eMedicine Specialties > Plastic Surgery > Craniofacial

Craniofacial, Postpalatoplasty Speech Dysfunction

Author: Peter D Witt, MD, Associate Clinical Professor, Department of Surgery and Pediatrics, University of California San Francisco; Medical Director, Department of Pediatric Plastic Surgery, Children's Hospital Central California
Contributor Information and Disclosures

Updated: Aug 26, 2008

The Problem: Velopharyngeal Dysfunction

Velopharyngeal dysfunction (VPD) is the constellation of speech production disorders that includes velopharyngeal insufficiency, incompetence, and incorrect learning. Anomalous velopharyngeal closure prevents appropriate speech production. Patients with VPD may present with hypernasality, nasal emission, or facial grimacing. In their attempt to be understood, affected patients often develop compensatory maladaptive articulations that are very difficult to reverse if left untreated. Many times, this failure of the sphincteric mechanism is the result of a structural defect of the pharyngeal walls or the velum (soft palate) at the level of the nasopharynx.

Increasingly, speech scientists and surgeons have converted to using the term VPD in place of the older and more entrenched term VPI,^1,2 which can be confusing because various authors use it to connote velopharyngeal insufficiency, velopharyngeal incompetence, velopharyngeal inadequacy, or velopharyngeal incorrect learning. In common parlance, VPI generally means that sphincteric closure is incomplete during production of oral sounds of speech. While the descriptors used in VPI are used synonymously, they are not necessarily equivalent. In contrast, the term VPD does not assume or exclude any possible origin of speech symptoms. Anatomic, myoneural, behavioral, or combinations of disorders are all possible causes of the dysfunction. VPD occurs in approximately 20% of children who undergo palatoplasty.³ In-depth evaluation of symptoms, causes, and treatment outcomes are critical aspects of treating patients with VPD.

Patients with VPD should be treated within in the context of multidisciplinary team care. In 1988, an international working group convened to standardize definitions and assessment methodologies.⁴ The working group strongly recommended implementing a multidisciplinary team approach and using multimodal instruments to evaluate preoperative and postoperative speech outcomes. The group asserted that comprehensive analysis of specific causes of speech production disorders, through perceptual and instrumental measures of velopharyngeal function, allows for customized treatment algorithms for specific patients.

Role of the speech pathologist

The surgeon, speech pathologist, and other health care providers work closely together to achieve the goal of optimal treatment for the patient. These practitioners collaborate in their reviews of the in-depth diagnostic assessment results and the individual patient's medical history. Consensus evaluation usually provides an appropriate course of management for affected individuals and may yield a differential diagnosis that leads to differential management.⁵ Ideally, this means that care providers attempt to match the gap size, shape, and velopharyngeal closing pattern to the most appropriate intervention.

Surgeons, as well as lay people, are usually capable of recognizing speech "differences." Perception of the difference does not require a sophisticated understanding of speech physiology, but discrimination of the causes and severity or magnitude of that difference and treatment planning do require a sophisticated understanding of speech physiology. Speech and language pathologists are particularly adept at sorting out the components of a communication disorder and their respective weights, which frequently dictates what receives surgical attention, not whether it receives attention. This is both a skill and a talent that surgeons and lay people rarely possess.

The relationship between speech pathologist and surgeon

Making the distinction between velopharyngeal valve dysfunction (structural defect) and speech dysfunction is critical. For example, consider the following analogy: If a newly licensed 16-year-old driver is involved in a motor vehicle accident, differentiating between a mechanical failure of the car and a failure of the just-learning driver to maintain control of the vehicle is important. Was the driver taking mind-altering drugs? Was a mechanical problem with the car at fault or was the driver's inexperience or misbehavior at fault? The answers to these questions are basic to the investigational algorithm. They tell the investigator where to look next, keeping in mind that both might sides may have been to blame.

The same logic holds true for the evaluation and management of VPD. Is the velopharyngeal valve (eg, car) at fault or the speech disorder (eg, driver) at fault? The answer to this question tells the investigator what to do next and who should do it. Treatment may involve fixing the velopharyngeal valve (ie, necessitating a surgeon's expertise) or teaching the patient (ie, necessitating speech and language pathologists' expertise). Examining the velopharyngeal valve should be quite straightforward. A simple mirror test at the bedside, with a definitive yes-or-no answer, can help determine if the patient can eliminate nasal escape.

The presence of hypernasality is much more difficult to evaluate. Using vocal, nonverbal testing can obviate such problems as phoneme-specific velopharyngeal insufficiency. An astute speech and language pathologist should be able to make the determination despite the confounding glottal stops, fistulae, and other factors. Surgeons must emphasize to parents, patients, and other providers that surgical success can be anticipated with respect to nasal escape and hypernasality. Then, the speech and language pathologists assume the responsibility of helping the patient achieve speech and language success, including articulation and other facets of verbal communication.

Anatomy

The anatomy of the velopharynx (ie, palate, posterior pharyngeal wall, airway) is depicted in Image 1.

The composite movements of the lateral pharyngeal walls, the velum, and posterior pharyngeal walls close the velopharyngeal port in deglutition and during oral speech sounds; they open the port for breathing and some nasalized articulations. Patterns of closure as observed on preoperative instrumental assessments include coronal, sagittal, bow-tie, circular, and Passavant (see Image 2).

Basic speech terminology for the surgeon:

Presumably, care providers representing the various disciplines of the cleft team use the same nomenclature so they can effectively organize and communicate their knowledge. Trost-Cardamone developed a useful taxonomy to classify possible causative factors of VPD.⁶

In velopharyngeal insufficiency, the tissue to accomplish closure of the velopharyngeal sphincter is insufficient. Additionally, velopharyngeal insufficiency can be due to structural etiologies, such as mechanical interferences with closure, including excessively large tonsils and/or webbing of the posterior tonsillar pillars.⁷

Velopharyngeal incompetence occurs with neurogenic etiologies such as motor disorders. Velopharyngeal incorrect learning may be the result of phoneme-specific nasal emission and deafness or hearing impairment.

Hypernasality and hyponasality are voice tones shaped by the mouth and oropharynx. Hypernasality is excessive resonance in the nasal cavity that is usually related to VPD due to a lack of a barrier between the oral and nasal cavities. While hypernasality usually refers to velopharyngeal sphincteric function, it may be secondary to a fistula or unrepaired cleft palate.

The following is a lexicon of additional terms used to describe some elements of cleft palate speech dysfunction:^8,9

• Nasalance: This is an acoustic correlate of nasal resonance. It is calculated as the ratio of nasal to nasal-plus-oral energy.
• Airflow (nasal emission): This is different from nasal acoustic energy associated with hypernasality. It involves a nasal, instead of oral, increase in airflow. Nasal emission and turbulence are disturbances of airflow that mostly affect the production of pressure consonants. Nasal air emission is an attempt to build up intraoral air pressure for the production of consonants in the presence of a leak in the system fistula or velopharyngeal valve. Some of the airflow is released through the nose, causing a disruption in the aerodynamic process of speech. It is noted on pressure sensitive phonemes, plosive fricatives, and affricates (not on vowels), and it can occur with normal resonance. Whether it is audible or not depends on the size of the opening. The bigger the opening, the less resistance to the flow.
• Nasal rustle or turbulence: This is a distracting sound that accompanies consonant production. Generally, small constriction in the nasopharynx produces a distinctive fricative sound on the voiced pressure consonants b, d, and g.
• Hypernasality: Nasally escaping air reverberating in a confined postnasal space defines hypernasality. Hypernasality is a resonance disorder that occurs when abnormal coupling (sharing of acoustic energy) of the oral and nasal cavities occurs during speech. Specifically, hypernasality is abnormal nasal resonance during the production of nonnasal oral sounds.
• Grimace: This aberrant facial muscle movement is a subconscious attempt by the patient to inhibit abnormal nasal airflow by constricting the nares.
• Hyponasality: A blocked up tone, hyponasality may occur with nasal obstruction from enlarged adenoids, a deviated septum, an inadequate nasal airway, or chronic catarrh.

Patient Assessment

When a patient is referred for surgical treatment of VPD, the first steps should be to try to elicit specific information germane to speech problems, cleft palate, or both.

Questions

Try to ascertain from both the parents and the patient whether the speech production disorder has caused psychosocial stigmatization, peer teasing, or frustration in not being able to communicate with others. Nasal regurgitation of liquids or solids and/or an associated hygiene problem may be the source of social embarrassment.

Findings

During intraoral inspection, look for palatal fistulae, enlarged tonsils, visibly aberrant carotid pulsations along the posterior pharyngeal wall, a prominent adenoid pad, palatal zona pellucida (trough), a palpable notch at the junction of the hard and soft palate, or a bifid uvula. Check for velar mobility (elevation) on speech tasks, and, thus, indirectly assess levator muscle status.

Provocative tests

Simple bedside maneuvers can help define the speech problem. A pocket-size, handheld mirror can be placed beneath the patient's nares in order to observe nasal airflow (audible air nasal emission). A straw may be placed at the corner of the patient's mouth while he or she recites a speech task. The listener at the other end of the straw can perceive amplified air sound, unmasked hypernasality, or both. (Click here to complete a Medscape CME activity on examining the ears, nose, and oral cavity in an older patient.)

Listen to both spontaneous speech and structured provocative samples. Provocative samples of speech are designed to elicit phonemes that require velopharyngeal closure. A representative sequence might include the following words or phrases: ma, ma, ma, puppy, puffy, muffin, pamper, sissy, go get a big egg, bye-bye Bobby, Katy likes cookies, Sally sees the sky. Production of voiceless consonants such as p, t, k, s, f, and sh require maximal pulmonary pressures and thus can be used as a brief screening for integrity of plosive sounds. Try to ascertain overall intelligibility in running, spontaneous, connected speech. Patients with suspected VPD are incapable of achieving velopharyngeal closure upon maximum effort when producing properly articulated phonemes that require closure.

Importantly, errors in these sequences of sounds should serve only as a red flag for the surgeon; interpretive significance of the errors should be left to the qualified speech and language pathologist. Most physicians are unfamiliar with the behavioral variables that can affect velopharyngeal function, such as oronasal discrimination proficiency, the presence of maladaptive articulations, the effects of coarticulation, the range of articulatory motion, and the contribution of speaking effort. The speech evaluation should include attention to error types and "stimulability" of performance during visualization of dynamic speech activity. Arguably, the speech pathologist best understands and interprets the movements and the articulatory and vocal structures.

The author usually concludes the interview with extemporaneous hand-drawn pictures of the velopharyngeal mechanism to explain the complex speech mechanism to the patient and family.

Airway evaluation

The tonsils and adenoids are often important components of the velopharyngeal closure mechanism. Occasionally, hypertrophic tonsils may herniate into the velopharyngeal port so that lymphoid obstruction may actually be a source of speech dysfunction. Other times, enlarged tonsils may limit the technical placement of pharyngoplasty flaps or their sheer size may efface the myomucosal pillars, making flap elevation difficult. Similarly, enlarged, friable, and hemorrhagic adenoids may inhibit performance of velopharyngeal surgery, and their presence may even compromise the outcome of surgical intervention if they contribute to flap dehiscence.

In these circumstances, preoperative tonsillectomy and/or adenoidectomy may be indicated. This decision, however, must be made cautiously and in conjunction with the team otolaryngologist and speech pathologist. Tonsillectomy, and particularly adenoidectomy, should be avoided in any patient with symptoms of VPD until a differential diagnosis is established and a management plan is formulated by care providers and accepted by the patient and family. Click here to complete a Medscape CME activity on tonsillectomy.

The clinical manifestations of VPD are likely to worsen after adenoidectomy. If performing an adenoidectomy is necessary to facilitate the technical execution of velopharyngeal surgery, the patient and family must be duly warned about this predictable deterioration. The author usually waits 3 months after adenoidectomy before proceeding with velopharyngeal surgery. Personally communicating with the team otolaryngologist to be certain he or she preserves the precious posterior tonsillar pillar tissue for later construction of the port is wise.

Instrumental assessment of speech

Several diagnostic modalities can be used to assess speech production in patients who demonstrate symptoms of VPD. Detailed descriptions of these modalities are found in published articles.¹⁰ These modalities include video-recorded standard perceptual speech screenings (ie, acoustic evaluation of sounds or listener judgments), nasendoscopy, nasometry, aerodynamics, and fluoroscopic speech evaluations. The studies have the advantage of being readily archived on digital media for review, for study, and for strobe analysis, among other tasks. Usually, test results are reviewed by the interdisciplinary velopharyngeal staff of specialists, including a speech and language pathologist, an otolaryngologist, a prosthodontist, and a plastic surgeon.

If cephalometric evaluations are available, they can facilitate diagnosis. Tracings can quantitatively assess the ratio of velar length to velopharyngeal depth, which is often a good predictor of patients who require physical management of the velopharynx.

Managing VPD

Nonsurgical and Treatment Options

In a small number of cases, prosthetic management may be the best solution for VPD. Prostheses include the following:

• Palatal lift: See Image 3. This device is good for patients with adequate tissue but poor control of coordination and timing of velopharyngeal movements.
• Speech bulb/obturator: This is an acrylic mass used for closing residual velopharyngeal gaps to achieve closure in patients with inadequate tissue.

Prostheses may be used as a temporary reversible trial for which they provide diagnostic information in patients with variable VPD in whom it is unclear whether surgery alone will provide significant improvement in speech quality. A prosthesis may be useful in some patients with a short, scarred velum or in other patients with a long supple paretic velum. Some authors have hypothesized that prostheses may stimulate neuromuscular activity,¹¹ although definitive proof for this is lacking.¹²

Contraindications to Surgery

Velopharyngeal narrowing procedures are not appropriate for patients who meet the following criteria:

• The patient declines surgical management by choice.
• The patient has a known or suspected risk for potential airway obstruction.
• The patient has intermittent or inconsistent closure that responds well to speech therapy.
• The patient has incomplete diagnostic results. With further studies and improvements in diagnostic technologies, speech production disorders should be more accurately assessed and individually managed to achieve optimal results.

The author does not believe that visible pulsations on the posterior pharyngeal wall, indicating aberrant carotid arteries, should be an absolute contraindication to surgery.¹³

What about aberrant carotid arteries?

Anomalous internal carotid arteries have been shown to be a frequent feature of velocardiofacial syndrome. These vessels pose a potential risk for iatrogenic injury and hemorrhage during velopharyngeal-narrowing procedures. Various forms of cervical vascular imaging studies (eg, CT scanning, angiography) have been advocated as aids to surgery by defining the preoperative vascular anatomy. Nevertheless, whether these studies alter the conduct or outcome of operations on the velopharynx remains unclear. Iatrogenic injuries to the carotid artery during velopharyngeal surgery are strikingly absent in the extant literature. Occasionally, transmission of vascular pulsations through floppy redundant mucosa may artificially masquerade as an ominous vessel. Additionally, tortuous mesially displaced vessels observed at one point have been shown to straighten out laterally on later studies.

The issue of how the should surgeon should approach the problem of aberrantly located carotid vessels is provocative and controversial and deserves overt answers from each participating surgeon; however, safety must prevail as the first priority. When displaced vessels are identified, surgeons are faced with a few options. In the first, the surgeon may abandon the procedure. In the second, the surgeon may "operate around" the vessels. In the third, the surgeon may choose to perform one procedure instead of another, ie, sphincter pharyngoplasty instead of pharyngeal flap. (Theoretically, performance of the latter procedure could expose a vessel over the full length of the flap.)

The author is more comfortable operating in the presence of these aberrant structures, provided he can reposition the flap(s) so as not to interfere with their presence, expose the vessel to oropharyngeal secretions, or compromise the execution of the procedure. The author does not routinely obtain preoperative vascular imaging studies on all patients. The author, in performing more than 150 velopharyngeal-narrowing procedures, has not been compelled to abort a single procedure. Awareness of the presence of aberrantly located carotid vessels comes from careful inspection of the small operative field, palpation of aberrant vessels intraoperatively, and cautious surgery.

Surgical Procedures for VPD

Partial obstruction, either temporary or permanent, of the velopharyngeal port is the unifying feature of most current operative management strategies for VPD. The choice of either of the 2 broad categories of options for VPD depends on the patient's specific diagnosis. The first option category is (1) lengthening the palate by retropositioning the velum, which can be achieved with a V-Y pushback procedure, an intravelar veloplasty,¹⁴ or a double-opposing Z-plasty;¹⁵ and (2) palatal re-repair.¹⁶ The second option category is reduction of the static opening between the nasal and oral pharynges,^17,18 which is considered a velopharyngeal-narrowing procedure. These may be accomplished with a pharyngeal flap or sphincter pharyngoplasty.

The pharyngeal flap creates a single subtotal central obstruction of the velopharyngeal port, leaving 2 open ports laterally. Alternatively, sphincter pharyngoplasty may be performed to diminish the cross-sectional area of the central port.

Pharyngeal flap

• Candidates for pharyngeal flap
• Lateral wall motion is important for effective valving after pharyngeal flap surgery.¹⁹ Performance of a pharyngeal flap procedure is most effective in patients with satisfactory lateral pharyngeal wall movement and sagittal or circular velopharyngeal closure patterns.
• The objective of the pharyngeal flap is to create a central static obstruction and to leave 2 lateral ports or openings, termed pharynges (see Images 5-7).
• Lateral openings should remain patent during breathing and nasal consonant speech production and closed during the production of oral consonants.
• Schoenborn originally published a description of this procedure in 1876. The pharyngeal flap was widely adopted in the 1950s and has been studied fairly extensively.
• Different types of pharyngeal flaps
• The pharyngeal flap has been modified widely, and variations in specific techniques abound. Key issues that stimulated the development of these modifications include (1) the appropriate width of the pharyngeal flap, (2) whether a superiorly or inferiorly based flap is more effective in achieving the ideal outcome, and (3) whether the flap should be lined with mucous membrane to prevent postoperative contraction/attenuation of the flap.
• Determination of the level of insertion and flap width may influence proper closure of the new lateral ports during speech. An excessively wide, nearly obstructive flap may induce untoward secondary consequences (ie, mouth breathing, hyponasality, sleep disturbances ranging from snoring to sleep apnea, retention of nasal secretions and mucus). Hypernasality may persist if the flap is too long and thin. Historically, flap width is determined at the time of surgery based on the surgeon's experience or preference. Many surgeons attempt to create a flap as wide as the field allows.
• Whether the flap is inferiorly or superiorly based has been the subject of lively debate among surgeons over the years, yet proof of significant differences between the 2 types is scant.²⁰ Currently, most surgeons favor a superiorly based flap. The disadvantages of an inferiorly based flap include length limitation and inferior tethering of the flap below the palatal plane and in the opposite direction of necessary motion for affecting velopharyngeal closure.²¹ Extrapolating from the information on failed sphincter pharyngoplasties, in which low flap placement correlated with failure, a superiorly based pharyngeal flap is preferred.²²
• If the flap is unlined, a broad, raw surface of pharyngeal tissue is left exposed after its elevation. Subsequent contraction (healing by secondary intention of unfulfilled mucosa) may diminish its efficacy. Thus, initial postoperative results may indicate improvement in velopharyngeal function, yet symptoms of the dysfunction may recur gradually thereafter. To reduce the tendency for contraction, "book flap" linings are usually raised from the nasal surface of the posterior velum and folded over to cover the unfulfilled surface of the flap (see Images 8-16).
• Another modification of the pharyngeal flap is the so-called lined pull-through procedure.²³ This involves demucosalization of the oral surface of the posterior soft palate that juxtaposes with the raw surface of the elevated pharyngeal flap. The author does not believe this is a sound operation, because, in his experience, it results in substantial downward migration/tethering and antagonizes normal velopharyngeal movement.
• Level of flap inset (affects outcome)
• The level of flap insertion is linked to surgical success. Insertion of a short, wide flap along the free margin of the soft palate may reduce the contraction of unlined flaps.
• Placing the flap at this level theoretically narrows the gaps between the base of the flap and the attached tonsillar folds where they merge with the pharyngeal wall.
• Presumably, this creates a velopharynx that is nearly completely obstructed and requires little contribution of movement from the lateral pharyngeal walls to achieve closure.
• Control of lateral port size
• Hogan devised a surgical technique to modulate the postoperative port size.²⁴ He introduced the concept of lateral port control in the 1970s, using indirect information of the size of the velopharyngeal port from differential nasal and oral airflow. Studies in the 1960s by Warren²⁵ and Warren and Devereau²⁶ corroborated this hypothesis and demonstrated that port size is related to the perception of nasal resonance. In 2003, Kummer et al more recently extended this concept.²⁷
• Hogan's technique involves placement of 10-mm² catheters that he assumed to be the crucial variable for anticipated normal resonance.²⁴ Although this technique may seem intuitive and logical, other uncontrolled variables such as the vagaries of wound healing, scarring, and postoperative migration of the flap lead this author to believe that port size cannot always be rigorously and reliably controlled.
• Tailoring the specifications of the pharyngeal flap to the patient's needs
• Whether appropriate flap width can be routinely determined intraoperatively remains unclear.
• In most cases of postpalatoplasty VPD, control of the flap width based on the morphology observed during the operation is ineffective. However, in cases of gross asymmetric closure patterns, focusing on correcting that asymmetry seems logical.^28,29 For example, patients with VPD secondary to hemifacial microsomia, stroke, or tumor resection may need specific skewing (tailoring) of flaps to affect closure.

Sphincter pharyngoplasty

The goal of sphincter pharyngoplasty is to narrow the central velopharyngeal orifice, thus minimizing airflow through the nose during speech. Theoretically, sphincter pharyngoplasty tightens the central orifice without creating lateral ports, resulting in an opposite configuration of the velopharynx compared with the pharyngeal flap.

Sphincter pharyngoplasty was first described more than 50 years ago, but it has only recently become the procedure of choice among many surgeons. Because of insufficient collation of data, a detailed description of risks, benefits, and long-term outcomes has not been confirmed. The original concept of sphincter pharyngoplasty was described by Hynes;³⁰ it has since been modified by others, including Orticochea.³¹ The procedure rearranges palatopharyngeus myomucosal flaps raised from the posterior tonsillar pillars, which are transposed to the posterior pharyngeal wall and to each other. This procedure may result in less airway morbidity than the pharyngeal flap and, conceptually, is more physiologic, although these impressions reflect the author's personal bias and remain unproven.³²

• Candidates for sphincter pharyngoplasty
• Sphincter pharyngoplasty may be an appropriate management option for patients with VPD who would not be treated with speech therapy alone and whose nasoendoscopy evaluations indicate a large-gap, coronal, circular, or bow-tie pattern of closure.
• Essentially, patients who demonstrate good velar elevation but poor lateral wall motion are good candidates for sphincter pharyngoplasty.
• Operative technique
• Pass a red rubber catheter transnasally and suture it to the uvula, and reflect the velum into the nasopharynx to achieve exposure of the posterior pharyngeal wall (see Image 17). Inspect the posterior pharyngeal wall for pulsations of aberrant carotid arteries.
• Next, plot lines of incision with indelible ink on both the anterior and, with the aid of a retractor, posterior aspects of the posterior tonsillar pillars, identifying the proposed myomucosal flaps (see Image 18). Infiltrate local anesthetic for hemostatic purposes. Beginning on the right and then repeating the same maneuver on the left, raise the posterior tonsillar pillar as a myomucosal flap, based cephalad (see Image 19). Elevate lateral palatopharyngeus myomucosal flaps to the height of attempted velopharyngeal closure, as documented on the preoperative speech videofluoroscopy images.
• Incise the posterior pharyngeal wall transversely at the proposed area of insertion in conjunction with the cephalad extent of the elevation of the flaps. The continuous cut extends from the superior end of the posterior limb of one lateral flap to the other and allows the lateral flaps to be fully inset. This design eliminates the bilateral fistulae inherent in Orticochea's original construction. Lay all sutures in sequence and subsequently secure from cephalad to caudad. Remove the red rubber catheter before securing knots.
• Attach the superior mucosa of the left flap to the mucosa of the superior incision of the posterior pharyngeal wall. Attach the caudal mucosa of the left flap to the superior mucosa of the right flap, overlapping the 2 flaps as described by Hynes. Attach the caudal mucosa of the right flap to the inferior mucosa of the posterior pharyngeal wall (see Image 20).
• Assist the integrity of the newly created sphincter by suturing the lateral flaps securely to one another and to the superior constrictor and pharyngobasilar membrane. Attempt to capture the mucosa, submucosa, and epimysium with each stitch to maximize its holding power. Approximate tissues without tension, and the close donor sites. Suture with 4-0 polyglactin. After construction of the sphincter pharyngoplasty, place an orogastric tube, aspirate gastric contents, and remove the tube.
• The central orifice of the sphincter pharyngoplasty port at the conclusion of the procedure should admit a small fingerbreadth (approximately 1 cm in diameter). A "tight" sphincter pharyngoplasty port usually measures approximately 0.5 cm in diameter, and a "loose" sphincter pharyngoplasty port usually measures approximately 1.5 cm in diameter.
• Long-term outcome of sphincter pharyngoplasty
• Riski et al demonstrated that the height of insertion appears to be a critical factor for success. They documented the emphasis on the importance of inset height for placement of the myomucosal flaps.²² In a follow-up study, Riski et al reported results in a large number of patients over a 15-year span.³³ Results showed a high success rate among patients who underwent sphincter pharyngoplasty before speech dysfunction developed fully. Success also seemed to correlate with patients who were younger than 6 years at the time of operation.
• Witt et al reported a study in which preoperative speech and instrumental assessments were separated to provide perceptual information and physiologic relationships. Only 18% of the patients involved in the study showed 100% resolution of hypernasality and nasal emission.³⁴ Approximately 30% of the patients developed hyponasality and/or obstructed speech and breathing patterns. Sphincter pharyngoplasty remains an effective treatment modality for VPD; however, the study does emphasize the need for further comparative data.

Posterior pharyngeal wall augmentation

Autogenous posterior pharyngeal wall augmentation is another method of treating resonance disorders, particularly in those patients with small velopharyngeal gaps. This procedure is used in various centers around the world with variable success. Operative technique involves raising a U-shaped posterior pharyngeal wall myomucosal flap to the tubercle of the atlas. The tip of the flap is connected by deep bites in the epimysium to the base of the flap. By securing the knots, the flap is rolled on itself, creating a soft tissue bulge or augmentation.

Autogenous posterior wall augmentation has been used in Europe and Russia but has not gained widespread acceptance in North America. Few data substantiate outcome of this procedure. In one report, results were analyzed with recorded (audio/video) perceptual, nasoendoscopic, and fluoroscopic standardized speech and airway evaluations, comparing preoperative and postoperative assessments.³⁵ A randomized master tape was presented in blinded fashion and random order to 3 skilled raters for independent assessment of numerous perceptual and instrumental parameters of speech. The raters were uninvolved in the care of the patients, and their intraobserver and interobserver reliabilities were known. Results of the extramural judgments of these parameters showed no statistically significant tendency for patients' speech to be rated as more normal after the augmentation procedure than before it.

Postoperative care 

• Patients are monitored overnight with pulse oximetry and are given oxygen by a nasal canula. They may resume a soft or liquid diet immediately.
• Most patients are discharged from the hospital after one night, although patients with 22q11 deletion often require at least 2 nights in the hospital.
• The author sees patients 3 weeks after surgery for a follow-up evaluation. In the meantime, parents are given information about sleep apnea and breathing signs that may be indicative of a problem.
• The team nurse communicates with parents by telephone, and a highly integrated and focused program of speech therapy usually resumes 3-6 weeks after surgery for VPD intervention.

Complications

Risks involved with surgical VPD treatment include acute obstructive sleep apnea, dehiscence (procedure failure), and a potential/theoretical risk of iatrogenic injury to anomalous internal carotid arteries.

Sleep disturbances as a consequence of sphincter pharyngoplasty may range from simple snoring to acute obstructive sleep apnea. Rarely is sleep apnea so severe as to require hospitalization. This adverse effect appears to occur in a substantial percentage of patients surgically managed for VPD, as suggested in a preliminary report by Witt et al in which the incidence was 13% of 58 patients observed.³²

Complete nasopharyngeal obstruction should be a rare complication, assuming that all raw surfaces were properly fulfilled at the primary pharyngoplasty. The author has not encountered it in his practice. He has seen patients in referral who presented with the unpleasant triad of sleep apnea/snoring, hyponasal resonance, and retained secretions/maxillary sinusitis (see Image 22).

Velopharyngeal surgery is still more of an art than a science. The goal is to create a subtotal obstruction that improves resonance but avoids airway morbidity. Still, in approximately 10% of cases, reoperation is necessary to treat residual hypernasality or nasal emission.

Axioms

• A finite number of patients will develop VPD, regardless of surgeon experience, palatoplasty technique, timing of operation, or early speech therapy intervention.^36,37,38
• Make sure the patient is managed by a team of specialists.
• Midface advancement may affect velopharyngeal function, particularly in persons with borderline function.
• Patients with 22q11 microdeletion (velocardiofacial syndrome) are notoriously difficult to treat; parents must be counseled carefully, usually on repeated occasions preoperatively, to temper their expectations about the outcome of interventions.^39,40,41
• Removing enlarged tonsils 3 months prior to velopharyngeal surgery makes the VPD surgical procedure easier.
• Place pharyngoplasty flaps at least as high as the atlas (C1), or higher if that is the place of attempted velopharyngeal contact as noted on preoperative speech videofluoroscopy images.
• Upon postoperative intraoral inspection, a well-placed pharyngeal flap is not visible.

Complex Problems Associated With VPD Research

Several studies have been published in support of each of the available options for management of velopharyngeal insufficiency; however, most of the data have not been validated by large numbers of patients, nor have these results been subjected to critical analysis. Most of these studies lack a multidisciplinary evaluation, standardized evaluation/treatment criteria, and methods for assessing surgical outcome.

For example, several different types of sphincter pharyngoplasties have been described, although they have commonly been grouped together as though they were the same. These procedures differ in terms of transposition of the flaps, use of muscle tissues, levels of insertion, and whether a synchronous pharyngeal flap is used. Other uncontrolled variables include the status of the tonsils and whether a full-thickness transverse cut is made in the posterior pharyngeal wall mucosa. This heterogeneity of sphincter pharyngoplasties explains some of the difficulty in describing postoperative outcomes.

Cleft palate populations, migratory patterns of treating physicians, and dogmatism among surgeons regarding the best technique are all inherently unstable. Additionally, the study designs often do not include rigorous documentation of the preintervention, peri-intervention, and postintervention states or the methodology for evaluation of the intervention. Achieving a high compliance rate from a patient population stratified for age, sex, socioeconomic factors, and number of surgical interventions is an arduous task. The outcome assessment instrument must be designed to allow analysis of intrarater and interrater reliabilities of all the extramural raters and, at the same time, not be so cumbersome and burdensome as to reduce compliance.

In the future, reporting outcomes based on clinical evidence are probably going to set the standards for cleft surgery. Surgeons who help manage postpalatoplasty velopharyngeal dysfunction will be judged by the evidence that their interventions are doing the most good for the most people at a price that patients and/or insurance agencies are willing to pay. Evidence-based practice is the integration of the best research evidence with clinical expertise and patient values. The "best evidence" comes from randomized clinical trials, which are expensive, time-consuming, and not always possible. Sometimes we must settle for good evidence, which may be the best evidence that is available. Nevertheless, the future demands good evidence, and as cleft surgeons and researchers, we need to supply that evidence.

Future diagnostic assessment and treatment modalities

Exciting new technologies are on the horizon, such as dynamic magnetic resonance imaging of the velopharynx, which may soon be available for clinical use. Magnetic resonance data can be reformatted to simulate endoscopy. Planar images may be converted to 3-dimensional volumes. While in its infancy, this technology may someday allow clinicians to feel as if they can actually go inside the anatomic structures they have scanned with "fly-throughs," focusing on specific pathologies. This has the potential of evolving into noninvasive endoscopy, assuming that it can meet or exceed the criteria standards currently available.⁴²

Importance of Palatal Fistulas in Management of VPD

Palatal fistulas are problematic for hygiene and speech. The contribution of fistulas to velopharyngeal dysfunction requires multidisciplinary assessment.

Recent lines of clinical and experimental evidence support the efficacy of decellularized dermal grafting as an adjunct to the closure of recurrent oronasal fistulas.^43,44,45 Early results with decellularized dermal grafts appear promising, when placed between the nasal mucosa and the levator veli palatine muscle, at decreasing the rates of infection, dehiscence, signs of rejection, extrusion, and fistula recurrence. All patients were followed for an average of 3 months. These authors have concluded that decellularized dermal graft should be considered for use in the treatment of recurrent oronasal fistula after cleft palate repair. Obviously, further clinical study is needed. This novel method offers promise as a simple and effective technique for tension-free closure of oronasal fistulas.  

"Medical Missions" and Velopharyngeal Surgery

A plethora of organizations provide humanitarian assistance to developing countries in which cleft care is not organized or coordinated. Most medical missions are undertaken on a short-term basis, so intermediate and long-term outcome assessments are not performed. Because velopharyngeal-narrowing procedures have significant potential airway morbidity, the author believes that they should not be performed in the setting of a foreign medical mission.

Multimedia

(Enlarge Image)

Media file 1: Schematic lateral view of the velopharynx illustrating anatomy.

(Enlarge Image)

Media file 2: Schematic bird's-eye view of the velopharynx illustrating directional movements of the representative closure patterns.

(Enlarge Image)

Media file 3: Palatal lift. Hard and soft palatal components are shown.

(Enlarge Image)

Media file 4: Palatal lift in situ.

(Enlarge Image)

Media file 5: Preoperative nasoendoscopic view of the velopharynx. One and 2 are the lateral pharyngeal wall, 3 is the velum, and 4 is a tonsil.

(Enlarge Image)

Media file 6: Postoperative nasoendoscopic view of the velopharynx, indicating an open pharyngeal flap as a central subtotal midline obstruction; 2 patent velopharyngeal ports are visible laterally.

(Enlarge Image)

Media file 7: Postoperative nasoendoscopic view of the velopharynx, indicating 2 lateral pharyngeal walls opposed against a pharyngeal flap to effect complete velopharyngeal closure.

(Enlarge Image)

Media file 8: Sutures are placed bilaterally in the soft palate to enhance visualization. A midline incision divides the soft palate to the posterior nasal spine.

(Enlarge Image)

Media file 9: Soft palate flaps are retracted.

(Enlarge Image)

Media file 10: An incision is made along the dotted line on the posterior pharyngeal wall down to the prevertebral fascia. A pharyngeal flap is created. A "book flap" incision that will line the lateral ports with mucous membrane is then made bilaterally on the nasal surface of the soft palate.

(Enlarge Image)

Media file 11: The pharyngeal flap is plotted with indelible ink and elevated to the prevertebral fascia. Two soft palate flaps are opened laterally.

(Enlarge Image)

Media file 12: The free inferior edge of the pharyngeal flap is sutured to the posterior edge soft palate.

(Enlarge Image)

Media file 13: Sutures are placed between the pharyngeal flap and the nasal edges of the soft palate. The raw surfaces arising from the origin of the pharyngeal flap are closed by simple approximation of tissue.

(Enlarge Image)

Media file 14: Two flaps from the soft palate used to cover the raw tissue of the pharyngeal flap are sutured to the base of the pharyngeal flap.

(Enlarge Image)

Media file 15: The oral side of the soft palate is sealed to conceal the pharyngeal flap.

(Enlarge Image)

Media file 16: Immediate postoperative view from the oral cavity.

(Enlarge Image)

Media file 17: Schematic of lateral view. A catheter has been passed transnasally and attached to the uvula.

(Enlarge Image)

Media file 18: Schematic showing proposed incisions (dashed lines).

(Enlarge Image)

Media file 19: Schematic showing elevation of both tonsillar pillar flaps.

(Enlarge Image)

Media file 20: Schematic showing rotation of palatopharyngeal flaps through 90°. It is ready for attachment to the posterior pharyngeal wall.

(Enlarge Image)

Media file 21: Schematic showing a completed sphincter pharyngoplasty. Flaps are overlapped and are sutured to each other and the posterior pharyngeal wall.

(Enlarge Image)

Media file 22: Complete nasopharyngeal stenosis.

Keywords

nasalance, airflow, nasal rustle, nasal turbulence, abnormal cavity coupling, acoustic energy, nasal resonance, grimace, hyponasality, airway evaluation, sphincter pharyngoplasty, postpalatoplasty speech dysfunction, VPD, velopharyngeal dysfunction, speech dysfunction, pharyngeal flap, sphincter pharyngoplasty, speech impediment, speech therapy, language therapy, mouth prosthesis, speech prosthesis, speech bulb, speech obturator, palatal lift, hypernasality, nasal emission, facial grimacing

The author wishes to acknowledge the two people who have had the biggest influence on his professional career, namely, Dr. Jeffrey L. Marsh, and Dr. Linda D'Antonio, who introduced me to Jeff. I hold them both in the highest admiration and esteem.

• Search for CME/CE on This Topic »

• Craniofacial Distraction Osteogenesis (Otolaryngology and Facial Plastic Surgery)
• Manifestations of Craniofacial Syndromes (Otolaryngology and Facial Plastic Surgery)
• Craniofacial, Distraction Osteogenesis (Plastic Surgery)
• Craniofacial, Orthognathic Surgery (Plastic Surgery)

• Predictors of Plagiocephaly Severity Identified
• Chorionic Villus Sampling May Raise Hemangioma Risk
• Adenotonsillectomy Reduces OSA in Infants and Toddlers

• Maternal Tobacco Use and Its Preimplantation Effects on Fertility: More Reasons to Stop Smoking
• Risk Factors and Treatment for Obstructive Sleep Apnea Amongst Obese Children and Adults
• Nurses Journal Scan, September 2008