Wednesday, December 8, 2010

Velopharyngeal Insufficiency

Speech is easy for some, but others have speech impediments. The types of speech impediments differ, and many circumstances may cause the problems. One particular circumstance causes hypernasal speech. This is where too much air escapes through the nose while talking. This condition is called Velopharyngeal Insufficiency (VPI). In this condition the soft palate, the soft flap of skin at the back of the roof of the mouth, does not function correctly. There can be many problems with the soft palate. It may not close correctly, or it may be too short; therefore, air escapes through the nose when it should not. Speech therapy helps but will not fix the problem if there are any structural deformities. These structural deformities can be fixed by an orthodontic prosthetic or surgery followed by intense speech therapy to retrain the patient how to talk correctly. A few different types of surgeries are done depending on the problem causing the VPI. There are different prosthetics used depending on the problem causing the VPI and the situation of the patient. VPI is most common in patients with prior medical problems, such as cleft palate or submucus cleft palate, but there can also be no apparent cause. “While VPI is not among the most serious of speech impairments, it can affect the child’s development and should be addressed through appropriate medical treatment and speech therapy.”

The main symptoms of VPI have to do with the nasal sounding speech and air leaking out the nose during speech. The types of symptoms are nasal sound substitutions, nasal voice quality, nasal air emission, and glottal sound substitutions ("Velopharyngeal Insufficiency"). With nasal sound substitutions, the child will substitute nasal sounds (m,n) for oral sounds (p,b,t,d). This is not the child's fault; he/she is medically unable to form those particular sounds. Nasal voice quality is the overall nasal sounding speech called hypernasality, which is the opposite of talking with a cold (hyponasality). With hypernasality, too much air escapes through the nose; with hyponasality not enough air gets out the nose ("Cleft Palate Repair, Pediatric Ear Nose and Throat 3"). If the child has nasal voice quality, it is possible that he/she could produce the nasal sounds correctly, just through the nose instead of the mouth. Nasal air emission is the actual escaping of the air through the nose. This can actually be heard with the ear and felt with the hand. Glottal sound substitutions happen when the child learns a different way to produce the nasal sounds. This may be easier for the child, but it is harder for a listener to understand. Cleftspeech.com says, “Since the child cannot control the air that comes out of his/her nose, he/she may try to 'stop' the air at the voice box (larynx) which creates a rough or breathy sound or may sound like the sound is being omitted. This type of speech pattern requires correction immediately through speech therapy. Surgery alone will not improve this type of speech pattern.” (Dixon-Wood 5)The child will have to go through intense speech therapy to correct the error and to learn the correct way to articulate the nasal sounds.

There are many causes for VPI, and it can occur in children and adults alike but is most common in children with cleft palate. Cleft palate is not visible from the outside of a child like cleft lip, which is a visual deformity of the lip. According to The Gale Encyclopedia of Childhood & Adolescence, “Cleft palate occurs when the right and left segments of the palate fail to join properly.” This separation in the hard palate prevents a tight seal from forming, therefore allowing air to escape through the nose. The Seattle Children’s Hospital Research Foundation states: “About 20% to 30% of children who have cleft palate with or without cleft lip will have persisting VPI after their palate repair.”

Other causes are submucous cleft palate, congenitally short palate or “velum,” adenoidectomy, motor speech disorders, and velo-cardio-facial syndrome. A submucous cleft palate is a type of cleft palate. “Submucous” refers to the cleft being covered by the lining, mucous membrane, of the roof of the mouth. A submucous cleft of the soft palate has to do with the lack of muscular tissue and incorrect positioning of the muscles ("Cleftline 2"). One of the effects of a submucous cleft palate is the soft palate not functioning properly. This results in the soft palate not being able to seal properly with the back of the throat, and in return causing VPI.

A congenitally short palate is simply where the soft palate is not long enough. The soft palate is too short to make contact with the back of the throat. This allows air to escape through the nose causing VPI. Adenoidectomy is a surgery to remove the adenoids, a single clump of tissue at the back of the nose. Children with cleft palate or any other syndrome associated with the palate are most at risk to develop VPI after adenoidectomy. Removing the adenoids open up the throat and allow for the air to escape through the nose. In some cases, removing the adenoids can help VPI instead of causing it.

Motor speech disorders result from damage in the brain that affect the control of muscles used for speech ("Communication Sciences and Disorders 1"). Sometimes the soft palate is affected and does not function properly, therefore causing VPI. Velo-cardio-facial syndrome (VCSF) is a genetic disorder with symptoms that are not constant and differ from individual to individual. There are a few symptoms that are common among individuals. These include; certain heart defects, effects on facial appearance, and lack of or underdeveloped thymus and parathyroid glands (“Velo-Cardio-Facial Syndrome Educational Foundation, Inc 2”). One of the features of VCSF is palatal abnormalities, such as cleft palate. These abnormalities can cause VPI.
Diagnosing VPI is not very difficult and sometimes does not require any special equipment. Listener judgment is the most important test of abnormal resonance and velopharyngeal dysfunction according to Ann Kummer. The examiner asks the child to repeat groups of sentences that contain the problem sounds. Single words are not used because they do not depict normal flowing speech. Even the most experienced examiners can have difficulty detecting the presence of nasal emission. The examiner can be more accurate by using something to amplify the sounds coming out of the nose. “A straw is the ultimate low-cost, low-tech instrument; yet, it is extremely helpful and reliable in detecting hypernasality and nasal emission. Using a bending straw, the examiner should place the short end of the straw in the child’s nostril and the other end near the examiner’s ear,” says Ann W Kummer, PhD. The examiner can also use a “Listening Tube.” This long plastic tube works exactly like the straw method. The only negative effect is that the examiner may have a hard time telling which end went in the child’s nose and which end went in the examiner’s ear. An Oral and Nasal Listener can also be used. This is a device like a stethoscope, only with a tube at the end that goes into the child’s nose. A SeeScape® can also be useful in measuring the pressure that comes out of the nose. “The SeeScape® is a commercial device with a length of tubing, attached by an L-shaped connector to a glass tube containing a Styrofoam piston. The end of the tubing is placed at the speaker’s most free-breathing nostril and the piston is viewed as the speaker repeats a speech sample containing oral pressure consonants. The lightweight piece of Styrofoam moves up with any nasal air entering the tubing” ("Velopharyngeal Assessment 12").

Fiber-optic nasopharyngoscopy is also another good way of diagnosing VPI, although it is not used in young children very often because it is invasive, and it can be hard to get the child to cooperate with the examiner. During this procedure the doctor sprays a topical numbing agent in the nose and inserts a fiber-optic scope. This scope goes down the nasal passage and is able to get a good view of the soft palate. The doctor then gets the patient to talk and say certain sounds to see how the soft palate reacts. The doctor will also be able to tell if there are any deformities or clefting of the soft palate.

X-rays are a great tool and are not as invasive as the fiber-optic nasopharyngoscopy. The child sits in front of an x-ray machine with the examiner, normally a speech pathologist, and the child is asked to make certain sounds, and then an x-ray is taken while these sounds are being produced. These x-rays can reveal adenoids, tonsils and the soft palate. The speech pathologist can tell immediately what is causing the problem and what the best course of treatment is. This information gets passed along to the doctor who makes the final say in the path of treatment.

There are three main types of treatment. Speech therapy, surgery and orthodontic prosthetics. Speech therapy is helpful in most cases and is needed no matter what path is chosen for the patient. Speech therapy helps the child learn to articulate correctly. The child plays games and repeats sayings to help him/her reach his/her goal. The frequency and length of therapy depends on the age and attention span of the child. Younger children, around the age of two, will probably only be able to tolerate a 30 minute session twice a week. Sometimes the speech pathologist can come to the home, but it will most likely cost the patient. This method may be more comfortable for the child, especially if there are other developmental problems involved. Some school systems will see children as young as two years old for speech therapy, and it is performed free of charge to the parent. Sometimes speech therapy is not enough, and the child needs a prosthetic or surgery to completely correct the problem. However, appliances and surgery alone are not enough; the child will have to continue speech therapy to learn how to speak correctly with the prosthetic or after the surgery.

A prosthetic is an artificial device that lengthens or strengthens the soft palate enabling it to function correctly and stop the leaking of air out of the nose. These appliances are similar to removable dental braces or a dental retainer. The prosthetic fits over the hard and soft palates and is anchored to the upper teeth much like a retainer. Prosthetics are most commonly used in children who are not good candidates for surgery. For example if the gap between the soft palate and the back of the throat is too large, that surgery may not be successful. These prosthetics are usually removable and can sometimes be used on a trial basis to see if surgery would be the correct route (Russell 11).

Three types of prosthetics used to treat VPI are a palatal lift, palatal speech bulb, and pharyngeal obturator. A palatal lift works in two ways. First, the palatal lift does just that; it lifts the soft palate up so it can touch the wall at the back of the throat. The second way that the palatal lift works is by positioning the soft palate so not much muscle is required to obtain closure between the soft palate and the back of the throat. This prosthetic is used in cases where there is no deformity with the soft palate, but it is too weak to move in cases of people with poor muscle function. Dr Bridgett Russell, in the department of speech Pathology and Audiology at State University of New York Fredonia, says, “Problems with this device include articulation precision after the initial positioning of the appliance and dental stress.”

Speech bulbs can be used to close off most of the gap leaving a small gap to allow the patient to breathe. The device improves closure in patient with poor movement of the soft palate. Bridgett Russell says, “Speech bulbs are recommended when surgery is not possible because of medical or anesthetic risks, or sometimes when the gap is so large that surgery is unlikely to work.”
Obturators can make up for the lack of muscle movement. Like palate lifts and speech bulbs obturators attach to the hard palate or teeth. Sometimes the patient can be weaned off of the obturator so the natural soft palate can strengthen and take over the work of the oburtrator (Biavati 9).

In cases where the soft palate is deformed or too short, surgery may be the best option. Most surgeons will not perform the operation on children younger than four, but they also do not want to wait too long from a developmental perspective. The cause of the VPI will determine which type of surgery will be used. There are seven types of surgery: pharyngeal flap procedure, palatal push-back, pharyngoplasty, augmentation pharyngoplasty, velopharyngeal sphincter reconstruction.

Pharyngeal flap procedure is the preferred method in patients with cleft palate. Schoenborn, a surgeon, was the first to come up with this procedure in 1876. “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” (Biavati 33). Palatal push-back surgery is also used in cleft palate patients. The surgery was introduced by cleft palate surgeons Dorrance, Bransfield, and Wardrill. In this procedure the lining of the nasal passages is used to complete the incomplete or cleft palate. This procedure essentially works in lengthening the soft palate and filling in the gaps of a cleft palate (Calvin and Owsley 30). Dr Hynes introduced pharyngoplasty in 1950. This surgery serves to be most helpful for patients with circular closures and slow pharyngeal wall movement. This procedure works to make the velopharyngeal opening smaller by tightening. This helps to reduce the loss of air through the nose (Biavati 28).

Augmentation pharyngoplasty is a type of reconstructive plastic surgery and is only used in mild cases of VPI. This procedure was introduced in 1926 by Dr von Gaza. Augmentation pharngoplasty is often done after a pharyngoplasty with the creation of a flap (Shiffman 358). Fat is harvested from around the belly button area and is purified of all connective tissue and blood. This purified fat is then injected into the back of the patient’s throat, either through the mouth or through the back of the neck via the atlas, the top vertebrae. The fat then works to close up the gap by creating a lump on the back of the throat, bringing it closer to the soft palate (359). Velopharyngeal sphincter reconstruction uses free muscle implantation to reconstruct the sphincter. All surgeries are aimed to cure hypernasality and nasal emission, but speech impediments continue and require speech therapy for full recovery. After the surgery, it is normal for antibiotics to be administered along with a clear liquid diet followed by a soft food diet (Lippincott, Williams and Wilkins 720).

In short, the symptoms of VPI all have to do with air coming out the nose instead of the mouth, due to the soft palate not making contact with the back of the throat. Nasal sound substitutions, nasal voice quality, nasal air emission and glottal sound substitutions are all indicators of VPI. The cause of VPI, also called cleft speech, is most commonly cleft palate or submucous cleft palate. The patient can also have a short palate, which does not make contact with the back of the throat. An adenoidectomy can either cause or help VPI. Motor speech disorders and velo-cardio-facial syndrome can also cause VPI. The diagnosis for VPI can be as simple as listener judgment, using a straw or tube to better hear the articulations. To view the actual soft palate fiber-optic nasopharyngoscopy, ultrasounds and x-rays are used. VPI can happen in with no other apparent medical problems but is most common in those with neurological disabilities and physical deformities of the hard or soft palate. VPI is a fixable medical problem that will always require speech therapy, sometimes accompanied by prosthetics or surgery.




Works Cited


Biavati, Michael J. Velopharyngeal Insufficiency: Treatment. Web MD, 2010. Web.
.

Calvin, Howard, and John Owsley. A Lining Vomer Flap for Palate Pushback in Unilateral Cleft

Palate Repair. San Francisco: Allen Press Publishing services, 1976. 30. Print.
"Cleft Palate Foundation." Cleftline. Cleft Palate Foundation, 2006. Web.
.

"CleftSpeech.com." Velopharyngeal Insufficiency. Department of Health, Florida, n.d. Web. .

Chromosomal and Genetic Conditions. Seattle Children's Hospital, 2010. Web. .

Gall & Kagan. "Cleft Lip and Palate." The Gale Encyclopedia of Childhood & Adolescence.
Detroit, New York, Toronto, London: Gale, 1998. Print.

Kummer, Ann W. "Resonance Disorders & Velopharyngeal Dysfunction: Simple Low-Tech and
No-Tech Procedures for Evaluation and Treatment." SpeechPathology.com. N.p., 12/10/07. Web. .

Lippincott, Williams & Wilkins, First. Professional guide to diseases. 9th. Ambler, Pa: Judith A Schilling McCann, RN, MSN 2009.720. Print.

"Motor-Speech Disorders." Communication Sciences and Disorders. North Arizona University,
n.d. Web. 2006. .

" Pediatric Otolaryngology." Cleft Palate Repair, Pediatric Ear Nose and Throat. Tampa Bay

Children's Hospital, 2004. Web. .

Russell, Bridget A. Treatment of Hypernasality in Children with Down Syndrome. Department of

Speech Pathology & Audiology State University of New York Fredonia, 2006. Web. 14 Nov 2010. .

Shiffman, Melvin. Autologous Fat Transfer: Art, Science, and Clinical Practice.

Berlin: Springer
Science & Business Media, 2010. 358. Print.

Velopharyngeal Assessment. Children's Healthcare of Atlanta, 2010. Web.
.

What is Velo-cardio-facial syndrome (VCFS)?. Velo-Cardio-Facial Syndrome Educational
Foundation, Inc., 2009. Web. http://www.vcfsef.org/about_vcfs/index.html

1 comment:

  1. Wow this is just stellar. Thank you. My daughter is having the pflap repair Thursday. Someone asked me if she was having the push back as well and that sent me to the Internet because I had no idea. This is a wealth of info. thanks

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