Royal Children Research Articles

Delayed diagnosis of anorectal malformations in neonates.

Anorectal malformations (ARM) are common congenital abnormalities of the terminal hindgut. Ideally, ARM should be diagnosed at, or shortly following, birth by careful physical examination of the perineum. Delayed diagnosis has been implicated as a risk factor for complications, including intestinal perforation. This study aimed to determine the rate of delayed diagnosis and associated intestinal perforation in ARM. A retrospective review was performed for all ARM patients managed at The Royal Children's Hospital over a 16-year period (2000-2015). Data collected included ARM type, timing of diagnosis and complications. Delayed diagnosis was defined as being at more than 24 h of age. A total of 243 ARM patients (male 146/243, 60%) were included. The most frequent ARM types were perineal fistula (83/243, 34%) and rectovestibular fistula (40/243, 16%). Diagnosis was delayed beyond 24 h of age in 92 of 243 (38%) patients. The ARM type most commonly delayed in diagnosis was perineal fistula (37/83, 45%). Two patients in whom diagnosis was delayed suffered an intestinal perforation. Delayed diagnosis in ARM patients remains a common, and potentially fatal, occurrence. Improved assessment of newborns is required to ensure timely diagnosis of ARM, and avoidance of complications associated with delayed diagnosis.

Century of the Angklung Journey and Its Establishment in Thailand

Both Thai and Javanese have been with lifelong cultural friendship for over 250 years. Thai people enjoyed the Panji storytelling since the late 1700’s andhave been using Panji as written Thai poetic literature and for court theatre over2 centuries. We shared the same brass knobbed gong culture with its pentatonic(Slendro) and septatonic (Pelog) equidistance scales. The angklung, bambooshaking instrument, were brought as a gift to royal children by HM King Rama the V to Siam since 1871 but the ensemble were not set up until our great court musician named Jangwang Sorn Silapa-banleng, took the instruments from Sunda West Java to Bangkok in 1908, later, he established the very first ThaiAngklung Ensemble played several songs he composed in pentatonic scalesame way he heard in the Javanese Islands. Since then, the bamboo angklungwere made with the Thai native bamboo followed by the nationwide uses. Thispaper describes the Angklung Journey from West Java to Thailand showing thehistoric photographs of Thai Angklung music teachers, their houses ofAngklungs and the music they composed. Today, Thai Angklung were used forchildren classroom music. DVD and Musical Disc of Thai Angklung will bedemonstrated, bringing both music knowledge and enjoyment to show thecross cultural examples of the two nations.

Rapid Assessment, Planning, Investigations and Discharge: Piloting the introduction of a senior doctor at triage model in an Australian paediatric emergency department.

We implemented a senior doctor at triage (SDT) pilot programme at The Royal Children's Hospital, Melbourne. We examined the impact on ED length of stay, seen on time and fail to wait (FTW) rates. A SDT model was piloted on Monday and Tuesday afternoons (pilot period) for 10 weeks, and compared with equivalent shifts for the preceding 10 weeks (pre-pilot period). We determined the differences between the proportions of patients seen on time, length of stay in the ED of less than 4 h and FTW rate, as well as the medians of time to clinician and length of stay in ED. A total of 2736 patients presented in the pilot period, and 2889 in the pre-pilot. The percentage of patients who were seen on time improved from 52.3% to 68.7% (absolute difference 16.4%, 95% confidence interval [CI] 13.6-19.2%, P < 0.001), the percentage of patients who had an ED length of stay of <4 h improved from 58.2% to 72.0% (absolute difference 13.8%, 95% CI 11.1-16.5%, P < 0.001) and the FTW rate reduced from 12.5% to 7.1% (absolute difference 5.4%, 95% CI 3.8-7.0%, P < 0.001) when the SDT model was operational. Implementation of a SDT model in a tertiary paediatric ED resulted in an increased proportion of patients being seen on time, having shorter length of stays in the ED and reduced the number of patients who FTW. Further studies are required to determine whether these improvements are sustained over time.

Utility of Endobronchial Ultrasound in Assessment of Intrathoracic Lesions in Paediatric Patients

Introduction: In adults, linear endobronchial ultrasound (EBUS) is the preferred modality to sample intrathoracic adenopathy and radial EBUS is a useful tool to biopsy peripheral pulmonary nodules. Utility in children is less well known. Objectives: The objective of this study was to review the EBUS experience of two specialist centres to better define the current role of EBUS in paediatric practice. Methods: A retrospective record review of EBUS procedures undertaken in patients aged 0–17 years at the Royal Children’s Hospital (Melbourne, Australia) and Thoraxklinik (Heidelberg, Germany) was performed. Data extracted included patient demographics, clinical presentation, bronchoscope (size and model), EBUS technique used, pathologic results, need for further invasive investigation, and complications. Results: Between 2008 and 2017, ten EBUS procedures were performed (6 linear EBUS and 4 radial EBUS). No complications were reported. Linear EBUS was performed on subjects who were between 4 and 15 years old, with a 100% diagnostic yield. Radial EBUS was non-diagnostic in three cases of non-malignant disease. In one case, it was used successfully for imaging alone. Conclusion: Both linear and radial EBUS are safe and feasible in children. Diagnostic yield of linear EBUS was 100%. Radial EBUS did not demonstrate utility, likely reflecting the pathologies of underlying parenchymal masses in paediatric populations.

Intravenous ceftriaxone at home versus intravenous flucloxacillin in hospital for children with cellulitis: a cost-effectiveness analysis

Outpatient parenteral antibiotic therapy after hospital admission is increasingly popular, but its use to avoid admission to hospital altogether by treating patients wholly as outpatients remains uncommon in children. One reason for the low use of treatment at home is the scarcity of evidence of its cost-effectiveness. In this planned follow-up analysis of the Cellulitis at Home or Inpatient in Children from the Emergency Department (CHOICE) trial, we aimed to assess the cost-effectiveness of an admission avoidance pathway, in which children were treated at home, compared with standard hospital care for the intravenous treatment of moderate or severe cellulitis. We did a cost-effectiveness analysis to compare home treatment with intravenous ceftriaxone versus hospital treatment with intravenous flucloxacillin in children aged 6 months to 18 years who had presented to the emergency department at The Royal Children's Hospital, Melbourne, VIC, Australia, with moderate or severe uncomplicated cellulitis. We included costs from two sources: institutional costs at a patient level and expenses incurred by families. We measured effectiveness with quality-adjusted life years (QALYs), which we derived from the Child Health Utility 9D questionnaire, and a clinical outcome of treatment failure, which was the primary outcome of the CHOICE trial. We planned to calculate the incremental cost-effectiveness ratio, defined as the difference between groups in total cost divided by the difference between groups in effectiveness. The CHOICE trial is registered at ClinicalTrials.gov, number NCT02334124. We included 180 children who comprised the per-protocol population in the CHOICE trial: 89 children in the home group and 91 children in the hospital group. The institutional cost per patient per episode was significantly lower in the home group than in the hospital group (AUS$1965 vs $3775; p<0·0001). The mean cost incurred per family was $182 for the home group and $593 for the hospital group (p<0·0001). Both measures of effectiveness were significantly better in the home group than in the hospital group: QALYs were 0·005 for the home group versus 0·004 for the hospital group (p<0·0001), and treatment failure occurred in one (1%) patient in the home group versus seven (8%) patients in the hospital group (risk difference -6·5%, 95% CI -12·4 to -0·7; p=0·029). Calculating the incremental cost-effectiveness ratio was thus deemed redundant. Treatment at home was less costly and more effective than standard hospital care for children with moderate or severe cellulitis. These findings support development of this admission avoidance pathway in hospitals. The Royal Children's Hospital Foundation, Murdoch Children's Research Institute.

The 13-Valent Pneumococcal Conjugate Vaccine Is Effective Against Hypoxic Pneumonia in Children in Lao PDR: A Novel Method Using a Hospital-Based Observational Study

Background: Pneumococcal pneumonia is a leading cause of childhood mortality. Pneumococcal conjugate vaccines (PCVs) have been shown to reduce hypoxic pneumonia in children. However, there are no 13-valent PCV (PCV13) vaccine effectiveness (VE) studies on hypoxic pneumonia from Asia. We describe a novel approach to determine the effectiveness of PCV13 against hypoxic pneumonia in children admitted with pneumonia in the Lao People's Democratic Republic, a low-resource setting. Methods: A prospective hospital-based observational study of children up to 59 months old admitted with pneumonia to a single tertiary hospital in Vientiane was undertaken over 54 months. Pneumonia was defined using the 2013 WHO definition. Hypoxia was defined as an oxygen saturation <90% in room air or requiring oxygen supplementation during hospitalisation. PCV13 status was determined by written record. VE was calculated using logistic regression comparing the odds of hypoxia between vaccinated and undervaccinated pneumonia cases. To handle potential confounders a propensity score (PS) analysis using inverse probability of treatment weighting was used; for missing data, multiple imputation (MI) analysis was undertaken. Findings: There were 826 children admitted with pneumonia, 285 had hypoxic pneumonia and 377 were PCV13-vaccinated. The unadjusted, PS adjusted and MI adjusted VE against hypoxic pneumonia were 23% (95% confidence interval [CI]: -9, 46%; p=0·14); 37% ([CI]: 6, 57%; p=0·02) and 35% ([CI]: 7, 55%; p=0·02) respectively. Interpretation: This is the first apparent study to show PCV13 is effective against hypoxic pneumonia in Asia. This single hospital-based approach will enable other similar settings to test for VE. Funding Statement: This study received funding from the Bill & Melinda Gates Foundation (OPP1115490). Declaration of Interests: KM and CN receive grants from Pfizer, outside the submitted work. The Institute of Research for Development (IRD) and Aix-Marseille University funds both ADP and the HRSV testing. PN receives grants from the Wellcome Trust. JL receives funding from Bill and Melinda Gates foundation. All other authors declare no competing interests. The opinion presented in this paper is that of the authors and does not reflect Pfizer. We have not been paid by a pharmaceutical company to write this article. Ethics Approval Statement: This study was conducted according to the study protocol approved by the Oxford Tropical Research Ethics Committee (OxTREC reference:1050-13), Laos Ministry of Health National Ethics Committee for Health Research (No 061NECHR), the Ethics Review Committee of the WHO Regional Office for the Western Pacific Region (WPRO-ERC) (reference ID:2013.30.LAO.2.EPI) and by The Royal Children’s Hospital Human Resources Ethics Committee (HREC) (reference number:33177B).

Neonatal neuroimaging after repair of congenital diaphragmatic hernia and long-term neurodevelopmental outcome

ObjectivePrevious outcome reports of congenital diaphragmatic hernia (CDH) have described neuroimaging anomalies and neurodevelopmental impairment. However, the link between imaging and outcome has not been described. We aimed to determine...

Skin Prick Test Predictive Values for the Outcome of Cashew Challenges in Children

Cashew is a common cause of tree nut allergy in children. To date there have been few studies of diagnostic tests for cashew allergy, and positive predictive values (PPVs) for cashew as well as other tree nuts are largely extrapolated from studies of peanut allergy. How relevant these cutoffs are for cashew has not been formally explored. We aimed to establish skin prick test (SPT) wheal sizes that correlated to 95% PPV for a positive food challenge for cashew. We included all cashew oral food challenges (OFCs) conducted as part of the HealthNuts (n= 108; age, 4-6 years) and SchoolNuts (n= 37; age, 10-14 years) studies, both recruited from the community (population cohort). A second cohort of all cashew OFCs conducted at the Royal Children's Hospital (RCH) allergy center (n= 343) (2011-2016) and a private allergy clinic based at RCH (n=43) was included via electronic medical record review (clinic cohort). The 95% PPV for cashew SPT was calculated for both cohorts. Among the population cohort (n= 145), 62% of cashew OFCs were positive compared with 20% of the clinic cohort (n= 386). The SPT cutoff for 95% PPV derived from the population cohort was 10 mm (95% confidence interval [CI], 7.5-12.0). For the clinic cohort, the 95% PPV was 14 mm (95% CI, 9.5-unknown). An SPT wheal size of 8 mm had a PPV of 89% (95% CI, 79-95) in the population cohort and 62% (95% CI, 45-78) in the clinic cohort. A higher SPT wheal size may be more appropriate than the commonly used 8 mm cutoff to guide clinical decisions around when to perform OFC for cashew.

Cost-effectiveness of epileptic surgery compared with medical treatment in children with drug-resistant epilepsy

BackgroundEpilepsy surgery is an alternative to continued antiepileptic drugs (AEDs) in children with drug-resistant epilepsy (DRE). ObjectiveThe objective of the study was to measure, model, and compare the medical costs and impacts on health-related quality of life (HRQL) of epilepsy surgery versus continued medical treatment with AEDs in children with DRE. MethodsA decision analytic model was created to estimate the cost-effectiveness of epilepsy surgery relative to continued medical treatment with AEDs. The model was based on costing and effectiveness data collected from 105 children with DRE who were operated on at the Royal Children's Hospital, Melbourne, Australia. The mean cost of conducting epilepsy surgery was AU$ 61,417 per person. Effectiveness of continued medical treatment was sourced from best available literature. In the absence of published utility values for pediatric patients with epilepsy and ethical approval to contact patients directly, HRQL was estimated by four clinicians using the Child Health Utility 9 Dimension (CHU9D). Outcome measures were seizure freedom and quality-adjusted life years (QALYs). ResultsThe costs over 7.6 years of follow-up were AU$ 219,297 for the surgical treatment group compared with AU$ 170,583 for the medical treatment group. The incremental cost-effectiveness ratio (ICER) for surgically vs medical treatment was AU$ 76,538 per additional patient attaining seizure freedom and AU$ 75,541 per additional QALY gained. ConclusionEpilepsy surgery resulted in a greater reduction of seizures and improvement in HRQL but was more expensive than continued medical treatment with AEDs. Including benefits outside of a healthcare perspective would likely lead to a more compelling cost-effective argument.

Does fluid bolus therapy increase blood pressure in children with sepsis?

To describe the effect of fluid bolus therapy (FBT) on blood pressure in children with sepsis. Secondary outcomes included the effect of FBT on systemic vascular resistance, shock index and shock phenotype (warm or cold). This was a prospective observational study in the ED of The Royal Children's Hospital, Melbourne, Australia. Participants were children meeting international consensus criteria for sepsis who received FBT for tachycardia or hypotension. FBT was defined as 10-20 mL/kg of 0.9% saline. Mean blood pressure (MBP) was recorded at baseline, 5 and 60 min after FBT. Total systemic vascular resistance index (TSVRi), shock index, and shock phenotype were derived for each time point. Hypotension was defined as MBP <55 + 1.5 × age (years). Warm shock was defined as TSVRi <800 dyne s/cm5 /m2 . Fifty fluid boluses were recorded in 41 children. Median MBP was 78 mmHg (interquartile range [IQR] 63-86) at baseline, 72 mmHg (IQR 60-82) at 5 min, and 75 mmHg (IQR 66-84) at 60 min. Hypotension was observed in 16% at baseline, 26% at 5 min and 17% at 60 min. Median TSVRi was 1580 dyne s/cm5 /m2 (IQR 1242-2206) at baseline, 1254 dyne s/cm5 /m2 (IQR 1027-1787) at 5 min, and 1850 dyne s/cm5 /m2 (IQR 1265-2140) at 60 min. Median shock index was 1.60 (IQR 1.34-1.90) at baseline, 1.49 (IQR 1.25-1.76) at 5 min and 1.37 (IQR 1.22-1.61) at 60 min. Two percent of cases had warm shock at baseline, 12% at 5 min and 2% at 60 min. MBP initially decreased following FBT for paediatric sepsis, returning towards baseline over the subsequent 60 min. The utility of FBT for increasing MBP and its effect on patient-centred outcomes in children with sepsis warrants further exploration.

Quality of life in adult survivors after paediatric heart transplantation in Australia.

Paediatric heart transplantation in Australia is centralised at The Royal Children's Hospital, Melbourne. Survival to adulthood is improving but the ongoing need for complex medical therapy, surveillance, and potential for late complications continues to impact on quality of life. Quality of life in adults who underwent heart transplantation in childhood in Australia has not been assessed. Cross-sectional quality of life data were collected from paediatric heart transplant survivors >18 years of age using Rand 36-Item Health Survey. Self-reported raw scores were transformed to a 0-100 scale with higher scores indicating better quality of life. Mean scores were compared to National Health Survey Short Form-36 Population Norms data using the independent sample t-test. A total of 64 patients (64/151) who underwent transplantation at The Royal Children's Hospital between 1988 and 2016 survived to adulthood. In total 51 patients (51/64, 80%) were alive at the time of the study and 27 (53%) responded with a mean age of 25 ± 6 years, being a median of 11 years (interquartile range 7-19) post-transplantation. Most self-reported quality of life subscale scores were not significantly different from the Australian normative population data. However, self-reported 'General Health' was significantly worse than normative data (p = 0.02). Overall, 93% (25/27) reported their general health as being the same or better compared to 1-year ago. Adult survivors after paediatric heart transplantation in Australia report good quality of life in multiple domains and demonstrate independence in activities of daily living and employment. However, lifelong medical treatment may affect perceptions of general health.

Unraveling the Association between Pediatric Traumatic Brain Injury and Social Dysfunction: The Mediating Role of Self-Regulation.

Social dysfunction is a common and disabling outcome of childhood traumatic brain injury (TBI); however, the mechanisms that link early brain injury to impaired social behavior are unclear. This longitudinal prospective study involved 129 children, including 86 children with TBI (53 mild, 33 moderate-severe TBI) and 43 age-matched typically developing control (TDC) children. Children with TBI were recruited via consecutive admissions to the emergency department of the Royal Children's Hospital, Melbourne Australia. At 6 months post-injury, all participants were assessed on measures of intellectual functioning, and parents provided ratings of children's self-regulation skills using gold standard measures of everyday executive function. At 12-month follow up, parents rated children's social adjustment using standardized measures of social skills, communication, and behavior. As expected, children with moderate-severe TBI were rated by parents as having greater self-regulation problems, worse social skills, and poorer communication than the mild TBI and TDC groups. In mediation models, greater self-regulation problems at 6 months post-injury were predictive of poorer social skills and worse overall social adjustment at 12-month follow-up. Moreover, self-regulation skills mediated the effect of moderate-severe TBI, but not mild TBI, on social skills and overall social adjustment. Our findings show that, while impaired social adjustment is common at 12 months following moderate-severe child TBI, the impact of TBI on these outcomes is likely mediated by its effect on self-regulation skills. Evidence for robust prospective associations between self-regulation deficits and later social adjustment difficulties have implications for early identification of children at high risk for chronic social problems.

DOZ047.63: Closing the gap: transitioning adolescents with esophageal atresia to adult care

Abstract Introduction Evidence supports engaging adolescents and carers in an individualized transition process to improve health outcomes through increased patient knowledge, skills, adherence, quality of life and ultimately, engagement with adult services. Further, a comprehensive transition process promotes confidence in navigating adult health services. While transition for chronic medical cohorts has received considerable attention over decades, there is increasing recognition of the importance of transition for adolescents with surgical conditions. Background It is widely held that the transition of adolescents born with esophageal atresia (OA) from pediatric to adult services is important, and if not managed well, may compromise outcomes. We designed and implemented a transition process for adolescent OA patients at our hospital (with establishment of a transfer pathway in partnership with adult health services) in 2017. Methods Following implementation of a transition process a retrospective review was undertaken in 2019 to identify themes from the dedicated Transition Support Service (TSS) Clinics (October, 2017–December, 2018) for OA patients aged 15–19 years. Patients were identified from an institutional database (Nate Myers Oesophageal Atresia Database, Royal Children's Hospital, Melbourne). These patients had previously been invited to: Attend TSS Clinics as part of routine clinical care.Re-engage in multidisciplinary follow-up (if necessary) including with a consultant surgeon, OA nurse and respiratory physician. Institutional ethics approval was obtained prior to commencement of the study (HREC #QA/51265/RCHM-2019). Results A total of 18 adolescent OA patients and their parents attended RCH TSS and associated follow-up clinics. We observed three key themes in attendees reports and experiences: Poor health knowledge: 56% of adolescents had poor knowledge about their OA, including previous surgery, possible complications and their symptoms;Poor compliance: 54% of adolescents reported poor compliance with prescribed regular medications (e.g. anti-reflux therapy);Mental health issues: 39% of adolescents identified as having mental health concerns, e.g. low mood or clinical depression, anxiety, anger or had engaged in self-harm. Anxiety about transfer to adult services was moderate to high in the majority (61%) of adolescents and their parents. Conclusions The themes identified in this study support the need for, and importance of, a structured process to transition adolescents born with OA to adult services.

PB2349 EXTRACORPOREAL PHOTOPHERESIS FOR PEDIATRIC PATIENTS WITH GRAFT‐VS‐HOST DISEASE AFTER HAEMATOPOIETIC STEM CELL TRANSPLANTATION: AN AUSTRALIAN EXPERIENCE

Background:Graft‐versus‐host disease (GVHD) remains a major cause of morbidity and mortality following pediatric allogeneic hematopoietic stem cell transplant (HSCT). Extracorporeal photopheresis (ECP) is a cellular immuno‐modulatory therapy licensed for the treatment of steroid‐dependent or refractory GVHD. ECP has been shown to be effective in the treatment of GVHD in the adult population, however there is little data in the pediatric setting. Here we present our Australian experience in paediatric patients with GVHD.Aims:To assess the outcome and feasibility of ECP as a treatment modality for GVHD in the pediatric allograft patients in Australia.Methods:A retrospective case note analysis of all patients receiving ECP for the treatment of GVHD between August 2010 and December 2018 at the Royal Children's Hospital and Peter MacCallum Cancer Centre, Victoria.Results:14 patients were identified, with a median age of 4.4 years (range 0.5‐14.8). Demographic and transplant characteristics are summarised in Table 1. ECP was used to treat both acute (aGVHD, n = 7) and chronic (cGVHD, n = 8) GVHD. One patient required two treatment episodes for acute and subsequently chronic GVHD. All patients were refractory to prior immunosuppressive therapy, with a median of 3 agents used (range 2‐5). Median steroid dose was 0.8 mg/kg/day methylprednisolone equivalent (range 0‐2). An overall response was obtained in 6 of 7 (86%) acute and 6 of 8 (75%) chronic GVHD patients. Overall survival was 79% (11 of 14 patients).Of those treated for aGVHD varying from grade II – IV, 86% (n = 6) showed a partial response or better, with 43% (n = 3) achieving a complete response. Median steroid dose was reduced by 94% from 1.8 mg/kg/day methylprednisolone equivalent to 0.1 mg/kg/day from beginning to end of ECP. ECP duration was a median of 1.9 months (range 1.4‐5.7), with a median of 16 treatments (range 12‐25).The majority of cGVHD patients had extensive disease (n = 7, 88%), with 75% (n = 6) achieving at least a partial response, with a complete response in 37.5% (n = 3). Median steroid dose was reduced by 88% from 0.18 mg/kg/day methylprednisolone equivalent to 0.02 mg/kg/day from beginning to end of ECP. ECP was used for a median period of 7.1 months (range 1.9‐39.1) with a median of 25 treatments (range 7‐61).Catheter‐related sepsis was the most common reason for premature discontinuation of ECP in our cohort, occurring in 6 patients (43%). Other noted complications included haemodynamic instability requiring admission, occurring in 3 patients (21%). This caused discontinuation of ECP in one patient weighing 7.5 kg. Of the 3 patients who died, two had grade IV aGVHD with progressive disease post‐ECP, and one with refractory extensive cGVHD. The causes of death were sepsis (n = 2) and relapsed primary malignancy (n = 1).Summary/Conclusion:This is the first report of ECP therapy in Australia for pediatric patients with GVHD. Our results demonstrate that ECP is an effective and feasible treatment modality for both acute and chronic GVHD post‐HSCT. In our cohort, ECP allowed for tapering of corticosteroids, which has been shown to be associated with improved survival. Although ECP was well tolerated overall, specific consideration should be given when used in the pediatric population, especially with regard to the risk of haemodynamic instability, the use of blood prime of the circuit and catheter‐related sepsis.image

DOZ047.64: Sham feeding promotes oral feeding success in long-gap esophageal atresia, even with traction sutures in situ

Abstract Introduction Sham feeding (SF) and staged repair using traction suture techniques (TST) were both introduced to the management of long-gap esophageal atresia (LGOA) at The Royal Children's Hospital, Melbourne (RCH) in January 2012. Previous studies report SF to reduce time to full oral feeding (FOF) postdefinitive repair. In our practice, SF is individually tailored, supervised, and involves offering 2–4 feeds daily. This single-center experience describes SF in newborns with LGOA, including those with staged TST prior to definitive repair, and the impact of SF on time to FOF. Methods Patients admitted 2000–2018 with LGOA were identified from the Nate Myers Oesophageal Atresia Database, RCH. Those with incomplete data or complications preventing oral feeds were excluded. Patients were grouped into two epochs: Group 1 (2000–2011, no SF, no TST) and Group 2 (2012–2018, all offered SF, some TST). Demographics and time to FOF were compared between groups. Parametric data (mean, SD) were analyzed with two sample t-test, and nonparametric data (median, IQR) with Mann–Whitney test; P &lt; 0.05 significant. Ethics approval was obtained (HREC#QA/51247/RCHM-2019). Results Of 37 LGOA patients, six met exclusion criteria leaving 31 for analysis; Group 1, n = 13; Group 2, n = 18. Groups did not differ in gestation (P = 0.63), birth weight (P = 0.91) or time to definitive repair (P = 0.85). In Group 2, 12/18 had successful SF, including 9 who underwent TST. Three additional patients with TST were unsuccessful with SF. Compared with Group 1, FOF was significantly reduced in those 12 patients with successful SF (median FOF 730 days, IQR 125–1100 vs. median FOF 75 days IQR 56–227; P = 0.03). Small sample size precluded meaningful subgroup analyses of successful versus unsuccessful SF patients. Importantly, no patient aspirated during SF, though one developed oral aversion post-TST and one ceased SF at parental request. Conclusions In LGOA, successful SF improves time to FOF postdefinitive repair, and was not associated with aspiration in our experience. Further, we report success with SF in patients undergoing staged repair with TST, including following traction suture placement. To our knowledge, this is the first report of SF in LGOA patients during the period between traction suture placement and definitive repair.

DOZ047.24: Predictors of early and late mortality in esophageal atresia patients

Abstract Background Esophageal atresia (EA) is associated with mortality, despite significant advances in neonatal care. Predictors of early mortality (EM) are well described in the literature. However, little is known about late mortality (LM). This study aimed to describe EM and LM rates and their associations in EA patients. Methods Patients with EA, managed at The Royal Children's Hospital, Melbourne (1980–2017), who subsequently died, were identified from the Nate Myers Esophageal Atresia database. Data collected included patient demographics, VACTERL associations, mortality risk factors, and preoperative, operative, and postoperative findings. Early mortality was defined as death during initial admission. Results A total of 90/628 (14.3%) patients died during the study period—EM occurred in 68/90 (75.6%), whilst LM occurred in 22/90 (24.4%). Causes of EM were most often palliation for respiratory anomalies (17/68, 25.0%), followed by associated syndromes (11/68. 16.2%) and neurological anomalies (10/68, 14.7%). The most commonly associated syndrome leading to palliation was trisomy 18 (7/68, 10.3%). Causes of LM were most often respiratory compromise (6/22, 27.3%), sudden unexplained deaths (6/22, 27.3%), and Fanconi's anemia (2/22, 9.1%). Of patients discharged from hospital, 22/560 (3.9%) subsequently died. There was no difference in VACTERL association between EM (32/63, 50.8%) and LM (11/20, 55%) (P = 0.80), and no difference in the incidence of twins between EM (8/58, 13.8%) and LM (2/18, 11.1%) (P = 1). Conclusion We identified predictors of early and late mortality in esophageal atresia in a large cohort. Parents of children with esophageal atresia must be counseled appropriately as to the likelihood of death following discharge from hospital.

Imperforate hymen: Retrospective review from a single tertiary centre of presenting symptoms and diagnostic process.

To review presentations, diagnostic processes and time to diagnosis of presentations of imperforate hymen at one tertiary paediatric hospital over a 10-year period. The aim was to improve knowledge, enable targeted education and reduce unnecessary tests and diagnostic delay for this congenital obstructive disorder of the female reproductive tract. A pragmatic, retrospective, observational study was conducted of all female patients, aged younger than or equal to 18 years of age, presenting to the Royal Children's Hospital, Melbourne, over an 18-year period. Presenting symptom(s), time to diagnosis, investigations ordered and number of health-care professionals consulted prior to diagnosis were recorded. A total of 27 patients met eligibility criteria, and 85% presented with pain (23/27). Pain was frequently associated with other symptoms (15/23). Over three quarters of patients consulted more than one health-care professional prior to diagnosis (22/27). Of the 27 patients, 19 had imaging undertaken prior to diagnosis. Paediatric and adolescent females managed at our tertiary paediatric hospital are experiencing diagnostic uncertainty and the unnecessary ordering of imaging prior to a diagnosis of imperforate hymen. Targeted education is recommended to health professionals managing female paediatric and adolescent patients.

Surgically Managed Ovarian Masses at the Royal Children’s Hospital, Melbourne –19 Year Experience

Background/PurposeTo describe the clinicopathological characteristics and management of surgically removed ovarian masses at the Royal Children’s Hospital, Melbourne from 1993 to 2012. MethodsMedical records were reviewed retrospectively. Data regarding clinical findings, imaging and surgical management were evaluated. ResultsThere were 266 ovarian masses found in 258 surgeries (eight had bilateral masses). Most were benign (246/266, 92.5%), 2.3% (6/266) were borderline, and 5.3% (14/266) were malignant. The most common presenting symptom was abdominal pain for benign masses (169/246, 68.7%), and a palpable mass for borderline and malignant masses (12/20, 60.0%).Sensitivity and specificity of ultrasound for detection of malignancy was 64.7% and 52.9% respectively. Ovarian torsion occurred in 22.1% (n=57), none with malignancy, with seven cases diagnosed under one year of age. Sensitivity and specificity of ultrasound for ovarian torsion was 22.0% and 91.9%, respectively.The proportion undergoing ovarian cystectomy rather than oophorectomy has increased from 56.3% during 1993-1997 to 93.8% during 2008-2012 (p<0.005). Ovarian torsion was managed with ovarian conservation in 82.6% of cases between 2008-2012. ConclusionThe majority of pediatric and adolescent ovarian masses were benign. Sensitivity of ultrasound was fair for detection of malignancy, and poor for ovarian torsion. Conservative surgeries are increasingly common. Level of EvidenceLevel IV – case series with no comparison group Type of StudyRetrospective Study

Impact of extreme prematurity or extreme low birth weight on young adult health and well-being: the Victorian Infant Collaborative Study (VICS) 1991–1992 Longitudinal Cohort study protocol

IntroductionInfants born extremely preterm (EP, <28 weeks’ gestation) or with extremely low birth weight (ELBW,<1000 g) in the era when surfactant has been available clinically are at high risk of...

Screening for associated anomalies in anorectal malformations: the need for a standardized approach.

Anorectal malformations (ARM) are common congenital abnormalities of the terminal hindgut. The high incidence of associated anomalies necessitates systematic screening, which should include renal and spinal ultrasonography, spinal radiography and an echocardiogram. This study aimed to determine the incidence of associated anomalies in ARM, and whether screening protocols were appropriately applied. A retrospective review was performed of all ARM patients managed at The Royal Children's Hospital, Melbourne over a 16-year period (2000-2015). Data collected included ARM type, presence of associated anomalies, as well as utilization of renal and spinal ultrasonography, spinal radiography and echocardiography. A total of 243 patients (male 146/243, 60%) were reviewed. The most frequent ARM types were perineal fistula (83/243, 34%) and rectovestibular fistula (40/243, 16%). Full screening was performed in 153/243 (63%), while 18/243 (7%) received no screening. In fully screened patients, associated anomalies were diagnosed in 143/153 (93%), with cardiovascular, renal and musculoskeletal anomalies being most frequent. The high incidence of associated anomalies identified in fully screened ARM patients highlights the importance of systematic screening. Clinically significant anomalies may have been overlooked in the more than one-third of ARM patients in whom screening was absent or incomplete. Standardized screening protocols for ARM patients have now been implemented.