Special Supplement

Recurrent Respiratory Papillomatosis

Juvenile recurrent respiratory papillomatosis (also known as laryngeal papillomatosis), a condition with benign, wartlike tumors in the respiratory tract, may be associated with upper airway obstruction.1 It occurs at all ages, with about half of all cases appearing in the pediatric age group. It is characterized by the growth of papillomatous lesions, particularly in the larynx, that have a high rate of recurrence after excision. Laryngeal papillomas are the most frequent masses to arise within the airway in older children, and morbidity can be severe.2

Disease Mechanisms. It is now recognized that human papillomavirus (HPV) causes most cases of recurrent respiratory papillomatosis.3 The clinical presentation and course of the disease have not been definitively linked to a particular HPV type.

The replicating virus may cause overgrowth of squamous epithelial cells. The papillomata are multiple projections, each with a connective tissue stalk covered by well-differentiated stratified squamous epithelium. The tumors are benign but present obstructive problems because of their localization in the vocal cords or other sites. At presentation, papillomata usually are present on one or both vocal cords with the anterior commissure, supraglottis, or subglottis also commonly affected.1,3

Clinical Manifestations. Recurrent respiratory papillomatosis can occur at any age, with the youngest reported patient being 1 month of age.4 In the pediatric age group, about half of patients have symptoms during the first year of life, although clinical recognition of the disease often is delayed. Patients usually come to medical attention late, with some degree of airway obstruction, including stridor, together with hoarseness or a weak cry.3 Life-threatening upper airway obstruction may occur. Although the lesions usually are localized within the larynx, spread to other areas (pharynx, esophagus, trachea, and lung parenchyma) may occur and indicates a more pessimistic outlook.2,3

The most usual course of the disease is for the papilloma to continue to grow locally despite surgical removal and without significant spread. Over time, the majority of cases in children undergo spontaneous remission (analogous to skin warts). It traditionally was believed that the onset of puberty is associated with remission, although this view has been challenged.1

The condition is diagnosed by inspection of the larynx, either by indirect means such as fiberoptic laryngoscopy in an office or by more formal laryngoscopy and bronchoscopy when tissue biopsies can be taken.

Multiple endoscopies usually are required for further investigation and management, and flexible bronchoscopy is the method choice for surveillance. Although ultrasound examination of the airway correlates with laryngoscopic findings, it is seldom used in clinical practice.

Management. During the acute phase of management, the airway should be monitored and otolaryngology consulted for both diagnosis and therapeutic intervention. Recurrent respiratory papillomatosis is frustrating to treat because lesions often are recurrent and sometimes aggressive. The focus of management is to ensure a safe airway without causing irreversible long-term scarring, especially affecting the voice. Total removal of the disease is impossible in most cases because undeclared viral infection occurs in apparently normal adjacent areas and the degree of destruction necessary to clear the field would in most cases require too great a degree of tissue damage.

In particularly aggressive phases of the disease, total removal may require laryngoscopies with excision as often as twice a week. The frequency of the surgery is dictated entirely by how rapidly the papilloma regrows and is individualized for each patient. All patients can expect multiple endoscopies and surgical removal.

The most widely used surgical method for removing recurrent respiratory papilloma is use of the carbon dioxide laser, which acts as a very precise "knife," vaporizing the papilloma with minimal damage to the underlying larynx. However, it does not prevent regrowth any better than the older surgical methods, such as direct removal. In the hands of experienced endoscopists, there is a low to moderate incidence of laryngeal scarring. If possible, tracheotomy should be avoided because of the risk of seeding of the disease to the tracheotomy site.3

References

1. Asher MI. Infections of the upper respiratory tract. In: Taussig LM, Landau LI, eds. Pediatric Respiratory Medicine. St. Louis: Mosby; 1999:530-547.

2. Rencken I, Patton WL, Brasch RC. Airway obstruction in pediatric patients. Radiol Clin of North Am 1998;36:175-187.

3. Bauman NM, Smith RJH. Recurent respiratory papillomatosis. Pediatr Clin North Am 1996;43:1385-1401.

4. Quiney RE, Hall D, Croft CB. Laryngeal papillomatosis: Analysis of 113 patients. Clin Otolaryngol 1989;14:217-225.