What is Velopharyngeal Insufficiency?
Velopharyngeal insufficiency (VPI) is a condition that affects a part of your body called the velopharyngeal sphincter or valve. This valve is like a door that separates your nose and mouth when you’re speaking, swallowing, vomiting, blowing air, or sucking on something. When the soft part of the roof of your mouth (soft palate) and the walls of your throat (pharyngeal walls) can’t form a good seal, it creates an abnormal connection between your nose and mouth.
This can lead to a few issues. Your speech may sound overly nasal. There might be an increased nasal sound in your voice. Your food or drink might come back up into your nose when you’re swallowing (regurgitation). Air might escape through your nose when you speak (nasal emission). And it could affect the air pressure in your mouth when you’re speaking, making it difficult for you to form certain sounds.
All these issues can make it hard for people to understand what you’re saying, which can cause problems in your daily life and social interactions. Specialists in speech-language pathology (who work on speech and language issues), otolaryngology (who deal with ear, nose, and throat disorders), and plastic surgery all work together to diagnose and treat this condition.
VPI is the term generally used for this condition. It hints at a problem with how the velopharyngeal valve closes but doesn’t suggest a specific cause. There are three subtypes: VPI from a structural issue, incompetence from a problem with how the nerves function, and mislearning from learned issues in how to form certain speech sounds.
What Causes Velopharyngeal Insufficiency?
Velopharyngeal inadequacy (VPI) can happen when part of the throat, called the velopharyngeal sphincter, doesn’t close fully. This part of the throat is responsible for controlling airflow during speech and helps avoid food or drink finding its way into the nose during swallowing. The reasons for VPI can include structural problems, issues connected to the nervous system, incorrect formation during learning, or as a feature of certain medical syndromes.
Structurally, VPI can occur due to conditions like a cleft palate, where there’s a gap in the upper lip or roof of the mouth, a naturally short soft palate, an imbalance in the size of the throat and nose, oversized tonsils, or scars from operations. Additional causes can result from surgery for correcting cleft palate, which might affect how well the soft palate moves, or alignment of specific throat muscles. Conversely, the removal of adenoids (small lumps of tissue at the back of the throat) may create an opening that’s too large for effective closure.
Neurological or musculoskeletal injuries can also lead to VPI, even when the structure of the sphincter is normal. Things like head injuries, strokes, cerebral palsy, progressive muscle conditions, nerve damage, or conditions like Parkinson’s disease or Down syndrome (Trisomy 21) can affect the closing of the velopharyngeal sphincter. Diseases that affect muscle conditions, like Myasthenia Gravis, can also lead to muscle weakening and VPI.
In some cases, children can learn incorrect pronunciation or articulation that might seem like VPI. They can also develop compensatory behaviors after surgery to repair cleft palate. These behaviors can mistakenly appear as VPI, but are typically best treated with speech therapy rather than surgical intervention.
Finally, VPI can be a symptom of various syndromes in children, such as Down syndrome, Klippel-Feil syndrome, Nevus syndrome, Turner syndrome, and VATER syndrome. One of the most common syndromic causes of VPI in babies without a cleft palate is Velocardiofacial syndrome, which can develop spontaneously or be inherited. Babies with this condition often have facial abnormalities, heart defects, calcium deficiencies due to poor parathyroid function, immune deficiencies, weak throat and palate muscles, or deformed palates. Many of these children experience VPI, difficulty with speech, and delays in feeding and language development.
Risk Factors and Frequency for Velopharyngeal Insufficiency
An overt cleft palate is a condition that is seen in roughly 1 in every 650 to 750 children born each year. This condition is the most common cause of Velopharyngeal Insufficiency (VPI). In about 20% to 30% of patients, some problems persist even after the primary cleft palate surgery. An adenoidectomy, a type of oral surgery, can also sometimes lead to VPI with the chances of occurrence ranging from 1 in 1,500 to 1 in 10,000. Other complications such as postoperative palatal fistula and VPI can occur at a rate of 3.4% to 15% after the primary cleft palate repair. If VPI occurs without an identifiable cause, the most common diagnosis is the 22q11.2 deletion syndrome. This syndrome occurs in 1 in 2000 to 1 in 4000 people, with 27% to 92% of diagnosed children having VPI.
Signs and Symptoms of Velopharyngeal Insufficiency
When a medical professional is examining a patient for oral or nasal issues, they need to consider many important factors. They should be aware of a patient’s medical history, including any known syndromes, previous surgeries in the mouth or throat region, a history of regular ear infections or other neurological conditions. They should also take into account if the patient has a cleft palate, has had previous episodes of food and liquid escaping back up the nose when eating, or has a risk of sleep apnea, which is characterized by breathing pauses during sleep.
In the physical examination, the doctor should pay close attention to various details in the oral and nasal regions. They should consider:
- The height, movement, and symmetry of the soft palate (the back part of the roof of the mouth)
- The size of the tonsils
- The movement and symmetry of the tongue
- The patient’s ability to keep their mouth closed
- Any issues with the nasal lining or nasal turns
- The condition of the nasal septum, which separates the nostrils.
The examination should also involve checking for possible physical abnormalities, such as a cleft (split) palate beneath the mucous membrane, a split uvula (the dangling flesh at the back of the throat), a clear zone in the soft palate, a notch at the back of the palate, or any muscle weakness or unevenness.
To assess the movement of the soft palate, they might ask the patient to sustain the pronunciation of “e” or “i”. Lastly, they should also perform an examination of the middle ear, to check for middle ear infection or inward pulling of the eardrum as these could indicate a problem with the Eustachian tube, which connects the middle ear to the back of the nose.
Testing for Velopharyngeal Insufficiency
When diagnosing velopharyngeal insufficiency (VPI), which is a problem with the coordination between the throat and soft part of the roof of the mouth, several steps are taken. These include getting your medical history, giving a physical exam, assessing how you produce speech, and using imaging tests such as video-nasal endoscopy or multiview videofluoroscopy. These methods help provide visual confirmation of the condition. The process is done by a team of professionals that may include ear, nose, and throat doctors, speech therapists, and radiologists.
The primary method for diagnosing VPI is through perceptual speech analysis performed by a speech-language pathologist. This involves noting how you say certain sounds and checking for signs like overly nasal speech, unusual facial expressions, or mispronunciation. You could be asked to say phrases like “pet the puppies” or “Kathy kissed the cat,” or to count from 60 to 80. Holding a dental mirror under your nose can help check for air coming out of your nose while you speak, as this can indicate VPI.
Video-nasal endoscopy uses a small camera to examine the nasal passage, adenoids, and the soft part of the back of the roof of your mouth. Live images are displayed on a screen for the speech therapist, who can use them to further assess your speech. This technique can even identify a hidden cleft palate, which is a gap in the roof of the mouth that could potentially cause VPI.
If a child might not be comfortable with a nasal endoscopy, multiview videofluoroscopy can be used. A substance called barium is administered through the nose to coat certain areas such as the soft part of the rear of the roof of the mouth. Multiple X-ray images are then taken from different angles while the speech therapist evaluates your speech.
Another method, Cephalometrics, uses multiple x-rays to study relationships within the mouth and throat during sustained speech. This method helps to identify any abnormalities in the soft tissue of the throat.
Magnetic Resonance Imaging (MRI) can be used in some cases to study the muscles that control the soft palate, particularly for suspected submucosal or occult (hidden) clefts. MRI provides high-quality images and doesn’t expose you to radiation. However, it is a bit costly and might require sedation for young children.
A method called nasometry measures nasal emission and works out a ratio between nasal and oral sound emissions. While helpful, it does not pinpoint the exact location or measure the size of the velopharyngeal gap, which hampers airflow and results in unusually nasal speech.
Treatment Options for Velopharyngeal Insufficiency
Treatment options for VPI, also known as Velopharyngeal Insufficiency, which affects the seal between your mouth and nose during speech, include speech therapy, custom mouthpieces called prosthetics, and surgery.
Speech therapy is the first option for patients who’ve learned to pronounce words incorrectly due to VPI or haven’t found effective ways to adapt to it. Apart from this, patients who have had surgery to treat their VPI are often recommended to continue speech therapy to improve the results.
Prosthetics are used to lift the soft part of the roof of your mouth, aka the soft palate. These are custom-designed by dental specialists called prosthodontists and are attached to your upper teeth. However, most people don’t like wearing them, so they’re commonly used as a temporary solution or for those who aren’t good candidates for surgery. Occasionally, prosthetics can be used for patients with holes in their palate after surgery, or as a temporary measure before the second stage of repairing a palate.
Surgery is the final, definitive treatment for physical causes of VPI. The aim is to create a working seal between your nose and mouth, while avoiding blocking your airway. Most surgeries occur when the patient is over 3 years old since they’ll be more cooperative during diagnosis. Various types of surgeries include re-positioning of palate muscles, creating a flap in the back of the throat, sphincter pharyngoplasty, and augmenting, or expanding, the back wall of the throat.
One re-positioning surgery is the Furlow double-opposing Z-plasty, which was originally for repairing a cleft palate but also serves to lengthen the palate and realign some muscles. Compared to other types of surgeries, it has a lower risk of causing sleep apnea, a condition where breathing stops and starts during sleep. There’s also intravelar veloplasty where the soft palate is opened along the midline, a line drawn down the center of the body, and muscles that were not properly aligned during the initial surgery are corrected.
Pharyngeal flap surgery works best for those whose palate closes like a book. It involves creating a flap from the back wall of the throat and attaching it to the soft palate in the back of your mouth. This creates two side openings for airflow and effectively narrows the opening at the back of the nasal cavity, helping to prevent air leakage during speech. It’s also effective for some people with neurogenic causes of VPI, such as those with certain genetic conditions.
Sphincter pharyngoplasty is a surgery for those who have a closure pattern that’s more like a set of blinds closing, than a book. It involves creating flaps from the muscles in the throat, rotating them, and then suturing or stitching them together, forming a smaller central opening and closing the distance between the soft palate and back wall of the throat.
Another type of surgery is posterior pharyngeal wall augmentation where materials like cartilage or silicone are used to expand the back wall of the throat, decreasing the distance between it and the soft palate. It’s generally recommended for mild cases of VPI. It can also be combined with other surgical methods where a small gap still exists.
What else can Velopharyngeal Insufficiency be?
Both faulty learning and regional influences can cause a slight nasal tone in children’s speech, even if there’s no physical or neurological issue. When these nasal accents are more pronounced in some languages like French, Portuguese, and Polish, speech therapists need to consider this when assessing a child’s speech.
Speech Sound Disorder (SSD) is another common issue in children, where they struggle to make certain word sounds correctly. This might lead them to change, miss out, or replace these sounds, making their speech unclear. When children first start to speak, SSD is common but most tend to outgrow it by the age of 4. SSD encompasses articulation disorders that impact the physical production of speech sounds, and phonologic disorders that affect a child’s ability to learn and distinguish between different speech sounds. If a child doesn’t naturally outgrow SSD, they may require speech therapy before they start school.
One rare neurological disorder is Childhood Apraxia of Speech (CAS), which limits a child’s ability to accurately and consistently make speech sounds. This issue arises due to problems in motor programming and planning, which makes it hard for children to use the necessary oral-motor movements to express themselves. These children often make inconsistent mistakes when making consonant and vowel sounds while repeating sentences. Treating CAS involves an individualized treatment plan coordinated with an experienced speech and language therapist and the child’s caregivers.
Large tonsils can also interfere with the closure of the velopharynx, leading to Velopharyngeal Insufficiency (VPI). Removing the tonsils can alleviate these symptoms. Therefore, evaluating and managing an enlarged tonsil is essential before undertaking any surgery for VPI. Performing a pharyngoplasty, a type of surgery for VPI, without addressing an enlarged tonsil may further block a child’s airway.
What to expect with Velopharyngeal Insufficiency
Several studies have shown that up to 85% of patients have seen a successful decrease in excessive nasal resonance or “hypernasality” with a procedure known as pharyngoplasty. However, about 4% to 12% of patients might not see improvement after the surgery for VPI – a condition where there’s not enough contact or closure between the back of the throat and the back of the roof of the mouth – and may need additional or a repeat surgery.
In these studies, factors like sex, age, defects of the roof of the mouth, previous adenoid removal surgery or intelligence quotient (IQ) did not influence the outcomes of speech after surgery.
Also, in a systematic review of patients with a genetic disorder known as the 22q11 microdeletion syndrome who also had VPI, the type of pharyngeal flap – a surgical technique to correct speech – used did not significantly affect the success of the surgery or cause additional health risks or challenges.
Furthermore, the rates of failure from the first round of the two types of surgeries for VPI – primary pharyngeal flap and sphincter pharyngoplasty – have been shown to be equal. Importantly, both of these techniques can be corrected with another round of surgery if required.
Possible Complications When Diagnosed with Velopharyngeal Insufficiency
Surgery-related complications may involve issues such as blocked nasal airflow, nasal speech problems, and obstructive sleep apnea (OSA). Use of a pharyngeal flap (a type of surgical procedure) can lead to certain issues like narrowing of side openings, development of hyponasal voice due to nasopharynx blockage, and post-surgery OSA in about 1 in 5 patients.
It’s important to ensure that any exposed muscle during flap elevation is covered by natural mucosa. This is to avoid tissue shrinkage and suboptimal closure of the velopharyngeal opening.
Post-surgery OSA risk increases with sphincter pharyngoplasty (another surgical procedure), which might need flap revision. There are also risks with posterior pharyngeal wall augmentation such as movement or extrusion of the injected material, foreign body reaction, and fat particles in the bloodstream.
Remaining issues with the velopharyngeal gap and hypernasal speech can be addressed with a combined surgical approach using sphincter pharyngoplasty and Furlow palatoplasty. This combined approach needs fewer surgical changes than using only a pharyngeal flap or sphincter pharyngoplasty.
Potential complications:
- Blocked nasal airflow
- Nasal speech problems
- Obstructive sleep apnea (OSA)
- Narrowing of side openings
- Nasopharynx blockage
- Tissue shrinkage
- Suboptimal closure of velopharyngeal opening
- Movement or extrusion of injected material in posterior pharyngeal wall augmentation
- Foreign body reaction
- Fat particles in the bloodstream
Recovery from Velopharyngeal Insufficiency
After their operation, patients typically stay overnight in the hospital where they are observed for issues such as low blood oxygen levels, snoring, nasal-sounding speech, and Obstructive Sleep Apnea (OSA). They are allowed to go home once they’re able to eat and drink adequately. If there are worries about sleep apnea, the patient should have a sleep study called polysomnography to check for OSA. They might also need to use a device that helps keep their airway open, known as continuous positive airway pressure, for a short period.
If the patient continues to have sleep apnea, problems with the flow of air, or a nasal quality to their speech, they may need further adjustments to the surgical area (flap revision) or removal of the repair (takedown) in the long run. After the surgery, the patient should continue speech therapy to improve their speech and manage any inappropriate behaviors they developed prior to surgery.
Preventing Velopharyngeal Insufficiency
Understanding VPI, or voice and speech problems due to issues in the throat, can be a bit tricky. This is because the causes can vary a lot from person to person, and handling it often requires a team of experts. One key professional in this team is a speech-language pathologist. This person plays a crucial role in diagnosing the problem and helping the patient overcome any negative behaviors tied to their speech problems.
It’s crucial that patients and their families understand the different ways their condition can be evaluated. These methods could include perceptual speech analysis (studying the quality of speech), video nasal endoscopy (a procedure using a tiny camera to view inside the nose and throat), video fluoroscopy (a type of X-ray that makes it possible to see the throat in motion), and nasometry (measuring the sound waves of nasal and oral speech).
It’s important that the results from these tests and images are shared with the patient and their families. This information helps to guide a conversation about the best possible way to manage the condition. And if a surgery is being considered, the patients and parents need to be informed about the risks involved, such as blockage of the airway, sleep apnea (a condition where breathing stops and starts while sleeping), and the possible need for more surgeries later on.