Patterns of Change in the Severity of Airway Obstruction with Robin Sequence in Early Infancy.

Previous studies suggest that infants with Robin sequence show a pattern of steady improvement in the severity of airway obstruction, and of their treatment requirements, during infancy. Methods: Three infants with Robin sequence and severe obstructive sleep apnea were managed with nasal continuous positive airways pressure (CPAP). Multiple measures of airway obstruction were made during infancy, including CPAP pressure evaluations and sleep studies (screening and polysomnography studies). Parameters reported include obstructive apnea-hypopnea index, oxygen desaturation parameters, and CPAP pressures required for effective airway management. Results: CPAP pressure requirements increased in all three infants during their first weeks of life. Apnea indices on polysomnography did not track with the CPAP pressure requirements. Peak pressure requirements were at 5 and 7 weeks for two patients, with subsequent gradual decline and cessation of therapy CPAP at 39 and 74 weeks, respectively. The third patient had a complicated course, jaw distraction at 17 weeks, and biphasic CPAP pressure requirement (first peak at 3 weeks, but maximum pressure at 74 weeks), with cessation of CPAP at 75 weeks. Conclusions: The observed pattern of early increases in CPAP pressure requirements for infants with Robin sequence adds to the complexities of managing this disorder. Factors that may lead to this pattern of change in airway obstruction are discussed.

Polysomnography in infants with clinical suspicion of sleep-related breathing disorders.

STUDY OBJECTIVES: Limited data exist concerning the indications, parameters, utility of daytime polysomnography, and treatment of infants with suspected sleep-related breathing disorders. METHODS: We retrospectively reviewed all polysomnography undertaken in a quaternary pediatric hospital for term infants up to 6 months of age between January 2017 and December 2019. Outcomes were evaluated, including a comparison among diagnostic groups. RESULTS: Of 161 infants (58% male), 77 (48%) were ≤ 2 months old, and 103 (61%) were referred for either craniofacial abnormalities or an airway malformation. Daytime (n = 100) vs nighttime (n = 61) studies showed no differences in sleep architecture or treatment rates. Apnea-hypopnea index was > 10 events/h in 137 (85%) and was similar across different diagnostic groups, and 97 (78%) were prescribed noninvasive ventilation, with a mean treatment duration of 13.4 ± 9 months. Of the infants who were commenced on noninvasive ventilation 75% did not require it beyond 24 months. CONCLUSIONS: Polysomnographic sleep parameters and the number of treatments prescribed were equivalent whether the polysomnography was performed during daytime or nighttime. Treatment with noninvasive ventilation was required in the short term for most infants with sleep-related breathing disorders, regardless of the indication for referral. CITATION: Singh J, Yeoh E, Castro C, Uy C, Waters K. Polysomnography in infants with clinical suspicion of sleep-related breathing disorders. J Clin Sleep Med. 2022;18(12):2803-2812.

Cheyne-stokes respiration in children with heart failure.

Cheyne-Stokes respiration (CSA-CSR) is a form of central sleep apnea characterized by alternating periods of hyperventilation and central apneas or hypopneas. CSA-CSR develops following a cardiac insult resulting in a compensatory increase in sympathetic activity, which in susceptible patients causes hyperventilation and destabilizes respiratory control. The physiological changes that occur in CSA-CSR include hyperventilation, a reduced blood gas buffering capacity, and circulatory delay. In adults, 25% to 50% of patients with heart failure are reported to have CSA-CSR. The development of CSA-CSR in this group of patients is considered a poor prognostic sign. The prevalence, progression, and treatment outcomes of CSA-CSR in children remain unclear with only 11 children being described in the literature. The lack of data is possibly not due to the paucity of children with severe heart failure and CSA-CSR but because they may be under-recognized, compounded by the absence of routine polysomnographic assessment of children with moderate to severe heart failure. Building on much broader experience in the diagnosis and management of CSA-CSR in adult sleep medicine and our limited experience in a pediatric quaternary center, this paper will discuss the prevalence of CSA-CSR, its' treatment options, outcomes in children, and the potential future direction for research in this understudied area of pediatric sleep medicine.

Motivation for change in the health care of children with developmental disabilities: Pilot continuing professional development-quality improvement project.

AIM: People with developmental disabilities (DDs) experience significant barriers accessing and receiving optimal health care resulting in poorer health-care outcomes. Continuing professional development (CPD) represents an effective means to alter health-care staff behaviour to improve the care of people with DDs. However, given the scepticism regarding the effectiveness of certain CPD models' ability to alter learner's workplace behaviour, the current pilot study developed and determined the feasibility of a novel CPD programme aimed at improving the health care provided to children with DDs. METHODS: Motivation for Change (MFC) is a novel CPD programme based on empirically based behaviour and educational strategies including motivational interviewing, flipped classroom and process mapping. It utilises input of patients, practitioners, and family members during administration of the programme. MFC was administered with 14 staff members in a Sydney Children's Hospital Sleep service. RESULTS: After MFC engagement, staff reported significant improvements in their knowledge of behavioural characteristics of children with DDs, the difficulties they face, how best to support them within the learner's work setting and confidence in working with children with DDs. There was a non-significant decline in their reported need for further training and expressed high level of satisfaction with the MFC programme. CONCLUSION: MFC represents a feasible means of providing CPD to health-care staff but further research is needed to determine objective clinical behavioural change. Evaluation of the impact on patient health outcomes, parent/child satisfaction, staff sustainability and overall system functioning is also needed. It may represent an effective model of CPD for other targets of health-care improvement.

The spectrum of obstructive sleep apnea in infants and children with Down Syndrome.

OBJECTIVE: Describe the spectrum of OSA across time in infants and children with Down syndrome. METHODS: Retrospective records review of children who underwent formal polysomnography (PSG) in one of two Australian tertiary sleep centres over selected 3.5-year periods. 152 children were identified, then all sleep study and treatment records were retrieved for the lifetime of the child through 2018. RESULTS: 3.8 ±â€¯3.2 studies (range 1-17) were retrieved per child and 38.2% had mild disease at worst. Children having only 1 study were more likely to have a normal or mild result than those having ≥2 (chi-square 11.25, p-value 0.0008) Studies were more often severe in children age <2 compared to those ≥2 years, (chi-square 12.87, p = 0.005). After age 2 years, OSA severity increased with age. Amongst 91 (56.4%) children with ≥2 studies, 71 (78.0%) had moderate or severe disease at some time. Studies evaluating the effects of surgery (most often adenotonsillectomy) showed resolution of disease to mild or normal in 53.3%. Where ≥2 studies were evaluated, the last study polarised towards normal or mild disease 40 (44.0%), or treatment titrations 34 (37.4%) with moderate or severe disease in 17 (18.7%). CONCLUSIONS: In a tertiary sleep unit, a full spectrum of sleep disordered breathing in Down syndrome was seen from infancy onwards. Children having only one study were more likely to have normal results. Children with multiple studies reflected disease surveillance, including follow-up after treatment interventions.

Is day stay adenotonsillectomy safe in children with mild to moderate obstructive sleep apnoea? A retrospective review of 100 patients.

OBJECTIVE: This study explored the perioperative course of 100 children with polysomnogram (PSG) proven mild to moderate OSA to evaluate if day stay adenotonsillectomy is safe. METHODS: A retrospective chart review of patients who had undergone tonsillectomy with or without adenoidectomy following an overnight PSG at The Children's Hospital at Westmead Sleep Laboratory. 263 records were reviewed. Patients with apnoea hypopnea index (AHI) ≥ 1 and <15/h and/or a final sleep study report of mild to moderate OSA were included. Exclusion criteria were age <3 years, weight <10 kg, or any significant co-morbidities or other surgery that would preclude day stay surgery. Demographic, PSG and post-operative data was analyzed. RESULTS: No major respiratory complications occurred. No patient required an unplanned medical review for respiratory concerns, or admission to a high care facility. Eleven children left recovery with oxygen prescribed. One child had a desaturation to 88% in recovery, and one child had laryngospasm. The nine other children required oxygen to maintain saturation >90%. Supplemental oxygen was prescribed to 7 patients on the ward. Of these, three patients received supplemental oxygen beyond 6h. The other 97 patients had an uncomplicated post-operative course and would have been suitable for day-stay surgery. Increasing severity of OSA grade on pre-operative PSG was significantly associated with post-operative supplemental oxygen use (p=0.003; Cochrane-Armitage test for trend). CONCLUSIONS: Children who are otherwise well with mild to moderate OSA have a sufficiently low risk of respiratory complications following adenotonsillectomy to permit day-stay surgery in the setting of appropriate facilities with careful post-operative monitoring for the first 6h to identify a small sub-group who require overnight observations.

Correlations between polysomnographic and lateral airway radiograph measurements in paediatric obstructive sleep apnoea.

AIM: To evaluate the ability of lateral airway radiography (LAR) to assess adenoidal hypertrophy in children and correlate with the severity of obstructive sleep apnoea (OSA). METHODS: This cohort study was undertaken in 72 children who presented consecutively for evaluation of OSA to the outpatients of the Children's Hospital at Westmead. All children had LAR and overnight polysomnography (PSG). Five assessors, with varying experience, were blinded to the PSG results and independently analysed the LAR. Inter-rater reliability was determined for four published assessment methods; Hibbert, Johannesson, Fujioka and Cohen and Konak. We then compared the four LAR results with PSG-determined criteria for OSA. RESULTS: Using intraclass correlations, inter-rater correlations were moderate to high for all four standardised evaluations of LAR with values ranging from 0.51 to 0.96. With the radiologist taken as the 'gold standard', individual assessors ranged from 0.05 to 0.91. LAR correlated best with PSG determined obstructive apnoea hypopnea index and minimum oxygen saturation for the anterior airway measurement (Hibbert) with r-values of -0.25 and 0.25 respectively (P < 0.05). CONCLUSION: Amongst four methods of evaluating adenoid size, the anterior airway size correlated best with PSG variables of obstructive respiratory index and minimum oxygen saturation. However, the methods are not able to be used as a predictor for OSA.