Phẫu Thuật Tạo Hình Grabb & Smith’s (Bản Cập Nhật – Kevin C. Chung)

Amputated Ear
Replantation
Replantation of amputated ears has been reported, and some excellent results have been obtained. If the
replantation is successful, it yields superior aesthetic result compared with secondary reconstruction.
However, the vessels are small and failure is common. Reattaching large pieces of auricular tissue as
composite grafts is doomed to failure. The superficial temporal artery or posterior auricular artery must
be available for microvascular anastomosis to consider replantation.
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Banking of Ear Cartilage
Amputated cartilage can be banked under postauricular skin, TPF, or volar forearm. The success of
banking is inconsistent.
Mladick’s pocket principle 34 : Dermabrased amputated cartilage is reattached to the remaining
auricle, then buried underneath the retroauricular skin pocket. The graft is left in place for 3 weeks,
then at the second-stage surgery, cartilage is exposed with soft tissue attached on the cartilage, and
the skin is grafted to complete the reconstruction.
Baudet’s fenestration techniques 35 : Posterior skin of the amputated part is removed. Fenestration is
made in avulsed auricular cartilage to increase the vascular recipient area.
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Cauliflower Ear
A hematoma may result from trauma and frequently occurs in aggressive contact sports such as
wrestlers. Unless evacuated, the blood tends to become cartilaginous, resulting in the so-called
cauliflower ear. Once fully developed, the cauliflower ear requires surgical correction. Hematomas may
require repeated aspirations or an incision to fully evacuate. Bolster sutures to the auricle to eliminate the
space between the skin against the cartilage usually prevent reoccurrence.
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Questions
1. A 5-year-old boy is brought to your office by his parents regarding their concern for the
appearance of his right ear. He is an otherwise healthy boy. On examination, the superior helix of the
right ear is abnormally adherent to the temporal skin. Gentle retraction on the helix allows it to be
pulled out to a normal position. What is the most likely diagnosis?
a. Stahl ear
b. Cup ear
c. Lop ear
d. Cryptotia
e. Helix-antihelix cartilage adhesion
2. Which of the following statements is correct regarding the normal size, dimension, and size of the
auricle?
a. The long axis tilt is consistently within 1° to 5° in most individuals.
b. Ear height varies between 5.5 and 6.6 cm in adults.
c. The auricle is located approximately one ear width posterior to the lateral orbital rim.
d. Maximum projection from the mastoid to the helix occurs superiorly.
e. Ear width is usually less than one-third the height.
3. A child presents with vestige lobule with conchal bowl, acoustic meatus, and tragus. Based on
current classification of microtia, what type of microtia does the child have?
a. Anotia type
b. Lobule type
c. Small concha type
d. Concha type
e. Atypical type
4. The lobule of the auricle arises from the:
a. first branchial arch
b. first branchial cleft
c. second branchial arch
d. second branchial cleft
e. third branchial arch
5. Which one of the following statements is correct regarding complications from autogenous
microtia reconstruction?
a. The risk of chest wall deformities can be reduced by harvesting rib cartilage without
perichondrium.
b. The procedure carries a relatively high risk of postoperative infection.
c. Hematoma formation is rare and is usually self-limiting.
d. The constructed auricle will remain a static size as the child continues to grow.
e. If the skin loss develops over the construct, a further surgical procedure will be almost always
required.
1. Answer: d. Cryptotia is characterized by an abnormal adherence of the superior helix to the
temporal skin, with varying degree of severity. Gentle retraction on the helix allows it to be pulled
out to a normal position. If the child is less than 6 months of age, ear molding may correct the
deformity according to Matsuo. Over 6 month of age, surgery is currently the 
 only option for
treatment. Cryptotia may be associated with helix-antihelix adhesion, which causes helix deformity.
2. Answer: b. The auricle is posteriorly tilted 15° to 20° (Farkas). The study shows that too upright or
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anteriorly inclined auricle to be aesthetically less pleasing. The vertical height of the auricle varies
between 5.5 and 6.6 cm, and the width is usually 0.5 to 0.55 of the height (Tolleth). Projection is
greatest inferiorly (∼2 cm) and smallest superiorly.
3. Answer: d. The currently favored classification system for microtia is based on the surgical
correction of the deformity, proposed by Nagata. Anotia: absence of auricular tissue. Lobule type:
Vestige ear with lobule, without concha, acoustic meatus, and tragus. Concha type: Vestige ear with
lobule, concha, acoustic meatus, and tragus. Small concha type: Vestige lobule with small indentation
of concha (need lobule-type construction). Atypical microtia: Cases do not fall into previous
categories. In concha-type microtia, 3D framework or may not accompany tragus portion.
4. Answer: c. The auricle develops from six auricular hillocks that form around the first branchial
groove or cleft. Hillocks one to three (anteriorly) are formed from the first branchial arch
(mandibular arch) and form the tragus, root of helix, and superior helix. Hillocks four to six
(posterior) are formed from the second arch (hyoid arch) and form the posterior helix, antihelix, and
lobule. The lobule is the last part of the auricle to develop. The external acoustic meatus develops
from the first branchial groove, and the middle ear and eustachian tube develop from the first
pharyngeal pouch. Part of the first arch cartilage (Meckel cartilage) ossifies and forms the malleus
and incus of the middle ear. The ventral portion of Meckel cartilage disappears, and the mandible
develops around it. Part of the second arch cartilage (Reichert cartilage) ossifies to form the stapes of
the middle ear and styloid process. The portion between the styloid process and the hyoid bone
forms the stylohyoid ligament. The third branchial arch forms the greater cornu and the lower part of
the hyoid bones. The fourth to sixth arch cartilages fuse to become the laryngeal cartilages.
5. Answer: a. The most common late complication in auricular reconstruction is chest wall deformity,
which occurs in approximately two-thirds of cases. Earlier surgery can cause more frequent chest
wall deformity. Because the perichondrium is the scaffold of cartilage regeneration, harvesting rib
cartilage without perichondrium will reduce the degree and frequency of chest wall deformity,
although even with this method, the chest deformity may occur. The most significant early
complications are skin loss, infection, and hematoma, all of which are rare; however, each must be
identified early and managed accordingly to prevent extrusion of cartilage framework. The area of
skin loss should be debrided and only reconstructed if the area is greater than 1 cm. Smaller defects
can be managed by dressing. This is the advantage of autogenous rib cartilage framework over
Medpor framework. Even a small defect in Medpor framework may be difficult to close
spontaneously. Hematomas are not self-limiting and must be drained immediately to minimize the
risk of skin loss. New constructed auricle commonly remains the same size over time (48%) but may
increase in size as the child grows, (42%) in Brent’s series.
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References
Tolleth, H. Artistic anatomy, dimensions, and proportions of the external ear. Clin Plast Surg. 1978;5(3):337-345. A must-read to understand the
aesthetics of the auricle for both cosmetic and reconstructive surgery.
Yamada, A. Anthropometric study of ear position and its clinical application to the total external ear reconstruction. Bull Osaka Med Coll.
2009;55(2):81-89.
Yanai, A, Tange, I, Bandoh, Y, et al. Our method of correcting cryptotia. Plast Reconstr Surg. 1988;82(6):965-972.
Tanzer, RC. The constricted ear (cup and lop) ear. Plast Reconstr Surg. 1975;55:406.The concept of constricted ear is described in detail.
Kon, M, van Wijk, MP. T-bar reconstruction of constricted ears and a new classification. J Plast Reconstr Aesthet Surg. 2014;67(3):358-361.
Park, C. A new corrective method for the Tanzer’s group IIB constricted ear: helical expansion using a free-floating costal cartilage. Plast
Reconstr Surg. 2009;123(4):1209-1219.
Nagata, S. Alternative surgical methods of treatment for the constricted ear. Clin Plast Surg. 2002;29(2):301-315.
Matsuo, K, Hirose, T, Tomono, T, et al. Nonsurgical correction of congenital auricular deformities in the early neonate: a preliminary report.
Plast Reconstr Surg. 1984;73(1):38-51.
Nagata, S. The modification stages involved in the total reconstruction of the auricle: part I. The modification in the grafting of the three-
dimensional costal cartilage framework (3-D frame) for the lobule type microtia. Plast Reconstr Surg 1994;93:221-230.
Nagata, S. The modification stages involved in the total reconstruction of the auricle: part II. The modification in the grafting of the three-
dimensional costal cartilage framework (3-D frame) for the concha type microtia. Plast Reconstr Surg. 1994;93:231-242.
Nagata, S. The modification stages involved in the total reconstruction of the auricle: part III. The modification in the grafting of the three-
dimensional costal cartilage framework (3-D frame) for the small concha type microtia. Plast Reconstr Surg. 1994;93:243-253.References 9 to
11 cover the original Nagata techniques in detail.
Wilkes, GH, Wong, JW, Guilfoyle, R. Microtia reconstruction. Plast Reconstr Surg. 2014;134(3):464e-479e.
Jahrsdoerfer, RA, Yeakley, JW, Aguilar, EA, et al. Grading system for the selection of patients with congenital aural atresia. Am J Otol.
1992;13(1):6-12.
Roman, S, Nicollas, R, Triglia, JM. Practice guidelines for bone anchored hearing aids in children. Eur Ann Otorhinolaryngol Head Neck Dis.
2011;128:253-258.
Yamada, A, Ueda, K, Yorozuya-Shibasaki, R. External ear reconstruction in hemifacial microsomia. J Craniofac Surg. 2009;20(suppl):1787-
1793.
Park, C. Balanced auricular reconstruction in dystopic microtia with the presence of the external auditory canal. Plast Reconstr Surg.
2002;109(5):1489-1500.
Oyama, A, Sasaki, S, William, M, et al. Salvage of cartilage framework exposure in microtia reconstruction using a mastoid fascial flap. J Plast
Reconstr Aesthet Surg. 2008;61(suppl 1):S110-S113.
Tanzer, RC. Total reconstruction of the external ear. Plast Reconstr Surg. 1959;23:1.Landmark article of the start of modern autogenous ear
reconstruction.
Brent, B. Technical advances in ear reconstruction with autogenous rib cartilage grafts: personal experience with 1200 cases. Plast Reconstr
Surg. 1999;104(2):319-334.The artistry of Dr. Brent is well described in this article.
Yamada, A. Autologous rib microtia construction: Nagata technique. Facial Plast Surg Clin North Am. 2018;26(1):41-55.
Kawanabe, Y, Nagata, S. A new method of costal cartilage harvest for total auricular reconstruction: part I. Avoidance and prevention of
intraoperative and postoperative complications and problems. Plast Reconstr Surg. 2006;117(6):2011-2018.
Kawanabe , Nagata, S. A new method of costal cartilage harvest for total auricular reconstruction: part II. Evaluation and analysis of the
regenerated costal cartilage. Plast Reconstr Surg. 2007;119(1):308-315.
Yamada, A, Imai, K, Fujimoto, T, et al. New training method of creating ear framework by using precise copy of costal cartilage. J Craniofac
Surg. 2009;20(3):899-902.
Reinisch, JF, Lewin, S. Ear reconstruction using a porous polyethylene framework and temporoparietal fascia flap. Facial Plast Surg.
2009;25:181-189.
Brent, B, Byrd, HS. Secondary ear reconstruction with cartilage graft covered by axial, random, and free flaps of temporoparietal fascia. Plast
Reconstr Surg. 1983;72:141.Decent secondary ear reconstruction was started from here.
Nuri, T, Ueda, K, Yamada, A. Application of free serratus anterior fascial flap for reconstruction of ear deformity due to hemifacial
microsomia: a report of two cases. Microsurgery. 2017;37(5):436-441.
Firmin, F. Ear reconstruction in cases of typical microtia: personal experience based on 352 microtic ear corrections. Scand J Plast Reconstr Surg
Hand Surg. 1998;32(1):35-47.
Nagata, S. Modification of the stages in total reconstruction of the auricle: part IV. Ear elevation for the constructed auricle. Plast Reconstr
Surg. 1995;93(2):254-266. 
 Nagata-type ear elevation technique is described.
Antia, NH, Buch, VI. Chondrocutaneous advancement flap for the marginal defect of the ear. Plast Reconstr Surg. 1967;39(5):472-477.
Talmi, YP, Wolf, M, Horowitz, Z, et al. “Second look” at auricular reconstruction with a postauricular island flap: “flip-flop flap”. Plast
Reconstr Surg. 2002;109(2):713-715.
Pearl, RA, Sabbagh, W. Reconstruction following traumatic partial amputation of the ear. Plast Reconstr Surg. 2011;127(2):621-629.
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Brent, B. Earlobe construction with an auriculo-mastoid flap. Plast Reconstr Surg. 1976;57(3):389-391.
Bastidas, N, Jacobs, JM, Thorne, CH. Ear lobule reconstruction using nasal septal cartilage. Plast Reconstr Surg. 2013;131(4):760-762.
Mladick, RA, Horton, CE, Adamson, JE, Cohen, BI. The pocket principle: a new technique for the reattachment of a severed ear part. Plast
Reconstr Surg. 1971;48(3):219-223.
Baudet, J, Tramond, P, Goumain, A. A new technic for the reimplantation of a completely severed auricle. Ann Chir Plast. 1972;17(1):67-72.
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C H A P T E R 3 4
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Management of Velopharyngeal Dysfunction
Asra Hashmi and Amanda A. Gosman
Key Points
In the normal palate, the levator veli palatini muscle rests horizontally within the middle third of the
soft palate and functions to elevate and lengthen the velum posteriorly, against the posterior
pharyngeal wall.
Velopharyngeal dysfunction may be from velopharyngeal insufficiency, mislearning, and/or
incompetence.
Velopharyngeal competence results from apposition of the velum against the posterior pharyngeal
wall and from motion of the lateral pharyngeal wall that causes closure of the velopharyngeal port.
Sphincter pharyngoplasty entails 90° transposition of the palatopharyngeal muscles from the
posterior tonsillar pillar to effectively obliterate the lateral ports of the velopharyngeal mechanism
and decrease the diameter of the central port while still maintaining the centric opening.
A sagittal or circular velopharyngeal closure pattern with good lateral wall motion is treated with a
pharyngeal flap, which elevates the flap from the posterior pharyngeal wall after inset into the soft
palate.
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Velopharyngeal Function
Normal Function
Velopharyngeal competence results from apposition of the velum against the posterior pharyngeal wall
and from motion of the lateral pharyngeal wall that causes closure of the velopharyngeal port.
Inadequate closure of the velopharyngeal port, broadly termed velopharyngeal dysfunction (VPD),
results in hypernasality and nasal air emission, which can be due to a variety of causes. In patients with a
history of cleft palate surgery, the incidence of VPD is approximately 20% to 30 %. 1,2 It is important to
accurately identify the etiology of VPD so that a customized treatment plan may be generated specific to
patient’s needs. A multidisciplinary team approach is essential in formulation of a plan. There are several
radiologic, invasive, and noninvasive diagnostic options that can be utilized to correctly identify the
problem. These include perceptual speech evaluation, nasal endoscopy, videofluoroscopy (VFS),
magnetic resonance imaging (MRI), and nasometry. Once the cause is identified, surgical and nonsurgical
treatment options can be employed to ensure best outcome for the patient.
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Velopharyngeal Dysfunction
VPD is typically classified into three main subtypes based on its cause.
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Velopharyngeal Incompetence
Velopharyngeal incompetence is physiologic VPD that results from neurologic or neuromuscular
dysfunction, for example, myotonic dystrophy or amyotrophic lateral sclerosis. Acquired causes of
velopharyngeal incompetence include head injury or stroke. It is important to note that even though most
patients with velocardiofacial syndrome (also known as 22q11.2 deletion syndrome, caused by a
microdeletion from chromosome 22 at the q11.2 band) present with cleft palate (overt or submucous
cleft), a subset of patients with this syndrome may have velopharyngeal incompetence resulting from
neuromuscular dysfunction, highlighting the necessity of correctly identifying the underlying problem. 3,4
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Velopharyngeal Mislearning
Mislearning results from inadequate closure of the velopharyngeal port, due to learned disarticulation.
Hearing loss or deafness can also contribute to velopharyngeal mislearning. Speech therapy is indicated
as there is no anatomic or physiologic cause associated with this type of VPD. 5
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Velopharyngeal Insufficiency
Velopharyngeal insufficiency (VPI) results from structural defect causing inadequate closure of
velopharyngeal port. Patients with history of cleft palate surgery may have a short velum resulting in
anatomic defect associated with VPD. Patients with submucous cleft (triad of bifid uvula, notch in the
hard palate, and zona pellucida) may also present with VPI. Other anatomic causes include deep pharynx
resulting from cervical spine or cranial base abnormalities, for example, in patients with Klippel-Feil
syndrome. 5
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Anatomy and Physiology
Muscles of the Velopharynx
There are six muscles that play an important role in the velopharyngeal closure and speech production.
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Levator Veli Palatini
This is the most important muscle in the soft palate, comprising middle 40% of the velum. Normally, the
levator veli palatini (LVP) takes origin from the petrous part of the temporal bone and the Eustachian
tube and decussates with the contralateral levator muscle forming a horizontally oriented sling in the soft
palate. It is innervated by the pharyngeal plexus from glossopharyngeal nerve (CN IX) and vagus nerve
(CN X) and functions to lengthen the velum and elevates it in the posterior direction. In patients with
cleft palate, this muscle is oriented in an anterior-posterior direction (instead of normal horizontal
orientation) and patients with submucous cleft tend to have diastasis of the LVP muscle (also known as
zona pellucida) resulting in VPI in 15% of the cases.
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Superior Pharyngeal Constrictor
This is a broad paired muscle that takes origin from the medial pterygoid and pterygomandibular raphe
and inserts onto the median pharyngeal raphe. Contraction of this muscle results in anterior and medial
movement of the posterior pharyngeal wall resulting in closure of the velopharyngeal port.
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Palatoglossus and Palatopharyngeus Muscles
These muscles lie in the anterior and posterior tonsillar pillars, respectively, and serve to depress the soft
palate. Some studies suggest that these muscles provide fine motor control of velar position during
speech, by acting as an antagonist to LVP muscle. 6
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Tensor Veli Palatini
Tensor veli palatini (TVP) originates from the Eustachian tube and from the scaphoid fossa of the
sphenoid bone, at the base of the medial pterygoid plate. Its tendon winds around the pterygoid hamulus
and then inserts into the palatal aponeurosis by joining the muscle of the opposite side in the anterior 25%
of the soft palate. As the name suggests, its action is to tense the soft palate, in addition to opening the
Eustachian tube.
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Musculus Uvulae
Musculus uvulae is the only intrinsic muscle of the soft palate and takes origin from the palatal
aponeurosis. It likely contributes to the VP closure by providing bulk to it, by acting as a space occupying
structure and a velar extensor. Studies suggest that anatomic reconstruction of musculus uvulae muscle
may improve VP port closure and assists in the production of plosive sounds. 7
All velopharyngeal muscles are innervated by the pharyngeal plexus, which receives contributions from
CN IX, CN X), and accessory nerve (CN XI). The only exception is the TVP muscle, which is innervated
by the mandibular branch of trigeminal nerve (CN V).
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Physiology
Velopharyngeal mechanism separates oral cavity from nasal cavity for the purpose of speech and
swallowing. During closure of velopharyngeal port, soft palate moves in a superior-posterior direction,
the posterior pharyngeal wall moves anteriorly, and the lateral pharyngeal wall moves medially to
achieve closure of the port. In some patients, the anterior motion of posterior pharyngeal wall forms a
distinct bulge called the Passavant ridge. Velopharyngeal valve is typically closed for oral sounds (most
consonants and vowels) and is open for nasal sounds (m, n, ng).
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Closure Patterns
Croft et al. examined the velopharyngeal closure pattern on VFS and endoscopy and identified five
closure patterns in normal and abnormal subjects 8 (Figure 34.1). Most common closure patterns in
normal subjects are coronal patterns, which are caused by posterior movement of velum with less
contribution from the lateral pharyngeal wall. Sagittal closure is predominantly attributed to medial
movement of the lateral pharyngeal wall. Circular closure is seen when there is good motion of velum as
well as lateral pharyngeal wall. Sometimes a Passavant ridge is also seen to contribute to this closure
pattern, resulting in circular Passavant ridge closure pattern. Bowtie closure is seen primarily with
movement of velum and posterior pharyngeal wall causing poor or minimal motion of the lateral
pharyngeal wall. Jordan et al. used three-dimensional MRI to examine the closure pattern of patients with
VPI and concluded that closure pattern also varied by sex with females demonstrating more circular
closure patterns and males demonstrating more coronal closure patterns. Their study also suggested that
length of the velum and thickness of adenoids may have the greatest impact on closure patterns. 9
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FIGURE 34.1 Velopharyngeal closure patterns.
(Adapted with permission from Croft, CB, Schprintzen, RJ, Rakoff, SJ. Patterns of velopharyngeal valving in normal and cleft palate subjects: a multi-view
videofluoroscopic and nasoendoscopic study. Laryngoscope. 1981;91:265-271.)
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Diagnosis
History and Physical Examination
Obtaining a detailed history in multidisciplinary setting is important. Information should be obtained
regarding feeding, swallowing difficulties, nasal regurgitation, hearing difficulties, and previous surgical
procedures including surgery for cleft lip and palate repair, tonsillectomy, and adenoidectomy. History
should also be obtained regarding any genetic testing for possible 22q11.2 deletion syndrome. If there is
any concern for sleep apnea, then polysomnography may be obtained to evaluate the severity of sleep
apnea and to identify whether it is central or obstructive in nature. Physical examination should assess
dentition, occlusion, soft palate length, movement during phonation, and presence of fistula. Patients
with clefts of only primary palate (especially those involving the alveolus) may have dental
abnormalities, which can also cause speech disturbances because of interference with tongue movement.
Jaw malocclusion may also be a major contributor to speech problems. Patients with history of cleft palate
repair often present with class III malocclusion, where tongue tip is positioned anterior to the maxillary
alveolar ridge. This can affect production of lingual-alveolar sounds (t,d,n,s,z) and even bilabial sounds
(p,b,m). 10 The information obtained from history and physical examination should be appraised with
speech evaluation to make a decision regarding the need for surgical intervention versus further
diagnostic testing. For example, if a patient with a history of submucous cleft presents with absence of
speech disturbances, then surgical intervention may not be necessary. On the other hand, if oral
examination reveals normal anatomy despite speech abnormality, then diagnostic tests should be
obtained to further investigate the cause of VPD.
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Perceptual Speech Evaluation
A formal perceptual speech evaluation by a speech and language pathologist (SLP) is perhaps the most
important modality in the evaluation of a patient with VPD. During speech evaluation, a skilled SLP
examines the speech sample of a patient during normal conversation and with provocative sentences, by
having the patient repeat some sentences with pressure-sensitive consonants (e.g., Buy bobby a puppy). The
main components of speech assessed include language (receptive and expressive), nasal air emission,
resonance (hyponasality or hypernasality), articulation, voice (hoarseness, high or low pitch), and
intelligibility. It is important to familiarize oneself with the terminology commonly used while assessing
a patient with VPD (Table 34.1). Some commonly used speech evaluation assessment tools are McWilliam
Scale and the Pittsburgh Weighted Speech Scale. The Pittsburgh scale rates five aspects of speech
including nasality, phonation, articulation, nasal air emission, and facial grimace. By assigning points to
each component, an overall score is reached which can be used as an objective assessment of speech.
CAPS-A (cleft audit protocol for speech—augmented) and UPS (universal parameters for reporting
speech outcomes) are some other protocols that are utilized in speech assessment. Even though several
protocols and speech assessment tools are available, the ear of the listener is considered to be the
standard, and use of a set of standard sentences that isolates each pressure consonant is recommended. 11
The typical speech sample of a patient with VPD often reveals poor intelligibility, hypernasality, nasal
substitution and glottal compensations, and weak pressure consonants.
TABLE 34.1
Commonly Used Terminologies for Perceptual Speech Evaluation
Intelligibility: Measure of comprehensibility of speech
Nasal emission: Escape of air through the nose during consonant production
Resonance: Relationship between sound reverberating in the oral versus nasal cavities, most perceptible during vowel production. In
hypernasality, too much sound resonates in the nasal cavity usually seen with velopharyngeal insufficiency or fistula. In hyponasality, not
enough sound resonates through nasal cavity, such as with the common cold.
Consonant: A speech sound produced by complete or partial closure of vocal cords (as apposed to vowels)
Fricatives: Consonants produced by forcing air through a narrow channel, such as (f) and (s)
Sibilant: A subset of fricatives where one still is forcing air through a narrow channel but, in addition, the tongue is curled lengthwise to
direct the air over the edge of the teeth. English (s), (z), and (ʃ) are examples of sibilants
Plosives (or stop consonants): Consonant sounds that are formed by completely stopping airflow, such as (p) (lips), (t) (teeth), and (k)
(back of the tongue)
Compensatory articulation: Articulation errors seen in velopharyngeal dysfunction patients resulting from an effort to compensate for
increased pressure and airflow during speech
Nasal substitution: Replacement of oral sounds (p), (b), (t), and (d) for nasal sounds (m) and (n)
Sibilant distortion: Incorrect production of (s) and (z) sounds resulting from incorrect tongue placement
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Speech Videofluoroscopy
A static lateral radiographic view obtained during quiet breathing and production of sound can give
information regarding palatal length, tonsil, and adenoids, as well as cranial base angle. Earliest reports
of radiographic assessment of velopharyngeal mechanism appeared in 1909. 12 The concept of speech VFS
was introduced nearly five decades ago by Skolnick, 13 which is a collaborative test between speech
therapist and radiologist that provides information regarding lateral and posterior wall of the pharynx as
well as length and movement of the soft palate based on sagittal and lateral radiographic views. Usually
barium is instilled through the nose to coat the posterior pharyngeal wall and nasal surface of the velum.
This highlights the velopharyngeal sphincter mechanism during its assessment. Subsequently speech
sample is obtained by an SLP as the patient repeats some standardized words and sentences. Information
can be obtained regarding velopharyngeal gap size, palatal length and stretch, pharyngeal depth, as well
as the size of tonsil and adenoids. Depending upon patients’ level of cooperation, this test can be
performed as early as 2 or 3 years of age. VFS seems to be better tolerated by patients compared with
nasopharyngoscopy (NPS), as it requires less level of cooperation from patients. Another advantage is
that some objective assessment of the velopharyngeal gap can also be obtained with this test, as opposed
to an NPS which does not allow any objective measurement. VFS does expose the patient to radiation,
which is a downside of this test.
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Nasopharyngoscopy
NPS directly visualizes the location of the defect and movement of velum during phonation. The test
begins by instillation of local anesthetic and decongestant into the nose to increase patient comfort. A
well-lubricated scope is then passed into the nose and views of velum, posterior and lateral pharynx, and
velopharyngeal gap can be obtained while the scope sits in the middle meatus. This test is usually
performed by the surgeon, and/or otorhinolaryngologist in collaboration with a trained SLP. An
assessment of velopharyngeal closure pattern is obtained while the patient is asked to repeat
standardized speech sample consisting of oral and nasal consonants and vowels. NPS is especially useful
for small and asymmetric gaps, submucous cleft, and VPD persisting post pharyngeal flap. The patient
ideally should be at least 4 to 5 years of age for this test, although it may be possible to successfully
perform NPS on a very mature three-year-old patient. 12
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Magnetic Resonance Imaging
There has been a recent surge of interest in the utility of MRI in the assessment of patients with VPD. 14,15
MRI allows morphologic assessment of velopharyngeal anatomy, especially the levator muscle. It also
provides information regarding the vascular anatomy of the neck which is especially important in
patients with 22q11.2 deletion syndrome who may have medialized internal carotid arteries. 16 The
advantage of MRI over NPS and VFS is that it is noninvasive and avoids the exposure to ionizing
radiation. A major hurdle is the poor image quality, when images are obtained during active phonation,
as children are usually only able to sustain phonation for 15 to 20 seconds, which limits the image
acquisition time. 15 A three-dimensional dynamic, fast MRI obtained in oblique coronal plane has been
proposed to provide better assessment and more precise measurements for patients with VPD. 14
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Nasometry
Warren and colleagues popularized the concept of nasometry, which allows measurement of oral and
nasal air pressure and flow and calculation of velopharyngeal port size. 17 In the original design, a
pressure transducer was placed in one of the nares and mouth and air flow meter was placed in the other
nares. Quantitative measurement of oral and nasal pressure and airflow was made which was
subsequently used to estimate velopharyngeal port size. 18 Warren in his study in 1985 suggested the use
of the word hamper for assessment of speech because this word has a nasal-plosive blend/mp/. An orifice
of >10 mm2 has been shown to correlate with hypernasality. 19
Nasalance (defined as the ratio of nasal sound energy divided by sum of oral and nasal sound) is the
acoustic index of nasality that is primarily measured during this assessment. As the patient reads (or
repeats) a standardized speech passage, computer-based analysis is done and a nasalance score is
calculated (range 0%–100%). Even though a variety of cut-off values for normal versus abnormal are used
(depending upon the speech stimulus), in general a higher score suggests more nasality. Nasometry is
one of the few tools that indirectly provide objective assessment of velopharyngeal mechanism but the
criticism to its use is its oversimplification of the VPD mechanism and its inability to predict the location
of gap size. 20
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Surgical Management
If a patient is deemed a surgical candidate, then the choice of surgical procedure should be individualized
and based on a host of factors. The goal of all surgical procedures is to decrease the area of
velopharyngeal port and reduce nasal air escape during phonation. Size of the defect, closure pattern, and
lateral wall motion are the three most important anatomic factors that should be considered in selecting
an optimal surgical procedure for the treatment of VPI. Figure 34.2 shows an algorithm for choosing the
suitable surgical procedure for the treatment of VPI. It is important to keep in mind that functional
repairs (see description later) yield a more anatomic local tissue rearrangement of structures in the soft
palate, without adding tissue from neighboring areas (e.g., in pharyngeal flap, sphincter pharyngoplasty,
or posterior wall augmentation procedures). Hence, functional repairs inherently have less risk of
obstructive sleep apnea (OSA) and should be attempted whenever possible to reduce the risk of long-
term airway complications as the patient ages.
FIGURE 34.2 An algorithm for choosing the optimal surgical procedure for the treatment of velopharyngeal insufficiency.
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Functional Palatal Lengthening
Functional palatal lengthening can be achieved by Furlow palatoplasty, or intervelar veloplasty. The
main anatomic objective of these procedures is to achieve lengthening of the soft palate with
retropositioning of levator muscle in the velum and closure of a small gap in the VP port. Sommerlad
popularized the concept of radical intervelar veloplasty, described as complete dissection of the velar
muscle complex off the nasal and oral mucosal layer in the soft palate as far laterally as the hamulus,
where tensor tendon is visualized and divided medial to the hamulus. The muscle is then divided from
the posterior border of the hard palate and then retropositioned and repaired in at least middle third of
the velum (or more posterior to it). 21 Sommerlad prospectively studied pre- and postoperative speech
outcomes in 129 patients with previous cleft palate repair, anterior insertion of levator muscle, and
symptoms of VPI, who underwent palatal repairs by radical intervelar veloplasty technique and noted
significant improvement in hypernasality, nasal air emission, and velopharyngeal gap closure. 22
Sommerlad et al. advocated that radical intervelar veloplasty should be considered as the first-line option
in patients with VPI (especially those with levator knee or point of maximum velar excursion on lateral
VFS more anteriorly located). However, if the palate is scarred and immobile, then rerepair is unlikely to
improve symptoms of VPI and should not be attempted.
The more commonly performed palatal lengthening procedure was introduced by Leonard Furlow in
1986. 23 This procedure entails creation of two double opposing Z-plasties of oral and nasal mucosa with
attached levator muscle. Lengthening is achieved by the concept of Z-plasty and reorientation of the
levator muscle to a more anatomic posterior position, which is achieved by its double opposition
technique. A recent study retrospectively analyzed the anatomic and speech changes of 29 patients with
VPI in the setting of previous cleft palate repair or submucous cleft and confirmed that Furlow resulted in
significant velar elongation, increased acuity of the genu angle, and retropositioning of the levator sling.
In their study, Furlow resulted in improvement of the resting genu angle which was noted to be 10° more
acute than preoperative assessment. Additionally, speech improvement and the absence of need for
reoperation were most consistently associated with tightening of the genu angle. 24
The advantage of functional repair (over more obstructive or nonanatomic surgical procedures for VPI) is
that it reduces the long-term distortion of the airway which places patients at a lower risk for OSA, as
they grow older and/or gain weight. This reduced risk of OSA is at the cost of up to 30% risk of failure (or
incomplete resolution of VPI), which may be a risk worth taking to avoid long-term morbidity of OSA.
21,22,25 However, it is important to counsel patients about the possibility of failure and need for a more
obstructive procedure in future if functional repair is selected for the management of VPI. Other methods
of palatal lengthening include interpositional buccal flap and V-Y pushback palatoplasty.
Next category of surgical procedures aims to physically decrease the area of velopharyngeal port and
resulting nasal air escape and hypernasality. These procedures include pharyngeal flap, sphincter
pharyngoplasty, or posterior wall augmentation.
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Posterior Pharyngeal Flap
Pharyngeal flap was first described by Schoenborn in 1876. This is a rectangular-shaped myomucosal
flap, raised from the posterior pharyngeal wall and insetted into the velum. 26 This flap converts a large
velopharyngeal port into two small lateral ports by creating a midline obstruction between oral and nasal
cavities (Figure 34.3). Even though Schoenborn’s original design for pharyngeal flap was inferiorly based,
various modifications of posterior pharyngeal flap have been described in the literature. In general,
inferiorly based flaps are considered easier to attach, and superiorly based flap have the advantage of
giving a larger flap; however, data are not conclusive on superiority of one flap design over another. 27
Some of the demerits of inferiorly based flap include limitation of its length and possible inferiorly
directed pull onto the velum, which is in contradiction to the goals of effective velopharyngeal closure
mechanism. Even though theoretically decreasing the port size by virtue of flap attachment into the
velum should decrease the symptoms of VPI secondary to all types of ports, pharyngeal flap is best suited
for patients with a sagittal or circularly oriented closure patterns. Additionally, adequate lateral wall
motion is also important for the success of the flap. 28 Other factors that may play a role in the success of
pharyngeal flap are flap width and level of attachment. Flaps that are too wide may be counterproductive
by causing symptoms of hyponasality and sleep apnea; on the other hand, flaps that are not wide enough
may be ineffective. Width of the flap is generally driven by surgeon experience and supplemented by
preoperative diagnostic workup and information gained by videoflouroscopy and/or nasal endoscopy
regarding port size, orientation, and lateral pharyngeal wall motion. 29 Pharyngeal flap is ideally inserted
at the level of C1 to allow adequate elevation of the velum.
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FIGURE 34.3 Pharyngeal flap.
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Sphincter Pharyngoplasty
Sphincter pharyngoplasty procedure was introduced by Hynes in 1950, who described the use of bilateral
salpingopharyngeus muscles for decreasing the dimension of velopharyngeal port. 30 Since the first
description, several modifications of this procedure have been reported in literature. 31,32 Currently, the
most commonly used design of this procedure entails 90° transposition of the superiorly based
palatopharyngeal muscles from the posterior tonsillar pillars to the posterior pharyngeal wall (Figure
34.4). It is best suited for a large posterior gap with coronal, circular, or bowtie closure pattern and/or
poor lateral wall motion. An advantage of sphincter pharyngoplasty procedure is the ability to adjust the
port size postoperatively, if needed by tightening or loosening the muscles.
FIGURE 34.4 Sphincter pharyngoplasty.
There is not enough evidence to suggest the superiority of sphincter pharyngoplasty or pharyngeal flap
for the treatment of VPI. 33,34 Large multicenter, randomized, blinded, and prospective studies are
required to determine the optimal procedure for VPI secondary to each type, size, and port orientation.
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Posterior Pharyngeal Wall Augmentation
Various autologous and alloplastic materials have been reported in the literature with a goal to reduce the
size of velopharyngeal port. Some of the synthetic materials used for posterior wall augmentation include
silicone, Teflon, proplast, etc. 35 Fat, fascia, cartilage, dermis, and rolled posterior pharyngeal flap are
some of the autologous materials that have been used for posterior wall augmentation. 36,37 Infection,
resorption, extrusion, migration, and unreliability of the results are some of the reported complications
and criticisms of posterior wall augmentation procedures. Another risk associated with posterior wall
augmentation with fat is its enlargement with weight gain and the consequent risk of OSA, at which
point the grafted fat may be difficult to remove. 36,37
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Complications
Complications after surgery for VPI include OSA, bleeding, dehiscence, airway obstruction, injury to
anomalous internal carotid artery, and persistent hypernasality, hyponasality, or reoperation. In a 10-year
review of perioperative complications following pharyngeal flap surgery, overall complication rate was
reported to be 6%. 38 Another series reported a 38% incidence of symptoms of upper airway obstruction
acutely after pharyngeal flap surgery, but persistent symptoms beyond five months was only seen in 6%
of the patients. 39 Witt et al. reported a 13% incidence of OSA following sphincter pharyngoplasty. 40
Symptoms of OSA can vary from snoring without any airway obstruction to obstruction with
desaturation, hence randomized controlled studies comparing complications secondary to various
surgical interventions utilized in the management of VPI patients are needed.
Another study on salvage of failed pharyngoplasties reported equivalent rates of failure with pharyngeal
flap (20%) and sphincter pharyngoplasty (16%) and successful denasalization of speech following
revision surgery. 41
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Special Considerations
Velopharyngeal Dysfunction and Chromosomal Syndromes
Many chromosomal syndromes are associated with VPD. Among the syndromic presentations, 22q11.2
deletion syndrome (sometimes referred to as velocardiofacial or Shprintzen syndrome) is known to be the
most common syndrome associated with cleft palate and VPI. These patients commonly have submucous
cleft; however, they may have a higher risk of associated VPD compared with patients with submucous
cleft in the absence of 22q11.2 deletion syndrome. This higher risk is thought to be multifactorial, and the
most commonly reported factor is neuromuscular dysfunction of the muscles of velopharyngeal
mechanism. Other contributing factors include increase in the width of space between velum and
posterior pharyngeal wall, asymmetric displacement of lateral pharyngeal wall, reduced adenoid size,
and intellectual disabilities. Hence, vigilance during interpretation of diagnostic tests such as VFS and
nasal endoscopy becomes even more important in the setting of syndromic presentation, so that the
accurate diagnosis can be made and precise prognosis of improvement with surgical intervention can be
established. During nasal endoscopy, attention should also be paid to the presence of pulsations in the
posterior or lateral pharyngeal wall which may suggest medialization of internal carotid arteries. Medial
displacement of internal carotid arteries can be frequently seen in patients with 22q11.2 deletion or
Kabuki syndrome. 42 Shprintzen and colleagues in their study of magnetic resonance angiography for
patients with 22q11.2 deletion syndrome noted anomalies in vertebral, carotid arteries or both, in all
patients with 22q11.2 deletion syndrome, highlighting the importance of obtaining imaging prior to the
performance of pharyngeal flap surgery in this patient population. 43 Ross and colleagues also studied the
risk of medially displaced carotid arteries in this patient population and concluded that a routine
superiorly based pharyngoplasty may be safe in 52%, whereas in others it should be custom designed
based on the individual anatomy of the patient. 44 Another concern for 22q11.2 deletion and other
syndromes with a developmental delay is that these patients may have intellectual disability, making it
more challenging for them to relearn and correct some of the articulation errors with speech therapy after
surgery. For this reason, in this patient population, physical management of VPI by utilizing pharyngeal
flap or sphincter pharyngoplasty may be warranted so that less reliance is placed on patient’s ability to
relearn, to control airflow, and to use their tongue, lips, and jaw correctly to make sounds, after surgery.
Other syndromes that may present with VPD include Opitz G/BBB syndrome, Kabuki syndrome, and
Jacobsen syndrome.
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Submucous Cleft
Controversy exists regarding the timing and optimal surgical intervention for submucous cleft (defined
as having varying degrees of phenotypic expressions of zona pellucida, notched hard palate, and bifid
uvula). In a study of 353 patients with submucous cleft, only 49.8 % were noted to have VPD, with the
rest of the patients demonstrating complete or marginal VPD. 45 Hence, it is reasonable to wait on the
surgical intervention till the patient is old enough to provide an adequate speech sample for perceptual
speech evaluation. If the patient is deemed a surgical candidate, then depending on the size of the defect,
it maybe reasonable to start with palatoplasty (either intervelar veloplasty or Furlow repair). Sommerlad
and colleagues performed a systemic review of 26 studies analyzing surgical management of VPI in
patients with submucous cleft. Speech outcomes were compared following sphincter pharyngoplasty,
pharyngeal flap, and palatal reconstruction (Furlow palatoplasty, intravelar veloplasty, and palatal
pushback), and little evidence was found to support one intervention over another. 46
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Tonsillectomy and Velopharyngeal Insufficiency
The effect of tonsils on VPI is complex. VPI can be associated with superior pole tonsillar hypertrophy,
especially when the upper pole extends above the level of the velum, into the lateral port and displaces
the palatopharyngeus muscle anteriorly. On the contrary and more importantly, VPI can worsen after
tonsillectomy, and hence these cases should be carefully evaluated with NPS prior to any surgical
intervention. If tonsillectomy is indicated, then it may be best to perform it prior to the performance of
surgical intervention for VPI, and after tonsillectomy, velopharyngeal closure should be carefully
reevaluated. Additionally, during tonsillectomy procedure, the posterior tonsillar pillar should be
protected, in case sphincter pharyngoplasty is indicated (which uses palatopharyngeus muscle from the
posterior tonsillar pillar). 47
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Nonsurgical Management
Palatal prosthesis is indicated for patients who have contraindications to surgery or for patients with poor
prognosis of improvement with surgery. 48,49 These devices are typically made in coordination with a
prosthodontist and a speech pathologist. The device fits along the hard palate and has a posterior
extension that pushes the soft palate upwards in a patient with hypomobile velopharyngeal mechanism
for closure of the port.
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Prognosis and Outcomes
Based on a systemic review with a goal to reach consensus in the literature regarding surgical
management of VPD, 71% of patients attained normal resonance and 65% attained normal nasal emission.
Additionally, no difference was noted in terms of speech outcomes, sleep apnea, or need for further
surgery, for patients treated with pharyngeal flap, sphincter pharyngoplasty, palatoplasty, and posterior
pharyngeal wall augmentation. 50 Once the patient undergoes surgery, speech therapy becomes important
in the postoperative period again so that the child can relearn and correct the articulation errors with
biofeedback and speech therapy.
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Questions
1. 1. A six-year-old boy is brought to the cleft clinic for a follow-up visit. The patient had intravelar
veloplasty at 10 months of age. On perceptual speech evaluation, he was noted to have hypernasality
and nasal emission. On physical examination, no fistula was noted. Nasal endoscopy showed a large
coronal gap with poor lateral wall motion. Which of the following is the most appropriate
management?
a. Continued speech therapy
b. Furlow palatoplasty
c. Sphincter pharyngoplasty
d. Posterior pharyngeal flap
e. Palatal lift prosthesis
2. A six-year-old patient with a history of bilateral complete cleft palate repair in infancy undergoes
sphincter pharyngoplasty for her VPI. Which of the following muscles was primarily incorporated
during pharyngoplasty for this patient?
a. Tensor veli palatini
b. LVP
c. Palatopharyngeus muscle
d. Palatoglossus muscle
e. Musculus uvulae
3. A four-year-old patient, adopted from China, is diagnosed with submucous cleft palate. He is
noted to have a notch of the posterior hard palate and a bifid uvula. Which of the following is an
additional feature that he is likely to have?
a. Incomplete cleft lip
b. Cleft of the anterior alveolus
c. Medialized internal carotid arteries
d. Zona pellucida
e. Syndactyly
4. A six-year-old boy presented to the multidisciplinary craniofacial team for evaluation of his VPI.
He is noted to have Tetralogy of Fallot, submucous cleft palate, learning disabilities, and
hypocalcemia. On perceptual speech evaluation, he is noted to have hypernasal speech with nasal air
escape. The most likely diagnosis is
a. Apert syndrome
b. Van der Woude syndrome (VWS)
c. 22q11.2 deletion syndrome
d. Nager syndrome
e. Stickler syndrome
5. A 5-year-old patient with a history of repaired cleft palate at 12 months of age presents to the clinic
for evaluation of her VPD. She is noted to have poor intelligibility, hypernasality, and weak pressure
consonants on perceptual speech evaluation. She undergoes nasal endoscopy which reveals a
moderate to large sized, sagittally oriented gap with good lateral wall motion and limited palatal
motion because of scarring. The patient has no contraindications for surgery. Which of the following
is the most appropriate treatment for this patient?
a. Intervelar veloplasty
b. Superiorly based pharyngeal flap
c. Posterior wall augmentation with fat
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d. Palatal obturator
e. Sphincter pharyngoplasty with transposition of salpingopharyngeus muscle
1. Answer: c. The patient described has VPI because of inadequate closure of the velopharyngeal
port. The first step in the evaluation of VPD is perceptual speech assessment, which is usually
performed around 3 or 4 years of age. Any nasal emission and/or hypernasality with speech are
indicative of inadequate approximation of velum against the posterior pharyngeal wall.
Nasoendoscopy provides qualitative assessment of the closure pattern and port size and can be
performed at 4 to 5 years of age. Surgical treatment is based on the severity of speech discrepancy
and the location and size of port closure defect. A large posterior gap with coronal, circular, or
bowtie closure pattern and poor lateral wall motion should be treated with a sphincter
pharyngoplasty, where palatopharyngeal muscles in bilateral posterior tonsillar pillars are elevated
and rotated through 90°. The flaps are overlapped and sutured to each other and to the posterior
pharyngeal wall.
Continued speech therapy will not correct hypernasality associated with a large coronal defect.
Furlow palatoplasty with its Z-plasty design provides palatal lengthening and is well suited for the
management of VPD in patients with unrepaired submucous cleft and with repaired overt clefts.
Although it can be done, it is not the best technique for patients in whom the levator muscles are not
sagittally oriented, as transposition would disrupt anatomically normal previously reconstructed
levator sling. Posterior pharyngeal flap requires raising a myomucosal rectangular flap from the
posterior pharyngeal wall and insetting it in the velum. It can be superiorly or posteriorly based and
is best suited for sagittal or circular closure patterns. Palatal prosthesis is indicated in cases of failed
speech therapy, when surgery is contraindicated.
2. Answer: c. Sphincter pharyngoplasty involves 90° transposition of the palatopharyngeus muscle
from the posterior tonsillar pillar and is best suited for a moderate to large coronally oriented gap.
TVP originates from the Eustachian tube and the scaphoid fossa of the sphenoid bone, and inserts
into the palatal aponeurosis by joining the muscle of the opposite side in the anterior 25% of the soft
palate. Its action is to tense the soft palate, in addition to opening the Eustachian tube. LVP takes
origin from the petrous part of the temporal bone and decussates with the contralateral levator
muscle forming a horizontally oriented sling in the soft palate. It functions to lengthen the velum and
elevates it in the posterior direction. Palatoglossus muscles lie in the anterior tonsillar pillars, where
the palatopharyngeus muscle is found in the posterior tonsillar pillar. They both serve to depress the
soft palate. Musculus uvulae is the only intrinsic muscle of the soft palate and takes origin from the
palatal aponeurosis. It likely contributes to the VP closure by providing bulk to it, by acting as a
space-occupying structure and a velar extensor. Among the muscles listed, palatopharyngeus muscle
is the only muscle that is used in sphincter pharyngoplasty procedure.
3. Answer: d. Patients with submucous cleft tend of have varying degrees of notched hard palate,
bifid uvula, and diastasis of the LVP muscle (also known as zona pellucida). This results in VPI in
15% of the cases. Incomplete cleft lip, cleft of the anterior alveolus, medialized internal carotid
arteries, and syndactyly are not standard features of a submucous cleft.
4. Answer: c. 22q11.2 deletion syndrome (commonly known as, DiGeorge syndrome, Shprintzen
syndrome, velocardiofacial syndrome) is a disorder caused by the deletion mutation on chromosome
22. The typical features of this syndrome include cleft palate, heart defects, recurrent infections,
unique facial characteristics, feeding problems, kidney abnormalities, hypoparathyroidism (resulting
in hypocalcemia), hearing loss, developmental delay, and learning disabilities. Apert syndrome is an
autosomal dominant syndrome characterized by bicoronal synostosis with complex syndactyly,
midfacial hypoplasia, exorbitism, etc. Patients with Apert syndrome can have cleft palate and 70%
have decreased IQ but they typically do not have hypocalcemia and cardiac abnormalities. VWS is an
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autosomal dominant syndrome characterized by lower lip pits, cleft lip with or without cleft palate,
or cleft palate alone. Patients with VWS can have congenital heart problems but do not tend to have
hypocalcemia. Patients with Nager acrofacial dysostosis are distinguished by radial-sided limb
abnormalities, malar hypoplasia, micrognathia, cleft palate, coloboma, and ear abnormalities. These
patients have many other anomalies including renal and cardiac anomalies but do not typically have
hypocalcemia. Stickler syndrome is another autosomal dominant syndrome with features of cleft
palate, Pierre Robin sequence, ocular malformations, hearing loss, and arthropathies. Patients with
Stickler syndrome can have mitral valve prolapse but hypocalcemia is not a typical feature of this
syndrome.
5. Answer: b. In a superiorly based pharyngeal flap, a rectangular-shaped myomucosal flap, based
superiorly, is raised from the posterior pharyngeal wall and insetted into the velum. This flap
converts a large velopharyngeal port into two small lateral ports by creating a midline obstruction
between oral and nasal cavities, and it is best suited for moderate to large sized gap that is a
sagittally oriented gap with good lateral wall. This patient has a surgical history of cleft palate repair
and now has limited palatal motion with scarring, so palatal rerepair with intervelar veloplasty is not
likely to help his VPI in the presence of a moderate to large sized gap. Posterior wall augmentation
with fat grafting will likely not resolve the VPI secondary to a moderate to large sized gap.
Additionally, fat grafting to posterior pharynx carries the risk of increase in size (and resulting
obstructive sleep apnea) if the patient gains weight. This patient has no contraindications to surgery
so palatal obturator is not the best long-term solution for his VPI. Sphincter pharyngoplasty uses
palatopharyngeus muscles and is best suited for a coronally oriented moderate to large sized gap.
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Lau, D, Oppenheimer, AJ, Buchman, SR, et al. Posterior pharyngeal fat grafting for velopharyngeal insufficiency. Cleft Palate Craniofac J.
2013;50(1):51-58.
Lypka, M, Bidros, R, Rizvi, M, et al. Posterior pharyngeal augmentation in the treatment of velopharyngeal insufficiency: a 40-year
experience. Ann Plast Surg. 2010;65(1):48-51.
Hofer, SO, Dhar, BK, Robinson, PH, et al. A 10-year review of perioperative complications in pharyngeal flap surgery. Plast Reconstr Surg.
2002;110(6):1393-1397.In a 10-year review of perioperative complications following pharyngeal flap surgery, the overall complication rate was reported
to be 6%.
Lesavoy, MA1, Borud, LJ, Thorson, T, et al. Upper airway obstruction after pharyngeal flap surgery. Ann Plast Surg. 1996;36(1):26-30.
Witt, PD, Marsh, JL, Muntz, HR, et al. Acute obstructive sleep apnea as a complication of sphincter pharyngoplasty. Cleft Palate Craniofac J.
1996;33(3):183-189.
Witt, PD, Myckatyn, T, Marsh, JL. Salvaging the failed pharyngoplasty: intervention outcome. Cleft Palate Craniofac J. 1998;35(5):447-453.A
study on salvage of failed pharyngoplasties reported equivalent rates of failure with pharyngeal flap (20%) and sphincter pharyngoplasty (16%) and
successful denasalization of speech following revision surgery.
Ysunza, A, Pamplona, MC, Molina, F, Hernández, A. Surgical planning for restoring velopharyngeal function in velocardiofacial syndrome.
Int J Pediatr Otorhinolaryngol. 2009;73(11):1572-1575.
Mitnick, RJ, Bello, JA, Golding-Kushner, KJ, et al. The use of magnetic resonance angiography prior to pharyngeal flap surgery in patients
with velocardiofacial syndrome. Plast Reconstr Surg. 1996;97:908-919.
Ross, DA, Witzel, MA, Armstrong, DC, Thompson, HG. Is pharyngoplasty a risk in velocardiofacial syndrome? An assessment of medially
displaced carotid arteries. Plast Reconstr Surg. 1996;98:1182-1190.
Heng, Y, Chunli, G, Bing, S, et al. Velopharyngeal closure pattern and speech performance among submucous cleft palate patients. Hua Xi
Kou Qiang Yi Xue Za Zhi. 2017;35(3):296-300.In a study of 353 patients with submucous cleft, only 49.8% were noted to have velopharyngeal
dysfunction, with the rest of the patients demonstrating complete or marginal VPD.
Gilleard, O, Sell, D, Ghanem, A, et al. Submucous cleft palate: a systematic review of surgical management based on perceptual and
instrumental analysis. Cleft Palate Craniofac J. 2014;51(6):686-695.The authors performed a systemic review of 26 studies analyzing surgical
management of velopharyngeal insufficiency in patients with submucous cleft. Speech outcomes were compared following sphincter pharyngoplasty,
pharyngeal flap, and palatal reconstruction (Furlow palatoplasty, intravelar veloplasty, and palatal ‘pushback’) and little evidence was found to support
one intervention over another.
Paulson, LM, Macarthur, CJ, Beaulieu, KB, et al. Speech outcomes after tonsillectomy in patients with known velopharyngeal insufficiency.
Int J Otolaryngol. 2012;2012:912767.
Raj, N, Raj, V, Aeran, H. Interim palatal lift prosthesis as a constituent of multidisciplinary approach in the treatment of velopharyngeal
incompetence. J Adv Prosthodont. 2012;4:243-247.
Aboloyoun, A, Ghorab, S, Farooq, MU. Palatal lifting prosthesis and velopharyngeal insufficiency: preliminary report. Acta Med Acad.
2013;42:55-60.
de Blacam, C, Smith, S, Orr, D. Surgery for velopharyngeal dysfunction: a systematic review of interventions and outcomes. Cleft Palate
Craniofac J. 2018;55(3):405-422.In a systemic review regarding surgical management of velopharyngeal dysfunction, 70.7% of patients attained
normal resonance and 65.3% attained normal nasal emission. Additionally, no difference was noted in terms of speech outcomes, sleep apnea, or need
for further surgery for patients treated with pharyngeal flap, sphincter pharyngoplasty, palatoplasty, and posterior pharyngeal wall augmentation.
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C H A P T E R 3 5
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Craniofacial Tumors and Conditions
James P. Bradley and Justine C. Lee
Key Points
Fibrous dysplasia, a benign, spongy bone disorder, may be monostotic (single area of bone
involvement) or polyostotic (multiple involved bony areas) and treated with operative recontour
burring or with partial resection and bone graft reconstruction.
Parry-Romberg syndrome, a progressive, acquired atrophy of the soft (skin/fat) and hard tissues
(cartilage/bone) of the face requires, depending on severity of the case, fat grafting, free adipofascial
flap, or even bony reconstruction.
Treacher Collins syndrome, a congenital bilateral deficiency of orbitozygomatic, mandibular, and ear
regions (Tessier no. 6, 7, and 8 clefts), may require staged reconstruction with bone grafts/eyelid
switch flaps for orbital reconstruction, total ear reconstruction for microtia, and mandibular ramus
distraction or orthognathic surgery for mandibular abnormalities.
Neurofibromatosis of the periphery (NF-1) or centrally (NF-2) is an autosomal dominant disorder
that causes (usually) benign growths within nerves and may require resection and the sacrificing of
involved nerves.
Pierre Robin sequence, a congenital anomaly with a triad of manifestations: micrognathia,
glossoptosis, and upper airway obstruction, may require early operative intervention in severe cases
with glossopexy or mandibular distraction to avoid a tracheostomy.
Moebius syndrome is characterized by bilateral absence of cranial nerves VI and VII resulting in
mask facies and inability to lateral deviate the eye and may be treated with functional muscle
transfers to give oral commissure animation.
Dermoid cysts are benign inclusion cysts typically located in the lateral supraorbital rim; but central
lesions require imaging to rule out intracranial extension that would require an intra- and
extracranial approach for resection.
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Fibrous Dysplasia
Definition and Clinical Findings
Fibrous dysplasia is a benign anomaly of the bone characterized by replacement of normal bone with
fibro-osseous tissue. It is the most common craniofacial tumor encountered by plastic surgeons. 1 Fibrous
dysplasia ranges from mild to severe and presents with monostotic or polyostotic distribution. 2,3 Skull
involvement occurs in 27% of monostotic patients and up to 50% of polyostotic patients. Clinical
manifestations of fibrous dysplasia depend on the location, duration, and the extent of disease.
Craniofacial fibrous dysplasia most commonly begins in childhood then progresses through adolescence.
It was previously thought that the disease progression halted after childhood, but this is not universally
true; the disease frequently continues well into adulthood. 2 Anatomic location of the monostotic disease
in the craniofacial skeleton most often affects the frontal and sphenoid bones and the maxilla 3,4 (Figure
35.1). Signs and symptoms of craniofacial disease are diverse and may include swelling of the affected
side (most commonly), facial pain, headaches, cranial nerve (CN) palsies, anosmia, deafness, blindness,
malocclusion with tooth displacement, diplopia, proptosis, and orbital dystopia. 3,5
FIGURE 35.1 Fibrous dysplasia limited to zygomatic maxillary complex (monostotic). A. Preoperative right oblique image. B.
3D model depicting fibrous dysplasia mass (arrow). C. Postoperative right oblique image after limited resection, cranial bone
graft to orbital rim/arch, and alveolar recontouring. (Courtesy of J.P. Bradley.)
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