Ds Urquhart

Six-year follow-up study on the effect of combined anterior and posterior spinal fusion on lung function and quality of life in young people with adolescent idiopathic scoliosis

Objectives The effects of spinal surgery on lung function and quality of life (QoL) are important patient outcomes. Long-term follow-up of lung function and QoL in those undergoing combined anterior and posterior spinal fusion (A/PSF) for adolescent idiopathic scoliosis (AIS) is poorly documented with only one study extending beyond 2 years, though available evidence points to a decrement in lung function. Our study evaluated long-term change in lung function and QoL following A/PSF for AIS. Design Prospective cohort study. Patients Patients with AIS. Setting Tertiary paediatric respiratory centre and national spinal service. Design Spirometry was performed along with QoL (Scoliosis Research Society-22 (SRS-22) questionnaire). Paired t test and one-way analysis of variance were used to compare pre-A/PSF and post-A/PSF data. Results Data were available for 12 patients (9 female) who underwent A/PSF at mean 13.8 (range 11.8–15) years. Mean follow-up was undertaken 5.8 (range 4.1–6.8) years postoperatively. Height increased from mean (SD) 169 (9) cm preoperatively to 175 (5) cm at follow-up (p<0.01). Scoliosis corrected from 100 (15)° to 29 (11)° (p<0.001). Mean (SD) forced expiratory volume in 1 s was −3.4 (1.4) z scores preoperatively versus −3.3 (1) z scores postoperatively (p=0.85); and forced vital capacity was −3.4 (1.7) ) z scores pre-A/PSF and −3.4 (1.1) z scores post-A/PSF (p=0.83). SRS-22 scores improved mean (SD) of 3.6 (0.3) preoperatively to 4.2 (0.3) at 2 years postoperatively, and 4.4 (0.4) at 6 year follow-up (p<0.001, analysis of variance). High patient satisfaction rates (4.8 (0.3)) were recorded. No correlation was noted between changes in forced expiratory volume in 1 s (r=−0.15, p=0.63) or forced vital capacity (r=−0.12, p=0.71) and change in long-term SRS-22 score. Conclusions Long-term follow-up of patients with AIS suggests no deficit in pulmonary function, while QoL shows incremental improvement and patient satisfaction is high over 6 years after A/PSF.

Airway clearance physiotherapy improves ventilatory dynamics during exercise in patients with cystic fibrosis: a pilot study

Background Airflow limitation and dynamic hyperinflation may limit exercise capacity in patients with cystic fibrosis (CF). The aim was to investigate whether the undertaking of airway clearance physiotherapy (ACT) prior to cardiopulmonary exercise testing (CPET) results in improvements in exercise capacity. Methods A prospective randomised, cross-over pilot study was performed in children aged >9 years. Spirometry, plethysmography and CPET were performed on two separate occasions—one test with ACT prior to CPET and the other without. Results 12 patients with CF were included in the study with a mean (SD) age of 12.83 (1.85) years. No significant difference in peak oxygen uptake (VO2) was found between the tests. However, lower minute ventilation (VE) and ventilatory equivalents (VEVO2 and VEVCO2) at ventilatory threshold (VT) were noted when ACT was undertaken prior to CPET. The mean(SD) VE (L/min) at VT was 26.67 (5.49) vs 28.92 (6.3) (p=0.05), VEVO2 (L/min) at VT was 24.5 (1.75) vs 26.05 (2.5) (p=0.03) and VEVCO2 (L/min) at VT was 26.58 (2.41) vs 27.98 (2.11) (p=0.03). Conclusions These pilot data suggest that ACT prior to exercise may lead to improved ventilatory dynamics during exercise in individuals with CF.

G378 Demographics and selected clinical features of paediatric human metapneumovirus infection

Aims This study aimed to characterise seasonal variation, co-infection rates, susceptible groups (by gender, age and co-morbidities) and selected clinical features of childhood human metapneumovirus (HMPV) infection. Methods The study was a retrospective analysis of 656 HMPV-positive respiratory samples collected from paediatric patients aged 0–15 years processed from January 2010 to December 2013 using real time PCR assays. In addition, 200 HMPV-positive samples from January 2012 to January 2013 were cross-referenced with electronic discharge summaries and descriptive statistical analyses of selected clinical features were performed. Results 377 of 656 (57%) HMPV-positive samples were from male patients. HMPV was most frequently detected in children aged 6–9 months and the median age of patients studied was 15 months. Seasonal patterns of HMPV infection varied from year to year. The peak in HMPV-positive isolates occurred between February and May. 117 of 656 (17.5%) samples were positive for other respiratory viruses in addition to HMPV. The most common co-infections were due to rhinovirus (58/656, 8.84%) and adenovirus (36/656, 5.49%). 81 (40%) of the 200 fully-characterised patients were hospitalised, 7 (3.5%) of whom required intensive care (ICU) or high dependency (HDU) admission. Rashes were reported in 14 (7%) and febrile seizures in 9 (4.5%) of 200 patients. These clinical features were more common in children with co-infections [6/40 (15%) and 4/40 (10%) respectively]. Conclusions Male gender is suggested as a predisposing factor for HMPV infection, along with younger age. Seasonal variation of HMPV infection in Scottish children appears different to the reported winter peaks of other studies, with peak incidence occurring between February and May in our dataset. Co-infection is common, and most frequently associated with rhinovirus or adenovirus. Rashes and febrile seizures are relatively common in HMPV-positive patients, especially those co-infected with other respiratory pathogens. Human metapneumovirus is a significant cause of morbidity in children. Further, larger-scale epidemiological research appears warranted, along with work to develop new therapies aimed at targeting HMPV.

G101(P) Incidence of scoliosis in adolescent cystic fibrosis patients

Background There are limited data on scoliosis in cystic fibrosis (CF) and the two most recent studies came to opposite conclusions. Reported prevalence ranges from 2%1 (within the normal range for the general population: 0.5–3.2%) to 15.5%.2 We felt that a recent study under-estimated the prevalence due to a very young population (median age 10.9 years), since scoliosis is most common in adolescents. We hypothesised that scoliosis is more prevalent in adolescents with CF compared to the general population. Objective To determine the incidence of scoliosis in adolescents with CF, and describe the spinal deformities. Population Patients in our CF clinic aged >10 years, and those who have transitioned to adult services in the last 10 years. Methods We conducted a retrospective observational study. Most recent chest radiographs, or those taken at transition to adult services, were reviewed by a Consultant Radiologist. Scoliosis was defined as a Cobb angle of greater than 10 degrees in the coronal plane.3 Results Our sample size was 143 CF subjects (48% male) with a median age at the time of chest radiograph of 18 years (range 10–18 years). 16 (6 male) subjects were noted to have scoliosis with a median (range) Cobb angle of 14 (10–38) degrees giving a prevalence of 11%. 13 were single curves, 2 double and 1 triple. The majority were short mid-thoracic curves, with the apex to the right. Conclusion We found a prevalence of scoliosis in a predominantly adolescent CF population that is significantly greater than expected in the general population. The negative effect of scoliosis on lung function is well-documented. With the progressive nature of CF lung disease, scoliosis may have further deleterious effects. Bone disease is increasingly recognised in CF patients, with osteopenia and osteoporosis occurring earlier and more frequently than in the general population (38% & 24% respectively in 18–32 year old CF patients).4 To date, studies have failed to show a correlation between scoliosis, lung function and bone mineral density. The paradigm of a radiologically significant (Cobb angle >10 degrees) versus a clinically important scoliosis remains. References Fainardi V, Koo SD, Padley SP, Lam SH, Bush A. Prevalence of scoliosis in cystic fibrosis. Pediatr Pulmonol 2013 Jun;48(6):553-555. Kumar N, Balachandran S, Millner PA, Littlewood JM, Conway SP, Dickson RA. Scoliosis in cystic fibrosis: is it idiopathic? Spine (PhilaPa 1976) 2004;29:1990–1995. Scoliosis Research Society criteria, www.srs.org Quon BS, Aitken ML. Cystic fibrosis: what to expect now in the early adult years. Paediatr Respir Rev. 2012;13(4):206-214.

G96 Epidemiology of Pierre Robin Sequence 2004–2012: a UK regional retrospective review

Aims Pierre Robin Sequence (PRS) is a rare, congenital disorder characterised by micrognathia, glossoptosis and cleft palate. Aetiology is reported to include genetic factors and early intrauterine exposure to environmental toxins or compressive forces (e.g. oligohydramnios, twin pregnancy). Epidemiological data are sparse, though available evidence suggests incidence is 1:8500 to 1:14000 live births with a possible trend towards increasing incidence over time. We aimed to determine the incidence of PRS over a 9-year period in a UK region with a 2.4 million population and to identify trends in incidence and geographical variation. Methods Children born between January 2004 and December 2012 with a diagnosis of PRS made by a Consultant Cleft Surgeon were identified by manually searching a patient database maintained by our regional Cleft Service. Case notes were retrospectively reviewed to verify the diagnosis and collect data relating to aetiology, clinical presentation, associated abnormalities, management and outcome. Results 91 PRS cases (52% male) were identified from a cohort of 254 000 live births during the 9-year study period, giving an incidence of 1:2790 live births. Annual incidence varied from 1:1570 to 1:5870 live births (5–19 cases per year; median 9, mean 10), with no significant incidence trends across the study period. Incidence varied across eight Health Boards within the region, ranging from 1:1440 to 1:5300 live births, and showed a trend of increasing incidence with decreasing latitude. Data analysis of secondary outcomes is ongoing. Conclusions This study identifies an incidence of PRS within a large UK region that is 3-fold higher than the last published UK epidemiological study (1983, Liverpool) and 5-fold higher than the only comparable national study (2004, Denmark). We identified no incidence trends over the study period but note clear geographical variations within an area spanning 59 000 km2 and including 42% of our national population. Further work is required to ascertain whether our findings are replicated across the UK, and to investigate possible aetiological causes for the geographical differences identified. The authors have been awarded a BPSU bursary to conduct a 1-year national UK surveillance study which will aim to address these questions.

G329(P) Prevalence of symptoms of disordered sleep in children with epilepsy

Aim To define prevalence of sleep-related breathing disorder (SRBD) as measured by Paediatric Sleep Questionnaire (PSQ) and Excessive Daytime Sleepiness (EDS) as measured by modified Epworth sleepiness scale (ESS) in a cohort of children with epilepsy compared with healthy controls. Methods Consecutive epileptic children attending for overnight video telemetry or outpatient clinics were recruited along with non-epileptic controls from paediatric surgical clinics in a tertiary children hospital setting. Additional information was collected on use of anti-epileptic drugs (AED). Those with additional neurological or respiratory problems were excluded. Parents were asked to complete both PSQ and modified ESS on behalf of their children. A score of >0.33 on the PSQ-SRBD scale was considered abnormal, and scores of >10 on the ESS were considered significant. Data were entered into Microsoft Excel database and analysed using SPSS v19.0. Non-parametric statistical comparison was made between groups by Mann-Whitney U-test (MWU). Results Seventy-five children were studied, 33 of whom had a diagnosis of epilepsy median (IQR) age 9 (5–12) years, along with 42 controls of median (IQR) age 6 (4–8.5) years. 49% epileptic subjects had an abnormal PSQ-SRBD score compared with 5% controls. The median (IQR) PSQ-SRBD score in the epilepsy group was 0.36 (0.22 to 0.46) versus 0.17 (0.09 to 0.23) in controls (p < 0.001, MWU). PSQ sub-scale scores were also significantly higher for snoring (p = 0.04), daytime sleepiness (p < 0.001), and inattention/behaviour (p = 0.007) in the epilepsy group compared with controls. Additionally, 30% epileptic subjects had an ESS >10 compared with 5% controls. Median (IQR) ESS score in epileptic subjects was 5 (1 to 10) versus 1 (0–3.25) in controls (p = 0.001, MWU). Within epileptic subjects, PSQ-SRDB (but not sub-scale scores) and ESS were higher in those on AED (p = 0.001 and p = 0.48 respectively). Conclusion This study suggests a higher prevalence of symptoms of SRBD and EDS in children with epilepsy compared with healthy children. Anti-convulsant therapy may be a confounding factor, but does not alone account for the associations seen. Further studies which should include polysomnography to verify the presence (rather than suggestion by questionnaire) of SRBD are warranted.