What is velopharyngeal dysfunction?

Velopharyngeal dysfunction (VPD) is a term used to describe disorders characterized by the abnormal function of the velopharyngeal valve, including velopharyngeal insufficiency (VPI).

The velopharyngeal valve includes the soft palate as well as the pharynx and the side and back walls of the throat. The purpose of these structures is to separate the oral and nasal cavities during swallowing and speech. When the velopharyngeal valve is not functioning correctly, it can cause complications with swallowing and speech.

Causes

VPD can result from structural, neuromotor or other functional causes. VPD can occur in isolation or with other medical or genetic conditions. CHOP’s Velopharyngeal Dysfunction Program treats patients with all forms of VPD resulting from the following:

• Cleft palate
• Submucous cleft palate
• Adenoidectomy, tonsillectomy
• Congenital VPD
• Disorders associated with genetic syndromes, some of which include:
  • The 22q11.2 deletion syndrome is the most common cause of syndromic palatal abnormalities. We are part of CHOP’s internationally recognized 22q and You Center, which specializes in the diagnosis and multidisciplinary management of children with a chromosome 22q11.2 deletion.
  • Neurofibromatosis (NF)
  • Kabuki syndrome
• Palatal tumors

Signs and symptoms

When velopharyngeal closure does not occur properly, food or excess air can escape through the nose. This can result in several speech disorders, such as:

• Hypernasal speech
• Inability to generate pressure for speech sounds
• Inability to form speech sounds correctly

These speech disorders can significantly interfere with your child’s ability to communicate and impact development and social functioning.

Children with VPD may also require treatment for laryngeal abnormalities, sleep apnea, voice disorders, hearing loss or ear disease, and problems with their tonsils and adenoids.

Evaluation and diagnosis

During your child’s first visit, we will conduct a comprehensive clinical examination to get a thorough understanding of his condition and any potential complications.

If needed, your child will undergo testing to evaluate the velopharynx during speech. This is done using video nasoendoscopy or video fluoroscopy.

• Video nasoendoscopy — Video nasoendoscopy involves placement of a small camera through the nose to visualize the velopharyngeal mechanism from above while your child is speaking. This study provides information about the anatomic structures involved in speech production and specific information about how they are working to produce speech. Our center is equipped with video nasoendoscopic equipment and can perform this study on the day of your visit if indicated.
• Video fluoroscopy — Video fluoroscopy is a good alternative study to evaluate the speech mechanism for children who may not tolerate nasoendoscopy. This imaging technique is performed in the Radiology Department with both a radiologist and a speech pathologist present. During this study, the anatomic structures involved in speech production are evaluated. A barium  solution is placed within the nose to help outline the structures better. A video is then taken during speech production.

Treatment

After your child’s initial evaluation, our team will collaborate to determine the most appropriate timing and course of treatment. We will provide recommendations and work with your family to schedule any necessary surgical intervention, as well as establish a plan for specialized speech therapy or other required therapies. Depending on your child’s condition, we may also recommend an evaluation by a geneticist. This can often be set up for the day of your visit, or we can make referrals as needed.

Surgical treatment of VPD

If surgery is indicated to correct velopharyngeal dysfunction, your child’s surgeon will collaborate with the other specialists on his care team to determine the best timing for pharyngeal surgery. Your child’s medical team will also work together to plan for additional pre- and perioperative treatment that will further your child’s progress and recovery.

Our plastic surgeons are experienced in a variety of surgical techniques and procedures to treat VPD, and will recommend the treatment option that is best for your child’s unique condition. Some common procedures include:

1. Furlow palatoplasty — The Furlow palatoplasty is a common technique used in cleft palate repair. This procedure can lengthen the palate and improve its function. It is also used to repair submucosal cleft palate and as a secondary revision of a previous cleft palate surgery where the muscles in the soft palate have not been repaired.
2. Palatal revisions — Revisions of the palate may be recommended if the palate has not healed properly after the initial cleft repair and an oronasal fistula (hole or opening) between the nose and the mouth is present. The hole can contribute to hypernasality and surgery to close the hole may be recommended to improve the quality of speech.
3. Posterior pharyngeal flap — This surgical procedure involves taking tissue from the back of the throat and attaching it to the soft palate. This prevents air from leaking out of the nose during speech.
4. Sphincter pharyngoplasty — During a sphincter pharyngoplasty, your child’s surgeon will take tissue from the sides of the throat behind the tonsils and sew them into the back of the throat. This will decrease the size of the space and prevent air from leaking out of the nose during speech.

VPD surgery in patients with 22q11.2 deletion syndrome

Up to 75 percent of patients with the 22q11.2 deletion syndrome are affected by palatal disorders, including velopharyngeal dysfunction, submucous cleft palate or cleft palate. These patients can have significant speech and language delays, hypernasal speech and articulation problems.

Many 22q11.2 patients with significant speech disorders related to palatal issues will seek surgical intervention in early childhood. Pharyngeal surgery to treat VPD in patients with the 22q11.2 deletion can result in substantial improvements in speech and communication, social functioning, and can significantly improve quality of life.

Thoughtful surgical decision making that includes the family is particularly important in patients with 22q11.2 deletion because of their multiple, often complex, congenital anomalies. Patients treated at CHOP will undergo a thorough preoperative evaluation to determine if VPD surgery is the best option.

This advanced screening frequently includes evaluation for cervical spine abnormalities, carotid artery abnormalities and obstructive sleep apnea. Additional screenings are based on each patient’s medical history. A thorough preoperative evaluation can help to minimize surgical risk factors and help the team determine the most appropriate treatment plan for your child. Our preoperative screening protocols are established to achieve the safest outcomes and help families decide what is best for their child.

Speech therapy for VPD

Children with velopharyngeal conditions require specialized speech therapy techniques. Our team of speech therapists is specially trained to evaluate and treat children with VPD, and will work with your family to make the appropriate treatment recommendations and referrals for therapy, both within CHOP in the Center for Childhood Communication and with specialists in your community.

Psychosocial support

Our team will refer your child to a psychologist if indicated to help them cope with any social challenges related to their condition, such as forming relationships or functioning in school. The Division of Plastic, Reconstructive and Oral Surgery has a dedicated psychologist who specializes in supporting children and adolescents with appearance differences or other conditions that have an impact on development or social activities. Learn more about the psychosocial support services available to your child.

Follow-up care

If your child is 3 1/2 years of age or older, is speaking, and is suspected of having VPD or has a diagnosis of VPD, he is a good candidate for The Children’s Hospital of Philadelphia’s (CHOP) multidisciplinary Velopharyngeal Dysfunction Program (VPD Program).

The VPD Program brings together all of the related specialties that may be needed to manage both the functional and social aspects of your child’s condition. During his clinic visits, he will receive coordinated care from a multidisciplinary team, including the core components of plastic surgery, speech pathology and psychology. Your child’s care team may also include experts from audiology, genetics, radiology, and more.

Referrals to other specialists can be made as needed, to address related issues such as sleep apnea, voice disorders, hearing loss, and other ear, nose and throat conditions. The VPD Program works closely with the Cleft Lip and Palate Program, the Division of Otolaryngology (also known as Ear, Nose and Throat, or ENT) and the Sleep Center in the Division of Pulmonology to manage your child’s care. If your child is non-verbal, he may be cared for by other clinics.

We work closely with your primary care physician, local speech pathologists and any school or community programs to provide the most coordinated, collaborative care for your child. Our team can make any referrals or recommendations for additional specialists or therapists as needed, including neuropsychologists or developmental pediatricians.

Depending on your child’s underlying condition, his long-term care may also be managed in coordination with one of our other specialty programs, such as the 22q and You Center.

Our team can make any referrals or recommendations for additional specialists or therapists as needed, including neuropsychologists or developmental pediatricians.