The prevalence in the general population is estimated between 1 and 4 cases / 100,000.

Recurrent tumoral lesions of viral origin (especially herpes type 6 and 11 papillomavirus) in the larynx. The average diagnosis is made at about 4 years of age but some cases already appear in infants: the peaks of diagnosis are between 2 and 5 years and between 20 and 30 years of age, with a male predominance. Mothers of affected children show histological and cytological signs of genital infection by the same virus. It is expected that the generalization of vaccination of girls against the papillomavirus around 11-12 years of age will greatly decrease the incidence of this devastating pathology and its oncologic complications (notably cervical cancers).

The viral DNA causes epithelial proliferation in the larynx, resulting in progressive dysphonia, stridor and/or progressive respiratory obstruction (laryngeal dyspnea, chronic cough, sometimes obstructive sleep apnea or dysphagia). Many more or less aggressive drug treatments have been used (interferon, acyclovir, ribavirine, cidofovir, anti-Cox2, retinoids, anti-reflux drugs, zinc, indole-3-carbinol, etc.) but often on the single basis of individual clinical cases, which does not allow clear recommendations of a general use. Different types of endoscopic treatments need to be repeated: thermal cauterization, cryosurgery, ablation with forceps, but the most commonly adopted method is debridement under suspended microlaryngoscopy, which is preferred to CO2 laser treatment. Derkay's classification is used to describe the extension of laryngeal involvement.

                Derkay's classification

The procedure poses significant technical problems due to laryngeal and and/or tracheal obstruction by the tumor: there is a major risk of ventilation and/or intubation difficulties . Most ENT surgeons prefer to proceed on an unintubated child kept in spontaneous ventilation by using either:

-        a supraglottic tube (or a nasopharyngeal catheter)

-        or a transglottic 7 French 2-way central venous catheter, enabling the measurement of teleexpiratory CO2 through one of the two channels

Jet-ventilation presents a (undocumented) risk of tissular remnants and potentially infectious viral particles being spread into the more distal respiratory tree and outside in the operating room. It is why it is no longer recommended.

Interventions are repeated according to the rate of the epithelial proliferation and the accompanying respiratory discomfort. Papillomatosis is considered to be very aggressive if the child requires more than 3 to 4 procedures per year. Bevacizumab, an inhibitor of the vascular endothelial factor, is used in addition to surgery in cases responding poorly to surgery or in case of lung disease. Sometimes a tracheostomy is necessary. The distal bronchial or parenchymatous spread of the lesions represents a severe form of the disease which can be life-threatening.

Anesthetic implications:

repeated endoscopic procedures (50 % of cases require more than 10 procedures).

Major risk of difficulties:

-        ventilation: maintain spontaneous ventilation, risk of obstruction of the upper airways (decreased tonus of the dilating muscles of the pharynx), risk of obstruction of the endotracheal tube or trachea if pieces of the tumor are detached during intubation, risk of 'reventilation' collapse during an procedure on a child admitted with respiratory failure

-        intubation: keep different types of tubes ready as well as a bronchoscope

-        and laryngospasm during induction, maintenance and recovery.

References :

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Recurrent respiratory papillomatosis: a review.
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Human papilloma virus (HPV) infection in children and adolescents.
Eur J Pediatr 2009; 168:267-73.

-        Gerein V, Schmandt S, Babkina N, Barysik N, Coerdt W, Pfister H.
Human papilloma virus (HPV)-associated gynecological alteration in mothers of children with recurrent respiratory papillomatosis during long-term observation.
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-        Gallagher TQ, Derkay CS.
Pharmacotherapy of recurrent respiratory papillomatosis: an expert opinion.
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Laser-assisted management of congenital and acquired pediatric airway disorders: case reports and review of the literature.
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Juvenile laryngeal papillomatosis: scary anaesthetic !
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Laryngeal papillomatosis with airway obstruction in an infant.
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-        Li S-Q, Chen J-L, Fu H-B, Chen L-H.
Airway management in pediatric patients undergoing suspension larygoscopc surgery for severe laryngeal obstruction caused by papillomatosis.
Pediatr Anesth 2010; 20: 1084-91.

-        Zhu Z-R, Hu Z-Y, Jiang Y-L, Xu L-L, McQuillan PM.
The use of a double-lumen central venous catheter for airway management in pediatric patients undergoing laryngeal papillomatosis surgery.
Pediatr Anesth 2014; 24: 157-63

-        Kelly-Ugarte LR, Munoz-San Julian C.
Laryngeal papillomatosis.
Anesthesiology 2014; 121: 1092

-        Aurégan C, Thierry B, Blanchard M, Chéron G.
Papillomatose laryngée récurrente compliquée d’insuffisance respiratoire décompensée chez deux enfants.
Arch Pédiatr 2015 ; 22 : 1171-5

-        Lawlor C, Balakrishnan K, Bottero S, Boudewyns A, Campisi P et al.
International Pediatric Otolaryngology Group (IPOG): juvenile-onset recurrent respiratory papillomatosis consensus recommendations.
Int J Pediatr Otorhinolaryngol 2020; 128: in press

Updated: December 2019