incision ( Fig. 13.19d). Fisher (2005) avoids the back
cut altogether, relying on the releasing incision at the vermi-
lion–cutaneous junction to obtain the desired length on the
medial element ( Fig. 13.19e). Incisions are carefully planned
between the columella, philtrum, and Cupid’s bow, thereby
treating each subunit of the lip separately.
Many surgeons continue to use the back cut as originally
described by Millard. Mohler’s back cut is restricted to the col-
umella, thus preventing extension into the lip. Mulliken pro-
poses a variation of this technique, using a “releasing incision”
that is perpendicular to Millard’s back cut, preventing the rota-
tional incision from either crossing the midline or extending
into the lip (Fig. 13.19d). The C-flap can then be used for hem-
icolumellar lengthening. Furthermore, it prevents the tip of the
advancement flap from crossing and e acing the philtral col-
umn. The back cut is avoided altogether by some surgeons,
championed by Nakajima and, more recently, by Fisher, releas-
ing incisions above the philtrum and at the level of the Cupid’s
Fig. 13.18 Correction of a microform cleft lip. (a,b) AP and submental views of a microform cleft lip and nasal deformity. (c) Final closure. (d) Markings
for correction. The vertical deficiency of the Cupid’s bow peak is noted, and an equilateral triangle of those dimensions is marked above the proposed
Cupid’s bow peak. A second such triangle may be required if a vermilion deficiency is present (double unilimb Z-plasty). The intervening tissue is
marked for excision. The alar base is marked for a Y–V type endonasal advancement. (e) Dissection in the subcutaneous tissue extends up to the nasal
floor and into the submucosa of the upper lip. The intervening fibrous band in the muscle is excised, and the orbicularis is apposed. Endonasal rotation
of the alar base and correction of the lower lateral cartilage with a single interdomal suture are performed. A dermal matrix or dermal graft may be
used to augment the mucosa of the free margin of the lip as well as to “create” a philtral column. (f) Final closure.
Unilateral Cleft Lip
145

bow allows for the correction of the medial segment. Triangular
flaps are used to fill these defects from the advancement side.
Extending the incision around the alar base was initially
described in the rotation–advancement technique to allow a
more independent positioning of the alar base and increased
length of the cutaneous upper lip. Detractors of this approach
claim that an incision around the alar base could stimulate scar
tissue, consequently narrowing the nostril and creating an
unattractive horizontal scar. A small perialar–crescentic exci-
sion can provide more cutaneous length for the upper lip, with-
out having to lateralize the proposed Cupid’s bow peak. This
can be especially helpful in infants with a long upper lip or if
the lateral lip discrepancy is great ( Fig. 13.15).
Originally proposed to form the nostril sill and to augment
the columella, variations of the C-flap have also been suggested.
Mulliken elevates and includes the medial crural footplate with
the C-flap by retrogressing it up the hemicolumella. Cutting
and Stal advocate the use of a wider C-flap, which when used to
backfill the entire medial rotational defect results in a straight-
line closure along the nasal sill.
Techniques for muscle repair have significantly evolved from
Millard’s original description, where the orbicularis is repaired
end to end. Both Cutting and Stal begin by advancing the
muscle from the tip of the lateral flap into the medial muscular
defect, correcting orbicularis orientation and still allowing a
straight-line cutaneous closure. Mulliken begins the repair infe-
riorly and works superiorly, progressively tightening the clo-
sure, resulting in a natural pout of the lower portion of the lip,
with the final, superior muscular suture, including the anterior
nasal spine periosteum.
13.10.1 Anesthetic Considerations
Several animal studies have shown an association between
early exposure to anesthetic agent administration and neuro-
toxicity. Long-term deficits in learning and cognitive behavior
after early exposure have been noted across several species.
Studies on humans have been less consistent. Some studies
have suggested that there may be adverse e ects on behavior,
learning, and memory from prolonged or repeated exposures
to anesthesia and surgery when children are younger than 2 to
4 years, presumably before myelination of neural tracts. Some
reports have implied a possible association between a single
anesthetic exposure and cognitive and behavioral deficits,
whereas other reports fail to show any association or show an
association only after multiple exposures. Such inconclusive
evidence has led to the conception of two large, multicenter
prospective human studies, currently under way.
It is still unclear whether it is the duration of a single anes-
thetic exposure or multiple separate exposures that may lead to
worse outcomes. Nonetheless, there is enough evidence in cur-
rent animal and human studies to be concerned. Although
correction of the cleft lip and palatal anomalies is elective, the
staging and duration of exposure to anesthesia can be titrated.
The cleft or craniofacial surgeon needs to take this into account
and be prepared to counsel parents when discussing the opera-
tion in an infant with a cleft.
13.11 Complications
13.11.1 Secondary Deformities of the
Lip
It is rare that final surgical results do not reflect the careful
planning and correct technique used to repair a cleft. However,
it is the rule that thoughtless planning and incorrect technique
lead to secondary deformity. It is important to evaluate the
landmarks of the lip and nose and also to take account of pre-
vious procedures, to be able to correct a secondary deformity.
Common secondary deformities of the lip can be classified into
mucosal, vermilion, Cupid’s bow, orbicularis, cutaneous lip
scars, philtral, and nasal deformities.
Fig. 13.19 Technical differences in cleft lip repair. (a) Tennison–Randall repair. (b) Millard repair. (c) Mohler repair. (d) Mulliken repair (e) Fisher repair.
Cleft Lip and Palate
146

13.11.2 Mucosa
Mucosal variance (thickness or weakness of the free margin of
the upper lip) is very common, given that it is di cult to pre-
dict the fullness of the lip during the primary repair. A trans-
verse excision along the vermilion–mucosal junction can
address fullness. The scar can be hidden along the red line.
Thinness of the free margin of the upper lip can be addressed
by augmenting with a dermal fat graft, acellular dermis, or fat
injection. Dermal fat grafts are reliable and predictable and are
the preferred method; they can be performed in conjunction
with other procedures, such as alveolar bone grafts.
13.11.3 Vermilion
Minor step-o s, mismatch, and notching are also encountered.
Notching, usually secondary to scar widening, can be corrected
by a simple diamond-shaped excision. Step-o s or mismatch at
the vermilion–cutaneous junction (white roll) is typically from
incorrect alignment during primary repair. A traditional Z-
plasty or a unilimb Z-plasty can be useful to correct these
deformities. Vermilion deficiency or step-o is encountered if
there has been no e ort to equalize the width during the pri-
mary repair. A triangular flap of vermilion from the wider side
can be inset into a releasing incision at the vermilion–mucosal
junction to achieve symmetry.
13.11.4 Philtrum
Adult philtral columns narrow under the columella (6 mm) and
widen at the Cupid’s bow (10 mm). The normal length of the
philtrum is 15 to 17 mm. During primary repair (age 3–5
months), the Cupid’s bow is 6 mm wide and 6 mm long. The
philtrum and the philtral column scars usually widen after
repair. Therefore, it is critical to plan the correct shape and size
of the philtrum during primary repair. Abnormal shape and size
should be corrected to age-appropriate norms.
13.11.5 Cupid’s Bow
An upswing of the Cupid’s bow, in a corrected unilateral cleft
lip, is usually an indication of inadequate rotation of the medial
element during primary repair. If the upswing is limited to 1 to
2 mm, it may be corrected with a traditional Z-plasty or a uni-
limb Z-plasty (Tennison’s triangle). If the discordance is worse
(short lip), a takedown and a rerotation will be required.
13.11.6 Orbicularis Oris
The continuity of the oral sphincter is paramount in the func-
tional repair of the cleft lip. The orbicularis oris has two parts:
the pars peripheralis and the pars marginalis. The pars periph-
eralis is the functional oral sphincter; the pars marginalis is
present in the vermilion–cutaneous junction or Cupid’s bow.
The most superior portion of the pars peripheralis attaches to
the anterior nasal spine. The pathology of the cleft lip results in
reorientation of muscle fibers that aberrantly insert into the
alar base rather than forming a continuous sphincter. Failure to
reorient the muscle by apposing the edges across the cleft may
result in a secondary deformity, that is, the contraction of the
muscle during animation, resulting in bulges under the alar
base. Failure to tighten and anchor the superior portion of the
pars peripheralis to the anterior nasal spine results in a flat
upper lip. Inadequate muscle bulk across the cleft can become
thin, allowing the philtral scar to contract. Incorrect approxi-
mation of the pars marginalis can result in white-roll abnor-
malities and notching. If the orbicularis discontinuity is
suspected, it is necessary to reopen the previous repair and
appropriately repair the muscle.
13.11.7 Lip Scars
The appearance of the philtral scar is dependent on the tension
across the cleft repair as well as on individual response to
injury. It is important to note that philtral scars undergo some
contraction immediately after repair. Parents must be coun-
seled to perform scar massage, and, typically, the lip will be bal-
anced after 3 months. Despite best e orts, scars may become
hypertrophic, in which case, they may have to be revised. A
short lip is usually a result of inadequate rotation of the medial
elements during the initial correction. A takedown of the origi-
nal repair and a rerotation are required. A long lip was tradi-
tionally seen after a geometric (triangular or quadrangular)
repair of the cleft lip. This is not seen with the advent of the
rotation–advancement repair. However, it can be encountered
after the correction of an incomplete cleft lip. A complete revi-
sion, with suspension of the buccal sulcus and crescentic exci-
sion along the alar base, is required to correct a long lip.
13.11.8 Secondary Deformity of the
Nose
Unlike secondary lip deformities, which usually result from
inappropriate or inadequate correction, secondary nasal
deformities are the rule, despite appropriate and adequate cor-
rection. The splayed alar cartilage that accompanies a complete
cleft lip retains the memory of its fetal position and reverts (to
some extent) in spite of the correction. Furthermore, the lack of
septal retaining ligaments on the side of the cleft allows the
deformity to progress throughout childhood and adolescence.
Therefore, it is important not only to correct the nasal deform-
ity primarily but also to revise it in childhood and adolescence
to maintain the correction. Similarly, it is important to realize
that improper secondary correction can lead to tertiary prob-
lems. The dogma of waiting until adolescence to correct the
cleft nasal deformity is applicable only for osteotomies.
The tripod theory implies progressive nasal tip and septal
deviation secondary to asymmetric displacement of the alar
base. Septal deformity and abnormal tension of the cartilage
due to malalignment of muscles and the skeletal base (maxilla)
can lead to the cleft nasal deformity. It is important to remem-
ber that the lower lateral cartilage, although misplaced, is
inherently normal anatomically and histologically. The unilat-
eral cleft nasal deformity is secondary to an inferolateral dis-
placement of the medial and middle crura of the lower lateral
(alar) cartilage as well as a hemicolumellar deficiency.
McComb and Potter first recommended primary sutural cor-
rection of the lower lateral cartilage to prevent progressive
deformity. Later studies showed that primary correction of the
nasal cartilage did not a ect growth. Many adjuncts have been
Unilateral Cleft Lip
147

suggested for maintaining the nasal correction. Cartilage can be
shaped permanently if molded in the first few months of life.
Preoperative NAM has utilized this principle to obtain superior
cleft nasal correction and maintenance. Postoperative nasal
conformers can be e ective but may be cumbersome and cause
columellar excoriation. The use of an internal, resorbable splint
to maintain the sutural correction of the lower lateral cartilages
has been successful in reducing the need for revisions.
In the quest for ideal position and symmetry, primary correc-
tion followed by timely revisions can be more e ective than
delaying nasal correction until adolescence. Gilles and Kilner
stated that a “well-mended harelip would go unnoticed at a
cocktail party were it not for the nose.” When we are judging
operative correction of a secondary lip or a nasal deformity, it is
also important to determine the impact on the patient. In other
words, substantiating a qualitative improvement rather than a
quantitative improvement should be proven to the child, espe-
cially in lieu of medical, academic, social, familial, and financial
obstacles. Marsh stated that the surgeon is often more critical
of the result than the child with a cleft. Therefore, it is impor-
tant to know “when enough is enough” in the search for the
ideal.
13.12 Conclusion
In parts of Africa, children with clefts are discarded as cursed
souls. An operation to correct the appearance of the face
can change the lifeline of these children. There are so many
important functional considerations such as feeding, hearing,
speaking, and chewing that are necessary for children with cleft
lip and palate. However, appearance seems paramount, as
explained by William Mayo’s oft-quoted canticle: “It is the
divine right of man to look human.” Repairing the cleft not only
addresses the appearance and function of the lip but also heals
the psyche of the a icted. Dr. Joseph Murray, Nobel laureate,
perhaps explains it best: “I prefer to think of our work as oper-
ating on the soul.”
Suggested Readings
Mulliken JB. Double unilimb Z-plastic repair of microform cleft lip. Plast
Reconstr Surg. 2005; 116(6):1623–1632
Mulliken JB, Pensler JM, Kozakewich HP. The anatomy of Cupid’s bow in nor-
mal and cleft lip. Plast Reconstr Surg. 1993; 92(3):395–403, discussion 404
Mohler LR. Unilateral cleft lip repair. Plast Reconstr Surg. 1987; 80(4):511–
517
Nakajima T, Tamada I, Miyamoto J, Nagasao T, Hikosaka M. Straight line repair
of unilateral cleft lip: new operative method based on 25 years experience. J
Plast Reconstr Aesthet Surg. 2008; 61(8):870–878
Thomas MD, Pfa MJ, Tonn J, Steinbacher DM. Normal nasolabial anatomy
in infants younger than 1 year of age. Plast Reconstr Surg. 2013; 131
(4):574e–581e
McComb H. Primary correction of unilateral cleft lip nasal deformity: a 10-
year review. Plast Reconstr Surg. 1985; 75(6):791–799
Noordho MS, Chen PK. Unilateral cheiloplasty. In: Mathes SJ, ed. Plastic Sur-
gery. 2nd ed. Philadelphia, PA: Elsevier; 2006:165–215
Grayson BH, Santiago PE, Brecht LE, Cutting CB. Presurgical nasoalveolar
molding in infants with cleft lip and palate. Cleft Palate Craniofac J. 1999; 36
(6):486–498
Mulliken JB, Martínez-Pérez D. The principle of rotation advancement for
repair of unilateral complete cleft lip and nasal deformity: technical varia-
tions and analysis of results. Plast Reconstr Surg. 1999; 104(5):1247–1260
Millard DR, Latham R, Huifen X, Spiro S, Morovic C. Cleft lip and palate treated
by presurgical orthopedics, gingivoperiosteoplasty, and lip adhesion (POPLA)
compared with previous lip adhesion method: a preliminary study of serial
dental casts. Plast Reconstr Surg. 1999; 103(6):1630–1644
Cutting CB, Dayan JH. Lip height and lip width after extended Mohler
unilateral cleft lip repair. Plast Reconstr Surg. 2003; 111(1):17–23, dis-
cussion 24–26
Mulliken JB, LaBrie RA. Fourth-dimensional changes in nasolabial dimensions
following rotation-advancement repair of unilateral cleft lip. Plast Reconstr
Surg. 2012; 129(2):491–498
Patel KB, Mulliken JB. Correction of the vestibular web during primary repair
of unilateral cleft lip. Plast Reconstr Surg. 2014; 134(4):600e–607e
Millard DR. Cleft Craft: The Evolution of Its Surgery. The Unilateral Deformity.
Vol 1. Boston, MA: Little Brown & Co; 1976
Farkas LG, Posnick JC, Hreczko TM, Pron GE. Growth patterns of the nasolabial
region: a morphometric study. Cleft Palate Craniofac J. 1992; 29(4):318–324
Bardach J, Cutting C. Pathogenesis of the cleft lip nasal deformity. In: Barach J,
Hughlett LM, eds. Multidisciplinary Management of Cleft Lip and Palate. Phil-
adelphia, PA: WB Saunders; 1990
Fisher DM. Unilateral cleft lip repair: an anatomical subunit approximation
technique. Plast Reconstr Surg. 2005; 116(1):61–71
Cleft Lip and Palate
148

14 Bilateral Cleft Lip
Alexander C. Allori
Sum m ary
Repair of bilateral cleft lip has been regarded by many to be
among the most challenging and rewarding of plastic surgical
procedures. Methods of repair have evolved considerably
over the past several decades. Presently, all repairs should be
soundly founded in the modern principles that have been dis-
tilled and clarified from this evolutionary process. This chapter
presents a practical synthesis and application of those princi-
ples to contemporary bilateral cleft lip repair, in the style of
Mulliken.
Keywords: bilateral cleft lip, cleft lip nasal deformity, orofacial
cleft
14.1 Introduction
A number of infants born with bilateral cleft lip undergo old-fash-
ioned, often multistaged, procedures, and later, they have to
endure sundry revisions throughout childhood and adolescence.
Despite the surgeon’s best e orts, the stigmata of the repaired
cleft lip and nose remain painfully obvious—even at a distance. To
the contrary, … the appearance of a child with repaired bilateral
cleft lip should be comparable to, and in many instances surpass,
that of a repaired unilateral cleft lip.
— John B. Mulliken
This chapter provides an introduction to the operative treat-
ment of bilateral cleft lip, with or without cleft alveolus and
with or without cleft palate. The history of bilateral cleft lip
repair is rich, interesting, and lengthy. For those interested, a
thorough recounting of this history is available. This chapter
focuses on a practical synthesis and application of the princi-
ples that have been distilled from this evolutionary process.
14.2 Anatomy, Terminology, and
Classification
A history of classification systems for cleft lip or palate summa-
rizes the major types of the bilateral paramedian orofacial cleft
deformity, as follows ( Table 14.1):
“Lesser-form” cleft lip is an “umbrella term” that may be fur-
ther described as minor-form, microform, or mini-microform
cleft lip.
If the two sides of the bilateral cleft lip are clefted to the same
degree (e.g., bilateral complete and bilateral incomplete), the
cleft is termed symmetric. Contrarily, if the two sides of the
bilateral cleft lip are clefted to di erent degrees (e.g., complete +
incomplete and complete +lesser form), then the cleft is termed
asymmetric. The ontological qualifier “symmetric” considers
the severity of only the preforaminal (labial and alveolar) clefts
on each side; the term does not encompass other aspects of
symmetry that are important to the surgeon, such as the possi-
bility of a deviated premaxilla that is situated closer to one side
than the other (Fig. 14.1).
Premaxillary malpositioning will occur only if the alveolar
arch is clefted. In the typical bilateral symmetric cleft lip (e.g.,
bilateral complete and bilateral incomplete), the premaxilla and
vomer usually remain in the midline (nondeviated). In the bilat-
eral complete case, the prolabium is separated from the lateral
labial elements and the premaxilla is separated from the lateral
alveolar processes; this allows unrestrained grow th of the
vomer and results in anterior projection and/or proclination of
the premaxillary segment. In the bilateral incomplete case, the
retention by the partial labial and/or partial alveolar attach-
ment may prevent projection and proclination. In bilateral
asymmetric cleft lip (e.g., complete +incomplete), an imbalance
of retention forces on one side versus the other results in devia-
tion of the premaxilla toward the lesser side.
The morphology of any palatal cleft that may be present will
also have an e ect on the position of the premaxilla and vomer.
Table 14.1 Classification systems for cleft lip and palate
Laterality

Severity

Anatomic involvement
Bilateral

Complete

Cleft

Lip
Incomplete

Lip and alveolus (primary
palate)
Lesser form

Lip, alveolus (primary pal-
ate), and secondary palate
Fig. 14.1 Bilateral complete cleft lip, alveolus, and Veau-IV palate.
Although ontologically “symmetric” (because the severity on each side
is complete), there is significant rightward deviation of the premaxilla.
Bilateral Cleft Lip
149

In the typical case of bilateral cleft lip with cleft palate, the pala-
tal cleft is morphologically Veau-IV (i.e., a midline cleft of the
secondary palate, with exposure of the vomer and bilateral ex-
tension through each alveolar process). Of course, it is possible
for the palatal cleft to be morphologically di erent (i.e., Veau-
III, Veau-II, and Veau-I), and it is therefore important to describe
the morphology of the cleft palate in the complete description
of the cleft phenotype. Veau-III cleft palate (unilateral complete
cleft through one side of the alveolar arch, with vomerine
attachment to the contralateral palatal shelf) results in appreci-
able deviation of the vomer and premaxilla.
The anatom ic details of the bilateral cleft are important
for preoperative planning and intraoperative treatment. In
the text that follows, we will use a shorthand notation to
describe specific phenotypic cases: bCL, bCLA, and bCLAP for
bilateral cleft lip; bilateral cleft lip and alveolus; and bilat-
eral cleft lip, alveolus, and palate, respectively. If m ore phe-
notypic detail is of significance to the discussion, it will be
described in longhand.
14.3 Planning
14.3.1 Principles
The modern approach to primary repair of the bilateral cleft lip
and related nasal deformity is founded on several guiding
principles:
● Maintain (or establish) symmetry.
● Prepare the projecting premaxilla.
● Anticipate fourth-dimensional changes that occur with
growth.
● Construct a full central lip by using lateral labial elements and
discard prolabial vermilion.
● Deepen the gingivolabial sulcus by using premaxillary
mucosa.
● Establish muscular continuity primarily.
● Address the nasal deformity synchronously.
The first three principles relate to operative planning or prepa-
ration, whereas the remaining principles relate to technical
details that should be executed skillfully during the operative
repair. For thorough exploration of these principles and the his-
tory behind them, the reader may refer to Mulliken and Allori
and Marcus. Disregard for some of these principles may lead to
inferior outcomes and the stigmata of bilateral cleft lip repair
(Fig. 14.2).
14.3.2 Treatment Algorithm
Presentation in Infancy
Ideally, a cleft team will first encounter an infant with cleft lip
and/or palate (CL/P) in the neonatal period, perhaps after previ-
ously having met the then-expectant parents in prenatal con-
sultation. The purpose of the first encounter is to examine the
child, document an accurate phenotype, and begin to formulate
and coordinate the treatment plan; to evaluate feeding and
nutrition and provide feeding education and support; and to
begin the process of preoperative education for the parents.
As described elsewhere in this textbook, protocols for treat-
ment of a child with CL/P may di er from center to center. Gen-
erally, the early surgical timeline for a child who presents with
the condition in infancy proceeds as follows ( Table 14.2):
For a child with bCL (with intact alveolus and palate), the sur-
geon may proceed with primary labial repair directly.
For bCLA or bCLAP, the cleft surgeon should first determine
whether presurgical orthopedics is indicated and available. If
so, this process should be initiated as early as possible in order
to achieve maximal e ectiveness in a timely fashion, so that
operative treatment is not unduly delayed. Presurgical orthope-
dics is described in detail in a subsequent section of this chap-
ter. In centers where presurgical orthopedics is not available,
bilateral labial or nasolabial adhesion may be performed
instead. Nasolabial adhesion achieves some of the same e ects
as presurgical orthopedics on premaxillary repositioning from
the natural retrograde pull of the labial elements. Nasolabial
Fig. 14.2 These two patients demonstrate some
of the stigmata of bilateral cleft lip repair when
some of the principles are not followed. (a) Here,
a very wide, shield-shaped philtrum is evident.
The median tubercle is deficient, and an absence
of vermilion is made up for by pseudokeratinized
mucosa. There is no white roll under the
philtrum. In full smile, the ptotic premaxillary
mucosa is evident. The alar base remains wide,
and the nasal tip is broad and flat. (b) In profile,
the columella is short, and the nasal tip lacks
projection and definition. Moreover, evident in
this photograph are the marked maxillary hypo-
plasia and retrusion, with negative overjet and
class III malocclusion. A late sequela of this is the
cleft lip lower lip deformity (CLLLD) that results as
it is chronically stretched to ensure oral closure.
Cleft Lip and Palate
150

adhesion is described in section “Adhesion Cheiloplasty: Labial
Adhesion and Nasolabial Adhesion.”
Nasolabial adhesion may be performed for another reason: it
may be utilized to great e ect in case of bilateral asymmetric
case, in order to preliminarily establish better symmetry before
proceeding with lip repair. For example, in case of complete +
incomplete bCLA, nasolabial adhesion may be performed uni-
laterally on the complete side, e ectively converting the asym-
metric phenotype into symmetric bilateral incomplete cleft lip
and alveolus. The primary labial repair that follows is thereby
facilitated in both the design and the execution of the repair. In
deciding whether to include nasolabial adhesion for this pur-
pose in his or her treatment protocol, the cleft surgeon must
weigh the relative benefit of improved symmetry with the risk
and burden of the extra anesthetic and operative procedure.
Late Presentation
For many reasons, including significant comorbidities and
international adoption, some children may first present to the
cleft team at an older age. At this time, malposition of the pre-
maxilla poses a greater challenge due to the strength and
rigidity of the vomer in an older child; that is, although the
vomer of the neonate may be “buckled” by presurgical orthope-
dic techniques or by nasolabial adhesion, this is no longer possi-
ble in the older child.
If the premaxillary projection or proclination in the older
child is minor, it may be possible to perform preliminary adhe-
sion or to proceed directly to labial repair, with minimal tension
on the labial repair. On the other hand, if the premaxilla is
severely projecting or proclined, this precludes a safe and e ec-
tive labial repair, because the gap that must be spanned by
the cutaneous and muscular closure is too great and will be
under excessive tension. In order to repair the lip over this
gap, the surgeon is forced to design an unaesthetically wide
philtral flap; moreover, the size of the gap makes continuous
muscular approximation di cult and gingivoperiosteoplasty
(GPP) impossible.
In addition to the challenges of premaxillary projection or
proclination, premaxillary deviation is also di cult to correct
in the older child. This deviation results in an asymmetry that
cannot be compensated for merely by alteration of the design of
the repair.
In such cases, the skeletal malposition requires a skeletal cor-
rection: premaxillary ostectomy and setback. This procedure is
described in section 14.4.3, "Premaxillary Ostectomy and Set-
back". Importantly, premaxillary setback must be done as a
preliminary procedure and should never be combined with pri-
mary cheiloplasty. In premaxillary ostectomy, the division of
the vomer disrupts the posterior blood supply to the premax-
illa, leaving the premaxilla totally dependent on anterior blood
supply from the prolabium. If the philtral flap is dissected and
raised, this will also eliminate the anterior blood supply, risking
avascular necrosis of the premaxilla. Consequently, if premaxil-
lary ostectomy and setback are necessary, they must be done 3
to 4 months before the lip repair. It is safe to combine premaxil-
lary ostectomy and setback with either nasolabial adhesion (in
case of bCLA) or palatoplasty with GPP (in case of bCLAP). These
procedures improve the fixation and support of the retroposi-
tioned premaxilla, while respecting its prolabial blood supply.
In additionally, in case of an older child with bCLAP, performing
palatoplasty at this time e ciently combines two procedures
that this child needs under one anesthetic. The definitive labial
repair (with inherent elevation of the philtral flap) may be
safely performed approximately 3 to 4 months following the
premaxillary setback.
14.4 Preparation
14.4.1 Presurgical Orthopedics
Presurgical orthopedics refers to the remodeling of the dentofa-
cial skeleton by way of orthodontic techniques. Generally, this
process may be either active or passive. Active processes use
mechanical gears and levers to exert force on the dentofacial
skeleton in order to remodel it quickly. An example of an active
device is the Georgiade–Latham device, or pin-retained elastic
chain premaxillary retraction (ECPR) device. In contrast, a pas-
sive device gradually remodels the dentofacial skeleton in res-
ponse to statically sustained extrinsic forces that are increased
at periodic intervals. Examples include lip taping, palatal
splints, and nasoalveolar molding (NAM). Both approaches
require the participation and expertise of craniofacial ortho-
dontists. The various techniques of presurgical orthopedics
have been debated fiercely. The merits and demerits of the
most common methods are worthy of review and have been
summarized recently in an excellent point/counterpoint article.
Both the Georgiade–Latham’s device and NAM are e ective
at widening a collapsed maxillary arch and at retropositioning
the projecting and retroclining the proclined premaxilla. As an
active technology, the Georgiade-Latham’s device is quite rapid,
with treatment completed over the course of 3 to 6 weeks. In
contrast, NAM requires 3 to 4 months. The Georgiade-Latham’s
device requires a separate anesthetic for initial device place-
ment, but it may be activated by parents (by turning a screw) at
home, with periodic clinical check-ups. Although NAM does not
require an anesthetic at any point during treatment, it does
require weekly clinical visits in order to modify the appliance.
One significant advantage that NAM has over the Georgiade-
Latham’s device is that midway through treatment, outrigger
nasal prongs are added to begin to round out the flattened
nasal ala. Although many single-center, long-term case series
have been published that demonstrate the e cacy of this tech-
nique, there remains considerable debate in the literature
regarding its widespread e ectiveness.
Table 14.2 Early surgical time-line for a child with cleft lip or palate
Age

Intervention
0–4 mo of age

Possible presurgical orthopedics
0–4 mo of age

Possible labial or nasolabial adhesion
3–5 mo of age

Primary cheiloplasty (labial repair), with or without
gingivoperiosteoplasty and with or without syn-
chronous correction of nasal deformity
10–12 mo of age Primary palatoplasty
Bilateral Cleft Lip
151

14.4.2 Adhesion Cheiloplasty: Labial
Adhesion and Nasolabial Adhesion
In circumstances where presurgical orthopedics are not avail-
able and where premaxillary protrusion, proclination, and devi-
ation are significant, the surgeon may opt to perform nasolabial
adhesion in order to indirectly achieve retropositioning and
centralization of the premaxilla. The basic principle behind
adhesion is to connect each side of the cleft, utilizing areas of
the cleft margin that will be discarded in the definitive repair.
To perform adhesion, it is advised that the surgeon first draw
the markings for the definitive cheiloplasty, which will be per-
formed later. The adhesion should be performed by utilizing
the tissues of the prolabium and lateral lip elements that will
be discarded in the future repair. In this fashion, any scar that
forms during the adhesion would be discarded. Importantly,
dissection is minimized in order to prevent excessive scarring,
which would complicate the future repair. Labial adhesion con-
nects the cutaneous lip in an “open-book” fashion, whereas
nasolabial adhesion extends this superiorly to preliminarily
close the nasal sill, utilizing the columellar flaps. In either case,
there is no muscular dissection, and the prolabial skin should
not be elevated o the premaxilla. The technique for adhesion
is described further in this textbook in the chapter on unilateral
cleft lip repair.
14.4.3 Premaxillary Ostectomy and
Setback
The method of premaxillary ostectomy and setback directly
achieves retropositioning and centralization of the premaxilla.
It is usually reserved for the older child who is no longer a can-
didate for presurgical orthopedics or nasolabial adhesion.
To perform the technique, the patient is placed on the operat-
ing room table in supine position, with a shoulder roll to pro-
vide gentle neck extension. A Dingman’s oral retractor is placed
to provide adequate exposure of the palate. In case of bCLA, the
secondary palate will be intact, but the vomer will be visible
through a defect in the primary (preforaminal) palate. In case of
bCLAP, the cleft palate will expose the vomer in its entirety.
For bCLA, the intention is to retroposition the premaxilla,
close the nasal mucosa, close the oral mucosa, and perform GPP.
After infiltration of local anesthesia with epinephrine, a midline
incision is made on the mucosa overlying the exposed region of
the vomer. A Cottle’s periosteal elevator is used to elevate the
mucoperiosteal vomerine flaps, exposing the underlying bone
and the premaxillary–vomerine suture. Next, incisions should
be made at the margin of the primary palatal cleft, and the Cot-
tle’s elevator should be used to separate the nasal mucosal lin-
ing from the oral mucosa, extending this dissection posteriorly
above and below the palatal shelves. Similarly, incision should
be made in the mucosa on the posterior aspect of the premax-
illa, and this should be gently reflected to provide flaps to
which the nasal and oral mucosa may be sutured. The ostec-
tomy is then performed immediately posterior to the premaxil-
lary–vomerine suture, using a rongeur. The amount of bone
that must be removed is dependent on how much it needs to
be moved backward, and the shape of bone removal (whether
rectangular or trapezoidal) is dependent on whether the
premaxilla needs to be rotated as well. In actuality, bone is
removed incrementally until the proper position is achieved. It
is critical not to remove too much bone because direct bone-to-
bone contact is required for osseous union. Rigid fixation is not
required. Some surgeons pass a Kirschner’s wire through the
premaxilla into the vomer, which provides temporary semirigid
fixation. It is the practice of the author to secure the bone with
one 2–0 Vicryl suture, which prevents undue movement during
the immediate postoperative period. Some surgeons do not fix-
ate the bone at all and just rely on the mucosal closure and GPP
to tether the premaxilla in its new position. Next, the nasal
mucosa should be closed by using 5–0 Vicryl or chromic suture,
and the oral mucosa should be approximated by using 4–0
Vicryl. The GPP should then be completed in the typical
fashion.
For bCLAP, the intention is to secure the retropositioned pre-
maxilla by way of the palatal repair and GPP. In this case, the
procedure starts by designing the typical two-flap palatoplasty
for repair of the Veau-IV cleft palate, as described in detail else-
where in this textbook. After elevation of the oral mucoperios-
teal flaps and elevation of the nasal mucosal lining from the
palatal shelves, the vomerine flaps are elevated from the under-
lying vomer. Prior to closure of the nasal floor, the premaxillary
ostectomy is performed, and the premaxilla is retropositioned,
as described earlier. The vomerine flaps are then used to close
the nasal floor on each side, and this nasal mucosal closure is
completed anteriorly at the premaxilla. Gingivoperiosteoplasty
is then completed to close the alveolar clefts and further stabi-
lize the new position of the premaxilla. The palatal repair is
then completed in the typical fashion ( Fig. 14.3).
For infants, the author recommends the placement of a tem-
porary petroleum gauze palatal splint for 1 day. It is removed
before discharge from the hospital.
For an older child with deciduous dentition, it is recom-
mended that a custom-made dental splint be made that will
protect the premaxilla in its new position. This is more impor-
tant to protect the older child (who may injure himself or her-
self during play) than for the purpose of semirigid fixation.
14.5 Primary Repair of Bilateral
Cleft Lip
Although several techniques for bilateral cleft lip repair have
been described, the Mulliken’s technique is described as
follows.
14.5.1 Marking
A wide double hook is placed in the nostrils to gently elevate
the nasal tip and establish symmetry, if needed. The standard
anthropometric landmarks should be marked:
● Nasion (n).
● Endocanthion (en).
● Pronasale (prn).
● Subnasale (sn).
● Ala nasi (al).
● Subalaris (sbal).
● Labiale superius (ls).
● Crista philtri superioris (cphs).
Cleft Lip and Palate
152

● Crista philtri inferioris (cphi).
● Stomion (sto).
● Chelion (ch).
When taking measurements for symmetry, sn should serve as
the main reference for prolabial markings and sbal for lateral lip
markings. In addition to the above landmarks, the vermilion-
mucosal junction (“red line”) of the lip is indicated with a dot-
ted line.
On the prolabium, the philtral flap is drawn. For a child
younger than 1 year, the philtral flap should be designed to be
2-mm wide superiorly (cphs–cphs), 4-mm wide inferiorly
(cphi–cphi), and 6 to 7 mm vertically (sn–ls). In complete cleft
lip, the total available height of the prolabium is often 6 to
7 mm. In incomplete cleft lip, the prolabium may actually be
longer, and in this case, the excess prolabial height will not be
included in the design of the philtral flap. The sides of the phil-
tral flap are concave. The philtral flap is bordered on each side
by 2 to 3 mm “flanking flaps” that will be deepithelialized.
These flanking flaps provide extra bulk for the philtral ridge
and also improve the vascular supply to the philtral flap. At the
top of the prolabium, horizontal lines are drawn to indicate the
C-flaps that are preserved and utilized in the closure of the
nasal floor.
On the lateral labial elements, Noordho ’s point is marked at
the medial-most location of the lip, where a distinct white roll
is present and where the height of vermilion is maximal. This
point may be adjusted slightly to ensure that there are 3 mm of
white roll medial to the proposed location of the cphi point of
the Cupid’s bow. It is important not to place cphi too medially,
where the white roll has become indistinct or where the vermi-
lion has begun to taper. In addition, it is important not to place
the point too laterally, as this will result in a tight closure and
possibly microstomia. Generally, the cphi–ch distance should
be greater than 15 mm on each side. The lateral labial cutaneous
advancement flaps are drawn as large as possible, given they
will be trimmed to fit later in the procedure. In marking these
flaps, the white roll should remain on the side of the vermi-
lion. At its superior aspect, the marking for the advancement
flap curves around the alar base up to, or just beyond, sbal
(Fig. 14.4).
Dilute anesthetic with epinephrine should be infiltrated. Key
landmarks are tattooed with methylene blue, and a scalpel may
be used to score the markings.
14.5.2 Operative Technique
The general operative sequence is shown as follows; however,
in actuality, some maneuvers are interdependent:
1. Philtral flap and lateral labial dissection.
2. Mucosal flap for deepening of the gingivolabial sulcus.
3. Gingivoperiosteoplasty.
4. Closure of the nasal floor.
5. Labial closure.
6. Nasal correction.
7. Skin closure.
Labial Dissection
The operation begins with dissection of the philtral flap. The
incision is made with a # 69a mini blade or # 15c scalpel. Great
care should be taken in the deepithelialization of the flanking
flaps, such that the dermis is preserved and remains attached
to the philtral flap. The remaining prolabial skin is removed
and discarded. The philtral flap (en bloc with the flanking flaps)
is then elevated superiorly, and sharp scissors are used to com-
plete the dissection in the subcutaneous plane to the level of
the anterior nasal spine.
The lateral labial flaps are incised as marked, and the vermi-
lion-mucosal flaps are separated sharply from the musculocu-
taneous portion of the lip. Near the alar base, sharp dissection
is performed submuscularly to the level of the maxilla and then
continued laterally in the supraperiosteal plane. The attach-
ment of the alar base to the piriform rim is released. A Tessier’s
elevator is used to complete this supraperiosteal mobilization
of the soft tissue of the cheeks as far laterally as the malar emi-
nences. This dissection is important for reducing the tension on
the lip repair.
The cutaneous advancement flap is secured with fine double
hooks, and scissors are used to sharply dissect the orbicularis
oris muscle from the overlying skin and the underlying oral
mucosa.
Fig. 14.3 A 10-month-old infant with bilateral complete cleft lip, alveolus, and Veau-IV palate, presented too late for presurgical orthopedics,
requiring premaxillary ostectomy and setback. (a,b) The premaxilla is significantly projecting and moderately proclined. (c) Premaxillary ostectomy
and setback were performed in combination with palatoplasty. Gingivoperiosteoplasty could not be completed due to the width of the alveolar cleft.
(d) The cleft lip repair is completed 3 months later. Healing in this case was complicated by scar hypertrophy, especially lateral to the left side of the
philtral flap.
Bilateral Cleft Lip
153

Mucosal Flap and Gingivoperiosteoplasty
Next, the subcutaneous or submucosal tissue overlying the pre-
maxilla is sharply removed. The thin strip of prolabial vermilion
is removed and discarded. The premaxillary mucosa is elevated
to form an inferiorly based mucosal flap, which is then pulled
upward and secured to the premaxillary periosteum at the level
of the anterior nasal spine by using Vicryl or polydioxanone
(PDS) suture. This flap forms the posterior wall of the new gin-
givolabial sulcus. If the mucosal flap is not lifted superiorly, the
gingivolabial sulcus will be very shallow and premaxillary
mucosa will hang down and create the appearance of a “double
lip” when the child smiles.
Gingivoperiosteoplasty is completed at the same time as the
mucosal flap creation. The gingiva and periosteum of the lateral
alveolar segments are incised vertically. The gingivoperiosteal
flaps are secured by using 6–0 or 5–0 chromic suture, begin-
ning posteriorly and proceeding anteriorly.
Closure of the Nasal Vestibule and Nasal Sill
Medial nasal mucosal flaps are elevated from the premaxilla,
and corresponding lateral nasal mucosal flaps are elevated from
the inferior turbinates. Closure of the vestibular mucosa pro-
ceeds from posterior toward anterior side.
Closure of the cutaneous nasal sill is completed later in the
procedure, after beginning the labial closure and repositioning
the nasal cartilages. At that time, the alar base is transposed
medially and sutured to the prolabial C-flaps by using 7–0
Vicryl suture for the dermis and 7–0 chromic suture for the
skin. The columellar flaps may require trimming to achieve the
ideal alar base width. An alar base cinch suture (5–0 Prolene,
passed through the dermis of each alar base near sbal and
through periosteum of anterior nasal spine) is used to precisely
control the alar base width. Myrtiform sutures (4–0 PDS suture
placed in maxillary periosteum just medial to each canine fossa
and passed through the midpoint of each nasal sill) may be
used to slightly depress the nasal sill and floor.
Labial Closure
After completion of the GPP and mucosal flaps, the lateral labial
mucosa is next to be closed. An inferolateral back cut may be
necessary for tension-free advancement of the closure. A 5–0
chromic suture may be used for the closure. In performing this
step, it is extremely important to apply firm traction medially
during the closure in order to adequately advance the flaps.
Next, orbicularis oris muscle is approximated in the midline
by using 4–0 Vicryl or PDS suture in horizontal mattress fashion.
The superior aspect of the orbicularis oris muscle is secured to
the periosteum of the anterior nasal spine by using 4–0 Prolene
suture to prevent it from sliding inferiorly with facial animation.
The vermilion flaps are then used for creation of the median
tubercle. The previously tattooed cphi points are identified on
each side, and a mark is made 3 mm medial to each. A 7–0 Vicryl
suture and a 7–0 chromic suture are used to approximate these
marks to create the new ls point, into which the point of the
philtral flap will later be inset. Vermilion closure proceeds down
the midline to create the median tubercle. Excess vermilion or
mucosa may be deepithelialized/demucosalized, with the sub-
mucosa used to augment the volume of the median tubercle.
Nasal Correction
As the cleft lip is repaired and the alar base width is narrowed,
the cleft lip nasal deformity (CLND) often worsens drastically.
Fig. 14.4 (a) A newborn with bilateral complete cleft lip, alveolus, and Veau-IVcleft palate, who underwent (b) nasoalveolar molding in preparation for
lip repair. (c,d) Following NAM, there was minor residual deviation of the premaxilla, which was compensated for in the design (e) of the repair. (f,g)
The final two photographs show the result at the time of suture removal.
Cleft Lip and Palate
154

Therefore, synchronous correction of the CLND has been advo-
cated. Several approaches have been described, including use of
percutaneous transfixion sutures and semiopen approach. In
the semiopen approach, the medial crura of the lower lateral
cartilages (LLCs) are mobilized beneath the C-flap through the
open labial incisions. Bilateral alar rim incisions or marginal
incisions are used to expose the inferior aspect of the LLCs.
Great care must be taken not to injure the cartilages during this
process. The LLCs are exposed and cleaned of fibrofatty tissues
on their superficial and medial surfaces. Similarly, the upper lat-
eral cartilages (ULCs) are exposed. A cotton-tip applicator may
be placed into the nasal vestibule, and gentle upward traction is
used to visualize the location of the genua to be created in each
LLC. Medial to each genu, two interdomal 5–0 PDS sutures are
placed between the domes of each LLC but are left untied.
Lateral to each genu, two intercartilaginous sutures are used to
re-create the interface between the LLC and the ULC (“scroll
region”). With the cotton-tip applicator assisting with proper
positioning of the cartilages in relation to each other, the
sutures are sequentially tied. The skin is then closed by using
7–0 chromic suture. Any redundant domal skin can be carefully
excised in crescentic fashion to improve the contour of the alar
rim. At this point, any preexisting vestibular web may be accen-
tuated. This can be corrected through lenticular excision of
mucosa and closure.
Skin Closure
To complete the procedure, the tip of the philtral flap is inset to
meet the ls point of Cupid’s bow. A 7–0 Vicryl suture is used to
reapproximate dermis, and a 7–0 nylon suture is used for skin
closure. The lateral labial cutaneous advancement flaps are
trimmed superiorly to conform to the shape of the nasal sill.
Closure is completed in two layers, with 7–0 Vicryl and 7–0
chromic sutures.
14.5.3 Postoperative Care
At the completion of the procedure, the face is washed and
dried. Bacitracin ointment may be applied to the incision line. It
is the author’s practice to place temporary vented stents (Xero-
form wrapped around 19-gauge butterfly tubing and cut to 1.25-
cm length) in each nostril. These are removed before the patient
is discharged from the hospital. Some surgeons tape a Logan’s
bow to the cheeks to support an iced saline gauze over the inci-
sion line to reduce bloody crusting in the perioperative period.
The patient should be observed overnight. The parents
should be educated on careful feeding techniques and on lip
hygiene. Any bloody crusting should be gently cleaned with
half-strength hydrogen peroxide and saline, and antibiotic oint-
ment should be reapplied. It is important that the parents feel
comfortable with suture-line care and are well aware of how to
keep the nostrils clean.
One week postoperatively, sutures are removed under gen-
eral anesthesia by using mask induction and insu ation. A
Steri-Strip is placed from side to side to help protect the healing
incisions. This Steri-Strip is replaced as needed over the course
of the next 2 to 4 weeks.
Further postoperative follow-up occurs at 1 month and 3
months. At 1 month, the parents are instructed on how to per-
form scar massage and are advised to apply sunblock (SPF≥50)
in the morning and skin lotion at night. At times, a thickened
scar that starts to cause contracture requires intralesional injec-
tion of Kenalog (triamcinolone). This is performed in the clinic
and may be repeated every 4 to 6 weeks up to three times. Scar
massage should continue throughout this period until the scar
softens and flattens.
Suggested Readings
Allori AC, Marcus JR. Modern tenets for repair of bilateral cleft lip. Clin Plast
Surg. 2014; 41(2):179–188
Allori AC, Mulliken JB, Meara JG, Shusterman S, Marcus JR. Classification of
cleft lip/palate: then and now. Cleft Palate Craniofac J. 2015:E-pub ahead of
print
Byrd HS, Ha RY, Khosla RK, Gosman AA. Bilateral cleft lip and nasal repair.
Plast Reconstr Surg. 2008; 122(4):1181–1190
Chen PK, Noordho MS. Bilateral cleft lip and nose repair. In: Losee JE, Kirsh-
ner RE, eds. Comprehensive Cleft Care. New York, NY: McGraw-Hill Professio-
nal; 2008:343–360
Fisher MD, Fisher DM, Marcus JR. Correction of the cleft nasal deformity:
from infancy to maturity. Clin Plast Surg. 2014; 41(2):283–299
Grayson BH, Garfinkle JS. Early cleft management: the case for nasoalveolar
molding. Am J Orthod Dentofacial Orthop. 2014; 145(2):134–142
Grayson BH. Discussion: limited evidence for the e ect of presurgical nasoal-
veolar molding in unilateral cleft on nasal symmetry: a call for unified
research. Plast Reconstr Surg. 2013; 131(1):75e–76e
Hathaway RR, Long RE , Jr. Early cleft management: in search of evidence. Am
J Orthod Dentofacial Orthop. 2014; 145(2):135–141
Marcus JR, Fisher DM, Lenz GJ, Magee WP, Zuker RM. Inadequate gingivolabial
sulcus remains an avoidable problem after bilateral cleft lip repair. Plast
Reconstr Surg. 2005; 115(1):220–225
Monson LA, Kirschner RE, Losee JE. Primary repair of cleft lip and nasal
deformity. Plast Reconstr Surg. 2013; 132(6):1040e–1053e
Mulliken JB. Bilateral cleft lip. Clin Plast Surg. 2004; 31(2):209–220
Mulliken JB. Repair of bilateral cleft lip. In: Neligan PC, Rodriguez ED, Losee JE,
eds. Plastic Surgery. Vol. 3. Philadelphia, PA: Saunders-Elsevier Health Scien-
ces; 2012:343–360
van der Heijden P, Dijkstra PU, Stellingsma C, van der Laan BF, Korsten-Meijer
AG, Goorhuis-Brouwer SM. Limited evidence for the e ect of presurgical
nasoalveolar molding in unilateral cleft on nasal symmetry: a call for unified
research. Plast Reconstr Surg. 2013; 131(1):62e–71e
Yuzuriha S, Mulliken JB. Minor-form, microform, and mini-microform cleft
lip: anatomical features, operative techniques, and revisions. Plast Reconstr
Surg. 2008; 122(5):1485–1493
Yuzuriha S, Oh AK, Mulliken JB. Asymmetrical bilateral cleft lip: complete or
incomplete and contralateral lesser defect (minor-form, microform, or mini-
microform). Plast Reconstr Surg. 2008; 122(5):1494–1504
Bilateral Cleft Lip
155

15 Cleft Palate
Ingrid Ganske and Carolyn R. Rogers-Vizena
Sum m ary
Cleft palate is a congenital craniofacial anomaly that a ects
feeding, hearing, and speech. The hard palate gives structural
support, and the soft palate provides velopharyngeal compe-
tence. The aim of cleft palate repair is to close the oronasal fis-
tula and to construct a dynamic velar sling, thereby establishing
oronasal competence and enabling control of airflow for intelli-
gible speech. With this goal in mind, palate anatomy, perio-
perative concerns, surgical technique, postoperative care, and
palatoplasty complications are described in this chapter.
Keywords: cleft palate, cleft palate repair, palatoplasty, Robin
sequence, velopharyngeal, speech surgery
15.1 Introduction
Detailed understanding of palate development, normal and cleft
palate anatomy, and epidemiology of clefting is essential to the
comprehensive care of children with cleft palate.
15.1.1 Embryology
Palatal processes form from the maxillary prominences during
the seventh week postconception. These vertically oriented
shelves elevate to a horizontal position by the ninth week post-
conception. They meet with each other to form the secondary
palate and also fuse with the primary palate. Failure of eleva-
tion and fusion of these shelves result in a cleft palate.
15.1.2 Anatomy
Normal Palate Anatomy
The incisive foramen is located at the midline, posterior to the
alveolus, at the junction of the primary and secondary palate.
Structures anterior to the incisive foramen are considered the
primary palate, and structures posterior to the incisive foramen
are considered the secondary palate. The secondary palate com-
prises both hard and soft palate. The hard palate is the grow th
center for the maxilla and is formed by paired palatine proc-
esses of the maxilla and palatine bones, articulating with the
vomer. The soft palate is a muscular sling formed by the paired
levator veli palatini, palatopharyngeus, palatoglossus, and uvular
muscles (innervated by the pharyngeal branch of the vagus
nerves) and is reinforced by the broad aponeurosis of the tensor
veli palatini (innervated by the medial pterygoid branch of the
mandibular nerves). These muscles work with the superior
constrictor to form the velopharyngeal sphincter.
Palatal and velopharyngeal blood supply comes from
branches of the paired internal maxillary arteries, recurrent
pharyngeal arteries, ascending pharyngeal arteries, and ascend-
ing palatine arteries. The soft palate and velopharyngeal
sphincter muscles have a rich blood supply from branches of
these arteries. The greater palatine branches of the internal
maxillary artery provide the major blood supply to mucoper-
iosteum of the hard palate ( Fig. 15.1).
Cleft Palate Anatomy
Isolated cleft palate involves secondary palatal structures. Max-
illary and palatine bones are separated at the midline, resulting
in an oronasal fistula. The tensor veli palatini aponeurosis, leva-
tor veli palatini muscles, and palatopharyngeus muscles are dis-
oriented parallel to the cleft, inserting on the posterior hard
palate ( Fig. 15.1). As a result, function of the velopharyngeal
sphincter is compromised. These muscles normally participate
in ventilation and pressure equalization of the middle ear.
Altered anatomy of these muscles results in Eustachian tube
dysfunction, frequently causing chronic middle ear e usion,
hearing loss, and otitis media.
Fig. 15.1 Palate anatomy.
Cleft Lip and Palate
156

In cleft lip and palate, the defect extends along the junction
of the primary and secondary palate, resulting in an alveolar
gap. When the alveolus is cleft, lateral incisors are frequently
absent and other dental anomalies, such as supernumerary
teeth, are common. The palatal component of orofacial clefts is
often described using the Veau classification (Fig. 15.2).
Submucous cleft palate is a lesser form of cleft palate involv-
ing muscular diastasis of the soft palate, with intact palatal
mucosa. The diastasis may be visualized as a bluish “zona pellu-
cida,” or furrow, in the soft palate. Other findings may include
a bifid uvula and/or a notch in the posterior hard palate
(Fig. 15.3). These three findings constitute Calnan’s triad. This
type of cleft is often diagnosed during evaluation of abnormal
speech.
15.1.3 Epidemiology
Traditionally, cleft lip with or without cleft palate (CL/P) and
isolated cleft palate (CP), also known as cleft palate only, have
been considered separate etiopathogenetic entities; however,
emerging evidence suggests that the delineation is less distinct.
In the United States, recent data from the National Institute of
Dental and Craniofacial Research estimate the incidence of CL/P
to be approximately 1 in 940 live births. The condition is twice
as common in male patients compared with female patients
and has ethnic heterogeneity, with Asians and Hispanics
demonstrating the highest incidence, Caucasians demonstrat-
ing intermediate incidence, and Africans manifesting the lowest
incidence. Estimated incidence of CP is approximately 1 in
1,570 live births. The condition is twice as common in female
patients and does not have the same ethnic variation that
CL/P has.
Clefts of the lip and/or palate result from a combination of
genetic and environmental factors. Environmental factors,
including maternal smoking, folate deficiency, alcohol con-
sumption, advanced maternal age, and retinoid use, are associ-
ated with increased risk. Mutations in multiple candidate
genes, such as IRF6, Wnt signaling, MSX1, and BMP signaling
factors, have also been associated with clefting. Cleft lip or pal-
ate is infrequently (10–14%) associated with a recognized
syndrome, but 29 to 45% of patients with a cleft lip have associ-
ated anomalies, suggesting possible underdetection. Among the
syndromes associated with CL/P are Van der Woude syndrome,
oral-facial-digital syndrome, branchio-oculo-facial syndrome,
and Opitz G/BBB syndrome. However, isolated CP is often (12–
54%) associated with an underlying syndrome, and as many as
47 to 72% of patients have associated anomalies. Among more
than 250 syndromes associated with CP are Stickler’s syndrome,
Fig. 15.2 Veau classification of cleft palate. (a)
Veau I: Soft palate cleft only. (b) Veau II: Soft and
hard palate cleft, posterior to the incisive fora-
men. (c) Veau III: Unilateral cleft lip and palate,
through alveolus, hard, and soft palate. (d) Veau
IV: Bilateral cleft lip, through the alveolus
bilaterally, hard palate, and soft palate.
Cleft Palate
157

22q11 deletion spectrum, Cornelia de Lange syndrome, Ema-
nuel syndrome, trisomy 13, Treacher Collins syndrome, and
Apert’s syndrome.
15.2 Diagnosis
Clefts of the lip and palate are increasingly diagnosed prena-
tally. Cleft lip is often noted on routine prenatal examination,
because the primary palate is well visualized on sonography
(Fig. 15.4a). Isolated CP is rarely diagnosed with sonography
but may be incidentally detected with fetal magnetic resonance
imaging (MRI) (Fig. 15.4b) performed as a part of the evalua-
tion of other congenital anomalies. In most cases, diagnosis of
CP is made by physical examination postnatally. As soon as the
diagnosis is made, the expectant mother or the newborn should
be referred to a cleft team for evaluation and counseling.
15.3 Nonoperative Management
The concept of team care is one of the most important charac-
teristics of modern cleft care. The American Cleft Palate-Cranio-
facial Association and Cleft Palate Foundation set standards for
designated cleft centers. A cleft team must include a speech and
language pathologist, a surgeon trained in cleft lip and palate
Fig. 15.3 Submucous cleft palate. (a) Intraoral view of an overt
submucous cleft palate demonstrating a bifid uvula and dark-colored
midline depression caused by muscular diastasis. (b) Nasal endoscopic
view of the same patient. With speech, a deep furrow is seen on the
nasal surface of the soft palate, also indicating muscular diastasis.
Fig. 15.4 Prenatal diagnosis of cleft lip and palate. (a) Fetal three-
dimensional sonography demonstrating a left unilateral incomplete
cleft lip. (b) Fetal magnetic resonance image demonstrating a Veau II
cleft palate. The fetal tongue rides high into the nasal cavity due to the
cleft hard and soft palate.
Cleft Lip and Palate
158

repair, and an orthodontist. In addition, to best serve the
unique needs of children with a cleft lip or palate, the team
should have access to practitioners in psychology, social work,
psychiatry, audiology, genetics, general and pediatric dentistry,
otolaryngology, and pediatrics or primary care.
Infants with CP often have feeding di culty early in life.
When a cleft palate is present, infants are not able to generate
suction due to the large oronasal fistula. Feeding instruction by
a speech and language pathologist or a nurse skilled in assess-
ing and feeding infants with cleft palate is initiated as soon as
the diagnosis is made, either prenatally or shortly after birth.
Infants with a cleft palate are best fed in semi-upright position,
using cleft feeders. No one feeding system is suited to every
infant. The two most commonly used at our center are Medela
SpecialNeeds (Haberman) Feeder and Dr. Brown’s Specialty
Feeding System. Other popular cleft bottles include the Mead
Johnson’s cleft lip/palate nurser and Pigeon cleft palate nipple.
The early management of infants with cleft palate is focused on
feeding, weight gain, and nutrition.
15.4 Operative Treatment
15.4.1 Timing of Surgery
Timing for palatoplasty varies widely between cleft centers.
Advocates for early repair cite improved speech development.
In one study, intervention at 6 months of age led to better
speech outcomes, with fewer compensatory articulations than
repair at 12 months of age. Another study correlated increasing
age at repair to velopharyngeal insu ciency (VPI) requiring
corrective surgery, with a major increase when intervention
was performed at 13 months of age or later. Because the maxilla
is the center of midfacial growth, advocates for later palato-
plasty are concerned that early palate manipulation substan-
tially inhibits midfacial growth. The surgeon must balance the
potential grow th restriction associated with early repair with
the poorer speech outcomes associated with late repair. For this
reason, a few centers perform hard and soft palate repair sepa-
rately. However, most surgeons (85%) perform cleft palate
repair in a single stage between 6 and 12 months of age. At our
center, palatoplasty is typically performed between 9 and 11
months of age.
Palate repair may be delayed if other medical issues are
present and not optimized, such as poor weight gain, cardiac
anomalies, and airway issues. Robin sequence (also called Pierre
Robin sequence or “syndrome”), defined by the triad of micro-
retrognathia, glossoptosis, and airway obstruction, deserves
special mention. Isolated CP is found in 69 to 90% of patients
with Robin sequence. Robin sequence is often associated with
an underlying diagnosis, most commonly Stickler syndrome,
which is present in one third to half of patients. Palatoplasty in
this subset should be approached cautiously. Airway problems
in the early postoperative period have been reported. Repair
should be delayed if the airway is not clearly secure.
15.4.2 Palatoplasty
The major goals of modern cleft palate repair are oronasal fis-
tula closure and speech optimization through proper muscula-
ture positioning. To close the hard palate, nasal lining is
elevated from the vomer medially and from the maxilla and
palatine bones laterally and is approximated to restore naso-
pharyngeal lining. Palatal mucosa is elevated in a subperiosteal
plane and is medialized. Von Langenbeck described elevating
the palatal mucosa as bipedicled flaps. The “two-flap palato-
plasty,” advocated by Bardach, employs a single pedicle based
on the greater palatine arteries. In both variations of hard palate
repair, a thin area of exposed palatal bone is left laterally to heal
by secondary intention. Soft palate repair is performed in three
layers: nasal mucosa, velar musculature, and palatal mucosa. In
a straight-line repair, the velar musculature is released from the
posterior hard palate, rotated posteromedially, and sutured at
the midline to create a muscular sling, the “intravelar velo-
plasty.” This technique is widely applicable to all variations of
cleft palate, regardless of width. An alternative is the Furlow
double-opposing Z-plasty, which both restores the velar sling
and lengthens the soft palate. At our institution, the Furlow
procedure is used for the initial repair of submucous cleft palate
and isolated clefts of the soft palate, as well as for secondary
lengthening procedures for VPI. These two techniques are
described in detail as follows.
Two-Flap Palatoplasty with Intravelar
Veloplasty
Preparation and Markings
The patient is positioned with neck in extension. A Dingman
mouth gag maintains retraction. The two-flap palatoplasty is
marked ( Fig. 15.5a, b). Local anesthetic with epinephrine is
injected into hard and soft palate mucosa for hemostasis and
analgesia.
Operative Technique
The soft palate mucosa is incised with a # 15 blade along the
junction of the nasal and oral mucosa, extending from hard pal-
ate to uvula. A # 12 blade may be used to complete the uvular
incision. The hard palate mucosa is incised laterally, at the bor-
der of the alveolus, and medially, at the junction of nasal and
oral mucosa. Hard palate mucoperiosteal flaps are elevated in
the subperiosteal plane ( Fig. 15.5c), taking care to preserve
the greater palatine arteries and dissecting circumferentially
around the vessels (Fig. 15.5d). For wide clefts, the arteries
may be cautiously skeletonized, and/or ostectomy at the greater
palatine foramen allows further medialization. An incision is
made down the vomer, and a periosteal elevator is used to ele-
vate just enough mucoperiosteum to close the nasal lining
(Fig. 15.5e). Attention is focused again to the soft palate. The
aggregates of the levator levi palatini and palatopharyngeus
muscles are sharply dissected, and their attachments to the
posterior hard palate are released (Fig. 15.5f).
Closure
Nasal lining is closed using 5–0 chromic suture and 4–0 Vicryl
suture, beginning anteriorly and progressing posteriorly
(Fig. 15.5g). Care is taken to evert the flaps and to avoid tear-
ing fragile tissue. The palatal muscles are approximated with
4–0 polydioxanone (PDS) suture in a figure-of-eight fashion.
Palatal mucoperiosteum and soft palate mucosa are closed
Cleft Palate
159

using 4–0 Vicryl simple interrupted sutures, beginning posteri-
orly at the uvula and progressing anteriorly. Mattress sutures
may be used when eversion is inadequate. The hard palate
mucoperiosteal flaps are loosely approximated to the alveolar
gingiva anteriorly ( Fig. 15.5h). Occasionally, a suture can be
placed through the soft bone of the hard palate to secure the
oral layer anteriorly, if needed.
Furlow’s Palatoplasty
Preparation and Markings
The oral mucosa is marked with a Z-plasty. The medial cleft
margin is marked. The patient’s left side is conventionally the
musculomucosal posteriorly rotating oral flap, and the right
side is the mucosal anteriorly rotating flap (Fig. 15.6a). The
nasal lining is incised in mirror image, with the musculomu-
cosal posteriorly rotating flap on the right and the mucosal
anteriorly rotating flap on the left. The incision on the left
extends from the junction of the hard and soft palate medially
toward the hamulus laterally, at an angle of ~ 60 degrees. The
right-sided oral mucosal flap is marked by joining the hamulus
laterally to the right side of the uvular base medially, at an angle
of ~ 70 to 80 degrees. We have been most successful waiting to
mark the exact placement of this incision until after the left
side has been cut and designing the flap based on how the tis-
sue best lies in a minimal-tension position. Local anesthetic
with epinephrine is injected into the hard and soft palate.
Operative Technique
The left-sided cleft margin is incised with a # 15 blade, followed
by the posteriorly rotating left oral musculomucosal flap. The
velar muscles are elevated with the flaps that rotate posteriorly,
oral on the left and nasal on the right, and ultimately, they over-
lap to create a muscular sling. The levator is sharply dissected
o the posterior edge of the hard palate, and the musculomu-
cosal flap is dissected o the nasal mucosal lining, leaving ad-
equate submucosa on that flap to preserve vascularity. The flap
is mobilized to the point of being inset with minimal tension
(Fig. 15.6b). The right-sided cleft margin and lateral limb are
incised. The anteriorly based mucosal flap is dissected in a sub-
mucosal plane, continuing anteriorly to the posterior edge of
the hard palate. Dissection along the edge of the hard palate is
performed with a Kramer elevator, freeing the nasal-sided
mucosa in this region. Next, the nasal flaps are incised in a mir-
ror image to the oral flaps, musculomucosal flap on the right
and mucosal flap on the left (Fig. 15.6c, d).
Closure
The uvula is approximated with 4–0 Vicryl sutures. The right-
sided musculomucosal flap is inset using 4–0 Vicryl suture, and
the left-sided mucosal flap is similarly inset (Fig. 15.6e). Next,
4–0 PDS sutures are used to approximate the musculus uvuli
and to overlap levator veli palatini muscles on each other. The
4–0 Vicryl sutures are used to inset the oral-lining z-flaps in a
similar fashion (Fig. 15.6f).
15.4.3 Postoperative Care
Cardiopulmonary monitoring is done routinely after palato-
plasty. Many surgeons use elbow immobilizers in the postope-
rative period; however, data supporting their use are lacking.
Postoperative diets vary among institutions. At our institution,
clear liquids or breast milk via a cleft feeder is allowed immedi-
ately postoperatively. A full-liquid, blenderized diet is initiated
on the first postoperative day, avoiding sharp utensils, straws,
crunchy food, or other items that are potentially injurious to
the repaired palate and is continued until postoperative day 10,
and then, a soft diet is continued until postoperative week 6.
Fig. 15.5 Two-flap palatoplasty (Bardach). (a) Palatal gingivoperiosteal flaps are marked out (blue lines) on either side, based on the greater palatine
arteries. The lateral marking is made at the junction of the alveolar gingiva and the palatal mucosa, and the medial marking is made at the junction of
the palatal and nasal mucosa. (b) The nasal–oral junction incision extends down the soft palate to the tips of the uvula. (c) The gingivoperiosteal flap is
elevated in a subperiosteal plane. (d) Circumferential dissection is performed around the greater palatine vessels (blue arrow). (e) Hard palate flaps are
elevated bilaterally. Next, vomerine mucosa is incised and elevated medially, and then, nasal lining is elevated laterally. (f) The velar muscles (*) are
dissected and posteromedially rotated. (g) Bilateral vomer and nasal lining flaps are approximated, followed by muscular repair. (h) The oral lining is
closed, and gingivoperiosteal flaps are tacked into place.
Cleft Lip and Palate
160

The patient is discharged when he or she is medically stable
and is taking enough liquid by mouth to stay adequately
hydrated, typically on the first or second postoperative day. Fol-
low-up varies by surgeon and cleft center. In the author’s prac-
tice, patients are typically evaluated 7 to 10 days and 6 weeks
postoperatively to assess the repair and help parents trouble-
shoot feeding issues. A full team evaluation with speech therapy
and dentistry is performed at 20 to 24 months of age and annu-
ally thereafter.
15.5 Complications
Acute complications of the cleft palate repair are extremely rare
but include dehiscence, bleeding, and airway-threatening
edema. Some surgeons leave a stitch in the tongue for retrac-
tion in case of an airway emergency. Chronic complications
include fistula formation (Fig. 15.7), VPI ( Fig. 15.8), maxil-
lary hypoplasia, and sleep apnea.
Incidence of palatal fistulae ranges from as low as 2 to 60%
and is reported to be more common with higher Veau classifi-
cation and repair at older age. Palatal fistulae occur at areas of
tension in the repair, with the hard palate–soft palate junction
being the most common site of unplanned fistulae. Minimizing
trauma during dissection, eversion of the suture line under
minimal tension, and good postoperative care are essential to
preventing fistulae.
Velopharyngeal insu ciency results from inadequate closure
of the velopharyngeal sphincter during speech, resulting in
nasal air escape. This may result from a short palate or inad-
equate muscular repair but often has no clear cause. Incidence
of VPI increases with greater Veau classification and increased
age at the time of palatoplasty. The association between palato-
plasty technique and incidence of VPI is unclear. With therapy,
many children will be able to achieve adequate speech, but
a small number of children require additional surgical
Fig. 15.6 Double-opposing Z-plasty (Furlow). (a) Oral-side rotation flaps are marked in blue in a patient with an overt submucous cleft palate. (b) The
palate has been split, and the left posteriorly rotating musculomucosal flap is elevated. (c) The nasal-side rotation flaps have been marked, roughly in a
mirror image of the oral flaps. (d) Musculomucosal flaps (*) and mucosal flaps (arrow) have been elevated. Note how the flaps orient themselves in
position when properly designed. (e) The nasal layer has been closed with the musculomucosal flap posteriorly and the mucosal flap anteriorly. (f) The
mirror-image oral flaps have been closed, similarly, with the musculomucosal flap rotated posteriorly and the mucosal flap rotated anteriorly.
Fig. 15.7 Palatal fistula. Slit-like fistula in the central hard palate.
Cleft Palate
161

procedures. These may include conversion to double-opposing
Z-plasty, sphincter pharyngoplasty, pharyngeal flap, and aug-
mentation of the posterior pharyngeal wall.
Children at the highest risk of developing sleep apnea are
those with syndromic diagnoses and Robin sequence. Other
complications of cleft palate repair detected late in childhood
include injury to tooth buds and maxillary hypoplasia. Maxil-
lary growth deficiency, thought to be related to periosteal
dissection, is most common in patients with bilateral cleft lip
and palate, and maxillary hypoplasia is treated by Le Fort I
advancement.
15.6 Conclusion
Successful palatoplasty hinges on separating the oro- and naso-
pharynx without fistula, achieving su cient velar length, and
creating a functional muscular sling. Successful comprehensive
cleft care entails preoperative optimization of feeding, aware-
ness and management of complications associated with palato-
plasty, and multidisciplinary postoperative care to achieve the
best functional outcome.
15.7 Key Points
● Cleft palate results from failure of fusion of facial prominences
early in embryologic development. There are genetic and
environmental influences. Isolated cleft palate is more likely
than cleft lip and palate to be associated with an underlying
syndrome.
● Cleft palate repair closes the oronasal fistula and establishes
continuity of the soft palate musculature to optimize speech
development.
● Secondary procedures may be required to address complica-
tions of surgery, such as poor speech and palatal fistulae.
Suggested Readings
Katzel EB, Basile P, Koltz PF, Marcus JR, Girotto JA. Current surgical practices
in cleft care: cleft palate repair techniques and postoperative care. Plast
Reconstr Surg. 2009; 124(3):899–906
Antony AK, Sloan GM. Airway obstruction following palatoplasty: analysis of
247 consecutive operations. Cleft Palate Craniofac J. 2002; 39(2):145–148
Kriens OB. An anatomical approach to veloplasty. Plast Reconstr Surg. 1969;
43(1):29–41
Salyer KE, Sng KW, Sperry EE. Two-flap palatoplasty: 20-year experience and
evolution of surgical technique. Plast Reconstr Surg. 2006; 118(1):193–204
Furlow LT , Jr. Cleft palate repair by double opposing Z-plasty. Plast Reconstr
Surg. 1986; 78(6):724–738
Smith DM, Losee JE. Cleft palate repair. Clin Plast Surg. 2014; 41(2):189–210
Sommerlad BC. Cleft palate repair. In: Losee JE, Kirschner KE, eds. Compre-
hensive Cleft Care. New York: McGraw Hill Medical; 2009:399–411
Sullivan SR, Marrinan EM, LaBrie RA, Rogers GF, Mulliken JB. Palatoplasty out-
comes in nonsyndromic patients with cleft palate: a 29-year assessment of
one surgeon’s experience. J Craniofac Surg. 2009; 20 Suppl 1:612–616
Ysunza A, Pamplona MC, Mendoza M, García-Velasco M, Aguilar MP, Guerrero
ME. Speech outcome and maxillary growth in patients with unilateral com-
plete cleft lip/palate operated on at 6 versus 12 months of age. Plast Reconstr
Surg. 1998; 102(3):675–679
Friede H, Enemark H. Long-term evidence for favorable midfacial growth after
delayed hard palate repair in UCLP patients. Cleft Palate Craniofac J. 2001; 38
(4):323–329
Fig. 15.8 Velopharyngeal insufficiency (VPI). (a) Nasal endoscopy
demonstrates nasal air escape during production of plosives in a
patient with mild VPI, as evidenced by air bubbles leaking at the left
lateral aspect of the palate. (b) Nasal endoscopy demonstrates a large
velopharyngeal gap in a patient with severe VPI.
Cleft Lip and Palate
162

16 Robin’s Sequence
Noopur Gangopadhyay and Kamlesh B. Patel
Sum m ary
Robin’s sequence is characterized by the triad of micrognathia,
glossoptosis, and airway obstruction; in 50% of cases, a cleft pal-
ate is associated with the sequence. The cleft of the secondary
palate is thought to arise from mechanical obstruction during
the elevation of the lateral palatine processes from a vertical to
a horizontal position. Conservative management of airway
obstruction should be attempted first, with prone positioning,
antireflux medications, and nasopharyngeal airway (NPA) as
the mainstays of treatment. Surgical management is required in
a small proportion of patients and should be preceded by air-
way evaluation by diagnostic endoscopy. Depending on the air-
way evaluation and relevant anatomy for each patient, tongue–
lip adhesion, mandibular distraction, or tracheostomy may be
required.
Keywords: Robin’s sequence, micrognathia, retrognathia, glos-
soptosis, airway obstruction, mandibular distraction, tongue–
lip adhesion
16.1 Introduction
Although initially described by St. Hilaire in 1822, the French
stomatologist Pierre Robin receives credit for defining this dis-
order in 1923. Robin wrote more than a dozen articles describ-
ing a disorder consisting of micrognathia and glossoptosis,
which resulted in airway obstruction and feeding di culties.
Unlike a syndrome in which multiple findings arise from a uni-
fying pathogenesis, Robin’s sequence arises from a chain of
events, in which one anomaly leads to the next. The micro-ret-
rognathia is thought to be due to an underlying genetic abnor-
mality or intrauterine growth restriction related to deformation
or an alteration in mandible position. This micrognathia then
leads to posterior displacement of the tongue, resulting in air-
way obstruction and poor oral intake. Approximately half of the
cases are associated with an isolated cleft palate, stemming
from mechanical obstruction by the tongue during the eleva-
tion of the lateral palatine processes from a vertical to a hori-
zontal position.
16.1.1 Epidemiology
The incidence of Robin’s sequence is reported as 1 in 8,500 to 1
in 14,000 live births, though the estimates vary between 1 in
5,000 and 1 in 50,000 births. There does not appear to be a gen-
der discrepancy. It usually presents in isolation and is referred
to as a nonsyndromic Robin’s sequence. A syndromic presenta-
tion occurs most commonly in association with Stickler’s syn-
drome and 22q deletion syndrome, though several other
syndromes share these findings. Mortality in the early half of
the 20th century was reported as high as 50%, largely due to
respiratory di culties and aspiration. With improvements in
diagnosis and treatment, mortality ranges from 1.8 to 30%, and
is usually related to the severity of airway distress.
16.1.2 Etiology
The cause of Robin’s sequence is unclear, and the majority of
studies point to a multifactorial etiology. The micro-retro-
gnathic mandible may be due to intrauterine growth restric-
tion, as may occur with a multigravid pregnancy or
oligohydramnios. External deformation results in constriction
of mandibular growth forward. The tongue is then retroposi-
tioned in the mouth and inhibits elevation of the lateral
palatine processes mechanically. Alternatively, delay of neuro-
muscular maturation can occur in the tongue and palate, lead-
ing to this sequence. Intrauterine exposure to ethanol and
hydantoin has also been implicated.
16.1.3 Genetics
A genetic basis is heavily supported by the high incidence of
twins with Robin’s sequence and a higher frequency of cleft lip
and palate in family members of patients with Robin’s sequence.
Deletions of 2q and 4p and duplications of 3p, 3q, 7q, 8q, 10p,
14q, 16p, and 22q are associated with cleft palate. Micrognathia
is linked to deletions in 4p, 4q, 6q, and 11q and duplications of
10q and 18q. Benko has reported on an autosomal dominant
locus of Robin’s sequence on chromosome 17q24.3–25.1, which
codes for regulatory elements of the SOX9 gene, a key protein in
chondrocyte di erentiation. Rainger describes loss-of-function
mutations in the SATB2 gene, which also results in micrognathia
and cleft palate in humans and in a mouse model.
Sonic hedgehog and Wnt signaling drive mandibular growth
from the first pharyngeal arch during embryogenesis. Absence
or downregulation of β-catenin reduces mandibular growth.
Inactivation of leukocyte-antigen related (LAR) family receptor
protein tyrosine phosphatase (RPTP) genes mimics the features
of Robin’s sequence in mouse model, resulting in aberrant jaw-
bone and cartilage and decrease in cell proliferation in the man-
dible. Missense mutations in DLX5 and DLX6 in a canine model
result in mandibular retrognathia and a cleft palate.
As early as 1978, Cohen recognized at least 18 syndromic
diagnoses associated with Robin’s sequence. This number has
grown to more than 40 syndromes. In a systematic review of
the literature, Izumi found that only 40% of cases were isolated
Robin’s sequence and that 60% of infants carried a syndromic
diagnosis. Stickler’s syndrome is the most commonly associ-
ated, with mutations in COL2A1, COL9A, COL11A1, or COL11A2,
resulting in abnormal type II or type XI collagen; these two
processes colocalize in 15 to 30% of patients. About 10% of
patients with Robin’s sequence are also a ected by 22q deletion
syndrome. This deletion in 22q11 leads to cleft palate, mandib-
ular retropositioning, immune dysfunction, and cardiothoracic
anomalies.
16.2 Diagnosis
The diagnosis of Robin’s sequence still relies on the classical
description from 1923—micrognathia, glossoptosis, and airway
Robin’s Sequence
163

obstruction. A cleft of the secondary palate is present in half of
the cases. A multidisciplinary approach for the workup and care
of these patients is required, with pediatric specialists in plastic
surgery, otolaryngology, neonatology, gastroenterology, pulmo-
nology, anesthesiology, speech pathology, and nursing all
involved in diagnosis and treatment planning.
16.2.1 Presentation
Prenatal diagnosis is quite rare because micrognathia is often
missed on a typical screening ultrasound. Micro-retrognathia is
evident at birth and is the initial feature that prompts explora-
tion for the diagnosis of Robin’s sequence. Mandibular hypopla-
sia is present in the vertical and horizontal directions, and
retrogenia—posterior displacement of the chin—is also notable.
The degree of glossoptosis—posterior displacement of the tongue
—is due to the structure and orientation of the mandible. The
tongue, while of normal size, takes up relatively more space in a
volumetrically smaller oral cavity. In addition, abnormal neuro-
muscular control of the tongue may lead to tongue prolapse.
Obstruction of the posterior pharynx by the tongue results in
occlusion of the airway during inspiration. This can lead to
repeated episodes of oxygen desaturation, apnea, and cyanosis.
Additional energy expenditure is required during breathing to
overcome these forces, with use of accessory muscles; supraster-
nal retractions are common during these desaturation events.
Furthermore, these episodes are not continuous given they may
not manifest while the infant is awake but are common during
sleep, while in the supine position, or during feeding. If untreated,
this may lead to hypoxia, respiratory failure, and death.
Eating is particularly challenging for these infants, given the
mechanics of breathing and feeding are in direct opposition.
This can result in gastroesophageal reflux and aspiration. In
addition, weight gain is poor because the energy required to
breathe and feed often outpaces the caloric intake that these
infants can acquire. Furthermore, in the population of infants
with Robin’s sequence who have a cleft palate, the inability to
generate nutritive suckling in a cleft is compounded by micro-
retrognathia and glossoptosis.
16.2.2 Adjunct Studies
Diagnostic fiberoptic endoscopy of the airway is critical to
determine optimum therapy. This is useful to determine the
level of airway obstruction, which may occur at the base of
tongue, epiglottis, vocal cords, or in the subglottic structures.
Laryngoscopy and bronchoscopy, if possible, should be per-
formed under anesthesia by an otolaryngologist, to evaluate for
laryngomalacia and tracheal stenosis as well as to assess for
vocal cord mobility and dynamic airway changes. It is critical to
perform these studies in di erent positions, given that prone
positioning may resolve airway obstruction related to the base
of tongue. In 1986, Sher described four types of airway obstruc-
tion in a cohort of 33 children, as noted on diagnostic endos-
copy. In type 1 and type 2 patterns, the dorsal tongue is
retropositioned, whereas type 3 and type 4 described lateral
pharyngeal wall motion and pharyngeal stenosis, respectively,
as the cause for airway obstruction. Sher’s schema was then
applied to infants with Robin’s sequence and has some predic-
tive and therapeutic value today. Sleep studies may also be
useful to evaluate for desaturation events during sleep, feeding,
or position change, when the clinical diagnosis is not clear. It is
not necessary to perform polysomnography routinely.
Cephalograms were used by Pruzansky to characterize man-
dibular shape and development in micrognathic patients; these
cephalograms also give a sense of the posterior airway space.
There is still controversy on whether the mandible stays persis-
tently hypoplastic or undergoes a period of accelerated “catch-
up” growth during the first few years of life. Airway obstruction
often improves during this period of mandibular growth. A
simple measurement of the distance between the maxilla
and mandible in the horizontal occlusal plane can be used
objectively to quantify the degree of mandibular hypoplasia.
Both three-dimensional computed tomographic (CT) scans and
three-dimensional surface photography are also useful in evalu-
ating micrognathia and planning for surgical treatment.
16.2.3 Syndromic Robin’s Sequence
Robin’s sequence can occur in conjunction with one of a multi-
tude of craniofacial anomalies in 60% of infants. Table 16.1
describes several of the familiar syndromes that are associated.
Stickler’s syndrome is the most commonly associated. This is
usually an autosomal dominant disorder, notable for short man-
dibular height, myopia, and concomitant retinal detachment,
hearing loss, and joint problems. An ophthalmologic examina-
tion is critical in infants presenting with myopia given that
most infants are hyperopic, and this points to the diagnosis of
Stickler’s syndrome. 22q11.2 deletion syndrome is distin-
guished by a retrognathic mandible; cleft palate, including sub-
mucous clefts; impaired immune system function; hypotonia;
and cardiothoracic malformations. Craniofacial microsomia and
Treacher Collins syndrome, with their dysplasia of the midface
and mandible, also occur with some frequency in patients
with Robin’s sequence. In several of these syndrom es, associ-
ated midface hypoplasia compounds the degree of airway
obstruction caused by glossoptosis. In these settings, a geneti-
cist may be helpful to guide future family planning and to assist
with expectations for long-term prognosis and development.
16.3 Nonoperative Management
Treatment of patients with Robin’s sequence is usually depend-
ent on the degree of airway obstruction. Conservative
Table 16.1 Common syndromic diagnoses associated with Robin’s se-
quence
Syndromes
22q deletion

Fragile X

TAR
Amniotic band
disruption
Kabuki

Treacher Collins
Beckwith–Wiedemann Marshall

Trisomy 13
Bruck

Moebius

Trisomy 18
CHARGE

Nager

Trisomy 21
Cornelia de Lange

Smith–Lemli–Opitz

VATER
Fetal alcohol

Stickler

Wolf–Hirschhorn
Cleft Lip and Palate
164

management is more e ective in nonsyndromic than in syn-
dromic patients with Robin’s sequence. The two critical features
are airway management and nutrition.
16.3.1 Airway Management
Prone positioning is the mainstay of therapy, relieving signs
and symptoms in up to 70% of patients. This is quite e ective
when airway obstruction is localized to the base of tongue,
given the prone position allows the tongue to fall forward. If
feeding is not compromised, often no other treatment is indi-
cated. This is successful in about half of infants with Robin’s
sequence. However, even these infants require rigorous obser-
vation because respiratory di culty, feeding problems, and fail-
ure to thrive can develop over the course of the first few
months of life.
More severe airway obstruction may require the placement
of an NPA and/or supplemental oxygen. The NPA acts to bypass
the site of upper airway obstruction given the distal end of the
NPA sits beyond the base of tongue. Placement can be con-
firmed by endoscopy via the NPA. This method can be individu-
alized for each infant, by modifying endotracheal tubes, chosen
based on the infant’s weight, as described by Chang. This allows
for the introduction of supplemental oxygen as well. The care
and cleaning of the NPA, as well as replacement, should be
demonstrated to the parents to allow for home management.
These adjuncts may be required for 2 to 4 months after dis-
charge from the hospital.
Laryngeal mask airway or endotracheal intubation is the last
resort to secure the airway in infants that do not respond to
prone positioning or NPA. However, this is a temporizing meas-
ure indicating more severe obstruction and pushes the clinician
toward surgical intervention for the patient.
16.3.2 Feeding
Even infants who do not exhibit respiratory di culties when at
rest may have airway distress while feeding. This is worsened if
there is an associated cleft palate. In addition, syndromic patients
may have underlying feeding di culty or neuromuscular issues
leading to di culty in swallow coordination and poor oral
intake. Increased metabolic rates require caloric supplementation
by gavage feedings via a nasogastric tube. This can decrease the
energy expenditure during feeding and facilitate appropriate
weight gain. In severe cases, a gastrostomy tube may be required
for feeding support. Evaluation by a speech pathologist and/or
clinical dietician is critical for feeding support.
Gastroesophageal reflux in these infants can lead to airway
inflammation, edema, and worsened airway obstruction.
Empiric treatment is useful to improve feeding and breathing.
In addition, sodium supplementation in infants has been e ec-
tive in some infants to reverse failure to thrive. A weight gain of
at least 20 to 30 grams per day is considered adequate.
16.4 Operative Management
16.4.1 Tongue–Lip Adhesion
Shukowsky was the first to describe tongue–lip adhesion (TLA)
in the early 20th century, but the technique was popularized by
Douglas 40 years later. Tongue–lip adhesion serves to correct
glossoptosis by pulling the base of the tongue forward and
securing it to the lower lip. This mucosal attachment serves as a
permanent tether, until the infant’s growth results in a more
stable airway. This must be done before the development of
mandibular dentition. A second surgery is required to release
the TLAwhen the airway is secure.
The GILLS scoring system, described by Rogers and Mulliken,
is one measure that can predict the patients with Robin’s
sequence who will be good candidates for TLA. Five factors—
gastroesophageal reflux disease (GERD), preoperative intuba-
tion, late presentation, low birth weight, and syndromic diag-
nosis—were determined to be significant in the success or
failure of TLA. Again, airway obstruction and subsequent man-
dibular growth remain important factors for the outcomes in
these patients, with TLA acting as a delaying type for the child
to outgrow the glossoptosis.
This technique is appropriate after imaging has ruled out
supraglottic and subglottic airway malformations. After naso-
tracheal intubation and supine positioning, a traction suture is
placed through the anterior tongue. Mirror-image rectangular
mucosal flaps are designed on the ventral tongue and lower lip
lingual surface. The incisions are infiltrated with local anes-
thetic with epinephrine. The genioglossus muscle should be
released subperiosteally from the lingual mandible via the ven-
tral tongue incision. A permanent suture is passed around the
mandible, through the lingual muscle, and buried across the
alveolar ridge, as the tongue is pulled forward with the traction
suture. The muscle and the mucosal flaps are then closed with
absorbable sutures. The traction suture is secured to the lower
lip and chin with adhesive strips; this can be pulled forward
postoperatively in the setting of desaturations ( Fig. 16.1). The
TLA is secured vertically to the external chin with two sutures
and a button to relieve tension as the mucosal flaps heal. The
traction stitch and button are removed 1 to 2 weeks postopera-
tively, and the TLA is taken down at 9 to 12 months of age.
This procedure is complicated by partial or complete dehis-
cence of the TLA, the need for reoperation, the need for a second
surgery for TLA release, soft tissue infection, abscess, base of
tongue scarring, and occasional conversion to tracheostomy. In
addition, data on feeding in this population are mixed, with some
cohorts experiencing worse outcomes due to altered tongue
mobility and swallowing and other cohorts showing increased
weight gain and a decreased need for nasogastric tube feeds.
Tongue–lip adhesion is appropriate for infants who fail prone
positioning and whose airway obstruction is limited to the base
of tongue. Success varies, with rates ranging from 36 to 100%.
Rogers and Mulliken showed that patients with a GILLS score of
2 or less have a high success rate with TLA, whereas patients
with a GILLS score of 3 or greater were five times more likely to
fail TLA; using this stratification, they have shown a 90% success
rate. However, patients with persistent symptoms and severe
respiratory distress have a high rate of conversion to a second
procedure such as mandibular distraction or tracheostomy in
the first year of life.
16.4.2 Mandibular Distraction
Mandibular distraction osteogenesis (MDO) was described and
popularized by McCarthy in 1989. It has become the mainstay
Robin’s Sequence
165

of surgical management for Robin’s sequence, given that it
addresses both airway obstruction and micro-retrognathia. The
mandible is lengthened in an anteroposterior vector, pulling
the base of tongue forward due to the lingual attachments to
the mandible. Mandibular distraction osteogenesis requires
bilateral mandibular osteotomies and placement of internal or
external distraction hardware with adjustable percutaneous
pins.
A preoperative CT scan can be advantageous to plan mandib-
ular osteotomies, based on the shape of the mandible, potential
tooth buds, and nerves. Denny describes the need for at least
17 mm of vertical height of the mandibular ramus to have ad-
equate space for the osteotomy and device placement. There is
significant variability in the orientation of mandibular osteoto-
mies and modifications in technique with regard to distraction.
Oblique osteotomies at the mandibular angle are quite com-
mon, whereas an inverted-L approach seems to minimize dam-
age to tooth buds. The deficiency in Robin’s sequence is in the
horizontal dimension, so a horizontal or slightly oblique vector
is preferred (though in cases associated with craniofacial micro-
somia, there are deficiencies in both dimensions).
The following technique, as described by Roy and Patel, illus-
trates a technique for MDO using internal distraction devices
(Roy and Patel 2006). Fiberoptic laryngoscopy is performed
before surgery and includes a jaw thrust to mimic MDO’s end
result. Bilaterally, the angle and inferior and posterior borders
of the mandible are marked on the skin, and the mandible is
approached 10 to 15 mm inferior to the border. Local anesthetic
with epinephrine is injected in the dermis. The subcutaneous
fat is swept superiorly and dissection proceeds deep to the
superficial layer of the deep cervical fascia to avoid the marginal
mandibular nerve. The pterygomasseteric sling is incised with
electrocautery to expose the buccal cortex, and subperiosteal
dissection frees the buccal and lingual surfaces of the mandible.
An inverted-L osteotomy is performed over the vertical ramus
of the mandible, at least 5 mm from the sigmoid notch. Internal
microdistractors are secured with at least three 1-mm screws.
Predrilling the holes is not required in infants. Activation of
the mandible is performed intraoperatively to check for
completeness of osteotomies and hardware function. The dis-
traction arm is brought through the skin posteriorly and infe-
rior to the ear. The skin is closed in layers with absorbable
suture.
The latency period can vary from 1 to 7 days, but most com-
monly, it is 2 to 3 days. Activation depends on patient’s age and
surgeon’s preference. Distraction rates are 0.5 to 2 mm per day,
spaced out in two to three intervals per day. The total amount
of mandibular advancement varies from patient to patient, but
the majority of craniofacial surgeons agree that overcorrection
in the horizontal vector is critical. Respiratory symptoms and
feeding are also evaluated as end points to completion of dis-
traction. The consolidation phase is 4 to 6 weeks after the com-
pletion of distraction ( Fig. 16.2). Radiographic evaluation
postoperatively is often obtained immediately after hardware
placement, during the activation phase, and after hardware
removal; delayed images are sometimes performed in follow-
up to assess for relapse and mandibular union. A second
procedure is required to remove the distraction hardware
(Fig. 16.3).
This procedure is complicated by pin site infections, unfavor-
able scarring, neuropraxia of the inferior alveolar nerve,
marginal mandibular nerve palsy, damage to tooth buds,
temporomandibular joint ankyloses, malocclusion, mandibular
malunion or nonunion, hardware failure, and failure to
resolve respiratory and feeding di culties. Several series have
described improvement in symptoms in up to 90% of patients,
with avoidance of tracheostomy and successful decannulation
of patients with tracheostomy in up to 70% of patients. Flores
described superior outcomes when comparing MDO with TLA
in nonsyndromic patients with Robin’s sequence, regarding
oxygen saturation and need for tracheostomy. In addition, there
appears to be significant cost savings in the first 3 years of life
for patients undergoing MDO as opposed to tracheostomy.
Mandibular distraction osteogenesis is appropriate for infants
who have failed prone positioning and conservative measures
and for those infants who would require a tracheostomy and/or
gastrostomy in the neonatal period. Again, this is not appropri-
ate for those infants who have subglottic airway anomalies.
Fig. 16.1 Postoperative appearance of patient
after TLA, with traction stitch and repair of
mucosal flaps shown.
Cleft Lip and Palate
166

16.4.3 Tracheostomy
Tracheostomy is often the last option for infants with Robin’s
sequence who fail conservative management and other surgical
interventions. It remains the gold standard for definitive airway
control and for those infants with subglottic stenosis,
tracheomalacia, or multilevel airway disease. The incidence of
conversion to tracheostomy in infants with Robin’s sequence is
higher in patients with an associated syndromic diagnosis.
This procedure is complicated by bleeding, airway obstruc-
tion, tracheitis, pneumomediastinum, granulation tissue at
the tracheal stoma, unfavorable scarring, unintentional
Fig. 16.2 Lateral radiograph showing device placement with distracted mandibular segments and clinical image of infant during the consolidation
period.
Fig. 16.3 Preoperative image and postactivation image of patient after mandibular distraction.
Robin’s Sequence
167

decannulation, tracheomalacia, subglottic stenosis, and fre-
quent hospitalizations.
16.5 Complications
Hypoxia, desaturations, asphyxia, and aspiration are common
without treatment in patients with Robin’s sequence. In addi-
tion, feeding di culties and recurrent respiratory infections are
associated morbidities in Robin’s sequence. Permanent brain
damage and sudden death can result for apneic episodes. Mor-
tality in patients with Robin’s sequence ranges from 1.7 to 65%.
Complications related to surgical technique are described
earlier.
16.6 Conclusion
Care of the patient with Robin’s sequence requires a multidisci-
plinary approach for diagnosis and treatment. Micrognathia,
glossoptosis, and airway obstruction are hallmarks of this dis-
ease process, but the degree of airway obstruction and feeding
di culty drive treatment and prognosis. Treatment ranges
from conservative measures such as prone positioning and
nutritional support to surgical interventions, including TLA,
MDO, and tracheostomy. The heterogeneity of presentations in
nonsyndromic and syndromic cases requires an individualized
approach to each infant with Robin’s sequence.
16.7 Key Points
● Robin’s sequence is a constellation of findings in which one
malformation leads to the next.
● It is classically described as glossoptosis, micrognathia, and
airway obstruction; a cleft palate is not necessary to make the
diagnosis.
● Respiratory di culties and feeding are important to address
optimal management.
● Conservative treatment remains the mainstay of therapy.
● Surgical management includes TLA, MDO, and, as the last
resort, tracheostomy.
Suggested Readings
Cladis F, Kumar A, Grunwaldt L, Otteson T, Ford M, Losee JE. Pierre Robin
sequence: a perioperative review. Anesth Analg. 2014; 119(2):400–412
Evans KN, Sie KC, Hopper RA, Glass RP, Hing AV, Cunningham ML. Robin
sequence: from diagnosis to development of an e ective management plan.
Pediatrics. 2011; 127(5):936–948
Flores RL, Tholpady SS, Sati S, et al. The surgical correction of Pierre Robin
sequence: mandibular distraction osteogenesis versus tongue-lip adhesion.
Plast Reconstr Surg. 2014; 133(6):1433–1439
Gangopadhyay N, Mendonca DA, Woo AS. Pierre robin sequence. Semin Plast
Surg. 2012; 26(2):76–82
Meyers AB, Zei MG, Denny AD. Imaging neonates and children with Pierre
Robin sequence before and after mandibular distraction osteogenesis: what
the craniofacial surgeon wants to know. Pediatr Radiol. 2015; 45(9):1392–
1402
Rogers GF, Murthy AS, LaBrie RA, Mulliken JB. The GILLS score: part I. Patient
selection for tongue-lip adhesion in Robin sequence. Plast Reconstr Surg.
2011; 128(1):243–251
Roy S, Patel PK. Mandibular lengthening in micrognathic infants with the
internal distraction device. Arch Facial Plast Surg. 2006; 8(1):60–64
Tahiri Y, Viezel-Mathieu A, Aldekhayel S, Lee J, Gilardino M. The e ectiveness
of mandibular distraction in improving airway obstruction in the pediatric
population. Plast Reconstr Surg. 2014; 133(3):352e–359e
Cleft Lip and Palate
168

17 Secondary Cleft Lip and Nasal Reconstruction
Michael Alperovich and Roberto L. Flores
Sum m ary
Secondary cleft lip and nasal deformities following primary cleft
surgery represent some of the greatest reconstructive chal-
lenges and have inspired a multitude of creative technical solu-
tions. Each secondary deformity is unique and must be
evaluated independently with reconstructive plans grounded in
principles rather than a formulaic approach. The number of
described techniques is vast with limited evidence-based
research to validate one method over another. However, techni-
ques that are reliable in the experience of the authors will be
presented to guide the plastic surgeon.
This review will discuss the important preoperative consider-
ations in cleft lip and nose repair. Key physical exam findings
will be presented. The philosophy of timing on secondary
reconstruction will be explained including what the authors
consider to be the major pitfalls in surgical approach.
Principles of secondary cleft lip reconstruction for unilateral
and bilateral cleft deformities will be discussed followed by
principles of secondary cleft nasal reconstruction of unilateral
and bilateral cleft deformities. While an exhaustive review is
impossible given the space limitations, this review will acquaint
the reader with the major challenges and the available techni-
ques in this field.
Keywords: cleft lip, cleft lip and palate, cleft, cleft rhinoplasty,
cleft nasal revision, cleft nose revision, cleft lip revision
17.1 Influence of Primary Cleft Lip
and Nasal Surgery
The first cleft operation is the best opportunity to achieve opti-
mal results. Any revisionary surgery will be compromised by
previously existing scars and violated tissue planes. When
results are suboptimal at the initial surgery, revisionary sur-
geries become more challenging. Therefore, it is imperative to
achieve the most aesthetic, functional, and lasting results at the
first operation.
Our center has benefited from the implementation of nasoal-
veolar molding (NAM) into our presurgical treatment plan.
NAM rotates the displaced alveolar segments into orthotopic
position, lengthens the columella, increases nasal tip projec-
tion, folds the lower lateral cartilage toward its native form,
medially transposes the lower lip and alar base, and stretches
the intranasal lining. Multiple studies have documented the
lasting benefit to nasal aesthetics following NAM and primary
cleft lip and nasal reconstruction. Patients with unilateral cleft
lip who undergo NAM demonstrate greater nasal symmetry
through age 9. Patients with bilateral cleft lip who undergo
NAM have a longer columella and near normal nasal morphol-
ogy through 12.5 years of age.
Presurgical orthopaedics facilitates gingivoperiosteoplasty
(GPP) and primary nasolabial fistula repair; however, NAM
requires frequent follow-up and diligent care on the part of
both the parents and the orthodontist. Alternative dentofacial
orthopaedics such as the Latham device are preferred by other
centers. As described by Millard, dentofacial orthopaedics
drives closer to completion the failed embryonic migration.
At the time of primary repair, exact intraoperative markings,
precise incisions, and gentle handling of the tissue are all crucial
for an optimal result. Cleft care delivered by high-volume cen-
ters with a multidisciplinary team approach is critical to main-
taining excellent results. Minimum guidelines for cleft teams
have been created jointly by the American Cleft Palate-Craniofa-
cial Association and Cleft Palate Foundation.
Reports by McComb and Salyer demonstrated the safety and
aesthetic advantage to primary cleft rhinoplasty. Long-term fol-
low-up supports the overall safety of primary rhinoplasty on
subsequent nasal growth. Although primary and revision cleft
rhinoplasty are standard practice, great variability exists in
approach. Plastic surgeons must remember that all primary
repairs must consider the final on-table result in the context of
long-term changes or the “fourth dimension” of time. Mulliken
and LaBrie have characterized the specific anthropometric
measurements that are altered in the fourth dimension. Cleft-
side alar base drifts laterally (sn-al distance) and should be
positioned more medially at cleft repair. Labial height (sn-cphi
distance) lags on the cleft side and should be slightly greater on
the cleft side at the time of repair. Finally, transverse labial
width (cphi-ch) grows more on the cleft side and should be set
shorter on the cleft side.
17.2 Timing of Secondary Cleft Lip
and Nasal Surgery
In this chapter, we define rhinoplasty as primary when per-
formed during the initial cleft lip repair, immature when per-
formed after primary rhinoplasty but prior to facial maturity,
and mature when performed at or after the time of facial
maturity. Limited secondary cleft lip revision and immature rhi-
noplasty can be performed as early as age 5 or 6 prior to the
child’s entry into school to reduce social stigma during peer
interactions. Early reconstruction is considered when patients
have significant deformity or are su ering socially in peer
interactions.
Because the nose grows until adolescence, rhinoplasty was
often deferred until the later teen years. Ortiz-Monasterio and
others have demonstrated excellent long-term follow-up even
when rhinoplasty was performed prior to age 12. However, at
this early age rhinoplasty precedes orthognathic surgery. Max-
illary advancement alters the anthropometric dimensions of
the nose, increases tip projection and nasolabial angle, and
restores aesthetic balance to the face. As facial proportions are
evolving and future rhinoplasty may still be required, the
authors caution against comprehensive nasal surgery prior to
facial maturity. Furthermore, patients prior to skeletal maturity
may not have the cognitive maturity to verbalize their goals
and concerns in immature rhinoplasty. At the time of facial
maturity, patients become willing participants in their own
care.
Secondary Cleft Lip and Nasal Reconstruction
169

The great enemy of the refined soft-tissue repair is scar.
Therefore, primary cleft rhinoplasty should achieve an aesthetic
and lasting change using the least amount of dissection. The
ideal is one rhinoplasty surgery during primary cleft lip repair
and a second at the time of facial maturity. Active cleft surgeons
are familiar with the scarred nasal tip resulting from excessive
operations that resists the most elaborate technical maneuvers.
The underoperated nose can be predictably improved although
perfection is elusive. The overoperated, scarred nose can pro-
duce unfortunate, complex, and irreversible hurdles to an
already challenging operation which, in many cases, can be
avoided with proper planning, and an appreciation of the larger
picture. Ultimately, the surgical philosophy of the authors is to
e ect the greatest change with the fewest number of surgeries
and new scars. Diligence at the primary cleft lip and nose repair
can reduce the number of surgeries required and avoid school-
age interventions.
17.3 Secondary Correction of
Unilateral Cleft Lip Deformities
Treatment of unilateral cleft lip deformities varies depending
on the etiology of the deformity. Obtaining operative reports
when the patient had the repair elsewhere may help with sur-
gical planning, but in the authors’ experience these reports add
limited value compared to a good history and physical exam.
Deficiencies can occur at each segment of the repair. Techniques
will be characterized by vermilion deficiency, white roll
misalignment, and a vertically short lip. When the deformity is
significant, revising the entire cleft lip repair is the better
intervention.
17.3.1 Vermilion Deficiency
Vermilion deficiency can result from insu cient attention dur-
ing reconstructive planning of a primary lip repair, inadequate
or incomplete repair of the orbicularis oris, or wound break-
down in the area of the vermilion. The two main categories of
vermilion deficiency are loss of the vertical height of the ver-
milion and misalignment of the red line separating the dry
from the wet vermilion. In Asians, African Americans, and
Latino patients, discrepancies of the red line can be quite
noticeable and therefore should be addressed at the time of
lip repair. A modification of Noordho ’s triangular flap can
restore the vertical height of the vermilion and reposition the
red line.
During reconstruction, attention should be directed to careful
repair of the orbicularis oris. Incomplete muscle repairs can
result in loss of vertical height of the upper lip and vermillion.
As scars in the vermilion tend to heal well, there should be a
low threshold for excision of the a ected vermilion and upper
lip as part of the reconstructive plan.
For minor deficiencies isolated to the vermilion, fat grafting
to the lip is a useful and minimally invasive technique that can
be performed in the o ce. Overcorrection is preferred with an
expectation that some fat will resorb, even in a well-vascular-
ized bed such as the lip. Although the technique is reproducible,
patients should be aware of the possible need for repeat sur-
gery, particularly in the scarred vermilion.
Dermal grafting to the deficient vermilion also can restore
volume. The most common criticism of dermal grafting is firm-
ness and poor integration with the surrounding lip architecture.
Local mucosal flaps have limited application outside mild
deformities as deficiencies in this area are rarely isolated to the
mucosa alone.
17.3.2 White Roll Misalignment
Misalignments of the white roll as small as 1 mm are noticeable
at conversational distance. The step-o can be corrected with
asymmetric Z-plasty or a diamond-shaped excision. Asymmet-
ric Z-plasty can e ectively align the two borders of the vermi-
lion, but introduces a new oblique scar along nonanatomic
landmarks of the upper lip. Patients with a predisposition for
poor scarring are at risk for additional morbidity.
Diamond-shaped excision e ectively lowers the white roll
border to provide an upper lip height match in small discrepan-
cies. As the incision line is hidden within the scar line of the
previous lip repair, no new scars are formed. For this reason,
the diamond excision technique is favored by the authors to
correct minor discrepancies of the white roll.
White roll discrepancies of greater than 2 mm require re-cre-
ating the cleft deformity and repairing the cleft de novo. In
these cases, the lip should be treated as a vertically short lip.
17.3.3 Vertically Short Lip
The vertically short lip can result from a planning error, techni-
cal error, or poor scar formation. Poor scar formation is related
to patient biology and unlikely to be a ected by revisionary
surgery. Because poor scarring can haunt the cleft surgeon,
careful physical examination should evaluate the presence of
scar in other parts of the body or cleft repair. Poor upper lip
scars share features of widening, placement lateral to the phil-
tral line, and have wide areas of white roll disruption.
When there is a vertical discrepancy of greater than 2 mm
between the cleft and noncleft philtral columns or when scars
are wide and prominent, a cleft lip re-repair is advocated in the
form of either a rotation-advancement or a straight-line repair.
Up to 25% of the upper lip can be excised without causing a
disturbance in lip dynamics. Every e ort should be made to
completely excise all scarred areas of skin and muscle, restore
vertical height of the lip, and locate the final scar along the phil-
tral line.
Widened and prominent scars of the lip skin commonly over-
lie wide scars of the orbicularis oris. Incomplete muscular
repair at the time of cleft repair may result in bulging of the
a ected upper lip with animation or vertical shortening. The
surgeon should have a low threshold for resection of scar tissue
in the orbicularis oris and complete reconstruction of the verti-
cal height of the muscle.
17.4 Secondary Correction of
Bilateral Cleft Lip Deformities
The secondary deformities a ecting the unilateral cleft lip can
occur in the bilateral cleft lip. However, the anatomic derange-
ment is more complicated, as are the surgical techniques
Cleft Lip and Palate
170

required for correction. Due to space limitations, deformities
related to alveolar clefts, displacement of the premaxilla, and
oronasal fistulae will not be covered in this review. A malposi-
tioned maxilla can have profound e ects on nasal and upper lip
form. The underlying bony foundation should be addressed
prior to definitive lip or nose surgery.
Common bilateral cleft lip deformities include excessive scar-
ring, a vertically short upper lip, and discontinuity of the white
roll. Bilateral cleft lip repair requires the formation of two paral-
lel, closely apposed incisions. Therefore, patients predisposed to
excessive scarring will be at risk for suboptimal results even in
the best of hands.
Some bilateral cleft lip repair techniques do not adequately
reconstruct the entire orbicularis oris. In some cases, the
muscle is not reconstructed at all. Absence of an intact oral
sphincter interferes with creation of an appropriate prolabial
width, adequate vertical height of the upper lip, and alar base
position. The absence of orbicularis oris continuity is high-
lighted during puckering or whistling.
The midline tubercle of the vermillion should be assessed for
presence and fullness. The height and width of the prolabium
and the quality, location and area of the philtral scars are scruti-
nized and documented. Manchester reconstructions of the mid-
line tubercle are antiquated, unaesthetic techniques that should
be abandoned.
If enough unscarred philtrum exists, a bilateral cleft lip re-
repair can adequately address the secondary deformities of
the bilateral cleft upper lip. Provided that there is adequate
tissue to create a columella-based philtral flap, surrounding
scar a ecting the upper lip skin, muscle, and mucosa is com-
pletely resected. If the lower lip is protrusive after orthog-
nathic correction, the patient may be better served by an
Abbe flap.
During bilateral cleft lip re-repair, vermillion turndown flaps
are created from the lateral lip elements similar to primary lip
repair. Care should be taken to reconstruct the entire length of
the orbicularis oris at the midline. Wide dissection across the
face of the maxilla may be necessary to relieve tension at the
midline. Through this controlled reconstruction, vertical height
of the upper lip and vermilion tubercle can be restored and
unaesthetic scars replaced. Surgeons must anticipate additional
widening of the prolabium with time. Philtral width is set nar-
rower than desired to allow for stretching of skin with healing
and activation of the orbicularis oris muscle. As will be dis-
cussed later, the forked flap advocated by Millard for primary
bilateral cleft lip repair has no role in the modern techniques in
cleft nasal reconstruction.
An Abbe flap can restore composite deficiencies in skin,
muscle, vermilion, and mucosa to the midline upper lip in cases
of excessively scarred or short philtrum (Fig. 17.1). The Abbe
flap serves to both augment the upper lip and reduce the lower
lip volume, thereby restoring aesthetic balance to the mouth.
The authors’ preference is to perform the Abbe flap following
definitive orthognathic surgery and secondary cleft rhinoplasty
revision except in cases of severe distortion of the upper lip.
The preference of the authors is to extend the Abbe flap onto
the columella to avoid a depressed scar at the lip–columella
junction. The fibrofatty tissue at the base of the prolabium is
rolled onto itself superiorly to provide bulk and take the place
of an absent anterior nasal spine.
The Abbe flap is designed narrower than needed for the same
reason that the prolabial segment is created narrower at the
initial bilateral cleft lip repair. Care is taken to prevent any ver-
milion misalignment of either the upper or lower lip during flap
inset and donor site closure. Loosely tied wires can be placed
between the maxillary and mandibular teeth if there is any
Fig. 17.1 Abbe flap for correcting deficient upper lip volume in a bilateral cleft lip deformity.
Secondary Cleft Lip and Nasal Reconstruction
171

concern regarding patient compliance. However, given this pro-
cedure is typically performed in patients who are at least ado-
lescents, there should be active involvement and investment on
the part of the patient. Flap division is performed in an o ce
setting one to two weeks later. The preference of the authors is
to perform an Abbe flap in isolation without any other concom-
itant adjustments to preserve the integrity of the final result.
However, other centers advocate combining the Abbe flap with
a secondary rhinoplasty in the same stage.
As in unilateral deformities, fat and dermal grafting may be
utilized for minor vermilion deficiencies that exist in isolation
of other upper lip deformities. Similarly, V-Y advancement can
be used to lengthen a deficient mucosa or vermilion although
with limited e cacy.
17.5 Secondary Correction of
Unilateral Cleft Nasal Deformities
Secondary reconstruction of unilateral cleft nasal deformity has
evolved over the past several decades. Despite the significant
variability in approach, most surgeons use the same set of key
techniques. Mature cleft rhinoplasty usually is one of the last
interventions for the cleft patient following maxillary advance-
ment and alveolar bone grafting. A description of the unilateral
cleft nasal deformity followed by commonly used techniques
will be presented.
17.5.1 Unilateral Cleft Nasal Deformity
Anatomy
The unilateral cleft nasal deformity is characterized by a defi-
cient maxilla on the cleft side resulting in the cleft side alar base
displaced posterolaterally and inferiorly. In a key historical
study evaluating the anatomy of fetuses with cleft lip, the lower
lateral cartilage of the cleft-sided nose was found to have dis-
torted rather than deficient tissue. The cleft side lower lateral
cartilage has a shortened medial crus and longer lateral crus.
The angle between the medial and lateral crura on the cleft side
is increased. The cleft-sided upper and lower lateral cartilages
lack typical overlap. The nasal dome on the cleft side is retrodis-
placed and less well-projected secondary to deficiencies in the
maxilla. The nasal floor is also caudal on the cleft side and fre-
quently absent. In addition, the lower lateral cartilage and alar
rim create hooding. The ala is oriented posteriorly, laterally, and
inferiorly on the cleft side relative to the noncleft side. The
cleft-side nostril may buckle due to webbing of the vestibular
lining. The shorter columella and caudal septum are deviated
toward the noncleft side, while the bony septum is deviated to
the cleft side. The nasal tip has a resulting asymmetry. Finally,
the inferior turbinate on the cleft side often is hypertrophic.
17.5.2 Goals of Cleft Nasal Deformity
Correction
The correction of the cleft nasal deformity in adults is generally
less satisfactory than the cleft lip deformity. The pathological
anatomy and reliable techniques are better characterized in the
cleft lip compared to the nose. Furthermore, the lip repair is a
largely two-dimensional dilemma that follows an ablative
approach to reconstruction; unacceptable and scarred lip is
completely excised and fresh tissue is borrowed from laterally
(or inferiorly in the bilateral cleft lip). The cleft nasal deformity
is a three-dimensional dilemma where previous surgeries
present cumulative obstacles in the form of scar and disrupted
cartilage. There are virtually no options to replace scarred skin
or mucosa from surrounding structures. Therefore, the most
e ective means of optimizing surgical results in the adult cleft
rhinoplasty is to avoid the creation of excessive scar by overop-
erating on the nose during development.
The functional impact on the nasal airway is an often over-
looked component of secondary rhinoplasty. Sixty percent of
patients with cleft lip nasal deformity have some di culty
breathing. The obstruction is related to external nasal deform-
ity, septal deviation, vomerine spurs, inferior turbinate hyper-
trophy, and maxillary hypoplasia. Secondary procedures such
as pharyngeal flaps or sphincter pharyngoplasties also impact
on airway resistance. Deviation of the bony septum can be an
underappreciated contributor to nasal airway obstruction. The
vomer is frequently deviated into the nasal airway on the cleft
side and the perpendicular plate of the ethmoid can cause air-
way stenosis in the posterior recess of the nose. After submu-
cous resection of the cartilaginous septum, additional resection
of the bony septum may be required to restore airflow through
the nose.
Multiple studies have developed assessment tools to evaluate
outcomes following secondary cleft nasal deformity repair, but
review of individual results remains largely subjective. The best
study to evaluate impact on quality of life used analogue scales
preoperatively and 1 year following secondary cleft rhinoplasty
to measure psychological distress and generic quality of life.
Interestingly, there was no correlation between subjective pa-
tient reported outcomes and surgeons’ scores.
17.5.3 Evaluation
Surgical planning for immature and mature cleft rhinoplasty is
dependent on an appropriately directed history and physical
exam. History of all previous nasal surgery including previous
rhinoplasty and septoplasty surgery is noted. Intranasal exam
evaluates the presence of the cartilaginous and bony septum,
septal deviation and dislocation, condition of the vomer and
perpendicular plate, and the size of the inferior turbinate bilat-
erally. Airflow is assessed in both nasal airways with and with-
out a Cottle maneuver. The skin and nostril rim are examined
and palpated to assess the quality of the scar and the integrity
of the lower lateral cartilages.
Visual inspection of the nose follows principles similar to tra-
ditional rhinoplasty. In the unilateral cleft rhinoplasty, dorsal
deviation of the lower, middle, and upper third of the nose is
closely scrutinized on frontal view. Lower lateral cartilage posi-
tion and definition are noted as nasal tip cartilages are com-
monly widened due to inferior displacement and rotation of
the cleft side lower lateral cartilage.
The nasal tip light reflex and the gullwing pattern of the
lower border of the nose can expose asymmetries in the lower
lateral cartilage position. The alar bases are compared for verti-
cal and horizontal position. The lateral view of the nose is
assessed on the right and left side as deficiency at the pyriform
Cleft Lip and Palate
172

aperture, tip projection, tip rotation, nasolabial angle. and col-
umellar lobular angle can di er between the cleft and noncleft
side.
In the basilar view, lower lateral cartilage position and devia-
tion are inspected. Medial footplate position, columellar length,
deviation of the columella and caudal septum, and nostril apex
collapse are evaluated. The alar base position on the horizontal
and anterior/posterior planes is noted, and the nasal floor is
inspected for depression.
17.5.4 Techniques in Unilateral Cleft
Nasal Deformity
The great majority of mature cleft rhinoplasty surgery is a sec-
ondary rhinoplasty involving extensive shaping of the nasal tip
cartilages. As a result, an open technique is used in virtually all
cases. Scar is the greatest impediment to successful manipula-
tion of the nose in the mature cleft rhinoplasty. Cleft surgeons
should avoid overoperating on the nose. Repeated “touch-up”
surgery to the nose in the form of immature rhinoplasty will
doom the patient (and surgeon) to a nose with fibrotic cartilage
encased in an envelope of scar that permeates the nasal skin
and mucosa and resists any meaningful manipulation. In order
to combat the force of scar, particularly at the nasal tip, many
surgeons advocate the use of costal cartilage grafts during rhi-
noplasty. In the authors’ experience, septal cartilage provides
adequate rigidity in the majority of mature cleft rhinoplasty
reconstructions.
Cutting grouped cleft rhinoplasty into bone, cartilage, and
skin envelope techniques. Deviation of the upper third of the
nose can be addressed with simple infracture and outfracture
techniques. In certain cases, the perpendicular plate of the eth-
moid is severely deviated, resulting in a tilt of the dorsal line of
the bony vault. In these cases, the authors favor the monobloc
osteotomy first described by Blair and Brown ( Fig. 17.2).
The technique requires a thorough resection of the deviated
cartilaginous and bony septum. Low to low lateral osteotomies
are employed followed by percutaneous osteotomy across the
radix, leaving an intact bony bridge on the side opposite the
deviation. Firm, laterally directed pressure rotates the nasal unit
as a single segment toward the midline. If the caudal septum is
dislocated from the anterior nasal spine, it is separated,
trimmed as needed, and secured to the midline with suture.
The monobloc osteotomy can address the bony dorsum as well
as deviations a ecting the middle vault.
Deviation of the middle vault is addressed using spreader
grafts. In cases of severe deviation, upper lateral cartilages are
completely separated from the septum, and the entire middle
vault is reconstructed to relocate the dorsal septum to the mid-
line. Spreader grafts may be extended as a combined columellar
strut to concurrently reinforce lower lateral cartilage position.
The emphasis of lower third nasal reconstruction is reposi-
tioning of the lower lateral cartilages. Columellar strut grafts
are used often to reinforce the lower lateral cartilage frame-
work. A floppy cleft-side lower lateral cartilage and scar within
the skin and mucosa leave the lower third of the nose vulner-
able to postoperative collapse. Although cartilage cap grafts
commonly are used to camouflage nasal tip asymmetries, they
will only exacerbate the deformity without rigid support from a
columellar strut graft. Cartilage repositioning and reinforce-
ment should be performed prior to any framework
augmentation.
For mild asymmetries, the nasal tip may only require dissec-
tion of the lower lateral cartilages followed by suture reinforce-
ment and a columellar strut graft. The tail of the cleft-side
lateral crura commonly has fibrous attachments to the pyriform
rim. These attachments should be completely excised prior to
repositioning the lower lateral cartilage. For more severe asym-
metries, composite rotation flaps from laterally or inferiorly can
recruit cartilage toward the midline.
In cases where the alar base position is normal, lower lateral
cartilage is recruited medially using a V-Y advancement flap
first described by Potter. Intracartilaginous and intercartilagi-
nous incisions are made on either side of the cleft-side lateral
crura merging in a “V” at the lateral limit of the cartilage
(Fig. 17.3). The composite flap of cartilage and mucosa is
advanced medially to restore tip projection followed by a V to Y
closure of the defect.
Fig. 17.2 Monobloc nasal osteotomy used to rotate the nasal bones and correct the upper third secondary cleft nasal deformity.
Secondary Cleft Lip and Nasal Reconstruction
173

A group recently reported their experience with a modified
V-Y chondromucosal composite flap similar to the Potter proce-
dure, but which also included additional sutures and occasional
use of a columellar strut graft. A shortcoming of the Potter tech-
nique is the cosmetic depression sometimes created by the
transection of lateral ligamentous attachments.
In cases where the cleft-side alar base is in a lateral position
and the nasal floor is depressed, the Dibbell procedure is used
to recruit cartilage superiorly ( Fig. 17.3). Intercartilaginous
and infracartilaginous incisions are extended medially as a
hemimembranous incision with a skin incision anterior to the
medial crura. These parallel incisions then extend medially just
anterior and posterior to the cutaneous portions of the nostril
floor and end laterally at the alar base.
This bipedicle composite flap is rotated superiorly, resulting
in a medially transposed alar base, obliterated nostril floor
depression, lengthened columella, and increased tip projection.
This powerful technique should be combined with a columellar
strut graft to reinforce the newly formed cartilaginous frame-
work. The authors have found that excessive medial transposi-
tion of the alar base is possible with the Dibbell technique, and
contralateral alar base resection may be required to restore
nasal symmetry.
The typical skin envelope deformity in unilateral cleft nasal
deformity includes nostril apex overhang, an absent soft trian-
gle and a shortened columella. The Tajima reverse-U incision is
a useful technique for tailoring and redraping the skin envelope
after repositioning of the lower lateral cartilages (Fig. 17.4).
The incision is made inside the nose at the junction of the col-
umella and membranous septum. The reverse-U incision is
made around the superior border of the nostril rim to reflect
the height and shape of the contralateral side. Wide subcutane-
ous undermining followed by suturing of the cleft lower lateral
cartilage to the cleft and noncleft upper lateral cartilages as well
as noncleft lower lateral cartilage is performed through a closed
rhinoplasty incision. Finally, the reverse-U flap is thinned,
reflected inferiorly, tucked within the nose, and inset to recre-
ate the soft triangle. Although the Tajima technique uniquely
increases the aperture of the nostril, precise markings are crit-
ical to success. An oversized flap will raid the nasal tip and rim,
creating a contour deformity that is di cult to correct.
The authors prefer to use the Tajima technique during imma-
ture rhinoplasty and combine the Tajima with an open rhino-
plasty during mature rhinoplasty. The open approach provides
better visualization of the lower lateral cartilages and exposure
for a columellar strut graft. The Tajima/open rhinoplasty can
also be combined with a Potter or a Dibbell technique depend-
ing on the individual patient needs ( Fig. 17.4). Through the
combination of di erent incisions and techniques, a customized
approach to the lower third of the nose is used for each patient.
This versatile approach is optimized through progressive expe-
rience with each of the listed procedures as a stand-alone ope-
ration and in combination with other techniques.
The micronostril is one of the most challenging deformities
associated with the cleft nose. A result of excessive medializa-
tion of the alar base, lower lateral cartilage collapse, and exces-
sive intranasal scarring, the micronostril lacks a reliable
solution. Avoiding this deformity with a properly executed pri-
mary cleft rhinoplasty is the best treatment.
As lateral repositioning of the ala is di cult to perform with-
out creating unaesthetic scars, the authors have relied more on
camouflage techniques to correct the micronostril deformity.
These techniques include a Tajima procedure to increase the
aperture of the nostril on the a ected side, alar base resection
on the contralateral side, and repositioning of the columella
toward the contralateral side.
17.6 Secondary Correction of
Bilateral Cleft Nasal Deformities
The challenges of bilateral cleft nasal deformity correction lie in
the restoration of tip projection, tip refinement, and increasing
columellar length. Surgical correction is similarly limited by the
force of scar upon the cartilaginous framework. Although the
struggle of restoring three-dimensional symmetry to the nose
is not typically present, the bilateral lack of tip projection, rota-
tion, and definition require more extensive repositioning of the
cartilage framework than the unilateral cleft nasal deformity.
Given the decreased relative incidence of this phenotype, lim-
ited data exist regarding the techniques of repair. Following an
explanation of the bilateral cleft nasal deformity, the principles
in bilateral cleft nasal reconstruction will be discussed.
Fig. 17.3 The (a) Potter and (b) Dibbell procedures to correct the position of the cleft lower lateral cartilage in secondary unilateral cleft nasal
deformity.
Cleft Lip and Palate
174

17.6.1 Bilateral Cleft Nasal Deformity
Anatomy
The bilateral cleft nasal deformity is characterized by a widened
inter-alar distance, shortened columella, lack of tip projection,
and a broad and shallow tip. The prolabium can appear attached
to the nasal tip. Similar to the cleft side in a unilateral cleft nasal
deformity, the medial crura are shortened, while the lateral
crura are longer with a hypoplastic maxilla bilaterally. The angle
between medial and lateral crura is increased bilaterally.
The alae are displaced posteriorly, inferiorly, and laterally,
and are flared in an S-shape. The vestibular lining of the alae
has webbing bilaterally and the nasal floor may be notched. The
caudal septum and anterior nasal spine are inferiorly displaced
with fibrous attachments to the premaxilla. The septum is typi-
cally midline in the anterior aspect, but septal deviation can be
present in the bilateral cleft lip patient.
17.6.2 Techniques in Bilateral Cleft
Nasal Deformity
Bilateral cleft nasal deformity techniques seek to lengthen the
columella, improve nasal tip projection, and narrow the alar
bases. Cronin advocated columella lengthening by using skin
from the nasal floor and ala. Forked flaps were popular for
lengthening the columella, but resulted in a rectangular and
long columella, enlarged nostrils, and a sharp columellar–labial
angle. These skin-based reconstruction techniques create exces-
sive cutaneous scarring, do not restore the anatomic form or
relationships to the cartilaginous framework, and, therefore,
should be abandoned.
Modern techniques in bilateral cleft lip rhinoplasty empha-
size alteration and reinforcement of the cartilaginous frame-
work to restore nasal form. Nostril rim incisions, advocated by
Mulliken, during primary rhinoplasty can be incorporated in
immature rhinoplasty, particularly when nose surgery was not
performed during primary lip repair. Tajima incisions may be
used at this time although the authors caution the reader
regarding the risks of overexcision of the nostril rim skin. At the
time of mature cleft rhinoplasty, an open rhinoplasty approach
is advocated with the use of Tajima incisions and Potter flaps as
needed. As tip support is a challenge in bilateral clefts, columel-
lar struts are obtained from septal or costal cartilage. Cutting
favored an L-shaped septal cartilage graft as a spreader graft
termed a “spreader-strut” to provide added projection. Onlay
grafts to the tip can be applied in addition for volume.
17.7 Key Points
● The primary cleft repair is the best opportunity to achieve
optimal results with any secondary surgery compromised by
previously existing scars and violated tissue planes.
Fig. 17.4 The Tajima reverse-U (left) and cutting open rhinoplasty with combination Dibbell/Tajima (right) procedures to address the cartilage and
soft-tissue orientations in secondary unilateral cleft nasal deformity.
Secondary Cleft Lip and Nasal Reconstruction
175

● Limited secondary cleft lip and nasal revision can be per-
formed prior to the child’s entry into school to reduce social
stigma during peer interactions.
● In the presence of significant vertical lip height discrepancy
or wide and prominent scarring, the cleft surgeon should not
hesitate to perform a cleft lip re-repair.
● Rhinoplasty ideally occurs once at primary repair and again
at facial maturity following any planned orthognathic sur-
gery. The guiding principle is doing the most in the fewest
number of surgeries.
● The majority of secondary cleft rhinoplasty surgery is per-
formed using an open technique as it involves extensive shap-
ing of the nasal tip cartilages.
Suggested Readings
Cutting CB, Warren SM. Extended Abbe flap for secondary correction of the
bilateral cleft lip. J Craniofac Surg. 2013; 24(1):75–78
Cutting CB. Secondary cleft lip nasal reconstruction: state of the art. Cleft Pal-
ate Craniofac J. 2000; 37(6):538–541
Dibbell DG. Cleft lip nasal reconstruction: correcting the classic unilateral
defect. Plast Reconstr Surg. 1982; 69(2):264–271
Flores RL, Sailon AM, Cutting CB. A novel cleft rhinoplasty procedure combin-
ing an open rhinoplasty with the Dibbell and Tajima techniques: a 10-year
review. Plast Reconstr Surg. 2009; 124(6):2041–2047
Henry C, Samson T, Mackay D. Evidence-based medicine: the cleft lip nasal
deformity. Plast Reconstr Surg. 2014; 133(5):1276–1288
Potter J. Some nasal tip deformities due to alar cartilage abnormalities. Plast
Reconstr Surg (1946). 1954; 13(5):358–366
Tajima S, Maruyama M. Reverse-U incision for secondary repair of cleft lip
nose. Plast Reconstr Surg. 1977; 60(2):256–261
Cleft Lip and Palate
176

18 Velopharyngeal Dysfunction
Jenny T. Chen, Maia N. Braden, Joseph Lopez, Timothy W. King, Anand Kumar, and Howard Wang
Sum m ary
Velopharyngeal dysfunction (VPD) describes the improper clo-
sure of the velopharyngeal valve leading to abnormal speech
production including hypernasality and nasal air emission. VPD
can be divided based on its underlying etiology into velophar-
yngeal incompetence (abnormal neurophysiology and move-
ment), velopharyngeal insu ciency (VPI; abnormal anatomy),
or velopharyngeal mislearning (articulation error). VPI fre-
quently results from overt cleft palates, submucous cleft palates,
or large palatal fistulas of the hard palate. Nonoperative ma-
nagement of VPI includes speech therapy and prosthetics such
as palatal lifts and palatopharyngeal obturators. However, the
primary treatment of VPI is surgical.
Keywords: velopharyngeal insu ciency, velopharyngeal dys-
function, velopharyngeal incompetence, velopharyngeal mis-
learning, sphincter pharyngoplasty, pharyngeal flap, Furlow
double-opposing Z-palatoplasty, radical intravelar veloplasty
18.1 Introduction
The velopharynx is composed of the soft palate, or velum (from
Latin which means veil), and pharynx. The velopharynx is made
of five muscles: the levator veli palatine (LVP), the tensor veli
palatine (TVP), the palatoglossus, the palatopharyngeus, and
the musculus uvulae. The superior pharyngeal constrictors and
the salpingopharyngeus contribute to pharyngeal wall move-
ment. The pharyngeal plexus (9th and 10th cranial nerves (CN
IX and CN X)) innervates all of these muscles, except for the
TVP, which is innervated by CN V ( Table 18.1).
Closure of the velopharynx separates the oral and nasal
cavities during both speech and swallowing. Opening of the
velopharynx is required for the consonants “m/n/ng.” Closure
of the velopharynx is needed for the normal production of all
other sounds in the English language. During closure of the
velopharynx, the palate is pulled superiorly and posteriorly by
the LVP and makes contact with the posterior pharyngeal wall
at a condensation of the superior pharyngeal constrictors
known as Passavant’s ridge. Normal speech requires the rapid
movement of the velopharynx in order to generate both oral
and nasal sounds.
Velopharyngeal dysfunction (VPD) in individuals with cleft
palate can result in audible stigmata, with hypernasal speech,
nasal air emissions, and inappropriate compensatory articula-
tion errors. The incidence of VPD after palatoplasty has been
reported from 5 to 40%. The impact of VPD is profound, and has
the potential to a ect social function and self-image.
Although the most common cause of VPD is anatomic/struc-
tural abnormalities, any pathology that results in abnormal
velopharyngeal function can cause VPD. Previous classification
of VPD was filled with confusion. The etiology of VPD was cate-
gorized as anatomic, iatrogenic, or neurogenic. VPD was further
divided into whether it resulted from structural, functional,
mechanical, or dynamic palatal dysfunction. More recently,
VPD is now classified into three major diagnostic types:
velopharyngeal incompetence (abnormal neurophysiology),
velopharyngeal insu ciency (VPI; abnormal anatomy), or velo-
pharyngeal mislearning (articulation errors; Table 18.2).
18.1.1 Velopharyngeal Incompetence
(Abnormal Neurophysiology)
This type of VPD is given to velopharyngeal pathology of
unknown origin or pathology secondary to abnormal
Table 18.1 Muscles of the velopharynx
Muscle

Origin

Insertion

Innervation

Function
Levator veli palatini

Cranial base (petrous tem-
poral bone)
Crosses the middle third of
the soft palate fusing with
the contralateral muscle to
form the levator sling
CN IX–CN X

Superior and posterior ele-
vation of the palate
Tensor veli palatine

Membranous wall of the
eustachian tube
Pterygoid hamulus

CN V

Opens the eustachian tube
Palatoglossus

Tongue

Anterior velum

CN X

Depresses palate
Palatopharyngeus

Posterior and lateral pha-
ryngeal walls
Velum via posterior tonsillar
pillar
CN X

Lateral wall motion
Musculus uvulae

Posterior velum

Mucous membrane of the
uvula
CN X

Upward movement and
shortening of the uvula
Superior pharyngeal con-
strictors
Median raphe (posterior
pharyngeal midline)
Medial pterygoid and the
pterygomandibular raphe
CN X

Lateral wall motion
Salpingopharyngeus

Cartilaginous eustachian
tube
Blends with the palatophar-
yngeus and constrictor fi-
bers
CN X

Elevates palate
Abbreviations: CN IX, ninth cranial nerve; CN X, 10th cranial nerve.
Velopharyngeal Dysfunction
177

neurophysiology. It is important to note that in velopharyng-
eal incompetence, no underlying anatom ical abnorm ality is
present. The neurophysiological abnorm alities associated
w ith velopharyngeal incompetence can be caused by under-
lying intracranial processes, acute cerebrovascular injuries,
palatal paralysis, or neurological injuries to the pharyngeal
plexus. Velopharyngeal incompetence m ay also be associ-
ated w ith congenital neurologic conditions such as m uscu-
lar dystrophy, hem ifacial m acrosom ia, or cerebral palsy. The
clinical m anifestation of velopharyngeal incom petence is
sim ilar to other types of VPD but also presents w ith weak-
ness, paralysis, or incomplete coordination of the velum
and pharyngeal m usculature, resulting in hypotonia, dys-
arthria, and loss of protective reflexes (i.e., gagging and
swallow ing).
In addition to neurophysiological abnorm alities m entioned
above, VPD can also present in patients with m otor speech
disorders, or apraxia. Since voluntary action of the velophar-
yngeal valve is mediated by coordinated signals from the
m otor cortex to the velopharyngeal m usculature, it is not
surprising that patients w ith apraxia can display abnorm al
coordination of the velopharynx during speech sound
production.
18.1.2 Velopharyngeal Insu ciency
(Anatomical Abnormalities)
The second major type of VPD is VPI. This categorization is
given to VPD cases that are the result of structural or anatomi-
cal velopharyngeal pathology. VPI more commonly results from
congenital abnormalities such as cleft palates, submucous clefts,
or palatal fistulas ( Fig. 18.1). These congenital conditions are
associated with gross tissue deficiency at the level of the velum
and pharyngeal walls, and therefore limit velopharyngeal valve
competence. Additionally, since velopharyngeal closure is
largely a function of the ratio of the pharyngeal depth to palatal
length, patients with disproportionally shorter palates or long
pharynxes may also demonstrate incomplete closure of the
velopharyngeal valve. Not only can VPI result from gross tissue
deficiencies, or abnormal structural proportions, but it can also
arise from congenital soft-tissue disturbances that produce
mechanical dysfunction of the velopharyngeal valve. For exam-
ple, hypertrophied tonsils can mechanically interfere with clo-
sure of the velopharyngeal valve and therefore cause VPI.
Lastly, VPI can stem from iatrogenic causes such as gross
tissue deficiency secondary to tumor resection, trauma recon-
struction, or adenoidectomy. Since removal of hyperplastic
Fig. 18.1 Velopharyngeal anatomy. A figure demonstrating the significant differences in the normal velar anatomy compared to the cleft palate. Note
the vertical position of both the levator and the tensor veli palatini muscles. The levator veli palatini muscle must be properly separated from the
tensor muscle and transposed posteriorly into a more normal position to produce a more effective velar closing mechanism.
Table 18.2 Types of velopharyngeal dysfunction
Type of velopharyngeal dysfunction

Definition

Examples
Velopharyngeal insufficiency

An anatomic or structural defect that prevents
velopharyngeal valve closure
Cleft palate, submucous cleft, deep pharynx,
adenoid abnormalities, hypertrophic tonsils
Velopharyngeal incompetency

A neurophysiological disorder in which poor
movement of the velopharyngeal structures
results in incomplete velopharyngeal valve clo-
sure
Neurogenic injury, hypotonia, apraxia, cerebral
palsy, global developmental delay
Velopharyngeal mislearning

Articulation errors secondary to inappropriate
opening of the velopharyngeal valve during
attempts to produce oral speech phonemes
Lack of auditory feedback, compensatory speech
production, developmental delay
Cleft Lip and Palate
178

adenoids is a common treatment for children with airway
obstruction or chronic otitis media, adenoidectomy can com-
monly cause VPI. Conversely, procedures that increase the soft-
tissue bulk of the velopharynx can also cause VPI. For instance,
excessive soft tissue at the nasopharynx level as a result of a
wide pharyngeal flap may limit apposition of the velum to the
pharyngeal walls.
18.1.3 Velopharyngeal Mislearning
Patients with this type of pathology appear to have normal ana-
tomical, neurogenic, and physiological velopharyngeal valve
competency. However, these patients exhibit phoneme-specific
nasal emissions; oral consonants are replaced by nasal airflow.
Commonly, these patients display nasal emissions with only a
select set of sounds such as CH, SH, Z, and S.
18.2 Diagnosis
Evaluation of VPD is necessary in order to identify the degree of
dysfunction, the reason for the dysfunction, and to determine a
course of appropriate treatment. Evaluation should be com-
pleted by a multidisciplinary team including a speech-language
pathologist with training, knowledge and experience specific to
the evaluation and treatment of VPD. Evaluation includes audi-
tory-perceptual and instrumental assessments.
18.2.1 Perceptual Evaluation
Resonance
Resonance must be evaluated in speech. Resonance is a phe-
nomenon that can only be perceived on vowels and voiced con-
sonants. It is based on the amount of energy present in the oral
cavity and/or nasal cavity. Hypernasality consists of too much
energy in the nasal cavity on sounds that are not supposed to
resonate in the nasal cavity. Hyponasality consists of too little
acoustic energy resonating in the nasal cavity on sounds such
as “m,” “n,” and “ng,” which are supposed to resonate in the
nasal cavity. Cul-de-sac resonance, sometimes called “hot
potato voice” is due to acoustic energy becoming trapped in the
pharynx, sometimes due to large tonsils. Ideally, evaluators will
be able to obtain a combination of structured and unstructured
speech tasks. These should include a connected speech sample,
sustained vowels, syllable strings of oral consonants and di er-
ent vowels, syllable strings of nasal consonants and vowels, and
words and sentences specifically useful in identifying a reso-
nance disorder. Di erent rating scales of hypernasality and hypo-
nasality are used, both clinically and in research. In a clinical
setting, most speech-language pathologists use an equal-appear-
ing interval scale, rating hypernasality on a scale of 0 to 4, or 0
to 5 of absent, mild, moderate, severe, and profound. Visual ana-
log scales, direct magnitude evaluation, and a visual rate and sort
method have all been used in the literature, and may be more
sensitive and reliable than the equal-appearing interval method.
Audible Nasal Emissions
Audible nasal emissions are perceived when air escapes
through the nose on a high-pressure consonant sound such as
“p” or “s.” They are due to incomplete closure of the velophar-
yngeal port during these high-pressure sounds, and the sound
is thought to be due to turbulence either at the velopharyngeal
port or in the nasal passages.
Articulation Errors
The speech pathology evaluation for VPD should always include
an evaluation of articulation. This will identify speech sound
errors that are developmental and unrelated to the VPD, but
will also identify any obligatory or compensatory errors related
to VPD. Individuals with VPD can have speech sound errors.
These may be obligatory errors, such as nasalization of conso-
nant sounds due to incomplete velopharyngeal closure. People
may also develop compensatory errors, such as glottal stop
substitutions, pharyngeal fricatives, and nasal fricatives, in an
attempt to approximate normal speech sounds in the absence
of a functioning velopharyngeal mechanism.
18.2.2 Instrumental Evaluation
Instrumental evaluation can be divided into indirect measures
and direct measures. Indirect measures evaluate the speech
output. Some examples of these include nasometry (Pentax),
which measures the ratio of nasal acoustic energy to total
acoustic output, and gives a percentage known as nasalance.
This is a method of quantifying hypernasality or hyponasality.
Pressure/flow measures are also used; these involve the place-
ment of a mask and tube on the speaker’s mouth and nose, to
measure the airflow and air pressure from the mouth and
nose during speech tasks. Other indirect measures used have
included nasal accelerometer spectrography. Nasometry is the
most widely used clinical measure.
18.2.3 Visualization
If perceptual and instrumental measures indicate that there is
VPD, visualization of the velopharynx may be necessary in
order to determine treatment. This is usually done by naso-
pharyngoscopy, but multiview fluoroscopy (MVF) is also widely
used.
Nasopharyngoscopy
Nasopharyngoscopy is performed by the speech-language path-
ologist or physician. Topical lidocaine and oxymetazaline are
often used for comfort. The procedure is not painful, and is gen-
erally very well tolerated. Very young children may be upset.
Careful preparation and a calm demeanor are key to patient
comfort and getting a good examination. The scope should be
introduced to the child in a calm and age-appropriate manner,
giving them plenty of time to become comfortable. In some
clinics, the child is given a coloring book and other preparatory
materials prior to the visit. Young children should be seated in
their parents’ lap, with someone available to steady their head
as needed. Patient participation is extremely important, given
we are evaluating the velopharyngeal mechanism during
speech; some speech is necessary. The examiner should pass
the scope through the nasal passage; passing through the mid-
dle meatus gives the best view. Once in place, and the VP port
Velopharyngeal Dysfunction
179

can be fully visualized, the patient is asked to repeat a series of
syllables, words, and sentences including oral and nasal sounds.
Closure is observed during these speech sounds. This might
reveal a submucous cleft palate, or incomplete closure for other
reasons (Fig. 18.2). Careful observation of closure pattern is
necessary to determine the appropriate surgical intervention.
Multiview Fluoroscopy
MVF can also be used to evaluate velopharyngeal closure. This
requires radiation exposure. If this is done, the child is posi-
tioned in the radiology suite and asked to repeat the speech
stimuli in three positions: lateral, frontal, and base (Towne’s)
views. This technique gives a three-dimensional view of the
velopharyngeal port, can provide actual measurements, and
does not require insertion of a tube (camera). However, it does
require a very cooperative patient, the study can take a long
time as it requires each view to be taken independently, is more
expensive than nasopharyngoscopy, and is most commonly
only available in the hospital.
Functional Magnetic Resonance Imaging
Functional magnetic resonance imaging (fMRI) is emerging as a
technology for visualizing velopharyngeal structure and func-
tion. With fMRI, imaging of the velopharynx is performed in
four planes: sagittal, velopharyngeal axial (aligned perpendicu-
lar to the “knee” of the velum), axial, and coronal. Palate motion
is shown using three-dimensional dynamic volumes created
from the four views. Real-time visualization of velopharyngeal
anatomy during its entire range of motion is possible. This tech-
nique, like MVF, also requires a very cooperative patient, is even
more expensive than MVF, and is currently only available a few
specialized centers. It does, however, eliminate the exposure of
the patient to ionizing radiation.
18.3 Nonoperative Management
18.3.1 Behavioral Speech Therapy
The speech-language-pathologist plays an important role in
not only the assessment but also the management of patients
with VPD. Behavioral speech therapy may be the primary treat-
ment for patients with velopharyngeal incompetence or velo-
pharyngeal mislearning. Speech therapy is also an important
adjunct to other treatment modalities for VPI and is often used
in combination with surgical intervention.
18.3.2 Prosthetic Devices
Prosthetic devices are an alternative to surgical management. It
may be used temporarily or serve as a permanent treatment
option for patients who are not surgical candidates, such as
those with sleep apnea or neuromuscular conditions. Several
studies have shown similar speech outcomes with prosthetic
devices as surgical management. Palatal lifts and palatophar-
yngeal obturators are the commonly used options. Each device
is custom-made by the prosthodontist for the individual patient
and is anchored to the maxillary dentition. Palatal lifts displaces
the velums superiorly to assist with velopharyngeal closure and
is usually indicated in cases of neuromuscular dysfunction
without anatomic deficit. In contrast, palatopharyngeal obtura-
tors have a posterior extension that occupies space in the velo-
pharyngeal gap and may be used to augment closure of the
velopharyngeal gap. The main advantages of prosthetic devices
are avoidance of the complications associated with surgery and
reduced cost. However, long-term use of prosthetics is compli-
cated by noncompliance, need for frequent device adjustments,
and emotional distress associated with wearing the prosthesis.
18.4 Operative Management
Surgical intervention is the primary treatment modality for
patients with VPI due to an underlying anatomic abnormality
such as those with a previous history of cleft palate repair.
Commonly used surgical options for the treatment of VPI
include radical intravelar veloplasty, Furlow double-opposing
Z-palatoplasty, posterior pharyngeal flap, and dynamic sphinc-
ter pharyngoplasty (Table 18.3). Posterior pharyngeal wall
augmentations have also been used to a lesser extent. Several
clinical trials have attempted to compare outcomes of the dif-
ferent surgical approaches, but no significant di erences have
been identified. Each operation aims to improve velopharyng-
eal competence through a di erent mechanism. Thus, the
choice of the operation should be selected based on the
patient’s pattern of velopharyngeal closure and the size of the
remaining pharyngeal gap.
Velopharyngeal closure patterns can be classified as coronal,
sagittal, circular, or bow tie. Pharyngeal flaps and sphincter
pharyngoplasty both aim to decrease the size of the velophar-
yngeal port. Pharyngeal flaps bring tissue to the central portion
of the velopharynx and thus are best utilized to correct central
gaps such as with cases of sagittal or circular patterns of clo-
sure. Sphincter pharyngoplasty mobilizes tissue from the lateral
aspects into the central portion and thus can correct lateral
defects from coronal and bowtie closure patterns. Furlow pala-
toplasty lengthens the soft palate and tightens/repositions the
levator sling. Radical intravelar veloplasty also tightens and
repositions the levator sling. Both have been shown to be suc-
cessful for small and medium central gaps less than 2 cm. Pha-
ryngeal augmentation is also used at times to address small
central defects less than 5 mm. Large “black hole” deformities
are the most challenging to address, and strategies to do so
include using static obturator, sphincter pharyngoplasty alone,
Fig. 18.2 Instrumentation view. Nasal endoscopic view demonstrating
the central gap in the soft palate associated with submucous cleft
palate (SP), the velopharyngeal gap (VPG), and the posterior pharyn-
geal wall (PPW). (These images are provided courtesy of open access
frontiers in Pediatrics, Front. Pediatr., August 11, 2014.
org/10.3389/fped.2014.00084.)
Cleft Lip and Palate
180

large posterior pharyngeal flap, or combining Furlow palato-
plasty with sphincter pharyngoplasty. The varied treatment
strategies for the large gap VPI is enigmatic and represents a
current treatment challenge.
18.4.1 Radical Intravelar Veloplasty—
Palatoplasty
Radical intravelar veloplasty is a well-described and useful
treatment to treat the palate and optimize function from abnor-
mal levator muscle position seen in submucous cleft palate and
undertreated prior palate repair. This is incised in the midline
from the junction of the hard/soft palate to the uvula with care
to preserve the muscularis uvulae muscle fibers ( Fig. 18.3a,
b). The oral mucosa is elevated to the lateral palate shelves in
order to visualize the entire levator veli palatini muscles, the
tensor tympani muscles, and the tensor tympani aponeurosis
bilaterally ( Fig. 18.3c, d). The tensor tympani aponeuroses is
then transected and the abnormally positioned levator veli
palatini muscles are then elevated from the nasal lining along
with the fibers of the pharyngeal sphincters. The levator veli
palatini muscles are then transposed to the midline and poste-
riorly at the level of the uvula and the central nonmuscular
excess area is resected. The levator muscles, after they have
been transposed posteriorly into a horizontal configuration, are
overlapped and sutured together with the levator muscles
under moderate tension ( Fig. 18.3e). The palate mucosa is
then closed after hemostasis is achieved (Fig. 18.3f).
18.4.2 Furlow Double-Opposing
Z-Palatoplasty
Furlow double-opposing Z-palatoplasty is a versatile technique
that leads to lengthening of the velum and repositioning of the
fibers of the LVP into a transverse orientation, which enhances
palatal mobility. The velum lengthening comes at the expense
of decreased palatal width, making a tighter velopharyngeal
port. The operation is performed by first making a midline inci-
sion along the soft palate and creating two Z-plasty flaps on the
oral mucosa side of the velum. A posteriorly based oromuscular
flap on one side and an oral mucosa–only flap on the opposite
side are elevated. During this dissection, care should be taken
to avoid injury to the greater palatine arteries that supply the
LVP muscles. After dissecting the oral flaps, a second set of nasal
mucosa Z-plasty flaps are elevated. This time with one nasal
mucosa–only flap on the side of the oromuscular flap and a
nasomuscular flap on the side of the previously raised oral
mucosa–only flap. The double Z-plasty flaps are then trans-
posed and sutured in layers. A distinct advantage of the Furlow
palatoplasty or radical intravelar veloplasty technique is that all
other surgical options such as pharyngeal flap or pharyngo-
plasty remain available and may be used as an adjunct or sal-
vage treatment for persistent VPI with this technique.
18.4.3 Posterior Pharyngeal Flap
The goal of the pharyngeal flap is to create a wall that connects
the soft palate to the posterior pharynx, reducing the airflow
through the velopharyngeal port. Nasal airway is maintained by
the two lateral openings on either side of the flap, which
implies that adequate lateral wall motion is critical to the suc-
cess of the pharyngeal flap. First, a superiorly based myomu-
cosal flap including the superior pharyngeal constrictor muscle
is raised just superficial to the prevertebral fascia. Then, inci-
sions are made at the nasal and the oral surface of the velum
just posterior the junction of the hard and soft palate, and the
two incisions are connected to create a pocket. The pharyngeal
flap is then passed through the pocket in the soft palate and
inset into the oral mucosa ( Fig. 18.4a). Primary closure of the
donor site should be attempted. To maintain patency and con-
trol the size of the lateral ports, small endotracheal tubes or red
rubber catheters are often inserted through each naris to main-
tain adequate airflow (Fig. 18.4b). The soft palate triangular
flaps are then inset to allow for complete closure and lining of
the flap. Ideally, the pharyngeal flap should be inset at the level
of the posterior nasal spine (PNS) parallel and in plane with the
maxillary palatine plane defined by anterior nasal spine (ANS)
to PNS to posterior pharyngeal wall ( Fig. 18.4c).
18.4.4 Sphincter Pharyngoplasty
Unlike posterior pharyngeal flap, sphincter pharyngoplasty cre-
ates an extension to the lateral and posterior pharyngeal wall.
Table 18.3 Surgical treatment of velopharyngeal insu ciency
Operative techniques

Anatomic effects

Indications
Furlow double-opposing Z-palatoplasty

● Lengthens the velum
● Reorients levator veli palatini into a transverse
orientation
● Small to medium central gaps
● Primary and revision cleft palate repair
Radical intravelar veloplasty (IVV)

● Lengthens the velum
● Reorients levator veli palatini into a transverse
orientation
● Small to medium central gaps
● Primary and revision cleft palate repair
Posterior pharyngeal flap

● Reduces the size of the airway with a static
wall of mucosa from the velum to the posterior
pharynx
● Large central gaps
● Revision cleft palate repair
● Requires adequate lateral wall motion
Sphincter pharyngoplasty

● Decreases velopharyngeal port size by exten-
sion of the lateral and posterior pharyngeal
wall
● Small to medium central gaps
● Poor lateral wall motion
● Requires adequate function of the levator veli
palatini muscle
Velopharyngeal Dysfunction
181

Thus, adequate function of the LVP muscle to close the central
port during speech production is important for the success of
this operation. The operation is performed by elevating bilateral
superiorly based myomucosal flaps of the palatopharyngeus
muscle. The flaps are then pivoted medially as high as possible
on the posterior pharynx. Achieving high inset leads to
improvements in speech outcomes but may be impeded by the
presence of prominent adenoid pad. In such cases, partial or
complete resection of the adenoid pad may be considered prior
to sphincter pharyngoplasty. A transverse incision is made on
the posterior pharynx where the two flaps are then sutured
together either end to end or overlapped and inset onto the
posterior pharyngeal wall. Primary closure of the donor site
should be performed to decrease risk for cicatricial contracture,
which can lead to inferior displacement of the myomucosal
flaps. A suggested advantage of sphincter pharyngoplasty is
that the sphincter formed by bilateral palatopharyngeus
muscles may have some dynamic function, allowing it to make
adjustments during speech to avoid hypernasality and hypona-
sality that may result from imperfectly sized flaps. However,
these claims have not been clearly proven.
18.4.5 Posterior Pharyngeal Wall
Augmentation
Posterior pharyngeal wall augmentation through the injection
of a variety of alloplastic or autologous material have been
described. The goal of these techniques is to bring the pharyn-
geal wall closer to the velum to aid closure during speech. Most
techniques have shown inconsistent success and carry risks for
numerous complications including infection, extrusion, migra-
tion, and even embolism. With the increasing popularity of fat
Fig. 18.3 Radical Intravelar veloplasty–palatoplasty. (a) Intraoperative view demonstrating a cleft of the hard and soft palate. (b) Standard markings
for Bardach hard palate flaps and soft palate exposure. (c) Intraoperative view demonstrating elevation of the hard and soft palate flaps prior to
closure. (d) Intraoperative view demonstrating closure of the nasal layer. Note the anterior position of the levator muscles after closure, in essence the
creation of a submucous cleft palate. (e) Intraoperative view demonstrating proper repositioning of the levator muscles into a horizontal position in a
proper radical intravelar veloplasty (IVV) procedure. The tip of the Gerald forceps is located at the junction of the lateral and vertically placed tensor
veli palatini and the horizontally placed levator veli palatini muscles. (f) Intraoperative view demonstrating final appearance of the repaired cleft palate
after closure of the hard palate and oral layer of the soft palate.
Cleft Lip and Palate
182