Recurrent respiratory papillomatosis (RRP) is an uncommon condition characterized by non-cancerous growths, known as papillomas, within the respiratory tract. These growths can emerge anywhere from the nasal passages down to the lungs. Their presence can lead to health challenges, particularly when they affect the larynx.
Understanding Recurrent Respiratory Papillomatosis
RRP is caused by infection with Human Papillomavirus (HPV), primarily types 6 and 11. These HPV types lead to the proliferation of cells in the respiratory passages, forming wart-like papillomas.
The condition presents in two forms: juvenile-onset RRP (JORRP) and adult-onset RRP (AORRP). JORRP typically manifests in children under the age of 12, often between two and six years old, and is generally considered more aggressive with a higher rate of recurrence and a greater need for surgical intervention. AORRP occurs in older individuals, commonly in their fourth decade of life, and tends to follow a milder course. JORRP is often linked to peripartum transmission from an infected mother.
Recognizing Symptoms and Diagnosis
The symptoms of RRP vary depending on the location and size of the papillomas within the respiratory tract. The most common symptom is hoarseness or a change in voice quality. As the papillomas grow and obstruct the airway, other symptoms may emerge, including noisy breathing (stridor), a persistent cough, and difficulty breathing.
Diagnosing RRP typically begins with a review of the patient’s medical history and a physical examination. Direct visualization of the airways is performed to confirm papillomas and determine their extent. This involves procedures such as laryngoscopy for the larynx, or bronchoscopy for the lower airways like the trachea and bronchi. Biopsies are often taken during these procedures to confirm the diagnosis.
Treatment Approaches
The primary goal of treating RRP is to remove the papillomas to maintain a clear airway and improve voice function. Surgical methods are the mainstay of treatment due to the recurrent nature of the growths. Common surgical techniques include microlaryngeal surgery using cold instruments, laser surgery (such as with a CO2 laser), and microdebriders.
Because papillomas tend to regrow, patients often require repeated surgical procedures. To manage severe or rapidly recurring cases, adjunctive therapies may be used in combination with surgery. These include antiviral medications like cidofovir, often injected directly into the lesions, and anti-angiogenesis drugs such as bevacizumab, which can be given systemically or injected. Other therapies like indole-3-carbinol, mumps vaccine, and photodynamic therapy have also been explored.
Managing Recurrence and Long-Term Care
RRP is a chronic condition characterized by the frequent regrowth of papillomas, necessitating ongoing medical observation and repeated treatments throughout a patient’s life. Patients with RRP often require multiple surgeries over many years to manage their disease. This persistent nature can lead to complications such as airway obstruction, which may require a tracheostomy in severe cases to ensure breathing. Papillomas can also spread to other parts of the respiratory tract, including the trachea and lungs.
While rare, there is a risk of malignant transformation, where the benign papillomas can develop into squamous cell carcinoma, particularly if the growths have spread to the lower respiratory tract. Regular follow-up appointments with an ear, nose, and throat (ENT) specialist are important to monitor disease progression and address any new growths or complications. The HPV vaccine, which protects against HPV types 6 and 11, can help prevent new HPV infections, thereby reducing the incidence of RRP in future generations. While it is not a cure for existing RRP, it may have a therapeutic benefit by reducing the number of surgeries needed and increasing the time between procedures.