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Juvenile Recurrent Respiratory Papillomatosis

The second most frequent cause of childhood hoarseness, Juvenile Recurrent Respiratory Papillomatosis (JRRP) is estimated to affect over 4 out of every 100,000 children in the US. It is the most common benign neoplasm (an abnormal growth) of the larynx, which is near or around the vocal cords, among children. This information is intended to give you a brief overview of the condition. Please follow up with the Mass. Eye and Ear's Pedi Airway team for questions. 

Causes

The main causes of JRRP are human papillomavirus (HPV) types 6 and 11. Children infected with HPV 11 appear to be at a higher risk of developing obstructive airway disease. HPV 31 and 33 exhibit intermediate malignant potential. There are 2 forms of RRP, with the most aggressive form occurring in children.  The presentation in the first 28 days of life poses higher right for tracheostomy and attendant morbidity and mortality. Diagnosis before versus after 3 years of age is associated with 3.6 times greater likelihood of needing more than four surgical procedures per year.

Presentation

The first and most predominant site of the papilloma in the vocal fold making hoarseness the principal presenting symptom. Stridor (noisey breathing) is often the second clinical symptom to develop.

 

Some of the less common presenting symptoms are chromic cough, recurrent pneumonia, failure to thrive, dyspnea, dysphagia, or acute respiratory distress, especially in infants with upper respiratory tract infection.

At times, JRRP can be misdiagnosed as asthma, croup, allergies, vocal nodules, or bronchitis.

Progression

Tracking of the JRRP is a challenge.  The history of the disease shows that is it highly unpredictable. It has a spontaneous remission rate and can only be stabilized when the patient undergoes periodic surgical treatments.  Those treatments must be aggressive and require surgical treatments every fews days to weeks and suggest an additional consideration of additional supportive medical therapy.

Diagnosis

The only way to properly diagnosis JRRP is through a biopsy. Once a biopsy is completed and the diagnosis made, a treatment plan can be made.  The goals for treatment are to improve the airway and voice and serial debulking (the removal of portion of a growth which can not be fully removed). Unfortunately, JRRP will reoccur but in order the try to control the disease, parents are encouraged to keep good records on the child to help understand how each child is doing in the longterm.

Treatment

There is no treatment that will cure JRRP as of yet. However, there are things to do to control the condition.
The treatment options include:

Cold Knife Excision
CO2 Laser
Powered Microdebrider
Angiolytic Lasers
Adjuvant Therapy
Combination of the above

Please click here for additional information on treatment options.