Recurrent Respiratory Papillomatosis (RRP) is a rare disease that is characterized by the growth of tumors in the respiratory tract caused by the human papilloma virus (HPV). Although the tumours typically occur in the larynx on and around the vocal cords, they may spread downward and affect the trachea, bronchi, and sometimes the lungs.

The RRP growths often resemble warts with cauliflower-like heads. They may be attached to the vocal cords by a slim stalk or they may adhere to mucosa in the region. They may even occur above or below the vocal cords. In fact, they can occur anywhere along the respiratory tract.

There is a lot of evidence showing that RRP in children results from transmission of HPV from mother to child via genital condylomas. HPV types 6 and 11, which are responsible for 80–90% of the condylomas, are responsible for nearly 100% of JoRPP.

Furthermore, it was found that mothers under the age of 20 who have condyloma during pregnancy and who deliver their first-born child vaginally, appear to be at greatest risk of infecting their newborn children.

In adults, RRP is also caused by infection with HPV-6 and HPV-11, but it is suspected that the virus is sexually transmitted.

Both children and adults can develop or have RRP.

In children, JORPP (Juvenile Onset RRP) is usually diagnosed before the age of five and almost always by the age of ten. In the case of adults, AORRP (Adult Onset RRP) may show itself at any age.

It is still not well understood why only certain individuals present with RRP. In the US population, it is estimated that approximately 5% of people may have HPV in their respiratory tract, but less than 1 in 1 000 of infected people develop RRP. In Africa, these statistics are currently not known.

Usually an ear, nose and throat (ENT) surgeon will perform an examination of the larynx (air passage to the lungs) and the vocal cords using a flexible fibreoptic camera through the nose or a rigid camera through the mouth. To make a definitive diagnosis, however, a direct laryngoscopy and surgical removal of the papilloma growths is performed with the patient under general anesthesia. A biopsy of the growth is taken and tested for HPV.

Often RRP is misdiagnosed by paediatricians and general practitioners who mistakenly assume that the shortness of breath and stridor are the result of asthma or croup. Misdiagnosis can be serious as the airway is partially obstructed by growths that should be removed straight away.

Unfortunately, the only current treatment for RRP is to surgically remove the growths. However, growths typically re-occur, which means that multiple surgeries are required over the course of a patient’s lifetime.

Research is ongoing and RRP Africa aims to be at the forefront of research in Africa with a view to preventing the transmission of the HPV virus.

Currently only surgery can remove growths that become problematic, and due to the recurring nature of the disease, multiple surgeries are typically required. This is often traumatic for the patient, and repeatedly risks scar formation.

Furthermore, any adjuvant drug therapies that are currently used to treat RRP patients are not approved by the FDA, nor by other accepted medical associations, which means that many medical aids do not cover them.

In Africa, most patients are reliant on under-resourced public healthcare facilities for diagnosis and treatment, with the result that RRP remains undiagnosed, misdiagnosed, or inadequately funded. Tracheostomies are often performed instead of surgery for those who cannot make it to theatre on a regular basis.

It is best to seek treatment from an ENT specialist who knows the most about RRP, as they will be familiar with all the latest research, treatments and adjunct therapies. They will be able to best advise the patient from both a surgical and voice preservation perspective.

Yes, it can. Recurring Respiratory Papilloma occurs from the human papilloma virus (HPV). HPV is usually transmitted sexually, and can be present in mothers and then transmitted to the child during pregnancy. It is typically the first child who is affected. RRP can be reduced through HPV vaccination in childhood and prior to the child’s first sexual activity, in order to prevent getting HPV as an adult.

There is currently no cure for RRP, and no FDA-approved pharmaceuticals are available to treat RRP. A distinguishing aspect of this disease is the tendency for the papilloma to recur after surgical procedures to remove them. This is why it is called “Recurrent” Respiratory Papilloma. It is typically self-limiting, in that the disease will eventually burnt out. However, there are children who continue to have recurrence into adulthood. In this case, there is a small risk of malignant transformation.

The most obvious approach is to introduce widespread HPV vaccination globally with Gardasil®, particularly in developing countries. Preventing the spread of HPV will, in turn, prevent the transmission of the virus between partners, and ultimately between mother and child. This approach requires extensive education and the mass roll-out of vaccination programmes by medical communities and clinics in each country.

For children and adults who already have RRP, ongoing research is also necessary. The development of new and safer treatments that do not involve surgery is preferable.

For now, the quality of life of patients who already have RRP can be enhanced through improved diagnosis and patient care. This is achieved by providing the appropriate training to ENT surgeons within the field and health professionals in paediatrics and child health, who are the entry point for the many children who present with airway symptoms. Further support and sharing of information pertaining to RRP across the African continent is necessary to ensure that collaborative experience is shared, and that patient care is optimised and streamlined.

ENT Surgeons in Africa who manage patients with RRP are encouraged to join our RRPAfrica Registry and contribute to RRP research and information on the African continent.