Laryngeal papillomatosis is infection of the airway with human papilloma virus (usually strains 6 and 11), leading to papillomas on the vocal cords. The juvenile form is thought to be caused by vertical transmission of human papilloma virus at birth. It has a peak incidence between 2 and 4 years, but has been diagnosed in neonates. Most juvenile cases are diagnosed before age 5 years. Risk factors include a vaginal birth, a young or primiparous mother, and a mother with genital warts during pregnancy.

The typical course is one of frequent relapses, requiring multiple surgeries over many years, usually every few months but for rapidly growing papillomas as often as every 1 to 2 weeks. Ultimately, spontaneous remission is common, but spread into the tracheobronchial tree, causing cystic lung lesions and even malignant transformation, is possible. Laryngeal papillomas have caused fatal airway obstruction even after diagnosis. JLP is a relatively rare disease, affecting 3 to 4/100,000 children, but the burden of disease for affected children is huge, with some requiring >40 surgeries during their lives. Stridor in infants and toddlers is common, is most often acute and in such cases is usually related to infection (e.g., croup) or aspiration. Chronic stridor has an extensive differential, including laryngomalacia, tracheoesophageal fistula, vocal cord paralysis, subglottic stenosis, hemangioma of the airway, and extrinsic compression from a tumor or vascular anomaly (“rings and slings”). The clinical clues in laryngeal papillomatosis are first, that the stridor is progressive, whereas that of laryngomalacia, the most common cause of chronic stridor in infants, should slowly improve; and second, the changes in the voice quality, which suggest insolvent of the
vocal cords. Children with fixed or progressive laryngeal obstructions often present with recurrent croup, because they are more prone to significant airway obstruction during viral illnesses; therefore, a child with hoarseness plus recurrent croup or episodes of stridor should always prompt a referral for laryngoscopy. Evaluation in a child with chronic or recurrent stridor should include plain films of the neck to visualize the upper airway, and an esophagram, which may offer clues to extrinsic compression (e.g., anomalous vasculature) or tracheoesophageal fistula.