Congenital laryngeal palsy

Other Names: Congenital vocal cord paralysis

Congenital laryngeal palsy is also known as congenital vocal cord paralysis. It represents 15%-20% of all cases of congenital anomalies of the larynx. It may affect one (unilateral) or both (bilateral) vocal cords. Vocal fold paralysis (also known as vocal cord paralysis) is a voice disorder that occurs when one or both of the vocal folds don't open or close properly. Single vocal fold paralysis is a common disorder. Paralysis of both vocal folds is rare and can be life threatening. The vocal folds are two elastic bands of muscle tissue located in the larynx (voice box) directly above the trachea (windpipe). When you breathe, your vocal folds remain apart and when you swallow, they are tightly closed. When you use your voice, however, air from the lungs causes your vocal folds to vibrate between open and closed positions. If you have vocal fold paralysis, the paralyzed fold or folds may remain open, leaving the air passages and lungs unprotected. You could have difficulty swallowing or food or liquids could accidentally enter the trachea and lungs, causing serious health problems.

Cause

The cause is often unknown (idiopathic). Congenital bilateral vocal cord paralysis may occur as a result of the immaturity of the nerve or muscle (neuromuscular) or as a result of central nervous system problems, such as Arnold-Chiari syndrome, cerebral palsy, hydrocephalus, myelomeningocele, spine bifida, hypoxia (lack of oxygen in the blood), or bleeding.

In other cases the vocal cords' paralysis is acquired. For example, a birth trauma may cause tension in the neck and lead to bilateral vocal cord paralyses that can last 6-9 months. Other causes may include:

• Surgical Trauma • Malignancies • Delayed endotracheal intubation • Neurological diseases • Strokes • Choking • Diseases that result in inflammation of the vocal cords or the laryngeal cartilage (Wegener's granulomatosis, sarcoidosis or polychondritis, gout, syphilis and tuberculosis (resulting in mechanical attachment of the vocal cords) • Diabetes mellitus, which may lead to a neuropathy resulting in vocal cord paralysis • Gastroesophageal reflux (GER).

The unilateral paralysis is usually idiopathic but may also be secondary to mediastinal lesions, such as tumors or vascular malformations or iatrogenic (caused by damage to the left recurrent laryngeal nerve during surgery in this area, such as heart surgery). It may also result from problems of the mechanical structures of the larynx as the cricoarytenoid joint.

Signs and symptoms

The signs and symptoms of bilateral vocal fold paralysis may include making a noise when breathing (inspiratory stridor) that worsens upon exercise, progressive obstruction of the respiratory airway, aspiration, recurrent chest infections, cyanosis, nose flaring and signs of cranial nerve deficits during the head and neck exam.

Diagnosis

Vocal fold paralysis is usually diagnosed by an otolaryngologist—a doctor who specializes in ear, nose, and throat disorders. He or she will ask you about your symptoms and when the problems began in order to help determine their cause. The otolaryngologist will also listen to your voice to identify breathiness or hoarseness. Using an endoscope—a tube with a light at the end—your doctor will look directly into the throat at the vocal folds. Some doctors also use a procedure called laryngeal electromyography, which measures the electrical impulses of the nerves in the larynx, to better understand the areas of paralysis.

Treatment

The most common treatments for vocal fold paralysis are voice therapy and surgery. Some people's voices will naturally recover sometime during the first year after diagnosis, which is why doctors often delay surgery for at least a year. During this time, a speech-language pathologist may be needed for voice therapy, which may involve exercises to strengthen the vocal folds or improve breath control while speaking. Patients may also learn how to use the voice differently, for example, by speaking more slowly or opening the mouth wider when speaking.

Treatment may include: Corticosteroids: When there is an associated disease such as Wegener's granulomatosis, sarcoidosis or polychondritis. Medical treatment of the disease that lead to an inflammation of the cricoarytenoid joint ( gout) or the laryngeal mucosa such as syphilis and tuberculosis (resulting in mechanical attachment of the vocal cords) to improve breathing.

Diabetes treatment: Can help to improve a neuropathy of the vocal cords caused by the diabetes mellitus.

Treatment of reflux: When the condition is caused by the gastroesophageal reflux.

Treatment of the eventual scarring of the arytenoid cartilages.

Several surgical procedures depending on whether one or both of the vocal cords are paralyzed. The most common procedures change the position of the vocal fold. These may involve inserting a structural implant or stitches to reposition the laryngeal cartilage and bring the vocal folds closer together. These procedures usually result in a stronger voice. Surgery is followed by additional voice therapy to help fine-tune the voice:

• Functional procedures as microflap, laryngectomy (similar to tracheostomy) with subsequent cricoidotomia (removal of the cricoid cartilage) and cartilage graft and stent (or stent placement only) or reconstruction of the local mucosa with scar removal. • Tracheotomy: May be required to help breathing. In a tracheotomy, an incision is made in the front of the neck and a breathing tube is inserted through an opening, called a stoma, into the trachea. Rather than occurring through the nose and mouth, breathing now happens through the tube. Following surgery, therapy with a speech-language pathologist helps you learn how to use the voice and how to properly care for the breathing tube • Permanent treatments with removal of the vocal cords (unilateral or bilateral) or the arytenoid cartilage (endoscopic or external, partial or complete) or changing the position of the vocal cords.

Other treatment may include: • Reinnervation techniques (experimental) • Electrical stimulation (experimental).

Most cases of unilateral vocal cord paralysis do not need any treatment. Adopting a vertical position is sometimes enough to relieve breathing problems but in some patients it may require an intubation.

NIH genetic and rare disease info

• NIH info on Congenital laryngeal palsy

Congenital laryngeal palsy is a rare disease.

Congenital laryngeal palsy Resources
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