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Address reprint requests and correspondence: Dhave Setabutr, MD, University of California Davis Department of Otolaryngology, 2521 Stockton Blvd., Suite 7200, Sacramento, CA 95817.
Velopharyngeal insufficiency can be a postoperative sequela or congenital. Velopharyngeal insufficiency is defined as a degree of nasality that interferes with resonance quality and comprehensibility. The diagnosis and preoperative evaluation is contingent on collaborative evaluation between the surgeon and speech-language pathologist. A thorough history and a complete head and neck examination including a videotaped nasopharyngoscopy optimize planning. Although each patient varies greatly in their presentation, we provide a review of the most commonly implemented procedures to improve velopharyngeal insufficiency and the best way to recognize which will likely improve a patient’s condition based on preoperative evaluation.
Velopharyngeal insufficiency is important to recognize in the long-term care of patients with a history of a cleft palate. Despite surgical repair of the cleft, approximately 20% of these patients demonstrate velopharyngeal insufficiency owing to either inadequate velar length following the repair or velopharyngeal incompetence due to poor muscle function.
Velopharyngeal insufficiency is defined as a degree of nasality that interferes with resonance quality and comprehensibility. This article describes the evaluation of these patients and discusses options for treatment.
Patient evaluation
Hypernasality typically becomes evident once speech becomes developed, and often caregivers or teachers initiate concerns regarding speech. A full evaluation is often impractical before 4 years of age owing to the absence of language development and patient cooperation. However, once a cooperative age is reached, the palate and oropharynx should be carefully examined noting tonsil size, the presence of any oronasal fistulas, and sites of previous surgeries. The presence of a zona pellucida of the soft palate and absence of a posterior nasal spine suggest a submucous cleft palate. Abnormal nasal air escape can be visualized with a dental mirror placed below the naris during nonnasal sounds.
Speech is best evaluated with a speech pathologist noting degree of overall intelligibility, articulation errors, and evidence of velopharyngeal dysfunction. The presence of hypernasality and hyponasality can at times be confusing. Occluding the nostrils producing hyponasality can aid in distinguishing the 2. After appropriate speech therapy, patients with intelligibility issues should be evaluated with a flexible nasopharyngoscopy and laryngoscopy following administration of topical lidocaine and oxymetazoline decongestant with a well-experienced speech-language pathologist. Vocal nodules and evidence of reflux are often seen in this patient population as well.
Velopharyngeal function is evaluated by placing the scope high in the nasopharynx via the middle meatus. The evaluation should include type of closure, coronal (most common), sagittal (least common), and circular, including the presence of Passavant ridge (horizontal contraction of the superior constrictor, which produces a bulge in the posterior pharyngeal wall). Lateral wall and palatal motion should be scored using the Golding-Kushner’s scale or similar
(Table 1). The presence of a midline palatal notch indicates a dehiscence of the levator veli palatini seen in submucosal cleft palates and can be noted after primary palate repair. The level of maximum closure, particularly as it relates to the adenoid bed also influences treatment. Ultimately, one needs to know where air is leaking and choose a surgical procedure directed there.
On nasopharyngoscopy, the palatal length, width, and tightness of closure are assessed. When insufficiency is present, the size and shape of the persistent defect should be noted. Some patients who may benefit from surgical intervention may have touch closure with bubbling with speech tasks. Air escape can be visualized at the level of the nasopharynx while asking the patient to say statements demonstrating velopharyngeal competence, such as “Sissy sees the sky,” and counting from “62 up to 68.”
An attempt is made to avoid sounds that typically have nasal escape, such as /m/, /n/, and /ng/. Using plosives, such as “Pop,” and “Cop,” can make further assessment, as these simple sounds promote closure. Using both more complex and lengthier sentences can further delineate function. This evaluation dictates the type of surgical repair. If a pharyngeal flap is indicated, port size should be determined at this time and documented in the child’s medical record (Table 2).
The 3 most common procedures used by cleft surgeons include the pharyngeal flap, sphincter pharyngoplasty, and Furlow palatoplasty. The pharyngeal flap is the original procedure of choice and is ideal for patients with good lateral wall motion where a relatively narrow flap can be used. The sphincter pharyngoplasty is ideal in a patient with excellent palatal motion and little lateral wall motion. The Furlow palatoplasty is ideal for patients who leak through a midline palatal notch or a shortened velum. There is debate among surgeons between pharyngeal flap and sphincter pharyngoplasty for those patients with little motion in any direction.
Pharyngeal flap
The patient should be screened for signs and symptoms of obstructive sleep apnea, and a polysomnogram should be used when there are concerns. If a patient does in fact have sleep apnea, severity may preclude proceeding with a pharyngeal flap. The goal of the pharyngeal flap is to create a central subtotal velopharyngeal obstruction, leaving 2 lateral ports for residual nasal airflow.
We typically recommend a staged procedure of tonsillectomy and adenoidectomy 3 months before the pharyngeal flap as respiratory disturbances can typically worsen after the flap. Some report proceeding without the need for a staged procedure.
The disadvantages of not removing the tonsils and adenoid include port obstruction by the tonsils or adenoid, and the adenoid pad limiting how high cephalad the base of the flap can be, typically missing the level of maximum velopharyngeal function.
Technique
The patient is placed supine in the Rose position. A shoulder roll is placed to provide adequate neck extension. Placing the patient in Trendelenburg position can assist with both decreased need for suctioning at the conclusion of the case and the surgeon’s ergonomics. It is important that a thorough examination and palpation of the posterior pharyngeal wall be done especially in patients with velocardiofacial syndrome to identify medialization of the carotid arteries that can occur in this subset of patients.
If the carotid becomes exposed, myomucosal coverage is necessary. The senior author typically injects the posterior pharyngeal wall with 0.25% bupivacaine with 1:200,000 of epinephrine. Adequate local injection can be indicated by evidence of mild hydrodissection of the mucosal plane. It is important to inject before proceeding with formal draping and instrument setup to allow for time for effective hemostasis by the vasocontrictor. With the use of a fine marking pen or methylene blue dye placed on a carved wooden tip of a cotton swab, one can then demarcate the lateral borders that will provide adequate width, taking into account contraction of the flap that will occur with healing. Narrow ports require that lateral incisions extend to the lateral gutter (junction of the posterior and lateral pharyngeal wall), whereas with wider ports, the lateral incision should be 5-8 mm from the lateral gutter. It is critical to avoid making the flap too narrow even if the inset is narrow as the flap contracts significantly. The distal demarcation will taper inferiorly to a triangular point and coincide with the site of attachment to the soft palate (or velum) (Figure 1). Riski et al
recommend positioning of the pharyngoplasty flaps with reference to the anatomical landmark of the first cervical vertebrate. The white prevertebral fascial plane determines the depth of the dissection. Hemostasis when necessary is controlled using spot electrocautery. During the procedure, it is important to intermittently release the Dingman retractor every 5-10 minutes to allowing lingual reperfusion preventing issues with postoperative obstruction from tongue edema.
Following adequate elevation, tension placed on the pharyngeal flap will allow the surgeon to test adequate release of the flap in reaching the velum.
Figure 1(A) The velum split sharply along the midline. (B) Posterior pharyngeal wall flap has been elevated with demarcation of the triangular mucosal flaps of the velum. (C) The elevated posterior pharyngeal flap is inset into the opened velum. (D) (1 and 2) Sagittal section depicting the inset and closure of the pharyngeal flap. (E) Completed pharyngeal flap.
Next, with the use of a #15-blade scalpel, the velum is split in the midline to the level of the hard palate (Figure 1). Using a forceps to pull carefully at the site of the uvula can assist with adequate tension and a clean incision. For better healing, the uvula is typically based on 1 side, unless it is bifid preoperatively. Elevation of the triangular nasal mucosa flaps is hinged posteriorly as outlined in Figure 1. Design of the triangular nasal mucosal flaps determines the port size.
The narrower the port, the more lateral the incision that will be made. The more inferior the incision, the more inferior the port will be. Care should be exercised as many ports heal inferiorly to the level of maximum velopharyngeal function. The distance from the lateral nasal mucosal flap to the lateral pharyngeal flap donor site will be equivalent to port size. This allows adequate attachment between the pharyngeal flap and the velum. Port sizes having been predetermined during the nasopharyngoscopy correlate with bilateral specific-sized endotracheal tubes through the nasal cavity, which are then visualized in the oropharynx (Figure 1). The endotracheal tubes are cut so that they end distal to the donor site. Next, 5 slowly absorbable 3-0 sutures are placed to be tied at the end of placement of all sutures. Sutures start in the midline oral mucosa passing out the raw nasal surface of the velum, through the raw surface ending on the mucosal surface of the donor flap. With a separation of 2-3 mm, the direction is reversed, passing from mucosa to raw of the donor flap, and raw to oral mucosa of the velum. Moreover, 2 additional sutures are placed lateral to the midline suture for a total of 5 sutures laterally securing the flap in place. The 2 nasal triangular flaps are sewn to the raw surface of the donor flap. The velum is approximated with vertical mattress sutures, which catch the midportion of the donor flap. The oral cavity is irrigated and suctioned dry. The senior author typically leaves the donor site to close via secondary intention, to prevent narrowing of the pharynx, which may increase the risk of obstructive sleep apnea. However, some surgeons may be inclined to reapproximate the edges with interrupted 3-0 absorbable sutures. From a potential sleep apnea risk standpoint, it is better to close the donor site from inferior advancement rather than from lateral advancement. Patients are admitted to the hospital and observed with continuous pulse oximetry. The endotracheal tube stents are removed on postoperative day 1. Patients are allowed a soft diet, and assurance of adequate oral intake is made before discharge.
Sphincter pharyngoplasty
Proceeding with a sphincter pharyngoplasty is ideal in patients who have excellent palatal movement and poor lateral posterior pharyngeal wall movement. The ultimate goal of the procedure provides patients with a smaller central oropharyngeal orifice.
Technique
The patient again is placed in Rose position and in slight Trendelenburg position. A Dingman retractor is placed so that there is an adequate view of the posterior oropharynx. Bupivacaine 0.25% with 1:200,000 of epinephrine is injected submucosally along the velum, posterior tonsillar pillars, and the posterior pharyngeal wall. With the use of a sharpened end of a Q-tip and methylene blue dye, the proposed incisions are marked as described in Figure 2. The myomucosal flaps are elevated in a cephalad direction using a #15-blade scalpel; the palatopharyngeus and likely some superior constrictor muscle with mucosa are incised sharply with the assistance of a Debakey forceps for retraction. The flaps are then elevated to a level of maximal velopharyngeal function determined on prior nasopharyngoscopy. This same level is marked on the posterior pharyngeal wall. If adenoids are present and interrupt the point of maximal closure, they need to be removed, and it may be done intraoperatively with a debrider or preoperatively at a separate setting. The posterior pharyngeal wall is incised sharply with a #15-blade scalpel through only mucosa to receive the 2 posterior tonsillar pillar myomucosal flaps. If the superior constrictor is completely transected, the receiving bed will migrate inferiorly. The superior edge of the left flap is sutured with interrupted 4-0 absorbable sutures to the superior edge of the incision of the posterior pharyngeal wall. The superior edge of the right-based flap will be sutured in a similar fashion to the inferior border of the left-based flap creating overlapping flaps. The inferior border of the receiving bed is closed to the right flap. It is important to include all layers during this suturing technique to assist in closure. The port size is determined by the amount of tension, which narrows the central port. The completed sutures are portrayed in Figure 2. The central orifice of the sphincter pharyngoplasty ranges from 0.5-1.5 cm in diameter depending on the preoperative workup. The patient is awakened and extubated in the operating room. Patients typically are admitted overnight and discharged the following morning if they have adequate oral intake.
Patients with a primary or secondary (following cleft palate repair) submucosal cleft palate or a slightly shortened palatal length are good candidates to undergo a Furlow palatoplasty.
Technique
The patient is placed in the Rose position and in slight Trendelenburg position. A Dingman retractor is placed inside the oral cavity and suspended on a rolled blue towel that is placed on the patient’s chest. Bupivacaine 0.25% with 1:200,000 of epinephrine is used for hemostasis and local anesthesia. After adequate visualization of the soft palate, a Z-plasty is designed along the midline of the palate using methylene blue dye as seen in Figure 3. The lateral extent of the Z lies over the hamulus on both sides creating slightly asymmetric flaps. A reverse nasal layer Z-plasty is planned to appose the oral mucosal layer (Figure 3). It is important to note that the oral mucosa containing glands and fibrofatty tissue is quite thick (1.5-3.0 mm) and the nasal mucosa is paper-thin as the mucosa is directly adherent to the muscle. A #15-blade scalpel is used to make the midline incision and to incise the left oral mucosa. A right-angle Beaver (Beaver-Visitec International, Inc, Waltham, MA) blade is then used to dissect the oral mucosa and muscle from the nasal mucosa and hard palate.
Figure 3(A) Depiction revealing planned incisions for Furlow flap. (B) Opposing nasal layers are demarcated following elevation of the oral layer. (C) Planned rotation of flaps. (D) Closure complete of nasal layer. Planned opposition of oral layers. (E) Completed Furlow palatoplasty.
The goal is to elevate the mucosa, fibrofatty tissue, and levator veli palatini muscle in 1 complete unit leaving the thin nasal mucosa intact. Laterally, tenotomy scissors are used sharply to dissect the levator muscle toward the eustachian tube. As the dissection moves superiorly, blunt dissection is favored over sharp dissection. Because innervation and blood supply arise from lateral and posterior direction, finesse should be used during dissection to allow appropriate repositioning. The left myomucosal flap is dissected until it easily crosses midline and ideally reaches the opposite side. The right mucosa and fibrofatty tissue flap are elevated leaving the levator veli palatini muscle attached to the nasal layer. Back elevation over the hard palate in the midline and on the left side will allow a tension-free closure. The 2 nasal limbs are made with scissors or a knife. On the right, spreading tenotomy scissors are used to bluntly free the levator muscle allowing repositioning.
Starting anteriorly, the nasal layer is closed with 4-0 dissolvable sutures in interrupted fashion. The remaining nasal layer is then closed. Beginning anteriorly, the oral mucosal flap is closed with special care in the midline. The right myomucosal flaps is sutured laterally overlapping the levator muscle. Any dead space between the muscles can be closed with coaptation sutures. The opposed oral mucosal layer is then reapproximated with 3-0 dissolvable sutures in interrupted fashion. The critical tension-free mucosal closure is midline. As in all Z-plasties, the increase in length of the palate comes from narrowing laterally. Care is taken to ensure the incisions in the midline are closed without tension. If there is significant tension, occasionally a small portion of the lateral incision can be left open to heal by secondary intention as there are no overlapping incisions.
The wound is irrigated with saline and suctioned dry. Children are admitted overnight, and oral intake is verified before discharge; adult patients typically can be typically discharged the same day.
Conclusion
Velopharyngeal insufficiency remains a common problem that otolaryngologists should be readily equipped to treat. A coordinated relationship between surgeon and a speech-language pathologist allows adequate identification of appropriate patients. Evaluation is key to determine the risks and benefits as well as success in treating a patient’s speech pathology. Those with velocardiofacial syndrome are particularly at risk for medialization of the carotid arteries and should be identified before surgery. Postoperative speech therapy and joint reevaluation with a speech-language pathologist will allow successful outcomes.
References
Kummer A.W.
Cleft Palate and Craniofacial Anomalies: Effects on Speech and Resonance. ed 2. Delmar Cengage Learning,
Clifton Park, NY2008
0 = no movement, 0.5 movement to midline.
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