Pediatric Recurrent Respiratory Papillomatosis: Fighting the Battle of a Rare But Serious Disease

The group also plans to look for genetic factors that influence the aggressiveness of the disease course. If you are one of these people who had one or two operations and then it went into remission, who would care? Dr. Buchinsky said. That is not nice, but it is not a big deal. On the other hand, if you are one of these people who undergoes 100 operations that is a huge deal. So we also want to look at whether there is any genetic influence over the type of course that one runs.

In the long run, Dr. Buchinsky hopes that identifying the susceptibility genes will help reveal the biology that underlies RRP, and lead to improved treatments. But that won’t happen quickly and in the meantime surgery remains the mainstay of therapy.

The Standard of Care-Surgery

Until recently most otolaryngologists used the carbon dioxide laser to remove papillomas from the larynx of children. A recent survey of American Society of Pediatric Otolaryngology (ASPO) members found that more than half are now using the laryngeal microdebrider as their surgical tool of choice.

It is as effective as laser for removing papillomas but safer because you don’t have any collateral thermal damage to the tissues nearby, said Craig S. Derkay, MD, Professor of Otolaryngology and Pediatrics at Eastern Virginia Medical School in Norfolk and Chair of the RRP Task Force, which is sponsored by ASPO. Additionally, the use of the microdebrider does not require specialized nursing, as do laser-based procedures, but it still allows for a delicate dissection of the papillomas.

Recently some physicians have started to use the 585-nm pulsed dye laser for the treatment of adult RRP patients. This laser has the advantage that it can be used with local anesthetic in the physician’s office, which saves the patient another trip to the operating room. There may be a role for the laser in the treatment of older children in the future, but it is not suitable for use in younger children, said Dr. Wiatrak.

Adjuvant Therapy for Severe Cases

For patients who have aggressive disease, adjuvant medical therapies may help increase the time between surgeries. The two most common adjuvant therapies used, according to the survey of ASPO members, are interferon and cidofovir. However, only interferon has been tested in randomized placebo-controlled trials.

In two randomized controlled trials, both of which were reported in 1988, systemic interferon therapy significantly reduced the severity of the disease, compared with controls. Following discontinuation of the drug, disease severity rapidly returned to baseline levels. As expected with interferon therapy, side effects included nausea, fatigue, headaches, and intermittent fever.