Recurrent respiratory papillomatosis (RRP) is a rare disorder characterized by the development of small, wart-like growths (papillomas) in the respiratory tract. Papillomas can develop anywhere along the respiratory tract, but most often affect the larynx and the vocal cords (laryngeal papillomatosis). Less often, the disorder affects the area within the mouth (oral cavity), trachea and bronchi. Only in rare cases do these growths spread to affect the lungs. Papillomas are noncancerous (benign), but in extremely rare cases can undergo cancerous (malignant) transformation. In RRP, papillomas have a tendency grow back after they have been removed. RRP can affect children or adults and is caused by infection with human papillomavirus (HPV), although exposure to the virus alone is insufficient to cause the disease.
Signs and symptoms of recurrent respiratory papillomatosis
Symptoms and severity of recurrent respiratory papillomatosis differ greatly from person to person. In some cases, the disease may resolve without treatment or it may remain stable requiring only periodic intervention.
The most common presenting symptom of RRP is hoarseness. Hoarseness may become progressively worse and the voice of an affected individual may be weak or sound low. Affected individuals may develop laboured, noisy breathing (stridor) due to obstruction of the airway. Initially, stridor may occur when breathing in, but overtime occurs both when breathing in and out.
Additional symptoms that can develop include a chronic cough, difficulty swallowing (dysphagia), shortness of breath or difficulty breathing
(dyspnoea), the sensation of a foreign body in the throat, and choking episodes. Left untreated, recurrent respiratory papillomatosis can eventually compromise the airways, resulting in life-threatening breathing difficulties (acute respiratory distress). If the disease spreads to the lungs, affected individuals can potentially experience recurrent pneumonia
, chronic lung disease
(bronchiectasis) and, ultimately, progressive pulmonary failure
A variety of conditions can produce signs and symptoms that are similar to those seen in RRP. Such conditions include asthma, allergies, chronic bronchitis
, croup, vocal nodules, and Gastroesophageal reflux
. Comparisons may be useful for a differential diagnosis.
Causes of recurrent respiratory papillomatosis
Recurrent respiratory papillomatosis is caused by the human papillomavirus (HPV) which is very common in human beings. HPV is passed through genital contact, most often during sex. Most individuals who are infected with HPV never develop any symptoms. There are more than 150 different subtypes of HPV and approximately 40 of these subtypes can affect the genital tract. Two specific subtypes, HPV 6 and HPV 11, account for more than 90% of cases of recurrent respiratory papillomatosis.
In children, the most likely cause of the transmission of HPV is passage from an affected mother to the child during labour as the child passes through the birth canal.
Most children born to women with HPV do not develop RRP. In addition, many individuals with HPV in the tissues of the respiratory tract never develop papillomas. This highlights the contribution of immunologic or genetic ones in the development of RRP in individuals with HPV.
Diagnosis of recurrent respiratory papillomatosis
A diagnosis of recurrent respiratory papillomatosis is based upon identification of characteristic symptoms (e.g. papillomas), a detailed patient history, a thorough clinical evaluation and specialized tests such as indirect or direct laryngoscopy. These procedures are performed by qualified ENTs.
Treatment of recurrent respiratory papillomatosis
There is no cure for recurrent respiratory papillomatosis. Treatment is directed toward removing papillomas, decreasing the spread of disease, creating safe and patent airway, preserving nearby anatomical structures, improving voice quality if necessary and increasing the time between surgical procedures
. Treatment may require the coordinated efforts of a team of specialists. Paediatricians
, ear-nose-throat specialists
that focus on children, anaesthesiologists
, speech pathologists, and other healthcare professionals may need to systematically and comprehensively plan a child’s treatment.
Specific therapeutic procedures and interventions may vary, depending upon numerous factors, such as frequency of disease recurrences specific location and spread of the disease papilloma size the presence or absence of certain symptoms an individual’s age and general health and other elements.
The mainstay of treatment is surgical removal of papillomas. However, these growths often return necessitating more surgery. The recurrence of papillomas is unpredictable. Some individuals may require surgery every few weeks while others may only require surgery twice a year or only a few times during their life.
In severe cases where tumour growth is aggressive, an affected individual may need a tracheotomy to keep the breathing airways open. A tracheotomy involves surgically inserting a tube into the windpipe (trachea). A tracheotomy is used only as a method of last resort because the procedure may allow for spread of the disease further into the respiratory tract.
After surgery, some individuals may receive certain medications designed to slow the re-growth of papillomas and increase the time between surgeries.