Lethal asphyxiating juvenile laryngeal papillomatosis. A case report with human papillomavirus in situ hybridization analysis

Chronic, progressive, and recurrent laryngeal papillomatosis is an uncommon problem that primarily occurs in children and has been shown (in the pediatric age group) to be caused by human papillomavirus (HPV) types 6 and/or 11. Juvenile laryngeal papillomatosis is hallmarked by recurrences, necessitating repeated surgical procedures to maintain airway patency, and is rarely encountered cause of sudden and unexpected death. This case describes a 22-month-old girl who had been treated for laryngeal and pharyngeal papillomata that were diagnosed 3 months prior to her death, and who died in her sleep the night before she was to be again laryngoscoped and reevaluated. The autopsy disclosed exuberant, complex exophytic papillomata that essentially obliterated the laryngeal lumen. In situ hybridization analysis was performed on paraffin-embedded tissue, which revealed HPV type 6/11 positivity. This is the first time that in situ hybridization studies have been performed on autopsy tissues from someone who died suddenly and unexpectedly from upper-airway obstructive papillomatosis. The pathology and clinical characteristics of juvenile laryngeal papillomatosis are also presented and discussed.