Paolo Campisi

Choanal atresia and choanal stenosis.

Congenital narrowing of the nasal airway at the posterior choanae, which can be uni- or bilateral, is an uncommon condition in pediatric patients. The surgical management of choanal atresia varies widely in different centers. This article discusses the different surgical strategies including: dilation and stenting; trans-palatal repair; and transnasal resection utilizing endoscopic sinus surgery (ESS) techniques. The merits of stents, lasers, CT-guided surgery, and the use of additional agents including mitomycin C are reviewed, as well as the particular problems associated with managing bilateral choanal atresia in neonates.

The epidemiology of juvenile onset recurrent respiratory papillomatosis derived from a population level national database.

To develop a national database of cases of juvenile onset recurrent respiratory papillomatosis (JoRRP) in Canada, to calculate trends in incidence and prevalence of JoRRP from January 1994 to December 2007 at the national and regional level, and to mathematically model the natural history of JoRRP.

Age of Child, More than HPV Type, Is Associated with Clinical Course in Recurrent Respiratory Papillomatosis

Background RRP 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 Findings Papilloma 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 (Fishers 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 Abstract The 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.

Update of respiratory tract disease in children with primary ciliary dyskinesia

Primary ciliary dyskinesia (PCD) is a rare genetic disease characterized by abnormal ciliary structure and function leading to impaired mucociliary clearance and chronic progressive sinopulmonary disease. Upper and lower respiratory tract manifestations are cardinal features of PCD. This review summarizes the current state of knowledge of respiratory tract disease in individuals with PCD and highlights the challenges in identifying and quantifying lung disease in very young children with PCD. No specific therapies are available to correct ciliary dysfunction in PCD. Treatment is not evidence based, and recommendations are largely extrapolated from cystic fibrosis and other conditions with impaired mucociliary clearance. There is a pressing need to develop and validate outcome measures, including patient-reported outcomes, that could be used to evaluate potential therapies in PCD. This review concludes with recommendations for clinical endpoints and outcome measures and a prioritized list of treatments to study in PCD clinical trials.

Acoustic analysis of the voice in pediatric cochlear implant recipients: a longitudinal study.

Objective: To characterize inherent acoustic abnormalities of the deaf pediatric voice and the effect of artificially restoring auditory feedback with cochlear implantation.

Pediatric aggressive fibromatosis of the head and neck: a 20-year retrospective review

UNLABELLED Aggressive fibromatosis in children is a rare, benign condition that is locally infiltrative and destructive. It often presents as a rapidly growing, painless lump in the head and neck region. To date, only small series and case reports have been reported, and the management of the condition remains unclear. Recently, nuclear beta-catenin expression has been suggested as a tumor-specific marker for aggressive fibromatosis (desmoid). AIM The aims of the study were to review our experience of the presentation, management, and treatment outcome of pediatric aggressive fibromatosis in the head and neck and to identify the presence of the desmoid tumor marker beta-catenin within this population. METHOD The study was conducted as a retrospective case review of children diagnosed with aggressive fibromatosis in the head and neck for a period of 20 years and a review of the literature. Pathologic review of the original tumor specimens was undertaken for evidence of positive tumor margins and presence of nuclear beta-catenin expression. RESULTS A total of 10 patients (6 males, 4 females) were identified. The age at presentation ranged from 12 months to 14 years. In total, 8 patients were treated with surgery alone. This included 7 patients with extension of the tumor to the resection margin; all had good long-term outcomes with no disease progression. Two patients received chemoradiotherapy, one as primary treatment, and the other as adjuvant treatment after gross incomplete resection. Both resulted in poor outcomes requiring further treatments. Within our series of pediatric fibromatosis, only 4 cases (40%) had positive results for any nuclear beta-catenin expression, and 6 (60%) of 10 patients had negative results for beta-catenin. CONCLUSION Our experience is that total gross resection and preservation of form and function is of higher priority than achieving a negative resection margin. Pediatric fibromatosis though aggressive is still a benign condition, and careful thought should be taken before considering adjuvant chemoradiotherapy. Nuclear beta-catenin expression should not be considered a specific tumor marker for pediatric aggressive fibromatosis of the head and neck. Pediatric aggressive fibromatosis in this region may be a distinct subtype of desmoid tumor from its adult form.

Time course of juvenile onset recurrent respiratory papillomatosis caused by human papillomavirus.

Background: With the recent licensure of a new quadrivalent vaccine, many diseases caused by human papillomavirus (HPV) can now be prevented, including recurrent respiratory papillomatosis (RRP). The purpose of this study was to describe the burden and time course of juvenile onset RRP. Methods: A retrospective chart review was conducted of children with airway papillomatosis at the Hospital for Sick Children in Toronto, Canada, between 1994 and 2004. Statistical methods included descriptive statistics of the cohort, a repeated events survival model, and nonlinear modeling equations to describe the time course of illness. Results: Nine hundred twenty-six surgical procedures in 67 patients were identified through a review of surgical records. The median age at diagnosis was 3.2 years (range, 0.1–14.8 years) and the most common presenting symptom was hoarseness (75%). Adjuvant pharmacologic therapy (interferon or cidofovir) was used in 13 cases (19%). HPV types 6 or 11 were identified most commonly as the etiologic agent. Nonlinear modeling equations (exponential and quadratic) fit the observed data well, and were superior to linear models. Repeated events survival analysis identified significant prognostic variables: surgeon, adjuvant therapy, and anatomic score. A decision rule is presented that allows the time to next surgery to be predicted based on the previous surgery and the anatomic score. Conclusions: Most patients have a decelerating rate of debulking surgeries over time, well described by our nonlinear modeling equations. Factors affecting the time course of RRP include: intersurgeon variability, the extent and severity of papillomas at the time of laryngoscopy, and the use of adjuvant medical therapies.

Role of adenosine in the ethanol-induced potentiation of the effects of general anesthetics in rats

Acetate, derived from ethanol metabolism in the liver, is released into the circulation and utilized in many tissues including the brain. The subsequent metabolism of acetate results in the production of adenosine that has a number of effects in the central nervous system. The purpose of the present studies, therefore, was to investigate the contribution of metabolically generated adenosine to the ethanol-induced potentiation of the inhalational agents isoflurane and sevoflurane. Changes in the anesthetic requirement for isoflurane and sevoflurane were determined in rats using the tail-clamp procedure. Both ethanol and sodium acetate reduced anesthetic requirement for isoflurane and sevoflurane in a dose-dependent fashion. The effect of acetate on anesthetic requirement was completely blocked by the administration of the adenosine receptor blocker, 8-phenyltheophylline. The ethanol-induced reduction in anesthetic requirement, however, was only partially blocked by 8-phenyltheophylline. Direct intracerebroventricular (i.c.v.) administration of the water-soluble adenosine receptor blocker, 8-sulfophenyltheophylline, also completely blocked the effect of acetate and partially blocked the effect of ethanol. This i.c.v. administration demonstrates that the actions of ethanol and acetate on anesthetic requirement are a central nervous system effect. The i.c.v. administration of the adenosine A1 receptor subtype agonist, R-phenylisopropyl adenosine, potentiated the anesthetic effects of isoflurane and suggests that the A receptor mediates the observed potentiation of anesthetic effect. This is further supported by the concomitant administration of 5-N-ethylcarboxamido adenosine, a non-selective adenosine agonist, with the selective A1 antagonist, 8-cyclopentyltheophylline, showing A1 receptor potentiation of anesthetic requirements. The studies show that (1) acetate potentiates the anesthetic effects of the inhalational anesthetics, sevoflurane and isoflurane; (2) acetate contributes in part to the effect of ethanol on anesthetic potency through metabolically generated adenosine; (3) these effects are likely mediated via adenosine A1 receptor subtypes.

Undergraduate Otolaryngology Education in Canadian Medical Schools

Objectives: To examine the quantity and nature of undergraduate otolaryngology instruction in the Canadian medical school system and to present the management of the undergraduate otolaryngology curriculum at the University of Toronto medical school with a yearly enrolment of 224 students.

The quality of life and health utility burden of recurrent respiratory papillomatosis in children

OBJECTIVE In this study we 1) measured the impact of juvenile-onset recurrent respiratory papillomatosis on health-related quality of life, voice-related quality of life, and family psychosocial well-being; and 2) compared these different measures, exploring their intercorrelation and their correlation with clinical disease severity. STUDY DESIGN Cross-sectional qualitative study. SETTING Tertiary academic pediatric hospital. SUBJECTS AND METHODS Twenty consecutive children with active juvenile-onset recurrent respiratory papillomatosis (JoRRP) were included. Standardized interviews were performed on parents and children with the use of four validated tools: the Health Utilities Index version 3; the Pediatric Voice-Related Quality of Life survey; the Impact on Family Scale; and a visual analogue health preference measure. Clinical disease severity and demographic data also were collected. RESULTS Subjects (13 male, 7 female) had a median age of 9.2 years, median age of JoRRP onset of 3.8 years, and averaged four procedures per year of disease. Mean health utility was 0.76 (95% confidence interval 0.68–0.84) on a scale of 0 (death) to 1 (perfect health). Marked impact on voice-related quality of life and family psychosocial health also was identified. Health burden correlated poorly with existing methods of severity scoring. CONCLUSION This is the first study to use validated measures of health utility, voice-related quality of life, and psychosocial impact. This information has public health implications, providing essential parameters for accurate modeling studies and cost-utility analysis of future interventions, including different human papilloma virus vaccination strategies.