Assessment of pulsed-dye laser therapy for pediatric cutaneous vascular anomalies.

IMPORTANCE: Although facial vascular malformations are often treated by facial plastic surgeons, no reliable validated assessment tool exists for surgeons to assess results. OBJECTIVES: To use our assessment tool to analyze results from pulsed-dye laser therapy used for pediatric facial vascular malformations, and to determine interrater reliability of our assessment tool in a standard clinical environment without the use of professional photography. DESIGN: A blinded retrospective review of pediatric patients who underwent pulsed-dye laser therapy for treatment of hemangiomas of infancy (HOIs) and port-wine stains. Three pediatric otolaryngologists and facial plastic surgeons independently rated all of the pictures using our assessment tool. SETTING: Tertiary care pediatric hospital. RESULTS: Pulsed-dye laser therapy was performed in 22 patients, 17 with HOIs and 5 with port-wine stains. Patients with HOIs treated only by pulsed-dye laser showed, on average, a 50% to 75% improvement in color, 1% to 24%, improvement in thickness, and 1% to 24% improvement in size of the lesion. Eight patients with HOIs underwent surgery after laser therapy, and of these, 100% saw improvement in color, thickness, and size. Port-wine stains showed a 1% to 24% improvement in color and no improvement in thickness or size. Interrater reliability for questions 1 to 6 was 0.92, 0.92, 0.93, 0.91, 0.70, and 0.10, respectively. CONCLUSIONS AND RELEVANCE: Currently, no standardized or validated methods exist to evaluate results from intervention of pediatric facial HOIs and port-wine stains. Our assessment tool is reliable to assess patients with HOIs and port-wine stains who undergo pulsed-dye laser therapy and surgical reconstruction. LEVEL OF EVIDENCE: 3.

Effect of endoscopic sinus surgery on pulmonary status of adults with cystic fibrosis.

OBJECTIVE: Functional endoscopic sinus surgery (FESS) provides symptomatic relief of sinus disease in patients with cystic fibrosis (CF), but it is unclear whether it has beneficial effects on lung disease in this population. This study assessed the effect of FESS on the respiratory status of adult patients with CF. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary medical center. SUBJECTS: Thirty-two adult patients with CF who underwent 45 operative cases. METHODS: Clinical information retrieved for the 12-month periods preceding and following to determine the effect of FESS on the rate of decline in lung function, as well as intravenous antibiotic use and hospitalization for pulmonary exacerbation. RESULTS: The rate of decline in forced expiratory volume in 1 second and forced vital capacity was not significantly different in the 12 months before and after FESS. Functional endoscopic sinus surgery did not reduce days hospitalized or days on intravenous antibiotics for a respiratory exacerbation in the pre- vs postoperative period. Limiting the analysis to the 30 surgeries that were performed in patients with concomitant respiratory symptoms (ie, excluding the 15 surgeries performed for sinus symptoms alone) did not significantly alter the results. Covariates of importance in CF, including CFTR genotype, gender, or microbiology, did not affect the study results. CONCLUSION: These results did not demonstrate an effect of FESS on progression of lung disease in patients with CF, but further research is needed because low statistical power has made some of the negative findings inconclusive.

Prevalence of dysplasia in juvenile-onset recurrent respiratory papillomatosis.

OBJECTIVES: To quantify the prevalence of dysplasia and to evaluate the impact of use of cidofovir in recurrent respiratory papilloma biopsy specimens obtained from a pediatric population. DESIGN: Retrospective review of patient medical records and histopathologic test results from January 1, 1998, through December 31, 2008. SETTING: Children's Hospital of Wisconsin. PATIENTS: Patients with a history of operation treated for recurrent respiratory papillomatosis. MAIN OUTCOME MEASURES: The presence or absence of dysplasia identified in a papilloma specimen and patient characteristics, such as age of initial presentation, number of operations, tobacco exposure, treatment for reflux, and use of cidofovir, were quantified. RESULTS: Treatment for recurrent respiratory papillomatosis was identified in 21 patients. Age at initial diagnosis ranged from 8 months to 14 years. A total of 123 recurrent respiratory papillomatosis specimens in 20 patients were identified. Dysplasia was seen in less than 1% of samples (1/123), which represents 5% of total patients. Seven patients (35%) received cidofovir treatment and none of them developed dysplasia. These data demonstrate a lack of correlation between cidofovir treatment and dysplasia, with the P value being nonsignificant (Fisher exact test, P=.4). CONCLUSION: Dysplasia is a rare event in pediatric recurrent respiratory papillomatosis, and there does not appear to be an association between the use of cidofovir and dysplastic changes.