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Juvenile Recurrent Respiratory Papillomatosis (JRRP) Overview and General Information

Click here for a printable PDF of this information.

JRRP Patient stats checkMass. Eye and Ear provides family-centered care to children with JRRP. It is one of several treatment centers in the country that uses KTP lasers and an Avastin (bevacizumab) protocol. Mass. Eye and Ear has established a team approach in which the physicians, nurses, child life specialists, social workers, speech therapists, parents, care givers, etc. work together for the care of the patient. Education is an integral part of the treatment plan in helping patients and family members come to terms with the disease.

What is JRRP and who is at risk?

 JRRP stands for Juvenile Recurrent Respiratory Papillomatosis. JRRP is a condition that causes the growth of viral papilloma (warts) on the surface of the respiratory tract. Papillomata may occur anywhere in the respiratory tract (nose, throat, or lungs) but mostly affect the larynx (voice box). The growths may vary in size and often grow very quickly. Papillomata in the larynx are benign (not cancerous) and can be managed with periodic surgical intervention. There is no cure for JRRP.

JRRP is caused by Human Papilloma Virus (HPV).  This virus is very common and there are more than 100 different strains. Many people have been infected with this virus, even if they do not have symptoms. Different strains can cause different types of illness. HPV is most familiarly associated with cutaneous warts on the hands or feet, genital warts, and cervical cancer. There are two strains of HPV that cause JRRP: 6 and 11, with 11 typically causing more severe disease.

Risk factors include being a firstborn child, children born to mothers younger than 20 years of age, and children born to mothers with active genital warts during pregnancy who deliver vaginally. Parents should check with obstetricians or midwives with questions. 

JRRP affects males and females equally, and the average age of diagnosis is 3.8 years of age. However, this does not exclude patients that are younger or older than 3.8 yrs. The incidence in the United States is estimated at 4.3 per 100,000 children and 1.8 per 100,000 adults. Age may be more indicative of prognosis than HPV type: children who develop the disease earlier in life often have worse prognoses.

In children older than 5 years of age who are diagnosed with respiratory papillomatosis, sexual abuse should be considered.

Diagnosis of JRRP

JRRP is a pediatric disease. Patients with JRRP are often initially diagnosed with one or more of the following signs and symptoms:

  • Stridor (noisy breathing)
  • Chronic cough
  • Failure to thrive
  • Shortness of breath
  • Snoring
  • Croup
  • Asthma
  • Difficulty speaking (Dysphonia)
  • Aphonia (loss of voice)
  • Hoarseness
  • Laryngomalacia

It is critically important to recognize the symptoms of JRRP before potential life threatening obstruction develops from the papillomata.

JRRP is diagnosed first by symptoms, and then either by viewing the larynx in the office with a scope or by taking the child to the operating room to inspect the airway while the child is asleep under anesthesia. The only way to absolutely confirm JRRP is by a biopsy.

Is JRRP Contagious Peer to Peer?

No.  Children cannot catch JRRP (or HPV) by playing with each other or by sharing utensils. Siblings cannot be infected through regular household contact.

Treatment

The goal in treatment is to clear the airway of papillomata and to prevent or reduce the occurrence of new papillomata. Surgical interventions include a debulking procedure to reduce papillomata, using a potassium titanyl phosphate (HTP) laser, carbon dioxide (C02) laser, microdebrider, or pulse dye laser (PDL).

A child's physician may also prescribe an antivirial medication, especially for children who require more than four surgical treatments a year.

Please click here for additional information on treatment options

Prevention

There are two vaccines currently approved and available in the US to prevent HPV, the virus that causes JRRP. Gardasil is a quadrivalent vaccine providing protection against HPV types 6, 11, 16 and 18. Types 6 and 11 are most associated with genital warts and 16 and 18 with cervical cancer. Cervarix is a bivalent vaccine containing only types 16 and 18. Although Gardasil does contain types 6 and 11, the ones associated with JRRP, the vaccine is approved for people 9-26 years of age. The recommendation is for both boys and girls to get vaccinated with the 3 required doses at age 11 or 12. Infants and young children could not be protected directly, but may be if their mothers were vaccinated as children/young adults prior to HPV infection. 

Photos

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Juvenile Recurrent Respiratory Papillomatosis: This photograph shows a child's airway and vocal folds with marked ingrowth of JRRP.
 

 

 

 

Acknowledgements:
Many thanks to Dr. Vandana Madhavan of the Pediatric Infectious Disease division of the Massachusetts General Hospital for Children for her critical read and input of this section regarding the infectious nature of JRRP.  Also, please see RRP Foundation for more information.