Recurrent respiratory papillomatosis (RRP), which is caused by human papillomavirus types 6 and 11, is the most common benign neoplasm of the larynx among children and the second most frequent cause of childhood hoarseness. After changes in voice, stridor is the second most common symptom, first inspiratory and then biphasic. Less common presenting symptoms include chronic cough, recurrent pneumonia, failure to thrive, dyspnea, dysphagia, or acute respiratory distress, especially in infants with an upper respiratory tract infection. Differential diagnoses include asthma, croup, allergies, vocal nodules, or bronchitis. Reports estimate the incidence of RRP in the United States at 4.3 per 100,000 children and 1.8 per 100,000 adults. Infection in children has been associated with vertical transmission during vaginal delivery from an infected mother. Younger age at diagnosis is associated with more aggressive disease and the need for more frequent surgical procedures to decrease the airway burden. When surgical therapy is needed more frequently than four times in 12 months or there is evidence of RRP outside the larynx, adjuvant medical therapy should be considered. Adjuvant therapies that have been investigated include dietary supplements, control of extra-esophageal reflux disease, potent antiviral and chemotherapeutic agents, and photodynamic therapies; although several have shown promise, none to date has "cured" RRP, and some may have serious side effects. Because RRP, although histologically benign, is so difficult to control and can cause severe morbidity and death, better therapies are needed. The potential for a quadrivalent human papilloma vaccine is being explored to reduce the incidence of this disease.
Statistical analysis of the relationships among epidemiological factors, HPV type, and clinical course revealed that patients with HPV-11 and patients younger than 3 years of age at RRP diagnosis are prone to develop more aggressive disease as represented by higher severity scores at endoscopic debridement, more frequent operative debridement procedures per year, a greater requirement for adjuvant therapy, and greater likelihood of tracheal disease with tracheotomy.
BackgroundRRP is a devastating disease in which papillomas in the airway cause hoarseness and breathing difficulty. The disease is caused by human papillomavirus (HPV) 6 or 11 and is very variable. Patients undergo multiple surgeries to maintain a patent airway and in order to communicate vocally. Several small studies have been published in which most have noted that HPV 11 is associated with a more aggressive course.Methodology/Principal FindingsPapilloma biopsies were taken from patients undergoing surgical treatment of RRP and were subjected to HPV typing. 118 patients with juvenile-onset RRP with at least 1 year of clinical data and infected with a single HPV type were analyzed. HPV 11 was encountered in 40% of the patients. By our definition, most of the patients in the sample (81%) had run an aggressive course. The odds of a patient with HPV 11 running an aggressive course were 3.9 times higher than that of patients with HPV 6 (Fisher's exact p = 0.017). However, clinical course was more closely associated with age of the patient (at diagnosis and at the time of the current surgery) than with HPV type. Patients with HPV 11 were diagnosed at a younger age (2.4y) than were those with HPV 6 (3.4y) (p = 0.014). Both by multiple linear regression and by multiple logistic regression HPV type was only weakly associated with metrics of disease course when simultaneously accounting for age.Conclusions/Significance AbstractThe course of RRP is variable and a quarter of the variability can be accounted for by the age of the patient. HPV 11 is more closely associated with a younger age at diagnosis than it is associated with an aggressive clinical course. These data suggest that there are factors other than HPV type and age of the patient that determine disease course.
Pediatric RRP continues to be a highly morbid disease process. New surgical and medical therapies offer hope for better control of this disease in affected patients. Recent advances in immunologic research offer the hope of immune system modulation and augmentation as potential future treatment modalities to better control this disease process.
Adenotonsillectomy in children may be performed safely on an outpatient basis in the majority of cases without an increase in complication rates. However, very young children comprise a unique patient subpopulation with regard to adenotonsillar surgery. Surgical indications in older children tend to be dominated by chronic recurrent infections, whereas younger children usually require surgery for chronic upper airway obstruction related to adenotonsillar hypertrophy. This study was undertaken to evaluate the adenotonsillectomy patient population under 3 years of age. Complication rates related to airway problems, hemorrhage, and dehydration were determined. Children under 3 years of age demonstrated an increased incidence of postoperative airway complications, manifested by oxygen desaturation and transient upper airway obstruction. It is recommended that adenotonsillectomy be performed on such patients on an inpatient basis with close postoperative monitoring including pulse oximetry.
Tonsillectomy subjects in the HS group showed a statistically significant ability to sleep soundly, suggesting that the subjects experienced less pain. These data correlate with the observed decrease in pain scores.
Immediate postoperative results indicate that the microdebrider may be as safe as and, at some institutions, might be more cost-effective than the CO2 laser for removal of recurrent respiratory papillomas.
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