Velopharyngeal Insufficiency Treatment & Management

Speech therapy

Speech therapy improves velopharyngeal function when velopharyngeal dysfunction (VPD) is minimal or due to articulation errors and in postoperative patients. Compensatory articulation techniques secondary to VPD also can be corrected with speech therapy. However, in patients with a specific anatomic deficiency that precludes adequate closure of the velopharynx, speech therapy cannot replace surgery.

Upon completion of speech and language testing, the SLP determines if VPD and hypernasality are related to articulation errors or if the condition is phoneme specific. If either is the case, the VPD usually is not related to structural abnormalities, and correction with speech therapy most likely is possible.

VPD also can be a result of decreased muscle tone in the oral musculature and soft palate. Decreased muscle tone can be observed on fiberoptic nasoendoscopy when the soft palate closes inconsistently or when closure appears slow in connected speech. ^[3] A period of speech/language therapy focusing on improving overall oral motor skills and improving strength and elevation of the velum may be able to correct VPD in this case.

Visual feedback

In some children, especially those with hearing impairments, visual feedback can assist in therapy to improve VPD. Several devices are available to assist with this method. Simple tools (e.g., cold mirror, paper paddle) can serve to show the patient when nasal escape occurs. Other devices are commercially available, such as the See-Scape, which is placed at the nose and causes a ball to rise when airflow is nasal rather than oral.

A more sophisticated method is the use of a Nasometer, which graphically displays a ratio of oral sound energy to nasal sound energy. The visual readout can help the therapist and patient develop compensatory techniques to reduce nasalance. In older children, videotaped nasopharyngoscopy has been used to achieve the same goals.

Nasal continuous positive airway pressure therapy

Continuous positive airway pressure (CPAP) therapy is beneficial for patients whose VPD seems related to oral motor issues or velar weakness rather than structural problems of the velum. CPAP therapy is a palate-strengthening program using CPAP equipment, including a nasal mask, that is carried out 6 days per week for 8 weeks in the patient's home. With the mask in place, the patient repeats a series of consonant-vowel combinations and sentences designed to cause the velum to open and close against air pressure from the nasal mask. CPAP pressures are increased on a regimented schedule, producing exercises similar to a weightlifting program for the soft palate.

CPAP therapy has the disadvantages of being very regimented and requiring cooperation and participation for up to 24 minutes, 6 days per week, which is difficult for young children. Wearing the nasal mask and speaking with nasal air pressure also can be frightening for some young children. Furthermore, this treatment program is still relatively new and needs further research to determine its effectiveness.

Prosthetics

These devices are helpful when surgery is contraindicated or the cause of VPI is neuromuscular in nature or as a temporizing measure until surgery can be performed.

Obturators can substitute for tissue deficiency and are attached to the palate or teeth. In certain cases, the obturator can be downsized gradually so that the native tissue, if adequate in bulk, can strengthen over time and compensate for the decreasing obturator size.

Palatal lifts are used when adequate palatal length exists but dynamic motion of the palate is poor due to neuromuscular etiologies. Palatal lifts reduce the distance the palate must traverse to produce adequate closure. ^[4]

An additional option is a nasal valve, an appliance fitted to each nostril. A one-way valve on either side allows the patient to breathe in through the nose, but stops nasal airflow with exhalation or speech.

As noted previously, the primary indications for surgical intervention include a structural defect of the velum or a functional problem that results in poor or inconsistent velar closure.

Maximum benefit can be achieved when surgical technique takes advantage of whatever native velopharyngeal function exists. Information obtained during objective evaluation via physical examination, nasopharyngoscopy, and/or multiview video fluoroscopy greatly aids in determining how best to accomplish velopharyngeal closure. Determination of a patient's predominant VPC pattern directs the surgeon to the treatment most appropriate for the patient.

Surgical techniques are illustrated in the videos below.

Velopharyngeal closure pattern

Several patterns of VPC have been described based on nasoendoscopy and VF examinations. The type of closure pattern is determined by the relative contribution of the palate and lateral pharyngeal walls to closure of the velopharyngeal sphincter.

Four basic types of closure patterns, coronal, circular, circular with the Passavant ridge, and sagittal, are used to describe velar closure as seen in the image below. Defining the type of closure pattern is important when considering surgical intervention for correction of VPD.

See the list below:

• Coronal: The most common closure pattern, coronal closure is present in 55% of patients with normal velar function. The major contribution to closure is from the soft palate as it contacts a broad area of the posterior pharyngeal wall. Little medial motion of the lateral pharyngeal walls occurs. A coronal closure pattern is often present with an enlarged adenoid pad.

• Circular: Present in approximately 20% of individuals with normal closure, circular closure involves contribution from both the soft palate and the lateral pharyngeal walls. This results in a closure that resembles a circle getting smaller.

• Circular with the Passavant ridge: This closure pattern occurs in 15-20% of the population and is a circular pattern that involves anterior motion of the posterior pharyngeal wall (known as Passavant's ridge).

• Sagittal: The least common pattern, sagittal closure is prevalent in 10-15% of people. Here, palatal elevation is minimal. The main contribution is from medial motion of the lateral pharyngeal walls. This is the pattern seen most commonly in patients with persistent VPD following repair of a cleft palate.

Determination of a patient's closure pattern is clinically significant, as is the determination of the vertical level of closure. The type of closure pattern determines the type of surgical intervention, because surgical intervention is most effective when it targets the point of maximal pharyngeal motion.

Tonsillectomy and adenoidectomy

Another preoperative consideration is the need for adenoidectomy and/or tonsillectomy. In most instances, preoperative adenoidectomy is necessary for either pharyngoplasty or pharyngeal flap. Indications for preoperative tonsillectomy vary.

In the case of pharyngoplasty, adenoidectomy makes room for placement of the lateral pharyngeal wall flaps. Failure to remove the adenoid makes attachment of the flaps to the posterior pharyngeal wall difficult. The need for tonsillectomy before pharyngoplasty is determined by the degree of obstruction. This determination is somewhat subjective and based on the size of the tonsils and whether they interfere with the raising of the lateral pharyngeal wall flaps. Leaving the tonsils in place often helps preserve the posterior tonsillar pilars, which are used to create the pharyngoplasty flaps.

With pharyngeal flaps, removing both the tonsils and adenoid often is necessary. Placement of a pharyngeal flap makes subsequent adenoidectomy difficult if not impossible, and residual adenoid tissue may obstruct the lateral pharyngeal ports. Similarly, the tonsils may obstruct the pharyngeal ports from below, so their removal typically is recommended.

Other considerations

One factor to consider in preoperative planning is whether breathing is obstructed preoperatively. Placement of a pharyngeal flap increases the degree of nasal airway obstruction and may worsen any preexisting obstructive sleep apnea. This is especially true in patients with Pierre Robin syndrome, in which retrognathia may result in upper airway obstruction. In such instances, consideration should be given to pharyngoplasty instead of pharyngeal flap, since the risk of worsening airway obstruction postoperatively is lessened with pharyngoplasty.

The location of the internal carotid arteries as they traverse the lateral pharynx is also an important consideration, especially in patients with velocardiofacial syndrome. In patients with VCF syndrome, the carotid artery can take a more medial position, placing it at risk of injury during pharyngeal surgery. Preoperative evaluation with contrast-enhanced CT scan or magnetic resonance angiography can settle this issue.

Following is an overview of the 3 main surgical approaches to velopharyngeal corrective surgery, which are pharyngoplasty, pharyngeal flap, and posterior pharyngeal wall augmentation.

Pharyngoplasty

This approach is advantageous when coronal or circular closure is present and lateral pharyngeal wall motion is deficient. The goal is to develop a more functional sphincter by improving the dynamics and bulk of the velopharyngeal tissues and by tightening and reducing the size of the velopharyngeal opening.

Hynes was the first to describe pharyngoplasty in 1950 as the elevation of 2 superiorly based flaps comprising the right and left salpingopharyngeus muscles and overlying mucosa. The flaps were rotated 90° and sutured to the mucosal edges of a transverse incision made across the nasopharynx just below the level of the tori tubariae.

In 1968, Orticochea described a modification of the pharyngoplasty procedure using 2 superiorly based flaps comprising the posterior tonsillar pillars with the underlying palatopharyngeus muscles. ^[5] A small inferiorly based posterior pharyngeal wall flap then was elevated, and the 2 lateral flaps were rotated 90° and inserted onto the posterior flap. Jackson further modified this method by sewing the lateral flaps onto a superiorly based posterior pharyngeal wall flap, allowing higher placement of the lateral wall flaps.

As noted above, the need for adenoidectomy and possible tonsillectomy is determined preoperatively. Adenoidectomy is performed several weeks (minimum 6 weeks) preoperatively to allow for healing of the velopharyngeal sphincter. If the tonsils are significantly enlarged so that they may make raising the palatopharyngeal flaps difficult, they must be removed. Velar closure is then reassessed following adenoidectomy (and tonsillectomy) as the muscular dynamics may have changed.

Other technical considerations include recognizing that more superiorly the palatopharyngeal flaps are elevated, the greater the degree of velopharyngeal obturation. If this is excessive, an obstructive pattern of breathing can develop. Even if the obturation is not excessive, some patients still develop some mild temporary obstructive sleep patterns in the immediate postoperative period. For this reason, most surgeons place a nasal trumpet through the sphincter intraoperatively and remove it the next morning.

Several studies have reported a success rate (ie, correction or significant reduction in hypernasality) from 78-100% with pharyngoplasty. The incidence of postoperative hyponasality is estimated to be 12-17%.

One study reported a 10-year experience on the efficacy of sphincter pharyngoplasty for velopharyngeal insufficiency repair, using need for surgical revision as the primary outcome measure. Six (13%) of 46 patients required revision, achieving a single-procedure success rate of 87%. With a single revision, 100% success was achieved. Further, they reported that compared with sphincter pharyngoplasty alone, Furlow palatoplasty at the same time as sphincter pharyngoplasty may lead to improved outcomes. Further study is needed to clarify this relationship. ^[6]

Pharyngeal flap

This is the preferred method in patients with good lateral pharyngeal wall motion who have a persistent central gap due to poor palatal motion, as is common following repair of a cleft palate. The procedure aims to develop a central flap of tissue to obturate the midline of the pharyngeal port and decrease the degree of air escape into the nasal cavity.

Schoenborn first reported this procedure in 1876 when he described an inferiorly based posterior wall flap sutured to the nasal surface of the soft palate. He later modified this procedure by using a superiorly based flap when he found that an inferiorly based flap tended to contract and tether the palate downward over time, worsening the patient's VPI.

The flap is raised down to the level of the prevertebral fascia, creating a tissue flap of superior constrictor muscle with its overlying mucosa. This flap is raised superiorly so that its base is at the level of the arch of C1. Typically, splitting the soft palate in the midline is necessary to provide better access to the nasopharynx during creation of the flap. The flap then is sutured to the nasal surface of the palate by incorporating it into the split repair.

Alternatively, the flap can be inset into the palate by performing a fish-mouth incision on its nasal surface. Raising a longer flap than necessary is advantageous to accommodate any future flap contracture. Red rubber nasal trumpets (4 fr) are placed through the resulting lateral ports to alleviate any immediate postoperative obstructive breathing patterns.

One large series of 500 patients reported normalization or resolution of hypernasality in 90% of patients. Failure to improve is related to inadequate flap width either by design or due to contracture. Conversely, a flap that is too wide narrows the lateral ports and produces hyponasal speech. Postoperative airway obstruction is most likely to occur within the first 24 hours and in one series resolved within 2 days in more than 90% of patients.

A single-institution study by Hon et al indicated that use of a superiorly based pharyngeal flap for VPI can safely and effectively be combined with tonsillectomy for hypertrophied tonsils. Of the 88 patients in the study, 18 (20%) underwent the simultaneous procedure, with none of the patients in the combined or flap-only groups suffering immediate postoperative complications (eg, upper airway obstruction, bleeding complications) that required reoperation. One patient in the combined group experienced pharyngeal flap dehiscence necessitating flap revision. Regarding efficacy, 58.3% and 75% of the patients who underwent simultaneous surgery achieved elimination of nasal airway emission and hypernasality, respectively. However, a significantly higher number of narcotic administration occurrences took place in the combined group than in the flap-only patients. ^[7]

Posterior wall augmentation

This approach is appropriate in the presence of a persistent gap in the central velopharyngeal port measuring at most 1-3 mm. It also is indicated when the patient can achieve touch closure that is not tight enough to prevent air escape with high oral pressure. Persistent postadenoidectomy VPI is also an appropriate indication for this procedure. Both autogenous tissue and foreign implants have been used for this approach.

A superiorly based pharyngeal flap incorporating the superior constrictor is raised down to the prevertebral fascia. The superior extent of elevation is defined just above the point of maximal closure. It then is buckled onto itself and sutured in place. Initially good results can become less favorable as the flap can atrophy with time, and this procedure is recommended only for small gaps.

A wide variety of implantable materials has been used to augment the posterior wall. Problems with extrusion, migration, resorption, and infection have been reported. Silicone has been noted to have a high extrusion rate. Teflon is not advised for use in the pharynx due to concerns over the risk of injection into large blood vessels. Both silicone and Teflon also have a tendency to migrate inferiorly along the prevertebral fascia. Autologous tissue, such as fat or rolled dermis, has been less problematic but tends to resorb with time.

More recent reports have described the use of calcium hydroxyapatite injected into the posterior pharyngeal wall. This technique has been demonstrated to have a reasonably high success rate with no migration or extrusion of the injected material. Polyethylene implants imbedded into the posterior pharynx has also demonstrated success in some adult patients.

For any of theses procedures, suturing is performed with 3-0 or 4-0 Vicryl sutures because chromic sutures tend not to provide sufficient holding strength and may result in repair dehiscence. Mucosal closure may be performed with chromic sutures as long as closure tension is not too great.

Immediate postoperative concerns deal mainly with upper airway obstruction. Placement of nasal trumpets overnight is helpful in management of airway obstruction while the patient is still under the effects of anesthesia. A 2-0 suture placed through the tip of the tongue and used to protrude the tongue can be helpful in temporary airway management in the postanesthesia recovery area.

Patients are admitted overnight primarily for airway management and intravenous hydration. Discharge home is on the first postoperative day as long as oral diet is tolerated and pain control by mouth is adequate.

Pain management postoperatively typically is limited to narcotics such as acetaminophen with either codeine or hydrocodone. Stronger narcotics, such as morphine or meperidine, may cause increased sedation worsening airway obstruction.

Once the patient is tolerating oral fluids and any postoperative nausea has cleared, the patient resumes a diet of thick liquids and soft foods. With pharyngoplasty, a diet similar to that for tonsillectomy patients is used and may include most foods as tolerated. Patients undergoing a pharyngeal flap procedure follow dietary restrictions similar to those for patients undergoing palatoplasty, since the soft palate is divided and repaired. Typically, these patients are restricted to thick liquids or soft foods (eg, mashed potatoes) until the palatal incision has healed (usually 10-14 days).

The initial follow-up appointment is at 7-10 days, at which time the wound is examined for proper healing. A determination regarding resuming a regular diet also is made at this time. Snoring and upper airway obstruction, if present at this point, usually resolve over the next several weeks as edema decreases and flap shrinkage occurs.

At 6 weeks, healing is usually complete, and a speech and resonance reassessment is performed. Surgical correction of structural defects in the velopharyngeal port does not necessarily change function, and articulation problems may persist following surgery. Based on the postsurgical speech and resonance evaluation findings, speech therapy is resumed at this point as necessary.

Pharyngoplasty

More superiorly raised palatopharyngeal flaps lead to a greater degree of velopharyngeal obturation. If this is excessive, an obstructive pattern of breathing can develop. However, even if the obturation is not excessive, some patients still develop some mild temporary obstructive sleep patterns in the immediate postoperative period. For this reason, most surgeons place a nasal trumpet through the sphincter intraoperatively and remove it the next morning.

Long-term considerations are shrinkage of the flaps and nasopharyngeal scarring. Over time, the flaps may shrink, reducing the bulk of the repair and altering speech results. Patients with return of velopharyngeal escape over time may be a candidate for flap augmentation with fat injection. ^[8] Nasopharyngeal stenosis may also result, especially if the dissection is carried up onto the soft palate. Resultant upper airway and nasal obstruction may require revision pharyngoplasty with tissue grafts.

Pharyngeal flap

Failure to improve is related to inadequate flap width either by design or due to contracture. Conversely, a flap that is too wide narrows the lateral ports and produces hyponasal speech. Postoperative airway obstruction is most likely to occur within the first 24 hours and in one series resolved within 2 days in more than 90% of patients.

A study by Campos et al indicated that cleft palate repair with a pharyngeal flap may not be associated with obstructive sleep apnea. The study involved 42 middle-aged patients who underwent cleft palate repair, including 22 whose procedure employed a flap and 20 whose repair did not. Although the majority of patients in the study were diagnosed with obstructive sleep apnea, the rate of occurrence did not differ significantly between the flap and nonflap groups (77% and 60%, respectively). The investigators suggested that the occurrence of sleep apnea may have been associated with the patients’ advancing age and with congenital upper airway abnormalities. ^[9]

Pharyngoplasty

Several studies have reported a success rate (ie, correction or significant reduction in hypernasality) between 78-100% with pharyngoplasty. The incidence of postoperative hyponasality is estimated to be 12-17%.

Pharyngeal flap

One large series of 500 patients reported normalization or resolution of hypernasality in 90% of patients.

A literature review by Wagner et al of VPI associated with 22q11.2 deletion syndrome found that treatment with a posterior pharyngeal flap resulted in successful outcomes in 85% of patients. ^[10]

A retrospective study by Rogers et al of 19 patients with VPI, 18 of whom had a history of cleft palate, found significant speech improvement following surgery using a wide, superiorly based pharyngeal flap. ^[11]

A retrospective study by Setabutr et al indicated that in patients who undergo pharyngeal flap surgery, older age at the time of surgery is a risk factor for revision. The study, of 61 patients who underwent the pharyngeal flap procedure, found improved speech postoperatively, with the mean nasal air emission score in patients being reduced from 2.0 to 0.8 and the mean resonance being reduced from 2.4 to 0.9. The revision rate was 20% (12 patients), with age being the only covariate found that significantly correlated with a greater chance of revision surgery. ^[12]

A study by Bohm et al indicated that in patients with velopharyngeal insufficiency (VPI) related to 22q11.2 deletion syndrome, concomitant palatoplasty and sphincter pharyngoplasty produces comparable speech outcomes to pharyngeal flap surgery. The two procedures produced significantly better results than sphincter pharyngoplasty alone, although outcomes from all three operative approaches were similar with regard to complication rates and the need for revision surgery. ^[13]