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P r i m a r y Cl e f t P a l a t e Re p a i r

Barry Daniel Long, MDa, Rajanya Shah Petersson, MD, MSa,b,*

KEYWORDS
 Cleft  Cleft palate  Palatoplasty  Surgical technique

KEY POINTS
 There are several techniques for cleft palate repair, with modifications of each.
 It is important to evaluate the type of cleft palate to choose the appropriate repair type.
 The Veau classification system is commonly used to describe varying degrees of cleft palate and
has implications for chosen technique and outcomes.
 The main outcome measure for palatoplasty is speech resonance. Other factors to consider are Eu-
stachian tube dysfunction, postoperative fistulas, velopharyngeal insufficiency, and maxillary
growth.

INTRODUCTION It is worth commenting on the submucous cleft


palate (SMCP), which does not fall within the
There are several factors that should be consid- Veau classification system. With SMCP, mucosa
ered when planning cleft palate repair. First, it is is intact along the entire hard and soft palate, but
important to review the patient’s associated there is disruption of the palatal sling musculature
comorbidities, because this can impact decisions in the midline. Although this is not as clearly
on perioperative management and counseling observed clinically as overt cleft palate, these pa-
that is provided to family members. Many comor- tients may present with a bifid uvula, a notch at the
bidities can change the risk of postoperative hard and soft palate junction, or translucent mu-
airway obstruction, velopharyngeal insufficiency cosa in the midline at the site of muscular discon-
(VPI), or fistula formation. It is also critical to deter- tinuity, called the “zona pellucida.” In cases of
mine the type of cleft palate that will be addressed, occult SMCP, there is no evidence of clefting in
because this influences the decision on which the oral examination, but furrowing of the nasal
technique is most appropriate for an optimal pa- surface of the soft palate is noted. McWilliams2
tient outcome. Although many different systems performed a study of 130 patients that reported
are available for describing palatal defects,1 the 44% of SMCP did not require surgical intervention
Veau classification is widely known and split into for speech development. However, when discus-
groups that follow well with surgical planning: sing management options, it is worth noting that
 Group I: cleft of the soft palate the rate of VPI with SMCP has been reported be-
 Group II: cleft of soft and hard palate, that tween 5% and 67%.3 Surgeons can counsel fam-
does not extend further than the incisive fora- ilies that VPI may be present up to 50% of the time,
men (secondary palate) necessitating repair.
 Group III: cleft of soft and hard palate that ex- Primary cleft palate repair is commonly per-
tends anterior to incisive foramen and through formed at 9 to 12 months of age, although should
the alveolus on one side be tailored to the patient based on size, weight,
 Group IV: complete bilateral cleft palate and comorbidities. Overall, the goals of this
facialplastic.theclinics.com

a
Department of Otolaryngology/Head and Neck Surgery, Virginia Commonwealth University SOM, Richmond,
VA, USA; b Department of Otolaryngology/Head and Neck Surgery, Children’s Hospital of Richmond at VCU,
Richmond, VA, USA
* Corresponding author. Department of Otolaryngology/Head and Neck Surgery, VCU Medical Center, 1200
East Broad Street, PO Box 980237, Richmond, VA 23298.
E-mail address: Rajanya.Petersson@vcuhealth.org

Facial Plast Surg Clin N Am 32 (2024) 55–62


https://doi.org/10.1016/j.fsc.2023.07.001
1064-7406/24/Ó 2023 Elsevier Inc. All rights reserved.
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56 Long & Petersson

operation are to close the defect, lengthen the pal- surgeons to stretch their necks and shoulders,
ate, and gain normal velopharyngeal function while because palate repair causes strain in these areas
minimizing negative growth effects. A key aim of (personal communication, Dr D. Kacmarynski,
palatal repair is normal speech resonance, so sur- 2022).
gery should ideally be performed before 12 to
15 months of age, before speech acquisition. TWO-FLAP PALATOPLASTY
Although there are instances where anatomic or
medical concerns cause delay in repair, this time- The two-flap palatoplasty is one of the most used
frame is ideal for most of the population. Patients techniques and is viable for any palatal defect that
who are otherwise healthy and have clefts involves the hard palate, whether unilateral or
involving the soft palate only can have surgical bilateral (Fig. 1). Bilateral hard palate flaps are
repair performed earlier, around 7 to 8 months, raised, each as a mucoperiosteal flap that receives
their size permitting. blood supply from the greater palatine artery.
There have been several techniques described Planned incisions are marked along the medial
for cleft palate repair.4–8 These include the unipe- edges of the soft and hard palate cleft edges,
dicled two-flap palatoplasty (Bardach and Salyer), with care taken to mark 1 to 2 mm lateral from
bipedicled flap palatoplasty (von Langenbeck), the edges for appropriate tissue length to close
V-Y pushback palatoplasty (Veau-Wardill-Kilner), the nasal layer. Markings for the lateral incisions
and double-opposing Z-plasty (Furlow). These are placed w1 cm posterior to the maxillary tuber-
techniques were developed and described by osity and are brought anteriorly just inside the
the named surgeons, but there have been many alveolar ridge until this meets the cleft edge ante-
modifications made to these original techniques. romedially. Typically, all incisions are made and
The choice of technique is based on surgeon’s the flap elevated on one side, then the other. The
comfort and preference, but it is important that medial incision is started at the tip of the uvula
technical details of these operations be described and carried forward to the anterior most aspect
by current surgeons for accurate portrayal of pro- of the planned incision, with the incision made
cedural steps and comparison of outcomes. It is down to bone over the hard palate. The lateral inci-
important to understand and be able to perform sion is begun at the anterior end, just behind the
multiple techniques, given that choice of repair is alveolus. This is taken down to bone as the dissec-
case dependent. Factors that weigh into this deci- tion continues posteriorly, but when nearing the
sion include anatomic considerations, such as the posterior end of the hard palate the incision should
Veau classification; quality of available tissue; dis- become more superficial to avoid damage to
tance from the medial edge of the cleft to the the greater palatine artery vascular pedicle. A sub-
vomer and alveolus (ie, width of the cleft); airway mucoperisoteal flap is then raised, beginning
concerns: and developmental prognosis. Further- anteromedially, typically with blunt dissection in-
more, an individual’s training and experience also struments and monopolar cautery as needed.
play into the decision-making process. Although This dissection is carefully brought posteriorly until
a multitude of closure options exist, holding to the greater palatine artery and associated neuro-
the appropriate timeline and managing associated vascular bundle are identified and preserved. The
comorbidities are necessary for optimal results, pedicle is circumferentially dissected without
regardless of operative technique. damaging the vessel, to allow for appropriate laxity
Although the separate techniques are covered in for later closure. Finally, musculature of the palatal
further detail, there are some commonalities sling that is abnormally inserted along the poste-
across cleft palate repair methods that are dis- rior hard palate edge is freed.
cussed here. Patients generally are intubated Now attention is turned to raising the nasal layer
with an oral endotracheal tube that is secured to of the hard palate. Mucoperiosteum is elevated at
the midline of the lower lip, and cases are per- the medial cleft edge and extended laterally along
formed with a Dingman mouth gag in place to the nasal floor until enough nasal mucosa is raised
obtain a wide, unobstructed view of the palate. for a tension-free closure. This mucosa must be
Local anesthetic with epinephrine is injected into raised carefully to avoid any tears that could affect
the palate, with special care taken not to exceed appropriate closure. This can easily occur at the
7 mg/kg if using 1% lidocaine with 1:100,000 bony margin if not careful. In a unilateral cleft pal-
epinephrine. To avoid ischemic injury to the ate, if necessary because of a wide cleft or lack of
tongue and postoperative edema and airway con- necessary tissue, the noncleft side is augmented
cerns, the Dingman retractor is loosened regularly with a vomer flap for nasal layer closure. If this is
(eg, every 20–30 minutes) to allow for tongue pursued, then a mucoperiosteal flap must be
reperfusion.9,10 This can also be a useful time for raised along the appropriate side of the vomer. In

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Primary Cleft Palate Repair 57

Fig. 1. (A) Complete right cleft palate defect, with incisions marked for two-flap palatoplasty and Z-plasty of soft
palate. (B) Careful dissection is performed to identify and preserve the greater palatine vascular pedicle. (C) Dou-
ble opposing Z-plasty (Furlow) palatoplasty was performed to lengthen palate posteriorly. Nasal layer reoriented
and closed first. (D) Nasal layer closed. (E) Muscular and oral layers are then closed. Stabilizing sutures are placed
laterally over exposed periosteum, which will mucosalize postoperatively. Note that a small double opposing
Z-plasty was performed for the soft palate.

bilateral cleft palate, a midline vomer incision is several factors. Special attention should be given
made to raise bilateral subperichondrial flaps to to completely dissecting free mucosa in this area
help close the nasal layer completely when faced to help provide tension-free closure. If tension is
with wide clefts or insufficient tissue. encountered, there are various maneuvers that
With the oral and nasal layers raised from the are performed to release this. If needed, the tensor
hard palate anteriorly, the next step is soft palate veli palatini is dissected off the hamulus, or the
dissection bilaterally. Depending on patient size hamulus is infractured. The neurovascular pedicle
and palate length, this portion is repaired with a is dissected further until there is sufficient tissue
double-opposing Z-plasty (also known as Furlow laxity. Also, further blunt dissection is performed
palatoplasty, described in further detail later) or in the space of Ernst, which is the plane between
with an intravelar veloplasty (IVV) and straight- the superior constrictor muscle and the pterygoid
line repair, which is described presently. Because muscle. Flap mobility must be checked after per-
the muscle fibers have been freed from the poste- forming each maneuver to assess when an appro-
rior hard palate, they are now dissected free from priate end point has been reached.
the nasal mucosal layer of the soft palate in a With adequate tissue release and laxity, closure
medial to lateral direction. These muscle fibers is performed in three layers with absorbable su-
are also freed, in varying degrees, from the edge tures per surgeon preference. First, the nasal layer
of the oral mucosal layer of the soft palate to allow is closed with interrupted or running sutures, or a
for necessary reorientation of the fibers into a combination thereof. Next, the palatal musculature
transverse plane. Whether a Furlow or IVV with is closed in the midline with horizontal mattress su-
straight-line repair is pursued, the goal should be tures. Then, the oral layer is closed in interrupted
to improve soft palate function by orienting the le- fashion, typically with simple interrupted and verti-
vator muscular sling into a transverse direction. cal mattress sutures to evert the wound edges for
Once flaps are raised along the soft and hard optimal healing. The uvula is also approximated
palate bilaterally, mobility should be assessed to and closed with simple interrupted sutures, along
make sure that the flaps can meet in the midline the oral and nasal layers and at the apex. This
without tension. A key area of tension, and multilayer midline closure helps prevent oronasal
possible complication such as oronasal fistula, is fistulae formation. At the end of midline closure,
at the hard and soft palate junction, because of lateral gaps are present, which heal by secondary

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58 Long & Petersson

intention over the exposed bone. The surgeon can when dissecting out the greater palatine artery
place one to three horizontal mattress “air- pedicle because it cannot be visualized as easily.
knotted” sutures bilaterally to stabilize these flaps Because this repair is best used in patients with
for further closure, but one should be careful not to incomplete clefting of the hard palate, dissection
create tension on the midline closure with these of the vascular pedicle and lateral releasing inci-
because it could affect surgical site healing. sions should provide enough laxity to close the cleft
Finally, hemostasis should be obtained before in the midline in layers as previously described.
complete closure to lessen risk of postoperative
bleeding. V-Y ADVANCEMENT (VEAU-WARDILL-KILNER)
PALATOPLASTY
VON LANGENBECK PALATOPLASTY This procedure is performed by creating bilateral
posteriorly based mucoperiosteal flaps, similar to
This technique uses a bipedicled mucoperiosteal the two-flap palatoplasty. It is most appropriate for
flap and is useful for clefts with a soft palate defect repairing secondary palate defects. The most ante-
and a subtotal degree of hard palate involvement
rior portion of the flap is raised starting opposite the
(Fig. 2). Because of the decreased anterior mobility
expected location of the primary canine teeth and
caused by the tethering of the anterior pedicle, raised posteriorly to expose the greater palatine ar-
clefts of the primary palate cannot be closed.
tery pedicle. The bilateral flaps are then brought
Similar to the prior repair technique, it is combined
together in the midline at the most anterior portion
with a Furlow palatoplasty if palatal lengthening is of the cleft. This brings the flaps slightly posterior,
desired or combined with the previously described which helps to elongate the palate without the
IVV for the soft palate. Medial cleft incisions are
need for concurrent Furlow palatoplasty of the soft
made like the previous description for two-flap palate, so IVV and straight-line closure are appro-
palatoplasty, but the surgeon only makes lateral priate. Although this technique with IVV elongates
releasing incisions around the alveolar ridge, pre-
the oral layer, it does not elongate the nasal layer.
serving the anterior-most mucosa of the hard palate
as the anterior pedicle. In a von Langenbeck palato- FURLOW (DOUBLE OPPOSING Z-PLASTY)
plasty, the lateral incisions do not connect with the PALATOPLASTY
medial incision anteriorly, thus the bipedicled flap.
Flaps are raised bilaterally as described in the This type of palatoplasty is often used for closing
two-flap palatoplasty, but extra care is needed clefts of the soft palate only, submucous clefts,

Fig. 2. (A) Wide, U-shaped cleft palate defect with incisions marked for von Langenbeck palatoplasty. (B) Lateral
incisions are made to allow for dissection and release of mucosperiosteal flaps. Greater palatine artery is identi-
fied and preserved. (C) Muscle released from hard palate edge, and nasal flaps developed such that muscle is
elevated with oral layer. (D, E) Nasal layer is closed first with absorbable sutures. (F) Muscular and oral layers
are then closed. Stablizing sutures are placed laterally over exposed periosteum, which will mucosalize postop-
eratively. Note that a small double opposing Z-plasty was performed for the soft palate to resist scar contraction.

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Primary Cleft Palate Repair 59

or as an adjunct for posterior palatal closure and Next, the nasal flaps are developed. Scissors
lengthening in conjunction with the techniques are used to cut the left nasal flap from the uvular
listed previously. However, Furlow palatoplasty base toward the hamulus and Eustachian tube,
can be modified for use in any type of cleft pal- and the right flap is cut along the hard palate shelf
ate.11 Although the original description of this tech- toward the hamulus and Eustachian tube. Similar
nique comments that lateral relaxing incisions are to the oral layer, it is important to leave a cuff of tis-
not necessary,12 they can often be useful for help- sue on the right side where flaps will be sutured
ing decrease tension with wider clefts. Because close to the posterior hard palate margin. If neces-
this form of repair is a Z-plasty, it lengthens the sary, flaps can be sutured to the bone in children
soft palate, which brings the soft palate closer to less than 1 year of age. With these incisions
the posterior pharyngeal wall and helps to reduce made, and because of how oral flaps were raised,
the risk of VPI, but at the cost of losing palatal the left side nasal layer should have an anteriorly
width growth potential. Of equal importance is based mucosal flap, whereas the right side should
that this Z-plasty works to reorient the levator veli have a posteriorly based myomucosal flap. This is
palatini muscle more posteriorly and in a trans- the opposite of the oral layer and allows for appro-
verse direction across the palate, leading to a priate reorientation so that an intact palatal sling is
functional muscular sling. It also narrows the velo- established along the posterior soft palate. Now
pharyngeal port when considering the area in three the nasal flaps are brought into their new, appro-
dimensions. All of these aspects have the potential priate orientation, and the three limbs are closed
to decrease velopharyngeal insufficiency. in a simple interrupted and/or running fashion
When designing the Z-plasty flaps, the posteri- with absorbable suture. The same process is
orly based flaps should be myomucosal, whereas then performed with the oral layer, which will rest
the anterior flaps are mucosal (with retained sub- in a mirror image of the nasal layer, so that there
mucosa for flap bulk). To avoid direct apposition is minimal overlap of suture lines, and thus a lower
of multiple layers of closure, and increased risk of risk of palatal fistula formation. Finally, the uvula is
fistula formation, the oral and nasal flaps are mirror closed at its apex and then along the nasal and
images of one another. The step-by-step proced- oral sides with absorbable sutures, in a simple
ure described here is best suited for the right- interrupted fashion. With a successful Furlow pal-
handed surgeon; left-handed surgeons may opt to atoplasty, the muscular sling has been established
plan incisions in the opposite direction as long as in the correct transverse direction posteriorly and
the musculature remains in the posteriorly based the soft palate has been lengthened.
flaps. Typically, the Z-plasty flaps are designed to If a Furlow palatoplasty is planned in conjunction
be 60 degrees (Fig. 3). Oral mucosal incisions are with any of the aforementioned techniques, it is
marked with the left side extending from the cleft worthwhile to make the Z-plasty incisions after
margin just posterior to the hard palate shelf, near complete dissection of the anterior mucoperiosteal
the hamulus. These incisions should be somewhat flaps is complete and the vascular pedicles can be
curvilinear to create broader flap tips, and a cuff assessed. If there is any damage or concern for
of tissue should be left anteriorly to allow for sutur- flap viability, a straight closure with IVV may be a
ing. On the patient’s right, the incision begins at the more successful option, because raising of addi-
base of the uvula and moves outward to the tional flaps may compromise vasculature.
hamulus.
Dissection typically begins on the left side, with OUTCOMES AFTER CLEFT PALATE REPAIR
scalpel incisions made through the submucosa
until palatal muscle is found. This is then released A primary complication to avoid through proper
from the hard palate edge posteriorly if there are surgical technique is fistula formation. This occurs
any abnormal attachments remaining. With tenot- if the viability of the repair is compromised, or
omy scissors, the palatal musculature is raised there is tension present in the closure. The junction
with the posteriorly based oral flap. Next, sharp of the hard and soft palate is the point of highest
dissection down to muscle is performed on the tension so it should be given adequate attention
right side, and the oral flap is developed anteriorly, during closure to prevent fistula formation. There
with tenotomy scissors used to leave muscle is a large variation reported in the rate of fistula for-
down on the anteriorly based nasal flap on this mation, from 3% to 60%, although 10% is an
side. Once again, care is taken to remove erro- agreed upon professional consensus.6,7,13 Specif-
neous muscular attachments from the hard palate ically among otolaryngologists, one study reported
edge, and submucosa and salivary gland tissue a fistula rate of 6.6% of 453 patient cases.14 This
should be taken up with the oral flap to aid with study also showed no difference in fistula forma-
flap bulk and adequate closure. tion among the operative surgeons, but rather

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60 Long & Petersson

Fig. 3. (A) Furlow (double opposing Z-plasty) palatoplasty incisions marked for a submucous cleft palate. (B) Left
oral myomucosal flap raised. Right oral flap is mucosal and submucosal only. (C) Z-plasty nasal flaps are reor-
iented and closed first with absorbable sutures. (D) The oral flaps are reoriented and closed in the opposite orien-
tation. Note lengthening of palate after Z-plasty.

that a significant risk factor was Veau IV classifica- patients, tympanostomy tubes are regularly
tion. The broader base of literature echoes this placed at the time of cleft palate repair to negate
same point, that risk factors related to higher rates a need for additional anesthesia. When consid-
of fistula formation are higher Veau classification ering which palatoplasty technique to perform
and wider cleft. with consideration for otologic outcomes, there
Velopharyngeal insufficiency (VPI) is present in are some conflicting data. Although some litera-
about 20% to 30% of patients after cleft palate ture suggests that performing Furlow palatoplasty
repair.6,15 Although it can be difficult to assess the can improve ETD over two-flap palatoplasty based
impact of surgical technique,16–18 cleft width and on number of myringotomy tubes placed after time
age at time of repair (especially age >2 years) shows of repair,21 other studies show no difference in
an associated higher risk of VPI after repair.17 There otologic outcomes when comparing Furlow and
is an increasing burden of evidence that Furlow von Langenbeck techniques.22 The latter results
palatoplasty of the soft palate can aid in obtaining were based on audiometry, rate of tympanostomy
velopharyngeal competency postoperatively.12,17,19 tube placement, and physical examination.22 ETD
Another area of interest is what effect palate is also improved when performing IVV versus not
repair has on facial growth. Although there have performing IVV.23 At age 5 to 6, 60% of these pa-
been several studies and systematic reviews, re- tients had abnormal otoscopic examinations,
sults prove to be conflicting when evaluating although children within the study did not undergo
maxillary growth and occlusion. Commonly stud- tympanostomy tube placement at the time of cleft
ied factors are timing of hard palate repair and palate repair. Carroll and colleagues24 demon-
technique performed, but a large variability among strated that at age 6, children with cleft palate
studies in relation to age range and timing of repair repair via Furlow palatoplasty had the best pure
makes for muddied results.20 Currently, the only tone average on audiometric testing when
clear consensus is that timing of repair does not compared with V-Y advancement, two-flap, or
affect the mandible’s protrusion. von Langenbeck palatoplasties.
Eustachian tube dysfunction (ETD) is consis- When evaluating other outcome measures for
tently present in patients with cleft palate and is children after cleft palate repair, there are sparse
linked to the abnormal orientation of the levator prospective randomized controlled data because
and tensor veli palatini muscles. Because of it is tough to randomly assign patients to one tech-
chronic middle ear drainage issues in these nique versus others. Therefore, the best option in

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Primary Cleft Palate Repair 61

this case is multicenter studies that can develop DISCLOSURE


and standardize methodology, study designs,
follow-up, outcome measures, and so forth. One The authors have nothing to disclose.
other improvement opportunity is described by
Sitzman and colleagues,25 who sought to improve REFERENCES
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