Summary about Disease
Juvenile Laryngeal Papillomatosis (JLP), also known as recurrent respiratory papillomatosis (RRP), is a rare condition characterized by the growth of benign (non-cancerous) tumors called papillomas in the larynx (voice box) and other areas of the respiratory tract. These papillomas are caused by the human papillomavirus (HPV). JLP typically affects children, although it can also occur in adults. The recurrent nature of the papillomas often necessitates multiple surgeries to remove them and prevent airway obstruction.
Symptoms
Hoarseness
Weak cry (in infants)
Chronic cough
Stridor (noisy breathing)
Difficulty breathing (dyspnea)
Voice changes
Airway obstruction (in severe cases)
Difficulty swallowing
Gagging or choking
Causes
JLP is caused by infection with the human papillomavirus (HPV), most commonly HPV types 6 and 11. Children typically acquire the virus from their mothers during childbirth if the mother has genital warts (caused by the same HPV types). However, other modes of transmission are possible but less common.
Medicine Used
There is no cure for JLP, and treatment focuses on managing the symptoms and removing the papillomas. While not curative, medications may be used adjunctively to surgery:
Cidofovir: An antiviral medication that can be injected into the papillomas to slow their growth.
Bevacizumab: An angiogenesis inhibitor that can be used to reduce the blood supply to the papillomas, slowing their growth.
Interferon: An immunomodulatory agent that has been used in some cases, but its effectiveness is variable.
Mitomycin C: topical application during surgery in some cases to prevent recurrence. The main treatment is surgical removal of the papillomas. Techniques include:
Microlaryngoscopy: Using a microscope to view and remove the papillomas with surgical instruments.
Laser surgery: Using a laser to vaporize the papillomas.
Microdebrider: Instrument that suctions and shaves the papillomas.
Is Communicable
Yes, JLP is caused by HPV, which is a communicable virus. The primary route of transmission in children is from mother to child during vaginal delivery. Transmission can happen but is rare outside of this scenario.
Precautions
Cesarean delivery: In pregnant women with genital warts caused by HPV, a cesarean delivery may be considered to reduce the risk of transmission to the baby, although this is controversial.
HPV vaccination: Vaccination against HPV is recommended for adolescents and young adults to prevent HPV infection and related diseases, including genital warts. While it doesn't directly prevent JLP in those already infected, it can help prevent the spread of HPV.
Avoidance of risk factors: Limiting exposure to HPV by practicing safe sexual behaviors can reduce the risk of HPV infection.
How long does an outbreak last?
JLP is a chronic, recurring condition. The papillomas will continue to grow back until either surgical removed or in rare cases a patient may outgrow the infection. There is no "outbreak" that resolves on its own. Papillomas recur at varying rates, requiring repeated interventions over months, years, or even a lifetime. The frequency of surgery varies widely among patients.
How is it diagnosed?
Laryngoscopy: A visual examination of the larynx using a flexible or rigid scope. This allows the doctor to see the papillomas directly.
Biopsy: A small sample of tissue is taken from the papilloma and examined under a microscope to confirm the diagnosis and rule out other conditions.
HPV testing: The tissue sample can also be tested for the presence of HPV and to determine the specific HPV type.
Timeline of Symptoms
Initial infection: Infection with HPV typically occurs during childbirth.
Latency period: There may be a period of time (weeks to months) between initial infection and the appearance of symptoms.
Onset of symptoms: Hoarseness is often the first symptom, followed by other voice changes, breathing difficulties, and stridor.
Recurrence: After surgical removal, the papillomas often recur, leading to a cyclical pattern of symptom improvement followed by worsening as the papillomas regrow.
Important Considerations
Airway management: JLP can cause life-threatening airway obstruction, so prompt diagnosis and treatment are essential.
Multiple surgeries: Due to the recurrent nature of the papillomas, patients often require multiple surgeries throughout their lives.
Psychosocial impact: JLP can have a significant impact on a child's voice, breathing, and overall quality of life. Psychological support may be helpful.
Rare cases of malignant transformation: In rare cases, the papillomas can become cancerous (squamous cell carcinoma). Regular monitoring and follow-up are important.
Multidisciplinary approach: Management of JLP requires a multidisciplinary team, including otolaryngologists (ENT doctors), pediatricians, pulmonologists, and speech therapists.