Phẫu Thuật Tạo Hình (Tập 3): Sọ Mặt, Đầu Cổ Và Nhi Khoa – Neligan (Ấn bản 4)

SECTION I • Clefts
SYNOPSIS
■ Individuals with known or suspected velopharyngeal dysfunction (VPD)
are best treated in the context of an interdisciplinary cleft/craniofacial
team.
■ Diagnosis of VPD requires obtaining a comprehensive patient history,
perceptual speech evaluation, physical examination, and appropriate
instrumental and imaging studies.
■ Successful surgical management of VPD requires precision in
diagnosis and individualization of treatment.
■ VPD may be the result of velopharyngeal insufficiency, velopharyngeal
incompetence, or velopharyngeal mislearning.
■ Flexible fiberoptic nasopharyngoscopy should be completed as part of
a standard preoperative evaluation to allow for direct visualization of
the velopharyngeal mechanism during speech and surgical planning.
■ Instrumental assessment of speech should always be interpreted in the
context of the results of a comprehensive perceptual speech
evaluation.
■ Aerodynamic assessment of speech can provide the surgeon with
information regarding velopharyngeal orifice size and timing to assist
with treatment decision-making and judgment of surgical outcome.
■ The primary goal of surgical management is to produce a competent
velopharyngeal mechanism for speech while avoiding the
complications of nasal airway obstruction.
Introduction
Normal speech is dependent upon the functional and struc­
tural integrity of the velopharynx, a complex and dynamic
structure that serves to decouple the oral and nasal cavities
during sound production. Dysfunction of the velopharyngeal
valve (referred to as VPD) may lead to hypernasality, nasal air
emission, and compensatory articulation errors, all of which
may impair speech intelligibility and lead to stigmatization.
There may also be “non-speech” sequelae of VPD including
nasal regurgitation. The goal of surgical intervention is to
produce or restore velopharyngeal competence while avoid­
ing the complications of upper airway obstruction. Successful
surgical management of VPD requires precision in diagnosis
and individualization of treatment. Thus, optimization of
surgical outcome is critically dependent upon a careful analy­
sis of each patient’s history, speech characteristics, structural
anatomy, and velopharyngeal dynamics – an analysis best
performed with close collaboration between the surgeon, the
speech pathologist, and the other members of the cleft/
craniofacial team.
Anatomy and physiology of
the velopharynx
Anatomy
The velopharyngeal port is defined by the soft palate, or
velum, by the lateral pharyngeal walls, and by the posterior
pharyngeal wall. Closure of the velopharynx during speech
is a subconscious and automated action that is mediated by
the motor cortex and that requires the coordinated action of
the velopharyngeal musculature. The muscles of the soft
palate include the levator veli palatini, the tensor veli palatini,
the palatoglossus, the palatopharyngeus, and the musculus
uvulae (Fig. 26.1). The levator takes its origin from the petrous
portion of the temporal bone and from the medial aspect of
the eustachian tube. Its fibers course anteriorly, inferiorly, and
medially, inserting into the palatal aponeurosis and decussat­
ing with the levator fibers from the opposite side (Fig. 26.2).
Contraction of the muscular sling formed by the paired leva­
tors is the primary mechanism for velar elevation and closure
of the velopharyngeal port, although evidence suggests that
the palatoglossus and palatopharyngeus muscles may act as
antagonists to the levators to provide fine motor control of
velar position during speech.1,2 The musculus uvulae is a
paired intrinsic muscle that likely contributes to velopharyn­
geal closure both by adding bulk to the dorsal surface of the
velum and by contributing to velar stretch.3–5 It is usually
26 
Velopharyngeal dysfunction
Richard E. Kirschner and Adriane L. Baylis
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620
SECTION I
CHAPTER 26 • Velopharyngeal dysfunction
With the exception of the tensor veli palatini, which is
innervated by the third division of the trigeminal nerve (V3),
all of the muscles of the velopharynx receive motor innerva­
tion from the pharyngeal plexus, which is composed of fibers
from the glossopharyngeal (IX), vagus (X), and accessory (XI)
nerves.9 Studies have suggested that the facial nerve (VII) may
also play a minor role in velopharyngeal motor function.10,11
It is important to note that, although the functional activity of
the velopharyngeal valve during speech and swallowing may
be similar, the neurological pathways for these activities are
distinct. Velopharyngeal movements for speech are learned,
automatized activities that are controlled by the motor cortex,
absent in patients with overt and submucosal clefts of the
palate.6
The superior pharyngeal constrictor is a broad, thin muscle
that takes origin from the velum, the medial pterygoid, and
the pterygomandibular raphe, inserting into the median
pharyngeal raphe along with the constrictor muscle fibers
from the opposite side. Contraction of the superior constrictor
may contribute to velopharyngeal closure by effecting medial
movement of the lateral walls and anterior movement of the
posterior wall of the velopharynx.7,8 The anatomy of the
superior constrictor and its contribution to velopharyngeal
closure, however, are highly variable.
Fig. 26.1  Muscles of the velopharynx.
Hamulus
Palatoglossus
Palatopharyngeus
Musculus uvulae
Levator veli palatini
Fig. 26.2  Schematic of the levator veli palatini.
(Cut)
Eustachian tube
Palatal aponeurosis
Normal newborn
Vomer
Maxilla
Alveolar process
of maxilla
Superior pharyngeal constrictor
Levator veli palatini

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Basic science/disease process of velopharyngeal dysfunction
patterns of velopharyngeal closure observed in normal sub­
jects (Fig. 26.4): (1) coronal, in which closure is effected pri­
marily by velar elevation; (2) circular (with or without
Passavant’s ridge), in which medial movement of the lateral
pharyngeal walls contributes to velopharyngeal closure in
near-equal proportion to the velum; and (3) sagittal, in which
closure is effected primarily by medial movement of the
lateral pharyngeal walls and the velum contacts the lateral
walls rather than the posterior wall. Of these, the coronal
pattern of closure is observed most commonly in both normal
individuals and in patients with VPD. In some individuals, a
localized transverse ridge of tissue may be seen to form on
the posterior pharyngeal wall during speech. This anterior
movement of the posterior pharyngeal wall during velopha­
ryngeal closure was first described by Passavant in 186318 and
is therefore frequently referred to as “Passavant’s ridge”.
Although some have written that its appearance is always
indicative of pathologic velopharyngeal function, Croft et al.17
have demonstrated that Passavant’s ridge may play a role in
velopharyngeal closure in both normal speakers and in those
with VPD.
Electromyographic studies support the notion that normal
velopharyngeal function requires the central coordination of
velopharyngeal muscle activity with other articulatory move­
ments.19 Changes in velar position during sound production
represent the end result of a complex interaction of several
interrelated variables, including auditory and proprioceptive
feedback. Moreover, for a single individual, there may be
significant flexibility in the system of sound production such
that there may be a limited but variable repertoire of velopha­
ryngeal movements that may produce the same perceived
sounds. Despite decades of speech science research, the
precise neurophysiology of both normal and abnormal velo­
pharyngeal function remains incompletely understood.
MRI has quickly gained ground as the primary method for
studying the anatomy, and emerging physiologic measures,
of velopharyngeal structures. Perry et al.20 and Bae et al.21 have
shown the application of both traditional and 3D MRI for
evaluation of velopharyngeal anatomy in persons with and
without clefts. MRI has also been utilized to explore sexual
dimorphism in velopharyngeal structures as well as how
velopharyngeal anatomy and physiology changes over time
with growth and aging.22 Lastly, dynamic MRI during speech
is now feasible in the clinical setting as a complementary tool
for clinical evaluation of VP function for speech.23,24
Basic science/disease process of
velopharyngeal dysfunction
Velopharyngeal insufficiency
The first major diagnostic category of VPD is velopharyngeal
insufficiency, a term used to denote an anatomic, or structural,
defect responsible for inadequate closure of the velopharyn­
geal valve. Such defects may be congenital, as in cases of cleft
palate or congenital velopharyngeal disproportion (i.e., a
short soft palate relative to the depth of the pharynx) (Fig.
26.5), or they may be secondary to surgical procedures that
alter velopharyngeal anatomy, as in cases of palatoplasty,
tumor resection, or adenoidectomy. The most common
whereas similar movements for swallowing are primarily
involuntary activities that originate from the brainstem.
Physiology
The velopharynx is a complex, three-dimensional valve which
serves to uncouple the oropharynx and nasopharynx during
speech and swallowing. This section will briefly discuss
velopharyngeal function during speech.
It is widely accepted that the levator veli palatini is the
muscle that is primarily responsible for velar motion and,
hence, for velopharyngeal closure.12 Fine motor control of
velar position may also be governed by the palatoglossus and
palatopharyngeus. As noted above, the paired musculus
uvulae may play an important role in velar stretch and in
filling the gap between the velum and the posterior pharynx
during velopharyngeal closure. The relative contribution of
the levator and of the superior pharyngeal constrictor to
lateral pharyngeal wall movement has been the subject of
some debate.
In normal individuals, the velum lifts posteriorly and
superiorly during velopharyngeal closure. The normal point
of contact with the posterior pharyngeal wall is located
approximately three-quarters of the way back on the velum
from the posterior nasal spine (Fig. 26.3). The site of velopha­
ryngeal closure is usually at or just inferior to the palatal plane
which is typically at or above the level of the first cervical
vertebrae (C1), but velar height, as well as the extent of velo­
pharyngeal contact, varies systematically depending upon the
phonetic context, speech accuracy, rate, and effort.13–15
The contribution of lateral pharyngeal wall movement to
velopharyngeal closure varies amongst individuals with or
without cleft palate and, as with velar movement, varies with
the phonemic task. Maximal lateral pharyngeal wall displace­
ment generally occurs at the level of velopharyngeal contact.
Skolnick et al.16 and Croft et al.17 have described three basic
Fig. 26.3  Lateral view of normal velopharyngeal closure for speech during
production of an oral pressure consonant.

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SECTION I
CHAPTER 26 • Velopharyngeal dysfunction
nasopharynx than controls, likely the result of alterations in
cranial base dimensions.26–28 Osborne et al.29 and Ross and
Lindsay30 have shown that a higher prevalence of upper cervi­
cal spine abnormalities in patients with clefts may result in
increased pharyngeal depth. Likewise, platybasia, or flatten­
ing of the cranial base angle, may contribute to VPD by
increasing pharyngeal depth, and thus the depth-to-length
ratio. Ruotolo et al.31 have shown that patients with 22q11.2
deletion syndrome, a condition associated with a high fre­
quency of severe non-cleft VPD, demonstrate several predis­
posing skeletal and soft-tissue anomalies, including increased
pharyngeal depth, platybasia, and cervical spine anomalies.
Velopharyngeal insufficiency may also be caused by post­
surgical changes in velopharyngeal anatomy. In young chil­
dren, velopharyngeal closure is most often velar-adenoidal.
Removal of hyperplastic adenoids for the management of
nasopharyngeal airway obstruction or chronic otitis media
results in an acute increase in pharyngeal depth. In the major­
ity of non-cleft patients, the capacity for velar stretch allows
the palate to accommodate for this change. In most cases,
postadenoidectomy velopharyngeal insufficiency is transient,
congenital structural defects associated with VPD are cleft
palate and submucosal cleft palate. The reported incidence of
persistent VPD after cleft palate repair varies widely and is
influenced by a large number of variables. In a recent report
of long-term outcomes following Furlow palatoplasty, sec­
ondary surgery for VPD was only clinically indicated in 8%
of children at five years of age.25 In the absence of an oronasal
fistula, however, VPD after palatoplasty is most commonly
the result of impaired velar mobility, velopharyngeal dispro­
portion, or a combination of both.
Since adequacy of velopharyngeal closure is largely a func­
tion of the ratio of pharyngeal depth to palatal length, patients
with a proportionally short palate or deep pharynx may
demonstrate incomplete velopharyngeal closure (see Fig.
26.5). Each of these conditions may be the result of either a
congenital anomaly or an iatrogenic alteration in velopharyn­
geal architecture. Cicatricial changes following palatoplasty,
for example, may lead to velopharyngeal insufficiency sec­
ondary to velar shortening. Congenital differences in skeletal
architecture may also play a role in postpalatoplasty VPD.
Patients with clefts have been demonstrated to have a broader
Fig. 26.4  Types of velopharyngeal closure patterns.
Coronal
Sagittal
Circular
Circular with Passavant’s ridge

623
Basic science/disease process of velopharyngeal dysfunction
velopharyngeal insufficiency, except that many of the indi­
viduals also demonstrate other features of dysarthria or other
motor speech impairments (e.g., apraxia). Abnormal innerva­
tion of cranial nerves IX, X, or XI, or abnormalities in muscle
tone/function may result in abnormal timing of palatal eleva­
tion which may also exacerbate the perception of hypernasal
speech, in excess of that predicted by velopharyngeal gap size
alone. In addition, adults with velopharyngeal incompetence,
depending on the etiology, frequently exhibit dysphagia and
varying degrees of nasal regurgitation.
There are extensive possibilities for neurologic diagnoses
that may be associated with congenital velopharyngeal
incompetence including, but not limited to, cerebral palsy,
myotonic dystrophy, and congenital hypotonia. Asymmetrical
velopharyngeal function, such as that typical of patients with
hemifacial microsomia, is also a common cause of velopha­
ryngeal incompetency. Acquired or “late” causes of velopha­
ryngeal incompetence may include traumatic brain injury;
cerebrovascular accident or brainstem stroke; and progressive
diseases such as Parkinson’s disease, amyotrophic lateral
sclerosis, muscular dystrophies, multiple sclerosis, and other
demyelinating diseases.35–39
Children and adults with motor speech disorders have also
been shown to demonstrate symptoms of velopharyngeal
incompetence of varying degrees of severity and consistency.
Apraxia of speech (also referred to as developmental apraxia
of speech or childhood apraxia of speech, in children) is a
neurologic condition resulting in difficulties with speech
motor programming and control.40 Apraxia may be character­
ized by inconsistent symptoms of VPD such as inconsistent
nasalization of vowels or consonants and inconsistent nasal
emission. In addition, many individuals with apraxia may
exhibit a combination of both inconsistent hypernasality and
hyponasality, providing additional evidence of the abnormal
coordination of the palate during speech sound production.
Younger children with apraxia may demonstrate some over­
lapping speech features often seen in children with congenital
velopharyngeal incompetence of other causes. For example,
children with a history of VPD may have a limited inventory
of sounds and demonstrate difficulties learning to produce
oral consonants in the first few years of life. These children
may produce a pattern of compensatory articulation errors
(i.e., glottal stop substitutions) or omit sounds completely.
Children with apraxia have difficulty with generating the
appropriate “motor program” (blueprint) for producing the
sequence of motor movements to produce a sound, which is
not typical of children with isolated VPD or clefting. The
importance of obtaining a thorough speech pathology evalu­
ation to diagnose these conditions differentially is critical for
appropriate treatment decision-making.
Lastly, stress velopharyngeal incompetence is a special case
of velopharyngeal incompetence for non-speech behaviors. It
is most commonly observed in wind musicians given the high
pressure demands.41,42 There may or may not be comorbid
hypernasality or nasal emission in speech. In some cases,
stress velopharyngeal incompetence may be an indicator of
an underlying physical cause of velopharyngeal incompe­
tence, which may have been masked, or very mild, in the past.
In some cases, neurologic or structural causes of VPD (e.g.,
submucous cleft palate) are diagnosed following the presenta­
tion of stress velopharyngeal incompetence, further empha­
sizing the importance of a thorough clinical evaluation of all
and resonance returns to normal within 6–12 months. VPD
may persist, however, in a small number of patients, some of
whom may have predisposing factors for VPD, including
submucosal cleft palate, a short velum, a deep pharynx, or
neuromuscular disorders. For patients with these conditions,
the adenoids may play a critical role in velopharyngeal
closure, and even their normal involution may result in velo­
pharyngeal insufficiency.12 Careful assessment of velopharyn­
geal anatomy is therefore essential in all patients prior to
adenoidectomy and should be avoided if possible when
anatomical factors that predispose to VPD are identified.
In some patients, irregularity of the surface contour of the
adenoid pad may interfere with the ability of the velum to
achieve complete velopharyngeal closure.32 In others, enlarged
tonsils may intrude between the velum and the posterior
pharyngeal wall, resulting in incomplete closure.33,34 In these
cases, the first step in management should be a selective
adenoidectomy or tonsillectomy, respectively, as such may be
sufficient to solve the problem, obviating the need for any of
the surgical procedures later described.
Velopharyngeal incompetence
The second major diagnostic category of VPD is velopharyn­
geal incompetence. This label is typically reserved for those
cases of VPD known or suspected to be due to congenital or
acquired neurological and/or neuromuscular causes such as
cerebrovascular incidents, traumatic brain injury, brain tumor,
abnormalities in muscle tone or function, and degenerative
neuromuscular diseases. In velopharyngeal incompetence,
there is typically no evidence of any underlying structural
abnormality and palatal length is sufficient; however, the
function of the velopharyngeal mechanism is suboptimal for
speech production and/or swallowing. The speech sequelae
of velopharyngeal incompetence are similar to that seen with
Fig. 26.5  Lateral view of inadequate velopharyngeal closure for speech due to a
short soft palate (velopharyngeal insufficiency). The arrows represent the escape of
air, pressure, and acoustic energy through the nasal cavity during speech.

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SECTION I
CHAPTER 26 • Velopharyngeal dysfunction
history and physical exam, perceptual speech evaluation,
imaging and acoustic measures, and team discussion for treat­
ment planning. The following information should be obtained
during a patient interview when undergoing evaluation for
VPD:
■ Current patient/family concerns with speech
■ Pregnancy history, complications, medication use, and
any exposure to teratogens
■ Birth and delivery history and complications
■ Primary medical diagnoses (e.g., cleft palate, syndromes,
cardiac defects, neuromuscular disease)
■ History of any feeding or swallowing difficulties during
infancy and any current swallowing concerns, including
nasal regurgitation and difficulty with breastfeeding or
bottlefeeding during infancy
■ History of hearing loss or ear disease, including history
of frequent ear infections or effusions
■ History of snoring or symptoms of sleep apnea
■ Surgical history, including prior tonsillectomy,
adenoidectomy, and, if appropriate, cleft-related surgical
history and timing
■ History of any genetic testing and results
■ Family history of cleft lip/palate, nasal speech, speech
delay or articulation/pronunciation difficulties, hearing
loss, learning disabilities, and medical conditions
■ Developmental history
■ Speech therapy history.
Every patient, regardless of age, should undergo direct cra­
niofacial and oral examination by the surgeon and speech
pathologist with experience in clefting/craniofacial anoma­
lies. The oral exam should be completed in an appropriate
examination room and with appropriate lighting. Compo­
nents of the examination should include an assessment of:
■ Craniofacial symmetry
■ Oral–facial movement and symmetry
■ Dentition and occlusion
■ Presence and location of any fistulae
■ Presence of signs of submucous cleft palate, including
bifid uvula, zona pellucida, and palpate for notch
■ Soft palate length, symmetry, and degree of elevation and
symmetry during phonation
■ Tonsil size and symmetry.
The observations from the physical exam should be inter­
preted together with the clinical speech evaluation results. For
example, if an exam reveals a submucous cleft palate but the
patient has normal speech, surgical management would not
be recommended. On the other hand, a normal oral exam with
clinical speech findings suggestive of severe VPD does not
negate the need for physical treatment. The findings from the
oral examination may provide hints regarding the source or
cause of VPD; however, imaging studies should be completed
to confirm the etiology, size/shape, and consistency of the
velopharyngeal gap, and to assess the surrounding anatomy
of the upper airway.
Perceptual speech evaluation
Perceptual speech assessment is considered the gold standard
in the diagnosis of speech disorders of persons with cleft
patients with any form of VPD.43 Treatment for stress velopha­
ryngeal incompetence may follow a similar course as that for
VPD for speech, although there have been reported cases of
spontaneous recovery after a period of rest.
Velopharyngeal mislearning
The third, and lesser known, type of VPD involves velopha­
ryngeal mislearning.44 In this category, the velopharyngeal
mechanism appears to be anatomically and physiologically
capable of consistent and complete velopharyngeal closure
for speech, despite the observation of inconsistent velopha­
ryngeal closure for speech. In this type of VPD, the patient has
mislearned how to produce certain speech sounds accurately.
The most common example is that of phoneme-specific nasal
emission, in which nasal airflow is produced as a complete
substitution for an oral consonant, despite adequate velopha­
ryngeal closure ability for other consonants.45 Clinically, this
will often be observed in a child with nasal emission heard
on a selected set of sounds, most commonly S, Z, SH, or CH,
but not on other sounds such as P, B, T, D, K, G. Another
example of velopharyngeal mislearning is the case in which a
child produces compensatory articulation errors (e.g., glottal
stops) which may prevent or interfere with the achievement
of adequate velopharyngeal closure for speech. The velopha­
ryngeal movements during the production of these aberrant
speech errors have been shown to be counterproductive (distal
versus medial movement of the lateral pharyngeal walls) to the
achievement of velopharyngeal closure for speech. A related
example is that seen in children with congenital hearing loss
with an inability to self-monitor their own speech production,
resulting in nasalized speech errors with an otherwise physi­
ologically intact velopharyngeal mechanism.
Velopharyngeal mislearning should be treated with behav­
ioral speech therapy, not surgery. It is critical that a well-
trained speech pathologist perform a thorough clinical
evaluation to diagnose such conditions differentially in order
to make the most appropriate treatment recommendations.
Combined types
In some cases, individuals with craniofacial anomalies and/
or clefting may exhibit a combined disorder with evidence of
velopharyngeal insufficiency, velopharyngeal incompetency,
and/or velopharyngeal mislearning, which poses a diagnostic
challenge. Some patients with 22q11.2 deletion syndrome
have been shown to demonstrate evidence of a combined type
of VPD due to the combination of structural clefting disorders,
increased pharyngeal depth, and hypotonia of the velophar­
ynx.46 Regardless of the type of VPD which is present, a
complete and thorough physical exam, clinical speech evalu­
ation, and any necessary instrumental or imaging studies
should be completed to confirm the etiology and identify the
most appropriate treatment plan.
Diagnosis/patient presentation
Patient history and physical exam
Individuals with known or suspected VPD are best treated in
the context of an interdisciplinary cleft palate team. Regard­
less of age, the clinical examination typically includes a brief

625
Diagnosis/patient presentation
reading passage is suggested for use with adolescent and
adult patients. Articulation skills should be assessed with
standardized measures (i.e., a standardized articulation test),
as well as word and sentence repetition tasks (e.g., American
English Sentence Sample50,51). Oral-only or nasal-only stimuli
(e.g., Buy baby a bib, Pet the puppy, Mama made muffins, etc.)
are also used to assess resonance, nasal emission (audible
and inaudible), and pressure for consonants. Special mirrors
or listening tubes may also be utilized to evaluate the pres­
ence of inaudible nasal emission. The speech parameters are
typically rated with five- or seven-point equal-appearing
interval scales or other ratio-based scaling methods (e.g.,
visual analog scales).47 The Cleft Audit Protocol for Speech-
Augmented (CAPS-A)52 is one rating tool that is now widely
used in many cleft centers with adaptations now available
for American English as well (CAPS-A-Americleft modifica­
tion53). Audio or video recording of the speech examina­
tion should be completed whenever possible for clinical
archiving, comparison pre–post treatment, assessment of
speech outcome, and for potential research purposes. If
clinical symptoms of VPD are present, additional diagnostic
testing should follow. Standard speech evaluations for the
cleft or VPD population should occur on at least an annual
basis and more frequently if there are changing needs (e.g.,
postsurgery, post-therapy). Speech evaluations after surgical
management (e.g., pharyngeal flap) should occur at least 3–6
months postsurgery to allow for adequate time for healing,
decrease in postoperative edema, and an initial period in
which patients can “practice” speech with their newly modi­
fied speech mechanism.
palate and VPD.47 Additional instrumental assessment and
imaging are considered adjunct to the perceptual speech find­
ings, which are the ultimate arbiter of a patient’s need for
treatment. Perceptual speech evaluation of this patient popu­
lation should be completed by a speech pathologist with
coursework, training, and continuing education in the area of
cleft palate and craniofacial anomalies.
During the speech evaluation, the speech pathologist
obtains the necessary clinical information regarding the pres­
ence and perceived severity of VPD, identifies and describes
speech characteristics, determines a suspected etiology, and
makes preliminary decisions regarding treatment recommen­
dations to discuss with the team. In addition, the speech
pathologist is making diagnostic decisions regarding the pres­
ence of comorbid conditions such as articulation disorders,
voice disorders, and language difficulties. Box 26.1 provides
a list of common speech pathology terminology used for
describing the speech characteristics associated with VPD.
The most common speech sequelae of VPD include reduced
speech intelligibility; articulation disorders ranging from mild
to severe articulation disorders (e.g., pervasive use of glottal
stop substitutions); reduced intraoral pressure for oral conso­
nants; audible nasal emission or nasal turbulence on oral
pressure consonants; hypernasal resonance; possible hoarse­
ness; and decreased loudness.47–49
The components of a standard speech evaluation for the
assessment of velopharyngeal closure for speech should
include an assessment of intelligibility, resonance, voice,
and articulation during a spontaneous speech sample, con­
versation, and/or picture description tasks.50 A standard
BOX 26.1  Common speech pathology terminology
Intelligibility: perceived amount of speech (i.e., number of words)
understood
Resonance: the perceptual balance of oral and nasal sound energy
in speech. In speakers with velopharyngeal dysfunction, there is
an abnormal escape of excessive nasal sound energy through
the velopharyngeal port and into the nasal cavity, which is
referred to as hypernasality
Hypernasality: perception of excessive nasal sound energy in
speech, typically on vowels, glides (W, Y), and liquid sounds
(L, R)
Hyponasality: perception of decreased nasal sound energy in
speech, typically on nasal sounds M, N, and usually due to
structural obstruction (e.g., enlarged adenoid pad, nasal
congestion)
Mixed resonance: combination of both hypernasality and
hyponasality perceived by a listener. Cul-de-sac resonance is
sometimes considered a form of mixed resonance in which
sound energy escapes to the anterior nasal cavity and becomes
trapped by some form of nasal obstruction or constriction, such
as a deviated septum
Nasal emission: abnormal escape of airflow through the nose during
consonant production (can be audible or inaudible). When
audible, may also be referred to as nasal turbulence
Compensatory articulation errors: a category of articulation errors
typically observed in populations with cleft palate or
velopharyngeal dysfunction, believed to result from an active
strategy to regulate pressure and airflow for speech. Typically
includes a pattern of producing sounds in a posterior place of
the vocal tract such as the pharynx or larynx, where pressure
and airflow can be “valved” prior to their escape to the level of
the velopharynx or oral cavity
Glottal stop substitutions: the most common type of compensatory
articulation errors seen in children with cleft palate or a history of
velopharyngeal dysfunction, produced by adducting the vocal
folds together and abruptly releasing the pressure beneath to
create the sound of an oral pressure consonant. Often used as a
replacement (substitution) for pressure consonants like P, B, T, D,
K, G
Nasal substitutions: the active replacement of oral sounds P, B, T, D
with nasal sounds M, N
Active nasal fricative: a learned articulatory behavior in which an oral
sound (usually S, SH, CH) is replaced with a voiceless nasal
sound (i.e., all airflow is emitted through the nose); this is
sometimes accompanied by a nasal grimace
Weak pressure consonants: the perception of decreased pressure in
oral consonants such as P, B, T, D, F, resulting from a fistula or
velopharyngeal gap, causing these sounds to take on
nasalization (e.g., the B sound is perceived as an M, the D is
perceived as an N), even though the speaker is accurately
attempting to produce the correct sound. Often co-occurs with
nasal emission
Sibilant distortions: incorrect tongue placement resulting from faulty
learning or malocclusion, resulting in imprecise production of
sounds S and Z

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CHAPTER 26 • Velopharyngeal dysfunction
the nasal sound energy divided by the sum of the oral plus
nasal sound energy in the speech signal.54 The patient wears
a specialized headpiece with nasal and oral microphones that
capture the speech signal while the patient reads or repeats a
standardized speech sample (see Fig. 26.6). Automated analy­
sis provides a nasalance score (expressed as a percentage),
which is then interpreted against the perceptual speech obser­
vations. Nasalance can range from 0 to 100%; higher numbers
represent a higher degree of nasality in speech. A variety of
normative and “cutoff” scores have been suggested, which are
dependent upon the type of speech stimuli used for the
nasalance score calculation.55–58 Surgeons and clinicians should
exercise caution in reliance on nasalance scores, however, due
to the variety of potential confounding variables that can
artificially inflate or reduce nasalance scores. These include
nasal turbulence, articulation errors, vocal hoarseness, mixed
resonance, and equipment placement variations, which may
reduce with the validity of this measure.58–60 Nasalance should
be considered a supplement to the perceptual speech evalua­
tion, not a substitute for it.
Pioneered by Warren and colleagues,61,62 pressure–flow
testing was developed to obtain quantitative measurement of
intraoral and nasal pressure and airflow, velopharyngeal
orifice size, and velopharyngeal closure timing for speech.
The schematic in Fig. 26.7 illustrates the type of instrumenta­
tion and set-up often utilized for aerodynamic assessment of
velopharyngeal closure for speech. Custom-designed and
commercially available aerodynamic systems, e.g., PERCI-
SARS (Microtronics), offer both clinical and research applica­
tions for the assessment of velopharyngeal closure for speech.
Warren and others61–66 suggested the use of the word “hamper”
as the speech stimulus for pressure–flow testing because of its
/mp/ sound sequence which requires the velopharynx to
open and close rapidly. An orifice of 10–20 mm2 (or larger)
during the /p/ sound of this stimulus has been shown to
correlate highly with perceptual observations of hypernasal­
ity.62 Some studies have suggested that even smaller gap sizes
may be clinically significant, especially when variations in
velopharyngeal timing are also present.46,64–66 Pressure–flow
Indirect measures of velopharyngeal closure
for speech
When clinical speech evaluation suggests the presence of
VPD, instrumental assessment of speech and velopharyngeal
closure may be useful as an adjunct to perceptual judgments.
Instrumental measures can provide confirmation of percep­
tual judgments and further evidence of the need for interven­
tion, as well as allow for objective pre–post treatment
measurements. The most popular clinical tools for indirect
instrumental evaluation include acoustic assessment of nasal­
ity and aerodynamic testing.
Nasalance is an acoustic index of nasality which has been
shown to correlate with perceptual judgments of resonance.54
Nasalance can be measured using commercially available
products such as the Nasometer (Kay Pentax) (Fig. 26.6),
Nasality Visualization System (Glottal Enterprises), Nasalview
(Tiger DRS), and other similar systems. Nasalance is a ratio of
Fig. 26.6  Nasometer II. (Courtesy of Kay Pentax.)
Fig. 26.7  Pressure–flow instrumentation for measuring intraoral pressure, nasal airflow, velopharyngeal orifice size, and velopharyngeal closure timing during speech.
Flow amplifier
Pressure
amplifier
Pressure
amplifier
Computer
Flow transducer
Heated flowmeter
Pressure
transducer
Pressure
transducer
Catheters

627
Diagnosis/patient presentation
no way of assessing connected speech and the dynamic func­
tion of the velopharyngeal mechanism.68
Multiview videofluoroscopy (Videos 26.1 and 26.2 )
During multiview videofluoroscopy, a connected speech
sample is recorded while motion fluoroscopy records the
movement of the velopharyngeal mechanism from multiple
angles. The benefit of this imaging approach is that it requires
a lower degree of cooperation (as compared to nasopharyn­
goscopy) and also provides information regarding palatal
length, pharyngeal depth, velopharyngeal gap size, and tonsil
and adenoid size. Clinicians can examine the velopharyngeal
mechanism during connected speech (as compared to static
images), which results in increased sensitivity to detect a
smaller or inconsistent velopharyngeal gap; however, the
radiation dose is higher than that of a traditional static radio­
graph. In this procedure, barium contrast is often instilled
through the nose to help highlight the nasal surface of the
velum and posterior pharyngeal wall, to aid in identification
of the velopharyngeal gap. Multiple angles can be obtained,
including lateral, frontal, base, and Towne’s, with the lateral
view as the most common.69 Due to radiation exposure and
the availability of other imaging options, videofluoroscopy
for speech is becoming less common at many cleft centers.
Nasopharyngoscopy
Nasopharyngoscopy involves the passage of a flexible fiber­
optic endoscope into the nasal cavity. Once the scope is
positioned slightly above the velopharyngeal port, the view
should allow for complete observation of all velopharyngeal
structures during speech and swallowing, including anteri­
orly, the soft palate; posteriorly, the posterior pharyngeal wall
or adenoid pad; and laterally, the lateral pharyngeal walls.
Nasopharyngoscopy during speech is usually conducted by
testing provides quantitative data that are easy to interpret for
diagnostic purposes. Some pressure–flow systems also offer
the ability to use the information for biofeedback purposes for
speech therapy. The disadvantages of pressure–flow testing
include its cost and increased cooperation required for use
with younger children.
Imaging
Imaging of the velopharynx is critical for making the most
appropriate treatment decision. It is important for the surgeon
to visualize the velopharyngeal mechanism in vivo during
speech in order to identify or confirm the etiology and extent
of the problem, as well as to determine which type of surgical
approach will best manage the speech problem. In addition,
imaging is helpful for clarifying the status of the adjacent
structures of the upper airway which could impact treatment
planning, such as the size of the tonsils and adenoid pad. It
is important that imaging be conducted with a trained speech
pathologist in order to ensure that an appropriate speech
sample is utilized, accuracy of speech attempts is interpreted,
and diagnostic decisions are accurate. Imaging is best com­
pleted when a patient has the ability to produce at least some
oral pressure consonants with accurate placement of the
articulators and not just compensatory errors, in order to view
the “best effort” of the velopharyngeal mechanism during
speech.
Static radiographs
A basic lateral cephalometric radiograph obtained at rest
(quiet breathing) and during sustained production of sounds,
such as /u/ or /s/, is one of the oldest approaches to evaluate
the velopharyngeal mechanism67 (Fig. 26.8). This type of
image may be helpful for confirming palatal length and velar
stretch, as well as tonsil and adenoid size; however, there is
Fig. 26.8  Static lateral cephalometric radiographs of the
velopharyngeal mechanism at rest compared with during
oral speech. PW, posterior pharyngeal wall; V, velum.
A
B
V
V
PW
PW

628
SECTION I
CHAPTER 26 • Velopharyngeal dysfunction
procedures for the management of VPD seek to reduce the
cross-sectional area of the velopharyngeal port and/or
improve the dynamic function of the velopharyngeal valve.
The procedures most commonly used for the management
of VPD include Furlow double-opposing Z-palatoplasty,
posterior pharyngeal flap, and sphincter pharyngoplasty.
Posterior pharyngeal wall augmentation has been used less
frequently.
Preoperative evaluation
In order to optimize surgical results while minimizing the
likelihood of complications, all patients considered candi­
dates for surgical management should undergo thorough
the surgeon, otolaryngologist, or a trained speech pathologist.
Regardless, a speech pathologist should be present during the
examination to model the correct speech stimuli for the patient
to imitate during the procedure and assist with the interpreta­
tion of the imaging results. Sufficient cooperation can be
anticipated in most 4–5-year-olds, and even some mature
3-year-olds, when they have been shown to be capable of
producing a sufficient speech sample. A topical anesthetic and
decongestant and scope lubricant are often utilized for
increased patient comfort and cooperation.
The scope should be inserted into the middle meatus of the
nasal cavity whenever possible, as the inferior meatus may
provide a view of the velopharyngeal port but is more prone
to artifact based on the viewing angle. The estimated size,
shape, and consistency of the velopharyngeal gap can be
viewed during speech, and the type of velopharyngeal closure
pattern can also be determined (Fig. 26.9). Tonsil and adenoid
size can be examined, as well as laryngeal structures, if
needed. The benefit of nasopharyngoscopy is the direct view
of the velopharyngeal mechanism from above the velopha­
ryngeal port during speech, in color. Nasopharyngoscopy is
also better for assessing small velopharyngeal gaps, asym­
metrical velopharyngeal function, and velopharyngeal inad­
equacy persisting post-pharyngeal flap, and is the most direct
assessment option for suspected occult submucous cleft
palate. Another benefit of nasopharyngoscopy is that it may
also be useful for slightly older children, adolescents, and
adults, who may benefit from biofeedback during speech
therapy (Video 26.3
).
For multiview videofluoroscopy and nasopharyngoscopy,
the speech sample should include words and phrases or
sentences, which are carefully selected by the speech patholo­
gist so that the patient’s best attempts at velopharyngeal
closure can be visualized during accurate articulation (and
may also be contrasted with the least amount of velopharyn­
geal closure during nasal sounds or compensatory errors).
Imaging exams should be audio- and video-recorded for later
review whenever possible. Standard procedures for acquir­
ing, rating, and interpreting videofluoroscopic and nasendo­
scopic images of the velopharynx during speech have
previously been reported.70
Two other imaging methods, computed tomographic scans
and magnetic resonance imaging (MRI), have been utilized
for the assessment of velopharyngeal closure for speech but
primarily for research purposes. Dynamic MRI is still in the
early stages of translation into the clinical setting for assess­
ment of the velopharyngeal mechanism, with preliminary
studies demonstrating the ability to capture the movement of
the velopharyngeal mechanism during the phonation of
vowels and consonants and limited speech tasks.71–73
Treatment/surgical techniques
The primary goal of surgical management is to produce a
competent velopharyngeal mechanism while avoiding the
complications of nasal airway obstruction, including hypo­
nasality, obligate mouth-breathing, snoring, and obstructive
sleep apnea. In all cases, surgical management should be
individualized, taking into consideration each patient’s velo­
pharyngeal anatomy and function, as well as any comorbid
conditions that may influence surgical outcome. All surgical
A
Fig. 26.9  Views of the velopharyngeal port during speech obtained by
nasopharyngoscopy. (A) Inadequate velopharyngeal closure during speech resulting
in a large central gap. (B) Complete closure of the velopharyngeal port during
speech.
B

629
Treatment/surgical techniques
for whom adenoidectomy or tonsillectomy is indicated as an
initial procedure. Previous research has suggested that when
a patient is observed to exhibit a short velum or decreased
palatal elevation, but at least an average degree of medial
movement of the lateral pharyngeal walls during speech, a
pharyngeal flap procedure may be most likely to result in a
better speech outcome. On the other hand, if a patient dem­
onstrates average palatal length and elevation with minimal
lateral pharyngeal wall contribution to velopharyngeal
closure, a sphincter pharyngoplasty may be the more appro­
priate procedure. Surgeon experience, skill, and preference, as
well as any potential concerns with airway obstruction in the
patient’s history, will also interact with imaging findings as
part of the treatment decision-making process.
Furlow double-opposing Z-palatoplasty
(Fig. 26.10)
Although originally described for the primary repair of palatal
clefts,74 the Furlow double-opposing Z-palatoplasty incorpo­
rates several features that make it an ideal procedure in
selected patients with VPD. Transposition of the posteriorly
based myomucosal flaps reorients the levator muscles from
the sagittal to the horizontal position, thereby reconstructing
the levator sling. The Z-plasty design provides for palatal
lengthening while avoiding velar shortening that may occur
preoperative evaluation. Individualization of surgical man­
agement is critical to optimizing surgical outcome. That is,
differential management based upon specific anatomic and
functional abnormalities allows for selection of the surgical
technique most likely to achieve velopharyngeal competence
in each patient.
The surgeon should elicit a thorough history, carefully
assessing each patient for prior surgery on the palate, velo­
pharynx, tonsils, and adenoids, as stated earlier in this chapter.
The presence of associated syndromes and comorbid condi­
tions should be noted, as should a prior history of upper
airway obstruction. Appropriate preoperative medical and
anesthetic consultation should be obtained. Patients with a
history of Pierre Robin sequence, loud snoring, or obstructive
sleep apnea should undergo a careful preoperative evaluation
of the airway, including polysomnography. In all such cases,
stabilization of the upper airway should precede surgical
management of VPD.
In addition to the perceptual speech evaluation, careful
physical examination should be performed in all patients who
may be candidates for surgical management of the velophar­
ynx. Patients should be assessed for stigmata of syndromic
diagnoses (e.g., 22q11.2 deletion syndrome) that may influ­
ence their management and outcome or those (i.e., Pierre
Robin sequence) that may increase their risk for postoperative
upper airway obstruction. Intraoral examination yields
important information regarding oropharyngeal anatomy.
Patients noted to have enlarged tonsils and/or adenoids
should undergo tonsillectomy and adenoidectomy prior to
posterior pharyngeal flap surgery in order to reduce their risk
of postoperative upper airway obstruction. Examination of
patients who have undergone prior tonsillectomy, however,
may reveal scarring of the tonsillar pillars that may preclude
sphincter pharyngoplasty. In patients who have undergone
prior palatoplasty, the palate should be inspected carefully for
velar dehiscence and oronasal fistulae. Patients with velar
dehiscence should undergo re-repair of the palate prior to
reassessment of velopharyngeal function and, if necessary,
pharyngoplasty. Likewise, fistulae large enough to be of
aerodynamic significance should be repaired and velopharyn­
geal function reassessed. Oropharyngeal examination may
also provide useful information in those patients who present
with persistent or recurrent velopharyngeal dysfunction after
pharyngoplasty. For example, a pharyngeal flap appropriately
positioned at the level of velar closure will be difficult to
visualize on oral examination. The presence of a pharyngeal
flap easily visible on the posterior pharyngeal wall below the
palate should raise suspicion that the flap may be tethering
the velum and impairing velopharyngeal closure.
In addition, preoperative imaging of the velopharynx is
also essential to surgical planning. The diagnosis of VPD
should be confirmed by nasendoscopy and/or multiview
videofluoroscopy, as earlier described. The site, pattern, and
symmetry of velopharyngeal closure should be noted, as
should gap size, shape, and location. Imaging further allows
the surgeon to assess orientation of the levator fibers, velar
anatomy and function, and adenoid and tonsillar size and
morphology. Markedly enlarged tonsils or irregularly shaped
adenoids may impair velopharyngeal closure in some patients.
Preoperative imaging allows the operator to assess the contri­
bution of adenoid or tonsillar abnormalities to VPD, thereby
avoiding misguided attempts at pharyngoplasty in patients
Fig. 26.10  Furlow palatoplasty.
Oral mucosal flap
Oral
musculomucosal flap
Nasal flaps rotated
Oral flaps rotated
Nasal mucosal flap
Nasal
musculomucosal flap

630
SECTION I
CHAPTER 26 • Velopharyngeal dysfunction
be determined to a large extent by the degree of velar length­
ening achieved,77 an effect that is related primarily to the
angles of the Z-plasty design. As noted above, however, the
Z-plasty angles are determined not by any specific geometric
design, but rather by the underlying palatal anatomy. As velar
length decreases, the Z-plasty angles increase, thereby reduc­
ing the amount of velar lengthening effectively achieved fol­
lowing transposition of the flaps. Velopharyngeal competence
after Furlow Z-palatoplasty, therefore, can be anticipated in
the patient with a small velopharyngeal gap and good velar
length but may be more difficult to achieve in patients with a
large gap and short velum.
Complications after Furlow Z-palatoplasty include bleed­
ing, oronasal fistula, and nasal airway obstruction. Fistula
formation can be minimized by ensuring that the repair is
completed with minimal tension. When necessary, lateral
relaxing incisions should be employed in order to achieve a
tension-free closure. Although mild obstructive apnea has
been documented in patients following Furlow Z-palatoplasty,
such has been noted to resolve in nearly all patients within 3
months of surgery.80 When compared to patients who have
undergone posterior pharyngeal flap surgery for the manage­
ment of VPD, patients treated by Furlow repair demonstrate
significantly lower incidence and severity of upper airway
obstruction 6 months or more postoperatively.81
Posterior pharyngeal flap (Fig. 26.11)
The creation of midline flaps from the posterior pharyngeal
wall represents the oldest surgical technique for the manage­
ment of VPD. In 1865, Passavant published the first report
describing the surgical management of VPD by adhesion of
the soft palate to the posterior pharyngeal wall.82 Schoenborn
described the use of an inferiorly based pharyngeal flap in
1875 and of a superiorly based flap a decade later.83,84 The
superiorly based pharyngeal flap was described in the US by
Padgett in 1930,85 and by the middle of the 20th century, the
procedure was widely employed as the standard surgical
treatment for VPD.
The pharyngeal flap functions primarily as a central obtu­
rator of the velopharyngeal port. Closure of the lateral side
ports during speech is dependent upon the medial movement
of the lateral pharyngeal walls. Hence, this technique is opti­
mally suited for patients with VPD that is characterized by
the presence of a central gap and that is associated with good
lateral pharyngeal wall motion. To be effective, the pharyngeal
flap should be carefully placed at the level of attempted
velopharyngeal closure as determined by preoperative
imaging. Flaps that have been created too low or that have
migrated inferiorly due to postoperative cicatricial changes
may tether the velum and interfere with velopharyngeal
closure. Patients with an asymmetrical velopharyngeal closure
pattern should have flap design altered accordingly.
The width of the pharyngeal flap should be tailored to the
functional and anatomic needs of each patient, again as
determined by preoperative imaging. That is, patients with
large gaps and those with relatively poor lateral pharyngeal
wall motion may require a wider flap design in order to
achieve velopharyngeal competence. Flap width is dependent
not only upon the breadth of the flap itself, but also on the
breadth of its inset on the posterior velum. Inset of the flap
may be accomplished either by dividing the soft palate in the
after straight-line closure. Thus, the Furlow Z-palatoplasty is
well suited for the management of VPD in patients with an
unrepaired submucosal cleft palate and in those who have
undergone cleft palate repair without levator reconstruction.
The technique is inappropriate, however, for patients in whom
the levator muscle fibers are not sagittally oriented, as trans­
position of the myomucosal flaps would disrupt the anatomi­
cally normal or the previously reconstructed levator sling.
The design of the Furlow palatoplasty incorporates mirror-
image Z-plasties on the oral and nasal aspects of the velum,
such that the posteriorly based flaps contain both mucosa and
the attached fibers of the levator veli palatini. In contrast, the
anteriorly based flaps contain mucosa and submucosa alone.
The Z-plasty design is determined by palatal anatomy, the
incisions extending from the hamulus to the junction of the
hard and soft palate at the cleft margin on one side and from
the base of the uvula to the hamulus on the other. The poste­
riorly based flap is elevated in the nasal submucosal plane,
thereby creating an oral myomucosal flap. Care must be taken
to divide the levator fibers completely from the posterior edge
of the hard palate in order to allow for complete posterior
rotation of the flap and for horizontal repositioning of the
levator. The contralateral flap is elevated in the oral submu­
cosal plane, creating an anteriorly based mucosal flap.
The anteriorly based nasal mucosal flap is then developed
by incising the nasal mucosa from the base of the uvula to a
point just medial to the orifice of the eustachian tube. On the
opposite side, the posteriorly based nasal myomucosal flap is
incised along the posterior edge of the hard palate, again
completely dividing the attachment of the levator to the bone.
The nasal flaps are then transposed and sutured in place.
Transposition of the oral flaps reconstructs the levator sling
and completes the repair.
Several reports confirm the efficacy of the Furlow
Z-palatoplasty in the management of VPD in selected patients
with unrepaired submucosal cleft palate and with repaired
overt clefts. In a series reported by Hudson et al.,75 85% of
patients with VPD after primary palatoplasty demonstrated
normal resonance after conversion to a Furlow Z-palatoplasty.
Chen et al.76 reported that the majority of patients with a
velopharyngeal gap of less than 5 mm achieve velopharyngeal
competence after Furlow repair, whereas the repair is far less
successful when the gap size exceeds 10 mm. D’Antonio
et al.77 reported normal resonance after conversion to a Furlow
Z-palatoplasty in six of eight patients with persistent VPD
after cleft palate repair. All patients in their series demon­
strated a central V-shaped notch in the posterior soft palate,
good velar motion, and a “small” velopharyngeal gap.
High success rates for the management of VPD by Furlow
palatoplasty have been reported in patients with submucosal
cleft palate. As for previously repaired overt clefts, the likeli­
hood of success in patients with submucosal clefts may be
related primarily to gap size. Seagle et al.78 reported that 83%
of patients with VPD and submucosal cleft palate achieved
velopharyngeal competence after Furlow repair, noting that
successful outcomes are far more frequent in those patients in
whom gap size measured less than 8 mm. Likewise, Chen
et al.79 reported that 97% of patients with submucosal clefts
achieved velopharyngeal competence when gap size did not
exceed 5 mm. Studies have confirmed that the Furlow
Z-palatoplasty increases palatal length in most patients and
have suggested that speech outcome after Furlow repair may

631
Treatment/surgical techniques
A
Suture in
uvula
Transverse
incision in
soft palate
Posterior
pharyngeal flap
Posterior
pharyngeal flap
Fig. 26.11  (A, B) Posterior pharyngeal flap.
B
midline or by dissecting a submucosal pocket through a
posterior transverse (“fishmouth”) incision. The latter allows
for somewhat greater flexibility in the design of flap width
and lateral port dimensions.86 Cicatricial change, or “tubing”,
of pharyngeal flaps may result in significant flap narrowing
and, consequently, in deterioration of velopharyngeal func­
tion.87 Pharyngeal flap narrowing can be minimized, however,
by lining the raw surface of the flap with mucosal flaps of
velar mucosa or by designing short, broad flaps. In all cases,
the need to create wide flaps must be judiciously balanced
against an associated increase in the risk of postoperative
obstructive sleep apnea.
Careful surgical planning and individualization of flap
design are essential to surgical success. Accurate interpreta­
tion of reported series is made difficult, however, by the use
of different surgical techniques, heterogeneous patient popu­
lations, and non-standardized, often unreliable measures of
surgical outcome. Argamaso86 reported that hypernasality
was eliminated after pharyngeal flap surgery in 96% of 226
patients. Similarly, normal or borderline sufficient velopha­
ryngeal function was achieved in 97% of 104 non-syndromic
patients described by Sullivan et al.88 Cable et al.89 reported
stability in resonance scores over 14 years of follow-up after
pharyngeal flap surgery, suggesting that surgical outcome
after pharyngeal flap surgery is durable.
Several authors have expressed concerns regarding the
negative impact of pharyngeal flap attachment on midfacial
growth. Although several studies have yielded conflicting
data, the majority of evidence from large series has failed to
demonstrate that attachment of pharyngeal flaps has any
significant long-term influence on maxillary development.90
Complications of pharyngeal flap surgery include bleed­
ing, dehiscence, and nasal airway obstruction, including
obstructive sleep apnea.91 Rarely, deaths have been reported
following the procedure, primarily related to airway compro­
mise.92 Fraulin et al.93 reported that predictive factors for
complications include the operator, associated medical condi­
tions, concurrent surgical procedures, and an open flap donor
site. Of all complications, upper airway compromise occurs
most commonly. Nearly all patients experience transient nasal
airway obstruction and perhaps mild obstructive apnea in the
early postoperative period. The vast majority of patients
demonstrate resolution of clinical and polysomnographic
evidence of nocturnal upper airway obstruction within several
months of surgery, as edema subsides. Wells et al.94 docu­
mented clinical evidence of nocturnal upper airway obstruc­
tion in 12 of 111 patients following pharyngeal flap surgery,
three of whom required takedown of the flap. Nine of the 12
patients underwent polysomnographic evaluation, and this
revealed obstructive apnea in only one patient. Thus, clinical
evidence of postoperative nocturnal airway obstruction may
not correlate with the presence of apnea. Syndromic patients
and those with a history of Pierre Robin sequence may be at
greater risk for airway compromise after pharyngeal flap
surgery due to the presence of associated functional or ana­
tomic airway abnormalities.94,95 Similarly, patients with tonsil­
lar enlargement may be at greater risk for postoperative upper
airway obstruction and should therefore undergo tonsillec­
tomy at or prior to the time of pharyngeal flap surgery.96
Postoperative monitoring of upper airway status, including
continuous pulse oximetry, should be considered the standard
of care for all patients following posterior pharyngeal flap

632
SECTION I
CHAPTER 26 • Velopharyngeal dysfunction
modified the procedure, eliminating the posterior pharyngeal
flap, instead insetting the palatopharyngeal flaps into a trans­
verse incision located higher on the posterior pharyngeal
wall.
The surgical technique used most widely today represents
a modification of the Hynes pharyngoplasty. Vertical incisions
are made anterior to both posterior tonsillar pillars, and the
palatopharyngeus muscles are exposed. The longitudinally
oriented muscle fibers are carefully dissected from the pos­
terolateral pharyngeal wall, so as to include the entire muscle
in each of the flaps. Vertical incisions are then made posterior
to the pillars, creating flaps that measure approximately 1 cm
in width. On each side, the parallel incisions are joined by a
transverse incision at the lowest aspect of the pillar, and the
flaps are elevated. The flaps are then rotated medially and
inset into a transverse incision that connects the most superior
aspect of the medial palatopharyngeal flap incisions.
Riski et al.102 have stressed the importance of preoperative
patient selection and of proper surgical planning for opti­
mizing surgical outcome. Flap inset should be high on the
posterior pharyngeal wall at the site of attempted velopharyn­
geal closure, as determined by preoperative imaging. Since
surgery. For patients at high risk for postoperative airway
obstruction, consideration should be given to the use of a
nasopharyngeal airway, placed intraoperatively through one
of the lateral ports, and to admission to the intensive care unit
for overnight monitoring. Patients may be discharged from
the hospital once they demonstrate adequate airway stability
and oral fluid intake.
Sphincter pharyngoplasty (Fig. 26.12)
In 1950, Hynes97 first described the technique of pharyngo­
plasty by transposition of musculomucosal flaps containing
the salpingopharyngeus muscles. He later modified the tech­
nique to include the palatopharyngeus muscles, noting that
success of the technique could be attributed to narrowing of
the velopharyngeal port and to augmentation of the posterior
pharyngeal wall with bulky, “often contractile” flaps.98,99
Orticochea100 stressed the concept of creating a true “dynamic
sphincter” in order to achieve velopharyngeal competence.
He dissected bilateral palatopharyngeal myomucosal flaps
and inset them into an inferiorly based mucosal flap on the
posterior pharyngeal wall. Jackson and Silverton101 later
Fig. 26.12  Sphincter pharyngoplasty.
Palatoglossal arch
Soft palate
Palatopharyngeal arch
Palatopharyngeus flaps
Flaps interdigitated
Posterior adenoid pad
Posterior pharynx

633
Treatment/surgical techniques
initial description, he sutured the palatopharyngeal muscles
together in the midline. In 1879, he described the use of a
pedicled flap of posterior pharyngeal mucosa, rolled upon
itself and inset across the posterior pharyngeal wall.112 After
disappointing results, the technique was abandoned. Hynes
reported the use of myomucosal flaps containing the salpin­
gopharyngeus (and later the palatopharyngeus) muscles
nearly a century later.97,98 In 1997, Witt et al. reported on the
use of a rolled, superiorly based pharyngeal flap for posterior
pharyngeal wall augmentation, noting no significant improve­
ment in the speech of 14 patients treated.113 In contrast,
however, Gray et al.114 reported good results using folded
flaps in young patients with good velar motion.
In 1912, Hollweg and Perthes115 described the use of autolo­
gous cartilage grafts inserted through a cervical incision. This
procedure was later modified by others utilizing a transoral
approach. Although many authors have reported some
success with cartilage grafts placed into the posterior pharynx,
the results and durability of these procedures have been vari­
able. Denny et al.116 reported elimination of hypernasality in
25% of 20 patients treated by retropharyngeal bone or cartilage
grafts with lesser degrees of improvement seen in another
65%. Follow-up studies of the same cohort of patients,
however, failed to document durability of the results achieved.
The body of evidence suggests that some degree of graft
migration and resorption after pharyngeal augmentation with
cartilage appears inevitable. Recent reports have documented
improvement in velopharyngeal function following injection
of autologous fat into the posterior pharynx in selected
patients,117 although the application and effectiveness of this
technique await greater experience and long-term follow-up.
The earliest attempts to augment the posterior pharyngeal
wall by injection of exogenous material may have been those
of Gersuny, who reported the use of petroleum jelly in 1900.118
Although the technique achieved some success in improving
patients’ speech, the technique was associated with several
serious complications, including blindness and death. In 1904,
Eckstein119 described injection of paraffin without untoward
complications. Blocksma120 reported augmentation pharyngo­
plasty using implantable blocks and injectable fluid Silastic.
Although he noted improvement of speech in many patients,
a high incidence of implant infection and extrusion led him
to recommend the use of autologous implants as the preferred
method for pharyngeal augmentation. Lewy published a
single case report on the use of Teflon injection for the man­
agement of VPD in 1965.121 Bluestone et al.122 later reported
success with Teflon injections in several patents, noting no
instances of infection, extrusion, or foreign-body reaction.
Smith and McCabe123 documented complete elimination of
hypernasality in 60% of 80 patients who underwent augmen­
tation pharyngoplasty by Teflon injection. Furlow et al.124
reported successful treatment of VPD by Teflon injection in
74% of 35 patients. Nevertheless, the risk of potentially serious
complications has led the Food and Drug Administration to
withdraw approval of its use for augmentation pharyngo­
plasty. Other exogenous materials that have been used to
augment the posterior pharyngeal wall in patients with VPD
include Proplast and calcium hydroxyapatite.
The majority of evidence suggests that posterior pharyn­
geal wall augmentation may be successfully used for the
management of carefully selected patients with good velar
motion and small-gap VPD. In order to be effective,
a dynamic sphincter may be achieved in only a subset of
patients,103,104 anatomic factors, such as reduced port size and
augmentation of the posterior pharyngeal wall, may be essen­
tial to achieving postoperative velopharyngeal competence.
In a retrospective review of speech outcome in 48 patients
who underwent sphincter pharyngoplasty, Shewmake et al.105
reported that 85.4% achieved normal resonance. Riski et al.106
reported that, of 139 patients who underwent sphincter pha­
ryngoplasty, 78% demonstrated resolution of hypernasality
and normal pressure–flow measurements. Most surgical fail­
ures were the result of the pharyngoplasty being placed too
low on the posterior pharyngeal wall. Similarly, Witt et al.107
noted that 16% of patients required pharyngoplasty revision,
although the primary cause of failure was noted to be complete
or partial flap dehiscence. In a series of 250 patients, Losken
et al.108 noted a revision rate of 12.8%, noting that persistent
VPD after pharyngoplasty was more common in patients with
22q11.2 deletion syndrome. Failure was seen more often in
patients with greater nasalance scores and velopharyngeal
valve area on preoperative instrumental evaluation.
Several studies have compared speech outcome after pos­
terior pharyngeal flap surgery and sphincter pharyngoplasty.
Ysunza et al.109 reported on 50 patients with cleft palate and
residual VPD who were randomized to undergo one or the
other procedure, finding no significant difference in the fre­
quency of persistent VPD (12% and 16%, respectively) between
the two groups. Similarly, Abyholm et al.110 reported no
significant difference in speech outcome 1 year following
posterior pharyngeal flap or sphincter pharyngoplasty in a
randomized multicenter trial.
Complications of sphincter pharyngoplasty are similar to
those of posterior pharyngeal flap surgery and include bleed­
ing, flap dehiscence, and upper airway obstruction. In a
multicenter, prospective randomized trial of 97 patients,
Abyholm et al.110 noted that polysomnographic evidence of
obstructive apnea 1 year after surgical management of VPD
was rare, with no significant difference between patients
undergoing posterior pharyngeal flap surgery and those
undergoing sphincter pharyngoplasty. Saint Raymond et al.111
examined polysomnograms of 17 patients before and after
sphincter pharyngoplasty and found that the procedure
induced no significant impairment in either the apnea–
hypopnea index or the nocturnal oxygen saturation. Despite
this, however, there was a notable reduction in slow-wave
sleep and an increase in cortical microarousals postopera­
tively, suggesting that the reduction in nasopharyngeal airway
diameter may increase airway resistance sufficiently enough
to cause fragmentation of sleep architecture even in the
absence of detectable sleep apnea.
Posterior pharyngeal wall augmentation
Augmentation pharyngoplasty, using both autologous tissues
and alloplastic materials, has long been used by surgeons
to reduce the size of the velopharyngeal orifice in patients
with VPD. Long-term results have been variable and have
depended upon both patient selection and the choice of
augmentation material. Overall, the most durable results have
been achieved in patients with good velar motion and rela­
tively small velopharyngeal gap size.
In 1862, Passavant became the first to describe the use of
local tissues to augment the posterior pharyngeal wall. In his

634
SECTION I
CHAPTER 26 • Velopharyngeal dysfunction
non-surgical options to be considered. Prosthetic or behavioral
speech treatment may be appropriate for a select set of patients
for whom surgery is not possible, not desired, or if the prog­
nosis for surgical outcome is guarded.
Prosthetic treatment
Speech prostheses may be an appropriate treatment option for
patients who have an unclear, guarded, or poor prognosis for
improvement with surgery, such as (1) when the diagnosis of
VPD is unclear based on perceptual speech and/or imaging
findings; (2) when the comorbid speech problems make it
difficult to determine if surgical intervention will result in
meaningful improvement in speech; and (3) when the patient
has a known neuromuscular or degenerative condition that
has been shown to result in suboptimal surgical outcomes. In
addition, the patient may have known medical contraindica­
tions to having surgery or have cultural, religious, or other
ethical conflicts with surgery. To be a good candidate for
prosthetic management, the patient and family must demon­
strate adequate compliance and dedication to completing the
prosthetic treatment plan, which may require several visits,
and be an appropriate dental candidate for fabrication of a
speech prosthesis (i.e., demonstrate good dental hygiene).
The palatal lift and the speech bulb are the most commonly
used speech prostheses125 (Fig. 26.13). A pediatric or general
dentist, orthodontist, or prosthodontist may fabricate the
device, usually with the input from the speech pathologist. A
palatal lift is basically a standard orthodontic retainer with an
extension posteriorly to “lift” up the soft palate. It is an
appropriate treatment option for patients with a soft palate of
sufficient length but lacks adequate movement during speech
and/or swallowing, such as in cases of velopharyngeal
incompetence. A speech bulb is more appropriate for patients
with velopharyngeal insufficiency in which the palate is too
augmentation should be performed precisely at the level of
attempted velar contact on the posterior pharyngeal wall as
determined by preoperative imaging. To date, no single allo­
plastic material has been found to be uniformly safe, effective,
and reliable, and single type of autologous graft has demon­
strated consistent long-term stability. Therefore, augmentation
pharyngoplasty should be considered only as a secondary
option in carefully selected patients with VPD.
Surgical management in the 22q11.2
deletion syndrome
Velopharyngeal dysfunction in persons with 22q11.2 deletion
syndrome (22q11DS) is multifactorial and typically results
from a combination of structural and functional causes such
as submucous cleft palate, palatopharyngeal disproportion,
obtuse cranial base, and/or hypotonia. Optimization of surgi­
cal outcomes in this specific patient population requires preci­
sion in diagnosis, surgical management, and multidisciplinary
team care. Children with 22q11DS tend to have more severe
hypernasal speech than that of other cleft and VPD popula­
tions. In addition, children with 22q11DS traditionally undergo
VPD surgery at slightly later ages than their peers with cleft
palate or non-syndromic VPD due to a variety of factors
including but not limited to (1) significant ongoing medical
issues (e.g., cardiac, airway); (2) delayed expressive language
development; and (3) severe articulation disorders, which
may interfere with the ability to obtain VP imaging with suf­
ficiently accurate articulation and adequate cooperation for
the study.
Non-surgical treatment options
While surgery is the preferred treatment option for most cases
of VPD, there may be patient-specific factors which cause
Fig. 26.13  (A) Schematic of a palatal lift (intraoral and lateral view). Note how the lift extends along the oral surface of the velum to lift it to the level of the palatal plane.
The length of the palate is sufficient, but in cases of velopharyngeal incompetency, the function of the velopharyngeal mechanism is impaired. The palatal lift places the
velum into position to provide adequate closure for speech and swallowing. (B) Speech bulb appliance assisting with velopharyngeal closure for speech. Note that the bulb
fits up into the velopharyngeal orifice to obturate the velopharyngeal port, as the length of the velum is insufficient to achieve closure independently.
A
B

635
Treatment/surgical techniques
short to contact the posterior pharyngeal wall. The speech
bulb is similar to the palatal lift, with an addition of a “bulb”
of acrylic material to fill in the remaining velopharyngeal gap
during speech. Other types of obturators, without any poste­
rior extension, may also be helpful for temporary or long-term
obturation of palatal fistulae.
Behavioral speech therapy approaches
In selected patients with borderline or inconsistent VPD and/
or velopharyngeal mislearning, a trial period of behavioral
speech therapy may be helpful prior to proceeding with
surgical management.126,127 Speech therapy is always the
most appropriate treatment for articulation errors, as surgery
cannot change lip and tongue placement for the production
of speech sounds. Speech therapy is also the most appropriate
treatment for phoneme-specific nasal emission or phoneme-
specific nasalization of sounds (velopharyngeal mislearning)
as surgery cannot correct these articulation disorders.128 There
are also some behavioral speech therapy methods which may
be effective for reducing mild or inconsistent hypernasality
or nasal air emission, as well, especially when biofeedback
is provided. If one considers the velum to be an articula­
tor, just as the lips and tongue are, the idea of changing the
“behavior” of the velum may be a reasonable goal. The patient
must then be provided with the right tools and feedback,
assuming that the underlying anatomy of the velopharyngeal
mechanism otherwise appears intact. The ideal patient for
such a treatment trial would have many of the following
characteristics:
■ Age 6–8 years or older
■ Intact cognitive skills
■ Intact motor skills
■ Adequate attention span and maturity
■ Normal hearing and vision
■ Good self-monitoring or speech self-correction skills
■ At least inconsistent velopharyngeal closure for speech
■ At least some accurate articulation skills already in the
speech repertoire
■ Can demonstrates measurable change within the first few
sessions of therapy.
Biofeedback is often a cornerstone of behavioral speech
therapy to improve velopharyngeal closure for speech. Bio­
feedback may be provided through enhanced auditory, visual,
or tactile–kinesthetic cues. Patients may benefit from technol­
ogy that provides information during online speech produc­
tion about oral pressure, nasal airflow, and nasalance.
Nasopharyngoscopy is also a useful biofeedback tool as it
allows for direct visualization of the velopharyngeal port and
its movement during speech. It can be used for treating
learned nasal emission and even glottal stop substitutions
in selected patients.129–131 Lastly, continuous positive airway
pressure (CPAP) has been proposed as a treatment modality
to improve velopharyngeal closure by “working” the muscles
against artificially increased nasal resistance (nasal pressure)
during speech for longer durations of time. The first round of
CPAP clinical trials has yielded mixed speech outcome
results.132 In contrast, it should be stated that, although the
velopharyngeal mechanism is a dynamic muscular system,
“oral–motor exercises” of the lips, tongue, and palate have not
been shown to be effective for improving long-term speech
outcomes. Multiple studies have shown that palatal massage,
electrical stimulation, swallowing exercises, blowing exer­
cises, and blowing against resistance (i.e., horn/whistle pro­
grams), are not effective for improving speech.133–135 Overall,
significantly more research is needed to identify the most
effective behavioral speech therapy approaches for improving
velopharyngeal closure for speech.
12. Peterson-Falzone SJ, Hardin-Jones MA, Karnell MP. Anatomy and
physiology of the velopharyngeal system. In: Peterson-Falzone SJ,
Hardin-Jones MA, Karnell MP, eds. Cleft Palate Speech. 3rd ed. St.
Louis: Mosby; 2001:69–86.
17. Croft CB, Shprintzen RJ, Rakoff SJ. Patterns of velopharyngeal
valving in normal and cleft palate subjects: a multi-view
videofluoroscopic and nasendoscopic study. Laryngoscope.
1981;91:265–271. The authors studied 80 control subjects and 500
patients with velopharyngeal dysfunction using direct
nasopharyngoscopy and multi-view videofluoroscopy. The incidence of
the different patterns of velopharyngeal closure was found to be similar
in frequency in both groups. The importance of these patterns is
discussed in relation to the surgical management of patients with
velopharyngeal dysfunction.
47. Kuehn DP, Moller KT. Speech and language issues in the cleft
palate population: the state of the art. Cleft Palate Craniofac J.
2000;37:348–383. This summary paper covers all aspects of speech
language assessment and treatment options relevant to cleft palate and
velopharyngeal dysfunction. A review of the anatomy and physiology and
instrumental assessment of the velopharyngeal mechanism is also
provided.
48. Peterson-Falzone SJ, Hardin-Jones MA, Karnell MP. Diagnosing
and managing communication disorders in cleft palate. In: Cleft
Palate Speech. 4th ed. St. Louis: Mosby; 2010:221–247.
50. Henningsson G, Kuehn DP, Sell D, et al. Universal parameters for
reporting speech outcomes in individuals with cleft palate. Cleft
Palate Craniofac J. 2008;45:1–17. The Universal Parameters System
(UPS) for rating speech in patients with cleft palate and velopharyngeal
dysfunction is discussed. Examples of standard speech stimuli, a rating
form, and various rating scales are included.
61. Warren DW, DuBois AB. A pressure-flow technique for measuring
orifice area during continuous speech. Cleft Palate J. 1964;1:
52–71.
70. Golding-Kushner KJ. Standardization for the reporting of
nasopharyngoscopy and multiview videofluoroscopy: a report
from an international working group. Cleft Palate Craniofac J.
1990;27:337–348. This manuscript describes a protocol for rating
velopharyngeal structures and movement during speech using multiview
videofluoroscopy and flexible nasopharyngoscopy. These standards were
published as a result of an International Working Group meeting of
experts in the field of clefting/velopharyngeal dysfunction and speech
pathology.
76. Chen PK, Wu JT, Chen YR, et al. Correction of secondary
velopharyngeal insufficiency in cleft palate patients with the
Furlow palatoplasty. Plast Reconstr Surg. 1994;94:933–941. The
results of this study demonstrate that a Furlow palatoplasty can
satisfactorily correct velopharyngeal dysfunction in carefully selected
patients. The most important factor is the size of the velopharyngeal gap.
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636
SECTION I
CHAPTER 26 • Velopharyngeal dysfunction
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The majority of patients with a successful surgical outcome had a
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98. Hynes W. The results of pharyngoplasty by muscle transplantation
in failed “cleft palate” cases, with special reference to the influence
of the pharynx on voice production. Ann R Coll Surg Engl.
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77. D’Antonio LL, Eichenberg BJ, Zimmerman GJ, et al. Radiographic
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function following Furlow Z-plasty. Plast Reconstr Surg. 2000;106:
539–549.
78. Seagle MB, Patti CS, Williams WN, et al. Submucous cleft palate: a
10-year series. Ann Plast Surg. 1999;42:142–148.
79. Chen PK, Wu JT, Hung KF, et al. Surgical correction of submucous
cleft palate with Furlow palatoplasty. Plast Reconstr Surg.
1996;97:1136–1146.
80. Liao YF, Yun C, Huang CS, et al. Longitudinal follow up of
obstructive sleep apnea following Furlow palatoplasty in children
with cleft palate: a preliminary report. Cleft Palate Craniofac J.
2003;40:269–273.
81. Liao YF, Noordhoff MS, Huang CS, et al. Comparison of
obstructive sleep apnea syndrome in children with cleft palate
following Furlow palatoplasty or pharyngeal flap for
velopharyngeal insufficiency. Cleft Palate Craniofac J. 2004;41:
152–156.
82. Passavant G. Ueber die Beseitigung der naeselnen Sprache bei
angeborenen Spalten des harten und weichen Gaumens
(Gaumensegel, Schlundnaht und Ruecklagerund des
Gaumensegels). Arch Klin Chir. 1865;6:333–349.
83. Schoenborn K. Ueber eine neue Methode der Staphylorrhaphie.
Verth Dtsch Ges Chir. 1875;4:235–239.
84. Schoenborn K. Vorstellung eines falle von Staphyloplastik. Verh
Dtsch Ges Chir. 1886;15:57–68.
85. Padgett EC. Cleft palate repair after unsuccessful operations, with
special reference to cases with extensive loss of palatal tissue. Arch
Surg. 1930;20:453.
86. Argamaso RV. Pharyngeal flap surgery for velopharyngeal
insufficiency. In: Jurkiewicz MJ, Culbertson JH, eds. Operative
Techniques in Plastic and Reconstructive Surgery. Philadelphia: WB
Saunders; 1995:233–238.
87. Vandevoort MJ, Mercer NS, Albery EH. Superiorly based flap
pharyngoplasty: the degree of postoperative “tubing” and its effect
on speech. Br J Plast Surg. 2001;54:192–196.
88. Sullivan SR, Marinan EM, Mulliken JB. Pharyngeal flap outcomes
in nonsyndromic children with repaired cleft palate and
velopharyngeal insufficiency. Plast Reconstr Surg. 2010;125:290–298.
89. Cable BB, Canady JW, Karnell MP, et al. Pharyngeal flap surgery:
long-term outcomes at the University of Iowa. Plast Reconstr Surg.
2004;113:475–478.

636.e3
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90. Semb G, Shaw W. The influence of pharyngeal flap on facial
growth. In: Bardach J, Morris HL, eds. Multidisciplinary
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91. Hofer SO, Dhar BK, Robinson PH, et al. A 10-year review of
perioperative complications in pharyngeal flap surgery. Plast
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92. Valnicek SM, Zucker RM, Halpern LM, et al. Perioperative
complications of superior pharyngeal flap surgery in children.
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93. Fraulin FO, Valnicek SM, Zuker RM. Decreasing the perioperative
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operation. Plast Reconstr Surg. 1998;102:10–18.
94. Wells MD, Vu TA, Luce EA. Incidence and sequelae of nocturnal
respiratory obstruction following posterior pharyngeal flap
operation. Ann Plast Surg. 1999;43:252–257.
95. Abramson DL, Marrinan EM, Mulliken JB. Robin sequence:
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Craniofac J. 1997;34:256–260.
96. Ysunza A, Garcia-Velasco M, Garcia-Garcia M, et al. Obstructive
sleep apnea secondary to surgery for velopharyngeal insufficiency.
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97. Hynes W. Pharyngoplasty by muscle transplantation. Br J Plast
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98. Hynes W. The results of pharyngoplasty by muscle transplantation
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99. Hynes W. Observations on pharyngoplasty. Br J Plast Surg.
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100. Orticochea M. Construction of a dynamic muscle sphincter in cleft
palates. Plast Reconstr Surg. 1968;41:323–327.
101. Jackson IT, Silverton JS. The sphincter pharyngoplasty as a
secondary procedure in cleft palates. Plast Reconstr Surg.
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102. Riski JE, Serafin D, Riefkohl R, et al. A rationale for modifying the
site of insertion of the Orticochea pharyngoplasty. Plast Reconstr
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insetting the sphincter pharyngoplasty at the site of attempted
velopharyngeal closure. With this modification, successful outcomes were
achieved in 93% of patients.
103. Kawamoto HK. Pharyngoplasty revisited and revised. In:
Jurkiewicz MJ, Culbertson JH, eds. Operative Techniques in Plastic
and Reconstructive Surgery. Philadelphia: WB Saunders;
1995:239–244.
104. Witt PD, Marsh JL, Arlis H, et al. Quantification of dynamic
velopharyngeal port excursion following sphincter
pharyngoplasty. Plast Reconstr Surg. 1998;101:1205–1211.
105. Shewmake K, Elias D, Fromwiller S, et al. Modification of the
Orticochea pharyngoplasty for the correction of velopharyngeal
insufficiency: seven-year experience. Portland, OR: American Cleft
Palate Craniofacial Association; 1992.
106. Riski JE, Ruff GL, Georgiade GS, et al. Evaluation of the sphincter
pharyngoplasty. Cleft Palate Craniofac J. 1992;29:254–261.
107. Witt PD, Myckatyn T, Marsh JL. Salvaging the failed
pharyngoplasty: intervention outcome. Cleft Palate Craniofac J.
1998;35:447–453.
108. Losken A, Williams JK, Burstein FD, et al. An outcome evaluation
of sphincter pharyngoplasty for the management of
velopharyngeal insufficiency. Plast Reconstr Surg. 2003;112:
1755–1761.
109. Ysunza A, Pamplona C, Ramirez E, et al. Velopharyngeal surgery:
a prospective randomized study of pharyngeal flaps and sphincter
pharyngoplasties. Plast Reconstr Surg. 2002;110:1401–1407.
110. Abyholm F, D’Antonio L, Davidson Ward SL, et al. Pharyngeal
flap and sphincteroplasty for velopharyngeal insufficiency have
equal outcome at 1 year postoperatively: results of a randomized
trial. Cleft Palate Craniofac J. 2005;42:501–511.
111. Saint Raymond C, Bettega G, Deschaux C, et al. Sphincter
pharyngoplasty as a treatment for velopharyngeal incompetence
in young people: a prospective evaluation of effects on sleep
structure and sleep respiratory disturbances. Chest.
2004;125:864–871.
112. Passavant G. Concerning the improvement in speech after
operation on the palate. Arch Klin Chir. 1879;23:771–780.
113. Witt PD, O’Daniel TG, Marsh JL, et al. Surgical management of
velopharyngeal dysfunction: outcome analysis of autogenous
posterior pharyngeal wall augmentation. Plast Reconstr Surg.
1997;99:1287–1296.
114. Gray SD, Pinborough-Zimmerman J, Catten M. Posterior wall
augmentation for treatment of velopharyngeal insufficiency.
Otolaryngol Head Neck Surg. 1999;121:107–112.
115. Hollweg E, Perthes G. Treatment of cleft palates. Tübingen: Franz
Pietzcher. 1912.
116. Denny AD, Marks SM, Oliff-Carneol S. Correction of
velopharyngeal insufficiency by pharyngeal augmentation using
autologous cartilage: a preliminary report. Cleft Palate Craniofac J.
1993;30:46–54.
117. Leuchter I, Schweizer V, Hohlfeld J, et al. Treatment of
velopharyngeal insufficiency by autologous fat injection. Eur Arch
Otorhinolaryngol. 2010;267:977–983.
118. Gersuny R. About a subcutaneous prosthesis. Zschr Heilk.
1900;21:199–204.
119. Eckstein H. Demonstration of paraffin prosthesis in defects of the
face and palate. Dermatology. 1904;11:772–778.
120. Blocksma R. Correction of velopharyngeal insufficiency by silastic
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121. Lewy R. Teflon injection in the correction of velopharyngeal
insufficiency. Ann Otol Rhinol Laryngol. 1965;74:874.
122. Bluestone CD, Musgrave RH, McWilliams BJ. Teflon injection
pharyngoplasty. Cleft Palate J. 1968;5:19–22.
123. Smith JK, McCabe BF. Teflon injection in the nasopharynx to
improve velopharyngeal closure. Ann Otol Rhinol Laryngol.
1977;86:559–563.
124. Furlow LT, Williams WN, Eisenbach CR, et al. A long term study
on treating velopharyngeal insufficiency by Teflon injection. Cleft
Palate J. 1982;19:47–56.
125. Reisberg DJ. Dental and prosthodontic care for patients with cleft
or craniofacial conditions. Cleft Palate Craniofac J. 2000;37:534–537.
126. Glaze LE. Behavioral approaches to treating velopharyngeal
dysfunction and nasality. In: Moller KT, Glaze LE, eds. Cleft Lip
and Palate: Interdisciplinary Issues and Treatment. 2nd ed. Austin, TX:
Pro-Ed; 2009:415–452.
127. Tomes LA, Kuehn DP, Peterson-Falzone SJ. Behavioral treatments
of velopharyngeal impairment. In: Bzoch K, ed. Communicative
Disorders Related to Cleft Lip and Palate. Austin: Pro-Ed; 1997.
128. Ruscello DM, Shuster LI, Sandwisch A. Modification of context-
specific nasal emission. J Speech Hear Res. 1991;34:27–32.
129. Witzel M, Tobe J, Salyer K. The use of videonasopharyngoscopy
for biofeedback therapy in adults after pharyngeal flap surgery.
Cleft Palate J. 1988;26:129–134.
130. Yamaoka M, Matsuya T, Miyazaki T, et al. Visual training for
velopharyngeal closure in cleft palate patients: a fiberscopic
procedure (preliminary report). J Maxillofac Surg. 1983;11:
191–193.
131. Ysunza A, Pamplona M, Femat T, et al. Videonasopharyngoscopy
as an instrument for visual biofeedback during speech in cleft
palate patients. Int J Pediatr Otorhinolaryngol. 1997;24:45–54.
132. Kuehn DP, Imrey PB, Tomes L, et al. Efficacy of continuous
positive airway pressure for treatment of hypernasality. Cleft Palate
Craniofac J. 2002;39:267–276.
133. Lof GL. Oral motor exercises and treatment outcomes. Perspect
Lang Learn Educ. 2003;10:7–11.
134. McCauley RJ, Strand E, Lof GL, et al. Evidence-based systematic
review: effects of nonspeech oral motor exercises on speech. Am J
Speech Lang Pathol. 2009;18:343–360.
135. Lass NJ, Pannbacker M. The application of evidence-based practice
to nonspeech oral motor treatments. Lang Speech Hear Serv Sch.
2008;39:408–421.

SECTION I • Clefts
27 
Secondary deformities of the cleft lip,
palate, and nose
Edward P. Buchanan, Laura A. Monson, Edward I. Lee, David Y. Khechoyan, John C. Koshy,
Kris Wilson, Lawrence Lin, and Larry H. Hollier Jr.
SYNOPSIS
■ How did we get here? What led to this secondary deformity?
• Spectrum of clefts: type and severity
• Primary operative techniques
• Technical expertise/experience
• Growth
• Timing of repair.
■ Cut as you go.
■ Prevention, prevention, prevention.
Introduction
Identifying and treating secondary cleft lip and palate defor­
mities is a challenging endeavor for even the most experienced
surgeon. Proper diagnosis involves an understanding of pre­
vious operations as well as a thorough physical examination.
Surgical timing depends on deformity severity, age of patient,
and understanding and willingness of family members.
Examination and evaluation
Children born with clefts of the lip and palate should ideally
be cared for by a multidisciplinary cleft team who adhere to
the international standards set forth by the American Cleft
Palate and Craniofacial Association.1 The standards set forth
by this organization provide for the proper care and follow-up
required to achieve successful long-term outcomes. Occasion­
ally, children do not have access to these teams, and their care
is performed outside of this standard. If these children are
initially treated elsewhere and are referred into a team,
there can be secondary deformities related to their initial
treatment.
A thorough history, including an understanding of all
previous interventions, must be obtained. Often, knowing
where the operations occurred will help to understand what
was previously done. Many parents are not familiar with
repair techniques, but surgeons are often aware of the types
of repairs performed in different areas of the world. The
physical examination can provide further clues as to what was
previously performed. Scars around the nose and lip can alert
the examiner to the primary or secondary techniques. Anima­
tion can help the examiner understand the status of the
underlying orbicularis oris muscle. Intraoral examination
may reveal which operations were done on the palate and
identify the presence of oronasal fistulas, the status of the
levator muscles, the length of the soft palate as well as the
presence of alveolar clefts. Intranasal examination will help
determine if a primary rhinoplasty was performed, the status
of the nasal septum and turbinates, and the patency of the
nasal cavity. A proper speech assessment will determine the
functionality of the palate and whether more tests need to be
done.
Unilateral cleft lip
Secondary unilateral cleft lip deformities are a common occur­
rence in cleft centers.2 The etiology of these deformities is
varied and can result from any number of contributing factors,
including but not limited to continued facial growth, scarring,
infection, or inaccurate primary repair.
Secondary deformities frequently require surgical interven­
tion for correction, spanning from minor revision to a complete
takedown and re-repair. Many different techniques have been
described, depending on the location and severity.2–6 Proper
treatment begins with an accuVrate analysis of the deformities
in relation to the involved anatomic structures (Video 27.3
).
A thorough history and physical examination should be
performed in a systematic fashion and documented in the
medical record. 2D photographs should be performed in a
standardized fashion so as to consistently and objectively
capture pre- and postoperative data for evaluation and out­
comes purposes. When examining the cleft lip deformity, a
Access video content for this chapter online at expertconsult.com

638
SECTION I
CHAPTER 27 • Secondary deformities of the cleft lip, palate, and nose
can help prevent further scar contracture and notching
during animation.
Augmentation with autologous fat has been used to correct
notching as well as vermilion deficiency in cleft patients. This
technique is indicated when the deformity results from soft-
tissue discrepancy only.
Mucosal excess
The symmetry of the upper lip can be compromised by an
increase in the amount of scar tissue produced, after the origi­
nal repair, at the level of the mucosa (Fig. 27.1). Scarring at the
lip margin can be unpredictable and result in an asymmetric
lip. Careful attention to aligning the mucosa directly below
the level of the vermilion and up into the gingivobuccal sulcus
is important. Furthermore, using a suture that does not cause
an excess amount of inflammation can help prevent a large
amount of postoperative scarring.
Mucosal thickening is a common occurrence and a hard
problem to prevent. Often, mucosal excess develops during
top down approach is advocated for simplicity’s sake. The
examiner starts with the nose and ends with the mucosa of
the upper lip. Care to evaluate symmetry between the affected
and unaffected sides is of major importance. Review of the
nose (dorsum, tip, alae, base, and sill), and lip (philtral unit,
Cupid’s bow, vermilion, and mucosa) should all be done in a
systematic fashion. A list of the involved anatomic structures
is recorded. Treatment planning should consider the anatomy
of all structures involved so that a plan to restore appropriate
form and function can be made.
The timing of cleft lip revision depends on the severity of
deformity, the age of the patient, and the preference of the
patient, family, and surgeon. Deformity severity and func­
tional disability is relevant to surgical timing. If the lip repair
is inadequate, affecting the ability to eat or drink properly,
impacting speech production, or is socially unacceptable, a
revision could be planned soon after evaluation. These find­
ings would represent the more severe cases and not the norm,
as most revisions can be delayed until pre-kindergarten years
(4 to 5 years of age), or prior to full social integration. This
allows for the previous lip surgery scar to fully mature and
the opportunity to combine other cleft-related operations if
needed, such as speech surgery.
Vermilion/Mucosa
Vermilion notching
Proper alignment and repair of the vermilion is key to creating
a symmetric and harmonious appearing lip. To accomplish
this, the height of the vermilion on the cleft side should match
the contralateral side. If this is not the case, and the vermilion
height is less on the cleft side, a vermilion notch will result.
The appearance of the lip with a vermilion notch can have the
initial appearance of a short lip, but on further inspection, the
vermilion discrepancy should be appreciated. The central or
lateral incisor on the affected cleft side can be more noticeable
than the opposite side. Furthermore, if mucosa is displaced
into the vermilion, an area of persistent dry skin will result.
This becomes extremely annoying to the patient and notice­
able for the family.
Vermilion notching can occur despite a perfectly restored
vermilion/mucosal “red line” junction. Scarring along the
repair site may result in vermilion notching and may require
revision. It is important to appreciate that this deformity is
not necessarily a result of a loss or lack of tissue, but may be
secondary to abnormal scarring or misalignment of the red
line.
This deformity may be prevented by properly choosing the
Cupid’s bow point on the lateral lip element (Noordhoff’s
point).7 An incision placed too laterally compromises an
already short lip in the transverse/horizontal orientation. An
incision placed too medially compromises the vertical height
of the lip and vermilion and also risks losing the white roll.
Therefore, adhering to Noordhoff’s point during the primary
repair is paramount in establishing the correct height of the
lip’s vermilion.
If lip notching results from scar contracture, a Z-plasty
maybe used to break up the scar. Failure to initially repair
the muscle or subsequent muscle dehiscence should be
appreciated during the preoperative visit and addressed
by dissection and re-suturing of the orbicularis oris. This
A
Fig. 27.1  A young female with lateral vermilion fullness. This affects the patient in
repose (A) with contour changes along the free vermilion border, as well as when
smiling (B).
B

639
Unilateral cleft lip
cleft-side peak of the Cupid’s bow. The use of permanent or
long-lasting resorbable sutures should be considered for
muscle repair to relieve tension on the closure and to prevent
postoperative scar widening.
Long lip
The appearance of a long lip as a secondary deformity in the
unilateral cleft patient is an unusual occurrence. It is more
commonly seen after a patient has been repaired with a
Z-plasty type repair technique (Fig. 27.3).8,9 This deformity
occurs when the cleft-side height has been overcorrected and
there has been overrotation of Cupid’s bow.
Minor vertical height excess can be reduced with a hori­
zontal wedge excision along the nasal base. The incision is
kept in this groove to prevent any noticeable scarring. Major
vertical height discrepancy should be addressed with a com­
plete revision and re-approximation of anatomic structures.
the healing process, and not being able to control how much
scar tissue will form is frustrating. Excising this mucosa can
be a satisfying operation, nonetheless resulting in an unsatis­
fying experience during the follow-up period. Secondarily, a
simple transverse wedge made at the level of the mucosa,
inferior to the vermilion mucosal red-line margin, is normally
done to achieve balance. If mucosal thickening recurs after
revisionary surgery, performing scar therapy and waiting a
full year to 18 months for maturation is indicated. Oftentimes,
with facial growth, this thickness will “even out” and further
revisions may not be required.
Skin
Short lip
The short lip, after a unilateral cleft lip repair, usually results
from postoperative scarring, but can result from failing to
balance the Cupid’s bow (Fig. 27.2). This deformity is one of the
most commonly seen and revised given its tendency to distort
the normal lip architecture. All straight-line scars can shorten;
and, with time, a perfectly repaired cleft lip can shorten and
result in an imbalanced repair and “under-rotated” Cupid’s
bow. Prevention of this deformity can be achieved by “break­
ing up” the incision during the original repair.
Minor vertical height deficiencies up to 1 mm can be cor­
rected using an elliptical excision of the previous cleft scar at
the red–white line.4,8 Due to the Rose–Thompson effect, this
will elongate the lip the necessary 1 mm to match the opposite
lip length and balance the Cupid’s bow. If a triangular flap
repair was initially performed and the lip is moderately short
(around 2–3 mm), a Z-plasty just above the white roll can be
made to level Cupid’s bow and break up the scar. Alternately,
an opening incision on the medial lip segment, above the
white roll, can be made and a triangular flap from the lateral
lip segment can fill the opening incision.8 Any major discrep­
ancy in vertical height requires complete revision of the lip
repair, including adequate release of all lip components
from abnormal attachments, “un-bunching” of the lateral lip
muscle, precise muscle alignment, and accurate leveling of the
Fig. 27.2  (A–C) Classic secondary cleft lip and nose deformities associated with the unilateral cleft lip, including
a short lip deformity, inferolaterally displaced alar base, as well as a depressed nasal tip. She also demonstrates
midface retrusion.
A
B
C
Fig. 27.3  This patient has the scar characteristics of a Randall–Tennison-type cleft
lip repair. Her lip demonstrates a nice three-dimensional shape, but the scar runs
transversely across the midportion of her lip. This is the primary critique of this
repair.

640
SECTION I
CHAPTER 27 • Secondary deformities of the cleft lip, palate, and nose
tension, infection, and genetic predisposition. Measures are
made to prevent or alleviate any factors that may contribute
to a poor scar. To help relieve any unnecessary tension, com­
plete dissection of involved structures, as well as a strong
muscle repair, is performed. This should allow for the skin
structures to be easily repaired without undue tension.
Aggressive postoperative wound care is done to prevent
infection. Scar management is employed to help achieve a
thin, flat, and well-blended scar (Fig. 27.5). This is normally
done with taping, scar massaging and sun protection. Scar
management lasts for 12–18 months, or until the scar has
fully matured.
Choice in the type of suture material has been much
debated and depends on the preference of the operative
surgeon. Permanent sutures need to be removed in 5–7 days
to prevent permanent scarring at the sutures sites. Removal
usually occurs in the procedure room, requiring another
anesthetic exposure. Patients, who get dissolvable sutures, do
not need to have their sutures removed; however, sutures
must be gone by the 5th–7th day. If they are not, notable scars
along the length of the incision can result.
White roll discontinuity
Misalignment of the white roll is another common problem
seen in unilateral cleft deformities, and one of the most easily
detectable from a conversational distance. A step-off at this
level is readily detected by the human eye, given its position
at the vermilion–cutaneous junction and its involvement in
the harmony of the entire upper lip contour (Fig. 27.4).
In cases of white roll mismatch, a diamond-shaped excision
of the white roll scar, extending above and below the roll, will
align the white roll. White roll mismatch may be associated
with under-rotation or vertical height deficiency of the medial
lip segment. Often, excising the scar is enough to lengthen the
lip and restore appropriate balance.
Poor scarring
Poor postoperative scarring is an adverse event, which
transcends the finest surgical technique and is completely
unpredictable. Despite that, there are a few factors believed
to be associated with poor scarring, including local wound
Fig. 27.4  This young man has a notable step-off at
the vermilion and significant cleft lip scarring. He also
has scarring in to the columella (A,B). Markings were
made to realign the white roll and excise the widened
scar (C), with the subsequent on-table result (D, E).
A
B
C
D
E

641
Unilateral cleft lip
mobilizing skin flaps for tension-free closure. The underly­
ing muscle is assessed at the time of scar revision. If the
muscle is scarred or abnormally oriented, then excision and
full-thickness revision is advised. If the muscle repair is intact,
then muscle imbrication, to reduce tension on the closure,
can be performed. It is important to understand that scar
revision also shortens an already deficient skin envelope and
should be approached with caution to prevent unnecessary
shortening.
It is important to assess the position of the alar base during
revision. If the alar base is asymmetric, usually posterior and
cephalic, the scar revision can facilitate correcting this problem.
Access to the alar base and the nasalis portion of the orbicu­
laris oris can be achieved, allowing for repositioning. During
any revisionary operation, care to fully assess all other defor­
mities should be performed with a mind towards an overall
aesthetic subunit correction.
Scar location is another important consideration in cleft
surgery. Most unilateral cleft lip operations attempt to place
the repair scar along the cleft-side philtral column to match
the normal side. Some repairs will incorporate a lateral rhi­
notomy incision to help reposition the alar base. This incision
should be approached with caution due to its visibility and
potential negative impact it can have on nasal alae growth.
Red or pink scars after surgery are expected and fall within
the realm of normal scar maturation. Parents and families
should be educated to prepare them for the amount of time it
will take for this scar to fade. If, however, the scar begins to
get thick, raised, and redder, a hypertrophic scar may be
developing, and treatment can include taping and injections.
More frequent follow-ups should be made to monitor the
progress of the scar.
Unsightly scars can be addressed with scar revision by
excising the scar down to underlying muscle and adequately
A
B
C
Fig. 27.5  This young man demonstrates hypertrophic scarring, notching and discontinuity of the white roll, as well as vermilion mismatch at the red line (A, B). Surgical
intervention required excision of the areas of hypertrophic scarring, as well as Z-plasty-based rearrangement of the red line (C, D). The immediate postoperative outcome is
demonstrated (E, F).
D
E
F

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SECTION I
CHAPTER 27 • Secondary deformities of the cleft lip, palate, and nose
the family in the operative plan so they are aware of what
will occur.
Vermilion/Mucosa
Vermilion deficiency – whistle deformity
Deficiency of the midline vermilion is primarily associated
with bilateral clefts and is the most common form of vermilion
deformity. The “whistle deformity” is a result of a number of
factors, such as failure to restore normal muscular continuity
at the level of the vermilion, failure to properly restore mucosal
and vermilion continuity, muscular dehiscence, or severe
postoperative scarring. Identification of each of these will help
dictate the surgical approach.
Treatment of a whistle deformity is probably one of the
most common reasons to revise a bilateral cleft lip (Fig. 27.6).
If the whistle deformity is due to vermilion deficiency alone,
then a simple vermilion revision re-approximating the vermil­
ion and creating a fuller lip is called for. This sometimes can
be done with a simple Z-plasty at the level of the vermilion
to break up a previous straight-line scar. If the deformity is
due to vermilion deficiency as well as muscle dehiscence, a
more in-depth revision should be planned. If the whistle
deformity includes scarring of the lip skin, a complete take-
down of the previous repair and revision should be performed.
This will allow for maximal access to the mucosa, muscle, and
skin. In older children, permanent suture muscle repair should
be considered to prevent gradual muscle dehiscence and
postoperative scar widening.
Vermilion excess
Vermilion excess is commonly seen in patients with bilateral
deformities after a Manchester repair. The original Manchester
repair incorporated the vermilion from the prolabial segment.
Furthermore, the orbicularis muscle sphincter and the anterior
gingivobuccal sulcus are not created. This repair usually
Muscle
Dehiscence
Failure to properly dissect and repair the muscle at the time
of primary repair, or muscular dehiscence after surgery, can
lead to lip asymmetry, unnatural animation, imbalance of the
nasal bases, and vermilion notching. Muscle continuity can be
assessed by evaluating for lateral muscle bulging during
animation.
Failure to repair the marginal component of the orbicularis
muscle, at the level of the muscle vermilion interaction, can
result in notching of the vermilion. Failure to properly identify
the nasalis portion of the orbicularis muscle, at the level of the
nasal base, can result in an abnormally positioned alae and
nasal asymmetry. Careful examination can reveal these very
subtle but easy to correct problems.
Lip revision is recommended when muscle dehiscence is
suspected. Revision should include complete dissection of the
muscle and proper orientation of the nasalis, marginalis, and
main portions of the orbicularis muscle. More so than in the
primary repair, the use of permanent or long-lasting absorb­
able suture should be entertained to prevent muscle dehis­
cence and scar widening.
Bilateral cleft lip
Treatment of patients with secondary bilateral cleft deformi­
ties requires an understanding of previous operations, as well
as a thorough evaluation of anatomy. Recognition of common
deformities will help facilitate the surgical planning process
and assist the surgeon during future procedures. Timing of
any surgical intervention is dependent upon the severity of
the deformity, age of the patient, and the understanding and
expectations of the family. There are characteristics specific to
the bilateral cleft lip that make secondary deformities distinct
from those seen in unilateral clefts, including a paucity of
central lip tissue, the size of the columella, and the limited
blood supply to the prolabium. It is helpful to think of bilateral
cleft lip deformities in terms of issues within the layers of the
lip: mucosa/vermilion, muscle, and skin. If these are taken
into consideration, a successful secondary correction can be
achieved.
As with unilateral clefts, the timing for bilateral lip revi­
sions can be controversial and should be decided by com­
munication with the patient’s family. Secondary revisions
have both functional and aesthetic components. If significant
issues with speech, nasal regurgitation, or socialization exist,
revision should be considered sooner than later. If the revision
is for aesthetic concerns, then it can be delayed until the
patient and family are interested. Revisions should be delayed
long enough to allow for maximal scar maturation. Revisions
are often delayed until the pre-kindergarten years (age 4–5
years), unless there are functional concerns.
When revising a bilateral cleft lip, diagnosing the
deformity and understanding the previous operations is of
paramount importance. The nose, upper lip, and gingivobuc­
cal sulcus should be considered in detail. Furthermore, the
components of the lip, including skin, mucosa, vermilion,
and orbicularis oris, should all be examined and addressed
prior to devising the surgical plan. It is important to include
Fig. 27.6  This figure demonstrates the whistle deformity, which is absent
apposition between the upper and lower lip centrally.

643
Bilateral cleft lip
will also require a more invasive muscle repair, as well as
addressing an insufficient gingivobuccal sulcus. Therefore, a
complete takedown and revision of the mucosa, vermilion,
muscle, and skin is recommended (Video 27.3
). The lateral
lip elements need to be brought together at the vermilion,
mucosa, and white roll level to create a harmonious and sym­
metric lip.
Mucosal excess
Excess mucosa and mucosal show with animation is a common
problem seen after bilateral cleft lip repair. Creation of the
results in an unsightly, dry, and flaky vermilion at the level of
the central tubercle. Furthermore, due to the inherent lack of
adequate white roll in the prolabial segment, there is no
continuity of the white roll across the upper lip. This defor­
mity becomes apparent from a conversational distance and a
constant source of dissatisfaction from patients and families.
The vermilion in this area can have a “pressed on” appear­
ance, without the impression of a symmetric and confluent lip
(Fig. 27.7). Patients who had a Manchester-type bilateral cleft
lip repair will have vermilion from the original prolabial
segment within the upper lip.10 If this is the case, at least the
skin and vermilion should be revised. Usually this revision
A
B
C
D
Fig. 27.7  A young male with the classic sequelae
associated with the Manchester repair. The central
prolabial segments were retained to reconstruct the
nasal sills, the white roll, and the central vermilion. He
has both a widened philtrum and a discontinuous white
roll (A, B). He subsequently underwent revisionary
procedure to excise the widened scars as well as
excess tissue in the nasal sills (C, D), with the
immediate on-table result (E, F).
E
F

644
SECTION I
CHAPTER 27 • Secondary deformities of the cleft lip, palate, and nose
Fig. 27.8  Preoperative image demonstrates correction of a discontinuous white roll
from previous bilateral cleft lip repair.
A
Fig. 27.9  This figure (A) demonstrates prominent suture marks associated with
previous bilateral cleft lip repair. Subsequent surgical correction required excision
and primary revision (B).
B
Fig. 27.10  This figure demonstrates scar widening associated with the bilateral
cleft lip deformity. Widened scars that were mature would generally be managed
with excision and primary closure.
anterior gingivobuccal sulcus during the initial repair is para­
mount to preventing this deformity (Video 27.5
). Some
older techniques did not include creation of this sulcus, and
the upper lip was tethered to the premaxilla. If the sulcus is
inadequately created, then redundant prolabial mucosa can
mask the upper central incisors during animation and repose.
If this is the case, a vestibuloplasty is performed. The anterior
mucosa of the prolabium is released and sutured to the raw
surface of the premaxilla. It is important that the prolabial
mucosa is thinned so that it will become adherent to the raw
surface of the premaxilla, recreating the attached gingiva, and
successfully creating the posterior surface of the gingivobuc­
cal sulcus. A 1–2 mm margin of mucosa is left free to suture
to the lateral mucosal lip segments in the depth of the sulcus.
The lateral mucosal lip segments create the anterior surface
of the gingivobuccal surface.
Skin
Poor scarring
Despite the most perfect surgical technique, a poor lip scar
can cause any cleft lip repair to be noticeable (Fig. 27.8). In
addition to the usual risk factors, those specific to the bilateral
cleft lip patient can include inadequate release and repair of
the orbicularis muscle, a protruding premaxilla, and excessive
cleft width – all contributing to an excess amount of wound
closure tension at the time of repair.
The use of permanent cutaneous sutures, kept in for too
long, or dissolving sutures that do not dissolve rapidly enough
can result in lip scars that have noticeable crosshatching
marks (Fig. 27.9). Assuring that sutures are gone within 5 days
will prevent them from causing permanent scars.
As with any unsightly scar, scar revision may be recom­
mended. If the scars are widened or have crosshatch marks,
an excision and re-approximation can be performed (Fig.
27.10). An attempt to prevent future scar widening can be
considered by imbricating the underlying orbicularis muscle
so as to create a tension-free closure at the level of the skin.
This can be done with a permanent suture, especially in older
children where muscle memory and coordination are well

645
Cleft palate
female philtrum.14 These measurements can be incorporated
into the surgical plan, but clinical judgment should be exer­
cised as it relates to the final appearance.
Muscle
Dehiscence
Failure to properly dissect and reapproximate the orbicularis
oris muscle at the initial repair, as well as postoperative
muscle dehiscence, accounts for many secondary bilateral
cleft lip deformities. This is diagnosed by lateral lip muscle
bulging during animation, representing discontinuity of the
muscular sling. Furthermore, failure to appropriately dissect
the orbicularis oris muscles from the nasal base can result in
widened alar bases. Careful attention should be made to
identify and repair the different components of the orbicularis
muscle in the upper lip.15 An attempt to repair the nasalis, the
marginal, and main components of the orbicularis oris are
important to restoring the normal contour to the upper lip. If
the muscle is repaired appropriately, the amount of tension
placed on the skin repair is minimal, and this can prevent
future scar widening.
If muscle dehiscence is suspected, then re-repair is recom­
mended to achieve normal lip appearance and function.
Restoring orbicularis oris continuity is one of the most impor­
tant components of a successful repair. Determining the dif­
ferent components of the orbicularis muscle during revision
is recommended. Permanent or a long-lasting absorbable
suture will help prevent postoperative muscle dehiscence and
prevent undue tension on the skin closure, helping prevent
scar widening.
Cleft palate
Some feel that outcomes in cleft palate surgery have been
overlooked, when compared with cleft lip, not only due to the
lack of visibility, but also due to poor definitions and incon­
sistent evaluation methods. Standardization of the evaluation
and characterization of speech outcomes is a difficult goal, in
part because of the subjective nature of speech evaluation;
however, it is a goal that will greatly assist the ability to
measure outcomes. Unlike a cleft lip repair, visible for all to
see, the results of a cleft palate repair require a more in-depth
evaluation. While the goal of cleft palate repair is normal
speech and resonance, fistulas and velopharyngeal insuffi­
ciency (VPI) can occur. Consistent and accepted definitions of
fistula and VPI will help cleft teams communicate more
effectively.
Velopharyngeal insufficiency
The diagnosis of VPI, of which hypernasality is one feature,
and the recommendation for secondary surgery can be differ­
ent for every patient and are highly dependent on cleft center.
Ideally, children should have at least an annual speech evalu­
ation prior to age six and have any surgery for VPI completed
by school age.16 However, there will be patients who are lost
to follow-up, experience involution of adenoids, have maxil­
lary growth or maxillary advancement, and will require
work-up and treatment at an older age. A symptomatic fistula
can also be a source of nasal air loss and may need to be
established. Scar revision is rarely done in isolation and
usually is involved with correction of other deformities, such
as a widened prolabium, white roll mismatch, vermilion
asymmetry, or alar base malposition.
Skin deficiency
A tight upper lip can be seen in patients who have had mul­
tiple revisions, who have had too much lateral lip skin
removed during the initial operation, or who have had prola­
bial necrosis after repair. Patients undergoing multiple revi­
sions require repeated skin excision to line up landmarks, and
this makes the upper lip horizontally shorter compared to the
lower lip, giving the upper lip a flat appearance.
The prolabium’s blood supply should be respected during
surgery to prevent ischemia. After surgical intervention, the
prolabium should be pink and perfused to ensure survival. If
it appears white, then sutures should be removed to help
prevent tissue ischemia. If the prolabium becomes ischemic
and necrotic, it will heal as a scar, result in a poor cosmetic
outcome, and potentially lead to a short and tight upper lip.
When local options for tissue rearrangement have been
exhausted and there is skin deficiency, an Abbe flap may be
incorporated (Video 27.1
).11,12 This procedure is used when
there is a loss of tissue from multiple operations or when the
prolabium has been discarded. The Abbe flap is being used less
frequently given the improvements in primary techniques. It
does have the ability to reconstruct the upper lip in all three
layers, restoring a normal-appearing Cupid’s bow, central
tubercle, and philtrum. In addition, it decreases the horizontal
size of the lower lip, helping camouflage upper lip deficiency.
When designing the Abbe flap, reconstructing the entire
philtral unit should be planned, improving upper lip tight­
ness and providing a balanced facial profile. The flap is
transposed from the center of the lower lip and inset into the
upper lip. This allows for the recreation of Cupid’s bow and
the entire philtral unit. Designing this flap slightly smaller
than normal will account for stretching of the flap once inset
and tension-free closure on the lower lip donor site. The flap
is based on the inferior labial artery, which can be divided at
least 10–14 days after inset. At the time of flap division and
inset, compressing the pedicle prior to division will confirm
viability of the flap.
Skin excess
In bilateral cleft lips, the philtrum can progressively widen
over time. Growth is the most likely reason for this widening,
but scarring and tension have been thought to contribute as
well. Contemporary primary repair techniques suggest the
prolabium design to be around 1.5–2 mm at the hemi-Cupid’s
bow and 2–3 mm at the base.13 These measurements account
for the widening that can occur with growth. Given the risk
of vascularity with a prolabium of this size, care is exercised
to keep the base intact so as to prevent devascularization.
A widened philtrum can be addressed at any point during
growth, but usually waiting until final growth is complete is
recommended, so as to prevent future revisions. If lip appear­
ance is a significant concern in a growing patient, revisions
can be made after consultation regarding future surgeries.
When designing the size of the philtral subunit, anthropomor­
phic studies have evaluated the size of the normal male and

646
SECTION I
CHAPTER 27 • Secondary deformities of the cleft lip, palate, and nose
These symptomatic fistulas are considered to be functional
and need to be surgically addressed.
Patients who have already undergone secondary speech
surgery and still display evidence of VPI are in a more
advanced stage of cleft care and will need to have another
speech evaluation to help determine the cause of their dis­
ability. As before, speech evaluation with objective measures
such as pressure flow testing and nasometry as well as
imaging should be considered. A thorough intraoral exam
should be performed to evaluate the architecture of the soft
palate and velopharyngeal structures. A nasoendoscopy is the
best way to see the velopharyngeal architecture during pho­
nation. This helps the treating physician understand the limi­
tations of the original cleft palate surgery as well as any other
surgeries that have been performed. Videofluoroscopy may
have limited benefit in the patient who has already undergone
secondary speech surgery, particularly if the suspected defect
is very small.
Fistula closure
Closure of palatal fistulas is a significant challenge for the cleft
surgeon. Small fistulas can be managed conservatively if they
are non-functional or asymptomatic. Fistulas are clinically
significant or functional when they lead to nasal air escape
(hypernasality and audible nasal emission), speech distortion,
or nasal regurgitation of liquids and solids. The published rate
of postoperative fistulas varies widely, from 0–70%.17–19 This
is in part due to the lack of a standard classification scheme,
which hinders both communication and research efforts. At
our institution, we prefer to use the Pittsburgh Fistula Clas­
sification System, described by Losee et al., due to its ease of
use.20 The Pittsburgh Fistula Classification System includes
seven fistula types: those at the uvula, or bifid uvulae (type
I); in the soft palate (type II); at the junction of the soft and
hard palates (type III); within the hard palate (type IV); at the
incisive foramen, or junction of the primary and secondary
palates – reserved for Veau IV clefts (type V); lingual–alveolar
(type VI); and labial–alveolar (type VII) (Fig. 27.12).
Fistulas are difficult to repair and have a reported recur­
rence rate approaching 65%.21 Many strategies for palatal
Fig. 27.11  This patient has a large type IV, V, and VI oronasal fistula associated
with a bilateral cleft palate. She has constant nasal regurgitation and air escape.
addressed alone or in conjunction with secondary speech
surgery.
Workup and algorithm
Patients who present with continued speech concerns require
a thorough work-up by an experienced speech and language
pathologist who is a member of the cleft team. A formal
speech evaluation will diagnose features of velopharyngeal
insufficiency including hypernasality, audible nasal emission,
light articulatory contacts, as well as compensatory articula­
tion errors. This evaluation is used to set goals for therapy as
well as determine the efficacy of surgical intervention. Naso­
endoscopy and videofluoroscopy visualize the pattern of
velopharyngeal closure during speech production. Indirect
measures, such as pressure flow testing and nasometry, are
often completed during a formal speech evaluation. These
studies are used as objective measurements to supplement
perceptual ratings. Guided by a thorough work-up, the speech
pathologist determines if the speech difference is due to a
structural problem, and would therefore be aided by a surgi­
cal solution, or if the speech difference may be corrected
through speech therapy. The speech pathologist will also help
to determine which surgical option would be the best, based
upon the pattern of velopharyngeal closure.
Outlining the complete speech evaluation for a child with
VPI is beyond the scope of this chapter; however, the opera­
tive surgeon should understand some basic considerations.
First, the patient needs to be mature enough to cooperate and
provide an adequate speech sample. For children without
significant speech delays, this can typically be done by age 3.
Pressure flow and nasometry may be achieved with a very
mature and cooperative preschool-aged child. Nasoendos­
copy, while our preferred method for imaging, may not yield
a representative speech sample until the child is older. Video­
fluoroscopy can be performed on a child as young as three or
four years old, or for those children who will not tolerate
nasoendoscopy.
In addition to perceptual and instrumental speech exams,
physical examination of the palate during phonation can
provide a great deal of information for the surgeon. A palate
previously repaired with a straight midline technique, without
complete dissection and repair of the levator palatine muscles,
will demonstrate a “vaulted V”-shaped pattern of elevation.
This is due to the sagittally oriented levator muscles that were
never completely dissected from the clefted position and
placed in the anatomic transverse orientation. When stimu­
lated (with phonation or a gag), the muscles bulge laterally in
the palate with no motion along the midline, resulting in the
vaulted V-shaped pattern of palatal elevation. This finding
should alert the examiner to the abnormal muscle orientation
and help them determine what the most appropriate interven­
tion will be.
Oronasal fistulas can result in significant speech difficulties
as well as velopharyngeal insufficiency. A formal speech
evaluation can help determine whether a fistula is contribut­
ing to any speech abnormalities. Fistulas can be characterized
as functional or non-functional. Non-functional fistulas
usually do not cause any speech disability and do not require
surgical intervention. These fistulas are usually small and are
not surgically corrected. As fistulas get bigger, they can cause
nasal regurgitation, air escape, and hypernasality (Fig. 27.11).

647
Cleft palate
Fig. 27.12  (A–C) Even with perfect surgical technique, postoperative scarring and inflammation vary, resulting in a broad spectrum of secondary deformities.
A
B
C
fistula repair have been reported over the years, the complex­
ity of which ascends the reconstructive ladder.21–40 From direct
cauterization by Obermeyer in 1967,41 to an appliance/
obturator by Berkman, to the myriad of local flap designs by
von Langenbeck to Gabka in 1964, to free conchal grafts by
Ohsumi,25,36,38,41 the options put forth for closure of palatal
fistulas have been numerous. Guerrero-Santos and Altami­
rano popularized the tongue flap in the 1960s and 1970s,
which has remained a mainstay at some centers for closure of
larger anterior fistulas.32,42 Multiple pedicle flaps, including
the facial artery myomucosal (FAMM) flap, have been
described to treat the most difficult anterior fistulas.23,28,32,42,43
Even free tissue transfer24,27,31,35,37,38 has been described for
when all other modalities have been exhausted. As with many
situations in plastic surgery, when there are numerous proce­
dures described, it is often because no one option works
universally. The most recent advances in fistula treatment
have been the use of acellular dermal matrix (ADM), as
described by Kirschner et al.,33,34 and the use of the buccal fat
pad flap.44
In general, the larger the fistula, the larger the surgery
required to address it. Small local flaps have the inherent
problem of utilizing already scarred, stiff tissue that is often
friable and difficult to work with due to the constant exposure
to regurgitating nasal and oral contents (Fig. 27.13). The well-
described procedures such as the FAMM flap and tongue flap
have the benefit of bringing in vascularized, unscarred tissue
but at the expense of considerable donor site morbidity,
technical difficulty, considerable postoperative care, and need
for a second surgery (Figs. 27.14 & 27.15). The preferred
method at our institution for fistulas of the soft palate only
(types I and II) is a direct excision and repair, often in conjunc­
tion with a conversion Furlow palatoplasty if the fistula occurs
in the presence of velopharyngeal insufficiency.
Fistulas at the junction of the hard and soft palate (type III),
and those of the hard palate (type IV) require considerably
more than direct excision and closure due to their nature and
paucity of malleable local tissue. The local transposition flaps
described in various textbooks are often inadequate for com­
plete closure, as the arc of rotation often falls short. If local
nasal tissue proves to be inadequate, palatally based hinge
flaps or nasal septal flaps may be utilized. Another option,
based on the work of Kirschner and Losee et al.,33,34,45–48 is the
use of acellular dermal matrix as an adjunct when local soft-
tissue options prove inadequate for a watertight closure (Fig.
27.16). They advocate for use of a thin piece of acellular
dermal matrix for augmenting the nasal lining closure and have
data to support its use in all fistula types, except those of the
soft palate only, with a decrease in recurrent fistulas to
3.6%.33,34,45
Another option, when local tissue is insufficient for closure
of either the nasal or oral lining, is use of the buccal fat pad
flap, which has been widely described for closure of palatal
defects both in the head and neck literature as well as in the
cleft palate literature.44,49–51 It has several advantages over
other options and has become the preferred method for
closure of larger fistulas of the hard palate at our institution.
It is a simple flap to raise, requires no tedious dissection, is
reliable, has minimal donor morbidity, does not require tun­
neling or a second operation, and can easily reach the midline
or the lingual central incisors. Additionally, it is well-
vascularized, autologous tissue that epithelializes readily and
can be used for either nasal or oral closure.
In the case of fistulas, as in most secondary cleft surgery,
prevention is the key. A well-executed primary palatoplasty
is the best defense against fistula formation. A tension-free
closure and careful postoperative care are crucial in preven­
tion. During the primary palatoplasty, whether it is a two-flap

648
SECTION I
CHAPTER 27 • Secondary deformities of the cleft lip, palate, and nose
Fig. 27.13  (A–F) The first option for closure of palatal fistulas is local random-pattern tissue, which can be utilized in either a unipedicle or bipedicle fashion. It is
important to note, however, that the majority of the peripheral hard palate is granulated mucosa after primary palatoplasty.
A
B
C
D
E
F
procedure with an intravelar veloplasty or a Furlow palato­
plasty, care should be taken to include the following elements
in order to prevent fistulization: relaxing incisions, complete
intravelar veloplasty, total release of the tensor tendon at the
level of the hamulus, complete dissection of the neurovascular
bundle with optional osteotomy of the bony foramen, meticu­
lous surgical precision to limit unnecessary handling or
crushing of the mucosal edges, and diligent postoperative
care.
Furlow palatoplasty
The double-opposing Z-plasty, as described by Dr. Leonard
Furlow, is one of the first-line treatments in secondary speech
surgery.52 It accomplishes the goals of anatomically overlap­
ping the levator veli palatine muscles in the posterior half of
the palate and placing them under maximal functional tension.
It lengthens the palate and, due to the nature of the Z-plasty
effect, results in narrowing the velopharyngeal port, resulting
in a built-in pharyngoplasty. The Furlow is the option of
choice for patients with a short but mobile palate, those who
have not had a previous complete repair of the levators, and
those with a coronal closure pattern.
It is drawn as double-opposing Z-plasties, one on the oral
side and one on the nasal side. The posterior flaps, the left/
oral side and the right/nasal side, contain the levator muscle
as well as the mucosa. The anteriorly based flaps (the right
side on the oral surface and the left side on the nasal surface)
contain mucosa only. The left-side myomucosal flap is drawn
at approximately 60° from the junction of the hard and soft
palate medially towards the point where the levator exits the
skull base laterally (or the hamulus) (Fig. 27.17). The right-
sided mucosal only flap on the oral side is drawn slightly
greater than 60° from the junction of the uvula and soft palate
towards the hamulus. On the left side the muscle is taken off
the posterior edge of the hard palate and dissected laterally
and posteriorly, taking care to take down all of the abnormal
lateral attachments, until the muscle can be rotated 90° to the
skull base and transposed across the midline easily. The nasal
Z-plasty on the left side is then incised from the junction of
the uvula and soft palate, at a 60° angle, heading towards
where the levator exits the skull base and the hamulus. On
the right side, the anteriorly based mucosa-only flap is raised
to the posterior edge of the hard palate. The levator is then
dissected off the posterior edge of the hard palate from medial
to lateral. The nasal lining is incised from medial to lateral

649
Cleft palate
transpose the right-sided posteriorly based musculomucosal
flap to be inset into the left side. The nasal limbs can now be
transposed and inset. The nasal lining is closed with inter­
rupted absorbable sutures. Some surgeons prefer to perform
an intermuscular repair with permanent or absorbable sutures.
from just behind the posterior edge of the hard palate along
the trajectory of the levator laterally at a 60° angle; care is
taken to leave the levator attached to the nasal lining and to
take down all of the abnormal lateral attachments. This inci­
sion is made laterally – only as far as needed to adequately
Fig. 27.14  (A–D) Buccal tissue can be harvested via an axial pattern flap based off the facial artery. FAMM, facial artery myomucosal.
A
B
C
D
Mucosa
Buccinator
Cross-section through cheek
Muscles of facial expression
Skin
Buccal fat pad
Submucosa
Buccal branch
of facial artery
Buccal branch
of facial artery
FAMM flap

650
SECTION I
CHAPTER 27 • Secondary deformities of the cleft lip, palate, and nose
The oral lining is then closed with interrupted absorbable
sutures (Fig. 27.18).
A nasal trumpet or tongue stitch is placed for postoperative
airway management per surgeon preference. The patient is
maintained on a liquid diet for one week postoperatively and
then advanced to a soft diet for an additional two weeks.
Posterior pharyngeal flap
The posterior pharyngeal flap (PPF) is a secondary speech
surgery (pharyngoplasty) that creates a static bridge of tissue
between the posterior free edge of the velum and the posterior
pharyngeal wall. The PPF relies on adequate lateral pharyn­
geal wall motion to achieve velopharyngeal competence. It is
ideally suited for patients with sagittal or circular closure
patterns.53 Variations exist in the design of the PPF; however,
today most surgeons utilize a superiorly based flap that is
lined with the nasal mucosa of the soft palate. An appropri­
ately executed PPF will not be visible on intraoral inspection,
as it should be placed at the level of velopharyngeal closure,
usually above the palatal plane. Of note, when planning a
PPF, especially in children with velocardiofacial syndrome,
the surgeon should remember the potential for medially
Fig. 27.15  The tongue offers another regional option for palatal fistula repair.
Fig. 27.16  (A–E) Placement of acellular
dermal matrix provides a tool to achieve and
bolster complete closure of the nasal lining.
A
B
C
Acellular
dermal
matrix
D
E

651
Cleft palate
flap is just above the level of the palatal plane typically and
should be placed at the level of predicted velopharyngeal
closure. The flap raised from the posterior pharyngeal wall
will become the nasal side of the PPF, and the oral lining of
the PPF will be the nasal mucosal flaps pedicled at the poste­
rior free edge of the soft palate.
Nasal mucosal lining flaps are then raised from the soft
palate and pedicled on the posterior free edge of the velum,
taking care to obtain adequate width for lining. The defect in
the posterior pharyngeal wall can be partially or completely
closed primarily. The flaps are inset with resorbable suture;
each stitch is placed and tagged until all are placed. The
authors’ preferred method is to use 4-0 chromic gut suture
with three sutures along the posterior edge of the hard palate,
three along the posterior pharyngeal wall, and two on either
side for a total of ten sutures in all.
Overnight monitoring in a step down unit with pulse
oximetry, a nasopharyngeal airway, and a nasogastric tube is
preferred. A full liquid diet for one week and then a soft diet
for an additional two weeks is recommended. Due to its static
nature, the PPF has a risk of creating obstructive sleep apnea
(OSA). Children who have had PPF surgery should be closely
monitored, not just in the immediate postoperative period,
but throughout growth and development, for signs of OSA.
Sphincter pharyngoplasty
Sphincter pharyngoplasty is a dynamic pharyngoplasty for
the treatment of velopharyngeal incompetence, utilizing the
palatopharyngeus muscles to decrease the overall size of the
velopharyngeal (VP) area. By reducing the transverse diam­
eter, the soft palate is better able to achieve VP competence.
After identifying the posterior tonsillar pillars, a vertical
incision is made in the anterior mucosa (between the tonsil
and the posterior pillar) and posterior mucosa (between the
posterior tonsil and the posterior pharyngeal wall) from the
upper third of the tonsil to its inferior pole. The palatopha­
ryngeus muscle is separated from the superior constrictor,
and the myomucosal flap is elevated, extending beneath the
inferior pole of the tonsil and pedicled superiorly. Care must
be taken near the superior attachment to the soft palate where
the muscle is innervated. This is performed bilaterally.
At the level of the second cervical vertebrae, a horizontal
incision is made on the posterior pharyngeal wall (connecting
laterally to the posterior mucosal incision) to receive the
resected posterior tonsillar pillars. The horizontal incision
must consist of mucosa, intra- and peripharyngeal aponeuro­
sis, and superior constrictor muscular fibers.54 The sphincter
flaps are brought together with sutures along the posterior
pharyngeal raw surface. They can be brought together end-
to-end or overlapped entirely, depending upon the desired
size of the VP port. A central, dynamic opening bounded by
the repositioned posterior tonsillar pillars, the soft palate, and
the posterior pharyngeal wall is created. If hypernasality is
still present after surgery, the VP port size can be secondarily
modified by tightening the sphincter flaps.
Posterior pharyngeal wall augmentation
Posterior pharyngeal wall augmentation is another form of
pharyngoplasty intended to decrease the velopharyngeal port
size by moving the posterior pharyngeal wall closer to the
displaced internal carotid arteries and should proceed with
care.
Surgery begins with splitting the soft palate down the
midline and laterally retracting each hemipalate. The extent
of the PPF is marked out along the posterior pharyngeal wall,
with adequate length to reach the posterior edge of the hard
palate. The width of the flap will be determined by the
patient’s anatomy, the size of the preoperative gap, and the
surgeon’s preference. Some surgeons place red rubber cathe­
ters, nasogastric tubes, nasopharyngeal airways, or any
combination of the three, at the time of surgery to assist with
sizing of the lateral ports. The superiorly based flap is raised
at the level of the prevertebral fascia. The cranial base of the
Fig. 27.17  This picture demonstrates the oral-side markings for the Furlow cleft
palate repair technique.
Fig. 27.18  Furlow palate repair. The same patient is presented as in Fig. 27.17.

652
SECTION I
CHAPTER 27 • Secondary deformities of the cleft lip, palate, and nose
deformity have been discussed extensively and continue to be
debated. The exact findings have been well measured and
documented.57 An appreciation of the pathologic anatomy in
the unilateral and bilateral cleft nose deformity is essential to
achieve a satisfactory aesthetic and functional result.
Patients should be thoroughly evaluated with a history and
physical exam prior to surgery. Previous surgeries should be
reviewed to prepare for possible findings intraoperatively.
Patient concerns regarding their nasal airway and nose and
lip symmetry should be addressed. Preoperative photographs
are taken to document the deformities; they are also helpful
when consulting the patient regarding operative goals.
Physical exam of the nose should be done in relation to its
position on the facial skeleton. Cephalometric analysis evalu­
ates jaw and chin position and the effect on overall facial
balance. These issues should be brought up with the patient
and family, and if they are not interested in orthognathic
surgery and are happy with the overall facial appearance, an
exam centered on nasal shape and symmetry is performed.
Nasal examination should always be performed in a very
systematic and repeatable fashion. Gunter diagrams are very
helpful for preoperative planning purposes. The secondary
cleft nasal deformity is related to what existed at primary
repair but altered by any previous surgery. It is important to
understand these changes, as it may affect the planned
operation.
The unilateral cleft nasal deformity involves the soft tissues
and skeleton of the nose (Fig. 27.19). Aberrant muscle inser­
tion results in an imbalance that is influenced by maxillary
skeletal hypoplasia.58 Rather than a horizontal orientation and
continuous orbicularis oris across the upper lip, the muscle
inserts in a discontinuous manner, into the base of the colu­
mella on the non-cleft side. This can create an unopposed
force that pulls the columella and caudal nasal septum to the
palate. Augmentation is typically less invasive than other
secondary speech procedures because it usually involves
injection or insertion of a durable, long-lasting material, not
requiring dissection and tissue rearrangement. Many different
materials have been described for augmentation, including
Vaseline, silicone, cartilage, acellular dermal matrix, and
autogenous fat. For very small holes in the velopharynx
during phonation, we prefer fat grafting for augmentation.
Fat can be harvested from the abdomen or buttock, depend­
ing on the abundance. Most children, during the early school
age years, are rather thin and will require a buttock donor site.
An abdominal donor site is most commonly used for older
children. Manual suction with 10 cc syringes and a large
cannula through small access incisions are commonly used.
Once harvested, fat can be processed in a number of different
ways, in an attempt to increase the vitality of the fat cells.55
Once harvested and processed, the fat is loaded into syringes
for injection. Our standard practice is to perform the proce­
dure with the assistance of the otolaryngology team. A naso­
endoscopy is performed at the time of injection, and
confirmation of injection is made by both surgeons. The pre­
operative nasoendoscopy is reviewed prior to the operation
and should be readily available for confirmation of injection
site. No less than 10 cc of fat is injected per patient to account
for graft resorption.56 Grafting is normally done as an outpa­
tient operation and patients are discharged on pain medica­
tion and oral antibiotics. Follow-up speech evaluation is done
at 1–3 months after surgery.
Cleft nasal deformity
The cleft nasal deformity involves the skin, cartilage, mucosa,
and skeletal platform. Theories regarding the etiology of the
Fig. 27.19  The key aspects of the cleft nose deformity involve abnormalities of the osseocartilaginous skeleton. AP, anteroposterior.
Deviated septum
Normal alar cartilage
AP
Worm’s eye
Displaced nasal
spine
Lowered nostril sill
Displaced alar base
Cleft-side alar cartilage
Depressed dome on cleft side
Slanted
columella

653
Cleft nasal deformity
in a posterior inferior direction resulting in a visibly thickened
and hooded ala.64 Attachment of the lateral nasal sidewall to
the depressed cleft side piriform results in a vestibular
webbing that is visible through the cleft-side nares.
Timing of cleft nasal surgery can be divided into primary,
intermediate, and secondary repairs. Studies have disproved
the idea that early manipulation of the nasal cartilage inter­
feres with growth.59 Original experimental studies demon­
strated that large submucous resections of the nasal septum
affected subsequent nasal and midface growth; however,
studies by McComb and Coghlan demonstrated that reposi­
tioning the lower lateral cartilage, without cartilage resection,
did not interfere with subsequent nasal and midfacial devel­
opment.65,66 As a result of this research, primary cleft rhino­
plasty today regularly occurs with the initial lip repair.67 The
benefit of early intervention is restoration of nasal shape with
the potential for more symmetric nasal growth. However, it
is important to note that surgery done at an early age will
result in scar tissue likely affecting future surgeries.
Intermediate rhinoplasty
Intermediate rhinoplasty is usually performed before school,
between 4 and 6 years old.68 The goal of surgery is to correct
the aberrant position of the cleft-side lower lateral cartilage so
that future nasal growth will not exacerbate the cleft nasal
deformity. At this time, the surgeon may also perform any
minor lip revision, as necessary. Septal surgery should be
deferred until post-adolescence so as not to prevent distur­
bance in nasal growth.64
In unilateral cleft deformities, the intermediate rhinoplasty
addresses the cleft-side lower lateral cartilage (LLC) as well
as any lateral vestibular webbing. An open rhinoplasty
approach can be performed to expose the lower lateral carti­
lages, directly observe anatomic differences, and correct them
with suture techniques. Cartilage grafting is not performed at
this young age, given the residual growth potential of the
nose.69 If there is lateral vestibular webbing, a V–Y type inci­
sion can lengthen the lateral nasal sidewall and advance the
lower lateral cartilage forward.68
In bilateral cases, the intermediate rhinoplasty addresses
the depressed lower lateral cartilages and lengthens the
shortened columella.13,68 This is done via an open rhinoplasty
technique where the lower lateral cartilages are exposed
through transcolumellar and infracartilagenous incisions. The
LLCs are sutured together with a transdomal suture decreas­
ing their angle of divergence and placing them in a more
projected orientation.13 Soft tissue that is commonly found
between the dome points of the LLC can be thinned to help
achieve better tip projection and definition. The incisions are
then closed with plain gut in the vestibule and permanent
suture on the columella.
Secondary cleft rhinoplasty – unilateral
Secondary rhinoplasty occurs after facial growth has com­
pleted, around 14–16 years old in female patients and 16–18
years old in male patients.70,71 Surgical techniques rely on
well-accepted rhinoplasty principles for unilateral or bilateral
cleft nasal deformities.72–74 The open approach is preferred for
better exposure and visualization. Placement of cartilage
grafts for support and reinforcement is a major component of
non-cleft side.4 On the cleft side, the orbicularis inserts into
the alar base, retracting it laterally, inferiorly, and posteriorly
(due to poor maxillary skeletal support) (Fig. 27.20).
The asymmetric nasal tip results from the irregular lower
lateral cartilage on the cleft side. While not ubiquitously
accepted, the length of the lower lateral cartilage on the cleft
side is considered equal to the non-cleft side, primarily differ­
ing in shape and position.59–62 On the cleft side, the medial crus
is shorter and the lateral crus is longer with a less defined,
wider dome.6 This results in an underprojected tip defining
point when compared to the non-cleft side (see Fig. 27.2).
Deviation of the nasal septum is seen bilaterally. On the
non-cleft side, the unopposed pull of the orbicularis oris
muscle, as well as the premaxillary ligament, results in caudal
septal deviation. However, the premaxillary ligament only
affects the caudal septum, permitting bowing of the majority
of the septum towards the cleft side. Whether alone or in
conjunction with hypertrophied nasal turbinates, the aperture
of the internal nasal valve is decreased, resulting in nasal
obstruction.63
Nasal obstruction at the external nasal valve from the
introverted lower lateral cartilage is common. The cephalic
edge of the lower lateral cartilage introverts into the vestibule
A
Fig. 27.20  These images (A, B) demonstrate classic deformities associated with
the cleft nasal deformity. This includes deviated nasal septum and tip, inferolaterally
and posteriorly displaced cleft-side alar base, and abnormal nasal sill.
B

654
SECTION I
CHAPTER 27 • Secondary deformities of the cleft lip, palate, and nose
Nasal vestibule
Nasal vestibule contracture, resulting from either the primary
repair or revisions, is a difficult and common problem
encountered during secondary rhinoplasty. Scarring can lead
to nostril stenosis and collapse of the external nasal valve.
Local tissue rearrangement with healthy tissue can help
reconstruct this area and disrupt the constrictive forces. Alter­
natively, the scar, along with redundant subepithelial soft
tissue, may be excised, followed by placement of a bolster to
encourage lateral healing.79 Redundant skin may also be used
to line a stenotic vestibule.80 A laterally or medially based
sliding chondrocutaneous flap, harvested using the previous
cleft lip scar, can be used for lining vestibular deficits due to
its healthy blood supply.80
A micronostril is a very difficult problem to correct, and the
creation of this deformity during primary repair should be
recognized and avoided at all costs. If there is significant
constriction of the nostril, and loss of tissue along the nostril
sill, the nasal alae should be repositioned. One way to correct
this problem is by introducing new skin into the nasal sill with
a peri-alar nasolabial transposition flap. This can increase the
size of the nostril, reposition the medially displaced alar base
laterally, and increase the length of the sill, more accurately
matching the opposite side.
Lateral vestibular webbing is a common problem seen in
secondary rhinoplasty. A V–Y advancement will move the
cartilage–mucosal flap into a more anatomic position. This
deformity can be limited during the primary lip repair by
releasing the lateral nasal sidewall from the piriform and
advancing the alar base to its correct position.
Nasal dorsum
The nasal dorsal root is usually directed toward the cleft side,
resulting in tip deviation away from the cleft. Dorsal humps,
if present, can be addressed prior to harvesting cartilage grafts
from the septum, thereby assuring an adequate 1 cm dorsal
septum. The nasal bones are commonly thick and wide;
therefore, low-to-low lateral osteotomies in combination with
medial to lateral osteotomies superiorly can be performed to
the cleft rhinoplasty operation. Cartilage grafts reinforce the
structural support of the nose, allow for improved tip defini­
tion, and prevent wound contracture and collapse.
Alar base support is essential for attaining definitive repair
and relies on the skeletal foundation of the face. Varying
degrees of unilateral or bilateral maxillary hypoplasia are
typically present in the cleft patient. Bridging the alveolar
cleft with bone graft is often performed prior to eruption of
the canine teeth, between the ages of 9 and 11 years (Video
27.2
).4,75,76 This serves to support, augment, and reposition
the alar base. When inadequate support of the alar base is
encountered, secondary augmentation using bone, cartilage,
and alloplastic implants may be applied. In severe maxillary
hypoplasia, maxillary osteotomies and advancement can be
used to reconstruct the anterior–posterior dimensions of the
face. Advancement should be completed prior to definitive
rhinoplasty.69
Incision
Cleft rhinoplasty via an open approach allows for direct
visualization of nasal structures as well as anatomic recon­
struction of abnormal components. Transcolumellar and
infracartilagenous incisions are most frequently employed to
visualize the lower lateral cartilages as well as the nasal
septum. Care should be taken when elevating the columella
not to injure the medial crura of the lower lateral cartilages.
Superficial dissection should proceed in these areas to prevent
injuring these structures. Once the skin has been elevated, the
nasal structures can be addressed (Video 27.4
).
The septum
The nasal septum can be approached by separating the medial
crura of lower lateral cartilages. The caudal septum is identi­
fied, and a subperichondrial dissection can be started, ensur­
ing that the dissection is in the proper plane, validated by the
blue appearance of the cartilage. A portion of the septum is
harvested to obtain cartilage for grafting as well as to correct
deviation causing nasal airway obstruction. A 1 cm dorsal and
caudal strut should remain to avoid nasal collapse. Any dis­
placement of the caudal septum should be corrected at this
time by release and repositioning in the facial midline. In
clefts, there is often a bony septal/vomer spur that needs
removal to achieve a patent nasal airway.
The nasal tip
The nasal tip is buttressed using a columellar strut, placed
in between the medial crura and sutured in place (Fig.
27.21). The LLCs are advanced and secured to the strut using
mattress sutures, obtaining appropriate tip projection (Fig.
27.22).77 The columellar strut is placed behind the medial
crura, so it does not increase the columella width.78 When
the lower lateral cartilages are wide, the tip may benefit from
cephalic trimming. Despite this, projection maybe inadequate
on the cleft side, and batten grafting with onlay cartilage
grafts on the cleft-side LLC can provide better support and
shape.78 Tip defining sutures are placed to reconstitute the
dome into a more cephalic position (Figs. 27.22 & 27.23).
Onlay shield grafts can augment nasal tip definition,
and other grafts can camouflage other tip asymmetries
(Fig. 27.24).78
Fig. 27.21  Columellar struts involve the placement of a graft between the medial
crura and suturing the structures together using mattress sutures. This improves
nasal tip support and projection, columellar length, and nasolabial angle.

655
Cleft nasal deformity
positioned alae are dropped by excision of superior lip skin.
Skin from the cheek is advanced superiorly to mobilize the
inferiorly positioned alae into a more symmetric position.
Bilateral cleft nasal deformity
Components of the bilateral cleft nose often resemble those of
the unilateral deformity; however, there is often greater sym­
metry in the bilateral deformity (Figs. 27.26 & 27.27). The colu­
mella can be short or nearly absent with decreased soft tissue
between the nasal tip and vermilion border of the lip.81 The
degree of the columellar shortening is related to the extent of
prolabial development, cephalic nasal tip rotation, and cleft
severity. The lower lateral cartilages have an increased inter­
domal distance due to the aberrant muscular insertions,
resulting in decreased projection and tip definition.64 The
septum may be midline in incomplete clefts; however, in
complete as well as asymmetric bilateral clefts, the septum is
reposition the nasal root to the midline and narrow the nasal
base width.78 If this does not adequately narrow the dorsum,
a central segment can be removed creating an open roof
via paramedian osteotomies. The nasal bones can then be
in-fractured to achieve adequate narrowing. If this is done,
spreader grafts may be necessary to reconstruct the internal
nasal valve.
Nasal alae
Addressing the malpositioned nasal alae is often one of the
final stages of secondary rhinoplasty (Fig. 27.25). Often later­
ally and inferiorly malpositioned, the alae can potentially be
in any orientation. With a full-thickness peri-alar incision, the
alae can be brought into a symmetric position. The lateral ala
can be repositioned using a V–Y advancement along the alar
facial groove. The ala can be moved laterally with a lateral
skin flap transposition into the nasal floor. Superiorly
Fig. 27.22  Pre- (A–C) and postoperative images
(D–F) demonstrate improvement in this patient’s
dorsal aesthetic lines, small dorsal hump, septal
deviation, wide boxy tip, nasal tip projection, and
nostril shape/symmetry.
A
B
C
D
E
F

656
SECTION I
CHAPTER 27 • Secondary deformities of the cleft lip, palate, and nose
Fig. 27.23  Shield grafts allow modification of the nasal tip and infratip lobule.
Sheen shield graft
Support graft and
anatomic tip graft
Peek onlay graft
Guyuron modification
Fig. 27.24  This figure demonstrates another example
of common deformities seen with the unilateral cleft
nasal deformity. Most significant in this patient is the
absent nasal tip projection and wide dorsal aesthetic
lines (A, B), which have been improved by performing
an open rhinoplasty and osteotomies (C, D).
A
B

657
Conclusion
Fig. 27.25  This figure demonstrates hypertrophic scarring associated with bilateral
cleft lip repair. Acutely, this might benefit from steroid injections; however, in the
mature setting, treatment would involve excision and primary closure.
Fig. 27.26  The bilateral cleft nose deformity involves bilateral displacement and
distortion of the osseocartilaginous skeleton of the nose. The shortened columella
plays a prominent role in the bilateral deformity and must be lengthened in
subsequent procedures.
Web alar rim
Flaring alae
Wide nostril
Short columella
Obtuse angle
Dislocation and separation
C
D
Fig. 27.24, cont’d
often displaced and obstructing. A wide septum is not uncom­
mon and may present as nasal obstruction.
Bilateral deformities require repositioning both LLCs with
reshaping techniques and tip suture techniques. Removing
the collection of fibrofatty tissue once separating the LLCs
helps with tip definition. If the lower lateral cartilages are not
strong enough to allow for appropriate projection, a columel­
lar strut as well as onlay grafts can be beneficial.78 Onlay
grafts, or Sheen grafts, provide tip definition for bilateral
clefts with typical thick nasal skin. Again, the goal for the
bilateral deformity is to decrease the angle of divergence
between the domal points of the LLCs as well as provide a
strong columellar framework for tip projection.
The nasal dorsum is usually too wide. Symmetric low
to high osteotomies, or lateral low-to-low combined with
medial-to-lateral superior osteotomies, are done to narrow the
dorsum and create more appropriate dorsal aesthetic lines. A
cartilage graft along the nasal dorsum can be considered if
these osteotomies do not provide an adequate amount of
dorsal narrowing. However, elevating the nasal dorsum will
increase the amount of tip projection required – an already
challenging task in the bilateral cleft nasal deformity.
Conclusion
Treating patients with cleft lip and palate is a challenging task
and should be undertaken by teams specifically experienced

658
SECTION I
CHAPTER 27 • Secondary deformities of the cleft lip, palate, and nose
Fig. 27.27  Pre- (A–C) and postoperative images (D–F) after cleft rhinoplasty demonstrate correction of dorsal aesthetic lines and improved nasal tip projection and
symmetry.
A
B
C
D
E
F
4. Fisher DM, Sommerlad BC. Cleft lip, cleft palate, and
velopharyngeal insufficiency. Plast Reconstr Surg. 2011;128:342e–
360e. Fisher and Sommerlad provide a comprehensive overview of cleft lip
and palate repair in the 21st century. Beginning with a foundation in
pathological anatomy of the cleft lip and palate, the authors step through
surgical management of the cleft alveolus, lip, and palate. The article
details multiple techniques for unilateral cleft lip repair, discussing their
origins and aesthetic outcomes in comparison to others. Similar attention
is given to techniques of palatoplasty. A discussion of velopharyngeal
insufficiency, a measure of success for palate repair, guides the reader in
choosing an appropriate reconstructive technique. It is important to
consider the outcome most significant to the patient to select the best cleft
lip and palate repair.
5. Stewart TL, Fisher DM, Olson JL. Modified Von Langenbeck
cleft palate repair using an anterior triangular flap: decreased
incidence of anterior oronasal fistulas. Cleft Palate Craniofac J.
2009;46:299–304. Oronasal fistulas are a frequent complication during
primary cleft palate repair with a high rate of recurrence. The authors
present a modified Von Langenbeck technique resulting in a fistula rate
close to 0%. Their inclusion of an anterior triangular oromucosal flap
reflected posteriorly reduces tension for nasal side mucosal closure. At
4 to 8 weeks postoperatively, 0 of 182 patients who had a modified Von
Langenbeck had a suspected oronasal fistula. The modification to the
common Von Langenbeck technique described provides a substantial
reported reduction in oronasal fistula rates for closure of U-shaped
fistulas. Even with a growing array of successful techniques for secondary
Access the complete reference list online at

in this care. The best treatment for secondary deformities is
prevention, with an appropriately performed primary opera­
tion. Cleft team care ideally decreases the number of opera­
tions by successfully addressing deformities during the
original operation. Secondary cleft surgery is ideally mini­
mized with this approach. Secondary operations can be
technically more challenging and the postoperative outcomes
more unpredictable. Centers with dedicated teams, treating
patients with clefts on a daily basis, should improve patient
outcomes over time.

659
Conclusion
substandard treatment, poor care was certain for patients seeking medical
treatment outside multidisciplinary teams.
52. Furlow LT. Cleft palate repair by double opposing Z-plasty. Plast
Reconstr Surg. 1986;78:724–736. Furlow proposed a primary cleft palate
repair of two opposing Z-plasties across the primary defect. At a time
when cleft palate repairs achieved either normal speech production or
matched midfacial growth, the proposed opposing Z-plasty repair
simultaneously achieved improved speech by palatal muscle mobilization
and palatal lengthening to counteract midface hypoplasia. In a series of 22
patients, 90% achieved velopharyngeal sufficiency. Although evidence of
crossbite, a criterion for midface growth, was present in 11 of 19 patients,
only three had more-than-mild crossbites. The Furlow palatoplasty
technique has become a commonplace surgical technique for the primary
repair of the cleft palate.
59. McComb H. Primary correction of unilateral cleft lip nasal
deformity: a 10-year review. Plast Reconstr Surg. 1985;75:791–797. In
1985, McComb addressed a long-standing concept of postponing cleft
nasal deformity repair until adulthood and facial maturity. The author
presented a series of 10 patients now 10 years of age or greater who had
combined primary lip and nasal corrections. During primary lip repair,
the displaced alar cartilages were elevated to improve symmetry. After ten
years of growth, the patients did not present with worse nasal deformities
or interference to nasal growth. Additionally, they benefitted from
improved nasal appearance during childhood. The evidence showed that
surgical intervention of nasal structures did not grossly alter the growth
pattern. This study along with many others has helped shift cleft lip and
nasal care towards fewer procedures and improved quality of life for
pediatric patients.
72. Mulliken JB. Principles and techniques of bilateral complete cleft lip
repair. Plast Reconstr Surg. 1985;75:477–486. In the 1980s, evidence was
slowly building to begin cleft nasal repair as early as cleft lip repair.
Mulliken described a two-stage repair supporting early cleft nasal
deformity correction with nasal repositioning in the second procedure. The
author performed the two-stage repair for 15 pediatric patients, presenting
follow-up pictures ranging from 1 to 6 years postoperatively. Five
principles for successful bilateral cleft lip were discussed: (1) attention to
symmetry, (2) primary muscle continuity, (3) prolabial shape and size, (4)
central tubercle construction from lateral lip tissue, and (5) early
reconstruction of nasal tip and columella. The concepts presented in this
text were critical to gradually improving bilateral cleft care to meet
satisfactory outcomes of its simpler sibling, the unilateral cleft lip.
73. Cutting CB. Secondary cleft lip nasal reconstruction: state of the art.
Cleft Palate Craniofac J. 2000;37:538–541. Cutting provides a thorough
and step-wise review of procedures for secondary cleft lip nasal
reconstruction. The article discusses treatment for the skeletal base, nasal
dorsal bone and cartilage, nasal tip cartilage, the skin envelope, and the
bilateral cleft nose. By altering the skeletal base through a Le Fort I
advancement or prosthesis, the lower maxilla can be projected forward
without altering nasal dorsum projection. Cutting also describes several
techniques for altering nasal dorsal bone and cartilage through single
block maneuvers or spreader-strut grafts. The use of cartilage flaps
through either Potter or Dibbell’s techniques can be used to revise nasal
tip projection. The author introduces Tajima’s incision as a method to
improve the skin envelope of the patient. As the nose is a focal point for
facial aesthetics, techniques of secondary cleft nasal repair can provide
considerable relief for the patient.
fistulas, prevention during the primary cleft repair remains the
ideal outcome.
7. Noordhoff MS, Chen YR, Chen KT, et al. The surgical technique for
the complete unilateral cleft lip-nasal deformity. Operat Tech Plast
Reconstr Surg. 1995;2:167–174.
8. Fisher DM. Unilateral cleft lip repair: an anatomical subunit
approximation technique. Plast Reconstr Surg. 2005;116:61–71. Fisher
presents a technique for repair of unilateral cleft lip based on meticulous
adherence to anatomical subunits of the lip. Upon observation of
Noordhoff and Thomson’s techniques, this repair attempts to place the
majority of the scar line along the reconstructed cleft-side philtral column.
There is no rotation incision unlike Millard’s technique. A total of 144
unilateral cleft lip repairs using the new technique are presented. In the
subsequent discussion, major benefits of the technique are presented such
as an exact approximation of vertical height on the lateral lip element.
This new method of repair has steadily gained use among cleft lip/palate
surgeons since its conception.
13. Mulliken JB. Primary repair of bilateral cleft lip and nasal
deformity. Plast Reconstr Surg. 2001;108:181–194. Mulliken set out to
describe the advances in bilateral cleft lip repair as well as the movement
towards single-stage cleft repairs such as primary nasolabial repair.
Bilateral cleft surgical care has lagged behind advances in unilateral
cleft care; however, recent surgical advancements have improved bilateral
cleft lip and nasal repair to match or overtake unilateral cleft repair.
Mulliken describes in-detail the primary nasolabial repair of a bilateral
cleft lip and nasal deformity, followed by consideration for anatomical
variants such as asymmetric or incomplete bilateral clefts. Objective
measurements for weighing surgical revisions are also addressed with a
section dedicated to common revisions after primary cleft repair. Lastly,
the author comments on the distribution of care and structure of
multidisciplinary teams for cleft patients in a rapidly changing healthcare
environment.
14. Farkas LG. Anthropometry of the Head and Face in Medicine. New
York: Elsevier; 1981. In the 1980s, Farkas created the first comprehensive
atlas of craniofacial surface anthropometry. The atlas outlines a
measurement system that is meticulously applied to the identification and
layout of anthropometric landmarks on the head and face. The text was
immediately useful for anthropological studies but has grown in
application to plastic and oral/maxillofacial surgery. Its utility has come to
include even medical and clinical genetics. For plastic surgery, Farkas’s
measurements are critical in preoperative evaluation, surgical planning,
and postoperative assessment. This text continues to be the reference for
standard and objective measurements in craniofacial surgery.
17. Bardach J, Morris H, Olin W, et al. Late results of multidisciplinary
management of unilateral cleft lip and palate. Ann Plast Surg.
1984;12:235–242. This study describes the status of 45 pediatric patients
with history of unilateral cleft lip and palate treated by a multidisciplinary
cleft care team. At 14 to 22 years of age, the patients were evaluated for
completion of treatment by the comprehensive team, composed of plastic
surgery, orthodontics, and speech pathology. It was uncovered that only
16% of patients had completed treatment for all three specialties. Factors
including lack of patient cooperation, socioeconomic factors, and changing
treatment algorithms were cited as possible culprits for delayed treatment.
However, the most significant effect in the authors’ opinion was that a
multidisciplinary cleft palate team experienced significant delay in overall
treatment for patients. With a well-run and organized team providing

659.e1
References
References
1. American Cleft Palate – Craniofacial Association. Standards for Cleft
Palate and Craniofacial Teams. Available from:
cpf.org/team_care/standards/; 2016.
2. Stal S, Hollier LH. Correction of secondary cleft lip deformities.
Plast Reconstr Surg. 2002;109:1672–1681.
3. Mathes SJ. Plastic Surgery. 2nd ed. New York: Elsevier; 2006.
4. Fisher DM, Sommerlad BC. Cleft lip, cleft palate, and
velopharyngeal insufficiency. Plast Reconstr Surg. 2011;128:342e–
360e. Fisher and Sommerlad provide a comprehensive overview of cleft lip
and palate repair in the 21st century. Beginning with a foundation in
pathological anatomy of the cleft lip and palate, the authors step through
surgical management of the cleft alveolus, lip, and palate. The article
details multiple techniques for unilateral cleft lip repair, discussing their
origins and aesthetic outcomes in comparison to others. Similar attention
is given to techniques of palatoplasty. A discussion of velopharyngeal
insufficiency, a measure of success for palate repair, guides the reader in
choosing an appropriate reconstructive technique. It is important to
consider the outcome most significant to the patient to select the best cleft
lip and palate repair.
5. Stewart TL, Fisher DM, Olson JL. Modified Von Langenbeck cleft
palate repair using an anterior triangular flap: decreased incidence
of anterior oronasal fistulas. Cleft Palate Craniofac J. 2009;46:299–304.
Oronasal fistulas are a frequent complication during primary cleft palate
repair with a high rate of recurrence. The authors present a modified Von
Langenbeck technique resulting in a fistula rate close to 0%. Their
inclusion of an anterior triangular oromucosal flap reflected posteriorly
reduces tension for nasal side mucosal closure. At 4 to 8 weeks
postoperatively, 0 of 182 patients who had a modified Von Langenbeck had
a suspected oronasal fistula. The modification to the common Von
Langenbeck technique described provides a substantial reported reduction
in oronasal fistula rates for closure of U-shaped fistulas. Even with a
growing array of successful techniques for secondary fistulas, prevention
during the primary cleft repair remains the ideal outcome.
6. Byrd HS, El-Musa KA, Yazdani A. Definitive repair of the unilateral
cleft lip nasal deformity. Plast Reconstr Surg. 2007;120:1348–
1356.
7. Noordhoff MS, Chen YR, Chen KT, et al. The surgical technique for
the complete unilateral cleft lip-nasal deformity. Operat Tech Plast
Reconstr Surg. 1995;2:167–174.
8. Fisher DM. Unilateral cleft lip repair: an anatomical subunit
approximation technique. Plast Reconstr Surg. 2005;116:61–71. Fisher
presents a technique for repair of unilateral cleft lip based on meticulous
adherence to anatomical subunits of the lip. Upon observation of
Noordhoff and Thomson’s techniques, this repair attempts to place the
majority of the scar line along the reconstructed cleft-side philtral column.
There is no rotation incision unlike Millard’s technique. A total of 144
unilateral cleft lip repairs using the new technique are presented. In the
subsequent discussion, major benefits of the technique are presented such
as an exact approximation of vertical height on the lateral lip element.
This new method of repair has steadily gained use among cleft lip/palate
surgeons since its conception.
9. Tennison CW. The repair of the unilateral cleft lip by the stencil
method. Plast Reconstr Surg. 1952;9:115–120.
10. Manchester WM. The repair of the bilateral cleft lip and palate. Br J
Plast Surg. 1965;52:878–882.
11. Koshy JC, Ellsworth WA, Sharabi SE, et al. Bilateral cleft lip
revisions: the Abbe flap. Plast Reconstr Surg. 2010;126:
221–227.
12. Wu D, Song T, Li H, et al. An innovative cross-lip flap with a
musculomucosal pedicle based on the vascular network of the
lower lip. Plast Reconstr Surg. 2013;131:265–269.
13. Mulliken JB. Primary repair of bilateral cleft lip and nasal
deformity. Plast Reconstr Surg. 2001;108:181–194. Mulliken set out to
describe the advances in bilateral cleft lip repair as well as the movement
towards single-stage cleft repairs such as primary nasolabial repair.
Bilateral cleft surgical care has lagged behind advances in unilateral cleft
care; however, recent surgical advancements have improved bilateral cleft
lip and nasal repair to match or overtake unilateral cleft repair. Mulliken
describes in-detail the primary nasolabial repair of a bilateral cleft lip and
nasal deformity, followed by consideration for anatomical variants such as
asymmetric or incomplete bilateral clefts. Objective measurements for
weighing surgical revisions are also addressed with a section dedicated to
common revisions after primary cleft repair. Lastly, the author comments
on the distribution of care and structure of multidisciplinary teams for
cleft patients in a rapidly changing healthcare environment.
14. Farkas LG. Anthropometry of the Head and Face in Medicine. New
York: Elsevier; 1981. In the 1980s, Farkas created the first comprehensive
atlas of craniofacial surface anthropometry. The atlas outlines a
measurement system that is meticulously applied to the identification and
layout of anthropometric landmarks on the head and face. The text was
immediately useful for anthropological studies but has grown in
application to plastic and oral/maxillofacial surgery. Its utility has come to
include even medical and clinical genetics. For plastic surgery, Farkas’s
measurements are critical in preoperative evaluation, surgical planning,
and postoperative assessment. This text continues to be the reference for
standard and objective measurements in craniofacial surgery.
15. Nicolau PJ. The orbicularis oris muscle: a functional approach to its
repair in the cleft lip. Br J Plast Surg. 1983;36:141–153.
16. American Cleft Palate – Craniofacial Association. Parameters for the
evaluation and treatment of patients with cleft lip/palate or other
craniofacial anomalies. Cleft Palate Craniofac J. 1993;30(suppl 1):
S1–S16.
17. Bardach J, Morris H, Olin W, et al. Late results of multidisciplinary
management of unilateral cleft lip and palate. Ann Plast Surg.
1984;12:235–242. This study describes the status of 45 pediatric patients
with history of unilateral cleft lip and palate treated by a multidisciplinary
cleft care team. At 14 to 22 years of age, the patients were evaluated for
completion of treatment by the comprehensive team, composed of plastic
surgery, orthodontics, and speech pathology. It was uncovered that only
16% of patients had completed treatment for all three specialties. Factors
including lack of patient cooperation, socioeconomic factors, and changing
treatment algorithms were cited as possible culprits for delayed treatment.
However, the most significant effect in the authors’ opinion was that a
multidisciplinary cleft palate team experienced significant delay in overall
treatment for patients. With a well-run and organized team providing
substandard treatment, poor care was certain for patients seeking medical
treatment outside multidisciplinary teams.
18. Maeda K, Ojimi H, Utsugi R, et al. A T-shaped musculomucosal
buccal flap method for cleft palate surgery. Plast Reconstr Surg.
1987;79:888–896.
19. Senders CW, Sykes JM. Modifications of the Furlow palatoplasty
(six- and seven-flap palatoplasties). Arch Otolaryngol Head Neck
Surg. 1995;121:1101–1104.
20. Smith D, Vecchione L, Jiang S, et al. The Pittsburgh Fistula
Classification System: a standardized scheme for the description of
palatal fistulas. Cleft Palate Craniofac J. 2007;44:590–594.
21. Schultz RC. Management and timing of cleft palate fistula repair.
Plast Reconstr Surg. 1986;78:739–747.
22. Thaller SR. Staged repair of secondary cleft palate deformities. J
Craniofac Surg. 1995;6:375–381.
23. Assuncao AG. The design of tongue flaps for the closure of palatal
fistulas. Plast Reconstr Surg. 1993;91:806–810.
24. Batchelor AG, Palmer JH. A novel method of closing a palatal
fistula: the free fascial flap. Br J Plast Surg. 1990;43:359–361.
25. Berkman MD. Early non-surgical closure of postoperative palatal
fistulae. Plast Reconstr Surg. 1978;62:537–541.
26. Brusati R, Mannucci N. Repair of the cleft palate without lateral
release incisions: results concerning 124 cases. J Craniomaxillofac
Surg. 1994;22:138–143.
27. Chen HC, Ganos DL, Coessens BC, et al. Free forearm flap for
closure of difficult oronasal fistulas in cleft palate patients. Plast
Reconstr Surg. 1992;90:757–762.
28. Coghlan K, O’Regan B, Carter J. Tongue flap repair of oro-nasal
fistulae in cleft palate patients: a review of 20 patients. J
Craniomaxillofac Surg. 1989;17:255–259.
29. Cohen SR, Kalinowski J, LaRossa D. Cleft palate fistulas: a
multivariate statistical analysis of prevalence, etiology, and surgical
management. Plast Reconstr Surg. 1991;87:1041–1047.
30. Denny AD, Amm CA. Surgical technique for the correction of
postpalatoplasty fistulae of the hard palate. Plast Reconstr Surg.
2005;115:383–387.

659.e2
SECTION I
CHAPTER 27 • Secondary deformities of the cleft lip, palate, and nose
31. Eufinger H, Machtens E. Microsurgical tissue transfer for
rehabilitation of the patient with cleft lip and palate. Cleft Palate
Craniofac J. 2002;39:560–567.
32. Guerrero-Santos J, Altamirano JT. The use of lingual flaps in repair
of fistulas of the hard palate. Plast Reconstr Surg. 1966;38:123–128.
33. Kirschner RE, Cabiling DS, Slemp AE, et al. Repair of oronasal
fistulae with acellular dermal matrices. Plast Reconstr Surg.
2006;118:1431–1440.
34. Kirschner RE, LaRossa DD, Losee JE, et al. Repair of oronasal fistulae
using acellular dermal matrices: preclinical study and clinical case series.
Presented at the 60th Annual Meeting of the American Cleft
Palate-Craniofacial Association, Asheville (NC), April 10–13, 2003.
35. Krimmel M, Hoffmann J, Reinert S. Cleft palate fistula closure with
a mucosal prelaminated lateral upper arm flap. Plast Reconstr Surg.
2005;116:1870–1872.
36. Millard DR. Cleft Craft: The Evolution of its Surgery. 1st ed. Boston:
Little, Brown; 1976.
37. Ninkovic M, Hubli EH, Schwabegger A, et al. Free flap closure of
recurrent palatal fistula in the cleft lip and palate patient. J Craniofac
Surg. 1997;8:491–495.
38. Ohsumi N, Onizuka T, Ito Y. Use of a free conchal cartilage graft for
closure of a palatal fistula: an experimental study and clinical
application. Plast Reconstr Surg. 1993;91:433–440.
39. Rintala AE. Surgical closure of palatal fistulae: follow-up of 84
personally treated cases. Scand J Plast Reconstr Surg. 1980;14:
235–238.
40. Wilhelmi BJ, Appelt EA, Hill L, et al. Palatal fistulas: rare with the
two-flap palatoplasty repair. Plast Reconstr Surg. 2001;107:315–318.
41. Obermeyer P. Early closure of suture dehiscence after uranoplasty
by means of a conservative method. Dtsch Stomatol. 1967;17:168–173.
[in German].
42. Guerrero-Santos J, Fernandez JM. Further experience with tongue
flap in cleft palate repair. Cleft Palate J. 1973;10:192–202.
43. Ashtiani AK, Emami SA, Rasti M. Closure of complicated palatal
fistula with facial artery musculomucosal flap. Plast Reconstr Surg.
2005;116:381–388.
44. Levi B, Kasten SJ, Buchman SR. Utilization of the buccal fat pad
flap for congenital cleft palate repair. Plast Reconstr Surg.
2009;123:1018–1021.
45. Smith DM, Vecchione L, Jiang S, et al. Progress in palatoplasty:
strategies to eliminate fistulae. Presented at the 64th Annual Meeting
of the American Cleft Palate-Craniofacial Association, Broomfield
(CO), April 23–28, 2007.
46. Clark JM, Saffold SH, Israel JM. Decellularized dermal grafting in
cleft palate repair. Arch Facial Plast Surg. 2003;5:40–45.
47. Cole P, Horn TW, Thaller S. The use of decellularized dermal
grafting (AlloDerm) in persistent oronasal fistulas after tertiary cleft
palate repair. J Craniofac Surg. 2006;17:636–641.
48. Steele MH, Seagle MB. Palatal fistula repair using acellular dermal
matrix: the University of Florida experience. Ann Plast Surg.
2006;56:50–53.
49. Hanazawa Y, Itoh K, Mabashi T, et al. Closure of oroantral
communications using a pedicled buccal fat pad graft. J Oral
Maxillofac Surg. 1995;53:771–776.
50. Baumann A, Ewers R. Application of the buccal fat pad in oral
reconstruction. J Oral Maxillofac Surg. 2000;58:389–393.
51. Samman N, Cheung LK, Tideman H. The buccal fat pad in oral
reconstruction. Int J Oral Maxillofac Surg. 1993;22:2–6.
52. Furlow LT. Cleft palate repair by double opposing Z-plasty. Plast
Reconstr Surg. 1986;78:724–736. Furlow proposed a primary cleft palate
repair of two opposing Z-plasties across the primary defect. At a time
when cleft palate repairs achieved either normal speech production or
matched midfacial growth, the proposed opposing Z-plasty repair
simultaneously achieved improved speech by palatal muscle mobilization
and palatal lengthening to counteract midface hypoplasia. In a series of 22
patients, 90% achieved velopharyngeal sufficiency. Although evidence of
crossbite, a criterion for midface growth, was present in 11 of 19 patients,
only three had more-than-mild crossbites. The Furlow palatoplasty
technique has become a commonplace surgical technique for the primary
repair of the cleft palate.
53. Armour A, Fischbach S, Klaiman P, et al. Does velopharyngeal
closure pattern affect the success of pharyngeal flap
pharyngoplasty? Plast Reconstr Surg. 2005;115:45–52.
54. Orticochea M. Construction of a dynamic muscle sphincter in cleft
palates. Plast Reconstr Surg. 1968;41:323–327.
55. Coleman SR. Structural fat grafting: more than a permanent filler.
Plast Reconstr Surg. 2006;118:108S–120S.
56. Lau D, Oppenheimer AJ, Buchman SR, et al. Posterior pharyngeal
fat grafting for velopharyngeal insufficiency. Cleft Palate Craniofac J.
2013;50:51–58.
57. Blair V, Brown JB. Nasal abnormalities, fancied and real surgery.
Surg Gynecol Obstet. 1931;53:797.
58. Rifley W, Thaller SR. The residual cleft lip nasal deformity: an
anatomic approach. Clin Plast Surg. 1996;23:81–92.
59. McComb H. Primary correction of unilateral cleft lip nasal
deformity: a 10-year review. Plast Reconstr Surg. 1985;75:791–797. In
1985, McComb addressed a long-standing concept of postponing cleft
nasal deformity repair until adulthood and facial maturity. The author
presented a series of 10 patients now 10 years of age or greater who had
combined primary lip and nasal corrections. During primary lip repair,
the displaced alar cartilages were elevated to improve symmetry. After ten
years of growth, the patients did not present with worse nasal deformities
or interference to nasal growth. Additionally, they benefitted from
improved nasal appearance during childhood. The evidence showed that
surgical intervention of nasal structures did not grossly alter the growth
pattern. This study along with many others has helped shift cleft lip and
nasal care towards fewer procedures and improved quality of life for
pediatric patients.
60. McComb H. Primary repair of unilateral cleft lip nasal deformity.
Oper Tech Plast Reconstr Surg. 1995;2:200–205.
61. Li AQ, Sun YG, Wang GH, et al. Anatomy of the nasal cartilages of
the unilateral complete cleft lip nose. Plast Reconstr Surg.
2002;109:1835–1838.
62. Wolfe SA. A pastiche for the cleft lip nose. Plast Reconstr Surg.
2004;114:1–9.
63. Latham RA. The pathogenesis of the skeletal deformity associated
with unilateral cleft lip and palate. Cleft Palate J. 1969;6:404–414.
64. Fisher DM, Mann RJ. A model for the cleft lip nasal deformity. Plast
Reconstr Surg. 1998;101:1448–1456.
65. Bernstein L. Early submucous resection of nasal septal cartilage. A
pilot study in canine pups. Arch Otolaryngol. 1973;97:273–278.
66. McComb HK, Coghlan BA. Primary repair of the unilateral cleft lip
nose: completion of a longitudinal study. Cleft Palate Craniofac J.
1996;33:23–30.
67. 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.
68. Shih CW, Sykes JM. Correction of the cleft-lip nasal deformity.
Facial Plast Surg. 2002;18:253–262.
69. Byrd HS, Salomon J. Primary correction of the unilateral cleft nasal
deformity. Plast Reconstr Surg. 2000;106:1276–1286.
70. Kohout MP, Aljaro LM, 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.
71. Mulliken JB, Burvin R, Farkas LG. Repair of bilateral complete cleft
lip: Intraoperative nasolabial anthropometry. Plast Reconstr Surg.
2001;107:307–314.
72. Mulliken JB. Principles and techniques of bilateral complete cleft lip
repair. Plast Reconstr Surg. 1985;75:477–486. In the 1980s, evidence was
slowly building to begin cleft nasal repair as early as cleft lip repair.
Mulliken described a two-stage repair supporting early cleft nasal
deformity correction with nasal repositioning in the second procedure. The
author performed the two-stage repair for 15 pediatric patients, presenting
follow-up pictures ranging from 1 to 6 years postoperatively. Five
principles for successful bilateral cleft lip were discussed: (1) attention to
symmetry, (2) primary muscle continuity, (3) prolabial shape and size, (4)
central tubercle construction from lateral lip tissue, and (5) early
reconstruction of nasal tip and columella. The concepts presented in this
text were critical to gradually improving bilateral cleft care to meet
satisfactory outcomes of its simpler sibling, the unilateral cleft lip.

659.e3
References
73. Cutting CB. Secondary cleft lip nasal reconstruction: state of the art.
Cleft Palate Craniofac J. 2000;37:538–541. Cutting provides a thorough
and step-wise review of procedures for secondary cleft lip nasal
reconstruction. The article discusses treatment for the skeletal base, nasal
dorsal bone and cartilage, nasal tip cartilage, the skin envelope, and the
bilateral cleft nose. By altering the skeletal base through a Le Fort I
advancement or prosthesis, the lower maxilla can be projected forward
without altering nasal dorsum projection. Cutting also describes several
techniques for altering nasal dorsal bone and cartilage through single
block maneuvers or spreader-strut grafts. The use of cartilage flaps
through either Potter or Dibbell’s techniques can be used to revise nasal
tip projection. The author introduces Tajima’s incision as a method to
improve the skin envelope of the patient. As the nose is a focal point for
facial aesthetics, techniques of secondary cleft nasal repair can provide
considerable relief for the patient.
74. Stal S, Brown RH, Hiquera S, et al. Fifty years of the millard
rotation-advancement: looking back and moving forward. Plast
Reconstr Surg. 2009;123:1364–1377.
75. Boyne PJ, Sands NR. Secondary bone grafting of residual alveolar
and palatal clefts. J Oral Surg. 1972;30:87–92.
76. Kane AA, Pilgram TK, Moshiri M, et al. Long-term outcome of cleft
lip nasal reconstruction in childhood. Plast Reconstr Surg.
2000;105:1600–1608.
77. Guyuron B, Ghavami A, Wishnek SM. Components of the short
nostril. Plast Reconstr Surg. 2005;116:1517–1524.
78. Guyuron B. MOC-PS(SM) CME article: late cleft lip nasal deformity.
Plast Reconstr Surg. 2008;121(suppl):1–11.
79. Sykes JM, Jang YJ. Cleft lip rhinoplasty. Facial Plast Surg Clin North
Am. 2009;17:133–144.
80. Madorsky SJ, Wang TD. Unilateral cleft rhinoplasty: a review.
Otolaryngol Clin North Am. 1999;32:669–682.
81. Penfold C, Dominguez-Gonzalez S. Bilateral cleft lip and nose
repair. Br J Oral Maxillofac Surg. 2011;49:165–171.

SECTION I • Clefts
28 
Cleft and craniofacial orthognathic surgery
Jesse A. Goldstein and Stephen B. Baker
SYNOPSIS
■ Dentofacial deformities, in particular maxillary retrusion resulting in
class III malocclusion, are typical of the cleft lip/palate population. Of
patients in this group, 25–30% have midface retrusion severe enough
to require orthognathic surgery
■ Orthognathic surgery should ideally be performed after facial growth is
complete. If surgery is performed earlier, the likelihood is high that
additional (though possibly less complicated) surgery may be required
when the patient reaches skeletal maturity
■ Treatment should favor expansive movements (anterior and inferior
repositioning) to achieve class I occlusion rather than contractile
movements (superior and posterior repositioning) in order to minimize
premature aging.
Access the Historical Perspective section online at

Introduction
Orthognathic surgery is the term used to describe surgical
movement of the tooth-bearing segments of the maxilla and
mandible. Candidates for orthognathic surgery have dentofa­
cial deformities that cannot be adequately treated with orth­
odontic therapy alone. Children with cleft lip and palate as
well as certain craniofacial anomalies are especially prone to
developing malocclusion. Indeed, where approximately 2.5%
of the general population have occlusal discrepancies that
warrant surgical correction, 25–30% of patients who undergo
surgical correction of cleft lip and palate in infancy will have
severe enough midface retrusion to require orthognathic
surery.1 Maxillary hypoplasia resulting in class III malocclu­
sion is the typical deformity seen in patients with cleft and
craniofacial deformities, but class II malocclusion, anterior
open bites, occlusal cants, and many other dentofacial defor­
mities can also occur. Regardless of the etiology, patient
examination and treatment-planning principles remain the
same. The goal of orthognathic surgery, therefore, is to estab­
lish ideal dental occlusion with the jaws in a position that
optimizes facial form and function.
Basic science
Growth and development
Timing of orthognathic surgery in the pediatric patient is key
to good and predictable outcomes and is mediated by the
development and maturation of the craniofacial skeleton. The
foundation of maxillofacial growth relies on a complex
interplay between genetic processes and micro- and
macroenvironmental factors which must be understood to
plan orthognathic procedures on patients with clefts and
craniofacial disorders.
The osteogenesis of the maxillofacial skeleton occurs by
way of two well-understood processes: intramembranous
ossification and endochondral ossification. The cranial vault,
upper face, midface, and a majority of the mandible arise from
the former mechanism. Although there is a great amount of
variability between individuals and genders, skeletal matura­
tion generally progresses in a cranial-to-caudal direction with
the cranial vault reaching close to adult size in early adoles­
cence, followed closely by the upper face in the early teen
years, the maxilla in the mid-teens, and the mandible in the
late teen years (Fig. 28.1).3
Dental eruption patterns proceed in a similar stepwise
fashion, and the transition from mixed dentition (6–12 years
of age) to permanent dentition (12–20 years of age) mirrors
the maturation of the maxillofacial skeleton. Indeed, midface
and lower face development is, in part, mediated by the
budding deciduous and permanent dentition, providing
regional signals to the alveolus and stimulating bony deposi­
tion. During this period, an alteration of tooth position can,
in turn, alter the direction of growth of both the maxilla and

660.e1
Historical perspective
Historical perspective
The history of orthognathic surgery is complex, spanning
two centuries and two continents and featuring some of
the innovators of the field of plastic surgery. Advances in
mandibular surgery preceded maxillary surgery by over
50 years. Although the first mandibular osteotomy was
reported in 1846 by Hullihan, Blair and Kostecka together
published the first large series of cases aimed at address­
ing mandibulofacial disproportion. These early cases were
performed in a seated dental chair with simple ether sedation.
Blind osteotomies using transcutaneous application of a Gigli
saw to the mandibular ramus took no more than 15 minutes
to perform.
Much has changed since those early surgeries. Perhaps the
greatest impact to the field was brought about by Hugo
Obwegeser, an Austrian-born dentist who is credited with
modernizing the field of orthognathic surgery and introduc­
ing it to the United States. Amongst his credits, the sagittal
split osteotomy for mandibular advancement and the intraoral
approach to the osseus genioplasty clearly advanced lower-
jaw surgery. But it was not until 1965, when Obwegeser2
demonstrated that the maxilla could be completely mobilized
in one procedure and reliably and stably repositioned, that
modern orthognathic surgery gained widespread appeal.