A Prospective Study of the Incidence of Juvenile-Onset Recurrent Respiratory Papillomatosis After Implementation of a National HPV Vaccination Program.

Background: Recurrent respiratory papillomatosis is a rare but morbid disease caused by human papillomavirus (HPV) types 6 and 11. Infection is preventable through HPV vaccination. Following an extensive quadrivalent HPV vaccination program (females 12-26 years in 2007-2009) in Australia, we established a method to monitor incidence and demographics of juvenile-onset recurrent respiratory papillomatosis (JORRP) cases. Methods: The Australian Paediatric Surveillance Unit undertakes surveillance of rare pediatric diseases by contacting practitioners monthly. We enrolled pediatric otorhinolaryngologists and offered HPV typing. We report findings for 5 years to end 2016. Results: The average annual incidence rate was 0.07 per 100000. The largest number of cases was reported in the first year, with decreasing annual frequency thereafter. Rates declined from 0.16 per 100000 in 2012 to 0.02 per 100000 in 2016 (P = .034). Among the 15 incident cases (60% male), no mothers were vaccinated prepregnancy, 20% had maternal history of genital warts, and 60% were first born; 13/15 were born vaginally. Genotyped cases were HPV-6 (n = 4) or HPV-11 (n = 3). Conclusion: To our knowledge, this is the first report internationally documenting decline in JORRP incidence in children following a quadrivalent HPV vaccination program.

Management of Recurrent and Delayed Post-Tonsillectomy and Adenoidectomy Hemorrhage in Children.

OBJECTIVE: To review our experience on post-tonsillectomy and/or adenoidectomy hemorrhage (PTAH) at a tertiary pediatric referral hospital and to evaluate the management and risk factors for recurrent postoperative hemorrhage and for delayed bleeding after day 14. METHODS: A retrospective chart review was performed for all pediatric patients admitted to The Children's Hospital at Westmead for PTAH between July 01, 2014, and June 30, 2019. Patients with recurrent hemorrhage and those with bleeding after day 14 were selected for subanalysis. RESULTS: Of the 291 patients admitted for PTAH, 31 (11%) patients had recurrent postoperative hemorrhage, and 11 (4%) patients had delayed bleeding after day 14. Surgical intervention for cessation of hemorrhage was required in 88 (30%) patients, including 2 patients who required return to the theater more than once. Nine (3%) patients received blood transfusions. The average number of days between bleeding episodes was 4 days. Recurrent postoperative hemorrhage occurred in 8.5% of patients who were managed operatively at their first presentation compared to 11.4% of patients who were managed nonoperatively (odds ratio: 1.1; 95% confidence interval 0.43-2.8). No association was found between abnormal coagulation profile, surgical indication, and risk of delayed postoperative hemorrhage. CONCLUSIONS: Recurrent or delayed postoperative hemorrhage represents a small proportion of children with postoperative bleeding and cannot be reliably predicted. Management of first presentations with either a conservative or a surgical approach is reasonable since the risk of recurrent of PTAH may be unrelated to the choice of management at initial presentation. Careful preoperative counseling of patients and their families is important to help set expectations in the event of PTAH.

Complications and outcomes following open laryngotracheal reconstruction: A 15 year experience at an Australian paediatric tertiary referral centre.

OBJECTIVE: To report on our experience with open pediatric laryngotracheal reconstruction (LTR) with cartilage interposition grafts over the past 15 years and document setbacks and factors that affect postoperative outcomes. METHOD: A retrospective chart review was performed on all pediatric patients who underwent single and double-stage LTR at The Children's Hospital in Westmead between August 2004 and July 2019. The outcomes measured include the overall decannulation rate, rates of postoperative complications, need for subsequent procedures and postoperative functional results (dyspnea, stridor, voice impairment). RESULTS: Between August 2004 and July 2019, a total of 51 open LTRs were performed on 46 patients, with mean age 3.9 years (range 1 month to 12.1 years), including 11 revision operations (11.8%). The most common indication for surgery was subglottic stenosis, followed by bilateral vocal cord motion impairment followed by anterior glottic web. 13 cases (25.5%) were complicated by ventilator associated pneumonia in the postoperative period and 11 patients (21.6%) experienced wound complications ranging from localised wound abscess to wound dehiscence and graft failure. Patients with viral infections had an increased risk of wound dehiscence and graft failure (OR 1.8, 95% CI 1.01 - 3.23). Patients with a greater decrease in albumin in the postoperative period were more likely to have wound complications (OR 1.8; 95% CI 1.17 -2.83). Ten patients failed extubation and required a subsequent tracheostomy. Severity of stenosis, age at time of surgery, history of prematurity and revision LTR were not predictors for need for reintubation and/or tracheostomy after surgery. 23 cases (45.1%) underwent subsequent endoscopic procedures such a balloon dilatation to manage restenosis following LTR. Though 45.7% had a very good outcome, a significant group of 21% had ongoing voice issues. CONCLUSION: Good outcomes were achieved following open LTR for pediatric laryngotracheal stenosis despite postoperative complications. At final follow up, 33 patients (71.7%) had good exercise tolerance with no or mild stridor. Forty-one of the forty-six patients (89.1%) were successfully decannulated.

Adenotonsillectomy in the context of obstructive sleep apnoea.

Adenotonsillectomy (T&A) is a common surgical procedure. Its frequency is highest in the paediatric age range and its most common current indication is obstructive sleep apnoea (OSA). Sleep studies are used to document the presence and severity of OSA. This review will focus on indications for and complications of T&A in the context of the age range and setting where this surgery is undertaken for OSA in children.

International Pediatric Otolaryngology Group: Consensus guidelines on the diagnosis and management of type I laryngeal clefts.

INTRODUCTION: The diagnosis and management of type I laryngeal clefts can be controversial and varies across centers and surgeons. Using existing peer-reviewed literature to develop an expert-based consensus will help guide physicians in the treatment of these patients as well as develop research hypotheses to further study this condition. OBJECTIVE: To provide recommendations for the diagnosis and management of type I laryngeal clefts. METHODS: Determination of current expert- and literature-based recommendations, via a survey of the International Pediatric Otolaryngology Group, using a modified Delphi method. SETTING: Multinational, multi-institutional, tertiary pediatric hospitals. RESULTS: Consensus recommendations include diagnostic workup, medical management, pre-operative, intra-operative and post-operative considerations for type I laryngeal clefts. CONCLUSIONS: This guide on the diagnosis and management of patients with type I laryngeal clefts is aimed at improving patient care and promoting future hypothesis generation and research to validate the recommendations made here.

Juvenile recurrent respiratory papillomatosis: 10-year audit and Australian prevalence estimates.

OBJECTIVES/HYPOTHESIS: To estimate the prevalence of juvenile onset recurrent respiratory papillomatosis (RRP) in Australia, describe its epidemiological profile, and assess the positive predictive value of International Classification of Disease, 10th revision (ICD-10) code D14.1 (benign neoplasm of larynx) in children for hospitalization due to RRP. STUDY DESIGN: Retrospective case series. METHODS: Retrospective case review undertaken at the three tertiary pediatric hospitals in New South Wales (Australia's largest state), by reviewing medical records of patients aged 0 to 16 years admitted during 2000-2009 containing the ICD-10 Australian modification code D14.1 or other possible disease (D14.2-4, D14.3, D14.4) and RRP-related procedure codes. For RRP diagnoses, we recorded treatment dates, length of stay, extent of disease, and surgical and adjuvant treatments. The positive predictive value (PPV) of code D14.1 and median number of hospitalizations per year were applied to national hospital separations data from 2000/2001 to 2012/2013 to estimate disease prevalence. RESULTS: We identified 30 cases of RRP using code D14.1, which had a PPV of 98.1%, with no further cases identified using other codes. Fifty-seven percent of cases were female, median age of onset was 36 months, and median treatment duration was 36 months (mean = 40 months, range = 1-118). There was one patient death. Between 2000 and 2013, the estimated national prevalence rate was 0.81 per 100,000 aged < 15 years, peaking at age 5 to 9 years (1.1 per 100,000). CONCLUSIONS: RRP prevalence can be monitored after human papillomavirus vaccination programs using routine hospital data. LEVEL OF EVIDENCE: 4 Laryngoscope, 126:2827-2832, 2016.

Low inter-arytenoid height: a subclassification of type 1 laryngeal cleft diagnosis and management.

OBJECTIVE: To report our experience of patients with type 1 laryngeal cleft, (including low inter-arytenoid height) who failed conservative management over a five year period. We describe the diagnostic elements of the history, examination at laryngobronchoscopy and provide a management algorithm including the use of inter-arytenoid submucosal injection of gelfoam as a temporary therapeutic as well as diagnostic tool. METHODS: A retrospective case note review over a five year period was undertaken to review all cases of type 1 laryngeal cleft who failed conservative management. Presenting symptoms, diagnostic procedures, surgical interventions and clinical outcomes were reviewed. RESULTS: Seventeen patients were identified. Chronic cough was the most consistent feature in the history (100%). All patients underwent a microlaryngoscopy with binocular microlaryngeal assessment. Six patients (35%) underwent gelfoam injection; four of these went on to a formal repair. The remaining 11 all had a repair performed without injection. The success of surgical repair was 80% (12/15) however in the other three, all had improvement in symptoms. CONCLUSIONS: Type 1 laryngeal cleft anomalies may extend beyond that described by Benjamin and Inglis. An appropriate history as well as binocular inspection at the time of laryngoscopy is essential. Injection augmentation offers a safe tool in the assessment and management, and endoscopic surgical repair remains the standard for definitive therapy in those that fail conservative management.

Vocal cord reconstruction to treat aspiration caused by a post-intubation posterior glottic furrow.

OBJECTIVE: To present the case of a premature child with a furrow in the posterior vocal cord as a result of prolonged intubation, with symptoms of aspiration and poor voice, treated with a novel method of vocal cord reconstruction. METHODS: The vocal cord was reconstructed endoscopically by freeing up the edge of cord remnant and suturing this to a flap of inter-arytenoid mucosa to create a new cord. RESULTS: The resulting neo-cord was able to achieve full glottic closure with resolution of aspiration, and this was also accompanied by improvement in voice quality. CONCLUSION: The described vocal cord reconstruction method proved to be a useful treatment for aspiration and poor voice caused by a post-intubation vocal cord furrow.

Distraction osteogenesis and glossopexy for Robin sequence with airway obstruction.

BACKGROUND: We present six cases of infants with Robin sequence and severe obstructive sleep apnoea who failed treatment with nasal mask continuous positive airway pressure (CPAP). Surgical intervention with mandibular distraction osteoneogenesis with glossopexy meant tracheostomy was avoided. Polysomnography (PSG) was used to document the severity of the obstructive sleep apnoea. The aim of this report was to assess the value of mandibular distraction osteogenesis with glossopexy in children with Robin sequence using PSG preoperatively and post-operatively. METHODS: Retrospective review of medical records to assess details of preoperative clinical progress, CPAP therapy, endoscopy, PSG and respiratory parameters. RESULTS: Between 2003 and 2008, 20 infants with Robin sequence were admitted to the neonatal intensive care unit of our tertiary paediatric hospital. Six infants (30%) failed a treatment trial of CPAP during that period, but were subsequently successfully managed with mandibular distraction osteoneogenesis and glossopexy. At endoscopy, all had associated airway problems which included congenitally hypoplastic epiglottis (n= 2), laryngomalacia (n= 2), unilateral choanal atresia (n= 1) and long-segment tracheal stenosis (n= 1). CONCLUSION: Mandibular distraction osteogenesis with glossopexy provides relief of severe upper airway obstruction for infants with Robin sequence. Epiglottic maldevelopment was present in two infants (33%). Endoscopy and PSG assisted clinical management, but highlighted ongoing respiratory issues.

Estimating the prevalence of and treatment patterns for juvenile onset recurrent respiratory papillomatosis in Australia pre-vaccination: a pilot study.

BACKGROUND: Recurrent respiratory papillomatosis (RRP) causes serious morbidity. RRP in Australia may be eliminated in the near future following the implementation of a national vaccination program using a human papillomavirus (HPV) vaccine that protects against infection with HPV types 6 and 11, those responsible for RRP. Baseline data on RRP prevalence and disease burden in Australia are lacking. METHODS: Three study methods were used to estimate the burden of juvenile onset RRP in Australia. We conducted a retrospective chart review of RRP cases treated at The Children's Hospital at Westmead over 10 years, examined the coding of these cases, and then calculated and applied the positive predictive value of the codes to national data to estimate the prevalence of RRP in Australia. We also conducted an online survey of otolaryngologists in Australia who manage RRP. RESULTS: Nineteen patients were treated at the hospital over 10 years, involving 359 admissions. We estimate that between 33 and 56 RRP cases aged <20 are being treated nationally per year (0.6-1.1 per 100 000 persons), with children 5-9 years having a higher estimated rate of 1.2-1.8 per 100 000. Among 39 otolaryngologists treating juvenile onset RRP, the majority (73%) treated RRP in a paediatric tertiary hospital, and used the microdebrider for ablation of lesions. CONCLUSIONS: Our estimates of RRP disease burden agree with international estimates. As a small number of clinicians treat RRP nationally, we believe that establishment of a national RRP register is both feasible and necessary to monitor the impact of vaccination.

Congenital anterior glottic webs with subglottic stenosis: surgery using perichondrial keels.

UNLABELLED: Four children with severe congenital anterior glottic webs required surgical reconstruction of their laryngeal airway to either avoid a tracheotomy or allow tracheotomy decannulation. The technique of re-establishing a glottic inlet allows both normal respiration, good cough and a satisfactory voice outcome. It utilizes an autogenous graft with perichondrium acting as an overlay keel to minimize glottic web reformation. All children were found to have Shprintzen syndrome. OBJECTIVE: The objective of this case series was to document the method of surgically using this perichondrial keel for airway reconstruction. It also examined the subsequent outcome and associated complications that were encountered. METHODS: A prospective analysis of four cases from 2001 to 2008 created a database of information. All cases were classified using the Cohen staging system. They were treated with the same surgical technique using auricular or costal cartilage graft with attached perichondrium, but the postoperative course was tailored to each individual case. RESULTS: All four children were successfully treated with removal or avoidance of a tracheostomy. All had an associated subglottic stenosis treated, and had no major complication requiring revision tracheotomy. They did not have any respiratory complications, and they produce a satisfactory voice albeit still slightly husky. CONCLUSION: All four cases had Shprintzen syndrome, and confirms the need to screen for VCFS in children with an anterior glottic web. Tracheotomy is still the gold standard of treatment in severe congenital anterior glottic webs. The described technique offers another good option to the paediatric airway surgeon in managing this condition.