Papillomatosis

Derek Pepiak, MD, FCCP, FAAP

Introduction/Etiology/Epidemiology

•Papillomatosis is a rare disease characterized by the growth of tumors in the respiratory tract that are caused by human papillomavirus (HPV).

•It is also referred to as recurrent respiratory papillomatosis.

•It may occur in children or adults.

•When diagnosed in children, the disease is referred to as juvenile-onset recurrent respiratory papillomatosis (JORRP).

•JORRP is the most common benign laryngeal tumor in children.

•Most children receive diagnoses by age 5, with a mean age of 3.8 years at diagnosis.

•A younger age at diagnosis is frequently associated with a more aggressive disease process.

•Male and female patients are affected equally.

•Laryngeal papillomatosis is most commonly caused by HPV-6 and HPV-11, although HPV-16 and HPV-18 have been observed, as well.

•Disease caused by HPV-11 is more severe.

•Incidence is estimated at 4.3 per 100,000 children in the United States.

Pathophysiology

•HPV infection is acquired during passage through the birth canal of an infected mother.

•Risk factors

—Firstborn child

—Vaginal delivery

—Infants born to mothers <20 years of age

•More than 95% of papillomas occur in the larynx, although they may be found anywhere along the respiratory tract.

•Consider a potential history of sexual abuse in children >5 years of age who develop JORRP.

Clinical Features

•History

—Hoarseness, the most common symptom

—Voice changes (dysphonia or aphonia)

—Weak cry

—Choking episodes

—Chronic cough

•Physical examination

—Often nonspecific findings