Velopharyngeal Insufficiency - Causes, Symptoms, Diagnosis, and Treatment

Introduction:

VPI or velopharyngeal insufficiencies are congenital (inborn) or genetic defects that occur due to improper seals between the oral and the nasal cavities resulting in a gap between them. The presence of a gap between the nasal and the oral cavities results in air escaping from the nose, which leads to nasal twang (tone) in the voice along with other speech difficulties as well.

How Do the Velopharyngeal Muscles Work During Normal Speech?

The muscles of the soft palate usually move upward and downwards to contact the back of the throat, creating an adequate seal between the nasal cavity and the oral cavity. The tight seal created between the nasal and oral cavities prevents the escape of air from the nose, thus resulting in a normal vocal tone and speech.

What Happens in Velopharyngeal Insufficiency?

In velopharyngeal insufficiency, there is the failure of the muscles of the soft palate to create a complete seal between the nasal cavity and the oral cavity as it does not contact the back of the throat tightly. This results in the escape of the air through the nose resulting in speech difficulties and nasal tone in the voice. Patients with velopharyngeal insufficiency find it difficult to pronounce oral consonants such as alphabets like p, w, and x.

What Are the Conditions That Can Result in Velopharyngeal Insufficiency?

VPI or velopharyngeal insufficiency can occur due to various conditions like:

• Genetic or Congenital Birth Defects: Like cleft palate and underdeveloped or short palate. Cleft palate is the most common reason for velopharyngeal insufficiency.

• Infections: Viral or bacterial infections resulting in enlarged tonsils and adenoids in children.

• Congenital Anomalies: Like Down syndrome, Turner syndrome, and neurofibromatosis due to underlying structural defects.

• Neurological Conditions: Neurological conditions affecting the muscles cause muscle weakness, like cerebral palsy or Parkinson's disease.

• Tumors: Tumors of the palate.

What Are the Signs and Symptoms Associated With Velopharyngeal Insufficiency?

Signs and symptoms associated with velopharyngeal insufficiency are:

• The nasal tone in the voice.

• Speech difficulties like problems in pronouncing a few alphabets like p, b, g, w, t, d, and x.

• Backflow or regurgitation of the stomach contents through the nose.

• Escape of air through the nose.

What Are the Diagnostic Techniques to Rule Out Velopharyngeal Insufficiency?

Various diagnostic modalities used to rule out velopharyngeal insufficiency are:

• Clinical Assessment: Clinical assessment and history is taken from the parents for the symptoms and difficulties faced by the child. Clinical evaluation is done by the analysis of the speech and nasal tone in voice if present, and a complete clinical examination of the palate is done to check for any structural abnormalities.

• Speech Assessment: Analysis of the speech is done by the speech and language pathologist. Patients are asked to produce some sounds with alphabets like p, d, x, and b to check for any nasal twang in voice and escape of air through the nose. Patients are also asked to do counting from 60 to 80 with simultaneous placement of a dental mirror near the nose. Fogging of the mirror is then checked to rule out any air escape from the nose while counting.

• Video-Nasal Endoscopy: A flexible endoscope is used to completely examine the nasopharynx, nasal cavities, tonsils, and adenoids to rule out the presence of any structural defects. The endoscope consists of an attached camera which presents the lie images of the internal body structures on the video monitor screen for clear visibility. The location, position, mobility, and size of the gap at the velopharyngeal sphincter are determined using endoscopy to plan the surgical management accordingly.

• Videofluoroscopy: It is a preferred technique in children who are not able to tolerate the nasal endoscopy procedure. In this method, an agent like barium is introduced in the patient through a trans-nasal route which coats the soft palate and pharyngeal walls laterally and on the backside. The image is then recorded in different views like lateral, frontal, and basal views to evaluate the pattern of velopharyngeal (tongue-pharynx) closure. There are three different patterns of velopharyngeal closure:

1. Coronal Closure: It involves only the soft palate and the back side (posterior) pharyngeal walls.

2. Sagittal Closure: It involves only the lateral or side walls of the pharynx.

3. Circular Closure: It has equal involvement in all the associated structures.

• Cephalometric Analysis: Serial radiographs are taken to evaluate the anatomy of the velopharyngeal (tongue-pharynx) relationship. Skull base and cervical spine deformities can also be evaluated using this method, especially in cases of congenital anomalies like Down’s syndrome.

• Magnetic Resonance Imaging: It helps in assessing the muscles of the soft palate for its size, position, and insertion.

• Nasometry: It helps in determining the ratio of emission of nasal and oral sounds. However, this technique does not help to measure the velopharyngeal gap.

How To Manage Velopharyngeal Insufficiency?

Velopharyngeal insufficiency can be managed by different methods:

• Speech Therapy: Speech therapy is given by speech-language pathologists to patients who have undergone surgical management of velopharyngeal defects. Speech therapy helps in the improvement of the speech of the patient.

• Oral Prosthesis: A customized palatal prosthesis is made by prosthodontics (specialists in oral and maxillofacial prosthesis) for temporary repair of the soft palate defect till the final stage of treatment.

• Surgical Management: Surgical treatment of the velopharyngeal gaps is the most accepted management of velopharyngeal insufficiency. It is usually performed in children after the age of three to four years. Different surgical repair procedures involve:

1. Posterior pharyngeal flap procedures.

2. Sphincter pharyngoplasty procedure.

3. Furrow palatoplasty.

4. Posterior pharyngeal wall augmentation.

What Are the Complications Associated With Velopharyngeal Insufficiency?

Few risks are associated with all surgical procedures. Complications related to surgical treatment of velopharyngeal insufficiency are:

• Nasal airflow obstruction.

• Obstructive sleep apnea (snoring while sleeping)-Mostly observed after pharyngeal flap surgery.

• Hypernasal speech (increased nasal tone in speech).

• Worsening of speech problems.

• Hyponasal speech (decrease nasal cavity resonance during speech).

Conclusion:

Velopharyngeal insufficiency can be due to multifactorial reasons. Velopharyngeal insufficiency hampers the quality of life of both the child and their parents. It occurs when the seal between the nasal cavity and oral cavities is not adequate resulting in a gap. It can lead to nasal twang in the voice along with speech deformities seen in the child as they are not able to pronounce oral consonants like p, x, d, b, and z. Early diagnosis and management of the underlying cause of velopharyngeal insufficiency should be made by a multidisciplinary approach to achieve acceptable treatment outcomes and prevent future complications.