What are the other Names for this Condition? (Also known as/Synonyms)

• Congenital Vocal Cord Paralysis

What is Congenital Laryngeal Palsy? (Definition/Background Information)

• 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
• The cause of bilateral paralysis of the vocal cords is often unknown (idiopathic). In some cases, paralysis may be secondary to the immaturity of the nerve or muscle (neuromuscular) or due to central nervous system damage (including the Arnold-Chiari malformation, cerebral palsy, hydrocephalus, myelomeningocele, spina bifida, hypoxia (lack of oxygen in the blood), or bleeding)
• Birth trauma that causes excessive tension in the neck can cause transient bilateral vocal cord paralysis that can last 6-9 months. Unilateral paralysis is usually idiopathic but can be secondary to problems with the vagus nerve or recurrent laryngeal nerve trauma
• The signs and symptoms of bilateral vocal cord 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
• Flexible endoscopy can be used to diagnose the condition
• The treatment of Congenital Laryngeal Palsy may include medication, surgery, and speech therapy

(Source: Congenital Laryngeal Palsy; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Who gets Congenital Laryngeal Palsy? (Age and Sex Distribution)

• Congenital Laryngeal Palsy is a rare congenital disorder. The presentation of symptoms may occur at birth 
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Congenital Laryngeal Palsy? (Predisposing Factors)

The risk factors for Congenital Laryngeal Palsy may include:

• Injury to vagus nerve
• Immature muscle or nerve in the larynx
• Trauma to laryngeal nerve
• Birth trauma

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Congenital Laryngeal Palsy? (Etiology)

• Congenital Bilateral Vocal Cord Paralysis may be caused by:
  • Immaturity of the nerve or muscle (neuromuscular) 
  • Central nervous system problems, such as Arnold-Chiari syndrome, cerebral palsy, hydrocephalus, myelomeningocele, spine bifida, hypoxia (lack of oxygen in blood)
  • Bleeding
• Acquired vocal cord paralysis: A birth trauma may cause tension in the neck and lead to bilateral vocal cord paralyses
• 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)
• 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) 
  • Problems of the mechanical structures of the larynx as the cricoarytenoid joint

(Source: Congenital Laryngeal Palsy; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

What are the Signs and Symptoms of Congenital Laryngeal Palsy?

The signs and symptoms of Congenital Laryngeal Palsy may vary among affected individuals in type and severity:

• A wheezing or vibrating sound while breathing
• Weak cry
• Inability to make noise
• Feeding difficulties
• Breathing difficulties

How is Congenital Laryngeal Palsy Diagnosed?

Congenital Laryngeal Palsy is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Congenital Laryngeal Palsy?

The complications of Congenital Laryngeal Palsy may include:

• Inadequate weight gain
• Respiratory distress

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Congenital Laryngeal Palsy Treated?

The most common treatments for Congenital Laryngeal Palsy are voice therapy and surgery.

• Voice therapy, 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
• Medications:
  • 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
• Surgery, followed by additional voice therapy 
• Functional procedures, such 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 
• 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

(Source: Congenital Laryngeal Palsy; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

How can Congenital Laryngeal Palsy be Prevented?

• Congenital Laryngeal Palsy may not be preventable, if associated with immature muscle and nerve fibers, trauma during birth, or if it occurs as part of a syndrome with a genetic basis
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as Congenital Laryngeal Palsy
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Congenital Laryngeal Palsy? (Outcomes/Resolutions)

• The prognosis of Congenital Laryngeal Palsy is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• Many cases are known to resolve on their own over the course of time

Additional and Relevant Useful Information for Congenital Laryngeal Palsy:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/