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MOC-CME

Evidence-Based Medicine: Cleft Palate


Kyle J. Chepla, M.D.
Learning Objectives: After reading this article, the participant should be able
Arun K. Gosain, M.D.
to: 1. Describe recent changes in treatment of cleft palate. 2. Compare the ef-
Cleveland, Ohio ficacy of different surgical treatments. 3. Assess their own knowledge of cleft
palate repair. 4. Determine where further individual in-depth study and devel-
opment are warranted.
Summary: The Maintenance of Certification in Plastic Surgery series is de-
signed to ensure professional development and measure continued competen-
cy within a specialty or subspecialty. The present article provides an evaluation
of the interval studies regarding the management of cleft palate with a specific
focus on craniofacial growth, speech outcomes, and obstructive sleep apnea
since the last Maintenance of Certification in Plastic Surgery article on the
subject published in 2010. This purpose of this article is to update plastic and
craniomaxillofacial surgeons on recent changes in treatment of cleft palate,
provide a means for accurate self-assessment, and guide further individual in-
depth study and development.  (Plast. Reconstr. Surg. 132: 1644, 2013.)

T
he incidence of isolated cleft palate is the normal muscular anatomy was first described
approximately 0.1 to 1.1 per 1000 births and by Braithwaite8 in 1964 and subsequently radically
varies by race, without a gender difference. modified in 1970 by Kriens,9 who coined the term
Multiple risk factors have been associated with “intravelar veloplasty.” Again, there are many com-
cleft palate and include genetic factors, terato- monly used techniques for recreating the levator
gens (e.g., maternal alcohol intake, maternal sling, including the double-opposing Z-plasty10,11
tobacco use, certain antiepileptic medications), and those described by both Cutting et al.12 and
and several syndromes (i.e., Pierre Robin, velocar- Sommerlad et al.13,14 Regardless of the technique
diofacial, Van der Woude, Apert, and Crouzon). used, the goals of cleft palate repair remain as
Cleft palate can involve only the soft palate follows: (1) lengthen the palate to minimize the
to varying degrees (incomplete cleft) or both the incidence of postoperative velopharyngeal insuffi-
soft and hard palate to the level of the incisive ciency and promote proper speech development,
foramen (complete cleft). Selection of the proper (2) minimize maxillary and alveolar growth dis-
corrective surgical technique is patient depen- turbances, and (3) prevent fistula formation.
dent and should be based on the degree of palatal The previous cleft palate Maintenance of Cer-
involvement and the distance between the palatal tification in Plastic Surgery article published in
shelves. Repair of the hard palate typically uses var- 201015 reviewed evidence-based literature with a
ious axial pattern flaps based on the greater pala- specific focus on preoperative assessment, anes-
tine arteries, and commonly used techniques for thesia/analgesia, surgical technique, and patient
repair of the hard palate include von Langenbeck outcomes. In this article, we evaluate the interval
palatoplasty,1 Veau-Wardill-Kilner pushback,2–5
Bardach two-flap palatoplasty,6 and others.7
Repair of the soft palate requires dissection of the Disclosure: The authors have no financial interest
anomalous palatal musculature to reposition the to declare in relation to the content of this article.
levator palatini with variable manipulation of the
palatoglossus, palatopharyngeus, and tensor veli
palatini to restore the levator sling. Restoration of
Supplemental digital content is available for
this article. Direct URL citations appear in the
From the Department of Plastic Surgery, University Hospi- text; simply type the URL address into any Web
tals Case Medical Center. browser to access this content. Clickable links
Received for publication June 29, 2012; accepted July 13, to the material are provided in the HTML text
2012. of this article on the Journal’s Web site (www.
Copyright © 2013 by the American Society of Plastic Surgeons PRSJournal.com).
DOI: 10.1097/PRS.0b013e3182a80952

1644 www.PRSJournal.com
Volume 132, Number 6 • Cleft Palate

literature and review articles with a high level of


evidence on cleft surgery with a specific focus on
maxillary growth, postoperative speech changes,
and obstructive sleep apnea. We also provide
a synopsis of our approach and treatment of a
patient with cleft palate, and videos demonstrat-
ing the Furlow double-opposing Z-plasty in a
patient with submucous cleft palate and a two-flap
palatoplasty in a patient with complete bilateral
cleft palate. (See Video, Supplemental Digital
Content 1, which demonstrates Furlow palato-
plasty, available in the “Related Videos” section
of the full-text article on PRSJournal.com or, for
Ovid users, at http://links.lww.com/PRS/A916; and Video 1. Supplemental Digital Content 1, which demonstrates
Video, Supplemental Digital Content 2, which Furlow palatoplasty, is available in the “Related Videos” section
demonstrates two-stage palatoplasty, available of the full-text article on PRSJournal.com or, for Ovid users, at
in the “Related Videos” section of the full-text http://links.lww.com/PRS/A916.
article on PRSJournal.com or, for Ovid users, at
http://links.lww.com/PRS/A917.)

ARTICLE SEARCH AND EVALUATION


A search of the PubMed database was per-
formed using the following terms: “cleft palate,”
“obstructive sleep apnea,” “maxillary growth,”
“maxillary development,” “craniofacial growth,”
“veloplasty,” “velopharyngeal insufficiency,” and
“speech.” The search was limited to studies pub-
lished since the last Maintenance of Certification
in Plastic Surgery article in 2009. All abstracts
were reviewed to determine level of evidence
as defined by the American Society of Plastic
Surgeons Evidence Rating Scale for Therapy Video 2. Supplemental Digital Content 2, which demonstrates
(Table 1) and were grouped by area of focus two-stage palatoplasty, is available in the “Related Videos” sec-
(maxillary growth, speech, obstructive sleep tion of the full-text article on PRSJournal.com or, for Ovid users,
apnea). Studies of patients with bilateral cleft lip at http://links.lww.com/PRS/A917.
and palate were excluded from review; however,
studies evaluating outcomes for patients with patients who are yet to undergo surgical repair,
either isolated cleft palate or unilateral cleft lip and nonclefted controls, to better elucidate skel-
and palate were included. etal changes. Chen et al.16 compared patients with
uncorrected cleft palate to noncleft controls and
MAXILLARY AND FACIAL GROWTH demonstrated a significant reduction in maxillary
Cleft palate is known to alter normal facial sagittal length (anterior nasal spine to posterior
skeletal development. Two recent studies com- nasal spine) and maxillary retrusion (decreased
pared facial growth in patients after surgery, sella, nasion, A point angle) in the uncorrected

Table 1.  American Society of Plastic Surgeons Evidence Rating Scale for Therapeutic Studies
Level of Evidence Qualifying Studies
I High-quality, multicenter or single-center, randomized controlled trial with adequate power; or systematic
review of these studies
II Lesser-quality, randomized controlled trial; prospective cohort study; or systematic review of these studies
III Retrospective comparative study, case-control study, or systematic review of these studies
IV Case series
V Expert opinion; case report or clinical example; or evidence based on physiology, bench research, or “first
principles”

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Plastic and Reconstructive Surgery • December 2013

cleft group. Another study of dental-arch mor- repair at 6 months of age (Level of Evidence:
phology in patients with surgically corrected cleft Therapeutic, II). Cephalometric data at age 15
palate demonstrated a significantly decreased years was used to compare the patients to non-
maxillary arch width and anterior arch length clefted controls. At 10-year follow-up, the two-
compared with nonclefted control patients and stage repair demonstrated significantly reduced
patients with uncorrected isolated cleft palate.17 anteroposterior maxillary growth compared with
More controversial is how surgical repair, par- controls; however, no significant difference was
ticularly the timing, number of surgical stages, and seen between the two surgical groups. Finally, no
technique used, impacts maxillary growth. Several difference in sagittal growth on lateral cephalo-
authors argue that flap elevation damages the grams was seen in a study evaluating single-stage
periosteum and that subsequent postsurgical scar- cleft palate repair versus two-stage repair at 12
ring negatively impacts maxillary growth, which months (soft palate) and 30 months (hard palate)
could be minimized using a two-stage approach in a study by Zemann et al. (Level of Evidence:
with delayed repair of the hard palate.18–20 How- Therapeutic, III).24
ever, proponents of early, single-stage repair main- Another study examined the extent of muco-
tain that early closure improves speech outcomes periosteal flap elevation, which is believed to cause
through promotion of proper phonologic devel- maxillary growth disturbances, in patients under-
opment and decreases articulation disorders asso- going single-stage repair. Kulewicz and Dudkie-
ciated with velopharyngeal insufficiency, and that wicz25 evaluated long-term morphologic outcomes
the benefits of improved speech outcome out- using lateral cephalometric analysis after single-
weigh potential maxillary growth restriction that stage surgical correction at age 7 months in 66
may ensue.21 patients with unilateral cleft lip and palate (Level
Several recent studies comparing one-stage of Evidence: Therapeutic, III). The patients were
versus two-stage cleft palate repair report conflict- divided into three groups based on the technique
ing results with respect to subsequent maxillary of hard palate repair: group I, mucoperiosteal flap
growth restriction. Yamanishi et al.19,22 compared elevated on both sides of the cleft; group II, mini-
growth outcomes following a two-stage repair mal (2 to 3 mm) mucoperiosteal dissection on
consisting of Furlow palatoplasty at 12 months the cleft side and normal dissection on the non-
followed by hard palate closure at 18 months, cleft side; and group III, single-layer closure using
to a single-stage Wardill-Kilner pushback at 12 vomer mucoperiosteal flaps and minimal palatal
months of age in 30 patients (Reference 19, Level mucoperiosteal elevation bilaterally. Cephalomet-
of Evidence: Therapeutic, III). Cephalometric ric analysis demonstrated a significant reduction
analysis at 4 years of age demonstrated a statisti- in anteroposterior maxillary prominence (group
cally significant increase in maxillary length and I versus groups II and III) and anterior vertical
anterior facial height in patients who underwent maxillary height (group I versus group III). Group
a two-stage repair. In contrast, other studies have III had the lowest number of significant growth
failed to demonstrate significant maxillary growth disturbances on cephalometric measurements
disturbance following single-stage elevation of compared with noncleft age-matched controls;
palatal mucoperiosteal flaps. Pradel et al.21 found however, there was no statistically significant dif-
an increase in anterior and posterior transverse ference between the three groups. Finally, evalu-
dental arch width at 6 years of age when single- ation of the impact of cleft size, as a percentage
stage intravelar veloplasty of the soft palate and of total palatal surface area in 39 infants, using
bipedicled mucosal flap closure of the hard pal- lateral and posteroanterior cephalometric radio-
ate was performed at 9 to 12 months compared graphs, demonstrated a significant correlation
with patients undergoing two-stage repair. Liao et between increased cleft size and decreased maxil-
al.18 randomized 72 patients to either one-stage or lary length and protrusion.26
two-stage cleft palate repair with delayed closure
of the hard palate. In this study, serial cephalo-
metric analysis until 20 years of age demonstrated SPEECH OUTCOMES
no difference in growth rate between the groups A properly functioning velopharyngeal mech-
but a decrease in maxillary length and protrusion anism is critical to proper speech development
in patients who underwent single-stage repair. De and remains one of the primary goals of cleft
Mey et al.23 randomized 72 patients to single-stage palate repair. Repositioning of the levator sling
closure at 3 months of age or two-stage repair with and recreation of the normal anatomy results in
soft palate closure at 3 months and hard palate velopharyngeal competence for most patients

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Volume 132, Number 6 • Cleft Palate

and reduces the need for secondary pharyngeal veloplasty (Level of Evidence: Therapeutic, II).
flaps.27 In a study by Sullivan et al.,28 persistent Postoperative velopharyngeal function, measured
postoperative velopharyngeal insufficiency was using a cul-de-sac test of hypernasality and mir-
shown to be associated with patient age at palato- ror test of nasal air emission, was significantly
plasty (timing), and degree of palatal involvement improved in the group that had undergone Fur-
(Veau score) (Level of Evidence: Therapeutic/ low double-opposing Z-palatoplasty.
Prognostic, IV). They conclude that early, single-
stage repair reduces the development of learned,
compensatory misarticulations that can persist OBSTRUCTIVE SLEEP APNEA
despite surgical correction of the palatal anatomy, A recent retrospective study examined the inci-
but must be weighted against altered facial growth dence of sleep-disordered breathing or obstruc-
secondary to early disruption of the maxillary tive sleep apnea in 459 cleft palate patients and
periosteum and soft-tissue envelope. demonstrated a significantly increased incidence
Willadsen29 recently reviewed the impact of of sleep-disordered breathing (37.5 percent) and
the timing of hard palate repair on speech out- obstructive sleep apnea (8.5 percent) compared
comes. Thirty-four children underwent two-stage with age-matched controls (Level of Evidence:
cleft surgery with velar repair at 4 months of age Prevalence, IV).31 No recent studies have evalu-
and repair of the hard palate at either 12 months ated how surgical repair of the cleft palate may
(early) or 36 months (late) (Level of Evidence: help or worsen this condition.
Therapeutic, II). Evaluation 18 months postop-
eratively demonstrated that the group that under-
went late repair of the hard palate produced fewer CONCLUSIONS
labial stops and more velar stops, fewer vocaliza- The present literature review demonstrates
tions, and consonants permissible in word-initial that many of the important issues surround-
position. Thirty-six months postoperatively, this ing repair of the cleft palate remain controver-
group demonstrated a severely restricted phono- sial. Since the last Maintenance of Certification
logic system with more cleft speech characteristics. in Plastic Surgery article on cleft palate repair
Pradel et al.21 evaluated speech outcomes after by Liau et al.,15 there is still no consensus in the
either a single- or two-stage cleft palate repair interval literature regarding the proper timing of
(Level of Evidence: Therapeutic, III). Twelve chil- repair, number of stages, or surgical technique(s)
dren underwent a two-stage repair with intravelar to maximize speech outcomes and minimize the
veloplasty at age 9 to 12 months and repair of the impact on long-term skeletal growth and facial
hard palate using bipedicled flaps at age 24 to 36 development. Although cleft severity will always
months, and 12 children underwent a single-stage vary between patients, we believe that there should
intravelar veloplasty and bipedicled flap repair not be an unstandardized approach to surgical
at age 9 to 12 months. At 4 years of age, patients repair. We echo the assessment by Yang and Liao32
who underwent single-stage repair demonstrated who, following a literature review of facial growth
less altered resonance and less nasal emission. after one- or two-stage palate repair, concluded
When reevaluated at age 6 years, the children who that there is still a need for well-designed, ran-
underwent two-stage repair had improved their domized, prospective, long-term studies (Level
speech skills but still had not attained a level equal of Evidence: Therapeutic, III). Studies with a
to patients who had undergone single-stage palate high level of evidence will possibly one day guide
repair. This is in contrast to a study by Yaminishi selection of surgical technique and the timing of
et al.19 that compared 30 patients who underwent repair to optimize surgical and patient outcomes.
early two-stage double-opposing Z-plasty at 12 In the absence of such a consensus, we advocate
months and hard palate repair at 18 months, to a single-stage repair of the hard and soft palate
42 patients who underwent single-stage Wardill- at 9 to 12 months of age as demonstrated in the
Kilner push-back palatoplasty at 12 months of age. accompanying videos. (See Video, Supplemen-
In this study, no statistically significant difference tal Digital Content 1, http://links.lww.com/PRS/
was seen in the incidence of velopharyngeal insuf- A916; and Video, Supplemental Digital Content
ficiency or articulation errors at 4 years of age. 2, http://links.lww.com/PRS/A917.) We believe that
Two techniques of palatal repair were com- early surgical repair significantly improves speech
pared by Williams et al.,30 who randomized patients outcomes and that there is currently insufficient
to either a Furlow double-opposing Z-plasty or evidence demonstrating a clinically relevant max-
a von Langenbeck palatoplasty with intravelar illary growth disturbance relative to two-stage

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Plastic and Reconstructive Surgery • December 2013

or late repairs. We institute regular orthodontic 16. Chen ZQ, Qian YF, Wang GM, Shen G. Sagittal maxillary
follow-up through a multidisciplinary team begin- growth in patients with unoperated isolated cleft palate. Cleft
Palate Craniofac J. 2009;46:664–667.
ning at age 3 years, allowing us to monitor facial 17. Ye B, Ruan C, Hu J, et al. A comparative study on dental-arch
and dental development carefully and initiate morphology in adult unoperated and operated cleft palate
palatal expansion with the onset of mixed denti- patients. J Craniofac Surg. 2010;21:811–815.
tion when necessary. Regular visits through the 18. Liao YF, Yang IY, Wang R, Yun C, Huang CS. Two-stage palate
multidisciplinary team can identify and prepare repair with delayed hard palate closure is related to favor-
able maxillary growth in unilateral cleft lip and palate. Plast
patients who may require subsequent orthogna- Reconstr Surg. 2010;125:1503–1510.
thic surgery. We believe that late correction of 19. Yamanishi T, Nishio J, Sako M, et al. Early two-stage dou-
skeletal deficiency can yield a normal facial pro- ble opposing Z-plasty or one-stage push-back palatoplasty?
file, whereas late correction of refractory errors Comparisons in maxillary development and speech outcome
secondary to prolonged velopharyngeal insuffi- at 4 years of age. Ann Plast Surg. 2011;66:148–153.
20. Friede H, Lilja J, Lohmander A. Long-term, longitudinal
ciency is a much more difficult prospect, with far follow-up of individuals with UCLP after the Gotenburg
less satisfying results. Primary Early Velopalsty and Delayed Hard Palate Closure
Protocol: Maxillofacial growth outcome. Cleft Palate Craniofac
Arun K. Gosain, M.D. J. 2012;49:649–656.
225 E. Chicago Avenue, Box 93 21. Pradel W, Senf D, Mai R, Ludicke G, Eckelt U, Lauer G. One-
Chicago, Ill. 60611 stage palate repair improves speech outcome and early max-
argosain@luriechildrens.org illary growth in patients with cleft lip and palate. J Physiol
Pharmacol. 2009;60(Suppl 8):37–41.
22. Yamanishi T, Nishio J, Kohara H, et al. Effect on maxillary
ACKNOWLEDGMENT arch development of early 2-stage palatoplasty by modified
The authors offer special thanks to Matt Sugerik for furlow technique and conventional 1-stage palatoplasty in
help in recording and editing the surgical videos. children with complete unilateral cleft lip and palate. J Oral
Maxillofac Surg. 2009;67:2210–2216.
23. De Mey A, Franck D, Cuylits N, Swennen G, Malevez C,
Lejour M. Early one-stage repair of complete unilateral cleft
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