Facial Function in Bell Palsy in a Cohort of Children Randomized to Prednisolone or Placebo 12 Months After Diagnosis.

BACKGROUND: Information on the medium-term recovery of children with Bell palsy or acute idiopathic lower motor neuron facial paralysis is limited. METHODS: We followed up children aged 6 months to <18 years with Bell palsy for 12 months after completion of a randomized trial on the use of prednisolone. We assessed facial function using the clinician-administered House-Brackmann scale and the modified parent-administered House-Brackmann scale. RESULTS: One hundred eighty-seven children were randomized to prednisolone (n = 93) or placebo (n = 94). At six months, the proportion of patients who had recovered facial function based on the clinician-administered House-Brackmann scale was 98% (n = 78 of 80) in the prednisolone group and 93% (n = 76 of 82) in the placebo group. The proportion of patients who had recovered facial function based on the modified parent-administered House-Brackmann scale was 94% (n = 75 of 80) vs 89% (n = 72 of 81) at six months (OR 1.88; 95% CI 0.60, 5.86) and 96% (n = 75 of 78) vs 92% (n = 73 of 79) at 12 months (OR 3.12; 95% CI 0.61, 15.98). CONCLUSIONS: Although the vast majority had complete recovery of facial function at six months, there were some children without full recovery of facial function at 12 months, regardless of prednisolone use.

Pain in children with Bell's palsy: secondary analysis of a randomised controlled trial.

OBJECTIVE: To describe the prevalence and severity of pain experienced by children with Bell's palsy over the first 6 months of illness and its association with the severity of facial paralysis. METHODS: This was a secondary analysis of data obtained in a phase III, triple-blinded, randomised, placebo-controlled trial of prednisolone for the treatment of Bell's palsy in children aged 6 months to <18 years conducted between 13 October 2015 and 23 August 2020 in Australia and New Zealand. Children were recruited within 72 hours of symptom onset and pain was assessed using a child-rated visual analogue scale (VAS), a child-rated Faces Pain Score-Revised (FPS-R) and/or a parent-rated VAS at baseline, and at 1, 3 and 6 months until recovered, and are reported combined across treatment groups. RESULTS: Data were available for 169 of the 187 children randomised from at least one study time point. Overall, 37% (62/169) of children reported any pain at least at one time point. The frequency of any pain reported using the child-rated VAS, child-rated FPS-R and parent-rated VAS was higher at the baseline assessment (30%, 23% and 27%, respectively) compared with 1-month (4%, 0% and 4%, respectively) and subsequent follow-up assessments. At all time points, the median pain score on all three scales was 0 (no pain). CONCLUSIONS: Pain in children with Bell's palsy was infrequent and primarily occurred early in the disease course and in more severe disease. The intensity of pain, if it occurs, is very low throughout the clinical course of disease. TRIAL REGISTRATION NUMBER: ACTRN12615000563561.

Evaluation of mild periorbital cellulitis and home-based therapy in children-EPOCH study: A prospective single centre cohort study.

BACKGROUND: Paediatric periorbital cellulitis is a common eye condition and warrants prompt management for the prevention of complications. International consensus on the approach to optimal management of children with mild periorbital cellulitis including ambulatory management is lacking. We aimed to prospectively investigate the safety and effectiveness of ambulatory management of children with mild periorbital cellulitis. METHODS: Over a 23-month period, we prospectively enrolled 70 children aged between 2 and 16 years who presented to the emergency department with mild periorbital cellulitis. Demographic and clinical data were collected. Eligible children were commenced on oral antibiotics and were discharged home with close outpatient ambulatory care and ophthalmology follow up. We used descriptive statistics for data presentation. RESULTS: Of the 70 children with mild periorbital cellulitis, 30 (43%) had unknown aetiology. Sixty-five (92%) children received a structured ambulatory follow up. Five children (7%) received inpatient parenteral antibiotics for worsening of local symptoms within 24 h of initial presentation. One child developed orbital cellulitis at follow up. There was no mortality or significant morbidity noted in this group and none of the children needed surgical intervention. CONCLUSIONS: Ambulatory care for children with mild periorbital cellulitis is an effective and safe management strategy. This might prevent hospitalisation, reduce the burden on hospital bed occupancy and promote patient care in the community.

Agreement Between House-Brackmann and Sunnybrook Facial Nerve Grading Systems in Bell's Palsy in Children: Secondary Analysis of a Randomized, Placebo-Controlled Multicenter Trial.

BACKGROUND: There is limited evidence on the use of facial nerve function grading scales in acute facial nerve paralysis in children. OBJECTIVE: To investigate the agreement between and the usability of the House-Brackmann and Sunnybrook scales in children with idiopathic facial paralysis (Bell's palsy) and to compare their ease of administration. METHODS: Data from a randomized controlled trial in children aged 6 months to <18 years with Bell's palsy was used. Children were recruited within 72 hours of symptom onset and assessed using the House-Brackmann and the Sunnybrook scales at baseline and at 1, 3, and 6 months until recovered. Agreement between the scales was assessed using the intraclass correlation coefficient (ICC) at each time point and using a Bland-Altman plot. Ease of administration was assessed using an 11-point Likert scale. RESULTS: Comparative data were available for 169 of the 187 children randomized. The ICC between the 2 scales across all time points was 0.92 (95% confidence interval [CI] 0.91-0.93), at baseline 0.37 (95% 0.25, 0.51), at 1 month 0.91 (95% CI 0.89-0.94), at 3 months 0.85 (95% CI 0.80-0.89), and at 6 months 0.96 (95% CI 0.95-0.97). The median score for the ease of administration for the House-Brackmann and Sunnybrook scales was 3 (interquartile range [IQR]: 1-5) and 7 (IQR: 4-8) respectively (P < .001, Wilcoxon signed-rank test). CONCLUSIONS: There was excellent agreement between House-Brackmann and Sunnybrook scales, with poorer agreement at baseline. Clinicians found the House-Brackmann scale easier to administer. These findings suggest that both scales can be applied in children.

Efficacy of Prednisolone for Bell Palsy in Children: A Randomized, Double-Blind, Placebo-Controlled, Multicenter Trial.

BACKGROUND AND OBJECTIVE: Corticosteroids are used to treat the early stages of idiopathic facial paralysis (Bell's palsy) in children, but their effectiveness is uncertain. We set out to determine if prednisolone improves the proportion of children with Bell's palsy with complete recovery at one month. METHODS: We conducted a double-blind, placebo-controlled, randomised trial of prednisolone in children presenting to emergency departments with Bell's palsy. Patients aged 6 months to less than 18 years, recruited within 72 hours after symptom onset, were randomly assigned to receive 10 days of treatment with oral prednisolone (approximately 1 mg/kg) or placebo. The primary outcome was complete recovery of facial function at 1 month rated on the House-Brackmann scale. Secondary outcomes included facial function, adverse events and pain up to 6 months. Target recruitment was n=540 (270 per group). RESULTS: Between 13 October 2015 to 23 August 2020, 187 children were randomised (94 to prednisolone and 93 to placebo) and included in the intention-to-treat analysis. At 1 month, the proportions of patients who had recovered facial function were 49% (n=43/87) in the prednisolone group compared with 57% (n=50/87) in the placebo group (risk difference -8.1%, 95% CI -22.8 to 6.7; adjusted odds ratio [aOR] 0.7, 95% CI 0.4 to 1.3). At 3 months these proportion were 90% (n=71/79) for the prednisolone group versus 85% (n=72/85) for the placebo group (risk difference 5.2%, 95%, CI -5.0 to 15.3; aOR 1.2, 95% CI 0.4 to 3.0) and at 6 months 99% (n=77/78) and 93% (n=76/82) respectively (risk difference 6.0%, 95% CI -0.1 to 12.2; aOR 3.0 95% CI 0.5 to 17.7) There were no serious adverse events and little evidence for group differences in secondary outcomes. DISCUSSION: In children with Bell's palsy the vast majority recover without treatment. The study, although underpowered, does not provide evidence that early treatment with prednisolone improves complete recovery. REGISTRATION: Registered with the Australian New Zealand Clinical Trials Registry ACTRN12615000563561, registered 1 June 2015, ://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=368505&isReview=true CLASSIFICATION OF EVIDENCE: This study provides Class I evidence that for children with Bell's palsy, prednisolone does not significantly change recovery of complete facial function at one month. However, the study lacked the precision to exclude an important harm or benefit from prednisolone.

Treatment patterns and frequency of key outcomes in acute severe asthma in children: a Paediatric Research in Emergency Departments International Collaborative (PREDICT) multicentre cohort study.

RATIONALE: Severe acute paediatric asthma may require treatment escalation beyond systemic corticosteroids, inhaled bronchodilators and low-flow oxygen. Current large asthma datasets report parenteral therapy only. OBJECTIVES: To identify the use and type of escalation of treatment in children presenting to hospital with acute severe asthma. METHODS: Retrospective cohort study of children with an emergency department diagnosis of asthma or wheeze at 18 Australian and New Zealand hospitals. The main outcomes were use and type of escalation treatment (defined as any of intensive care unit admission, nebulised magnesium, respiratory support or parenteral bronchodilator treatment) and hospital length of stay (LOS). MEASUREMENTS AND MAIN RESULTS: Of 14 029 children (median age 3 (IQR 1-3) years; 62.9% male), 1020 (7.3%, 95% CI 6.9% to 7.7%) had treatment escalation. Children with treatment escalation had a longer LOS (44.2 hours, IQR 27.3-63.2 hours) than children without escalation 6.7 hours, IQR 3.5-16.3 hours; p<0.001). The most common treatment escalations were respiratory support alone (400; 2.9%, 95% CI 2.6% to 3.1%), parenteral bronchodilator treatment alone (380; 2.7%, 95% CI 2.5% to 3.0%) and both respiratory support and parenteral bronchodilator treatment (209; 1.5%, 95% CI 1.3% to 1.7%). Respiratory support was predominantly nasal high-flow therapy (99.0%). The most common intravenous medication regimens were: magnesium alone (50.4%), magnesium and aminophylline (24.6%) and magnesium and salbutamol (10.0%). CONCLUSIONS: Overall, 7.3% children with acute severe asthma received some form of escalated treatment, with 4.2% receiving parenteral bronchodilators and 4.3% respiratory support. There is wide variation treatment escalation.

Assessing the adherence to guidelines in the management of croup in Australian children: a population-based sample survey.

OBJECTIVE: To determine the extent to which care received by Australian children presenting with croup is in agreement with Clinical Practice Guidelines (CPGs). DESIGN: Retrospective population-based sample survey. Croup clinical indicators were derived from CPGs. DATA SOURCES/STUDY SETTING: Medical records from three healthcare settings were sampled for selected visits in 2012 and 2013 in three Australian states. DATA COLLECTION: Data were collected by nine experienced paediatric nurses, trained to assess eligibility for indicator assessment and adherence to CPGs. Surveyors undertook criterion-based medical record reviews using an electronic data collection tool. RESULTS: Documented guideline adherence was lower for general practitioners (65.9%; 95% CI: 60.8-70.6) than emergency departments (91.1%; 95% CI: 89.5-92.5) and inpatient admissions (91.3%; 95% CI: 88.1-93.9). Overall adherence was very low for a bundle of 10 indicators related to assessment (4.5%; 95% CI: 2.4-7.6) but higher for a bundle of four indicators relating to the avoidance of inappropriate therapy (83.1%; 95% CI: 59.5-96.0). CONCLUSIONS: Most visits for croup were characterized by appropriate treatment in all healthcare settings. However, most children had limited documented clinical assessments, and some had unnecessary tests or inappropriate therapy, which has potential quality and cost implications. Universal CPG and clinical assessment tools may increase clinical consistency.

Out-of-hospital arrests attending an Australian tertiary paediatric emergency department over 13 years: An observational study.

OBJECTIVE: In paediatric cardiopulmonary arrest, International Liaison Committee on Resuscitation (ILCOR) states, 'there are no simple guidelines to determine when resuscitative efforts become futile'. Considerations to assist this decision-making include cause of arrest, pre-existing medical conditions, age, site of arrest, duration of untreated cardiopulmonary arrest, witnessed arrest and presence of shockable rhythm. Outcomes are poor in out-of-hospital cardiac arrests (OHCA), particularly for infants. This single-centre observational study describes the characteristics and outcomes of the subgroup of children presenting to our hospital's ED following OHCA still receiving cardiac compressions, to assist development of guidelines for future resuscitation efforts in our ED, particularly for cessation of cardiopulmonary resuscitation (CPR). METHODS: The ED database was searched for children presenting in cardiopulmonary arrest receiving cardiac compressions. Data were reviewed on pre-hospital, ED and hospital management and outcome, particularly looking at considerations outlined by ILCOR. RESULTS: From January 2000 to December 2013, 60 children were identified: median age 1.71 years; 87% arresting at home; 68% with bystander CPR; median CPR duration pre-hospital 42 min, and in ED 19.5 min; total CPR median 61 min. Fifty patients (83%) died in ED, 10 (17%) were admitted to intensive care but all died within 4 days. CONCLUSION: Children presenting to ED still receiving cardiac compressions following OHCA had a universally poor outcome, regardless of age and underlying cause. This implies resuscitative efforts could be discontinued earlier in this subgroup. A national, multicentre study is needed to determine if this finding is reproducible with a larger population.

Bell's Palsy in Children (BellPIC): protocol for a multicentre, placebo-controlled randomized trial.

BACKGROUND: Bell's palsy or acute idiopathic lower motor neurone facial paralysis is characterized by sudden onset paralysis or weakness of the muscles to one side of the face controlled by the facial nerve. While there is high level evidence in adults demonstrating an improvement in the rate of complete recovery of facial nerve function when treated with steroids compared with placebo, similar high level studies on the use of steroids in Bell's palsy in children are not available. The aim of this study is to assess the utility of steroids in Bell's palsy in children in a randomised placebo-controlled trial. METHODS/DESIGN: We are conducting a randomised, triple-blinded, placebo controlled trial of the use of prednisolone to improve recovery from Bell's palsy at 1 month. Study sites are 10 hospitals within the Australian and New Zealand PREDICT (Paediatric Research in Emergency Departments International Collaborative) research network. 540 participants will be enrolled. To be eligible patients need to be aged 6 months to < 18 years and present within 72 hours of onset of clinician diagnosed Bell's palsy to one of the participating hospital emergency departments. Patients will be excluded in case of current use of or contraindications to steroids or if there is an alternative diagnosis. Participants will receive either prednisolone 1 mg/kg/day to a maximum of 50 mg/day or taste matched placebo for 10 days. The primary outcome is complete recovery by House-Brackmann scale at 1 month. Secondary outcomes include assessment of recovery using the Sunnybrook scale, the emotional and functional wellbeing of the participants using the Pediatric Quality of Life Inventory and Child Health Utility 9D Scale, pain using Faces Pain Scale Revised or visual analogue scales, synkinesis using a synkinesis assessment questionnaire and health utilisation costs at 1, 3 and 6 months. Participants will be tracked to 12 months if not recovered earlier. Data analysis will be by intention to treat with primary outcome presented as differences in proportions and an odds ratio adjusted for site and age. DISCUSSION: This large multicenter randomised trial will allow the definitive assessment of the efficacy of prednisolone compared with placebo in the treatment of Bell's palsy in children. TRIAL REGISTRATION: The study is registered with the Australian New Zealand Clinical Trials Registry ACTRN12615000563561 (1 June 2015).

An innocuous duplication of 11.2 Mb at 13q21 is gene poor: sub-bands of gene paucity and pervasive CNV characterize the chromosome anomalies.

A boy with autistic spectrum disorder without dysmorphisms was found to have a chromosome duplication of part of band 13q21. His mother and grandfather both of normal intellect had the same chromosomal duplication. Comparison was made with the Chromosome anomaly database www.som.soton.ac.uk/research/geneticsdiv/anomaly%20register which revealed similar cases. Mapping on DNA microarray for the proband and mother showed the duplication to be of length 11.2 Mb, encompassing the 13q21.1-13q21.32 region. The duplicated region is profoundly gene poor, with a mean gene density of 0.45 genes/Mb. We estimate, that the mean gene density in the sub-bands of the chromosome anomalies is 2.4-2.5 genes/Mb. In addition the percentages of the sub-bands reported as copy number variants (CNV) was estimated from the Database of Chromosome Genomic Variants (http://projects.tcag.ca/variation/). It was found that for some of these sub-bands, gene paucity was likely to be a major contributor to their innocuous phenotypic effect, for example, the gene densities were for: 1p31.2 (1.25 genes/Mb); 2p12 (1.7); 4p15.31 (1.3); 5p14.1 (0.22); 5p14.3 (0.8); 5q21.2 (0.6); 5q21.3 (1.2); 8p23.2 (0.25); 13q21.1 (0.9); 14q31.1 (1.4); 18q22.1 (1.4); 21q21.1 (1.2); and 21q21.2 (0.7). For other sub-bands the percentage of the band in which CNV have been reported was found to be markedly increased, for example, 8p23.2 (94.7% of the band is defined by reported CNV); 3p26.3 (81.6); 5p14.3 (59.3); 8p22 (48.8); 2p12 (44.0); 5q21.1 (43.6); 6q24.2 (41.4); 9p23 (38.8); 10q21.1 (36.5); 5q21.2 (35.4), and 11q14.3 (33.8). We argue that both gene paucity and pervasive CNV are major indicators of bands conforming to the Chromosome Anomaly phenomenon.