Cleft Palate Repair and Velopharyngeal Dysfunction€¦ · for velopharyngeal insufficiency. Mirror image Z-plasty incisions are made across the cleft on the opposing oral and nasal

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TECHNIQUES OF CLEFT PALATE REPAIRKey points in the evolution of cleft palate repair

are listed in Table 1.1–3 Early techniques focused on hard palate closure, culminating in von Langen-beck recognizing the benefit of a subperiosteal dis-section.4 Veau and subsequently Kriens recognized the importance of soft palate reconstruction.5 The current techniques in cleft palate repair share the goals of (1) separation of the oral and nasal com-ponents without fistula; (2) sufficient velar length; and (3) functional transverse orientation of the levator muscle sling. The timing of cleft palate repair varies among centers but is typically as late as possible to minimize the potential negative effect on facial growth, yet early enough to provide a func-tional velum during the critical period of speech sound acquisition. With these considerations, the most common time is around the first year of life. Some authors have advocated a two-stage repair

with early soft palate closure with use of an anterior obturator to allow delayed hard palate closure in an attempt to minimize facial growth restriction.6,7 This technique has become less common because of the increased risk of oronasal fistula and worse speech results than with single-stage techniques8,9 ( Reference 8 Level of Evidence: Risk, III).

von Langenbeck PalatoplastyThe von Langenbeck palatoplasty combines

incisions along the medial cleft margins with lat-eral relaxing incisions (Fig. 1).2,10 Elevation of mucoperiosteum produces bipedicled flaps that are approximated for oral side closure. Muscle

Disclosure: The authors have no financial interest to declare in relation to the content of this article.

Copyright © 2014 by the American Society of Plastic Surgeons

DOI: 10.1097/PRS.0000000000000184

Richard A. Hopper, M.D., M.S.

Raymond Tse, M.D.James Smartt, M.D.

Jordan Swanson, M.D.Sara Kinter, M.A.,

C.C.C.-S.L.P.

Seattle, Wash.

Learning Objectives: After studying this article, the participant should be able to: 1. Describe the technical details common to all cleft palate repairs that optimize outcomes and minimize complications. 2. Explain the subjective and objective evaluation of speech in children with cleft palate. 3. Practice with an increased awareness of the management of complications associated with cleft palate repair. 4. Design a treatment plan for velopharyngeal dysfunction.Summary: Goals of a successful cleft palate repair include separation of the oral and nasal components without fistula, achieving sufficient velar length, and creating functional transverse orientation of the levator muscle sling. A number of techniques have been described to achieve these goals, but they all have the following technical details in common: elevation of oral mucosal flaps based on the greater palatine arteries, tension free nasal lining mobiliza-tion, and functional intervelar muscle dissection. After palate repair, speech evaluation needs to be performed by an objective interdisciplinary team fol-lowing a standardized protocol. Identification of velopharyngeal insufficiency secondary to an incompetent nasopharyngeal port will necessitate secondary speech surgery. These secondary techniques include pharyngeal flaps, soft pal-ate lengthening, or pharyngeal sphincters, which should be tailored to opti-mize speech, while minimizing the risk of obstructive sleep apnea. (Plast. Reconstr. Surg. 133: 852e, 2014.)

From the Craniofacial Center, Seattle Children’s Hospital.Received for publication July 10, 2012; accepted February 5, 2014.

Cleft Palate Repair and Velopharyngeal Dysfunction

Related Video content is available for this ar-ticle. The videos can be found under the “Re-lated Videos” section of the full-text article, or, for Ovid users, using the URL citations pub-lished in the article.

CME

Volume 133, Number 6 • Cleft Palate Repair

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repair is accomplished through intravelar velo-plasty, and nasal mucosa is mobilized adjacent to the cleft margins for nasal side closure. The repair is best suited for repair of clefts of the secondary palate because the bipedicled flaps are inadequate for closure of the primary palate behind the alveolus. Although advocates of the von Langenbeck palatoplasty suggest that pre-serving the anterior attachment offers increased vascularity and less future constriction of facial growth, this has not been substantiated by past studies11–15 (References 13 and 14 Level of Evidence: Therapeutic, III).

Veau-Wardill-Kilner (Oxford) PalatoplastyThe Veau-Wardill-Kilner palatoplasty16 is a vari-

ation of the von Langenbeck design that incorpo-rates a V-Y pushback closure of the anterior hard palate (Fig. 2). The bipedicled flaps become uni-pedicled with an anterior release at the junction of the primary and secondary palates, leaving the mucoperiosteum anterior to the incisive foramen in place. The flaps are transposed posteriorly, pro-ducing a degree of palatal lengthening in the pro-cess. This posterior movement is accomplished at the expense of large denuded areas of the anterior palate, which may have a detrimental effect on facial growth. The pushback also leaves oral clo-sure of the anterior primary palate unaddressed.

Bardach Two-Flap PalatoplastyThe Bardach palatoplasty elevates the entire

hard palate behind the alveolus as two flaps based off the greater palatine pedicles (Fig. 3).17 Although this technique requires the largest amount of pala-tal elevation of the techniques discussed so far, it facilitates two-layer closure along the entire length of the cleft, including the fistula behind the alveo-lus. It is well suited for repair of complete bilateral clefts and can be used for complete unilateral clefts.

Hybrid PalatoplastyA variation of the above repairs, popularized

by Gillett and Clark,18 uses a hybrid technique for

Fig. 1. Markings for the von Langenbeck palatoplasty. Lateral releasing incisions along with a submucoperiosteal dissection of the hard palate create paired bipedicled flaps for closure across the cleft. The advantage is increased vascularity of the flaps; the disadvantage is inability to close the fistula of the pri-mary palate. [Reprinted from van Aalst JA, Kolappa KK, Sadove M. MOC-PSSM CME article: Nonsyndromic cleft palate. Plast Reconstr Surg. 2008;121(Suppl):1–14.]

Fig. 2. Veau-Wardill-Kilner palatoplasty. The secondary pal-ate mucoperiosteum is elevated based on the greater palatine arteries. The unipedicled flaps are “pushed back” in a V-Y pat-tern to increase the palate length. The advantage is improved visibility of the pedicle; the disadvantage is potential facial growth restriction from the denuded anterior dissection. [Reprinted from van Aalst JA, Kolappa KK, Sadove M. MOC-PSSM CME article: Nonsyndromic cleft palate. Plast Reconstr Surg. 2008;121(Suppl):1–14.]

Table 1. Evolution of Cleft Palate Repair

Year Advancement Surgeons

1810s Staphylorrhaphy (soft palate closure)

von Graefe and Roux

1826 Uranoplasty of hard palate cleft Dieffenbach1859 Mucoperiosteal advancement

flapsvon Langenbeck

1863 Velopharyngeal insufficiency described

Passavant

1920s Pushback operations Gillies, Fry, and Dorrance

1920 V-Y palatal posterior advancement

Ganzer

1922 Midline approximation of “cleft muscles”

Veau

1937 Posterior advancement with pharyngoplasty

Wardill and Kilner

1969 Intravelar veloplasty Kriens

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Plastic and Reconstructive Surgery • June 2014

Fig. 3. Bardach palatoplasty. (Left) The markings are similar to the Veau technique. (Center) The mucoperios-teum from the primary and secondary palates is elevated as unipedicled flaps that allow closure of the nasal lining and the intervelar veloplasty. (Right) Opposed to the Veau technique, tips of the flap are secured to the back of the alveolus, with the soft palate lengthening achieved by the intervelar veloplasty. The advantage is the ability to close the anterior fistula of the primary palate; the disadvantage is increased sensitivity of the flap vascularity to surgical technique. [Reprinted from van Aalst JA, Kolappa KK, Sadove M. MOC-PSSM CME article: Nonsyndromic cleft palate. Plast Reconstr Surg. 2008;121(Suppl):1–14.]

Fig. 4. Furlow palatoplasty. The double-opposing Z-plasty technique con-sists of a posterior based musculomucosal flap on both the oral and nasal sides of the soft palate. When these two flaps are transposed, the attached levator muscles are brought from a sagittal orientation to a transverse one. The soft palate length is increased at the expense of a narrowing of the intertonsillar pillar distance. The advantage is a reliable lengthening and muscular repositioning of the soft palate; the disadvantage is increased tension on closure of wide clefts. [Reprinted from van Aalst JA, Kolappa KK, Sadove M. MOC-PSSM CME article: Nonsyndromic cleft palate. Plast Reconstr Surg. 2008;121(Suppl):1–14.]


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complete unilateral clefts in which the cleft side has a unipedicled Bardach flap and the noncleft side involves a bipedicled von Langenbeck flap to take advantage of the strengths of the two procedures.

Furlow Double-Opposing Z-PlastyThe Furlow double-opposing Z-plasty pal-

ate repair19 provides for closure and lengthen-ing of the soft palate (Fig. 4) (Level of Evidence: Therapeutic, IV). It is particularly well suited for isolated clefts of the secondary soft palate and is also used as a secondary lengthening procedure for velopharyngeal insufficiency. Mirror image Z-plasty incisions are made across the cleft on the opposing oral and nasal surfaces of the soft pal-ate. The two posteriorly based limbs are muscu-lomucosal, leaving the levator muscles attached to the mucosa, whereas the anteriorly based flaps are mucosa only. When transposed, the levator assumes a transverse orientation, and the palate is lengthened. The lengthening, however, is at the expense of a decrease in the transverse width of the soft palate, which can tether and deform the posterior pillars. The Furlow palatoplasty provides reliable lengthening, maintains musculomucosal attachment, and causes the nasal lining to take on a tubular shape. The net effect is movement of the velum posterior and superior, which places it in a more functional position relative to the Passavant ridge.

The advantages of the Furlow palatoplasty have led to its combined use with other techniques (e.g., Bardach palatoplasty).2,20 This application can be technically demanding, as the Z-plasty

results in a near island flap of the anterior palatal flap. Particular caution should be exercised if it is applied to wide complete bilateral clefts.

COMMON TECHNICAL DETAILS OF CLEFT PALATE REPAIRS

Greater Palatine Pedicle MobilizationIn all techniques, mobilization of the hard

palate requires preservation and mobilization of the greater palatine vascular pedicle. During subperiosteal dissection, three bony spikes signal the proximity of the pedicle at the posterolateral corner of the hard palate (Fig. 5). On the lateral side of the foramen, the periosteum invaginates between the maxilla and the pterygoid process of the sphenoid, and a leaf of the tensor muscle inserts into the oral mucosa. These require sharp release. Medially, the posterior nasal spine is vari-ably formed. Just posterior and lateral to this is the cleavage plane between the tensor and leva-tor muscles, where the submucosa and mucosa tend to be tethered and a surface dimple often is seen. Deliberate elevation of the submucosa from between the muscles prevents mucosal tears. After lateral and medial release, circumferential dissection of the pedicle can be completed. If fur-ther mobility of the pedicle is required, it can be achieved by release of the periosteal cuff posterior to the pedicle by gentle stroking with a small knife followed by a gentle stretch with opening of scis-sors. Sudden relaxation signals completion of the periosteal release. Further mobility can be gained by removal of the bone posterior to the pedicle using a 2-mm osteotome. After the osteotomy, the pedicle can lie more transversely, but care must be taken that the medial bone edge is removed so it does not compress the flow.

Nasal Lining DissectionWith appropriate release of the nasal mucosa

off the medial pterygoid plate of the skull base, tension-free closure of the nasal lining can be achieved in all clefts. Subperiosteal access is gained along the caudal edge of the tensor fas-cia, at the juncture of the posterior border of the hard palate and the lateral nasal wall. Posterior to the pedicle, medial to the hamulus, there is an adherence of the mucosa at the pterygomaxillary junction. Careful release in the sagittal plane is followed by easy dissection of the nasal lining of the medial pterygoid plate up to the skull base. With the tip of an elevator at the skull base, the mucosa can be levered medially, creating a large

Fig. 5. Circumferential dissection of the greater palatine pedicle (g). The anterior three bony spikes of the palatine bone herald the proximity of the pedicle during dissection.

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sheet of mucosa that meets the midline (Fig. 6). Care must be taken to first fully release the adher-ence of the mucosa and levator at the posterior nasal spine; otherwise, the lining can tear at this point during the levering maneuver.

Muscle DissectionThe normal levator palatini muscle forms

a sling across the middle third of the soft pal-ate. Clefts result in an abnormal anteroposterior

course and insertion onto the posterior hard pal-ate. This sagittal orientation of the muscle impairs closure of the velopharyngeal port during speech. Cleft repair requires realignment into a transverse orientation. For adequate mobilization of the levator, there are two options. One is to release the tensor tendon as it wraps around the hamulus so that the levator and tensor palatini are mobi-lized en bloc (Fig. 7). The other option is to sep-arate the levator from the tensor palatini within the velum, which may allow greater mobility and retroposition. Once released, the levator course can be traced cranial to the superior constrictor and followed toward the cranial base (Fig. 8). The intervelar separation of the levator may increase mobility, and retain the intrinsic tension of the tensor palatini for eustachian tube function, but the dissection must be meticulous to avoid dam-age to the muscle fibers. The use of a microscope can aid in visualization. If the tensor cannot be readily visualized, the two muscles are released en bloc. Although the access or dissection may differ among techniques, the same release and transpo-sition of the levator sling is required regardless of what repair is used (Fig. 9).

ModificationsSommerlad has popularized the use of a

microscope for better visualization of the soft palate muscle dissection.21,22 Some have advo-cated buccal mucosa flaps based anteriorly or posteriorly to patch small defects of oral or nasal mucosa.23,24 Acellular dermal matrix has also been used to patch defects.25 Although its effectiveness has been reported and it imparts no donor-site morbidity, it is avascular and can potentially act as a foreign body if it is not quickly revascular-ized. A recent report has questioned the need for nasal lining closure26; however, this remains controversial.

Velopharyngeal InsufficiencyThe velopharyngeal sphincter is created by the

soft palate or velum, the lateral pharyngeal walls, and the posterior pharyngeal wall. The muscles that constitute the velopharyngeal sphincter are the levator veli palatini, tensor veli palatini, palatoglos-sus, palatopharyngeus, musculus uvulae, salpingo-pharyngeus, and superior pharyngeal constrictors. The key contributors to velopharyngeal sphincter closure are the levator veli palatini, palatopharyn-geus, and superior pharyngeal constrictors.27 The palatoglossus does not contribute to velopharyn-geal sphincter animation in speech, but does play a

Fig. 6. Nasal lining release. Once the greater palatine pedicle and posterior nasal spine have been dissected, the nasal lining can be released back to the skull base with a subperiosteal dissection off the medial pterygoid plate. With this technique, tension-free closure of the nasal lining is possible, even in wide clefts.

Fig. 7. The tensor aponeurosis. The tensor wraps over the hamu-lus and can be released at this point to mobilize the levator and tensor en bloc.


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role in deglutition. The tensor veli palatini is inner-vated by the trigeminal nerve (fifth cranial nerve), whereas the rest are innervated by the pharyngeal plexus (ninth and tenth cranial nerves).

The role of the velopharyngeal sphincter is to separate the oral and nasal cavities for speech and swallowing. The velopharyngeal sphincter should remain open for normal breathing and for production of nasal consonants (/m/, /n/, and /ng/). Complete closure of the velopharyngeal sphincter is required for production of oral pres-sure consonants (e.g., /p/, /b/, /t/, /d/, /k/, /g/, /s/, /z/, /f/, and /v/), sucking, swallowing, and blowing. During connected speech, velophar-ynx function is a complex coordination of varying

degrees of velopharyngeal sphincter opening and closure influenced by both the target sound and the surrounding phonetic context.28–32

Velopharyngeal dysfunction is a general term and includes many causes that may or may not be associated with cleft palate (Fig. 10). Velopha-ryngeal insufficiency is a type of velopharyngeal dysfunction and is the most common cause of reduced intelligibility of speech in patients with clefts. Velopharyngeal insufficiency is character-ized by nasal air emission and/or hypernasal-ity from mechanical restriction, malposition, or insufficiency of velar tissue. In addition to a short palate or a lack of transverse orientation of the levator mechanism, velopharyngeal insufficiency

Fig. 8. (Left) Intervelar dissection and posterior transposition of the levator muscle (a) off of the tensor palatini (b) and pharyngeus (c). (Right) Artist’s rendition of dissection. GPP, greater palatine pedicle; TVP, tensor veli palatini; PP, palatopharyngeus; LVP, levator veli palatini.

Fig. 9. Intervelar veloplasty with transposition of the levator sling (a) from a sagittal (left) to a transverse (right) orientation. This muscle movement is required with all techniques, regardless of the oral lining incisions or dissection.

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can also be the result of palatopharyngeal dispro-portion. This can occur with skull base anomalies (as in 22q11 deletion), scarring or strictures from previous surgery, or mechanical obstruction from tonsillar abnormalities. Although velopharyngeal insufficiency is the most frequent diagnosis in cleft palate patients, velopharyngeal dysfunction can also result from velopharyngeal mislearning with compensatory misarticulation. Finally, nasal air emission may not be related to velopharyngeal insufficiency or velopharyngeal dysfunction. In the case of a patent oronasal fistula and a func-tioning velopharyngeal sphincter, patients can have nasal air escape through the fistula.

SPEECH EVALUATION OF THE PATIENT WITH A CLEFT PALATE

The most common methods to evaluate velo-pharyngeal function are: (1) perceptual assess-ment by a trained speech-language pathologist; (2) transoral examination; (3) nasoendoscopy; (4) multiview video fluoroscopy; and (5) aerody-namic vocal tract measurements (e.g., nasometry and pressure-flow testing). The relative ability of

the techniques to detect abnormalities in velopha-ryngeal structure, movement, closure, and timing is shown in Table 2.

Perceptual evaluation forms the cornerstone of the evaluation process and includes subjec-tive assessment of the presence of nasal air emis-sion, definition of resonance characteristics (i.e., hypernasality versus hyponasality), and overall rating of degree of velopharyngeal insufficiency. Standardized articulation testing is also included to differentially diagnose type of speech errors (developmental, obligatory, or compensatory). Speech, resonance, and airflow control character-istics should be assessed in both short phrases, rote speech (counting), and connected speech when-ever possible, as velopharyngeal function may vary depending on the complexity of the speech task and degree of fatigue. It is imperative that percep-tual velopharyngeal insufficiency assessment be completed by a clinician trained in diagnosis and management of velopharyngeal insufficiency, as some speech characteristics can be misperceived as velopharyngeal insufficiency. This assessment can be completed as early as 2 years of age in many children. Table 3 lists the resonance, articulation,

Fig. 10. Velopharyngeal dysfunction classification. The differential of velopharyngeal dysfunction includes velopharyn-geal insufficiency, incompetency, or mislearning (orange boxes). Potential causes of nasal air emission in a patient with cleft are in the green boxes. Causes in blue boxes are typically not found in patients with cleft palate.


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and phonation disorders often associated with cleft palate and velopharyngeal dysfunction that can be discerned by a speech-language pathologist expe-rienced in velopharyngeal insufficiency diagnosis and management. Ideally, ratings of velopharyngeal insufficiency characteristics should be reported in a standardized way to enhance communication between team members. An example of such a tool is the Pittsburgh Weighted Speech Scale.33

A transoral examination is typically performed along with the perceptual evaluation to provide information on the presence and size of oronasal fistulas, the contour and length of the velum at rest and during phonation, the status of tonsillar tissue, and the state of dentition and occlusal rela-tionship. Atypical dentition can result in obliga-tory sound distortions or substitutions. Transoral examination does not provide information on competency or timing of velopharyngeal closure.

The evaluation pathway followed at Seat-tle Children’s Hospital Craniofacial Center is described in Figure 11. Detected velopharyngeal insufficiency on perceptual evaluation will lead

to an instrumental evaluation to further evaluate velopharyngeal function. Velopharyngeal mis-learning alone will instead be referred to speech therapy, with subsequent instrumental evaluation only if velopharyngeal insufficiency is present fol-lowing acquisition of some pressure consonants.

The instrumental evaluation performed at Seattle Children’s Hospital includes nasoen-doscopy and video fluoroscopy. These provide information on the timing, closure pattern, and competency of the velopharynx.34 (See Video, Supplemental Digital Content 1, which displays lateral fluoroscopy of a patient with normal velo-pharyngeal function, http://links.lww.com/PRS/A995. Note the strong contact of the velum to the pharyngeal wall during oral pressure consonants. See Video, Supplemental Digital Content 2, which displays a nasopharyngoscopic view of a velophar-ynx with normal closure pattern, http://links.lww.com/PRS/A996. Note the opening of the velopha-ryngeal port during nasal consonants and the clo-sure during oral pressure consonants. See Video, Supplemental Digital Content 3, which displays a

Table 2. Comparison of Common Evaluation Techniques for Velopharyngeal Structure, Movement, Closure, and Timing

Technique Structure Movement Closure Timing Patient Tolerance Expense

Perceptual No No Yes No Good LowOral examination Limited Limited No No Good LowNasoendoscopy Yes Yes Yes Yes Fair HighFluoroscopy Yes Yes Yes Yes Good HighNasometry No Yes Yes Yes Good Moderate

Table 3. Brief Definitions of Resonance, Articulation, and Phonation Disorders Often Associated with Cleft Palate and Velopharyngeal Dysfunction*

Disorder Definition

Hypernasality The perception of unregulated nasal resonance during the production of vowels resulting from inappropriate connection of the oral and nasal cavities

Nasal air emission Nasal air escape that accompanies the production of consonants requiring high oral pressure (i.e., /p/, /b/)

Hyponasality A reduction in normal nasal resonance resulting from nasal blockage (i.e., turbinate hypertrophy, septal deviation, obstructing pharyngeal flap)

Hypernasality and hyponasality

Simultaneous hypernasality and hyponasality usually resulting from incomplete velopharyngeal closure in the presence of high nasal resistance (not complete blockage)

Cul-de-sac resonance A variation of hyponasality often associated with tight anterior nasal constriction producing a muffled quality to sounds

Nasal substitution The articulating structures are placed appropriately for an intended oral consonant but incomplete velopharyngeal closure causes the sound to be produced as a nasal consonant (i.e., /b/ becomes /m/; /d/ becomes /n/)

Compensatory misarticulation

The articulating structures are placed inappropriately so that the individual can create the plosive (e.g., /p/) or fricative (e.g., /s/) characteristics of the sound they want to make in the presence of velopharyngeal dysfunction; examples include glottal stops and pharyngeal stops that are used to build up the oral pressure that is usually created by a functioning velopharynx

Laryngeal symptoms A variety of phonation disorders that accompany velopharyngeal dysfunction; they are thought to be a compensation for the inability to achieve complete velopharyngeal closure with compensatory activity at the level of the larynx; examples include hoarseness, low speaking volume, strained or strangled voice quality, and unusual pitch alterations

*Adapted from D’Antonio LL. Evaluation and management of velopharyngeal dysfunction: A speech pathologist’s viewpoint. Probl Plast Reconstr Surg. 1992;2:86–111.

http://links.lww.com/PRS/A995




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lateral fluoroscopic view of a patient with velopha-ryngeal insufficiency, http://links.lww.com/PRS/A997. Note the consistent absence of contact of the velum to the posterior pharyngeal wall during oral pressure consonants. See Video, Supplemen-tal Digital Content 4, which displays a nasopha-ryngoscopic view of a velopharynx with abnormal triangular closure pattern, http://links.lww.com/

PRS/A998. Note the V-shaped defect in the velum, consistent with sagittal levator orientation, and the consistent opening of the velopharyngeal port during oral pressure consonants. This patient was subsequently treated with a Furlow secondary lengthening procedure. All videos are available in the “Related Videos” section of the full-text article

Fig. 11. Seattle Children’s Hospital velopharyngeal insufficiency evaluation protocol. Perceptual speech evaluation determines whether patients have hypernasality and/or nasal air emission (NAE). If they do, an instrumental evaluation with nasopharyngos-copy is performed to determine whether physical management (surgery or obturator) is indicated. If there is no hypernasality or nasal air emission, speech therapy is instituted with repeated perceptual evaluation.

Video 1. Supplemental Digital Content 1 displays lateral fluo-roscopy of a patient with normal velopharyngeal function. Note the strong contact of the velum to the pharyngeal wall during oral pressure consonants. This video is available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A995.

Video 2. Supplemental Digital Content 2 displays a nasopha-ryngoscopic view of a velopharynx with normal closure pattern. Note the opening of the velopharyngeal port during nasal con-sonants and the closure during oral pressure consonants. This video is available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A996.









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on PRSJournal.com or, for Ovid users, at the URLs provided.)

These images are used to score and docu-ment the status of the velopharynx and pro-vide treatment recommendations. We use the Golding-Kushner scale35 to subjectively rate the closure pattern and size of the velopharyngeal gap. Briefly, the scale rates lateral wall movement from 0 (no movement) to 0.5 (movement to the midline), right and left palate movement from 0 (no movement) to 1.0 (contact at the posterior

pharyngeal wall), and posterior wall movement from 0 (no movement) to 1.0 (movement suffi-cient to reach the palate).

Nasometry is a noninvasive objective evalua-tion of nasalance, which is defined as an acous-tic ratio of nasal to oral resonance. A higher nasalance score is therefore expected for phrases containing nasal consonants; conversely, a low score is expected with oral phoneme phrases. There are published nasalance normative means and deviations for comparison of an individ-ual’s score with the noncleft population. The McKay-Kummer sensory nerve action potential test is a picture-cued evaluation of hypernasality and hyponasality. Short phrases composed of oral (closed velopharynx) pressure consonants (i.e., “Pick up the book,” “Go get a cookie,” “Suzy sees the scissors”) are used to detect hypernasality and nasal air escape, whereas phrases with nasal conso-nants (“Mama made some mittens”) can be used to detect hyponasality.36

MANAGEMENT OF VELOPHARYNGEAL INSUFFICIENCY

Based on the speech evaluation described above, a standardized protocol should be followed to recommend physical treatment based on instru-mental assessment (Fig. 11).34 Nasoendoscopy is used to determine candidacy for a secondary Fur-low lengthening palatoplasty (in cases of sagittally oriented levators) or a pharyngoplasty (in cases of transverse oriented levators). If a pharyngoplasty is planned, the videofluoroscopy and nasoendos-copy results are used to help the surgeon tailor the size and position. If the soft palate is short and/or scarred and the orientation of the levators can-not be determined, the palate is explored intraop-eratively. If the muscles are found to be sagittally oriented, a Furlow lengthening procedure is per-formed. Perceptual velopharyngeal insufficiency evaluation is performed 3 months postoperatively, with referral for instrumental evaluation made only in the case of residual velopharyngeal insuf-ficiency, or if there are symptoms of obstructive sleep apnea. The outcomes of this standardized approach to velopharyngeal insufficiency have been reported previously.34

The two workhorse pharyngeal procedures are the superiorly based pharyngeal flap and the sphincter pharyngoplasty. Pharyngeal augmenta-tion and injection37 have also been described but are less commonly used. The superiorly based pharyngeal flap converts the velopharyngeal gap into two smaller lateral ports, to facilitate closure

Video 3. Supplemental Digital Content 3 displays a lateral fluo-roscopic view of a patient with velopharyngeal insufficiency. Note the consistent absence of contact of the velum to the posterior pharyngeal wall during oral pressure consonants. This video is available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A997.

Video 4. Supplemental Digital Content 4 displays a nasopha-ryngoscopic view of a velopharynx with abnormal triangular closure pattern. Note the V-shaped defect in the velum, consis-tent with sagittal levator orientation, and the consistent open-ing of the velopharyngeal port during oral pressure consonants. This patient was subsequently treated with a Furlow secondary lengthening procedure. This video is available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A998.




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in less dynamic velopharyngeal sphincter. More recently, attempts have been made to tailor the flap to the velopharyngeal sphincter gap to opti-mize speech outcomes while minimizing the risk of sleep apnea.38–40 Some centers have used the superiorly based pharyngeal flap preferentially for sagittal closure patterns (isolated lateral wall movement sufficient to reach the edges of the flap) and sphincter pharyngoplasty for coronal closure patterns (to take advantage of dynamic pharyngeal constrictors in the sphincter)31,41 (Reference 40 Level of Evidence: Risk, II). Bleed-ing and airway complications related to superi-orly based pharyngeal flaps have been shown to decrease through the use of palatal mucosal flaps to resurface the raw surface of the flap and by limiting the number of surgeons performing the procedure.42 Advocates of the sphincter pharyn-goplasty technique feel that it provides a more physiologic reconstruction of the velopharyngeal sphincter27; however, two randomized prospective studies comparing sphincter pharyngoplasty and superiorly based pharyngeal flaps did not dem-onstrate any significant difference in outcome between the two procedures43,44 (Reference 43 Level of Evidence: Therapeutic, II; Reference 44 Level of Evidence: Therapeutic, I). Both studies, however, did recognize small study size and inad-equate power.

For the modified Hynes sphincter pharyngo-plasty, two lateral superiorly based mucosal flaps are raised containing the palatopharyngeus mus-cles (Fig. 12).45,46 The flaps are rotated at right angles to attach to a demucosalized transverse strip on the posterior pharynx, just below the ade-noid pad. The location of the transverse strip is based on the desired point of palatal contact seen on preoperative videofluoroscopy. In some cases, the adenoid pad is displaced inferiorly and inter-feres with insetting of the flaps. In these cases, a microdebrider can be used to create space for the flap or, less commonly, a separate adenoidectomy may be required followed by second-stage inset-ting of the flaps into the scar. The degree of over-lap (tightness) of the two flaps is determined by the size of the gap seen on preoperative nasopha-ryngoscopy. In very large gaps, a portion of the posterior tonsillar pillar can be incorporated into the flaps to create a more substantial sphincter, but this can predispose to sleep apnea, especially when used with small or moderate gaps. Although the sphincter has been described as “dynamic,” electromyographic testing demonstrates no intrin-sic function within the transposed flaps. However, the adjacent pharyngeal constrictors still function

after sphincter creation.47 Sphincter pharyngo-plasty revision can be achieved through eleva-tion, tightening, or lateral release of the sphincter based on postsurgery evaluation.

Patients who are not considered good surgical candidates, because of either medical comorbidi-ties (sleep apnea) or neuromuscular conditions

Fig. 12. Sphincter pharyngoplasty techniques. (Above) Hynes pharyngoplasty. Lateral pharyngeal wall flaps that include the posterior tonsillar pillars (palatopharyngeus, salpingopharyn-geus, and superior pharyngeal constrictor) are sutured high on the posterior pharyngeal wall in a crossover fashion. (Center) Orticochea pharyngoplasty. The palatopharyngeus is trans-posed into a low, inferiorly based flap in the oropharynx. (Below) The Hynes pharyngoplasty can be modified by suturing the tips of the flaps with their underlying muscle in end-to-end fashion to tailor the degree of port reduction. In addition, the flaps can be reduced in size if a smaller sphincter is required by reducing the amount of posterior tonsillar pillar and palatopharyngeus in the flaps. (Reprinted from Moss AL, Pigott RW, Albery EH. Hynes pharyngoplasty revisited. Plast Reconstr Surg. 1987;79:346–355.)


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(velopharyngeal incompetence) can be offered nonsurgical prosthetic management with an obtu-rator (fills residual velopharyngeal sphincter gap) or palatal lift (statically elevates the palate). Fam-ily preference may also drive recommendation for an obturator. It has been reported that in some cases muscle strengthening can occur with use of an intraoral prosthesis, allowing some patients to be weaned from the appliance or become candi-dates for surgical intervention.

OBSTRUCTIVE SLEEP APNEAPatients with a repaired cleft palate are at

increased risk of obstructive sleep apnea. Con-tributing factors may be the associated midface retrusion or iatrogenic obstructive sleep apnea from previous palate and pharyngeal operations. Studies have estimated the risk of obstructive sleep apnea in the cleft lip–cleft palate popula-tion to range from 8.5 to 65 percent.48–50 The rate of occurrence of iatrogenic obstructive sleep apnea from pharyngeal speech surgery is low and reported at two of 104 patients who underwent tai-lored pharyngeal flaps51 and eight of 58 patients who underwent sphincter pharyngoplasty51,52 (Reference 51 Level of Evidence: Therapeutic, IV). Patients with Pierre Robin sequence and/or a history of perinatal respiratory difficulties may be at highest risk.52 Management of obstructive sleep apnea in a cleft palate patient requires multidisci-plinary evaluation. The most common procedure is tonsillectomy and/or adenoidectomy followed by pharyngeal flap takedown or sphincter relax-ation.53 Families need to be made aware that treat-ment of obstructive sleep apnea can exacerbate speech problems.

Richard A. Hopper, M.D., M.S.Division of Plastic Surgery

Craniofacial CenterSeattle Children’s Hospital

4800 Sand Point Way NESeattle, Wash. 98105

[email protected]

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mailto:[email protected]

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Documents

Cleft Palate Repair and Velopharyngeal Dysfunction€¦ · for velopharyngeal insufficiency. Mirror image Z-plasty incisions are made across the cleft on the opposing oral and nasal