Recurrent Respiratory Papillomatosis (RRP) :: Nationwide Children's Hospital

Recurrent Respiratory Papillomatosis (RRP)

What is recurrent respiratory papillomatosis (RRP)?

RRP is caused by the Human Papilloma Virus (HPV), which is a highly prevalent virus. HPV causes wart-like lesions that most commonly grow on or next to the vocal folds. They also can grow anywhere in the airway, above or below the vocal cords. In particularly severe cases, the lesions can spread into the lungs.

RRP can be diagnosed in childhood (juvenile onset RRP) or adulthood (adult onset RRP). When found in children, it is most commonly passed from mother to child, possibly during pregnancy or childbirth.

What are the signs and symptoms of recurrent respiratory papillomatosis?

RRP most commonly causes a hoarse voice. This can be present very early on, and a child may have a hoarse cry as an infant. Children are generally diagnosed around three to four years of age.

As the lesions become larger, they can begin to cause airway obstruction. When this happens, you may notice noisy breathing, difficulty breathing with exertion or during illness. If left untreated, the airway can become critically narrowed. It is important to seek evaluation if you are noticing these signs or symptoms persisting in your child.

What causes recurrent respiratory papillomatosis?

Human Papilloma Virus (HPV) causes Recurrent Respiratory Papillomatosis. This virus is very common, but it is not well understood why some people exposed to the virus develop RRP and others are completely without symptoms. Once a person has the virus in his or her body, it is always there, but it may lay dormant or may begin to cause symptoms.

How is recurrent respiratory papillomatosis diagnosed?

RRP is diagnosed by visualization of lesions in the airway. Lesions can be seen by flexible nasopharyngoscopy or laryngoscopy and bronchoscopy. Once noted, diagnosis is confirmed by biopsy.

How is recurrent respiratory papillomatosis treated?

Unfortunately, we do not currently have a cure for HPV or RRP. Lesions can be excised by multiple techniques, but are typically removed during a rigid laryngoscopy and bronchoscopy procedure.

There are multiple different instruments that can be used to remove the lesions, and the child’s surgeon will discuss the risks and benefits of the techniques that be recommended. While lesions are removed as completely as possible, the virus remains in the body, and the lesions could grow back over time. Sometimes this happens very slowly and a child requires only one to two procedures a year to remove the lesions.

In other cases, lesions can grow very rapidly, and a child may require procedures monthly or even more often to prevent airway obstruction. As a rule, every child with RRP will require multiple procedures throughout life to remove lesions.

Growth of the lesions may vary throughout a child’s lifespan, so it is important to be aware of developing symptoms even if your child has been doing well. For those children who have very rapid growth of the lesions, there are additional treatment options that can be considered. The child’s surgeon will discuss treatment options. The pediatric ENT surgeons at Nationwide Children’s have received additional training in techniques for managing RRP.

If my child has recurrent respiratory papillomatosis, when should I be concerned?

It can be difficult for a parent or family member to discern the severity of a breathing problem when a child has a history of RRP. If you are concerned, seek medical evaluation immediately.

In particular, signs that indicate more severe conditions include: irritability, poor feeding, pulling in of the skin at the collar bone, between the ribs, or under the ribs, flaring of the nose, increasing effort to breathe and poor weight gain or weight loss, especially in infants.

Symptoms that should trigger emergency evaluation include: pauses in the breathing, color change of the skin (particularly if the lips, face or hands are turning blue), appearing lethargic or tired, or any other sudden change from a child’s normal breathing pattern. If any of these or other concerning signs develop, please seek immediate medical attention.

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