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Special Feature
April 1998

Pathological Case of the Month

Arch Pediatr Adolesc Med. 1998;152(4):408. doi:

Diagnosis and Discussion: Recurrent Respiratory Papillomatosis

Figure 1. Multiple surgically removed papillomas.

Figure 2. Microscopic section. Squamous papilloma (original magnification ×40).

Figure 3. Squamous papilloma showing koilocytosis due to human papillomavirus (original magnification ×250).

Figure 4. A computed tomographic scan of the chest showing multicystic cavitary lesions bilaterally.

Papillomas are the most frequent benign neoplasms of the larynx in children.1 Papillomas may affect any age group, including adults. Most children present with papillomas before 5 years of age. Presentation before 2 years of age is associated with a recurrent and progressive disease. Papillomas affect 1500 to 2000 infants and children in the United States each year and are now known to be caused by the human papillomaviruses (HPV); HPV-6 and HPV-11 are the most commonly identified types.2-4 These are the same types found in greater than 90% of genital condylomas. It is believed that these lesions are acquired from an affected mother. While greater than 50% of children with laryngeal papillomas have a history of maternal condylomas, most women with condylomas do not have affected children. The reason is unknown. Cesarean section seems to be somewhat protective.5 Adult and childhood laryngeal papillomas were previously believed to have a different pathogenesis, but new research now points to a common cause.3,6

The most common presenting symptoms are hoarseness, stridor, and airway obstruction. Patients have been treated in the past for recurrent croup or asthma. Laryngoscopy, the investigation of choice, is indicated to rule out respiratory papillomatosis in any young child with symptoms of voice change, obstructive airway symptoms, or recurrent croup. While the larynx is the most commonly affected site, the lesions may spread to the lower airways in approximately 20% of cases, and in the rare case, to the lung's parenchyma. Increased risk of spread to the lower respiratory tract has been noted with placement of a tracheostomy, and therefore, such placement should be avoided for as long as possible. The clinical course varies from spontaneous remission to ruthless progression and the associated complications.7 In some, malignant degeneration to squamous cell carcinoma may occur. Once thought to uniformly regress during puberty, this has not been observed by some investigators.8

The treatment of respiratory papillomatosis is frustrating, as lesions are frequently recurrent and relentless. Surgical excision is the only definitive therapy. Most investigators use the carbon dioxide laser. Other therapies include interferon, isotretinoin, antibiotics, antiviral agents, hormones, steroids, podophyllin, and antimetabolites (fluorouracil and methotrexate). Interferon has proven to be the most helpful.

Prognosis is affected by age at onset. The earlier the onset, the greater the likelihood of lower airway involvement. However, progressive disease can even occur in adults. A spontaneous remission is not infrequent. The factors associated with remission are not well understood. Childhood papillomatosis may persist into adulthood. Squamous cell carcinoma may occur without previous irradiation in patients with a long history of recurrent respiratory papillomatosis.9

Accepted for publication December 18, 1996.

Corresponding author: Enid Gilbert-Barness, MD, Department of Pathology, Tampa General Hospital, PO Box 1289, Tampa, FL 33061.

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