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Paediatric clinic - recurrent respiratory papillomatosis

Paediatric ENT specialists Mr Adam Donne and Mr Ravi Sharma advise on this uncommon but serious cause of airway obstruction

 

A two-year-old boy presents to the GP with breathing difficulty. His Mum reports that he has always had a weak and hoarse voice. He does not have any features of infective airway pathology, for example croup. On examination he has significant difficulty breathing with tracheal tug, sternal recession and subcostal recession. His oxygen saturation is around 90%. The GP refers him to hospital where nebulised adrenaline is administered, but does not give noticeable relief. He has an emergency airway endoscopy to diagnose the airway obstruction.

The endoscopic image of the larynx indicates the almost complete obstruction of the airway by a papillomatous lesion arising from the right vocal cord which moves in and out of the laryngeal inlet with respiration. There are additional lesions on the left vocal cord causing a thickening of the cord. The papillomas are removed using a microdebrider to open the airway. The parents collect their son and explain it is the first time they have “heard him cry.”

The problem

Recurrent respiratory papillomatosis is a rare condition in children – with 4/100,000 cases per year.1 It is caused by HPV types 6 and 11. The HPV typing in this case was reported to be HPV 11. It is a highly problematic condition since the only effective treatment is the repeated surgical removal of the lesions under general anaesthetic. Children may require monthly procedures until the condition is more stable.

Features

Papillomas most commonly affect the vocal cords and presents as hoarseness. Stridor is also a common feature but can be variable given the changing shape of papillomas on the vocal cords. If ignored, the papillomas grow slowly and obstruct the airway. Death due to obstruction has been reported in children. The unusual feature of this case was that the parents felt the voice had been affected since birth - this age of symptom occurrence is very rare indeed.

Examination

  • Hoarseness is an important symptom in children and adults and should be considered to be significant, especially if there are no additional features of acute infection.
  • Stridor only occurs when greater than 50% of the airway is obstructed - because the airflow changes from laminar to turbulent and turbulence generates noise.
  • One of the earliest signs of increased work of breathing because of obstruction in children is tracheal tug, sternal and subcostal recession follow. These signs can occur readily with croup but likewise respond quickly to steroids and nebulised adrenalin.

Treatment

The best current treatment is ‘cold-steel’ removal of the papillomas to keep the airway patent. Lasers have previously been used but studies demonstrate worse voice outcomes long-term. Unfortunately this requires frequent general anaesthetics. There are obvious practical implications for the families of affected children and a significant cost burden for this condition despite the low prevalence.2 There are numerous adjuvant therapies but none of these are curative or predictable. Cidofovir has been used and appeared to show some clinical improvement, but these studies were not controlled and cidofovir has now effectively stopped being used for this condition. Alpha-interferon has been used and around two thirds of patients show an improvement, but the side-effects are significant.

Recently, the national HPV prophylactic vaccination program has changed from the bivalent vaccine (that did not cover HPV 6 & 11) to the quadrivalent vaccine (Gardasil). Gardasil is designed to generate antibodies against HPV 6 and 11 in addition to HPV16 & 18.  Gardasil is a therapeutic vaccine and the hope is that it may confer protection against the virus that causes RRP.

 

Mr Adam Donne and Mr Ravi Sharma are consultant paediatric ENT surgeons at Alder Hey, Liverpool

Alder Hey is one of Europe’s biggest and busiest children’s hospitals providing care for over 275,000 patients each year.  Alder Hey has a broad range of hospital and community services for direct referral from primary care.  It is the designated national centre for head and face surgery and a Centre of Excellence for children with cancer, spinal and brain disease. Alder Hey has been chosen to be a national centre for heart surgery, a respiratory ECMO surgery centre and one of just four specialist centres to provide surgery for drug-resistant epilepsy. For more information, go to www.alderhey.nhs.uk.

 

References

1 Derkay CS. Task force on recurrent respiratory papillomas. A preliminary report. Arch Otolaryngol Head Neck Surg 1995;121:1386–91

2 Donne AJ and Clarke R. Recurrent respiratory papillomatosis: an uncommon but potentially devastating effect of human papillomavirus in children. International Journal of STD & AIDS 2010;21:381–385

 

 

 

 

 

 

 

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