Conservative Airway Management Successful in Majority of Infants With Pierre-Robin Sequence at Queensland Children's Hospital: A Retrospective Review.

PURPOSE: Pierre-Robin Sequence (PRS) is a congenital abnormality characterized by micrognathia, glossoptosis, and variable severity upper airway obstruction. Clear management algorithms are lacking, particularly the indications for surgical versus nonsurgical intervention. The authors reviewed the management of these children in Queensland. METHODS: All children diagnosed with PRS at Queensland Children's Hospital from April 2014 to October 2019 were identified (n=45), and their charts were retrospectively reviewed. Three management patterns emerged: prone/lateral positioning, nasopharyngeal airway (NPA) use, and surgery (tracheostomy or mandibular distraction). RESULTS: Most children (n=30; 67%) were managed successfully nonsurgically with an NPA (median age of insertion 0.25 mo, median duration 5.0 mo). Of these, 12 patients (40%) also required supplemental oxygen. The median age of NPA cessation was 5.5 months, with oxygen therapy ceasing at a median 8.25 months, upon which no further support was required. The remaining majority (n=13; 29%) of children were managed without an NPA, using positioning alone (10/13; 77%) or positioning combined with supplemental oxygen (1/13), CPAP (1/13), or both adjunct measures (1/13). Only 2 patients underwent surgical intervention. Feeding supplementation using nasogastric tube was necessary in 78% of patients for a median duration of 4 months. Cleft palate co-existed in all but one patient. CONCLUSION: Management of upper airway obstruction in PRS children is variable between units. Over a 5-year period, 96% of children with PRS were successfully managed without surgical intervention at the Queensland Children's Hospital. These findings contrast with some other literature and may suggest that more careful consideration of surgical intervention in PRS patients is prudent.

Longitudinal change in sleep, functional, and behavioural characteristics in a cohort of children with Down syndrome.

This paper describes the longitudinal change in sleep, functional, and behavioural characteristics in a cohort of children with Down syndrome, including the effect of sleep interventions in a subset. A prospective longitudinal cohort study was undertaken in children with Down syndrome aged 3-16 years comparing (1) children referred to a tertiary sleep medicine clinic who received sleep hygiene advice and an additional sleep treatment (DSref_I) with (2) children attending the same clinic who only received sleep hygiene advice (DSref_N) and (3) children recruited from the community who, were not receiving any treatment (DScomm). Data collected included demographic and medical history information, Child Sleep Habits Questionnaire-Abbreviated (CSHQ-A), Life-Habits Questionnaire (Life-H) and Child Behaviour Checklist (CBCL) at baseline and then 6-monthly for a total of 18 months. Any sleep interventions during this time were recorded. A total of 57 children were included (DSref_I, n = 16; DSref_N, n = 25; DScomm, n = 16). At recruitment, the median CSHQ-A total score was high (>41) in all three subgroups, but highest in the DSref_I subgroup (median [interquartile range] Dsref_I score 58 [53-66] versus DSref_N score 49 [43-53], p = 0.019). Although improved, 80% of participants in the DSref_I subgroup still had a CSHQ-A total score >41 at the last assessment point. The median total Life-H and total CBCL scores were not significantly different between groups at baseline and there was no significant time, group, or interaction effect seen through the study. Over an 18-month period, sleep problems were seen to persist in children with Down syndrome. Treatment resulted in only modest improvements in sleep.

Extended Reality Use in Paediatric Intensive Care: A Scoping Review.

Background: Extended reality (XR) technology such as virtual and augmented reality is increasingly being utilised in paediatric medicine due to its role in medical education and reported positive impacts on outcomes including pain, anxiety, and sleep. To the author's knowledge, no previous reviews investigating the use of XR in paediatric intensive care have been undertaken. Objectives: To scope the use of XR in paediatric intensive care, and assess its barriers to adoption, including safety considerations, cleaning and infection control. Eligibility criteria: All articles of any methodological design discussing the use of XR within paediatric intensive and critical care were included. Sources of evidence: Four databases (EMBASE, CINAHL, PsychInfo, PubMed) and Google Scholar were searched without any limitations on publication year. Charting methods: Data was extracted into Microsoft Excel by two authors independently (AG & SF) and cross-checked for completeness. Results: One hundred and eighty-eight articles were originally identified. Following the application of eligibility criteria 16 articles utilising XR in clinical interventions (n = 7) and medical education (n = 9) were included. Articles utilised VR and AR for highly variable purposes within both medical education (eg disaster preparedness, intubation) and clinical interventions (eg decrease pain, nausea, anxiety and improve Glasgow Coma Scale). Conclusions: While research into the use of XR in paediatric intensive care is still in its infancy it has increased dramatically over the past 5 years within two key areas. Firstly, in healthcare education, to assist in the acquisition of PICU-specific knowledge and practice of skills such as intubation of difficult airways. Secondly, studies have evaluated and demonstrated that with appropriate use, VR appears to be a safe and feasible intervention to decrease pain and anxiety in PICU patients.

Parents' Experiences of Having a Child with Down Syndrome and Sleep Difficulties.

OBJECTIVES: Sleep disorders are prevalent in children with Down Syndrome (DS). However, sleep treatment is not always readily accessed by this group. This study aims to understand families' experiences of having a child with DS and sleep difficulties, and in particular, their healthcare experiences, with the goal of informing practice improvements. METHODS: We conducted semi-structured interviews with 34 parents (fathers n = 4 and mothers n = 30) with open-ended questions about parents' experiences of sleep, family dynamics, and healthcare. We operationalized a reflexive Thematic Analysis. RESULTS: Parents normalized their experiences of having a child with DS and sleep problems. Parents acknowledged that sleep disruption has adverse and pervasive impacts on their wellbeing and family dynamics, but also found this difficult to identify as a health problem. They accepted sleep difficulties as a regular part of bringing up any child, particularly one with a disability. When they did seek treatment for their child's sleep difficulties, parents often reported encountering insensitive and inadequate care and described that, at times, healthcare professionals also normalized children's sleep difficulties, resulting in sub-optimal treatment. This included at times failure to refer to tertiary sleep medicine services when required. CONCLUSIONS: Parents' and healthcare professionals' normalization of sleeping difficulties denies that they are both deleterious and modifiable. Practice implications include raising healthcare professionals' awareness of the importance of proactively addressing sleep, with sensitivity to families' normalization strategies, recognizing that families may require prompting to report concerns.

Ventilatory support at home for children: A joint position paper from the Thoracic Society of Australia and New Zealand/Australasian Sleep Association.

The goal of this position paper on ventilatory support at home for children is to provide expert consensus from Australia and New Zealand on optimal care for children requiring ventilatory support at home, both non-invasive and invasive. It was compiled by members of the Thoracic Society of Australia and New Zealand (TSANZ) and the Australasian Sleep Association (ASA). This document provides recommendations to support the development of improved services for Australian and New Zealand children who require long-term ventilatory support. Issues relevant to providers of equipment and areas of research need are highlighted.

Cognitive parameters in children with mild obstructive sleep disordered breathing.

PURPOSE: Sleep disordered breathing (SDB) in children is commonly described as a continuum from primary snoring (PS) to obstructive sleep apnea (OSA), based on apnea indices from polysomnography (PSG). This study evaluated the difference in neurocognitive and behavioral parameters, prior to treatment, in symptomatic pre-school children with PSG-diagnosed OSA and PS. METHODS: All children had positive Pediatric Sleep Questionnaire (PSQ) results and were deemed suitable for adenotonsillectomy by an ENT surgeon. Neurocognitive and behavioral data were analyzed in pre-school children at recruitment for the POSTA study (The Pre-School OSA Tonsillectomy Adenoidectomy Study). Data were compared between PS and OSA groups, with Obstructive Apnea-Hypopnea Index, OAHI < 1/h or 1-10/h, respectively. RESULTS: Ninety-one children were enrolled, including 52 with OSA and 39 with PS. Distribution of IQ (using Brief Intellectual Ability, BIA) was slightly skewed towards higher values compared with the reference population. No significant differences were found in neurocognitive or behavioral parameters for children with OSA versus those with PS. DISCUSSION: Neurocognitive and behavioral parameters were similar in pre-school children symptomatic for OSA, regardless of whether or not PSG diagnosed PS or OSA. Despite having identical symptoms, children with PS on PSG are often treated conservatively, whereas those with OSA on PSG are considered for adenotonsillectomy. This study demonstrates that, regardless of whether or not PS or OSA is diagnosed on PSG, symptoms, neurocognition, and behavior are identical in these groups. We conclude that symptoms and behavioral disturbances should be considered in addition to OAHI when determining the need for treatment. TRIAL REGISTRATION: Australian and New Zealand Clinical Trials registration number ACTRN12611000021976.

Using continuous overnight pulse oximetry to guide home oxygen therapy in chronic neonatal lung disease.

AIM: The aims of this study are: (i) to survey the knowledge of paediatric clinicians using overnight continuous pulse oximetry data to guide management of infants with chronic neonatal lung disease (CNLD); (ii) to assess the ability of paediatric clinicians to interpret overnight continuous pulse oximetry data; and (iii) to describe the overnight oximetry interpretation practices of paediatric respiratory specialists. METHODS: Paediatric clinicians from three tertiary teaching hospitals completed an anonymous survey regarding overnight continuous pulse oximetry in chronic neonatal lung disease. Using a modified Delphi technique, paediatric respiratory specialists participated in a concordance exercise and discussions to establish consensus interpretations for 25 oximetry studies. Paediatric clinicians were invited to complete the same exercise as a comparison. RESULTS: Self-rated knowledge from 74 surveyed clinicians was proportional to clinical experience. Twenty paediatric clinicians and nine paediatric respiratory specialists completed the oximetry exercise with scores of 64% (κ = 0.25) and 80% (κ = 0.45), respectively. Individual parameters like a mean peripheral arterial haemoglobin saturation (SpO2 ) below 93% and percentage time spent below SpO2 93% correlated poorly with the consensus interpretations. Paediatric respiratory specialists instead relied on visual analysis of SpO2 waveforms, utilising the frequency and depth of desaturations to guide management. CONCLUSION: Interpretation of overnight oximetry data is variable amongst both paediatric clinicians and respiratory specialists. This likely reflects inadequate evidence defining clinically significant intermittent hypoxaemia, whether in terms of desaturation duration, frequency or nadir.

Rationale for and design of the "POSTA" study: Evaluation of neurocognitive outcomes after immediate adenotonsillectomy compared to watchful waiting in preschool children.

BACKGROUND: IQ deficits are linked to even mild obstructive sleep apnoea (OSA) in children. Although OSA is commonly first diagnosed in the pre-school age group, a randomised trial is still needed to assess IQ outcomes after adenotonsillectomy in the pre-school age-group. This randomised control trial (RCT) will primarily determine whether adenotonsillectomy improves IQ compared to no adenotonsillectomy after 12 months, in preschool (3-5 year-old) children with mild to moderate OSA. METHODS: This protocol is for an ongoing multi-centred RCT with a recruitment target of 210 subjects (105 in each arm). Children age 3-5 years with symptoms of OSA, are recruited through doctor referral, at the point of referral to the Ear Nose and Throat (ENT) services. Screening is initially with a questionnaire (Paediatric Sleep Questionnaire, PSQ) for symptoms of obstructive sleep apnoea (OSA). Where questionnaires are positive (suggestive of OSA) and ENT surgeons recommend them for adenotonsillectomy, they are invited to participate in POSTA. Baseline testing includes neurocognitive testing (IQ and psychometric evaluation with the neuropsychologist blinded to randomisation) and overnight polysomnography (PSG). Where the Obstructive Apnoea-Hypopnea Index (OAHI) from the PSG is <10/h per hour, consent for randomisation is sought; children with severe OSA (OAHI ≥ 10/h) are sent for immediate treatment and excluded from the study. After consent is obtained, participants are randomised to early surgery (within 2 months) or to surgery after a usual wait time of 12 months. Follow-up studies include repeat neurocognitive testing and PSG at 12 (with the waiting list group studied before their surgery) and 24 months after randomisation. Analysis will be by intention to treat. The primary outcome is IQ at 12 months' follow-up. DISCUSSION: If IQ deficits associated with OSA are reversible 12 months after adenotonsillectomy compared to controls, future clinical practice advise would be to undertake early surgery in young children with OSA. The study could provide data on whether a window of opportunity exists for reversing IQ deficits linked to OSA in the pre-school age-group. TRIAL REGISTRATION: Australian and New Zealand Clinical Trials Registration Number ACTRN12611000021976 .

Interstitial lung disease in infancy: A general approach.

Childhood Interstitial lung disease (chILD) is an umbrella term used to define a broad range of rare, diffuse pulmonary disorders with altered interstitial structure that leads to abnormal gas exchange. Presentation of chILD in infancy can be difficult to differentiate from other common causes of diffuse lung disease. This article aimed at paediatricians provides an overview of interstitial lung disease presenting in infancy and includes key clinical features, a suggested approach to investigation and a summary of management. An overview of three clinical cases has been included to demonstrate the diagnostic approach, characteristic investigation findings and varied clinical outcomes.

Snoring in children.

Chronic snoring (≥4 nights per week) is not benign. Otherwise healthy children with chronic snoring and evidence of adenotonsillar hypertrophy can be referred directly for adenotonsillectomy. Snoring children <30 months or with significant medical comorbidities should be referred for specialist sleep evaluation. Older children with intermittent snoring or without significant medical comorbidities can be managed with a combination of medical and surgical interventions listed herein.

Monitoring of physiologic features and treatment aspects of children on home invasive mechanical ventilation.

Pediatric home invasive mechanical ventilation patients are a small but resource-intensive cohort, requiring close monitoring and multidisciplinary care. Patients are often dependent on their ventilator for life support, with any significant complications such as equipment failure, tracheostomy blockage, or accidental decannulation becoming potentially life-threatening if not identified quickly. This review discusses the indications and variations in practice worldwide, in terms of models of care, including home care provision, choice of equipment, and monitoring. With advances in technology, optimal monitoring strategies for home, continue to be debated: In-built ventilator alarms are often inadequately sensitive for pediatric patients, necessitating additional external monitoring devices to minimize risk. Pulse oximetry has been the preferred monitoring modality at home, though in some special circumstances such as congenital central hypoventilation syndrome, home carbon dioxide monitoring may be important to consider. Children should be under regular follow-up at specialist respiratory centers where clinical evaluation, nocturnal oximetry, and capnography monitoring and/or poly(somno)graphy and analysis of ventilator download data can be performed regularly to monitor progress. Recent exciting advances in technology, particularly in telemonitoring, which have potential to hugely benefit this complex group of patients are also discussed.

Optimising sleep in adolescents: The challenges.

BACKGROUND: Adolescence is a stage of significant transition as children develop into young adults. Optimal sleep is crucial during this period to ensure physical, emotional and mental wellbeing. However, it is well recognised that insufficient quality and quantity of sleep is common among adolescents worldwide. OBJECTIVE: This article aims to provide general practitioners with an overview of the key issues encountered in adolescent patients relating to sleep and summarises approaches to assessment and evidence-based management of sleep problems in this population. DISCUSSION: This review highlights the physiological changes that affect sleep during adolescence and how other factors, including unhealthy sleep behaviours, influence these. It discusses the importance of healthy sleep and the consequences of sleep disturbance in adolescents. Management strategies are outlined, focusing on the key common issues that affect sleep in the teenage years, and guidance on when to consider co-management with specialist care is provided.

An approach to common sleep presentations in infants and toddlers.

BACKGROUND: Healthy sleep is vital for optimal child development, yet over 30% of Australian parents report having children with disrupted sleep affecting all family members. These sleep difficulties might co-exist with sleep breathing disorders, contributing to morbidity and reduced quality of life. OBJECTIVE: This article aims to provide general practitioners (GPs) with an evidence-based, biopsychosocial approach to managing common sleep problems in infants and preschool-aged children. DISCUSSION: Strategies and techniques are outlined to aid GPs in promoting healthy sleep during infancy, educating parents on typical sleep patterns and supporting families in managing problematic sleep patterns in toddlers. Emphasis is placed on a tailored approach to developing a healthy sleep environment to meet the child's needs and parental values. Valuable resources and indications for specialist consultation are included.

Real-world utility of overnight oximetry for the screening of obstructive sleep apnea in children.

BACKGROUND: Obstructive sleep apnea (OSA) is a common problem in children and can result in developmental and cognitive complications if untreated. The gold-standard tool for diagnosis is polysomnography (PSG); however, it is an expensive and time-consuming test to undertake. Overnight oximetry has been suggested as a faster and cheaper initial test in comparison to PSG as it can be performed at home using limited, reusable equipment. AIM: This retrospective case control study aims to evaluate the effectiveness of a home oximetry service (implemented in response to extended waiting times for routine PSG) in reducing the time between patient referral and treatment. METHODS: Patients undergoing diagnostic sleep evaluation for suspected OSA who utilized the Queensland Children's Hospital screening home oximetry service in the first year since its inception in 2021 (n = 163) were compared to a historical group of patients who underwent PSG in 2018 (n = 311). Parameters compared between the two groups included time from sleep physician review to sleep test, ENT review, and definitive treatment in the form of adenotonsillectomy surgery (or CPAP initiation for those who had already undergone surgery). RESULTS: The time from sleep physician review and request of the sleep-related study to ENT surgical treatment was significantly reduced (187 days for the HITH oximetry group vs 359 days for the comparable PSG group; p-value <0.05), and time from sleep study request to the report of results was significantly lower for patients in the oximetry group compared to those in the PSG group (11 days vs 105 days; p-value <0.05). CONCLUSION: These results suggest that for children referred to a tertiary sleep center for possible obstructive sleep disordered breathing, a home oximetry service can be effective in assisting sleep evaluation and reducing the time to OSA treatment.

A growth chart of brain function from infancy to adolescence based on EEG.

BACKGROUND: In children, objective, quantitative tools that determine functional neurodevelopment are scarce and rarely scalable for clinical use. Direct recordings of cortical activity using routinely acquired electroencephalography (EEG) offer reliable measures of brain function. METHODS: We developed and validated a measure of functional brain age (FBA) using a residual neural network-based interpretation of the paediatric EEG. In this cross-sectional study, we included 1056 children with typical development ranging in age from 1 month to 18 years. We analysed a 10- to 15-min segment of 18-channel EEG recorded during light sleep (N1 and N2 states). FINDINGS: The FBA had a weighted mean absolute error (wMAE) of 0.85 years (95% CI: 0.69-1.02; n = 1056). A two-channel version of the FBA had a wMAE of 1.51 years (95% CI: 1.30-1.73; n = 1056) and was validated on an independent set of EEG recordings (wMAE = 2.27 years, 95% CI: 1.90-2.65; n = 723). Group-level maturational delays were also detected in a small cohort of children with Trisomy 21 (Cohen's d = 0.36, p = 0.028). INTERPRETATION: A FBA, based on EEG, is an accurate, practical and scalable automated tool to track brain function maturation throughout childhood with accuracy comparable to widely used physical growth charts. FUNDING: This research was supported by the National Health and Medical Research Council, Australia, Helsinki University Diagnostic Center Research Funds, Finnish Academy, Finnish Paediatric Foundation, and Sigrid Juselius Foundation.

The management of residual OSA post-adenotonsillectomy in children with down syndrome: The experience of a large tertiary sleep service.

INTRODUCTION: Obstructive Sleep Apnoea (OSA) is common in children with Down Syndrome (DS). Adenotonsillectomy is regarded as first line treatment for OSA but does not always lead to resolution of symptoms in this group of children. Management of residual OSA is variable with no existing recommendations to guide clinical practice. AIM: To describe the experience of a large tertiary sleep service in managing residual OSA in children with DS following upper airway surgery (adenotonsillectomy, adenoidectomy or tonsillectomy). METHODS: A retrospective study of children who were under evaluation at the Queensland Children's Hospital sleep medicine department between October 2013 to April 2022 for residual OSA, after upper airway surgery was undertaken. RESULTS: 148 children with DS who underwent polysomnography for evaluation of OSA were identified. 100 were included in this study and of these, 77 underwent adenotonsillectomy, 19 adenoidectomy, and 4 tonsillectomy. Post-surgical PSG data of all 100 children showed residual mixed sleep disordered breathing in 68 children. 41 were recommended CPAP following surgery, while 21 underwent further surgery. CONCLUSIONS: Residual OSA was confirmed to be highly prevalent in children with DS who had already undergone upper airway surgery for OSA. This study identified that CPAP is possible to establish in most children with DS and can be used to manage residual OSA in this population. Approximately one-third of this group were able to discontinue therapy at a median duration of 18 months, suggesting resolution of disease with time can occur in some children.

Communicating the complex lives of families that include a child with Down syndrome.

Families of children with Down syndrome experience complex lives and needs, yet the few existing studies on these families are written in conventional academic prose that is not optimal for knowledge translation beyond academia, particularly for busy healthcare professionals. In this paper, we Depart Radically in Academic Writing (DRAW) (Mackinlay, 2022) and present data poetry and two case studies that draw upon semi-structured interviews with mothers, fathers, and siblings, who were interviewed separately about their experiences of having a child/sibling with Down syndrome. We introduce our interdisciplinary team that includes academics and clinicians to contextualise our focus on research translation. We demonstrate that writing with creative criticality (i.e. 'DRAWing') contributes an embodied and affective understanding of research participants' stories, which is largely lacking in the academic literature on families of children with Down syndrome and the sociology of health and illness field more broadly. Moreover, DRAWing can impact audiences emotionally as well as intellectually (Richardson, 2003, p. 924), which has important knowledge translation implications for both healthcare professionals and these families. DRAWing can capture healthcare professionals' attention, prompting them to critically reflect on their practices and opportunities for improving care and treatment for these families.

Use of extended reality in sleep health, medicine, and research: a scoping review.

STUDY OBJECTIVES: This scoping review explores the use of extended reality (virtual, augmented, and mixed reality) within sleep health, sleep medicine, and sleep research. It aims to provide insight into current uses and implementation considerations whilst highlighting directions for future research. METHODS: A systematic scoping review was undertaken informed by the preferred reporting items for systematic reviews and meta-analyses for scoping reviews and Johanna Briggs Institute. RESULTS: The use of virtual reality (VR) as a research tool in the investigation of areas such as dreaming and memory reactivation is growing. Thirty-one articles were identified in total with 20 utilizing VR to improve sleep as a clinical intervention. CONCLUSIONS: Research exploring the utility of VR as a clinical intervention in various patient populations and clinical settings is therefore warranted. Researchers and clinicians should ensure that extended reality interventions are developed based on clinical reasoning and informed by evidence of both sleep medicine and the effects of virtual and augmented reality. Where possible future research should utilize up-to-date technology and reporting frameworks to assist in the translation of research into clinical practice.

Tolerance of polysomnography in children with neurodevelopmental disorders compared to neurotypical peers.

STUDY OBJECTIVES: Diagnostic polysomnography (PSG) is the gold standard test to evaluate sleep-disordered breathing in children. Little is known about how children with neurodevelopmental disorders (NDD) tolerate electrodes and sensors in PSG compared to neurotypical children. METHODS: In this retrospective cohort study of children > 12 months of age who underwent diagnostic PSG at our center from 01/01/2021-30/06/2021, we used sleep technician and physician reports to determine how PSG was tolerated in children with NDD compared to neurotypical children. Subanalyses included tolerance of individual electrodes and sensors and subgroups of NDD (eg, Trisomy 21). RESULTS: A total of 132 children with a NDD and 139 neurotypical children underwent diagnostic PSG. The median age of all children was 8 years, 39% were female, and 50% had a sleep disorder identified on PSG, with no significant differences between NDD and neurotypical groups. The most poorly tolerated sensors for all children were the nasal prongs (poorly tolerated in 30% of all children), followed by thermistor (14%) and electroencephalography electrodes (6%). Children with NDD were > 3 times more likely (odds ratio 3.1, 95% confidence interval 1.8-5.3) to experience problems tolerating any study leads than neurotypical children. Subgroup analysis revealed children with Trisomy 21 had the greatest difficulty tolerating PSG set-up and leads. CONCLUSIONS: This retrospective study demonstrates that children with neurodevelopmental disorders are less likely to tolerate PSG monitoring than neurotypical children and highlights the need to develop alternative measures for evaluation of sleep disorders in this population. CITATION: Lanzlinger D, Kevat A, Collaro A, Poh SH, Pérez WP, Chawla J. Tolerance of polysomnography in children with neurodevelopmental disorders compared to neurotypical peers. J Clin Sleep Med. 2023;19(9):1625-1631.