Velopharyngeal insufficiency

Velopharyngeal insufficiency (VPI) is a failure of the body's ability to temporarily close the communication between the nasal cavity and the mouth, because of an anatomic dysfunction of the soft palate or of the lateral or posterior wall of the pharynx.

The effect of such a dysfunction leads to functional problems with speech (hypernasality), eating (chewing and swallowing), and breathing.[1] This gap can be treated surgically, although the choice of operational technique is still controversial.

The terms velopharyngeal "incompetence", "inadequacy" and "insufficiency" historically have often been used interchangeably, although they do not necessarily mean the same thing (sense distinctions can be made but sometimes are not). Velopharyngeal insufficiency includes any structural defect of the velum or pharyngeal walls at the level of the nasopharynx with insufficient tissue to accomplish closure, or there is some kind of mechanical interference with closure.[2] It is important that the term insufficiency is used if it is an anatomical defect and not a neurological problem.

Velopharyngeal insufficiency (VPI) can be caused by a variety of disorders (structural, genetic, functional or acquired) and is very often associated with a cleft palate. Abnormal physiological separation of the oropharynx from the nasopharynx can lead to VPI and hypernasality.[3]

Anatomy

Diagnosis of velopharyngeal insufficiency

An incorrect diagnosis can lead to insufficient differential management. Most surgeons have their own vision on diagnosis and differential management. If it is thought, for instance, that palatal length is the only variable necessary for achieving normal closure, differential management will be insufficient. Therefore, differential diagnosis is important, because otherwise treatment of the velopharynx may not be appropriate.

There is not one single operative approach to VPI because anatomical structures diverge a lot between individuals.[4] With diagnostic tools the surgeon is able to decide which technique should be used based on the anatomical situation of the individual. The goal of every operation is to achieve the best possible result with the technique assigned to each individual case.[4]

Several methods can be used to diagnose velopharyngeal inadequacy.

Speech analysis

The first thing that is evaluated is the quality of speech of the patient, also known as speech analysis.[5][6] To come to the right diagnosis this is the gold standard in VPI evaluation. The speech scientist listens to the voice, articulation, motor speech and the velopharyngeal function of the patient. The main symptom is hypernasality of the voice. The patient is unable to create normal resonance because of nasal air emission.

Nasometry

These different emissions can be measured with nasometry. Nasometry is a test which calculates a ratio between the nasal and oral sound emissions. The ratios of the patient will be compared with a normal ratio and standard deviation. These ratios will help determine whether the operation was a success. Preoperative ratios will be compared with postoperative ratios.[7]

The physician also examines the patient for obstructive sleep apnea syndrome (OSAS). When this is positive, the patient will be treated for OSAS first. When there is no sign of oral sleep apnea the physician will conduct a speech analysis. If the patient has an indication for surgical treatment, the next step will be visualization of the mouth and pharyngeal cavity.[6] Often visualization is combined with audiometry or speech analyzation.

Nasoendoscopy

Nasoendoscopy is a non-radiographic technique in which the physician uses a scope to enter the mouth of the patient. Usually the examiner uses a flexible scope, but in certain situations a rigid scope is used. Nasoendoscopy provides an overview of the anatomy of the velopharynx during phonation. With nasoendoscopy the vocal tract but especially the soft palate and the lateral wall of the pharynx can be visualized.[8] Not only the location but also the movement can be visualized with nasoendoscopy.[9]

There are some limitations to nasoendoscopy. Firstly it is hard to get an overview with nasoendoscopy with a rigid scope in small children,[6] especially when there are abnormalities or obstructions in the nasal cavity, which are frequently found in children with a history of cleft palate.[9] Secondly the nasoendoscope can cause irritations of the mucosa when the child does not cooperate.[6]

Videofluoroscopy

Multiview videofluoroscopy is a radiographic technique, mostly to demonstrate the lateral and posterior wall of the pharynx. This is a questionable technique considering these children undergo radiographic examinations frequently. Also known is that children are more sensitive to radiographic examinations than adults. Most of the time barium is used in multiview videofluoroscopy. Besides the fact that videofluoroscopy provides an overview of the lateral and posterior walls of the pharynx, this technique also provides information about the length and movement of the soft palate, the posterior and the lateral walls.[8][10]

A limitation of multiview videofluoroscopy is the possibility of misinterpreting certain shapes of gaps and anatomic structures.[9][11]

The most frequently used diagnostic tools are videofluoroscopy and nasoendoscopy. Some studies conclude that the first step in the process of diagnosis is videofluoroscopy in combination with nasometry.[6] Other studies show a favour for nasoendoscopy.[9][12] But in general there is no preference for which tool should be used as a standard. Most studies conclude that it is necessary to make an individual decision on which diagnostic tool should be used.[6][9][12]

Magnetic resonance imaging

A relatively new approach in the diagnosis is magnetic resonance imaging (MRI), which is noninvasive. MRI uses the property of nuclear magnetic resonance to image nuclei of atoms inside the body. MRI is non-radiographic and therefore can be repeated more often in short periods of time.[7] In addition, different studies show that the MRI is better as a diagnostic tool than videofluoroscopy for visualizing the anatomy of the velopharynx.

On the contrary there are still a few limitations of the MRI. Firstly artefacts can be shown on the images when the patient moves while imaging. Also artefacts will also be shown if the patient has orthodontic appliances. Secondly the MRI is limited in children who are claustrophobic.[12][13]

Furthermore, in the MRI scanner movement of the sphincter leads to artefacts on the images. Therefore, nasoendoscopy is still needed for information about the sphincter’s movement.[13] Finally, the MRI is a more expensive diagnostic tool than the combination of nasoendoscopy and videofluoroscopy.

Because of these limits MRI is currently not widely used. However, MRI is used for a “bird's eye view” of the child in the planning of the operation, but not in the progress of diagnosis.

Treatment of velopharyngeal insufficiency

Physical therapy

The main objective of physical treatment is to achieve adequate velopharyngeal (VP) function and normal oral-nasal resonance.

Speech scientist

The patient can learn to pronounce the words better with the help of a speech scientist. Also a speech scientist can help a patient to learn how to use the VP-port after surgery. Only if the VP mechanism is not working properly after surgery, speech therapy will be of little improvement.[2]

Operation techniques

Inferior based pharyngeal flap
Superior based pharyngeal flap

Normally when the patient has VPI, first of all the palatum will be closed. If speech is not as aimed, palatoplasty will take place. This is an operation in which the velopharyngeal port is narrowed down and functionally improved.

Velopharyngeal insufficiency remains in 5-20% of the patients who underwent surgery for closing the palatal cleft.[11] Therefore, a secondary operation is necessary. Nowadays the procedure that is chosen the most from the palatoplasties is the pharyngeal flap or sphincter palatoplasty.[2]

Pharyngeal flap

When a pharyngeal flap is used, a flap of the posterior wall is attached to the posterior border of the soft palate. The flap consists of mucosa and the superior pharyngeal constrictor muscle. The muscle stays attached to the pharyngeal wall at the upper side (superior flap) or at the lower side (inferior flap).[11] The function of the muscle is to obstruct the pharyngeal port at the moment that the pharyngeal lateral walls move towards each other.[2][11] It is important that the width and the level of insertion of the flap are properly constructed, because if the flap is too wide, the patient can have problems with breathing through the nose, which can result in sleep apnea.[14] Alternatively, a postoperative situation can be created with the same symptoms as before surgery. Some complications are possible; for example, the flap's width can change because of contraction of the flap. This results in a situation with the same symptoms of hypernasality after a few weeks of surgery.[2] Also a fistula can occur in 2.4% of the cases.[2][15]

Sphincter palatoplasty

Sphincterplasty

When the sphincter pharyngoplasty is used, both sides of the superior-based palatopharyngeal mucosa and muscle flaps are elevated.[16][17] Because the distal parts (posterior tonsillar pillars, which the palatopharyngeal muscles are attached to)[2] are sutured to the other side of the posterior wall, the pharyngeal port will become smaller. As a result, the tissue flaps cross each other, leading to a smaller port in the middle and a shorter distance between the palate and posterior pharyngeal wall.[11]

There are a few advantages with using this technique. First of all the procedure is relatively easy to execute. This makes the operation cheaper, also because of a reduced anesthesia time. Secondly the dynamic sphincter can be moved as result of a remaining neuromuscular innervation, which gives a better function of the velopharyngeal port. Finally there is a lower complication rate, although obstructive sleep apnoea syndrome (OSAS) is associated.[2][11][18]

Both techniques are used often, but there is no standard operation. Pharyngeal flap surgery is not better than sphincter palatoplasty.[11] It is more upon the surgeon's experience, knowledge and preference which operation will be done. Also the patient’s age,[19][20] and the size and nature of the velopharyngeal defect, contribute to which technique is used.[20][21]

Posterior wall augmentation

Posterior wall augmentation

Another option for diminishing the velopharyngeal port is posterior wall augmentation. This technique is not often used.[2] Additionally this technique can only be used for small gaps.[22] When this operation is performed there are several advantages. It is possible to narrow down the velopharyngeal port without modifying the function of the velum or lateral walls.[22] Furthermore, the chance of obstructing the airway is lower, because the port can be closed more precisely. Many materials have been used for this closure: petroleum jelly, paraffin, cartilage, adjacent soft tissue, silastic, fat, Teflon and proplast.[2] But results in the long term are very unpredictable. There are problems with tissue incompatibility and migration of the implant.[2][22] Even migration to the brain is noticed.

Non-operative techniques

Prosthesis

Prostheses are used for nonsurgical closure in a situation of velopharyngeal dysfunction.[2][23] There are two types of prosthesis: the speech bulb and the palatal lift prosthesis.[2] The speech bulb is an acrylic body that can be placed in the velopharyngeal port and can achieve obstruction. The palatal lift prosthesis is comparable with the speech bulb, but with a metal skeleton attached to the acrylic body.[23] This will also obstruct the velopharyngeal port.[23] It is a good option for patients that have enough tissue but a poor control of the coordination and timing of velopharyngeal movement.[2][23] It is also used in patients with contraindications for surgery. It has also been used as a reversible test to confirm whether a surgical intervention would help.[2]

Conclusion

Pharyngeal flap and sphincter pharyngoplasty seem to be safe and reliable procedures for treating velopharyngeal dysfunction. Although not in all patients complete closure will be achieved after the surgical procedures, they do show a reduction of the size of the velopharyngeal defect. The planning of the surgical procedure seems to be the most important aspect of the surgery for correcting velopharyngeal dysfunction.[24] To clarify the problems of each individual patient, diagnosis will be confirmed by hearing (speech analysis) and imaging (videofluoroscopy and nasoendoscopy) the defect. It is important to think about that these different diagnostic procedures can give a various result, because of the fact that what you hear does not necessarily correlate with what is seen.[2]

The preoperative planning will give you important information about the movement of the soft palate, the lateral pharyngeal walls and posterior pharyngeal wall. The part of the soft palate with the maximal movement, gives you the precise level where tissue has to be attached. This information decides the length of the pharyngeal flap or lateral flaps. Likewise, the size and shape of the gap is considered for determining the width of the pharyngeal flap or lateral flaps. The dissymmetry will be corrected by placing an asymmetric flap or creating unilateral a wider flap in the sphincteroplasty procedure. This is why diagnostic tools contribute immensely in the approach of the problem, and consequently decide if the operation can be called a success.[24] Moreover, it is concluded that the diagnostic tool that should be used has to be chosen on the aspect of the velopharyngeal defect of the individual.[6][9][12]

A recent meta-analysis which used two RCT's (Randomised Controlled Trial), currently provides the highest quality data in comparing pharyngeal flap with sphincter pharyngoplasty. This study suggests a possible trend favouring the pharyngeal flap.[11] However, in this meta-analysis only one out of two RCT’s shows a better outcome for the pharyngeal flap, the other RCT doesn’t show any difference. What’s needed next is more research to be done, so that the result of the meta-analysis can be supported.

Etymology and pronunciation

The word velopharyngeal uses combining forms of velo- + pharyng-, referring to the soft palate (velum palatinum) and the pharynx.

References

  1. ↑ Hirschberg J. Results and Complications of 1104 Surgeries for Velopharyngeal Insufficiency. ISRN Otolaryngol. 2012.181202
  2. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 Peter D. Witt, D’Antonio. Velopharyngeal insufficiency and secondary palatal management. Clinics in plastic surgery. 1993. Oct;20(4):707-21.
  3. ↑ Wermker K, Lünenbürger H, Joos U, Kleinheinz J, Jung S et al. Results of speech improvement following simultaneous push-back together with velopharyngeal flap surgery in cleft palate patients. J Craniomaxillofac Surg. 2013 Sep 13. pii: S1010-5182(13)00219-9.
  4. 1 2 Ysunza A, Pamplona C, Ramírez E, Molina F, Mendoza M, Silva A. Velopharyngeal surgery: a prospective randomized study of pharyngeal flaps and sphincter pharyngoplasties, Plast Reconstr Surg. 2002 Nov;110(6):1401-7.
  5. ↑ Emara TA, Quriba AS. Posterior pharyngeal flap for velopharyngeal insufficiency patients: A New Technique for Flap Inset. Laryngoscope. 2012 Feb;122(2):260-5.
  6. 1 2 3 4 5 6 7 Havstam C, Lohmander A, Persson C, Dotevall H, Lith A, Lilja J. Evaluation of VPI-assessment with videofluoroscopy and nasoendoscopy. Br J Plast Surg. 2005 Oct;58(7):922-31.
  7. 1 2 Ruda JM, Krakovitz P, Rose AS. A Review of the Evaluation and Management of Velopharyngeal Insufficiency in Children. Otolaryngol Clin North Am. 2012 Jun;45(3):653-69
  8. 1 2 Lam DJ, Starr JR, Perkins JA, Lewis CW, Eblen LE, Dunlap J, Sie KC. A comparison of nasoendoscopy and multiview videofluoroscopy in assessing velopharyngeal insufficiency. Otolaryngol Head Neck Surg. 2006 Mar;134(3):394-402.
  9. 1 2 3 4 5 6 Henningsson G, Isberg A. Comparison between multiview videofluoroscopy and nasoendoscopy of velopharyngeal movements. Cleft Palate Craniofac J. 1991 Oct;28(4):413-7
  10. ↑ Willging JP. Velopharyngeal insufficiency. Curr Opin Otolaryngol Head Neck Surg. 2003 Dec;11(6):452-5.
  11. 1 2 3 4 5 6 7 8 Jessica Collins et al. Ontario, Canada. Pharyngeal flap versus sphincter pharyngoplasty for the treatment of velopharyngeal insufficiency: A meta-analysis. J Plast Reconstr Aesthet Surg. 2012 Jul;65(7):864-8.
  12. 1 2 3 4 C. Drissi & M. Mitrofanoff & C. Talandier & C. Falip & V. Le Couls& C. Adamsbaum. Feasibility of dynamic MRI for evaluating velopharyngeal insufficiency in children. Eur Radiol. 2011 Jul;21(7):1462-9.
  13. 1 2 S. Vadodaria, T. E. E. Goodacre and P. Anslow; Does MRI contribute to the investigation of palatal function?; Br J Plast Surg. 2000 Apr;53(3):191-9.
  14. ↑ Mc Williams BJ, Morris HL, Shelton RL: Cleft Palate speech. Philadelphia. BC Decker 1990, p 71
  15. ↑ Murthy AS, Parikh PM, Cristion C, et al. Fustila after 2-flap palatoplasty: a 20-year review. Ann Plast Surg. 2009. 63(6):632-5
  16. ↑ Dec W, Shetye PR, Grayson BH, et al. Incidence of oronasal fistula formation after nasoalveolar molding and primary cleft repair. J Craniofac Surg. 2013 . 24(1):57-61.
  17. ↑ Losken A, Williams JK, Burstein FD, et al. An outcome evaluation of sphincter pharyngoplasty for management of velopharyngeal insufficiency. Plast Reconstr Surg. 2003. 112(7):1755-61
  18. ↑ Witt PD, Marsh JL, Muntz HR, et al. Acute obstructive sleep apnea as a complication of sphincter pharyngoplasty. Cleft Palate Craniofac J. 1996. 33(3):183-9.
  19. ↑ Kirschner RE, Randall P, Wang P, et al. Cleft palate repair at 3 to 7 months of age. Plast Reconstr Surg 2000;105:2127e32.
  20. 1 2 Peat BG, Albery EH, Jones K, Pigott RW. Tailoring velopharyngeal surgery: the influence of etiology and type of operation. Plast Reconstr Surg 1994;93:948e53.
  21. ↑ Shprintzen RJ, Lewin ML, Croft CB, et al. A comprehensive study of pharyngeal flap surgery: Tailor made flaps. Cleft Palate J 1979;16:46e55.
  22. 1 2 3 Gray SD, Pinborough-zimmerman J, Catten M, et al. Posterior wall augmentation for treatment of velopharyngeal insufficiency. Otolaryngol Head Neck Surg. 1999. 121(1):107-12.
  23. 1 2 3 4 Aboloyoun AI, Ghorab S, Farooq MU. Palatal lifting prosthesis and velopharyngeal insufficiency: preliminary report. Acta Med. Acad. 2013; 42(1):55-60
  24. 1 2 Ysunza A, Pamplona C, Ramirez E, et al. Velopharyngeal surgery: A prospective randomised study of Pharyngeal Flaps and Sphincter Pharyngoplasties.Plast Reconstr Surg 2002 110(6):1401-7
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