Juvenile Onset Recurrent Respiratory Papillomatosis

A Rare Side-Effect of Genital Warts During Pregnancy

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Lungs made of flowers. JW LTD/Stone/Getty Images

Juvenile recurrent respiratory papillomatosis (JO-RRP) is a rare type of HPV infection. With JO-RRP, warts (benign tumors) grow in a child's airway. Similar to adult onset RRP (AO-RRP), RRP is defined as juvenile onset if it first appears before the age of 12. That is usually considered the lower limit for consensual sexual activity. However, most cases of JO-RRP are diagnosed before an infected child is five years old.

Risk Factors for JO-RRP

The major risk factor for a child to become infected with JO-RRP is the child's mother having genital warts during pregnancy, or a history of genital warts, and choosing to have a vaginal delivery. JO-RRP is somewhat more common in first children than in children from later pregnancies. It is also more common in children of teenage mothers.

It is thought that most children with JO-RRP are exposed to HPV as they pass through the birth canal. Not all mothers of children with JO-RRP have a history of genital warts or other HPV infection. However, it's likely that many women who think they are uninfected may simply have been asymptomatic. However, there is also the possibility of non-perinatal exposure to HPV leading to JO-RRP. This is a particular risk for children with late onset disease.

It is relatively rare to get a first diagnosis of JO-RRP in children over five years of age. Therefore, a late diagnosis is sometimes considered to be a warning sign of potential child sexual abuse.

Symptoms of JO-RRP

The most common early symptom of JO-RRP is hoarseness or other difficulty speaking. If not managed properly, JO-RRP can sometimes also lead to more serious consequences. These include difficulty breathing or a blocked airway.

Less than 5 percent of JO-RRP cases eventually become cancerous.

Cancer may be more likely when the JO-RRP is caused by one of the oncogenic HPV variants instead of the (far more common) HPV-6 or HPV-11.

Treatment of JO-RRP

A variety of surgical techniques can be used to debulk the airway of children with JO-RRP. This process does exactly what it sounds like - it reduces the size, or bulk, of tumors in the airway. Unfortunately, while surgery is reasonably effective in some children, in others the tumors regrow so fast that the procedure needs to be repeated as often as every two weeks. Why tumors regrow quickly in some children and not in others is not yet well understood. However, the number of surgeries necessary to treat JO-RRP can vary from two to over 100

A number of medical treatments are also under investigation for JO-RRP. The could be used either alone or in combination with surgical debulking. However, none of these treatments have yet been shown to be effective in repeated, high quality randomized controlled trials.

Can JO-RRP Be Prevented?

For women who have symptomatic genital warts during pregnancy, some scientists have proposed that Cesarean sections could be useful in preventing JO-RRP.

Such a method is theoretically cost-effective. However, there is no conclusive evidence that C-sections are a necessary or medically effective precaution.

HPV vaccination of children is also unlikely to be useful in preventing JO-RRP, even if such a use of the vaccine were to be approved. Most exposures to the viruses that cause JO-RRP happen before or during birth, before there is a chance to vaccinate. However, Gardasil vaccination of young women during their teenage years could reduce their risk of giving birth to a child with JO-RRP in the future.

Note: The absolute risk of JO-RRP is extremely low on a population basis. It is considered to be an orphan disease.


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