549
Operative techniques
distractions such as scheduled meetings, patients waiting in
the office, and other obligations.
The technical details for repair of a bilateral complete cleft
lip and nasal deformity are given below, followed by modifi
cations used for the major anatomic variants of bilateral cleft
lip. The reader will note that descriptions of the procedures
do not follow the usual sequence of marking, dissection, and
closure. Instead, the operative steps are portrayed as they
proceed, which often requires turning attention from the lip
to the nose, then back to the lip again, and ending with the
final touches to the nose. Some leeway in the sequence of the
operation is possible, but in general this is not advisable until
considerable experience is gained in these procedures.
Bilateral cleft, complete cleft lip, and palate
Markings
A sharpened tooth pick is used for drawing and the ink is
brilliant green dye (tincture) rather than methylene blue dye
(aqueous). The anatomic points are designated using the
standard anthropometric initialisms (see Fig. 22.3).6 With the
nostrils held upward with a double-ball retractor, the philtral
midfacial retrusion.52,53 Others have failed to document such
an effect beyond that expected inhibition of vertical and
forward maxillary growth in children with repaired bilateral
cleft lip/palate followed through the age of the mixed denti
tion.54 There is also recent controversy about a possible delete
rious effect of alveolar gingivoperiosteoplasty.
Traditional treatment protocols place undue emphasis on
efforts to minimize inhibition of midfacial growth. Some
degree of maxillary retrusion is an unavoidable consequence
of closure of the cleft lip and cleft palate or both. The first
priorities should be nasolabial appearance and speech. Mid
facial retrusion is predictably corrected by maxillary advance
ment with the additional aesthetic benefits to nasal, labial, and
malar projection.
Operative techniques
The day of repair of a bilateral cleft lip and correction of the
nasal deformity is the most important day in the child’s life.
It should be the first case in the morning, and probably the
only operation for that day. The surgeon must work slowly
and deliberately, taking as much time as necessary, without
Fig. 22.2 (A) Latham appliance; (B) prior to insertion of device; (C) 6 weeks
following dentofacial orthopedic manipulation.
A
DB
DC
550
SECTION I
CHAPTER 22 • Repair of bilateral cleft lip
labial elements are disjoined from the alar bases, and the
basilar flaps are freed from the piriform attachments by inci
sion along the lower section of the vestibular cutaneo-mucosal
junction.
The mucosal incisions are extended distal along the gingi
volabial sulcus to the premolar region. With a double hook on
the muscular layer, the lateral labial elements are widely dis
sected off the maxillae in the supraperiosteal plane. The
non-dominant ring finger is held on the infraorbital rim (to
protect the globe) as the dissection is further extended over
the malar eminence (Fig. 22.4). Extensive release of the lateral
labial segments is a critical maneuver so as to minimize
tension at the muscular and cutaneous closure. The orbicularis
oris bundles are dissected in the subdermal and submucosal
plane for 1 cm, or a little further if necessary (Fig. 22.5).
Alveolar closure
The lateral nasal mucosal flaps are released from beneath the
inferior turbinates, the medial nasal mucosal flaps are elevated
from the premaxilla, and the nasal floors are closed. The
premaxillary mucosal incisions are continued on each side
and vertical incisions are made in the facing gingiva of the
lesser segments. Often digital pressure on the premaxilla is
necessary to permit the alveolar gingivoperiosteal closure.
The alar base flaps are advanced medially and the inner
edge is sutured to the anterior edge of the constructed nasal
floor. The thin strip of vermilion is trimmed off the premaxil
lary mucosa and the remaining mucosal flange is secured high
to the premaxillary periosteum to construct the posterior side
of the central gingivolabial sulcus (Fig. 22.6).
Fig. 22.3 Markings for synchronous repair of bilateral cleft lip and nasal deformity.
Open circles denote tattooed dots. Anthropometric points: pronasale (prn); highest
point of columella nasi (c); subnasale (sn); ala nasi (al); crista philtri superior
(cphs); crista philtri inferior (cphi); labiale superius (ls); stomion (sto).
prn
c
sn
cphs
cphi
ls
al
sto
Fig. 22.4 Lateral labial elements dissected off maxilla in supraperiosteal plane,
extending over the malar eminences.
flap is drawn first. Its dimensions are determined by the
child’s age at repair (usually 5–6 months), and less so by
ethnicity. The length of the philtral flap (sn–ls) is set at 6–7 mm
(normal male 11.4 ± 1.3 mm at 6–12 months); usually it is the
same as the height of the cutaneous prolabium. If the prolabial
element is overly long, the philtral flap can be shortened
appropriately. The width of the philtral flap is set at 2 mm at
the columellar–labial junction (cphs–cphs) and 3.5–4 mm
between the proposed Cupid’s bow peaks (cphi–cphi) (normal
male 6.7 ± 1.0 mm at 6–12 months). The sides of the philtral
flap should be drawn slightly concave in anticipation of slight
bowing with growth. Flanking flaps are drawn; these will be
de-epithelialized and will come to lie beneath the lateral labial
flaps in an effort to simulate philtral columns. These flanking
flaps also add width and increase the vascularity of the phil
tral flap.
The proposed Cupid’s bow peaks are carefully noted on
the lateral labial elements and marked just atop the white roll
above the vermilion–cutaneous junction. These points are situ
ated so that there is medial extension of white roll to form the
handle of the Cupid’s bow and sufficient vermilion height to
construct the median tubercle and raphe. Curvilinear lines
are drawn at the juncture of the alar bases and lateral labial
elements. Anthropometric dimensions are measured and
recorded before proceeding. Lidocaine with epinephrine is
injected into the nose and labial segments, and after the con
ventional 5–7 min waiting time, the critical points, including
the vermilion–mucosal junctions, are tattooed with tincture of
brilliant green dye (Fig. 22.3).
Labial dissection
First, all labial lines are lightly scored. The philtral flanking
flaps are de-epithelialized, the extra prolabial skin is discarded,
and the philtral flap is elevated (including subcutaneous
tissue) close to the anterior nasal spine. Leave fibrous attach
ments to maintain the columellar-labial angle. The lateral
551
Operative techniques
suture is inserted through each upper lateral cartilage and
then through the ipsilateral lateral crus. Often it is possible to
place a second intercartilaginous suture. Holding an intrana
sal cotton-tipped applicator beneath the genu and tenting the
nostril roof facilitates the insertion and tying of these sutures
(Fig. 22.9).
The C-flap on each side of the columellar base is trimmed
to 3–5 mm in length (Fig. 22.10A). The alar bases are advanced
medially, rotated endonasally, and sutured side-to-end to the
C-flaps. Next, the tips of the alar base flaps are trimmed and
closure of the sills is completed. A “cinch suture” of polypro
pylene is placed through the dermis of each alar base, passing
under the philtral flap, and tied to narrow the interalar dimen
sion (al–al) to less than 25 mm (normal male 26 ± 1.4 mm at
6–12 months). The premaxillary sutures placed earlier are
brought above the muscular layer, inserted through the alar
bases (near the “cinch suture”) and tied. These sutures simu
late the depressor alae nasi and also: (1) form the cymal shape
of the sills; (2) prevent alar elevation with smiling; (3) mini
mize postoperative nasal widening (Fig. 22.10B).
Labial closure
It is critical to emphasize advancement of the lateral labial
elements during closure of the sulci. A back-cut is made at the
distal end of the sulcal incision, and each sulcus is closed
while the labial flap is being pulled mesially with a double
hook. The advanced lateral labial mucosa forms the anterior
wall of the central gingivolabial sulcus.
The orbicular bundles are apposed (end-to-end), inferiorly
to superiorly, using simple polydioxanone sutures. Prior to
completion of the muscular closure, a polydioxanone suture
is placed on each side of the premaxilla and left untied.
A polypropylene suture suspends the uppermost muscular
elements to the periosteum of the anterior nasal spine
(Fig. 22.7A).
Construction of the median tubercle begins with placement
of a fine chromic suture, about 3 mm medial to the tattooed
lateral Cupid’s bow peak-point, joining the white-roll–
vermilion–mucosal flaps in the midline (Fig. 22.7B). The
excess vermilion–mucosa is successively trimmed on each
side, and the flaps are accurately aligned to form the median
raphe. There is a natural inclination to save too much of
the vermilion–mucosal flaps, resulting in a furrowed raphe.
Attention is next focused on nasal repair before insetting the
philtral flap.
Nasal dissection and positioning the lower
lateral cartilages
The slumped/splayed lower lateral cartilages are exposed
through bilateral rim incisions (“semi-open” approach).
Fibrofatty tissue is dissected off the anterior surface of and
between the cartilages; this is aided by elevation with a
cotton-tipped applicator on the mucosal underside. Dissec
tion is continued across the dorsal septum to expose the upper
lateral cartilages (Fig. 22.8).
With direct visualization, a horizontal mattress suture of
5-0 polydioxanone (P2 reverse cutting needle, Ethicon) is
placed between the genua and left untied. Another mattress
Fig. 22.5 Dissection of orbicularis oris muscle bundles in subdermal and
submucosal planes.
A
Fig. 22.6 (A) After completion of gingivoperiosteoplasty, redundant premaxillary
vermilion is trimmed. (B) Remaining premaxillary mucosal flange sutured to
periosteum forming posterior wall of anterior gingivolabial sulcus.
B
552
SECTION I
CHAPTER 22 • Repair of bilateral cleft lip
Final touches
Fashioning a philtral dimple seems just beyond the surgeon’s
skill; nevertheless, it is worth trying. One way to simulate
this depression is to suture the dermis in the lower one-third
of the philtral flap to the orbicularis layer. The tip of the
philtral flap is inset into the handle of Cupid’s bow. The
leading edge of the lateral labial flaps need not be trimmed
before apposition to the philtral flap. A little extra lateral
labial tissue helps to simulate the columns. There should be
no tension at the philtral closure, which is done with fine,
interrupted dermal and percutaneous sutures. The cephalic
margin of the labial flaps must be trimmed, corresponding
to the position and cymal configuration of the sills (Fig.
22.11). Closure of the labial flaps to the sills proceeds laterally
to medially.
A
Fig. 22.7 (A) Apposition of orbicularis oris from inferior to superior; uppermost
suture placed through periosteum of anterior nasal spine. (B) Lateral white-roll–
vermilion–mucosal flaps trimmed to construct median tubercle and Cupid’s bow.
B
Fig. 22.8 Lower lateral cartilages exposed through rim incisions. Cotton-tipped
applicator elevates nostril and helps display genua.
Fig. 22.9 Positioning dislocated and splayed
lower lateral cartilages: (A) Apposition of genua
with interdomal mattress suture; (B) suspension
over ipsilateral upper lateral cartilage with
intercartilaginous mattress sutures.
B
A
553
Operative techniques
constructed columella (sn–c) is usually 5–6 mm, (normal male
4.7 ± 0.8 mm at 5 months). After the measurements, a strip of
14 -inch Xeroform gauze is wrapped around a 19-gauge sili
cone tubing and a 1 cm segment is inserted into each nostril.
These vented “stents” are removed after 48 h. Prolonged
nostril splinting is difficult to maintain, likely to damage the
sills, and probably unnecessary.
Postoperative care
A Logan bow is taped to the cheeks: (1) to protect the labial
repair and (2) to hold an iced-saline sponge over the wound
for 24 h postoperatively. The infant is discharged from
After anatomic positioning of the lower lateral cartilages,
it is obvious that there is redundant domal skin in the soft
triangles and in the upper columella. This extra skin is excised
in a crescentic fashion from the leading edge of the rim inci
sions and extending inferiorly along each side of the columella
(see Fig. 22.11). This resection narrows the nasal tip, defines
and tapers the mid-columella, and elongates the nostrils.
Apposition of the genua also accentuates the extra lining
(oblique webbing) in the lateral vestibules. Lenticular excision
on the cutaneous side of the intercartilaginous junction flat
tens this lateral vestibular ridge (see Fig. 22.11, inset).
Immediately postoperative nasolabial anthropometry is
documented and placed in the child’s record (Fig. 22.12).6 The
A
Fig. 22.10 (A) Columellar flaps shortened and alar bases trimmed. Note bilateral sutures in premaxillary periosteum below alar bases – these were inserted prior to
completion of muscular closure. (B) Alar base flaps rotated endonasally and secured (side-to-end) to C-flaps. Interalar distance narrowed with cinch suture. Right
premaxillary suture to alar base has been tied – note cymal configuration (depression) of lateral sill.
B
Fig. 22.11 Crescentic excision of expanded domal skin
and lining extended into upper columella. Cyma-shaped
resection of superior margin of lateral labial elements to fit
curve of the alar base and sill. Lenticular excision of lateral
vestibular web (inset).
554
SECTION I
CHAPTER 22 • Repair of bilateral cleft lip
lary set-back, gingivoperiosteoplasties, and palatoplasty, is
the safer procedure. It is recommended if the child is nearing
age 1 year or older when speech is the first priority. Bilateral
nasolabial repair on a solid maxillary foundation is scheduled
later.
Primary premaxillary retropositioning is likely to accentu
ate midfacial retrusion; however, nasolabial appearance and
speech take precedence. The majority of children with bilateral
complete cleft lip/palate will need maxillary advancement.
Binderoid bilateral complete cleft lip/palate
Nasal features in this rare bilateral variant include: orbital
hypotelorism,
hypoplastic
bony/cartilaginous
elements
(including short septum and absent anterior nasal spine), and
conical columella. Labial features are: hypoplastic prolabium/
premaxilla (with a single incisor) and thin vermilion in the
lateral labial segments.55 The floppy premaxilla usually pre
cludes an attempt at dentofacial orthopedic manipulation;
furthermore, it is often unnecessary because the premaxilla is
not procumbent.
Synchronous nasolabial repair is accomplished as described
above, but there are some slight differences. Sometimes the
premaxilla is so small that alveolar gingivoperiosteoplasties
cannot be accomplished during labial closure. If necessary, a
passive palatal plate can be used to maintain anterior maxil
lary width following nasolabial closure. The philtral flap need
not be drawn overly small in either height or width; it will
expand very little with growth. The interalar dimension
should be narrowed to slightly below age-matched normal
because it will widen. Although the lower lateral cartilages
are hypoplastic, usually they can be dissected, positioned, and
secured. The thin and tapered columella can be augmented
when the child is older (Fig. 22.14).
Secondary procedures are likely in a patient with the
binderoid variant. These include dermal grafts to augment
the median tubercle and to widen the narrow columellar
base; cartilage grafts to the nasal tip, and a costochondral
graft to build-up the nasal dorsum and project the tip; and
maxillary advancement along with augmentation of the fossae
praenasale.55
Bilateral complete cleft lip and intact
secondary palate
Bilateral complete cleft lip/alveolus with intact secondary
palate is another very rare form. The premaxilla is solid,
thus obviating dentofacial orthopedics. If the premaxilla is
not severely protrusive, it may be possible to accomplish
synchronous nasolabial repair and closure of the posterior
premaxillary cleft. If this strategy is chosen, alveolar gingi
voperiosteoplasties should be delayed in order to preserve
premaxillary blood supply. But, if the premaxilla is pro
cumbent, consider a first-stage premaxillary ostectomy and
set-back, along with closure of the alveolar clefts, and closure
of the defect at the anterior edge of the hard palate. Bilateral
nasolabial repair can be done safely at the second stage later in
infancy. The alternative plan is premaxillary set-back, bilateral
nasolabial repair, and closure of the posterior premaxillary–
palatal defect, leaving the facing edges of the alveolar clefts
intact (to maintain blood supply to the premaxilla). The third
strategy is premaxillary set-back, gingivoperiosteoplasties,
the hospital on the second postoperative day. The parents are
instructed in suture-line care and how to keep the nostrils
clean. Percutaneous sutures are removed 6 days postopera
tively under general anesthesia using mask induction and
insufflation. A 12 -inch transverse Steri-Strip (3M Health Care,
St. Paul, MN) is trimmed and placed over the labial scars; the
tape is changed as needed for 6 weeks. Thereafter, the parents
are instructed to perform digital massage (with Pedi-Mederma,
Merz North America, Raleigh, NC) for several months and
counseled about the importance of application of sunblock
ointment (Fig. 22.13).
Technical modifications for
bilateral variations
Late presentation of bilateral complete cleft
lip/palate
Dentofacial orthopedics may not be available or practical in
developing countries. Even in developed nations, a child with
bilateral complete cleft lip/palate can present in late infancy,
and by that age the premaxilla is rigid and preoperative
manipulation is not possible. Rather than attempt labial
closure over a protrusive premaxilla, consider ostectomy and
set-back. There are two alternatives: (1) premaxillary set-back
and nasolabial repair or (2) premaxillary set-back and palato
plasty. With careful attention to mucosal blood supply, pre
maxillary retropositioning can be safely done along with
bilateral nasolabial closure. Another uncommon indication
for the first alternative is partially successful dentofacial
orthopedic preparation. Caution: Only a bilateral narrow
isthmus of septal mucosa nourishes the premaxilla after the
mucosal incisions and elevation necessary for alveolar gingi
voperiosteoplasties and for resection of the premaxillary neck
and inferior septal cartilage. The second alternative, premaxil
Fig. 22.12 Completed bilateral complete cleft lip/nasal repair. Pronasale (prn);
highest point of columella nasi (c); subnasale (sn); ala nasi (al); crista philtri
superior (cphs); crista philtri inferior (cphi); labiale superius (ls); stomion (sto).
prn
c
sn
cphs
cphi
ls
al
sto
555
Technical modifications for bilateral variations
prominent central white roll, the prolabial vermilion–mucosa
may be utilized as the central segment. The next consideration
is columellar length: measure sn–c. If columellar length is
normal for the infant’s age and the lower lateral cartilages are
in nearly normal position, it may be unnecessary to position
the cartilages and sculpt the tip. Nevertheless, interalar nar
rowing is always needed as this dimension is overly wide and
will increase with growth. If the columella is short and the
alar domes are splayed, the lower laterals should be apposed
through the semi-open approach (Fig. 22.16).
Asymmetrical bilateral (complete/incomplete)
cleft lip
Symmetry, the first principle of bilateral labial repair, should
be foremost in mind when planning and executing closure of
nasolabial repair, and delayed closure of the premaxillary–
palatal cleft (the latter is technically difficult). Midfacial retru
sion is very unlikely following primary premaxillary set-back
because the secondary palate is intact (Fig. 22.15).
Bilateral incomplete cleft lip
One-quarter of all double labial clefts are incomplete and
most are symmetrical.45 Of all the bilateral variants, this is the
most easily repaired. The design and execution are the same
as for the bilateral complete form, including adjustments
based on expected nasolabial changes with growth. There are
two technical considerations that need to be underscored. The
first relates to construction of the median tubercle. Usually,
the tubercle should be formed using the lateral white-roll–
vermilion–mucosal flaps. However, in the rare instance of a
bilateral lesser-form (<50% of cutaneous labial height) and a
Fig. 22.13 (A) Bilateral complete cleft lip/palate. (B) Following synchronous nasolabial repair at age 6 months. (C,D,E) At 4 months postoperative. Note columella/tip
projection, hint of a philtral dimple, and normal columellar–labial angle.
A
B
C
D
E
Immediate postoperative anthropometry
documents undercorrected fast-growing
features and overcorrected slow-growing
features compared with normal values
Intraoperative
Patient
(7 months)
Normal
(6–12 months)
n–sn
20.0a
26.9 ± 1.6
al–al
24.5
25.4 ± 1.5
sn–prn
10.5
9.7 ± 0.8
sn–c
6.0a
4.7 ± 0.8
cphs–cphs
1.5
NA
cphi–cphi
4.5a
6.5 ± 1.1
sn–ls
5.5a
10.7 ± 1.1
sn–sto
11.2a
16.0 ± 0.8
ls–sto
6.2
5.3 ± 1.4
Normal values expressed as norm ± SD.
aValues outside SD.
SD, standard deviation; NA, not available.
556
SECTION I
CHAPTER 22 • Repair of bilateral cleft lip
is in near-normal position on the incomplete side, use bilateral
rim incisions and overcorrect the cartilage on the complete
side. There may be inequality of vermilion height at the junc
tion forming the median raphe. This can be adjusted by a
vermilion unilimb Z-plasty, usually with the triangular flap
designed on the lesser side vermilion (Fig. 22.18).
If the contralateral side is a microform cleft, it is best first
to narrow the complete side by preoperative dentofacial
orthopedics and repair it either in one stage or two (after
preliminary nasolabial adhesion) with alveolar gingivoperios
teoplasty. In so doing, observe the contralateral microform
when designing the arc and position of the medial incision for
rotation-advancement repair. Try to limit the incision at the
columellar base and advancement of the lateral labial element
so that the philtral suture line will match the configuration of
the contralateral microform. After the scar has remodeled, the
contralateral microform is corrected using the double unilimb
Z-plastic technique, including muscular apposition and
an asymmetrical variant. An algorithm for timing and tech
niques for repair of asymmetrical bilateral cleft lip is shown
in Fig. 22.17. If both greater and lesser side clefts are incom
plete or the lesser side is a minor-form, synchronous bilateral
repair is indicated. However, if the greater side is incomplete
and the contralateral side is microform or mini-microform, the
incomplete side should be repaired first.
If the greater side is a complete cleft, it is initially managed
by unilateral dentofacial orthopedics, followed by nasolabial
adhesion and alveolar gingivoperiosteoplasty. Thus, the
greater side is converted from a complete to an incomplete
cleft. This levels the surgical field, and the options for the next
stage become clear. If the contralateral (lesser side) cleft lip is
a minor-form or a more severe incomplete, the second stage
is simultaneous bilateral nasolabial repair. Keeping symmetry
in mind, technical maneuvers on the complete side must be
exaggerated because the distortions and tensions are greater
than on the incomplete side. Even if the lower lateral cartilage
A
B
C
Fig. 22.14 (A) Female infant with binderoid bilateral complete cleft lip/palate. (B) Floppy, deviated, diminutive premaxilla centralized by Latham device. (C) After
synchronous repair. Note thin vermilion in lateral labial elements. Midline tip incision is no longer used. (D–F) Appearance at age 5 years.
D
E
F
557
Technical modifications for bilateral variations
Fig. 22.15 (A) Female infant with bilateral complete cleft
lip/alveolus (van der Woude syndrome). (B) Intact secondary
palate. (C) Markings for synchronous repair at age 6 months.
(D) Following premaxillary set-back, alveolar
gingivoperiosteoplasties and nasolabial repair. Palato-
premaxillary defect closed and lower labial sinuses excised in
second stage. (E,F) Appearance at age 2 years.
A
B
C
D
E
F
Intraoperative anthropometry: slow-growing
nasal protrusion and columella were crafted
larger than normal but just within 1 SD
Intraoperative
Patient
(6 months)
Normal
(6–12 months)
n–sn
21.0a
26.9 ± 1.6
al–al
24.5
25.4 ± 1.5
sn–prn
10.5
9.7 ± 0.8
sn–c
5.0
4.7 ± 0.8
cphs–cphs
2.1
NA
cphi–cphi
4.5a
6.5 ± 1.1
sn–ls
5.5a
10.7 ± 1.1
sn–sto
13.0a
16.0 ± 0.8
ls–sto
7.0a
5.3 ± 1.4
Normal values expressed as norm ± SD.
aValues outside SD.
SD, standard deviation; NA, not available.
558
SECTION I
CHAPTER 22 • Repair of bilateral cleft lip
mini-microform. Sometimes, a minor correction is needed to
correct asymmetry of the nasal tip. Often a contralateral mini-
microform does not need attention.45
Outcomes
The surgeon’s responsibility does not end with completion of
nasolabial repair. There is an obligation to periodically assess
the outcome of the procedure and, if possible, to continue to
do so until skeletal growth is complete. Only by so doing can
the surgeon come to understand changes in the fourth dimen
sion, learn from these observations, and apply this knowledge
to succeeding infants with bilateral cleft lip.
dermal graft to augment the philtral ridge, and nasal correc
tion.56 If the position of the contralateral microform cannot be
matched by rotation-advancement on the greater side, syn
chronous bilateral repair is the best strategy. Mirror-image
symmetry is the goal with attention to philtral shape, Cupid’s
bow position, alar base placement, and nostril axis.
If the lesser side cleft is mini-microform, often this can be
corrected by vertical lenticular excision at the same time as
repair on the greater side, although nothing is lost by waiting.
Nasolabial asymmetries between the greater and lesser sides
can best be judged when the child is older. Augmentation of
the median tubercle is almost always necessary. If the Cupid’s
bow peak is too high on the repaired greater side, it is best
to lower it (by unilimb Z-plasty) rather than adjust the
Fig. 22.16 (A) Bilateral symmetrical incomplete cleft lip. (B) Markings for synchronous closure at age 6 months. (C) Following nasolabial repair. (D,E) Appearance at age
1.5 years.
A
B
C
D
E
Intraoperative anthropometry: fast-growing
features made smaller and slow-growing
dimensions made larger than normal age/
sex-matched values
Intraoperative
Patient
(6 months)
Normal
(6–12 months)
n–sn
20.0a
27.0 ± 1.7
al–al
24.5a
26.5 ± 1.4
sn–prn
12.3a
9.1 ± 1.2
sn–c
6.0a
4.3 ± 0.9
cphs–cphs
2.0
NA
cphi–cphi
5.0a
6.7 ± 1.0
sn–ls
7.0a
11.4 ± 1.3
sn–sto
14.5
15.8 ± 1.5
ls–sto
7.0a
4.4 ± 1.0
Normal values expressed as norm ± SD.
aValues outside SD.
SD, standard deviation; NA, not available.
559
Outcomes
Standardized photographs can be used for panel-assessment
of repaired bilateral cleft lip.57 Although visual-perceptive
analysis using a predetermined rating scale can be reliable, it
is clumsy, time-consuming, and still subjective.
Revision rate
Most surgeons keep a mental tally of the kinds of revi
sions that are necessary after cleft lip repairs. Documenting
the types of secondary corrections guides the surgeon to
make technical alterations during subsequent primary pro
cedures. The surgeon’s goal is to minimize the number of
revisions. For a repaired bilateral deformity, the cutaneous
lip should never need to be reopened. The nasal cartilages
may require repositioning, the mucosal free margin often
Photography
Preoperative photographs are the basic minimum for docu
mentation. Images are unacceptable if taken when the child
is on the operating table with the endotracheal tube in place.
The surgeon must find the time and have the patience to take
preoperative frontal, submental, and lateral views of the
infant. Intraoperative photographs taken after cutaneous
markings and immediately after the repair are also useful. The
standard angle for the submental view should align the nasal
tip on a line sighted half-way between the medial canthi and
eyebrows. The submental photograph is essential for assess
ment of nasal configuration and symmetry. Photographs must
be taken periodically during childhood, as well as before and
after adolescent growth.
Fig. 22.17 Algorithm for correction of asymmetrical
bilateral cleft lip (complete/incomplete) and contralateral
incomplete or lesser-form cleft. DFO, dentofacial
orthopedics; GPP, gingivoperiosteoplasty. (Modified from
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;122:1494–1504.)
Greater side
Lesser side
Minor-form
Incomplete
Complete
DFO
Labial adhesion and GPP
Synchronous bilateral
nasolabial repair
Incomplete
Complete
DFO
Labial adhesion and GPP
Rotation-advancement
Incomplete
Lesser side: delayed double unilimb Z-plasty
Rotation-advancement
Lesser side: immediate/delayed lenticular
labial excision and nasal correction
Complete
DFO
Labial adhesion and GPP
Incomplete
Microform
Mini-microform
Type of repair
560
SECTION I
CHAPTER 22 • Repair of bilateral cleft lip
vermilion and securing the remaining mucosa to the premax
illary periosteum. Augmentation of a weak median tubercle
with a dermal–fat graft is also common; this was usually done
at the time of alveolar bone grafting (age 9–11 years). Using
the posterior iliac donor site, a thick dermal–fat graft is easily
taken along with harvest of cancellous bone.59
The most frequent nasal distortion in this series was dis
proportionate widening of the interalar dimension; however,
this rarely required correction during childhood. None of
the children in this study required a secondary “columellar
lengthening” or revision for an abnormally wide or long
philtrum.
Given preoperative asymmetry, the revision rate for asym
metrical bilateral cleft lip would be expected to differ slightly
from that for the more common symmetrical complete forms.
Indeed, our study showed the rate of nasolabial revision cor
related with the degree of preoperative asymmetry between
must be adjusted, and, sometimes, nasal width has to
be narrowed.
Symmetry is the one major advantage of a bilateral com
plete cleft lip as compared to a unilateral complete cleft lip.
Any nasolabial asymmetry following closure and further
distortions with growth will become increasingly obvious
before the child attends school. These asymmetries remain
relatively unchanged during childhood, but they often become
magnified during adolescence. The age for kindergarten is a
good time to assess the child for revision.
In a study of 50 consecutive non-syndromic children
(median age 5.4 years), the revision rate was 33% for those
with bilateral complete cleft lip/palate as compared to 12%
for those with bilateral complete cleft lip/alveolus and intact
secondary palate.58 The most common labial revision was
resuspension of prolapsed anterior gingivolabial mucosa.
This problem has since been minimized by trimming prolabial
Fig. 22.18 (A) Asymmetrical bilateral cleft
lip: right complete and left incomplete.
(B) Bilateral complete cleft of secondary
palate, submental view. (C,D) Age 6 years
following first-stage right labial adhesion/
gingivoperiosteoplasty and second-stage
synchronous nasolabial repair.
A
B
C
D
561
Outcomes
Direct anthropometry can be repeated as the child grows
and compared to normal values based on sex and age. This
was shown in a retrospective longitudinal analysis of 12
children with bilateral complete cleft lip who underwent
repair with the same procedure as described herein.4 Through
age 4 years, nasal height and protrusion, as well as columellar
length and width, were within 1 standard deviation of normal
values. The interalar dimension was overly wide, about 1
standard deviation above normal. Nevertheless, the nose
often did not appear wide because children with bilateral cleft
lip tend to have minor orbital hypertelorbitism. The cutane
ous lip was intentionally set short because this is less eye-
catching than the typical long lip appearance in these patients.
Thus, total lip height was 1–2 standard deviations below
normal, whereas, height of the median tubercle was 1 stan
dard deviation above normal at 4 years. Fullness of the central
red lip usually falls below normal in older children. Depend
ing on the show of the permanent incisors, the median tubercle
either is augmented or excess mucosa is trimmed. An example
of an 8-year-old boy with repaired bilateral complete cleft lip/
palate is shown in Fig. 22.19 with intraoperative and postop
erative anthropometry.
Serial direct anthropometry in 32/48 patients with repaired
bilateral incomplete cleft lip was compared to Farkas’s norma
tive values.63 Females, African-Americans, and Asians were
excluded because of their small numbers, leaving 22 Cauca
sian males in the long-term study. Nasal measurements
demonstrated: interalar dimension (al–al) widened in early
childhood and followed line of normal growth thereafter;
nasal tip protrusion (sn–prn) grew in parallel above the
normal line; columellar height (sn–c) crossed the normal line
in early childhood and remained slightly below into adult
hood. Labial measurements showed: Cupid’s bow width
(cphi–cphi) was normal into late adolescence; upper philtral
width (cphs–cphs) remained below the normal line; philtral
height (sn–ls) stayed below the normal line; median tubercle
(ls–sto) grew slowly, sometimes dropping below the normal
line during adolescence; total labial height (sn–sto) closely
approximated Farkas’s normal line throughout childhood and
adolescence.
Normative anthropometric measures are needed for the
major ethnic groups. Farkas’ book, in addition to Caucasian
norms, includes data from Singapore for three age groups: 6
and 12 years (taken in school children) and 18 years (taken in
army recruits and university students).62 Indian anthropom
etry from infancy to adulthood is also available.64 Kim and
colleagues measured six nasolabial features in normal Korean
children age 5 years and younger, and compared the mean
values to 30 children who had a modified “Mulliken repair”
for three variants of bilateral cleft lip.65 They found all naso
labial features were within 2 standard deviations of normal.
Nasal tip protrusion and columellar length were below,
whereas nasal width was slightly above, in normal age-
matched Korean noses.
Indirect anthropometry
Photogrammetry
Photogrammetry is the original type of indirect anthropom
etry. It eliminates any inaccuracies introduced in trying to
measure the lip and nose in a frightened or fidgety child.
the greater and lesser sides.45 The lowest frequency of nasola
bial revision was in the contralateral minor-form subgroup
because symmetry is more likely achieved by synchronous
bilateral correction. In contrast, the highest frequency of both
nasal and labial revision (usually on the greater side) was in
the contralateral mini-microform subgroup. This was predict
able because the nearly normal configuration of a mini-
microform makes it more difficult to attain primary symmetry.
Conceptually, the complete/mini-microform type of bilateral
cleft lip approaches the unilateral complete cleft lip deformity.
In all of the contralateral lesser-forms, the most common
labial deformity was a thin median tubercle, accentuated by
a full mucosal free margin on the greater side. This deficiency
of the tubercle can be explained by insufficient mesodermal
contribution from the lesser side.
Reassessment of the frequency and types of nasolabial
revisions must be undertaken after skeletal maturity. We
analyzed the frequency of Le Fort I osteotomy and maxillary
advancement in the major forms of bilateral cleft lip: 50% for
bilateral incomplete or bilateral asymmetrical complete/
incomplete and 75% for bilateral complete cleft lip/palate.60
The high rate of maxillary advancement in our unit reflects
our preference for operative correction for all patients with
midfacial retrusion whatever the occlusal relationship. Le Fort
I advancement is often combined with alloplastic onlay to the
malar eminences to give a normal convex facial profile.
Attempts to compensate for a small degree of maxillary retru
sion at the dental level results in a relatively flat facial profile.
The need for secondary nasolabial correction is based on
subjective criteria, determined by the surgeon’s opinion and
often made in agreement with the family and the older patient.
Cephalometry has long been used by dental specialists to
document skeletal growth in children with repaired cleft lip/
palate. Similar quantitative methodology is needed to permit
assessment of nasolabial appearance during childhood and
adolescence.
Direct anthropometry
Farkas was the first to apply medical anthropometry to chil
dren with repaired bilateral cleft lip.61 His reference book is
invaluable.62 It contains normative values for 28 nasal and 18
labial linear/angular measurements in a North American Cau
casian population from birth (0–5 months and 6–12 months)
and each year up to age 18. Direct anthropometry requires
training, practice, and patience. The tools are a sliding Vernier
caliper and a Castroviejo caliper. Locating the soft-tissue
landmarks and measuring nasolabial dimensions are usually
easily accomplished in a child older than 5 years, but difficult
to impossible in a younger child. Intraoperative anthropom
etry is used to assess severity of the deformity prior to the
procedure and immediately after repair as a record of baseline
nasolabial dimensions.6 In an analysis of 46 consecutive repairs
of bilateral complete cleft lip, intraoperative anthropometry
confirmed the strategy of design in three dimensions in antici
pation of the fourth dimension. All fast-growing nasolabial
features were set smaller than age and sex-matched normal
infants. The only exception was central vermilion–mucosal
height (median tubercle) that purposely was made overly
full (on average 155% of normal). The slow-growing features,
nasal protrusion, and columellar height, were also constructed
longer than normal, 130% and 167%, respectively.
Fig. 22.19 (A) Bilateral complete cleft lip/palate. (B) Submental view following closure. (C–E) Appearance at age 8 years. Note nasal protrusion and columellar length are
longer than normal; however, nasal width and Cupid’s bow width are above normal.
A
B
C
D
E
Intraoperative anthropometry: note overcorrected nasal
protrusion and columellar length; nasal width is narrower
than normal
Intraoperative
Postoperative
Patient
(6 months)
Normal
(6–12
months)
Patient
(8 years)
Normal
(8 years)
n–sn
22.0a
27.0 ± 1.7
44.0
42.1 ± 2.4
al–al
26.0
26.5 ± 1.4
36.0a
29.8 ± 1.5
sn–prn
15.0a
9.1 ± 1.2
17.7a
15.9 ± 1.3
sn–c
7.0a
4.3 ± 0.9
10.5a
8.0 ± 1.1
cphs–cphs
2.0
NA
7.7
NA
cphi–cphi
4.0a
6.7 ± 1.0
10.4a
8.8 ± 1.1
sn–ls
7.5a
11.4 ± 1.3
9.0a
14.0 ± 2.2
sn–sto
16.5
15.8 ± 1.5
16.0a
19.7 ± 1.8
ls–sto
7.0a
4.4 ± 1.0
7.8
8.0 ± 1.2
Normal values expressed as norm ± SD.
aValues outside SD.
SD, standard deviation; NA, not available.
563
Outcomes
However, measurements on two-dimensional photographs
likely introduce errors due to magnification, variation in
lightening, angulation, head position, and subject-to-camera
distance. The magnification factor can be eliminated by
including a standard metric in the photograph. Photogram
metry can only be used for linear measurements of certain
nasolabial features, as well as for proportions and angles.66
Examples of photogrammetric assessment in 15 patients with
bilateral complete cleft lip repaired by the operative tech
niques described herein are found in a study by Kohout and
colleagues.67 The ratio of columellar length (sn–c) to nasal tip
protrusion (sn–prn), the two slow-growing dimensions, was
0.47 ± 0.08, i.e., very close to the normal of 0.53 ± 0.02 at a mean
age of 2 years. Changes in the columellar–labial angle were
analyzed in a small cohort of children. This angle was obtuse
in early childhood (128.5 ± 6.5°) (normal 102.5 ± 5.2°), but
began narrowing after age 7 years and normalized by adoles
cence. Possible explanations for this change include either
growth of the anterior-caudal septum, increasing obliquity of
the lip, or increasing maxillary retrusion.
Liou and colleagues employed basilar photogrammetry to
assess 22 young children with repaired bilateral complete cleft
lip using the NAM protocol.68 They determined that columel
lar length decreased in the first and second years postopera
tively, then started to increase in the third year, but still lagged
behind the normal length by 1.9 mm, whereas all other nasal
dimensions increased significantly. Lee and co-workers also
used photogrammetry to assess children who had undergone
the NAM protocol and primary “retrograde” nasal correc
tion.69 They noted that columellar length was slightly less, but
not statistically different, from a control group of children at
an average age of 3 years.
Morovic and Cutting adapted some elements of primary
nasal correction described herein and used photogrammetry
with intermedial canthal distance as the scaling standard.70 In
25 children, they calculated near-normal columellar length
and tip projection, but nasal width, columellar width, and
nasolabial angles were significantly greater than the control
values.
Stereophotogrammetry
Three-dimensional stereophotogrammetry is the most ad
vanced way to quantitatively assess nasolabial appearance.
Fig. 22.20 (A,B) Three-dimensional photogrammetry (Vectra 3D imaging system): anthropometric points located on frontal and submental images. (Same patient as in
Fig. 24.18.)
A
B
Indirect anthropometry (3D photogrammetry) at age 6 years
(same patient as shown in Fig. 22.18). Note changes in
fast- and slow-growing features
Postoperative
Patient (6 years)
Normal (6 years)
n–sn
37.6
39.3 ± 2.7
al–al
26.5
27.5 ± 1.3
sn–prn
15.8a
14.5 ± 1.2
sn–c
L, 7.0; R, 6.4
7.5 ± 1.0
cphs–cphs
3.2
NA
cphi–cphi
6.7a
8.4 ± 1.3
sn–ls
11.5a
12.6 ± 1.3
sn–sto
20.3a
18.7 ± 1.7
ls–sto
9.7
8.0 ± 1.1
Normal values expressed as norm ± SD.
aValues outside SD.
SD, standard deviation; NA, not available.
Direct intraoperative anthropometric data on patient shown in
Fig. 22.18 (age 6 months)
Intraoperative
Patient
(6 months)
Normal
(6–12 months)
n–sn
23.0a
26.9 ± 1.6
al–al
24.0a
25.4 ± 1.5
sn–prn
12.3a
9.7 ± 0.8
sn–c
6.0a
4.7 ± 0.8
cphs–cphs
2.0
NA
cphi–cphi
4.0a
6.5 ± 1.1
sn–ls
6.8a
10.7 ± 1.1
sn–sto
13.4a
16.0 ± 0.8
ls–sto
6.5
5.3 ± 1.4
Normal values expressed as norm ± SD.
aValues outside SD.
SD, standard deviation; NA, not available.
564
SECTION I
CHAPTER 22 • Repair of bilateral cleft lip
Several systems are currently available: 3dMDface (3dMD,
Atlanta, GA) and Vectra (Canfield Imaging Systems, Fair
field, NJ). The validity and reliability of these systems have
been documented.71,72 Synchronized high-resolution digital
cameras capture images in milliseconds. Software algorithms
merge the different overlapping images into a single three-
dimensional image that can be viewed, turned in any direc
tion, and analyzed on a computer. Standard anthropometric
points are easily located after the image is appropriately
maneuvered and the nasolabial dimensions are recorded (Fig.
22.20). Furthermore, the digital images can be manipulated to
calculate soft-tissue projection and produce a numeric value
for mirror-image symmetry of the nose or lip.
Conclusion
Every year, a cleft lip/palate team will treat only a few chil
dren born with a bilateral deformity or a variant. Every infant
with this defect deserves a surgeon who has the requisite
patience, precision, and passion to undertake the first opera
tion. Indeed, the primary procedures are the major determi
nants of the child’s appearance and ability to communicate.
The surgeon also has the obligation to care for and assess
these children well into adulthood – this requires singular
commitment.
6. Mulliken JB, Burvin R, Farkas LG. Repair of bilateral complete cleft
lip: intraoperative nasolabial anthropometry. Plast Reconstr Surg.
2001;107:307–314.
13. Millard DR Jr. Cleft Craft: The Evolution of Its Surgery. Vol. II. Boston,
MA: Little, Brown; 1977. One definition of a “classic” is a great book
that is often cited, but seldom read. In his conversational style of writing,
Millard recounts the history of bilateral cleft lip repair as if he was an
observer. The novice may find the organization of the book a little difficult
to follow. Nevertheless, reading Millard’s text is analogous to watching a
master surgeon in the operating room. The more experienced the visitor,
the more gained by the experience.
44. Cutting CB, Grayson BH, Brecht L, et al. Presurgical columellar
elongation and primary retrograde nasal reconstruction in one-stage
bilateral cleft lip and nose repair. Plast Reconstr Surg. 1998;101:630–
639. The article describes the prototype of a nasoalveolar molding
appliance in preparation for synchronous nasolabial repair by Cutting’s
technique. The authors underscore that expansion of nasal lining is as
important as stretching columellar skin. The principle of primary
positioning the lower lateral cartilages is applied as described in this
chapter; however, the technique differs.
45. 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;122:1494–
1504. This paper focuses on a subgroup of asymmetrical bilateral clefts
that present with a lesser-form variant that is contralateral to a complete
or incomplete cleft lip. The lesser-forms are defined based on extent of
disruption at the vermilion–cutaneous junction: minor-form; microform,
and mini-microform. These designations determine the methods of repair
and correlate with frequency and types of revisions that are usually
necessary.
55. Mulliken JB, Burvin R, Padwa BL. Binderoid complete cleft lip/
palate. Plast Reconstr Surg. 2003;111:1000–1010. The authors define a
rare subset of patients who have complete cleft lip/palate,
nasolabiomaxillary underdevelopment, and orbital hypertelorism. One-half
of the patients have a bilateral complete deformity, characterized by a
diminutive single-toothed premaxilla. Necessary modifications in primary
repair and in secondary correction of the hypoplastic soft tissue and
skeletal elements are described.
57. Lo L-J, Wong F-H, Mardini S, et al. Assessment of bilateral cleft lip
nose deformity: a comparison of results as judged by cleft surgeons
and laypersons. Plast Reconstr Surg. 2002;110:733–741.
58. Mulliken JB, Wu JK, Padwa BL. Repair of bilateral cleft lip: review,
revisions, and reflections. J Craniofac Surg. 2003;14:609–620.
62. Farkas LG, ed. Anthropometry of the Head and Face. 2nd ed. New
York, NY: Raven Press; 1994.
69. Lee CT, Garfinkle JS, Warren SM, et al. Nasoalveolar molding
improves appearance of children with bilateral cleft lip–cleft palate.
Plast Reconstr Surg. 2008;122:1131–1137. This study provides further
proof of the principle of primary nasal correction. Photogrammetry was
used to document columellar length in patients with bilateral cleft lip/
palate who had nasal repair by the two-stage forked flap method versus
primary nasal correction after nasoalveolar molding; both groups were
compared to age-matched controls. Measurements to age 3 years showed
nearly normal columellar length in the primary repair group without need
for further nasal procedures, whereas secondary operations were
recommended for all children who had forked flap columellar lengthening.
72. Wong JY, Oh AK, Ohta E, et al. Validity and reliability of
craniofacial anthropometric measurements of 3D digital
photogrammetric images. Cleft Palate Craniofac J. 2008;45:232–239.
Access the complete reference list online at
564.e1
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639. The article describes the prototype of a nasoalveolar molding
appliance in preparation for synchronous nasolabial repair by Cutting’s
technique. The authors underscore that expansion of nasal lining is as
important as stretching columellar skin. The principle of primary
positioning the lower lateral cartilages is applied as described in this
chapter; however, the technique differs.
45. 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;122:1494–
1504. This paper focuses on a subgroup of asymmetrical bilateral clefts
that present with a lesser-form variant that is contralateral to a complete
or incomplete cleft lip. The lesser-forms are defined based on extent of
disruption at the vermilion–cutaneous junction: minor-form; microform,
and mini-microform. These designations determine the methods of repair
and correlate with frequency and types of revisions that are usually
necessary.
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Reconstr Surg. 1989;83:32–38.
564.e2
SECTION I
CHAPTER 22 • Repair of bilateral cleft lip
51. Millard DR Jr, Latham RA. Improved primary surgical and dental
treatment of clefts. Plast Reconstr Surg. 1990;86:856–871.
52. Henkel K-O, Gundlach KKH. Analysis of primary
gingivoperiosteoplasty in alveolar cleft repair. Part I: facial growth.
J Craniomaxillofac Surg. 1997;25:266–269.
53. Berkowitz S, Mejia M, Bystrik A. A comparison of the Latham–
Millard procedure with those of a conservative treatment approach
for dental occlusion and facial aesthetics in unilateral and bilateral
complete cleft lip and palate: part I. Dental occlusion. Plast Reconstr
Surg. 2004;113:1–18.
54. Bitter K. Repair of bilateral cleft lip, alveolus and palate. Part 3:
follow-up criteria and late results. J Craniomaxillofac Surg.
2001;29:49–55.
55. Mulliken JB, Burvin R, Padwa BL. Binderoid complete cleft lip/
palate. Plast Reconstr Surg. 2003;111:1000–1010. The authors define a
rare subset of patients who have complete cleft lip/palate,
nasolabiomaxillary underdevelopment, and orbital hypertelorism. One-half
of the patients have a bilateral complete deformity, characterized by a
diminutive single-toothed premaxilla. Necessary modifications in primary
repair and in secondary correction of the hypoplastic soft tissue and
skeletal elements are described.
56. Mulliken JB. Unilimb Z-plastic repair of microform cleft lip. Plast
Reconstr Surg. 2005;116:1623–1632.
57. Lo L-J, Wong F-H, Mardini S, et al. Assessment of bilateral cleft lip
nose deformity: a comparison of results as judged by cleft surgeons
and laypersons. Plast Reconstr Surg. 2002;110:733–741.
58. Mulliken JB, Wu JK, Padwa BL. Repair of bilateral cleft lip: review,
revisions, and reflections. J Craniofac Surg. 2003;14:609–620.
59. Steinbacher DM, Padwa BL, Mullliken JB. Simultaneous harvesting
cancellous iliac bone for alveolar cleft closure and dermis for
augmentation of the median tubercle. Cleft Palate Craniofac J.
2009;46:295–298.
60. Good PM, Mulliken JB, Padwa BL. Frequency of Le Fort I
osteotomy after repaired cleft lip/palate or cleft palate. Cleft Palate
Craniofac J. 2007;44:396–401.
61. Farkas LG, Lindsay WK. Morphology of the adult face follow repair
of bilateral cleft lip and palate in childhood. Plast Reconstr Surg.
1971;47:25–32.
62. Farkas LG, ed. Anthropometry of the Head and Face. 2nd ed. New
York, NY: Raven Press; 1994.
63. Mulliken JB, Kim DC. Repair of bilateral incomplete cleft lip:
techniques and outcomes. Plast Reconstr Surg. 2013;14:609–620.
64. Khandekar B, Srinivasan S, Mokal N, et al. Anthropometric analysis
of lip–nose complex in Indian population. Indian J Plast Surg.
2005;38:128–131.
65. Kim S-K, Lee J-H, Lee K-C, et al. Mulliken method of bilateral cleft
lip repair: anthropometric evaluation. Plast Reconstr Surg.
2005;116:1243–1251.
66. Farkas LG, Bryson W, Klotz J. Is photogrammetry of the face
reliable? Plast Reconstr Surg. 1980;66:346–355.
67. Kohout MP, Monasterio Aljaro L, Farkas LG, et al. Photogrammetric
comparison of two methods for synchronous repair of bilateral cleft
lip and nasal deformity. Plast Reconstr Surg. 1998;102:1339–1349.
68. Liou EJ-W, Subramanian M, Chen PK. Progressive changes of
columella length and nasal growth after nasoalveolar molding in
bilateral cleft patients: a 3 year follow-up study. Plast Reconstr Surg.
2007;109:642–648.
69. Lee CT, Garfinkle JS, Warren SM, et al. Nasoalveolar molding
improves appearance of children with bilateral cleft lip–cleft palate.
Plast Reconstr Surg. 2008;122:1131–1137. This study provides further
proof of the principle of primary nasal correction. Photogrammetry was
used to document columellar length in patients with bilateral cleft lip/
palate who had nasal repair by the two-stage forked flap method versus
primary nasal correction after nasoalveolar molding; both groups were
compared to age-matched controls. Measurements to age 3 years showed
nearly normal columellar length in the primary repair group without need
for further nasal procedures, whereas secondary operations were
recommended for all children who had forked flap columellar lengthening.
70. Morovic CG, Cutting C. Combining the Cutting and Mulliken
methods for primary repair of the bilateral cleft lip nose. Plast
Reconstr Surg. 2005;116:1613–1619.
71. Weinberg SM, Naidoo S, Govier DP, et al. Anthropometric precision
and accuracy of digital three-dimensional photogrammetry:
comparing the Genex and 3dMD imaging systems with one another
and with direct anthropometry. J Craniofac Surg. 2006;17:477–483.
72. Wong JY, Oh AK, Ohta E, et al. Validity and reliability of
craniofacial anthropometric measurements of 3D digital
photogrammetric images. Cleft Palate Craniofac J. 2008;45:232–239.
SECTION I • Clefts
23
Cleft palate
Jason H. Pomerantz and William Y. Hoffman
SYNOPSIS
■ Normal speech is the primary goal of cleft palate repair; minimizing
effects of maxillary growth is also important but ultimately secondary.
■ Cleft palate repair prior to 1 year of age (ideally 9–10 months) results
in better speech outcomes than later repairs.
■ The levator veli palatini muscle is longitudinally oriented in the cleft
palate patient. Realignment of the muscle to a transverse and posterior
position in the soft palate is the key to a successful functional result.
■ Eustachian tube function is abnormal in cleft patients due to abnormal
position of the tensor veli palatini muscle; this must be addressed in
every cleft palate patient, usually with ventilating tubes.
Access the Historical Perspective section online at
Basic science
Embryology
The embryology of maxillary and palatal development is
reviewed in detail in Chapter 20. In broad terms, the failure
of fusion of the frontonasal and maxillary processes gives rise
to the cleft of the primary palate, which includes the lip,
alveolar process, and the hard palate anterior to the incisive
foramen. This results in a cleft in the typical location between
the premaxilla and the lateral maxilla, on either one or both
sides. The lateral palatal shelves fuse later than the primary
palate, around 7–8 weeks’ gestation, as they rotate from verti
cal to horizontal orientation. This fusion proceeds from
anterior to posterior, which helps us to understand the spec
trum of clefts of the secondary palate.
The levator palatini and other pharyngeal muscles are
derived from the fourth branchial arch and are innervated by
the pharyngeal plexus, primarily cranial nerve X (vagus). The
sole exception to this is the tensor palatini muscle, which
arises from the first branchial arch and is innervated by cranial
nerve V (trigeminal).
Anatomy of the velar muscles
Careful anatomic evaluation of each patient is of paramount
importance in considering palatoplasty. Anatomic variability
within the broad diagnosis of cleft palate will influence the
timing and sequence of surgical repair as well as the type of
repair. Optimal functional results depend directly on accurate
analysis of the available structures and understanding of their
long-term significance to function and facial growth (Fig. 23.1).
It is critical to understand the anatomy of the levator palatini
muscle and the derangement of this anatomy that occurs in all
clefts of the palate, including submucous cleft palate. Normally
the levator muscle forms a transverse sling across the posterior
half of the soft palate, and contraction causes the soft palate to
move superiorly and posteriorly, contacting the posterior
pharyngeal wall for velar closure, usually at the level of the
adenoid pad. This action forms the characteristic “genu” seen
on lateral views of the palate in motion.
In addition to being discontinuous across the cleft, the
levator muscle runs more or less longitudinally along the cleft
margin before it inserts aberrantly into the posterior border
of the hard palate.11–13 This results in ineffective contraction
and inability to close the palate against the posterior pharyn
geal wall. Air escape through the nose during speech produces
a characteristic hypernasal quality. In addition, aberrant
levator positioning, as well as an abnormal fusion with the
tendon of the tensor veli palatini muscle, is thought to impair
the function of the tensor muscle in assistance with Eustachian
tube function, and is thought to be contributory to cleft oto
pathology.14 A review of cleft otopathology is presented in the
next section.
Ear pathology
Alt was the first physician to note a correlation between ear
disease and cleft palate in 1878.15 Numerous studies have
565.e1
Historical perspective
Historical perspective
The first recorded operative treatment of a cleft patient has
been attributed to the period of the Chin (Tsin) Dynasty
(c.390 AD). The repair was of a cleft lip only, and no mention
of cleft palate repair was made. Palatal clefts were confused
with the more common fistulas resulting from tertiary syphilis
and were not addressed surgically because of this association.
The greater technical challenges of cleft palate repair no doubt
presented a barrier to surgical treatment as well. Although the
first known cleft palate repair was performed in the early 19th
century, the introduction of anesthesia permitted a quantum
leap in treatment as it did for many diseases.1,2
John Stephenson (1797–1842), a physician who was born
with incomplete cleft palate, wrote the earliest recorded
description of palatoplasty. As a medical student in Edin
burgh, he traveled to Paris to observe the renowned surgeon
Philibert Roux (1780–1854). While he was there, Roux noted
his abnormal speech pattern; he performed the primary repair
of his velum when Stephenson was 22 years old. In his thesis,
Stephenson wrote in remarkable detail of his speech quality:
I always pronounced /th/ like /s/. As I grew up, the adjacent
parts tended to close the defect, and by speaking slowly I
articulated better. Thanks to the nasal quality of the language, I
used to speak French more clearly than English. Nature is kind
and trying to correct her mistakes, did her best to improve my
unpleasant voice by contracting as strongly as possible the
muscles of the nasopharynx … I believe that the nasal sound
was due to faulty vibration of the air in its passage from lungs
to nares, because the fissure prevented the soft palate from
functioning.3
Following the description of his speech, Stephenson wrote in
detail of the procedure, including the positioning (seated
upright in front of Dr. Roux), anesthetic (none), difficulties
(breathing and bleeding), and postoperative course (respira
tory difficulty, nothing to eat or drink for 29 h, and discomfort
of suture removal). He also described the quality of the post
operative speech:
It must be confessed that some of the nasal quality is still
present. Old habit and the above-mentioned muscular
contraction were too much for me. The repaired instrument is
not yet fulfilling its proper duties to giving the help it should to
my vocal faculty. Who can deny the all-importance of habit?
The same problem often occurs today when cleft palate repair
is delayed until later in life.
After the acceptance of his doctoral thesis, Stephenson
returned to his home in Canada and eventually became a
principal founder of McGill Medical College, where he served
as Professor of Anatomy, Physiology, and Surgery.4 Roux later
published his account of the procedure in 1825, describing the
simple suture closure of the surgically freshened edges, which
he termed “staphyloraphie”.5
The notoriety surrounding the publication of Stephenson’s
thesis angered Carl Ferdinand von Graefe (1787–1840), who
proclaimed that he, rather than Roux, was the first surgeon to
perform velar closure. Indeed, he had devised the surgery and
performed it (unsuccessfully) before 1819. He had briefly
mentioned the simple suture of the velum to the medical
society of Berlin in 1816 but had failed to report it formally in
the medical journals of the time. For von Graefe, “who
appeared to have little interest in this procedure at the time,
apparently deeming it of no importance, it now became the
chief concern of his life”.6 Competition and national honor
between surgical groups in Berlin and Paris split the European
medical community on this topic. Von Graefe eventually
reported a successful repair in 1820.7 The first successful
closure of the soft palate in America was by John Collins
Warren in 1820 in Boston.2
Johann Friedrich Dieffenbach (1792–1847) studied under
von Graefe at the University of Berlin.8 Dieffenbach expanded
the technique of soft palate repair to include closure of the
hard palate. Dieffenbach’s palatoplasty method involved
bringing together the cleft bony segments by a series of twist
ing silver or lead sutures passed through punch holes in the
bony palate.9 He eventually designed and advocated lateral
mucosal relaxing incisions to aid successful soft-tissue closure.
Later, following von Graefe at Charité Hospital in Berlin in
1840, he not only improved the technique of palatoplasty but
also advanced techniques of local flap closure and transplan
tation. Dieffenbach’s possibly most important contribution to
plastic surgery was the introduction of ether anesthesia to
plastic surgical procedures.9 Dieffenbach had been introduced
to anesthetic technique during a demonstration at Massachu
setts General Hospital in 1846 and began applying it to routine
surgical procedures before his death in 1847. He was so
revered in Germany that he received an official state burial.
After the death of Dieffenbach, Bernhard von Langenbeck
(1810–1887) succeeded to the position at the University of
Berlin. Incorporating his extensive work with bone and peri
osteum of the extremities during the Franco-Prussian war, he
was the first to describe the mucoperiosteal plane of dissec
tion and to use its advantage in mobility to cleft palate
closure.10 His technique, with various modifications, is still
widely used today. The combination of Dieffenbach’s intro
duction of general anesthesia and von Langenbeck’s use of
mucoperiosteal flaps ushered in the modern era of cleft palate
surgery.
The ensuing century has brought greater understanding of
the anatomy of the cleft palate, as well as improved surgical
approaches. Dorrance’s introduction of a pushback technique,
Veau’s dissection of the levator muscles, and the more recent
techniques introduced by Bardach and Furlow are particularly
significant. As many of these operations are still in use, we
review them in the appropriate technical areas of the chapter
rather than in this historical overview.
566
SECTION I
CHAPTER 23 • Cleft palate
Both radiologic and manometric testing techniques have
demonstrated abnormalities in active dilation of the Eusta
chian tube.20 In addition, intrinsic abnormalities of tubal car
tilage framework rendering a Eustachian tube more collapsible
have been noted. Although anatomic studies show that the
levator veli palatini muscle does not directly actively open the
Eustachian tube orifice, it is likely to have a secondary effect
by influence on the tensor veli palatini and also with passive
position of the orifice. The levator and tensor do share a
common tendinous insertion near the hamulus and pulley
position around the hamulus. In the cleft patient, the levator
is connected solidly against the rigid posterior hard palate;
the pulley effect around the hamulus cannot be activated and
this impairs opening of the tube. In addition, some theorize
that constant bathing of the tube orifice with oropharyngeal
refluxed material leads to inflammation and obstruction of
drainage. Other studies have demonstrated adenoidal tissue
at the level of the tubal dilator that could potentially contrib
ute to mechanical obstruction.21 Anatomic paratubal abnor
malities and risk for serous otitis media and chronic audiologic
sequelae are present, regardless of cleft type, although Pierre
Robin sequence has been postulated to be associated with
even higher risk for hearing loss because of potential for
concomitant ossicular malformation.22
Chronic obstruction of drainage leads to serous otitis
media, and long-standing effusion can result in hearing loss.
Estimates are 20–30% incidence of pure tone hearing loss in
cleft patients by audiography23; decreased hearing has been
found in as many as half of cleft palate patients by other
authors.24 Untreated children with clefts and severe effusions
may have total deafness. Whereas hearing loss is significant
in any child, it may be even more so in a cleft patient in whom
speech development may be abnormal.
It has long been suggested that closure of the palate reduces
risk of permanent hearing loss. In retrospective studies, chil
dren who had undergone palatoplasty had significantly lower
incidence of permanent hearing loss than did children with
unrepaired cleft palates.24 Although still controversial, palato
plasty is thought by most surgeons to reduce risk of chronic
serous otitis media and hearing loss. Nevertheless, serous
otitis persists in most cleft patients for several years after
palate repair. Although not currently recommended for every
cleft palate patient (e.g., absence of effusion during examina
tion under anesthesia), myringotomy with placement of
ventilating tubes remains the mainstay of treatment for this
difficult problem.25
Patient presentation
Cleft palate with cleft lip and alveolus
Although the primary palate and the secondary palate form
at different stages of embryonic development, cleft palate is
most commonly seen in combination with cleft lip. The alveo
lar portion of the cleft lies between the maxillary lateral incisor
and canine tooth roots. This results in malposition of the
maxillary lateral incisor and cuspid in both the deciduous and
permanent dentition.26,27 The maxillary lateral incisor on the
cleft side is absent in 80–90% of cleft patients; when present,
it may be smaller than the contralateral tooth or significantly
dysmorphic27–29 Absence of other teeth is not uncommon,30 but
linked the presence of a cleft palate to abnormalities in Eusta
chian tube function. In multicenter studies, incidence of otitis
media effusion has been found to be 96–100% in cleft palate
patients, measured by both middle ear effusions on otoscopy
and impedance testing and middle ear aspiration.16,17 In the
cleft palate, impairment of tubal dilation is thought to occur
from complex misalignment of the paratubal musculature.18,19
A
Aponeurosis
Hamulus
Tensor muscle
Levator muscle
Uvulus muscle
Fig. 23.1 (A) Normal anatomy: The levator veli palatini muscle can be seen
forming a sling across the soft palate; the tensor veli palatini is shown coming
around the hamulus to fuse with the levator. (B) Cleft palate: The muscles are seen
running more or less parallel with the cleft margin.
B
Aponeurosis
Hamulus
Tensor muscle
Accessory muscle
Levator muscle
567
Patient presentation
it may be related to the surgery itself because adults who have
not been operated on do not show the same patterns of
hypodontia.31 Asymmetry is common, resulting in alterations
in first maxillary molar position.32–34 In comparison to non-
cleft individuals, patients with unilateral cleft lip and palate
have overall reduction in crown size35 and delay of eruption
of permanent dentition.36 These findings support the theory
of global dental growth potential disturbance associated with
clefting. Interestingly, eruption of deciduous dentition is not
significantly delayed.37
Unilateral complete cleft palate is characterized by direct
communication between the entire length of the nasal passage
and oropharynx (see Fig. 23.3D). Absence of a portion of the
inferior piriform aperture, along with hypoplasia of the lateral
nasal bony platform at the maxillary wall, contribute to the
cleft nasal deformity; the nasal base is depressed, the ala col
lapses, and the floor widens. The superior and posterior nasal
septum is deviated and buckled toward the cleft side, and
when viewed intraorally from inferiorly, the vomer and
caudal septum slants towards the non-cleft side. The soft
palate muscles are discontinuous in the midline and insert
along the cleft margins in an abnormal manner. The unilateral
complete cleft is thus a full-thickness palatal defect of nasal
mucosa, bony palate, velar musculature, and oral mucosa; all
of these deficiencies must be addressed during the cleft palate
repair or later at the time of alveolar cleft bone grafting.
In the bilateral complete cleft lip and palate, the premaxil
lary segment, containing the central and lateral incisor tooth
roots, is discontinuous from the alveolar arch. The lateral
segments often collapse inward and lingually, resulting in
“locking out” of the premaxilla. Preoperative management
with presurgical infant orthopedics (PSIO) may help prevent
or treat lateral segment collapse and correct the anterior posi
tion of the premaxilla. If untreated, the anterior location of the
premaxilla in this situation may result in anterior fistulas,
which may in turn cause significant speech problems and
nasal regurgitation of fluid. Later orthodontic treatment of the
maxillary arch can expand the lateral segments before bone
grafting; however, repositioning of the premaxilla at this age
is usually not possible.
Kernahan and Veau proposed standardized reporting
schemes, each of which is commonly used to distinguish the
variable presentations of clefts of the palate, shown in
(Fig. 23.2
). Clinical examples representing the spectrum of
palatal clefts are shown in Fig. 23.3.
Fig. 23.2 appears online only.
Clefts of the secondary palate
Also called incomplete cleft palate, a cleft of the secondary
palate may be variable, from an opening in the posterior soft
palate to a cleft extending up to the incisive foramen (see Fig.
23.3B,C). There is almost always a separation of the bony
shelves of the hard palate, which is variable but may extend
anteriorly to the incisive foramen. Most commonly, dentition
is normal and symmetric (see Fig. 23.3C).
Submucous cleft palate
Submucous cleft palate occurs when the palate has mucosal
continuity but the underlying levator palatini muscle is dis
continuous across the midline and longitudinally oriented,
similar to the muscle anatomy in overt clefts of the palate.
Calnan’s classic triad of a midline clear zone (zona pellucida),
a bifid uvula, and a palpable notch in the posterior hard palate
is diagnostic of this condition. With contraction of velar
musculature, a distinct midline muscle diastasis may be seen
(see Fig. 23.3A).
The significance of a submucous cleft may be difficult to
assess clinically; the child with submucous cleft palate is often
undiagnosed in infancy. In a study screening a large popula
tion of schoolchildren in Denver, the overall incidence of
submucous cleft palate in the population was found to be
1 : 1200.38 Another similar cohort study reported that 45–55%
of patients with isolated submucous cleft palate were symp
tomatic with regard to speech, serous otitis media, or hearing
loss.39,40 In a large series of children with submucous cleft
palate in Mexico, examination with fiberoptic nasoendoscopy
showed about one-third to have velopharyngeal insuffi
ciency.41 Other attempts to identify risk of speech problems
with submucous cleft palate have been problematic because
referrals to cleft palate clinics are usually based on the iden
tification of speech issues rather than of the submucous cleft
itself. Therefore, an infant identified with submucous cleft
palate need not routinely undergo repair because a significant
number of individuals with submucous cleft palate will not
develop velopharyngeal insufficiency. Rather, these patients
should be closely monitored with serial speech evaluations
and audiometric surveillance.
Patients who present with velopharyngeal insufficiency
and submucous cleft palate on examination require full evalu
ation, including speech evaluation and endoscopy.42 Even in
the absence of obvious findings on clinical examination,
anatomic abnormalities are found in most patients (>90%) at
the time of surgery43,44; thus, the so-called “occult submucous
cleft palate” is simply one that is less obvious on clinical
evaluation. There will still be rare patients, however, who
have true palatopharyngeal disproportion and present with
velopharyngeal insufficiency without any cleft, submucous or
otherwise.
Corrective surgical technique for submucous cleft palate is
focused on anatomic correction of the velar muscle diastasis.
Although pharyngeal flaps and sphincter pharyngoplasty
have been proposed as primary means of treatment,41 most
surgeons focus on repair of the abnormal levator muscle posi
tion.45 The Furlow double opposing Z-plasty (see below) is an
ideal procedure for patients with isolated submucous clefts
and velopharyngeal insufficiency in the absence of 22q dele
tion syndrome, because there is no width discrepancy to be
overcome (Fig. 23.4).46 This avoids the potential for nasal
obstruction and sleep apnea in these patients if a pharyngeal
flap is used.
Pierre Robin sequence
Pierre Robin described the triad of micrognathia, glossoptosis,
and respiratory distress.47 Of the children diagnosed with
Pierre Robin sequence, 60–90% have cleft palate48,49; the palatal
cleft is usually isolated to the velum and can be “V” shaped
or, more typically, “U” shaped.50,51 In the past, this has been
thought to be secondary to hyperflexion of the head in utero
with resultant displacement of the tongue between the palatal
shelves, preventing their fusion. More recently, extensive
analysis of multiple syndromes associated with Pierre Robin
sequence has delineated genetic associations, indicating that
567.e1
Patient presentation
Fig. 23.2 Kernahan’s (A) and Veau’s (B) classification of clefts allows for
standardized reporting of the severity of both cleft lip and palate.
9
10
11
Hard
palate
Soft
palate
Nose
1
2
3
5
6
7
4
8
Lip
Nasal floor
Alveolus
Hard
palate
A
Veau classification
The Veau classification system divides into 4 groups, which are
as follows and illustrated below
Group I – defects of the soft palate only
Group I – defects involving hard
palate and soft palate
Group IV – complete bilateral clefts
Group III – defects involving the soft palate to
thee alveolus, usually involving the lip
B
568
SECTION I
CHAPTER 23 • Cleft palate
Fig. 23.3 Cleft palate spectrum. (A) Submucous cleft palate. (B) Incomplete
cleft of the secondary palate involving mainly the soft palate. (C) Incomplete
cleft involving the entire secondary palate extending to the incisive foramen.
(D) Left unilateral complete cleft lip and palate. Note vomer contiguous with the
right maxillary segment. (E) Complete bilateral cleft lip and palate. Vomer is
straight, in the midline, and separated from both maxillary segments.
B
D
A
C
E
569
Patient presentation
adhesion has been used as an alternative to tracheostomy
and is generally effective.58,59 More recently, mandibular dis
traction osteogenesis has been used in neonates with success
in averting tracheostomy.60 Although definitive long-term
outcomes with regard to mandibular growth and dental
development are not yet available, existing outcomes data
do not suggest major deleterious effects of neonatal distrac
tion on mandibular growth and dental development.61,62 In
all cases, if management is focused on the upper airway,
bronchoscopy should be performed to rule out any intrinsic
subglottic problems (e.g., laryngomalacia) that might neces
sitate tracheotomy.
the etiology may not be such a simple mechanical event.52–54
Infants with Pierre Robin sequence also have increased inci
dence of associated anomalies, particularly cardiac and renal
problems.55
Newborns with Pierre Robin sequence may have severe
respiratory and feeding difficulty because of the posterior
displacement of the tongue. Initial treatment consists of
placing the child prone and use of gastric lavage feeding
tubes to push the tongue forward.56 Nasal airways have been
used for the same purpose with reported success rates
of 80–90%.57 If these conservative measures fail, surgical
management of the airway may be required. A tongue–lip
Fig. 23.4 Submucous cleft palate. (A) Note bifid uvula and thinning of central
palate. On palpation, there is a notch in the posterior hard palate rather than a
posterior nasal spine. (B) Furlow double opposing Z-plasty repair of submucous cleft
palate. (C) Appearance of double opposing Z-plasty after repair.
A
B
C
570
SECTION I
CHAPTER 23 • Cleft palate
developmental delay, and various cardiac conditions. The
same deletion gives rise to DiGeorge syndrome with associ
ated B-cell and immune dysfunction; these children all need
appropriate immunology referral and follow-up. Most chil
dren with 22q deletions do not have overt cleft palate and may
not even have submucous clefts; usually the velar dysfunction
is related to absence of movement of an otherwise normal
soft palate. This may complicate decision-making regarding
surgery for speech in these patients, and soft palate proce
dures alone are generally not sufficient to correct the velopha
ryngeal insufficiency.
Growth
At birth, the average weight is the same for cleft and unaf
fected newborns.68,69 However, cleft infants have been shown
to exhibit poor weight gain in early infancy. Studies observing
infants with cleft lip and palate show initial growth retarda
tion by the time they undergo surgical lip repair. When the
same children reach the age for palatoplasty, they have sig
nificantly lagged on the growth curve. However, longitudinal
studies show that after repair of the palate, average growth
returns to normal compared with unaffected children by the
age of 4 years. Stratification of risk within the longitudinal
cohort shows pronounced growth retardation in associated
syndromes and also in children with isolated clefts of the
secondary palate.70,71
Children with orofacial clefting stabilize and continue
normal growth to at least 6 years of age, with no statistically
significant differences in height and weight when compared
to unaffected children. In later childhood, however, average
weight and height of children with cleft appear to diminish
compared with those of control subjects. In a Danish study of
skeletal maturity assessing both body height and radius
length as an index of growth in male cleft patients, onset of
puberty was found to be delayed on average by 6 months,
and the velocity of skeletal growth during puberty was
blunted. However, duration of puberty and pubertal skeletal
growth were prolonged an average of 1 year, resulting in final
attained body height and radius length the same as those of
control subjects.72
Numerous causes are thought to contribute to these differ
ences in growth patterns. There are certainly feeding difficul
ties early in life before palate repair,73 but it has also been
suggested that intrinsic growth disturbances may be respon
sible for slow postnatal growth.74 The increased frequency of
ear and airway infections has been implicated in early growth
retardation,68 as have the multiple operative procedures that
these children undergo.73 Adding more confusion to the etiol
ogy is the fact that growth hormone levels may be diminished
in cleft children. In summary, any growth disturbance is likely
to be of multifactorial origin throughout infancy, childhood,
and puberty. Fortunately, families can be counseled that most
children attain norms of height, weight, and development.
Feeding and swallowing
The intact palate provides a barrier between the respiratory
tract and the alimentary tract. To understand the difficulty
cleft infants have with feeding, one must understand the
normal role of the palate in sucking and swallowing. Oral
intake is divided into two separate activities, generation of
suction force (negative intraoral pressure) and swallowing.
Palatoplasty in children with Pierre Robin sequence must
be carefully timed with growth of the child, particularly the
mandible. There is some controversy about “catch-up” growth
of the mandible in children with Pierre Robin sequence; basi
cally, there is good documentation of extra growth in the first
year of life,63,64 but growth is subsequently commensurate
with that of normal children. Children with syndromes tend
to have less growth than in non-syndromic cases. Late cepha
lometric evaluation has consistently shown that the children
with Pierre Robin sequence have smaller mandibles than
normal.65 Closure of the palate narrows the effective area for
respiration and can lead to respiratory distress. If the mandible
attains reasonable size in the first year of life, palate repair can
still be performed safely before 1 year of age. In the rare
patient who has previously undergone tracheostomy, the
palate should be repaired before decannulation. The risk of
airway compromise after palatoplasty reaches 25%, with an
emergent tracheostomy or reintubation rate of 11% at one
institution.66 However, careful preoperative evaluation incor
porating the use of polysomnography and upper endoscopy
informs palate repair decision-making and minimizes risk.
Syndromes
Multiple malformations or syndromes have been found fre
quently in cleft patients.67 Cleft palate without associated cleft
lip has been reported to be associated with a syndrome in as
many as 50% of cases, while cleft lip and palate together have
an incidence of syndromes of about 30%. Van der Woude
syndrome is associated with a mutation in the interferon regu
latory factor 6 (IRF6) gene that also causes popliteal pterygium
syndrome; this is an autosomal-dominant syndrome associ
ated with lower lip sinus tracts (“lip pits”), and has variable
penetrance including the full range of cleft lips as well as
palates.
Children with cleft palate associated with an identified
syndrome must be evaluated thoroughly and have individu
alized planning and timing of therapy. Infants with a profound
developmental delay and severely shortened life span projec
tion should have surgical intervention delayed or should
undergo palatoplasty under special circumstances only. In
addition, syndromic children may have increased incidence
of cardiac anomalies, requiring specific anesthetic and post
operative considerations. Repair of cleft palate in the hope
that this will stimulate or allow a severely disabled child to
speak gives unreasonable expectations and hope to parents;
as noted before, it is critical to explain that palate repair may
aid speech production but not speech development. The
majority of syndromic infants with cleft palate can attain
adequate weight gain and growth with proper nutrition and
feeding assistance. Therefore feeding benefit associated with
palate repair is often outweighed by the risk of airway com
promise and absence of speech-related indications for repair.
Palate repair in severely disabled children can lead to altered
airway status and obstructed upper airway in those with
neuromuscular delay.
22q chromosomal deletion
Velocardiofacial syndrome, which is associated with 22q
chromosomal deletions, is diagnosed by fluorescent immuno
hybridization (FISH). These children have a characteristic
“bird-like” facial appearance, velopharyngeal dysfunction,
571
Patient presentation
ulceration. In the older child, a persistent communication
through a fistula in the palate may result in regurgitation of
food through the nose, which is socially unacceptable.
Weight gain and skeletal growth confirm success of the
feeding regimen. Once the palate is successfully closed surgi
cally, special feeding methods are generally unnecessary.
Speech
The primary goal of palatoplasty is normal speech. Patients
can grow and even thrive, despite feeding difficulties, but
speech cannot be normalized if the palate is not repaired. The
ability to partition the oropharynx and nasopharynx is crucial
for normal speech production. The palate elevates during
production of any sounds requiring positive pressure in the
oropharynx; the levator palatini is primarily responsible for
this movement (Fig. 23.6).
Speech is a complex issue, and many factors may influence
speech development in a child with a cleft palate. In addition
to the importance of the palate itself, speech development
may be influenced by motor or neurologic developmental
delay (often seen in syndromes), by hearing, and by environ
mental stimuli. It is important to distinguish between speech
production, which is primarily dependent on normal ana
tomic function, and speech development, which is influenced
more by global developmental issues. Speech production also
requires normal articulation, which in turn depends on tongue
placement, lip competence, and dental position, all of which
may be abnormal in cleft patients.79
If palate function is not corrected, velopharyngeal insuffi
ciency results, with speech that is hypernasal, often with
hoarse quality due to difficulty in directing airflow through
the mouth. When complete closure cannot be anatomically or
functionally obtained, compensatory mechanisms for sound
production are learned. These are maladaptive patterns that
interfere with global intelligibility and include glottal stops
For negative intraoral pressure to be produced, the velum
seals off the pharynx posteriorly; the lips close anteriorly, and
negative pressure is produced by moving the tongue away
from the palate and by opening the mandible. This effectively
increases the intraoral volume within a closed system, result
ing in generation of negative pressure. If the individual is
unable to close the nasopharynx or to generate a seal of the
lips, or if the palate is not intact at the point of contact with
the tongue, negative pressure cannot be produced. This failure
of velopharyngeal closure is the basis of difficulty with breast
feeding or normal bottle-feeding in the patient with cleft
palate.
Suction on the nipple in a breastfeeding infant is thought
to stabilize the nipple position while motion of the tongue
against the nipple pushes fluid into the mouth. Ingestion
through an artificial nipple is different; infants learn to
manipulate the nipple and flow rates by closing the alveolus
against the artificial nipple. Tongue motion is primarily used
to transfer the bolus to the pharynx for swallowing. In the
cleft infant, the communication between the oropharynx and
nasopharynx prevents a seal of the tongue against the palate,
and negative pressure cannot be generated. Suckling is there
fore not productive, and breastfeeding is ineffective.
Most infants with clefts are unable to breastfeed. Infants
with clefting limited to the posterior velum can often use
posterior tongue position to generate a partial negative seal.
The exception to this is the child with Pierre Robin sequence
and isolated velar cleft, who can develop respiratory distress
or ineffective suction from glossoptosis. Obviously, patients
with isolated cleft lip and an intact palate should and do have
little difficulty with breastfeeding.
Infants who are unable to breastfeed because of cleft palate
have a number of options for feeding. Regimens and devices
have evolved to nourish the infant, including specialty nipples
such as lamb’s nipples, crosscutting of standard nipples, and
long soft nipples that place the liquid at the posterior tongue.
Special flow bottles such as gravity flow and squeeze bottles
allow the caregiver to carefully control the flow rate (Fig.
23.5). The strategy of each technique is low resistance to flow,
with controllable flow rate for optimal volume and minimal
effort by the infant. All of these techniques are effective, and
selection is generally by personal preference and the baby’s
acceptance of the method. Other key considerations are ele
vated head positioning during feeding and careful observa
tion of feeding time and volume ingested.75–78
Swallowing involves a complex interaction of the tongue
and pharynx. Coordinated swallowing is dependent on neu
romuscular control and rhythmic coordinated contraction of
the tongue and pharynx. Children with clefts generally do not
have difficulty with swallowing and aspiration unless intrin
sic neuromuscular abnormality of the tongue or pharynx is
present. It is an error to ascribe aspiration simply to the pres
ence of a cleft palate; indeed, aspiration with swallowing
should stimulate an appropriate diagnostic evaluation,
including thin barium swallow studies, bronchoscopy, and
gastroscopy. Children may cough or sputter with reflux of the
ingested material into the nose, particularly if volume or rate
of feeding is excessive. During normal deglutition, the tongue
moves the food bolus to the pharynx by complex interaction
of tongue against palate. When the palate has an open cleft,
food may reflux into the nasal passage. Nasal reflux is irritat
ing to the nasal mucosa and can predispose to sinusitis and
Fig. 23.5 Specialized bottles for cleft palate feeding. Left, Haberman feeder with
reservoir in continuity with nipple. Right, Mead–Johnson feeder, which requires
squeezing the bottle to improve flow of fluid.
572
SECTION I
CHAPTER 23 • Cleft palate
A
9
9
3:
3:
Fig. 23.6 (A) In the lateral cephalogram, the soft palate or
velum (“V”) is shown at rest (above left) and during
speech, making contact with the posterior pharyngeal wall
(“PW”) (above right). (B) This line drawing shows the air
flow during normal speech (left), with the velum making
contact with the posterior pharynx to direct air out the
mouth. If the velum is too short or movement is inadequate
(right), air can escape through the nose during speech,
creating velopharyngeal insufficiency.
B
and pharyngeal fricatives. In addition, tongue placement for
various phonemes is altered to achieve the most normal
production of sounds. Eliminating these learned compensa
tory articulations is difficult, even with the best of speech
and language therapy. Compensatory articulations may
persist even in the face of a functional palate repair, especially
in later repairs or secondary correction of velopharyngeal
insufficiency.
Timing of palate repair
The normal palate serves several important functions, all
related to functional separation of the oropharynx from the
nasopharynx. Whereas the sine qua non for palate repair is
normal speech, consideration of cleft palate surgery must
balance developmental, dentofacial growth, and otologic
issues as well.
Speech
The driving force for palatoplasty is the development of
normal speech. Two crucial aspects of palatoplasty are impor
tant in optimal speech outcome: (1) surgical technique and (2)
timing of palate repair. Victor Veau11 first made the observa
tion of a correlation between age at repair and speech outcome
in 1931. He noted that children who had undergone repair
before 12 months of age were much more likely to have
normal speech than those with repair between 2 and 4 years
of age. Children who underwent repair after 9 years of age
had the worst speech outcome. Adults with unrepaired cleft
palate (see Fig. 23.3C) have little or no possibility of neuromo
tor adaptation to a newly repaired palate and unfortunately
do not exhibit significant speech gains. Therefore palate repair
in adults is indicated for correction of nasal regurgitation
alone. The optimal time of palatoplasty still remains scientifi
cally unproven. Confounding variables of technique, sur
geon’s skill, lack of standardization of speech evaluations,
and therapies preclude exact determination of optimal age at
repair.80
Most would agree that the best speech results are correlated
with closure of the palate near the time of the infant’s begin
ning language acquisition, which for the normal-developing
child is before 12 months of age.81,82 Indeed, there is a body of
evidence that phonologic development actually begins earlier,
at 4–6 months of age.83,84 Most studies of the timing of palatal
repair have looked at secondary outcomes; increased compen
satory articulations were shown in one study and increased
need for pharyngeal flaps in another when the palate was
repaired after 1 year of age. Although repairs before 1 year of
age are now common, this is not a rigid chronologic milestone;
rather, repair should be related to the child’s speech develop
ment. Some studies have shown that if correction occurs even
as late as 21 months, compensatory maladaptive patterns are
infrequent. Despite the absence of hard evidence supporting
earlier palate repairs, a growing body of opinion seems to
support palate repair around 9–10 months of age for children
573
Treatment/surgical technique
commonly used instrument for exposure, compresses the
tongue and causes ischemia; if it is used for longer than 2 h,
significant postoperative tongue swelling can occur. Lidocaine
0.5% and epinephrine 1 : 200 000 are infiltrated into the palate
7–10 min before incision; use of a 3-mL syringe makes the
injection into the hard palate somewhat easier than with a
larger syringe. A maximum of 1 mL/kg is used.
Palate repair is performed with the surgeon at the child’s
head, with use of a fiberoptic headlight or retractor. A rolled
towel under the shoulders will extend the neck; it is important
to assure that the child does not have any syndromes that
predispose to cervical spine anomalies. The use of curved
needle holders facilitates suture placement without obstruc
tion of vision.
The most important aspect of surgical anatomy is the loca
tion of the greater palatine neurovascular bundle, which
emerges through the greater palatine foramen through the
lateral posterior hard palate. Incisions on each side are best
made with the surgeon’s contralateral hand to bevel the inci
sion away from the vascular pedicle. Circumferential freeing
of the palatal attachments around the pedicle and gentle
stretching of the pedicle out of the foramen can be essential
to obtain a tension-free closure of the oral flap.103 In general,
the goal is to obtain complete nasal and oral closure from front
to back. This may not be possible in wide clefts, particularly
on the nasal surface, or in cases of complete clefts with poorly
aligned alveolar segments. The most difficult area for closure,
around the junction of the hard and soft palate, is the most
common location for fistulas.
Hard versus soft palate closure
It is easier to understand cleft palate surgery by separating
techniques used for the hard palate from those used for the
soft palate. In general, all techniques use some form of muco
periosteal flap for the hard palate closure; the soft palate
repair emphasizes correction of the abnormal position of the
levator palatini muscles. The location of the incision along the
cleft margin can be varied to include more or less mucosa to
be turned over for nasal lining. In the discussion below, the
hard palate techniques are discussed first followed by soft
palate closure.
von Langenbeck
Bernhard von Langenbeck introduced the use of mucoperios
teal flaps to close clefts of the secondary palate in the late
1800s. The initial description of the technique involved a
simple approximation of the cleft margins with a relaxing
incision that began posterior to the maxillary tuberosity and
followed the posterior portion of the alveolar ridge. Some
variation of the Langenbeck repair is still used commonly for
clefts of the secondary palate. Intravelar veloplasty, or repair
of the levator palatini muscle, as described below, is added
today to reproduce the normal muscle sling (Fig. 23.7).
V-Y pushback (Veau–Wardill–Kilner)
George Dorrance (1877–1949) of Philadelphia realized that a
distinct number of patients with cleft would develop velopha
ryngeal dysfunction caused by inability of the soft palate to
touch the posterior pharyngeal wall.104,105 In fact, he advocated
muscle transposition but did so by fracturing the hamulus,
with apparently normal development.85,86 Very early repair of
the palate (6 months or younger) has been proposed by some
surgeons,87–90 primarily as a means of improving feeding;
however, long-term results are lacking for any large cohort of
these patients. Prospective longitudinal assessment is cur
rently in progress to attempt to better define the optimal
timing of cleft repair.
Maxillary growth
Palatoplasty is generally assumed to detrimentally affect
maxillary growth, and evidence in support of this is that
cephalometric analysis of adults with unrepaired cleft palate
has shown normal maxillary dimensions and growth.91,92
There is experimental evidence that the lip repair may restrict
sagittal growth of the maxilla,93,94 but in most patients it seems
that the palate repair is more significant. Many children with
repaired cleft palate display typical findings of transverse
maxillary deficiency requiring orthodontic widening of the
maxilla once permanent teeth have erupted.
Transverse growth of the maxillary arch is narrowed in
comparison with that in non-cleft patients, resulting in typical
malocclusion traits of crowding, lateral crossbite, and open
bite.32,95–97 Whether the narrowed arch and maxillary growth
inhibition result from surgical scarring98,99 or intrinsic maxil
lary underdevelopment remains a matter of debate; most
likely it is a combination of the two. There may be a sagittal
growth deficiency as well; whereas 35–40% of children will
develop an anterior crossbite, as many as 15–20% of children
with cleft palate go on to require a Le Fort I maxillary advance
ment in some series. There is some evidence that the develop
ment of crossbite and maxillary hypoplasia may be related to
the severity of the original cleft.100,101
Although it might seem preferable to wait until a more
advanced age for palate repair, given the growth effects on
the maxilla, it is far more difficult to establish normal speech
in older children after cleft repair than to correct occlusion
with a combination of orthodontic treatment and orthognathic
surgery.
Treatment/surgical technique
Technical considerations
A number of perioperative considerations must be addressed
regardless of the type of repair used. The general health and
the developmental status of the child play a role in the timing
of the palate repair and are also important for anesthetic and
surgical management. Audiology evaluation is routinely
obtained preoperatively so that the otolaryngologist can place
ventilating tubes in the tympanic membranes if indicated,
saving the child an additional anesthetic. Most surgeons use
preoperative antibiotics, although consensus is lacking. One
recent randomized, placebo controlled study demonstrated a
lower fistula rate with the use of additional postoperative
antibiotics.102
The use of a Ring–Adair–Elwyn (RAE) endotracheal tube
facilitates placement of the Dingman gag without kinking the
tube. The airway must be assessed constantly for problems; if
lower central teeth are present, this can be a source of tube
compression against the retractor. The Dingman gag, the most
574
SECTION I
CHAPTER 23 • Cleft palate
The essence of the pushback repair is the central “V” inci
sion on the hard palate that is then closed in a straight line,
creating length on the oral side of the closure (Fig. 23.8). The
initial description included osteotomy of the posterior hard
palate at the greater palatine foramina to release the palatine
vessels; circumferential dissection, with release of the perios
teum behind the vessels, was subsequently advocated to
stretch the vessels, which works as well with less risk of injury
which he believed would change the vector of muscle contrac
tion and in combination with techniques of Langenbeck
would lengthen the palate. He also advocated division of the
major palatine neurovascular bundles to assist with the push
back. Thomas Kilner (1896–1964) was important in develop
ment of the V-Y palate repair along with Victor Veau and
William E.M. Wardill (1893–1960). In addition, he pushed for
palate repair at an earlier age, 12–18 months.106
Fig. 23.7 Von Langenbeck repair. (A) Relaxing incisions are made behind the alveolar ridge, creating bilateral
bipedicle flaps for midline closure. The greater palatine vessels must be preserved. (B) The cleft margins are
incised in a manner to leave adequate nasal mucosa for complete closure. (C) Closure of the nasal mucosa and
muscle repair. (D) Final appearance.
A
B
C
D
Fig. 23.8 Pushback repair. (A) Design of anterior “Y” incision. (B) Elevation of bilateral mucoperiosteal flaps
based on palatine vessels. The levator veli palatini muscles are freed from the posterior border of the hard palate.
(C) The muscles are repaired across the midline of the soft palate. (D) The “Y” closure creates additional length
but also leaves large raw areas bilaterally.
A
B
C
D
575
Treatment/surgical technique
anteriorly in a complete cleft is a single layer of nasal mucosa
only, which gives rise to a higher fistula rate in pushback
repairs than in other techniques.110
Two-flap palatoplasty
Bardach and Salyer111 originally described a technique of
freeing mucoperiosteal flaps from the cleft margins only,
arguing that the arch of the cleft would provide the length
needed for central closure. This is certainly not a universal
finding, and it is probably most applicable in relatively narrow
clefts. The more extensive two-flap palatoplasty is a modifica
tion of the Langenbeck technique, extending the relaxing
incisions along the alveolar margins to the edge of the cleft.
This designs flaps entirely dependent on the circulation from
the palatine vessels but also much more versatile in terms of
their placement. In a complete unilateral cleft, the flap from
the greater (medial) segment can be shifted across the cleft
and closed directly behind the alveolar margin. This virtually
eliminates fistulas in the anterior hard palate.112
The soft palate closure is accomplished with a straight line
closure in the typical two-flap technique. Intravelar veloplasty
is an essential part of this closure (Fig. 23.10).
Vomer flaps
There is confusion about the terminology applied to anterior
closure of the nasal mucosa in complete cleft lip and palate.
The original vomer flap is described as inferiorly based; an
incision is made high on the septum, and the flap is reflected
downward to provide a single-layer closure on the oral side.
A number of European centers noted a high number of
patients with maxillary retrusion, presumably from injury to
the vomer–premaxillary suture, as well as a high fistula rate,
and changed to a two-layer anterior closure.113–115
Similar problems have not been found with superiorly
based vomer flaps. This technique involves reflecting the
mucosa from the septum near the cleft margin, dissecting only
enough to close the nasal mucosa of the opposite side. In
bilateral cleft palate, this requires a midline incision along the
septum, and two flaps are reflected in each direction. This
technique results in a two-layer closure of the hard palate
mucosa, with low fistula rates and less effect on maxillary
growth.110
Intravelar veloplasty
Although Victor Veau first advocated midline reapproxima
tion of the levator palatini muscle, Braithwaite116 was the first
to perform more extensive muscle dissection for posterior
repositioning and tension-free approximation. He emphasized
careful dissection and freeing of the levator palatini from the
posterior edge of the hard palate before approximation in the
midline.
Cutting117 has described a technique of veloplasty that
includes division of the tensor palatini tendon at the hamulus
and repositioning of the muscle. This method, known as
radical levator transposition, requires an extensive dissection
of the levator muscle, freeing it from both nasal and oral
mucosa (Fig. 23.11). Although there is a reasonable thickness
of oral mucosa, the adherence of the nasal mucosa to the
muscle makes the dissection difficult on this side and at times
results in perforation of the mucosa. Sommerlad described the
to this vital blood supply.103,107 The nasal tissue is released and
left open; some authors have proposed providing nasal lining,
either with septal flaps108 or with buccal mucosa (Fig. 23.9).109
The soft palate is addressed with repair of the cleft margins
and transverse closure of the levator muscle.
The pushback technique has the advantage of providing
increased length for the palate and placing the levator muscle
in a more favorable position. Large open areas are left anteri
orly and on the nasal surface; as these close by contraction, a
good deal of the length gain is lost. Additionally, the contrac
tion of the oral mucosal defects results in loss of maxillary
width anteriorly, a situation more difficult to correct than
posterior maxillary narrowing. The arch may also be flattened
anteriorly, a difficult problem for the orthodontist. The closure
A
Fig. 23.9 (A) Buccal mucosal flap. Kaplan advocated use of this flap to elongate
the nasal mucosa. (B) Flap transposed into the nasal surface. In some situations
bilateral flaps can be used with the second flap lining the oral surface.
B
576
SECTION I
CHAPTER 23 • Cleft palate
Fig. 23.11 Cutting’s radical levator transposition. (A) The
levator veli palatini is freed completely from the posterior
hard palate as well as from nasal and oral lining. The tensor
tendon is cut medial to the hamulus to effect a complete
release of the muscular sling. (B) The muscles are repaired
in the midline after being transposed into a horizontal
position and situated in the posterior portion of the soft
palate.
Levator veli palatini
Tensor veli palatini
Point at which tensor
aponeurosis is divided
Cut tensor aponeurosis
Radical levator transposition
A
B
Fig. 23.10 Two-flap palatoplasty. (A) The incisions are similar to the Langenbeck repair but meet the cleft margin
just behind the alveolar ridge. (B) Mucoperiosteal flaps are developed on both sides with preservation of the greater
palatine vessels. (C) The levator veli palatini muscle is freed from the posterior border of the hard palate and
sutured across the midline. (D) Final closure. It is often possible to close much of the lateral incision and minimize
raw areas.
A
B
C
D
use of the microscope for dissection of the muscle to reduce
injury of the nasal mucosa. The blood supply of the levator veli
palatini is derived from the ascending palatine and ascending
pharyngeal arteries with generous collateralization.118 Precise
identification of the vascular pedicles is not performed during
cleft surgery and significant devascularization of the levator
is thought to be uncommon. However, the surgeon should
be aware that the majority of the velar blood supply enters
from the region lateral to the hamulus, in the space of Ernst,
and the region immediately posterior (through which the
motor innervation passes as well), and excessive dissection
in these areas is probably best avoided. The tensor tendon is
577
Treatment/surgical technique
Although Furlow described the use of relaxing incisions
when necessary, Peter Randall and other surgeons at The
Children’s Hospital of Philadelphia incorporated their use
routinely, with elevation of bipedicled mucoperiosteal flaps in
their modification of the Furlow procedure.124 The chief
problem may arise in very wide clefts, in which the distance
to be traversed by the Z-plasty may be excessive. The anteri
orly based oral flap can be joined to the relaxing incision to
design an island flap based on the greater palatine vessels and
giving considerably greater mobility, although this will shift
the closure to the side of the posteriorly based flap. Another
alternative is to extend the relaxing incision along the lateral
border of the soft palate to allow oral closure and to use
acellular dermal matrix for any nasal defects. A third alterna
tive is to employ a straight line closure in wide cases, reserving
the Z-plasty as a secondary procedure if needed for speech.
The rationale for the latter is that the added length gained
with Z-plasty in very wide clefts, come at the expense of
increased contracture by secondary healing laterally. As
the child grows, adequate soft tissue develops such that sec
ondary Z-plasty of a straight line repair is a relatively easy
procedure that results in significant improvement in muscle
position and palate function.
Direct comparison of Z-plasty and intravelar veloplasty
has not been done controlling for surgeon, cleft type, and
other significant variables. A review of published studies
showed overall better speech outcomes reported by groups
using the Furlow technique or its modifications.125 However,
there is a wide range of reported speech outcomes, and, as
discussed above, a number of surgeons have reported excel
lent speech results using intravelar veloplasty with straight
line repairs. Thus, available data to date have not led to a
consensus recommendation for either individual technique
over the other for all soft palate repairs. The authors’
released just medial to the hamulus, and the levator muscle is
overlapped to provide appropriate tension on the repair. This
is presumably similar to the muscle overlap accomplished by
Furlow’s double opposing Z-plasty method. This technique
has shown excellent speech outcomes in early evaluations.
Both Cutting and Sommerlad119 have proposed “re-repair” of
the levator muscle when primary palatoplasty still results in
velopharyngeal insufficiency.
Double opposing Z-plasty (Furlow)
Furlow120 first described his technique for palate closure in the
1980s, adapting the Z-plasty principle to palatal closure. By
alternating reversing Z-plasties of the nasal and oral flaps and
keeping the levator palatini within the most posterior flaps,
he reported initial early success in both speech outcomes and
skeletal growth.
A Z-plasty is developed on both the oral and nasal surfaces
of the soft palate but in opposite directions. For both of the
Z-plasties, the central limb is the cleft margin, and the poste
riorly based flap is designed to include the levator muscle.
Furlow recommended that the posteriorly based oral flap be
on the left side for a right-handed surgeon because the eleva
tion of the muscle from the nasal mucosa is the most difficult
part of the dissection (Fig. 23.12).121
This technique addresses closure of the soft palate in a
manner that provides complete nasal and oral closure as it
re-establishes the levator sling. Because the nasal Z-plasty is
placed more laterally, a higher and presumably more func
tional sling is formed. The theoretical disadvantage of this
technique is that it is non-anatomic, in that it completely
ignores the small longitudinal uvular muscle, but overall
speech results have been comparable to or better than those
with other techniques.122,123
A
B
C
Fig. 23.12 Furlow double opposing Z-plasty. (A) The oral
flap design is shown with the posteriorly based flap on the
left side. If necessary, the relaxing incisions can be
continued up to the cleft margin behind the alveolus, similar
to the two flap palatoplasty (Fig. 23.10) for the hard palate
closure. (B) The left-sided oral flap is raised with the levator
muscle, the right-sided flap above the muscle. The reverse
pattern is planned for the oral side. A vomer flap is shown
closing the nasal mucosa anteriorly. (C) The nasal flaps are
transposed and the anterior oral mucosa closed. (D) The
final appearance after transposition of the oral flaps. (E) A
two-flap palatoplasty can be combined with a Z-plasty of the
soft palate.
D
E
578
SECTION I
CHAPTER 23 • Cleft palate
A
B
Fig. 23.13 Authors’ general approach to technique selection. (A) Moderate width complete unilateral cleft lip and palate. (B) Two-flap palatoplasty combined with double
opposing Z-plasty. The two-flap approach gives maximum mobilization of the mucoperiosteal flaps and is particularly effective for minimizing the occurrence of an anterior
fistula. (C) Wide cleft of the secondary palate. (D) Two-flap palatoplasty with straight line soft palate closure and intravelar veloplasty.
C
D
preference for primary repair of complete clefts is to use a
two-flap technique for the hard palate combined with a
double opposing Z-plasty of the soft palate, or in the case of
very wide clefts, a straight line repair with a radical levator
transposition (Fig. 23.13). This approach has resulted in a very
low fistula rate126 and excellent speech results.
Two-stage palate repair
The problem of maxillary growth after cleft palate repair has
led some surgeons to advocate a two-stage approach to pala
toplasty, with earlier repair of the soft palate only and later
repair of the hard palate. The general protocol, originally
introduced by Schweckendiek and Doz,127 entailed repair of
the soft palate at the same time as the cleft lip repair, around
4–6 months. The hard palate was obturated and repaired at
about 4–5 years of age. Earlier ages have subsequently been
proposed for hard palate repair, usually around 18–24
months.128
The rationale for this approach has been that the hard
palate cleft narrows during the time between procedures,
requiring less dissection and thus resulting in less maxillary
growth disturbance. Although it is appealing on a theoretical
basis, numerous studies have shown significantly poorer
speech results from these two-stage procedures, with marginal
if any salutary effect on the growth of the maxilla.129–131 As
noted before, good speech outcomes become more difficult to
achieve with increasing age, whereas most maxillary growth
problems can be addressed with orthodontic treatment and,
if needed, surgical maxillary advancement.
579
Outcomes of cleft palate repair
a source of nasal regurgitation of fluids. In one particularly
extensive review of fistulas at a single institution, use of the
Furlow repair was shown to markedly reduce fistulas relative
to the V-Y pushback or von Langenbeck technique. The width
of the cleft was the other factor in this multivariate analysis.110
Late closure of fistulas may be difficult, especially in the hard
palate, and two-layer closure with lining turnover and the use
of large mucoperiosteal flaps or facial artery musculomucosal
(FAMM) flaps135 on the oral side are recommended to give the
best outcome. Closure of fistulas at the soft–hard palate junc
tion can also be incorporated into double opposing Z-plasty
palate-lengthening procedures when indicated for treatment
of velopharyngeal insufficiency (Fig. 23.14
).136 If there is
reason for delay of repair, a palatal plate may aid in obturating
a fistula for speech purposes until surgical correction can be
achieved.
Fig. 23.14 appears online only.
Speech outcomes/velopharyngeal
insufficiency
Normal speech is the primary goal of cleft palate repair.
Specifically this refers to the lack of velopharyngeal insuffi
ciency (VPI) and nasal air loss in speech. It can be difficult to
interpret some studies, as it is common that “good” and
“excellent” results are grouped together, or that the nasality
is rated on a numerical scale. In general, it is preferable to look
at the presence or absence of nasality and ideally to use a
binary outcome measure for analysis.
In the majority of studies in which some form of muscle
repair is utilized, good speech results are obtained about
85–90% of the time. These results are in non-syndromic cleft
patients; syndromic patients will always have poorer results
for a variety of reasons, but their good outcomes may be in
the 50–60% range. It appears that width of cleft is related
to outcome, as bilateral clefts often have worse speech
outcomes, but there have been few studies looking at this
in detail.
Some studies have examined the long-term stability of
speech outcomes. In some patients, nasality may develop in
puberty or later, probably related to involution of the adenoid
pad, but this is rare in our experience.
Maxillary growth
Normal maxillary growth is a secondary goal of palate repair.
While some orthodontists might contest this, it is much more
difficult to correct nasal speech after puberty than to perform
a LeFort I maxillary advancement. It is clear that avoidance
of large, raw surfaces on the hard palate will improve maxil
lary growth long term, and that minimizing scar tissue will
have a salutary effect as well. However, the use of relaxing
incisions has not been shown to independently affect maxil
lary growth or to induce posterior crossbite.137 Fistula forma
tion requiring additional procedures will increase scar tissue
and often decrease maxillary growth.
The need for maxillary advancement is highly variable,
from 10–40% in non-syndromic cleft patients. Because differ
ent centers have different surgeons as well as different proto
cols, it is difficult to tease out the critical differences in practice
that might account for these variations in outcome. Wider
clefts and bilateral clefts have a higher rate of maxillary
Postoperative care
In the immediate postoperative period, breathing is the criti
cal concern. The child with an open cleft palate has become
accustomed to a larger than normal nasal airway, which is
virtually occluded after cleft palate repair, especially with the
introduction of a small amount of blood or mucus in the nose.
Postoperative hypoxemia is not uncommon, but generally
resolves after 24–48 h.132 The use of a traction suture in the
tongue during the immediate period after extubation may
avoid the need for utilizing any oral devices for maintaining
the airway. Some centers use nasal trumpets routinely to
improve ventilation. Monitoring with continuous pulse oxim
etry and minimizing narcotic use will help to avoid cata
strophic problems. Acetaminophen 15 mg/kg alternating
with ibuprofen 10 mg/kg will usually give adequate pain
relief. Recently, use of intravenous acetaminophen has been
associated with decreased opioid requirements.133 Any patient
who has had prolonged surgery (over 2 h) with the mouth
gag in place should be observed for at least 48 h for tongue
edema. Some surgeons routinely release the mouth gag at set
intervals throughout the procedure, analogous to releasing
the tourniquet during hand surgery.
Children with Pierre Robin sequence are a special group.
These and any other children with syndromes that may affect
breathing must be observed closely, even in an intensive care
unit setting, to be sure that there is no significant respiratory
obstruction. Most Pierre Robin children stay additional time
in the hospital for this reason.
Bleeding is not uncommon after palate repair. There are
inevitably raw surfaces, which may ooze for 12–24 h. Hemo
static rolls (e.g., Surgicel) are sutured into raw areas by some
surgeons to reduce postoperative bleeding (see Fig. 23.13B,D).
Bleeding can also be reduced by surgery that takes less than
90–120 min because the epinephrine will still have some effect
during emergence from anesthesia. Light pressure on the hard
palate repair at the conclusion of the procedure will often
control bleeding when necessary. The authors have found that
application of ice packs to the posterior neck is almost always
effective in stopping postoperative bleeding in recovery or on
the ward; this is a technique that has not been documented
previously but has been used by experienced surgeons.134
Postoperative feeding is generally limited to liquids for
10–14 days, to prevent particulate matter lodging in the areas
that are left open at the end of the procedure. Reducing or
stopping intravenous fluids the morning after surgery causes
some thirst, and almost all patients start to take adequate
liquids under these circumstances. The parents must learn to
time feeding 30 min or so after analgesic administration.
Arm splints may be used as well to prevent children from
putting their fingers or, more likely, foreign objects in the
mouth.
Outcomes of cleft palate repair
Fistula
Fistula formation is one complication that has been studied
in some detail. Fistulas may be a source of persistent nasal air
loss even in the face of a functioning soft palate; they are also
579.e1
Outcomes of cleft palate repair
A
B
C
D
E
F
Fig. 23.14 Palatal fistulas. (A) Fistula in hard palate. (B) Large mucoperiosteal flap for oral closure. (C) Repaired fistula. (D) Fistula at junction of hard and soft palate. (E)
Fistula closure incorporated into double opposing Z-plasty palate lengthening. (F) Fistula in anterior hard palate and alveolus.
579.e2
SECTION I
CHAPTER 23 • Cleft palate
G
H
(G) Left facial artery musculocmucosal (FAMM) flap for oral closure. (H) Repaired fistula.
Fig. 23.14, cont’d
580
SECTION I
CHAPTER 23 • Cleft palate
from the other specialists involved in cleft care. The increased
application of methods that incorporate reconstruction of the
levator palatini muscle has produced much more predictable
speech results. Current trends of earlier surgical intervention
for cleft palate and presurgical alignment of the dental arches
should result in still more predictable outcomes.
hypoplasia, possibly related to greater need for dissection at
the time of palate repair.138
It is clear that syndromic patients have a higher rate of
maxillary hypoplasia, which may well be genetically deter
mined. In our review of van der Woude syndrome, the need
for maxillary advancement was about 85% compared to less
than 10% in a matched group of non-syndromic unilateral
cleft patients.
Conclusion
Cleft palate repair has undergone major changes in the past
quarter century. Overall, results have improved as far as
speech outcomes; this is probably due to the growth of centers
for cleft care as well as to refinement of techniques. The team
approach has decreased the number of operations needed to
obtain better outcomes as the surgeon has gained knowledge
Fig. 23.2 Kernahan’s (left) and Veau’s (right) classification of clefts allows for
standardized reporting of the severity of both cleft lip and palate.
Fig. 23.14 Palatal fistulas. (A) Fistula in hard palate. (B) Large mucoperiosteal
flap for oral closure. (C) Repaired fistula. (D) Fistula in anterior hard palate and
alveolus. (E) Left facial artery musculomucosal (FAMM) flap for oral closure.
(F) Repaired fistula. (G) Fistula at junction of hard and soft palate. (H) Fistula
closure incorporated into double opposing Z-plasty palate lengthening.
Bonus images for this chapter can be found online at
14. Huang MH, Lee ST, Rajendran K. A fresh cadaveric study of the
paratubal muscles: implications for Eustachian tube function in
cleft palate. Plast Reconstr Surg. 1997;100:833–842. Cadaveric
dissections were performed to clarify possible ramifications of palatal
clefting on Eustachian tube function. Functional hypotheses are drawn
from morphological findings.
44. Kaplan EN. The occult submucous cleft palate. Cleft Palate J.
1975;12:356–368.
46. Chen PK-T, Wu J, Hung KF, et al. Surgical correction of sub-
mucous cleft palate with Furlow palatoplasty. Plast Reconstr Surg.
1996;97:1136–1146. Sleep apnea is a recognized adverse outcome of
pharyngeal flaps performed for velopharyngeal insufficiency (VPI). This
report demonstrates that Furlow palatoplasty is a reliable alternative to
pharyngeal flaps for the correction of VPI in the context of submucous
cleft palate.
47. Robin P. Glossoptosis due to atresia and hypotrophy of the
mandible. Am J Dis Child. 1934;48:541–547.
60. Denny AD, Talisman R, Hanson PR, et al. Mandibular distraction
osteogenesis in very young patients to correct airway obstruction.
Plast Reconstr Surg. 2001;108:302–311. This clinical series correlates
airway measurements before and after distraction with functional
outcomes. The authors conclude that distraction improves tongue base
position such that airway space is effectively increased.
63. Figueroa AA, Glupker TJ, Fitz MG, et al. Mandible, tongue, and
airway in Pierre Robin sequence: a longitudinal cephalometric
study. Cleft Palate Craniofac J. 1991;28:425–434.
94. Bardach J. The influence of cleft lip repair on facial growth. Cleft
Palate J. 1990;27:76–78.
117. Cutting C, Rosenbaum J, Rovati L. The technique of muscle repair
in the soft palate. Operative Techniques Plast Surg. 1995;2:215–222.
120. Furlow LT Jr. Cleft palate repair by double opposing Z-plasty. Plast
Reconstr Surg. 1986;78:724–738. Furlow describes his palatoplasty in
the context of a 22-patient case series. Optimistic speech outcomes are
reported.
128. Rohrich RJ, Byrd HS. Optimal timing of cleft palate closure.
Speech, facial growth, and hearing considerations. Clin Plast Surg.
1990;17:27–36.
136. Emory RE Jr, Clay RP, Bite U, et al. Fistula formation and repair
after palatal closure: an institutional perspective. Plast Reconstr
Surg. 1997;99:1535–1538. The authors report an 11.5% post-palatoplasty
fistula rate. Local flaps are advocated to repair these lesions.
Access the complete reference list online at
580.e1
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Palate Craniofac J. 2001;38:582–586.
102. Aznar ML, Schonmeyr B, Echaniz G, et al. Role of postoperative
antimicrobials in cleft palate surgery: prospective, double-blind,
randomized, placebo-controlled clinical study in India. Plast
Reconstr Surg. 2015;136:59e–66e.
103. Edgerton MT. Surgical lengthening of the cleft palate by dissection
of the neurovascular bundle. Plast Reconstr Surg. 1962;29:551–560.
580.e3
References
104. Dorrance GM. Lengthening the soft palate in cleft palate
operations. Ann Surg. 1925;82:208–211.
105. Dorrance GM, Bransfield JW. The pushback operation for repair of
cleft palate. Plast Reconstr Surg. 1946;1:145–169.
106. Wallace AF. A history of the repair of cleft lip and palate in Britain
before World War II. Ann Plast Surg. 1987;19:266–275.
107. Dellon AL, Edgerton MT. Correction of velopharyngeal
incompetence by retrodisplacement of the levator veli palatini
muscle insertion. Surg Forum. 1969;20:510–511.
108. Cronin TD. Method of preventing raw areas on the nasal surface
of soft palate in push-back surgery. Plast Reconstr Surg. 1957;20:
474–484.
109. Kaplan EN. Soft palate repair by levator muscle reconstruction
and a buccal mucosal flap. Plast Reconstr Surg. 1975;56:129–136.
110. Cohen SR, Kalinowski J, LaRossa D, et al. Cleft palate fistulas: a
multivariate statistical analysis of prevalence, etiology, and
surgical management. Plast Reconstr Surg. 1991;87:1041–1047.
111. Bardach J, Salyer K. Surgical Techniques in Cleft Lip and Palate.
Chicago, IL: Year Book; 1987.
112. Bardach J. Two-flap palatoplasty: Bardach’s technique. Operative
Techniques Plast Surg. 1995;2:211–214.
113. Friede H, Johanson B. A follow-up study of cleft children treated
with vomer flap as part of a three-stage soft tissue surgical
procedure. Facial morphology and dental occlusion. Scand J Plast
Reconstr Surg. 1977;11:45–57.
114. Delaire J, Precious D. Avoidance of the use of vomerine mucosa in
primary surgical management of velopalatine clefts. Oral Surg Oral
Med Oral Pathol. 1985;60:589–597.
115. Molsted K, Palmberg A, Dahl E, et al. Malocclusion in complete
unilateral and bilateral cleft lip and palate. The results of a change
in the surgical procedure. Scand J Plast Reconstr Surg Hand Surg.
1987;21:81–85.
116. Braithwaite F, Maurice DG. The importance of the levator palatini
muscle in cleft palate closure. Br J Plast Surg. 1968;21:60–62.
117. Cutting C, Rosenbaum J, Rovati L. The technique of muscle repair
in the soft palate. Operative Techniques Plast Surg. 1995;2:215–222.
118. Huang MH, Lee ST, Rajendran K. Clinical implications of the
velopharyngeal blood supply: a fresh cadaveric study. Plast
Reconstr Surg. 1998;102:655–667.
119. Sommerlad BC, Henley M, Birch M, et al. Cleft palate re-repair – a
clinical and radiographic study of 32 consecutive cases. Br J Plast
Surg. 1994;47:406–410.
120. Furlow LT Jr. Cleft palate repair by double opposing Z-plasty. Plast
Reconstr Surg. 1986;78:724–738. Furlow describes his palatoplasty in
the context of a 22-patient case series. Optimistic speech outcomes are
reported.
121. Furlow L. Cleft palate repair by double opposing Z-plasty.
Operative Techniques Plast Surg. 1995;2:223–232.
122. Randall P, LaRossa D, Solomon M, et al. Experience with the
Furlow double-reversing Z-plasty for cleft palate repair. Plast
Reconstr Surg. 1986;77:569–576.
123. Gunther E, Wisser JR, Cohen MA, et al. Palatoplasty: Furlow’s
double reversing Z-plasty versus intravelar veloplasty. Cleft Palate
Craniofac J. 1998;35:546–549.
124. Jackson O, Stransky CA, Jawad AF, et al. The Children’s Hospital
of Philadelphia modification of the Furlow double-opposing
Z-palatoplasty: 30-year experience and long-term speech
outcomes. Plast Reconstr Surg. 2013;132:613–622.
125. Timbang MR, Gharb BB, Rampazzo A, et al. A systematic review
comparing Furlow double-opposing Z-plasty and straight-line
intravelar veloplasty methods of cleft palate repair. Plast Reconstr
Surg. 2014;134:1014–1022.
126. Maine RG, Hoffman WY, Palacios-Martinez JH, et al. Comparison
of fistula rates after palatoplasty for international and local
surgeons on surgical missions in Ecuador with rates at a
craniofacial center in the United States. Plast Reconstr Surg.
2012;129:319e–326e.
127. Schweckendiek W, Doz P. Primary veloplasty: long-term results
without maxillary deformity – a 25 year report. Cleft Palate J.
1978;15:268–274.
128. Rohrich RJ, Byrd HS. Optimal timing of cleft palate closure.
Speech, facial growth, and hearing considerations. Clin Plast Surg.
1990;17:27–36.
129. Witzel MA, Salyer KE, Ross RB. Delayed hard palate closure: the
philosophy revisited. Cleft Palate J. 1984;21:263–269.
130. Bardach J, Morris HL, Olin WH. Late results of primary
veloplasty: the Marburg Project. Plast Reconstr Surg. 1984;73:
207–218.
131. Cosman B, Falk AS. Delayed hard palate repair and speech
deficiencies: a cautionary report. Cleft Palate J. 1980;17:27–33.
132. Iida S, Kogo M, Ishii S, et al. Changes of arterial oxygen saturation
(SpO2) following push-back operation. Int J Oral Maxillofac Surg.
1998;27:425–427.
133. Nour C, Ratsiu J, Singh N, et al. Analgesic effectiveness of
acetaminophen for primary cleft palate repair in young children: a
randomized placebo controlled trial. Paediatr Anaesth. 2014;24:
574–581.
134. Ousterhout DK. pers. comm. 1986.
135. Pribaz J, Stephens W, Crespo L, et al. A new intraoral flap: facial
artery musculomucosal (FAMM) flap. Plast Reconstr Surg.
1992;90:421–429.
136. Emory RE Jr, Clay RP, Bite U, et al. Fistula formation and repair
after palatal closure: an institutional perspective. Plast Reconstr
Surg. 1997;99:1535–1538. The authors report an 11.5% post-palatoplasty
fistula rate. Local flaps are advocated to repair these lesions.
137. LaRossa D, Jackson OH, Kirschner RE, et al. The Children’s
Hospital of Philadelphia modification of the Furlow double-
opposing Z-palatoplasty: long-term speech and growth results.
Clin Plast Surg. 2004;31:243–249.
138. Ross RB. Treatment variables affecting facial growth in complete
unilateral cleft lip and palate. Cleft Palate J. 1987;24:5–77.
SECTION I • Clefts
24
Alveolar clefts
Richard A. Hopper and Gerhard S. Mundinger
SYNOPSIS
■ Treatment of the alveolar cleft remains one of the most controversial
topics in cleft care.
■ Treatment protocols have varied in timing, technique, and selection of
graft material.
■ Currently the “gold standard” is secondary bone grafting with
autogenous cancellous graft at the time of mixed dentition.
■ Outcomes of proposed alternate treatments such as
gingivoperiosteoplasty, primary bone grafting, and use of inductive
proteins need to be compared to documented outcomes of the gold
standard.
■ Regardless of which technique is employed, coordination and
communication between surgeon and orthodontist are essential for
success.
■ Failed or complex alveolar bone graft sites remain a considerable
challenge. These recalcitrant alveolar clefts can benefit from recent
applications of distraction osteogenesis techniques.
Access the Historical Perspective section online at
Introduction
■ Compared to soft-tissue repair techniques, surgery on the
bony alveolar defect in cleft lip and palate patients is
relatively new.
■ Early approaches including primary grafting in infancy
fell into disfavor due to iatrogenic impairment of facial
growth.
■ Secondary bone grafting in mixed dentition and primary
gingivoperiosteoplasty (GPP) were introduced in the
1960s.
■ Secondary bone grafting has become the gold standard
for comparison of other techniques.
■ Primary bone grafting and GPP remain controversial.
Basic science/disease process
Anatomy of the alveolar cleft
The alveolar cleft is more than a linear gap in the maxillary
arch. With soft tissue removed, the cleft is best visualized as
a tornado, increasing in size from incisal to apical, becoming
widest as it extends into the nasal cavity and distorts the
surrounding anatomy (Fig. 24.1). The soft-tissue distortion
caused by this skeletal deficiency can be minimized by a cor
rectly performed cleft lip repair, but not completely eradicated.
In the patient with an untreated alveolar cleft, the alar base of
the nose lacks the bony support of the non-cleft side, and if
release of the lateral nasal wall and reconstruction of the nasal
component of the orbicularis oris muscle have not been
achieved at the primary lip repair, the nasal base will remain
attached to the hypoplastic piriform rim, with inferior and
posterior malposition.
The fistula between oral and nasal cavities has three distinct
boundaries. The nasolabial fistula is located at the apex of the
cleft, high up in the labial sulcus, and consists of loose wet
labial mucosa transitioning to nasal mucosa. The oronasal
fistula extends from the incisive foramen to the alveolar
process, and is a transition of attached palatal mucoperios
teum to nasal mucosa. At the corner of these two fistulas, at
the alveolar process itself, attached alveolar gingiva is located,
which is the only appropriate support lining of erupting teeth.
Whichever technique is employed to close the soft tissues of
the nasolabial and oronasal fistulas, attached gingival tissue
must be present at the anticipated site of eruption to ensure
long-term support of the adult teeth.
Dental development
An alveolar cleft is associated with variable anomalies in
dental development that must be taken into consideration
with presurgical preparation, timing of surgery, surgical
technique, and post-surgical orthodontic planning. Anomalies
Access video content for this chapter online at expertconsult.com
581.e1
Historical perspective
BOX 24.1 Goals of treatment of the alveolar cleft
• Stable bone continuity of maxillary arch
• Separation of oral and nasal cavities
• Appropriate maxillary arch form and transverse width
• Stable environment for eruption of cleft-side canine
• Maintenance and bone support of all erupting teeth
• Keratinized gingival environment for erupted teeth
• Piriform bone support of nasal base
• Preserved anterior vestibule
• Uninhibited facial growth
• Minimized donor site morbidity
Historical perspective
Although the visible soft-tissue deformity associated with
cleft lip and palate has been described and treated for centu
ries, the importance of treating the underlying skeletal
pathology has only recently been realized. Primary bone
grafting in infancy and early childhood was advocated by
Schmid and others in the mid 20th century,1–5 but soon fell
into disfavor, with long-term follow-up demonstrating iatro
genic impairment of facial growth. In the midst of this
backlash to alveolar grafting, Boyne introduced the concept
of secondary alveolar bone grafting in the 1960s, advocating
treatment towards the end of the first decade of life to mini
mize growth impairment while still supporting eruption of
the adult dentition.6 The same decade, Skoog published his
experience with “boneless bone grafting”, which was the
birth of the concept of GPP.7
All of these three treatment options of alveolar reconstruc
tion – primary grafting, secondary grafting, and GPP – are
currently practiced in modern cleft care; however, most prac
titioners would accept that secondary bone grafting remains
the “gold standard”. Despite this predominance of secondary
grafting, treatment of the alveolar cleft remains the most
controversial topic of discussion at national cleft meetings.8
New technology in this century has introduced even more
variables in the form of inductive proteins that could avoid
the need for graft material, and devices that can generate bone
formation through the principles of distraction osteogenesis.
Practitioners in cleft care should understand the pros and
cons of all options of alveolar cleft treatment regardless of
their protocol of choice. Only in this way can the merits of
new technologies and treatment protocols be fairly evaluated.
The overriding goals of alveolar cleft treatment listed in Box
24.1 should be shared by all management protocols. The
purpose of this chapter is to introduce the reader to the con
cepts of each treatment and to discuss the available literature
regarding the success of each in achieving these treatment
goals.
582
SECTION I
CHAPTER 24 • Alveolar clefts
cleft with molding and a GPP would unnaturally constrict the
arch form. Isolated clefts of the primary palate are also diffi
cult to predict if a GPP is possible. Due to the bony fusion of
the secondary palate, the alveolar segments are more resistant
to presurgical molding, and in some cases cannot be adequately
aligned. Finally, in bilateral complete clefts, it is not always
possible to align both sides of the premaxilla with the alveolar
segments. In this case, one alveolar cleft can undergo a GPP
to convert the arch form to a lesser and greater segment
similar to a unilateral cleft. Assessment of parallel alveolar
molding can be difficult, and benefits from a team presurgical
evaluation (Fig. 24.2). If the alveolar anatomy and presurgical
molding outcome are favorable, a GPP can be offered to the
family at the same time as the primary lip repair.
Primary bone grafting
Grafting at the time of primary dentition is practiced by
relatively few centers, with the most published group being
that of Rosenstein and Dado, who have used primary graft
ing as their approach to the alveolar cleft for over 20 years.
Patient selection is based on the family’s ability to undertake
the staged surgical and orthodontic appliance protocol
necessary to maintain arch relationship before and after
grafting.16
Secondary bone grafting
As the patient is approaching the time of secondary bone
graft, the craniofacial orthodontist and surgeon should discuss
plans for timing of the graft, the fate of adjacent teeth, and the
timing of arch expansion. Any dental morbidity such as poor
hygiene or caries should be addressed by a pediatric dentist
prior to the surgery. In some cases, primary teeth adjacent to
the cleft should be extracted 3–6 weeks prior to grafting in
order to ensure a viable mucosal seal of the oral surface of the
grafted area. In most cases, however, the teeth can be pre
served until the time of grafting to maximize bone retention,
avoid an additional procedure, and be extracted at the graft
ing surgery.
Arch expansion can be performed either before or after the
graft surgery. Preoperative arch expansion allows the ortho
dontist to take full advantage of the mobility of the two or
three ungrafted segments of the alveolus to achieve appropri
ate maxillary anterior and posterior arch width. When the
graft is then placed and heals in the expanded cleft, the con
tinuous maxillary arch form has an optimal relationship with
the mandible. A collapsed overlapping alveolar cleft can also
benefit from expansion by increasing the access and visibility
of the surgeon to the fistula at time of operation. The disad
vantages of presurgical expansion, however, include overex
pansion, such that the alveolar cleft becomes challenging to
treat due to simultaneous expansion of the oronasal fistula
and resulting excessive tension on any soft-tissue repair. If an
expander is in place at the time of surgery and obstructs the
site, then replacement of the device with a custom acrylic
retention splint at the time of surgery is indicated. Postopera
tive expansion should wait 6–8 weeks from the date of surgery,
and conventional orthodontic movements should not be
attempted before the cleft is grafted, but can start within 3
weeks of surgery.
can include the number of teeth (missing teeth, supernumer
ary teeth), the location (mesial or distal to cleft), the shape
(pegged or conical), the size (microdontic), the time of forma
tion and/or eruption, and crown and root malformations.9,10
A goal of successful alveolar cleft treatment is to provide a
stable supporting environment for eruption of the permanent
canine. The adjacent lateral incisor, however, must be taken
into consideration prior to surgery with a coordinated
surgical–orthodontic plan. The lateral incisor is often missing
in complete clefts; however, if present, it can be positioned
either mesial or distal to the cleft. Although still debated,
many consider the lateral incisor to be supernumerary when
distal.11
It is important that the patient and family be informed
before the bone graft procedure that in many cases a perma
nent lateral incisor is congenitally absent, will not erupt, or
will be extracted as part of the treatment protocol.12–15 If
needed, the lateral incisor is extracted to create space for the
permanent canine to migrate and erupt through the newly
grafted area. The orthodontist can then perform canine
substitution of the lateral incisor if it was absent or not
supernumerary.
Diagnosis/patient presentation
Gingivoperiosteoplasty
Prior to undergoing a GPP, the infant with a cleft must be
evaluated by the practitioner coordinating the presurgical
molding, as well as the surgeon who will be performing the
GPP. It must be emphasized that not all infants will be candi
dates for GPP due to individual variations of anatomy. Some
infants with particularly wide unilateral clefts can be “mesen
chymally deficient”. Compressing and closing the alveolar
Fig. 24.1 Bony anatomy of an untreated unilateral cleft lip and palate in a
4-year-old. The lateral piriform rim is hypoplastic, increasing the width of the
piriform opening on the cleft side, creating a tornado-shaped defect. The maxillary
nasal crest is deviated away from the cleft, carrying the bony septum with it. The
collapse of the ungrafted maxillary arch is apparent with a lingual crossbite of the
lesser segment.
583
Diagnosis/patient presentation
Debate continues regarding the appropriate timing of sec
ondary bone grafting. The mixed dentition phase is variable
among patients, but typically falls between ages 6 and 11
years. Proponents of grafting early in mixed dentition believe
that a stable, healed graft prior to canine eruption results in a
superior bone environment (Fig. 24.3). El Deeb et al.17 found
that successful eruption of cuspids though the graft occurred
when root formation of the canine adjacent to the cleft was
one-fourth to one-half formed at the time of graft placement.
Bergland et al.18 found a higher proportion of graft failures
and cases with lower interdental septa when grafting was
done adjacent to fully erupted teeth compared to just before
eruption. In comparison, Long et al. retrospectively performed
detailed periapical radiograph analysis of bone formation and
found no significant correlation between final graft success
and the amount of canine crown eruption in the cleft at the
time of grafting.19
Fig. 24.2 (A) An appropriately molded unilateral alveolar cleft candidate for
gingivoperiosteoplasty (GPP). There is parallel alignment of the alveolar cleft edges
with a smooth arch form. (B) Gingival hypertrophy in the alveolar cleft is masking a
bone gap that is too wide for a GPP. (C) A collapsed arch form not amenable to
GPP. Although the edges are touching, the opposing bone segments within the cleft
are not aligned for bone formation. (D) A mesenchymal-deficient arch form that
should not undergo GPP. If this cleft was approximated with molding followed by a
GPP that formed a bone fusion, it would unnaturally constrict the projection of the
alveolar arch. Arch expansion with bone grafting of the alveolar gap is the indicated
treatment.
A
B
C
D
1mm
1mm
2mm
2mm
1mm
5mm
1mm
5mm
Gingiva
Bone
X
X
X
Fig. 24.3 (A) A unilateral alveolar cleft in mixed dentition ready for preoperative
arch expansion and secondary bone grafting. The permanent canine is descending
but is still covered with bone. The cleft extends from the alveolus up into the
piriform cavity. (B) The same cleft after preoperative palate expansion and
successful autogenous bone grafting. With the expansion and with orthodontic
uprighting of the adjacent central incisor, there will be sufficient bone support and
space for the erupting permanent canine.
A
B
584
SECTION I
CHAPTER 24 • Alveolar clefts
is in place. Alcohol, smoking, and drug abuse must be ruled
out, and the expectations on the patient must be discussed
and documented. All current distraction devices require
activation by the patient or caregiver and frequent follow-up
during the period of activation. Therefore, a patient who is
considered unreliable, non-compliant, or unable to return to
clinic regularly is not a suitable candidate. TDO is currently
Bone morphogenetic protein-2
Recombinant human bone morphogenetic protein-2 (rhBMP2)
is a mitogen that has been demonstrated to stimulate osteo
blastic activity and induce bone nodule formation in animals.
It has been approved by the US Food and Drug Administra
tion for clinical use in human spine fusion procedures,
and has been shown to decrease nonunion, donor site morbid
ity, and operating time over autogenous grafting in this popu
lation.20 More recent clinical applications have been on patients
undergoing alveolar augmentation and implant placement,21
and early trials at individual centers for the treatment of
alveolar clefts have been completed. The risk–benefit profile
of rhBMP2 in these patients remains largely unknown, and
elucidation thereof will require further high-quality random
ized controlled trials.22
Late bone grafting
Some centers have advocated delaying alveolar bone grafting
in patients who are known to need a LeFort I osteotomy pro
cedure at skeletal maturity in order to perform both simultane
ously. This, however, has largely been abandoned in the
presence of an erupting canine, since eruption into a bone graft
is felt to provide better bone support and periodontal status.12
However, in any cleft center, patients can present in per
manent dentition without having had their alveolar cleft
treated. In these cases, a combined segmental orthognathic
and bone grafting procedure is required. Outcomes of these
techniques are not as favorable as grafting in mixed dentition
followed by dental eruption into the graft and orthodontic
movement.
Alveolar distraction
Successful primary or secondary treatment of the alveolar
cleft should obviate the need for alveolar distraction in this
patient population. Unfortunately there exist patients with
“ungraftable” or “recalcitrant” alveolar clefts that have few
options available to them other than undergoing transport
distraction osteogenesis (TDO) of alveolar bone.23 The typical
patient who falls into this category has unhealthy, scarred
gingiva, a large nasolabial and/or oronasal fistula, and a
history of repeated unsuccessful bone grafts with infections
and exposure. Another possible presentation is a previously
grafted maxilla that has severe vertical deficiency along with
scarred mucogingiva preventing additional graft augmenta
tion. In both these cases, TDO is a useful tool to have in the
cleft armamentarium.
In the wide “ungraftable” cleft patient (Fig. 24.4), a tooth-
bearing transport segment can be slowly moved into the gap,
closing the fistula and converting the problem into a narrow
cleft amenable to traditional secondary grafting. In the verti
cally deficient alveolus (Fig. 24.5), a transport segment of
superior maxillary bone, with or without prior bone augmen
tation, can be slowly lowered, leveling the alveolar ridge by
gradually bringing the scarred attached gingival tissue along
with the advancing bone front.
Factors that may have contributed to previous failed sur
geries in patients with “recalcitrant” clefts must be carefully
considered to avoid the plan for TDO suffering the same fate.
Any dental caries or periapical disease must be addressed to
minimize the infection risk during the period when the device
Fig. 24.4 A good candidate for interdental horizontal transport distraction. The
patient had undergone three previous attempts at treatment of bilateral alveolar
clefts, including transfer of a facial artery musculomucosal (FAMM) flap, seen
hanging from the anterior palate. The patient has extensive scarring and a large
tornado-shaped oronasal and nasolabial fistula. The recommended treatment was
simultaneous closure of the nasal lining using the FAMM flap tissue with transport
distraction of a three-tooth segment from the lesser segments, as shown in Fig.
24.10, followed by secondary bone grafting of the residual cleft.
Fig. 24.5 A good candidate for vertical transport distraction. The patient had
undergone six previous attempts of alveolar grafting with infection and graft loss.
The result was vertical deficiency of the alveolus, loss of the adjacent three
permanent teeth, and tight scarred gingival covering. The patient underwent staged
cortical grafting and vertical transport distraction, as shown in Fig. 24.12.
585
Treatment/surgical technique
to the minimum needed to achieve closure of the nasal floor.
After the mucosal incision, most of the vomer dissection can
be achieved with an elevator, with the exception of the region
of the premaxillary suture, which will require sharp dissection
with a small blade. This nasal floor closure separates the nasal
from the oral cavity back to the incisive foramen and provides
the superior barrier of the guided tissue regeneration tunnel
between the alveolar segments.
The “floor” of the GPP tunnel is created by elevating
inferiorly based mucoperiosteal flaps from the oral edges of
the alveolar cleft (oral flaps). These flaps are contained within
the cleft itself, and extend from the labial surface of the alveo
lus back to the incisive foramen. They are typically 2–3 mm
in vertical height, such that when they are inferiorly rotated,
they meet across the oral boundary of the cleft and can be
sutured together with everting resorbable 5-0 gut or Vicryl
sutures.
The attached mucoperiosteum that remains within the
alveolar cleft between the superior anteroposterior incision
made to create the nasal closure flaps and the inferior one
made to create the oral closure is the tissue used to close the
anterior border of the GPP. The alveolar cleft can be visualized
as a pyramid with the apex at the incisive foramen, where the
superior and inferior vomer incisions converge. Anteriorly,
these two incisions diverge, creating anteriorly based triangu
lar flaps that are brought out from between the alveolar seg
ments, pedicled anteriorly, and flipped across the cleft, closing
the labial border of the GPP (labial flaps). In designing the
GPP flaps, the incisions on one side of the cleft are shifted
slightly superiorly, so that the labial flap covers the upper half
reserved for patients past mixed dentition due to the risk to
unerupted tooth follicles during the segmental osteotomies.
Coordination with the patient’s orthodontist or prosthodontist
is essential in order to agree upon the desired goals of the
procedure and the endpoint of activation. Frequently the
treatment plan may include arch expansion or tooth extrac
tion, which should be performed prior to the TDO procedure.
Written directions and a device-turning log should be care
fully explained to the patient to avoid complications during
activation. Dental hygiene must be maintained during activa
tion with perioperative chlorhexidine mouthwash and a soft
toothbrush.
Treatment/surgical technique
Gingivoperiosteoplasty
The GPP can be performed at any point of the primary cleft
lip repair, but is easiest after all other dissection has been
completed, and before repair of the lip elements. The “roof”
of the GPP is the repair of the anterior palate (nasal floor) from
the nasal sill back to the incisive foramen that is typically done
with most modern cleft lip repairs. This is achieved by sutur
ing the inferior edge of the reconstructed lateral nasal wall to
a superiorly based mucoperiosteal vomer flap (nasal flap)
(Fig. 24.6). The incision along the vomer to create the leading
edge of the vomer flap is at the level of the oral–nasal mucosa
demarcation. This demarcation is also visible on the opposing
lateral nasal wall. The vomer flap vertical dissection is kept
Fig. 24.6 Gingivoperiosteal flap design and elevation for the Millard-type gingivoperiosteoplasty. The dissection is limited to the tissues within the cleft. The flaps are
named by the part of the periosteal tunnel they construct. See text for details. LR, right labial flap; OR, right oral flap; NR, right nasal flap; LL, left labial flap; OL, left oral flap;
NL, left nasal flap; A, anterior; P, posterior.
C
A
L
N
O
P
OR OL
Oral bridge
D
LR
LL
Labial bridge
B
NR
NL
Nasal bridge
A
Oral
Nasal
NR
NL
LR
LL
OR
OL
1-2mm
Gingiva
Bone
586
SECTION I
CHAPTER 24 • Alveolar clefts
used by Rosenstein and Dado, a maxillary appliance is used
prior to lip repair to align the alveolar segments.16,24 Following
a lip repair at 6–8 weeks of age, the appliance is modified
to prevent posterior collapse of arch width while allowing
the repaired lip musculature to mold and close the anterior
cleft as a “butt joint”. Once the cleft is molded to approxima
tion, at around 4–6 months, the segments are stabilized with
an autogenous split rib graft. The appliance is continued for
6–8 weeks post-surgery and the palate is closed at or before
1 year of age.
Secondary bone grafting
When performing secondary bone grafting, we recommend
chlorhexidine mouthwash and nasal antibiotic ointment in
the days before surgery to decrease the bacterial load. The
patient is positioned supine with a small support under the
posterior iliac crest to facilitate bone graft harvest. There
should be two sterile fields to avoid contamination between
the oral field and donor field.
We follow a modification of the technique described by
Abyholm et al.25 (Fig. 24.7 and Video 24.1
). A superiorly
based mucoperiosteal flap is raised off the lesser segment of
the alveolus with a back cut into the loose mucosa anterior to
Stenson’s duct. If the site immediately adjacent to the cleft has
sufficient attached gingiva, this is included in the tip of the
flap. If not, the attached gingiva of the one or two teeth distal
to the cleft must be included in the flap, and when the flap is
advanced, the papillae are shifted by one tooth width towards
the cleft.
of the labial border of the cleft, while on the contralateral side,
the incisions are shifted inferiorly for the labial flap to cover
the lower half. The superior edge of the upper labial flap is
sutured to the lip mucosa, and the inferior edge of the lower
labial flap is sutured to the anterior edge of the two oral flaps.
In this fashion, a guided tissue regeneration “chamber” is
sealed nasally, orally, and labially by mucoperiosteal flaps,
and contains two opposing bone surfaces on the mesial and
distal walls. It is essential that this end goal is visualized
throughout the GPP procedure to ensure that all soft-tissue
interference is removed from the alveolar cleft during creation
of the flaps, and that the flaps are positioned correctly to direct
bone growth across the cleft.
Technical pitfalls of the GPP include: elevation of the flaps
in a submucosal instead of subperiosteal plane; inaccurate
planning of flaps such that viability of the anteriorly based
labial flaps is compromised; and trauma to the flaps from
compressive handling with forceps. Frequently, a deciduous
tooth follicle is encountered during the flap dissection. Careful
sharp dissection between the follicle and the periosteum is
required to prevent disruption of dental eruption. If the flaps
appear too thin or non-viable during the follicle dissection,
the GPP should be aborted and the mucosa replaced.
Primary bone grafting
Protocols for primary bone grafting vary, but typically involve
a staged approach of molding the maxillary arch segments in
the first year of life followed by stabilization of the maxillary
arch with an autogenous bone graft in infancy. In the protocol
A
B
C
E
Fig. 24.7 Modified Abyholm technique for secondary bone grafting of a unilateral alveolar cleft. See text for details. (A) The superior-based lesser-segment flap for labial
surface closure has been marked. The tip is covered with attached gingiva for transfer into the cleft. (B) The flap is raised off the lesser segment in a subperiosteal plane
and cleft mucosa separated from the labial mucosa with an angled blade. (C) The opposing surfaces of mucosa within the cleft have been separated from alveolus to
incisive foramen. The superior flaps (hook in place) are used to repair the nasal lining. (D) The two reflected superior mucosal flaps have been sutured together to close the
nasal fistula and the two inferior flaps closed to repair the oral fistula. (E) Cancellous iliac crest bone graft has been packed within the cleft. (F) The lesser-segment
mucoperiosteal flap is transposed across the labial surface of the graft without tension. The frenum was released secondarily.
D
F
587
Treatment/surgical technique
dissection proceeds directly down to the cartilage cap of the
crest, without any muscle dissection or stripping. The carti
laginous crest is opened as an H-shaped incision to gain
access to the underlying cancellous bone. The bone is har
vested using a curette or gouge and stored in blood. If a corti
cal strut is planned to “reconstruct” the pyriform rim and
provide a barrier between the graft and nasal lining, it is
harvested from the inner table of the ilium. The graft donor
site is then packed with gel foam soaked with plain bupiva
caine to minimize postoperative pain27 and the cartilage
tightly approximated prior to closing the skin.
If a cortical barrier strut is used, it is placed under the nasal
lining repair, resting on the opposing edges of the piriform
rim at the superior aspect of the cleft. The graft is then care
fully packed into the prepared graft site, using a bone tamp
to compress the pieces. If there is graft remaining after filling
the cleft from incisive foramen to labial surface and from nasal
lining to oral lining, the remaining bone is used to augment
the deficient maxillary bone in the region of the cleft-side
piriform rim. This onlay graft helps support the alar base of
the nose, and can improve symmetry in the unilateral cleft
nasal deformity. The lesser-segment mucoperiosteal buccal
sulcus flap is then mesially advanced over the labial surface
of the graft and carefully secured with fine resorbable sutures
to the mesial alveolus and oral palate. The most common site
of dehiscence is at the junction of the lesser-segment flap and
the oral lining repair, often at the edge of the central incisor
crown. Meticulous atraumatic suture technique must be used
in this area.
Postoperative care includes gentle oral hygiene with a soft
manual toothbrush and antibiotic mouthwash. A mechanical
soft diet is followed for 6 weeks. Radiographic preservation
of the graft is assessed with a periapical film at 8–10 weeks.
If a small portion of the graft site becomes exposed in the early
postoperative course, it can often be salvaged with debride
ment of the exposed bone chip and antibiotic rinses. If the
entire graft itself becomes contaminated or purulent, it must
The lesser-segment flap is raised in a subperiosteal plane
and then mobilized by performing a periosteal release across
the undersurface of the flap, parallel to the occlusal surface,
approximately 2.5 cm from the inferior edge. Since the peri
osteum contributes to the perfusion of the flap, the further the
release is performed from the edges of the flap, the better. This
periosteal release is continued towards the cleft separating the
labial mucosa from the cleft mucosa. For this release of labial
mucosa, an angled blade is used to undermine the incision at
the superior extent of the alveolar cleft labionasal fistula,
taking care to cut up into the lip and not towards the nasal
floor. This mobilizes the oral mucosa above the cleft along
with the lesser-segment flap without violating the nasal cavity
(see Fig. 24.7C).
The opposing alveolar cleft mucosal surfaces are then cut
from incisive foramen to labial surface of the alveolus, sepa
rating them into upper (nasal lining) and lower (oral lining)
flaps. The upper mucoperiosteal nasal lining flaps are
reflected out of the cleft and up into the piriform aperture
and sutured to each other with a small curved needle repair
ing the labionasal fistula (see Fig. 24.7D). If the space is too
tight for a needle, the repair is done through the nose,
passing a large needle from the nose into the cleft through
one of the lining flaps, and then passing it back from the
cleft into the nose through the opposing lining flap and tying
the knot inside the nostril. The lower oral lining flaps within
the cleft are then reflected down into the mouth, such that
they can be closed to each other with everting sutures to
repair the oronasal fistula. If the cleft is too wide, then ante
rior hard-palate tissue is raised as posteriorly based muco
periosteal flaps pedicled on the greater palatine vessels, and
sutured together to close the oral surface of the cleft without
tension.
With closure of the oronasal fistula, the graft can now be
harvested. Numerous donor sources of bone graft have been
described,26 but iliac crest cancellous bone graft is considered
the gold standard (Fig. 24.8). After the skin incision, the
A
B
Fig. 24.8 Incision placement for harvesting iliac crest cancellous bone graft. (A) The anterior superior iliac spine and crest have been marked in dotted lines. (B) Upward
traction is placed on the skin over the crest and the incision is marked along the iliac crest such that when the skin is released, the scar will lie off the crest and below the
pant line. The incision should be 2 cm behind the anterior superior iliac crest to avoid iatrogenic damage to the lateral femoral cutaneous nerve. Excessive traction or
transaction of the nerve will result in meralgia paresthetica.
588
SECTION I
CHAPTER 24 • Alveolar clefts
the orthodontic force is not competing with the distraction
vector; otherwise, the device footplates and screws will be
under strain. When performing simultaneous bilateral dis
traction for bilateral clefts, care must be taken to control
protrusion of the ungrafted premaxilla as the transport seg
ments contact it towards the end of activation (Fig. 24.11). The
device can be removed after 8–12 weeks of consolidation with
simultaneous secondary grafting of the remaining approxi
mated cleft.
Vertical alveolar TDO
Vertical alveolar TDO is useful for augmentation of a previ
ously grafted cleft when the gingiva or previous surgeries
have made augmentation with standard grafting techniques
not possible (Fig. 24.12). Ten millimeters of vertical bone
height below the maxillary sinus is the minimum required to
perform an osteotomy and distraction without prior onlay
grafting. If there is no or minimal bone below the maxilla, a
cortical graft can be harvested as a separate procedure from
the mandible angle, iliac crest, or outer calvarium and rigidly
lag screwed as an onlay graft over the planned future oste
otomy. The osteotomy and distraction can be performed 2–3
months later, transporting the increased bone volume from
the incorporated onlay graft into the defect. The same activa
tion and consolidation protocol for horizontal TDO is fol
lowed. During vertical distraction, however, the activation
arm can often be covered with a custom temporary prosthesis
created by the patient’s prosthodontist.
Outcomes, prognosis, and
complications
Gingivoperiosteoplasty
Long-term evaluation is now available of the Millard GPP
protocol that used the Latham device to narrow the cleft such
that a limited subperiosteal GPP could be performed. In
evaluating their own patients, Millard et al.29 found that a
bony bridge formed in 63% of unilateral and 83% of bilateral
clefts and a very low percentage (3%) required secondary
bone grafting. An increase in anterior crossbite was reported
in patients treated with GPP, but they did not seem to require
orthognathic surgery more frequently than did controls,
although the follow-up was admittedly too short to tell
definitively.
Others, however, were less optimistic in their evaluations
of these same patients. Henkel and Gundlach reported verti
cal growth disturbance of the maxilla in 42% of patients with
unilateral and 40% of patients with bilateral clefts treated with
Millard’s technique.30 Berkowitz analyzed the occlusion of
these patients and found crossbites in 100% of patients treated
with GPP, and he reported more difficulty in treating the
crossbite deformity.31 Matic and Power32,33 retrospectively
reviewed 65 unilateral and 43 bilateral clefts treated with
Latham active molding and GPP. The clinical success rate
compared to a historical group treated with secondary bone
grafting was 41% for unilateral GPP and 58% for bilateral GPP,
compared to 88% and 90% respectively for the secondary graft
comparisons.
be removed and the site allowed to heal before attempting a
repeat graft.
For bilateral clefts, the same technique is used, but care
must be taken to ensure that the U-shaped fistula behind the
premaxilla is closed. If this has not been done adequately
at the time of the palate repair, an angled blade is used to
incise the posterior aspect of the premaxilla transversely,
separating the mucosa into oral and nasal components. The
nasal lining is repaired as in primary cases. The anterior hard-
palate mucosa is then raised as unipedicled mucoperiosteal
flaps and sutured to the oral mucosa of the posterior premax
illa. If the vascular supply of the premaxilla is tenuous, then
the repair should be staged, doing each graft at different set
tings. Alveolar cleft repair can be staged with an initial soft-
tissue oronasal fistula repair followed by standard bone
grafting. A stabilization splint is essential for a bilateral cleft
repair, or the mobility of the premaxilla post-surgery will
prevent healing of the grafted clefts.
Occasionally at the time of bone grafting, unrestricted
overgrowth at the premaxillary suture in patients with bilat
eral complete cleft lip and palate prevents the orthodontist
from achieving the appropriate arch alignment needed for
successful grafting. This can be seen in delayed or untreated
clefts found in adopted children, when the lack of a repaired
oral muscle sphincter did not mold the projecting premaxilla
in early childhood. In these rare cases, premaxilla setback is
required. This technique involves separating the premaxilla
from the vomer and resecting a portion of the vomer and
septum, allowing the entire premaxilla to be set back into
alignment with the lesser segments (Fig. 24.9 and Video 24.2
).28 This procedure carries the risk of necrosis of the premax
illa and loss of the anterior dentition, and should therefore
only be performed when other options are not available.
Horizontal TDO of a tooth-bearing
alveolar segment
This procedure is typically performed for a recalcitrant alveo
lar cleft with large oronasolabial fistula. The principle is to
create a transport segment by separating an adjacent two- or
three-tooth-bearing segment of the distal alveolus from the
maxilla without damage to the tooth roots and without viola
tion of the attached gingiva (Fig. 24.10). The distraction device
is then applied to the stable maxilla and the transport segment
with an anterior/mesial vector, such that when the device is
activated, the segment gradually closes the alveolar cleft until
it is touching the premaxilla. At this point, the remaining cleft
is amenable to standard grafting techniques. The device is
then left in place and not activated during the 8- to 12-week
consolidation period, during which the generate formed in
the distraction gap distal to the transport segment gradually
ossifies and stabilizes the transport segment. For large
naso-orolabial fistulas, performing the nasal lining closure
at the time of the device placement may be easier due to
the improved visibility and access via the wide cleft. As the
transport segment advances and the cleft and fistula are
compressed, the repaired nasal lining will fold on itself and
seal any remaining small holes, allowing the secondary graft
ing procedure to focus solely on closing the oral lining.
Activation of the device is initiated 5 days post-operation
at 0.5–1 mm/day. Orthodontic guidance can be provided with
brackets and an arch wire as long as the direction dictated by