Velopharyngeal Dysfunction in Pediatric Population

Before talking about velopharyngeal dysfunction, is important to understand what is typical velopharyngeal function:

• The velopharyngeal valve is made up of:
  •  “Velum”, also known as the soft palate
  • “Lateral Pharyngeal Walls”, also known as the side walls of the throat
  • “Posterior Pharyngeal Wall”, also known as the back wall of the throat
• Velopharyngeal function closes for oral sounds, and opens for nasal sounds. It is used for producing pressure-sensitive sounds (plosives like “b”, fricatives like “s”, affricates like “ch”)
• A typically functioning velopharyngeal valve also closes off the nasal cavity from the oral cavity during swallowing, vomiting, blowing, sucking and whistling.

So, let’s talk about velopharyngeal dysfunction. Velopharyngeal Dysfunction (aka as VPD) is a condition where the velopharyngeal valve does not close consistently and completely during the production of oral sounds.

There are 3 types:

Who has Velopharyngeal Dysfunction? 

 You may be wondering who are the individuals who have velopharyngeal dysfunction.

• Velopharyngeal dysfunction is often associated with individuals who have craniofacial disorders containing cleft palates.

What is a Cleft Palate?

• A fistula in the palate of the oral cavity
• A cleft palate may be:
  • Unilateral
  • Bilateral
  • Complete
  • Medial
  • Transverse
  • Oblique

• Submucous cleft palate: Occurs when the skin on the palate looks typical, but the muscles below the skin are not connected properly. When the individual speaks, there’s poor elevation of the velum.

The most common syndrome related to a cleft palate is velocardiofacial (VCF) syndrome. Approximately 1 in 2,000-5,000 are born with it each year. It is an Autosomal Dominant condition where the microscopic segment on the long arm of chromosome 22 is missing.

• Characteristics:
  • a long face with a prominent upper jaw
  • flattening of the cheeks
  • an underdeveloped lower jaw
  • a bluish color below the eyes
  • a prominent nose with narrow nasal passages
  • a long thin upper lip and a down-slanting mouth
  • cleft palate or submucous cleft palate

Another condition in which a cleft palate is commonly observed includes Pierre Robin Sequence. Pierre Robin Sequence is a condition in which the infant has micrognathia (i.e., small jaw) present at birth, glossoptosis (i.e., a tongue that is placed further back than normal), and a cleft palate (i.e., fistula in the palate)

• Approximately 7-10 weeks into pregnancy, the lower jaw of a fetus grows rapidly, allowing the tongue to descend. If the jaw does not lower properly, however, the tongue can prevent the palate from forming – which causes a cleft palate. The tongue is also displaced in the back, which may cause feeding/breathing difficulties.

What are the Signs of Velopharyngeal Dysfunction? 

• 1)  Resonance Disorders
  • What is a Resonance Disorder? 
    • The abnormal coupling of oral/nasal cavity during speech
    • What types of resonance disorders are there?
      • Hypernasality (most commonly seen with cleft palates)
        • Too much sound resonating in the nasal cavity during speech
      • Hyponasality 
        • Not enough resonance in the nasal cavity due to upper airway obstruction
      • Mixed
        • Mix of hypernasality or nasal air emission on oral consonants and hyponasality on nasal consonants
      • Cul-Du-Sac
        • Sound resonates in the pharynx or nasal cavity, but it is not released due to obstruction
        • Palate is working pretty well, but somewhere along the way, the air flow is ending inappropriately

• 2) Articulation Errors
  • Compensatory Errors – How individual speaks to compensate for low air pressure by the velopharyngeal region
    • Glottal Stops
    • Pharyngeal Stops
    • Pharyngeal Fricatives
    • Velar Fricatives
    • Posterior Nasal Fricatives
    • Nasal Sniff
    • Generalized Backing
    • Mid Dorsum Palatal Stop
  • Obligatory Errors – Articulation errors directly because of structural deficit
    • Nasalized Phonemes
    • Weak Consonants, Short Utterance Length
  • SLP’s can fix compensatory errors by working on placement – however, they cannot fix obligatory errors. 

• 3) Feeding Difficulties
  • The presence of velopharyngeal dysfunction has the potential to interrupt normal feeding process/outcome in infants, due to lack of ability to create suction (or negative pressure) because of the absence of velum elevation when being breastfed/bottle-fed.
  • Infants may display:
    • Poor Suction
    • Poor Intake
    • Lengthy Feedings
    • Nasal Regurgitation
    • Choking, Gagging
    • Poor Weight Gain
    • Excessive Air Intake

When is Surgery Warranted? 

The timing of surgery is influenced by function, speech, feeding, airway management and psychosocial needs, but it usually occurs between 9 and 18 months of age.

When a child exhibits obligatory speech errors, which is when a resonance disorder is the product of structural abnormality, resonance disorder cannot be modified through speech therapy and surgery is warranted 

• Before surgery, it would behoove the individual to work on placement of articulation, because many individuals display compensatory errors, which are speech errors usually articulated in the pharynx (i.e., where there is more air pressure).

A direct assessment (e.g., nasoendoscopy) is necessary before surgery

Other options other than Surgery? 

Yes… Prostheses!

• Palatal Obturator 
  • Prosthetic that is used to close defects of the hard/soft palate that may affect speech production
  • Completely occludes oronasal fistula
  • Similar to dental retainers, but without the front wire
• Speech Bulb
  • A speech bulb aids with velopharyngeal closure (it does not merely obturate a fistula)
  • Used when palate is short; the bulb contacts posterior pharyngeal wall, while still providing air flow
  • Can take it out at night to breathe well
• Palatal Lift 
  • A palatal lift, like a speech bulb, is provided when there is not enough palatal movement
  • Palatal lift allows you to raise your velum a third of a way up and down
  • Can take appliance out at night

What is the Speech Language Pathologist’s Role? 

The Speech-Pathologist is essential to continue reinforcement of correct speech and prevent residual articulation errors. Following surgery/prosthesis, the child may continue to have hypernasality or nasal emission, despite correct articulation

• Why? 
  • Changing structure does not change function 
  • This is essential to mention to family – surgery alone will not correct speech 
• Child will need to learn to use the new structure through auditory feedback
• Specific speech therapy techniques may be found:
  • https://www.asha.org/Events/convention/handouts/2011/Kummer/

If you live in the state of Florida and have concerns regarding velopharyngeal function, or overall speech-language development, please call Exceptional Speech Therapy (786-717-5649) to schedule a comprehensive speech-language evaluation.

Andrea Scola, M.S., CF-SLP
Exceptional Speech Therapy Blog Writer

References:

Brigger, M. T., Ashland, J. E., & Hartnick, C. J. (2010). Injection Pharyngoplasty With Calcium Hydroxylapatite for Velopharyngeal Insufficiency. Archives of Otolaryngology–Head & Neck Surgery,136(7), 666. doi:10.1001/archoto.2010.110

Cole, P., Banerji, S., Hollier, L., & Stal, S. (2008). Two Hundred Twenty-Two Consecutive Pharyngeal Flaps: An Analysis of Postoperative Complications. Journal of Oral and Maxillofacial Surgery,66(4), 745-748. doi:10.1016/j.joms.2007.12.020

Home. (n.d.). Retrieved from http://faces-cranio.org/

Khajehahmadi, S., & Rahpeyma, A. (2013). Buccinator-based myomucosal flaps in intraoral reconstruction: A review and new classification. National Journal of Maxillofacial Surgery,4(1), 25. doi:10.4103/0975-5950.117875

Khosla, R. K., Mabry, K., & Castiglione, C. L. (2008). Clinical Outcomes of the Furlow Z-Plasty for Primary Cleft Palate Repair. The Cleft Palate-Craniofacial Journal,45(5), 501-510. doi:10.1597/07-063.1

Kilpatrick, L. A., White, D. R., Kline, R., Hufnagle, K., & Vanlue, M. (2009). SP359 – Postoperative management following sphincter pharyngoplasty. Otolaryngology – Head and Neck Surgery,141(3). doi:10.1016/j.otohns.2009.06.668

Kummer, A.W. (2007). Resonance Disorders and Velopharyngeal Dysfunction: Evaluation and Treatment. A four hour self study course on DVD with manual. Rockville, MD: American     Speech-Language-Hearing Association.

Kummer, A.W. (2007). Oral & Nasal Listener. Greenville, SC: Super Duper Publications.

Kummer, A.W. (2008). Cleft Palate and Craniofacial Anomalies: The Effects on Speech and Resonance, 2nd Edition. New Albany, NY: Delmar Cengage Learning. .

Kummer, A.W. (2014). Cleft Palate and Cranioÿaclal Anomalies, The Effects on Speech and Resonance, 3rd Edition. Clifton Park: Cengage Learning

Lierop, A. C., & Fagan, J. J. (2007). Buccinator myomucosal flap: Clinical results and review of anatomy, surgical technique and applications. The Journal of Laryngology & Otology,122(02). doi:10.1017/s0022215107008353

Miller, S. (2013). Pharyngeal flap versus sphincter pharyngoplasty for the treatment of velopharyngeal insufficiency: A meta-analysis. Yearbook of Plastic and Aesthetic Surgery,2013, 7. doi:10.1016/j.yprs.2012.07.025

Surgical Treatment of Velopharyngeal Dysfunction. (2018, October 09). Retrieved from https://emedicine.medscape.com/article/1279928-overview

Yamashita, R. P., & Trindade, I. E. (2008). Long-Term Effects of Pharyngeal Flaps on the Upper Airways of Subjects with Velopharyngeal Insufficiency. The Cleft Palate-Craniofacial Journal,45(4), 364-370. doi:10.1597/07-031.1