Juvenile recurrent respiratory papillomatosis (JRRP) is a condition that causes the growth of papilloma (warts) in the airway. These warts may occur anywhere, including the throat, windpipe (trachea), or lungs, but mostly affect the voice box (larynx). They are benign (not cancerous), but can vary in size and can grow very quickly. There currently is not a cure, but the condition can be managed with periodic surgical intervention.
Patients are often initially diagnosed with one or more of the following signs and symptoms:
- Abnormal cry
- Loss of voice
- Difficulty speaking
- Noisy breathing
- Chronic cough
- Shortness of breath
It is critically important to recognize the symptoms of JRRP before potential life threatening obstruction develops from the papilloma.
JRRP is frequently diagnosed in children. When JRRP is suspected by symptoms, it’s often diagnosed either by viewing the voice box with a scope or by taking the child to the operating room to inspect the airway. The only way to absolutely confirm JRRP is with a biopsy.
The goal of treatment is to clear the airway of the warts, which may be done with a special laser and/or suction device. Sometimes, special medications are used, especially for children who require more than four surgical treatments a year.
While the primary treatment is surgery, a speech-language pathologist can help by providing support and education about the effects of JRRP and surgeries on the voice. In addition, they can provide voice therapy to help manage these changes.
There is currently only one vaccine available in the US to prevent HPV, the virus that causes JRRP. Gardasil9 is a vaccine that provides protection against the four HPV types included in the first Gardasil (6, 11, 16, 18) as well as five additional types (31, 33, 45, 52, 58). Types 6 and 11 are most associated with genital warts, as well as JRRP, and the others, especially16 and 18, are associated with cervical cancer. Cervarix, bivalent vaccine containing only types 16 and 18, is no longer available in the United States and would not have provided any protection against the HPV types causing JRRP.
Gardasil9 is approved for people 9 to 26 years of age. It's safe, typically well-tolerated, and causes a strong immune response providing long-term protection against HPV. The current recommendation is for both boys and girls to receive two doses of the vaccine at least six months apart starting at age 11. Adolescents and young adults 15 and older need to receive the previous standard of three required doses. Infants and young children cannot be protected directly, but may be if their mothers were vaccinated as children/young adults prior to HPV infection.
JRRP Overview and General Information for Families, Schools, and Health Care Providers Handout
Current Ongoing Clinical Research
We are actively recruiting children with symptomatic JRRP for a study that aims to identify drug sensitivity and gain a better sense of a child’s immune state and how this may affect disease progression. For more information, please email firstname.lastname@example.org.
Learn more about other ongoing research initiatives involving JRRP.
Efficacy of treating children with anterior commissure and true vocal fold respiratory papilloma with the 585-nm pulsed-dye laser
Use of 532-nm pulsed potassium titanyl phosphate laser and adjuvant intralesional bevacizumab for aggressive respiratory papillomatosis in children
PD-L1 expression and CD8þ infiltration shows heterogeneity in juvenile recurrent respiratory papillomatosis
Tracheomalacia is a congenital or acquired condition in which the windpipe (trachea) is floppy, often leading to respiratory problems.
- Expiratory stridor (high-pitched noise while breathing out) that is often worse with crying, laying on back, or feeding
- Cyanotic episodes (turning blue)
- Retractions (sucking in of chest/neck with each breath)
- Apneic episodes (stopping breathing)
Tracheomalacia is usually diagnosed by an endoscopy, a procedure in which a small scope is passed into the airway while the patient is asleep.
Most patients with tracheomalacia are asymptomatic and do not require treatment. Some may only become symptomatic during upper and lower respiratory tract infections. Some children with tracheomalacia have greater frequency and severity of respiratory infections. Bronchodilators, commonly used to treat children with asthma, may be used.
For trouble breathing, sometimes adjustments in positioning can help or your physician may suggest the use of devices to keep the airway open, especially during sleep, such as CPAP and BIPAP, which are machines that provide breathing assistance using pressure.
Surgical management is rarely needed, and only when symptoms are not controlled by medical management and cause life-threatening events.
Every child has a breathing tube starting at the nose and mouth, which extends into the lungs. He or she also has a feeding tube just behind the breathing tube that brings food and liquid to the stomach. If there is a connection at the very top of these two tubes, we call this a laryngeal cleft.
A laryngeal cleft can cause a wide range of symptoms, including: cough, wheezing, recurrent lung infections, trouble swallowing, poor feeding, and failure to gain weight.
A modified barium swallow study (MBS) may show certain swallowing problems where liquid moves toward or into the airway (penetration/aspiration), which is concerning for a laryngeal cleft. The only way to diagnose a laryngeal cleft is through an endoscopy, a procedure in which a small telescope is passed into the airway while the patient is asleep.
Laryngeal clefts begin at the very top of the two tubes. Imagine a zipper unzipping—if the zipper unzips only to the level of the vocal cords, then it is labeled a type 1 cleft and if the zipper unzips all the way down into the chest it is a type 4 cleft.
Management of type 1 cleft is usually conservative with anti-reflux medications and feeding modifications. There are also options for a temporary surgical procedure with injection of a slowly dissolving material that may help the child overcome their symptoms until their swallowing improves. Otherwise, a surgical procedure using the microscope or endoscope can be performed to close the cleft. This same procedure can be used for type 2 clefts.
All type 3 and 4 clefts require surgical repair and this is generally done through an open approach.
Current Ongoing Clinical Research
We are actively recruiting children with laryngeal clefts to identify whether younger children would benefit from an early cleft injection to allow them early transition to thin liquids and that might lower the frequency whereby laryngeal clefts need definitive repair later on. For more information, please email email@example.com.
Radiation exposure from videofluoroscopic swallow studies in children with a type 1 laryngeal cleft and pharyngeal dysphagia: A retrospective review
Type 1 laryngeal cleft: A multidimensional management algorithm
Type 1 laryngeal cleft: Establishing a functional diagnostic and management algorithm
Management of Type I and Type II laryngeal clefts: Controversies and evidence
Modified best-practice algorithm to reduce the number of postoperative videofluoroscopic swallow studies in patients with type 1 laryngeal cleft repair
Short-term swallowing outcomes following type 1 laryngeal cleft injection
Hemangiomas are the most common benign tumor of infancy, often appearing as a red birthmark. In some cases, they can occur in the airway, usually right under the vocal cords. When this happens, it can cause breathing difficulties that can be mild or severe (the narrower the airway, the more difficulty the child will experience when breathing).
The best way to diagnose a hemangioma is through an endoscopy, a procedure in which a small telescope is passed into the airway while the patient is asleep.
Most of the time, hemangiomas do not require treatment. However, in some cases, they can cause concerning symptoms, including obstructing a child's airway. When this happens, medical management can include medications, such as steroids or beta-blocker (propanalol).
In severe cases or cases that do not respond to medications, surgery is considered. Depending on the case, tracheostomy or open airway excision may be recommended.
Initial experience using propranolol as the sole treatment for infantile airway hemangiomas
Open excision of subglottic hemangioma
Subglottic stenosis is a condition in which a child’s airway is obstructed due to congenital (present at birth) narrowing of the airway or from scarring just below the vocal cords. This narrowing can also occur lower down in the airway, which is known as tracheal stenosis. The scar typically develops early in childhood, often because the child was born premature and needed a breathing tube as their lungs developed.
Airway stenosis causes breathing difficulties for children. These difficulties can be mild or severe—the narrower the airway, the more difficulty your child might experience when breathing.
The best way to diagnose a stenosis is through an endoscopy, a procedure in which a small telescope is passed into the airway while the patient is asleep. In some cases, an imaging study, such as a CT scan, may be performed if the stenosis is so narrow that a telescope cannot be passed through it.
Significant stenosis may require placement of a tracheostomy tube to bypass the narrow part of the airway. The tracheostomy is generally placed to allow the child to breathe, feed, and grow until they become healthy and stable enough for further treatment. Pediatric airway reconstruction in the form of either laryngotracheal reconstruction or cricotracheal resection is designed to rebuild the airways and to allow the tracheostomy to be removed. For a child without major heart, lung, or other medical issues, airway reconstruction has an approximately 90 to 95 percent success rate for tracheostomy removal.
Current Ongoing Clinical Research
We are actively recruiting children with lower subglottal collapse and upper suprastomal tracheal collapse to identify the role of less invasive bioresporbable plate placement. For more information, please email firstname.lastname@example.org.
Surgery for pediatric subglottic stenosis: Disease-specific outcomes
Management of severe suprastomal collapse with bioabsorbable microplates
Comparison of hybrid laryngotracheal reconstruction to traditional single- and double-stage laryngotracheal reconstruction
Pediatric partial cricotracheal resection: A new technique for the posterior cricoid anastomosis
Subglottic stenosis complicated by allergic esophagitis
Congenital high airway obstruction syndrome and airway reconstruction
Novel method for laryngotracheal reconstruction: Combining single- and double-stage techniques