HPV Strain Predicts Severity of Juvenile-Onset Recurrent Respiratory Papillomatosis with Implications for Disease Screening.

Juvenile-onset recurrent respiratory papillomatosis (JoRRP) is the most common benign neoplasm of the larynx in children, presenting with significant variation in clinical course and potential for progression to malignancy. Since JoRRP is driven by human papillomavirus (HPV), we evaluated viral factors in a prospective cohort to identify predictive factors of disease severity. Twenty children with JoRRP undergoing routine debridement of papillomas were recruited and followed for ≥1 year. Demographical features, clinical severity scores, and surgeries over time were tabulated. Biopsies were used to establish a tissue bank and primary cell cultures for HPV6 vs. HPV11 genotyping and evaluation of viral gene expression. We found that patients with HPV11+ disease had an earlier age at disease onset, higher frequency of surgeries, increased number of lifetime surgeries, and were more likely to progress to malignancy. However, the amplitude of viral E6/E7 gene expression did not account for increased disease severity in HPV11+ patients. Determination of HPV strain is not routinely performed in the standard of care for JoRRP patients; we demonstrate the utility and feasibility of HPV genotyping using RNA-ISH for screening of HPV11+ disease as a biomarker for disease severity and progression in JoRRP patients.

The impact of persistent pediatric obstructive sleep apnea on the Quality of Life of Patients' families.

OBJECTIVES: To (1) quantify the quality of life (QOL) for families of children affected by persistent obstructive sleep apnea (OSA), and (2) assess factors contributing to the negative impact of OSA on families. METHODS: Prospective case series in a multidisciplinary upper airway center at a tertiary pediatric institution. Our study included patients with persistent OSA referred to our clinic from 2014 to 2016. Both patients and their families completed validated questionnaires assessing QOL and OSA symptoms, including the Family Impact Questionnaire (FIQ), Pediatric Sleep Questionnaire, the Pediatric Quality of Life Inventory, the OSA-18, and the Epworth Sleepiness Scale. RESULTS: Families of 67 patients were included. The mean patient age was 12.5 (95%CI 11.9-13.1); 23 (34.3%) were female, and the mean BMI percentile was 80.2 (95%CI 73.6-86.8). The single most common comorbidity was Down syndrome (45.6%). The mean obstructive apnea-hypopnea index was 9.7 ±â€¯10.3 events/hour. A significant association exists between OSA severity and 18 FIQ negative subscore (P < 0.001). Financial impact was the primary negative concern for parents of patients with OSA compared to those without OSA (P = 0.03). There were no other significant differences between those with and without OSA. CONCLUSIONS: There was a significant correlation between persistent OSA severity and the FIQ negative impact of disease score on patients' families. Concern regarding financial burden was more common for families of children with OSA than for those without OSA. This suggests that targeted interventions, particularly regarding the financial burden of persistent OSA diagnosis and management, may provide some relief to families.

Clinical outcomes after shared decision-making tools with families of children with obstructive sleep apnea without tonsillar hypertrophy.

OBJECTIVES: To determine if shared decision-making tools (SDMTs) improve clinical outcomes for these children. Shared decision making (SDM) is a collaborative process in which patients and clinicians jointly establish treatment plans that integrate clinical evidence and patient values/preferences. We previously reported less decisional conflict using a SDMT for families of children with obstructive sleep apnea (OSA) without tonsillar hypertrophyl; however, the clinical impact of this finding is unknown. METHODS: Prospective single-blind randomized controlled trial for consecutive patients referred to a multidisciplinary upper airway center. The study group used a SDMT, whereas the control group did not; all were followed until their next appointment and polysomnogram. RESULTS: We assessed 50 families (24 study, 26 controls); mean age of patients was 8.8 (95% confidence interval 6.9-10.6) years, and 44% were female. After their initial visit, there was agreement between families and providers on the best treatment option for 22 of 24 (91.7%) study patients and 12 of 26 (46.2%) controls (P < 0.001). Before the first follow-up, four control families (15.4%) modified their treatment plan, whereas none of the study families did so (P = 0.04). Continuous positive airway pressure (CPAP) compliance was 27% (3 of 11) for controls and 57% (5 of 8) for study patients (P = 0.11). The median obstructive apnea-hypopnea index significantly improved in study patients from 13.4 (range, 20.0-57.2) to 3.5 (range 0.4-45.5, P = 0.01] events per hour, but not in controls, with 9.4 (range, 0.9-76.2) to 4.9 (range, 0-116, P = 0.10) events per hour. CONCLUSION: Families of children with OSA without tonsillar hypertrophy who were counseled regarding treatment options using SMDTs were more likely to undergo agreed upon treatment and had higher CPAP compliance. LEVEL OF EVIDENCE: 1b. Laryngoscope, 129:2646-2651, 2019.

Comparison of Patient- and Parent-Reported Quality of Life for Patients Treated for Persistent Obstructive Sleep Apnea.

Objectives To evaluate the impact of the treatment of persistent pediatric obstructive sleep apnea (OSA) on quality of life (QOL) with patient-reported outcomes tools and to compare parent- and self-reported Pediatric Quality of Life Inventory (PedsQL) scores. Study Design Prospective case series. Setting Multidisciplinary upper airway center at a tertiary pediatric institution. Subjects and Methods Children with persistent OSA referred to our multidisciplinary upper airway center from 2014 to 2016. Patients and their families completed validated questionnaires for QOL, including the Family Impact Questionnaire, the Obstructive Sleep Apnea-18, the PedsQL, and the Epworth Sleepiness Scale for Children and Adolescents. They completed the same surveys after treatment. Results Twenty-three children (7 females) and their families were included in the study. Patients had a mean age of 12.7 years. Pretreatment, the mean obstructive apnea-hypopnea index was 15.0 events/hour (95% CI, 8.7-21.3); after treatment, the mean was 3.9 events/hour ( P = .003). The mean PedsQL score for children improved from 60.5 to 74.2 ( P = .04). The PedsQL score for parents did not significantly improve (61.6 to 63.8, P = .39). There was a significant though weak association between OSA severity ( R2 = 0.25, P = .03) and PedsQL scores. The Family Impact Questionnaire negative subscore and Epworth Sleepiness Scale for Children and Adolescents scores did not change with treatment. Conclusions For children treated for persistent OSA, we found that self-reported QOL significantly improved after treatment; however, parent-reported QOL did not significantly change. It is unclear if parents underestimate or patients overestimate QOL after treatment. We suggest that patient-reported outcomes be obtained when feasible.

Characterizing Decisional Conflict for Caregivers of Children With Obstructive Sleep Apnea Without Tonsillar Hypertrophy.

STUDY OBJECTIVES: The goals of this study were to (1) evaluate the degree of decisional conflict (DC) experienced by caregivers of children with obstructive sleep apnea (OSA) without tonsillar hypertrophy; and (2) describe the association between DC, quality of life (QOL), and OSA severity. METHODS: This study comprised children evaluated in the multidisciplinary upper airway center at the Cincinnati Children's Hospital Medical Center from December 2014 to May 2016. Caregivers were asked to complete surveys (Pediatric Quality of Life Inventory 4.0 [PedsQL], OSA-18, Epworth Sleepiness Scale, Family Impact Questionnaire, Decisional Conflict Scale, CollaboRATE scale, and SURE questionnaire) during a clinic visit. Polysomnography data were collected. Analysis included Kruskal-Wallis, Wilcoxon rank-sum, and regression testing. RESULTS: Caregivers of 76 children participated; 16 (21.1%) had high DC. There were no significant differences in demographics between those with low and high DC; the low DC group had a higher obstructive apnea-hypopnea index (13.2 versus 12.3 events/h; P = .013). Overall and disease-specific QOL, sleepiness, family impact scores, and DC did not differ by OSA severity except for the PedsQL physical subcategory (P = .02). DC was associated with the total PedsQL (P = .043) on univariate regression; however, this did not persist (P = .61) after controlling for demographic variables. DC scores correlated well with CollaboRATE and SURE throughout the analysis (P < .001). CONCLUSIONS: The proportion of caregivers of children with OSA without tonsillar hypertrophy who experienced a high level of DC regarding their child's treatment was 21.1%. Neither DC nor OSA severity was related to QOL in children with OSA. The briefer SURE or CollaboRATE scales were adequate tools to measure DC in these children.

Systematic Review of Drug-Induced Sleep Endoscopy Scoring Systems.

Objective To systematically review the scoring systems used to report findings during drug-induced sleep endoscopy (DISE) for adults and children with obstructive sleep apnea. Data Sources PubMed, CINAHL, EBM Reviews, Embase, and Scopus databases. Review Methods This is a systematic review of all indexed years of publications referring to scoring of DISE for children and adults with obstructive sleep apnea. The type of DISE scoring system utilized was the primary outcome. PRISMA guidelines were followed to carry out this review; articles were independently reviewed by 2 investigators. All pediatric and adult studies that utilized ≥1 DISE grading systems were included. Results Of 492 identified abstracts, 44 articles (combined population, N = 5784) were ultimately included; 6 reported on children, 35 on adults, and 1 on children and adults. Twenty-one reporting methods were used in these studies, with the most common being the VOTE system (velum, oropharynx, tongue base, and epiglottis; 38.6%) and the Pringle and Croft classification (15.9%). The sites of obstruction most commonly included in a scoring system were the tongue base (62%), lateral pharynx/oropharynx (57%), palate (57%), epiglottis/supraglottis (38%), and hypopharynx (38%). Less commonly included sites were the larynx (29%), velum (23%), nose (23%), tongue (14%), adenoids (10%), and nasopharynx (10%). Conclusion There is no consensus regarding which scoring system should be utilized to report findings during DISE. The VOTE system and the Pringle and Croft classification were the most frequent scoring systems reported for patients undergoing DISE. Standardization of the reporting of DISE findings would improve comparability among studies.

A shared decision-making tool for obstructive sleep apnea without tonsillar hypertrophy: A randomized controlled trial.

OBJECTIVES/HYPOTHESIS: Shared decision-making is a process whereby patients and clinicians jointly establish a treatment plan integrating clinical evidence and patient values and preferences. Although this approach has been successfully employed in numerous medical disciplines, often using shared decision-making tools, otolaryngologic research assessing its use is scant. Our primary objective was therefore to determine if the tools we developed reduced decisional conflict for children with obstructive sleep apnea without tonsillar hypertrophy. STUDY DESIGN: Prospective, single-blind, randomized controlled trial. METHODS: We enrolled consecutive patients meeting inclusion criteria who were referred to our multidisciplinary upper airway center. Study patients used a shared decision-making tool whereas controls did not. Measures of decisional conflict (SURE [Sure of myself, Understanding information, Risk benefit ratio, Encouragement], CollaboRATE, and the Decisional Conflict Scale [DCS]) were obtained pre- and postvisit. RESULTS: We assessed 50 families (study group = 24, controls = 26). The mean age was 8.8 ± 6.6 years, 44% were female, 86% were white, and the mean obstructive apnea-hypopnea index was 12.7 ± 15.6 events/hour. The previsit mean DCS score was similar for controls (42.7) and study patients (40.8) (P = .38). The postvisit mean DCS score for controls was 13.3 and for study patients 6.1 (P = .034). Improvement in this score was greater in the study group (P = .03). At previsit evaluation, 63% of controls and 58% of study patients were unsure about their options. Postvisit, this improved to 4.1% and 0%, respectively. CONCLUSIONS: Families counseled regarding treatment options using shared decision-making tools had significantly less decisional conflict than those who did not use these tools. These positive outcomes suggest that clinicians should consider integrating this approach into clinical practice. LEVEL OF EVIDENCE: 1b. Laryngoscope, 128:1007-1015, 2018.

Outcomes of Drug-Induced Sleep Endoscopy-Directed Surgery for Pediatric Obstructive Sleep Apnea.

Objectives To determine the effectiveness of pediatric drug-induced sleep endoscopy (DISE)-directed surgery for children with infant obstructive sleep apnea (OSA) or OSA after adenotonsillectomy. Study Design Case series with chart review. Setting Tertiary care pediatric hospital. Subjects and Methods We included 56 children undergoing DISE from October 2013 to September 2015 who underwent subsequent surgery to address OSA. The primary outcome was successful response to DISE-directed surgery based on the postoperative obstructive Apnea-Hypopnea Index (oAHI). Wilcoxon matched-pairs signed-ranks tests were used to compare polysomnography variables before and after surgery, and regression was used to model response to surgery. Results We evaluated 56 patients with a mean age of 5.9 ± 5.5 years (range, 0.1-17.4) and mean body mass index of 21.2 ± 7.9 kg/m2 (percentile, 77 ± 30). The most commonly performed surgical procedures were adenoidectomy (48%, n = 27), supraglottoplasty (38%, n = 21), tonsillectomy (27%, n = 15), lingual tonsillectomy (13%, n = 7), nasal surgery (11%, n = 6), pharyngoplasty (7%, n = 4), and partial midline glossectomy (7%, n = 4). Mean oAHI improved from 14.9 ± 13.5 to 10.3 ± 16.2 events/hour, with 54% (30 of 56) of children with oAHI <5 and 16.1% (9 of 56) with oAHI <1. There was a significant improvement in oAHI ( P = .001) and saturation nadir ( P < .001) but not in time with end tidal carbon dioxide >50 mm Hg ( P = .14). Multivariable modeling, controlling for age, race, body mass index, sex, and baseline polysomnography variables, revealed that white race predicted success of DISE-directed surgery. Conclusion Fifty-four percent of children with infant OSA or persistent OSA after adenotonsillectomy had oAHI <5 events per hour after DISE-directed surgery. Only white race was predictive of oAHI <5 events per hour.

Gaps in evidence: Management of pediatric obstructive sleep apnea without tonsillar hypertrophy.

OBJECTIVES/HYPOTHESIS: Persistent obstructive sleep apnea (OSA) is demonstrated in 40% of children after adenotonsillectomy. We previously evaluated the basis of management decisions in children with OSA without tonsillar hypertrophy and found that 61% of decisions were non-evidence based. The aim of this study was to identify gaps in evidence for the management of children with OSA without tonsillar hypertrophy. STUDY DESIGN: Case series. METHODS: We recorded all real-time decisions made by pediatric subspecialists from eight disciplines that participated in an upper airway clinic and management conferences. Practitioners were immediately queried regarding the basis of their decisions, and non-evidence-based decisions were categorized. RESULTS: During 10 case conferences and five clinics, 507 decisions were made for 63 children (43% with Down syndrome, 20% with Pierre Robin sequence). The 309 non-evidence-based decisions most commonly pertained to follow-up timing and appropriate subspecialty clinic location (116/309, 38%) as well as timing for repeat polysomnography (35/309, 11%), especially in children at high risk for persistent OSA after treatment. Additional gaps identified included the likelihood of OSA improvement from weight loss, and effectiveness of sleep surgical procedures (i.e., lingual tonsillectomy, posterior midline glossectomy, and craniofacial surgery) alone or in combination. CONCLUSIONS: Identified gaps in evidence included timing and location of follow-up, appropriate use of polysomnography for surveillance, effectiveness of specific surgical procedures performed alone and in combination, and the use of oral appliances and continuous positive airway pressure therapy in children with Down syndrome. We also found a need for studies to compare the effectiveness of these treatment options in diverse patient populations. LEVEL OF EVIDENCE: 4 Laryngoscope, 126:758-762, 2016.