Royal Children Research Articles

P18 An audit on the delivery of Bristol Royal Children's Hospital paediatric orthopaedic services in response to BOAST COVID-19 guidance

IntroductionThe COVID-19 pandemic raised concerns regarding the spread of infection by asymptomatic children. Guidance from the British Orthopaedic Association Standards for Trauma (BOAST) for the ‘management of patients with urgent orthopaedic conditions and trauma during the coronavirus pandemic’, helped structure our service in response to the pandemic. We assessed our compliance with ‘BOAST COVID-19 standards’ pertaining to children to determine whether it is possible to run a safe and effective paediatric orthopaedic service.MethodsBetween the 16th March and 30th April 2020, we performed a prospective audit of clinic and theatre data from the paediatric orthopaedic department at the Bristol Royal Children’s Hospital against the ‘BOAST COVID-19 standards’. We also performed a retrospective audit between 16th March and 30th April 2019 for comparison.ResultsPatients booked into acute fracture clinic (AFC) and fracture clinic follow-up (FFO) reduced by 40% and 48% respectively from 2019 to 2020. A virtual fracture clinic (VFC) was implemented with increasing trend in VFC consultations. From 2019 to 2020, the number of patient initiated follow-up appointments increased in AFC and FFO from 16% to 75% and 12% to 35% respectively. Radiography was reduced; only 17% and 39% of AFC and FFO patients respectively required radiographs. On-call referrals and trauma cases reduced by almost 50% with a similar case mix year-on-year. All elective operating was cancelled in 2020.ConclusionBy reducing clinic admissions and theatre throughput, it was possible to run an effective paediatric orthopaedic service in a busy tertiary referral centre. Our aim now is to determine the long-term efficacy.

The clinical features that contribute to poor bone health in young Australians living with cystic fibrosis: A recommendation for BMD screening

For Australians living with cystic fibrosis (CF), increased longevity means greater consideration needs to be given to long-term endocrine sequelae such as CF-related bone disease. Deficits in bone mass accrual are most likely to occur during childhood and adolescence. Current guidelines in Australia suggest repeat dual-energy X-ray absorptiometry (DXA) scans every 2 years. This study aims to stratify clinical factors that determine future bone health in the Australian CF population and use this to guide a more streamlined approach to bone health screening. This study was a retrospective audit of all patients diagnosed with CF who were treated at the Royal Children's Hospital Melbourne, Australia from 2000 to 2016 (n = 453). Two hundred and two patients had a DXA scan in the study period (191 with height-adjusted data) and 111 patients had more than one scan (108 with height-adjusted data). An investigation into the associations between bone mineral density (BMD) Zscore and potential risk factors was conducted using DXA and historical data. The main predictor of future BMD was the previous BMD Zscore (p < .001). Other factors found to be determinants of BMD included nutritional status, lung function (FEV1 ), age, history of previous fracture, oral corticosteroid use, and the number of hospital admissions. However, after adjusting for previous BMD, evidence of an association remained only with nutritional status, FEV1 , and number of hospital admissions. Second yearly scans may be unnecessary in children with an adequate DXA score on the initial scan who remain clinically stable. However, clinical deterioration in those whose BMD was previously normal, may require closer monitoring of bone health. We propose a guideline for the frequency of DXA monitoring in relation to clinical risk factors.

Characteristics and assessment of children undergoing upper limb surgery for management of cerebral palsy

Background: Surgical intervention for improvement of upper limb function in children with cerebral palsy is reported to be of benefit where careful patient selection is taken into consideration. Currently there are no clinical guidelines to help determine selection of individuals best suited for operative management, nor does research exist that identifies characteristics of the patient cohort currently being selected as appropriate for surgery. The aim of this study is to determine the current trends in selection, assessment and management of patients with cerebral palsy undergoing upper limb surgery.&#x0D; Methods: This retrospective study included data from 102 individuals with cerebral palsy who had undergone upper limb surgical management during a 10-year time period at the Royal Children’s Hospital, Melbourne, Australia. There were 138 separate surgical events involving 579 procedures during this period. Data regarding the characteristics of the individuals and their perioperative assessment was collected and analysed. Results: The cohort was 56.9% male, the median age at time of surgery was 14.2 years old. Motor involvement was 51.0% bilateral and 49.0% unilateral and 96.0% of individuals had a spastic component. The most common surgical goal was position care (50.4% of cases), followed by activity and participation (47.9% of cases). Preoperative outcome measures were used in 69.6% of cases.Conclusions: Currently, individuals are selected for surgery using variable preoperative assessment. Selection has great potential to differ between clinicians. These compounding issues provide rationale for initiating further research into understanding the characteristics of this patient group and promoting better standardisation of perioperative assessment.

Helen Rae Noblett

Helen Rae Noblett was a pioneering paediatric surgeon who came from Australia in 1976 to establish the specialty in Bristol. She trained in Brisbane and Melbourne. She had already made...

Magnitude and significance of interarm blood pressure differences in children and adolescents.

An interarm difference (IAD) in blood pressure (BP) of 10 mmHg or more is a potential cardiovascular risk factor in adults, given its association with cardiovascular events/mortality. In children and adolescents, accurate BP assessment is critical for identifying risk of end organ damage. However, IAD has not been systematically studied in paediatric patients; if present and of significant magnitude, measuring BP in only one arm could lead to misclassification of hypertensive status. In 95 children/adolescents with a normal aorta (including 15 with a history of tetralogy of Fallot) aged 7-18 years attending the Royal Children's Hospital, Melbourne, we aimed to determine the magnitude of IAD, frequency of IAD of at least 10 mmHg, difference in BP classification between arms, and influence of repeat measures on IAD in a single visit. After 5 min rest, simultaneous bilateral BP was measured in triplicate with an automated device. Absolute systolic IAD was 5.0 mmHg (median, interquartile range 2-8 mmHg) and was 10 mmHg or more in 14%, with no change on repeat measures. In patients with a history of aortic surgery, IAD of 10 mmHg or more occurred in 27% (transposition of the great arteries, n = 15) and 75% (aortic coarctation, n = 8). Differences in BP classification, based on initial left vs. right arm measures, occurred in 25% (normal aorta) and 40%/63% (aortic surgery), or 17% and 33%/50%, respectively if second and third measurements were averaged. Substantial interarm BP differences were common, even in apparently healthy children and adolescents: evaluation of IAD may, therefore, be important for BP classification in the paediatric setting.

Atypical Lemierre's syndrome complicated by transcalvarial brain herniation

Journal of Paediatrics and Child HealthVolume 57, Issue 10 p. 1707-1709 Instructive Case Atypical Lemierre's syndrome complicated by transcalvarial brain herniation Nelson Y Wang, Corresponding Author Nelson Y Wang nywan3@gmail.com orcid.org/0000-0002-2538-6072 Department of General Medicine, The Royal Children's Hospital, Melbourne, Victoria, Australia Correspondence: Dr Nelson Y Wang, Department of General Medicine, The Royal Children's Hospital, Melbourne, Victoria, Australia. Fax: +61 (03) 9345 4751; email: nywan3@gmail.comSearch for more papers by this authorDavid P Burgner, David P Burgner orcid.org/0000-0002-8304-4302 Department of General Medicine, The Royal Children's Hospital, Melbourne, Victoria, Australia Murdoch Children's Research Institute, The Royal Children's Hospital, Melbourne, Victoria, Australia Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia Department of Paediatrics, Monash University, Melbourne, Victoria, AustraliaSearch for more papers by this authorKatherine Y-H Chen, Katherine Y-H Chen orcid.org/0000-0003-3301-8031 Department of General Medicine, The Royal Children's Hospital, Melbourne, Victoria, Australia Murdoch Children's Research Institute, The Royal Children's Hospital, Melbourne, Victoria, Australia Department of Paediatrics, University of Melbourne, Melbourne, Victoria, AustraliaSearch for more papers by this author Nelson Y Wang, Corresponding Author Nelson Y Wang nywan3@gmail.com orcid.org/0000-0002-2538-6072 Department of General Medicine, The Royal Children's Hospital, Melbourne, Victoria, Australia Correspondence: Dr Nelson Y Wang, Department of General Medicine, The Royal Children's Hospital, Melbourne, Victoria, Australia. Fax: +61 (03) 9345 4751; email: nywan3@gmail.comSearch for more papers by this authorDavid P Burgner, David P Burgner orcid.org/0000-0002-8304-4302 Department of General Medicine, The Royal Children's Hospital, Melbourne, Victoria, Australia Murdoch Children's Research Institute, The Royal Children's Hospital, Melbourne, Victoria, Australia Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia Department of Paediatrics, Monash University, Melbourne, Victoria, AustraliaSearch for more papers by this authorKatherine Y-H Chen, Katherine Y-H Chen orcid.org/0000-0003-3301-8031 Department of General Medicine, The Royal Children's Hospital, Melbourne, Victoria, Australia Murdoch Children's Research Institute, The Royal Children's Hospital, Melbourne, Victoria, Australia Department of Paediatrics, University of Melbourne, Melbourne, Victoria, AustraliaSearch for more papers by this author First published: 18 February 2021 https://doi.org/10.1111/jpc.15366 Conflict of interest: None declared. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume57, Issue10October 2021Pages 1707-1709 RelatedInformation

Diplomatic Gifts as Ordering Devices

Summary This conclusion to the forum on diplomatic gifts goes on to note two historical types of such gifts. They are, first, fostering of a royal child at another royal court and, second, royal and noble marriage exchanges. Using these examples as a stepping stone, this essay goes on to formulate a co-ordination system of giver and receiver assessments of gift value, ranging from low to high. This yields four types of gifts: personalised gifts (low value to giver, high value to receiver); unique gifts (high value to both parties); culturally irrelevant gifts (high value to giver, low value to receiver) and fluff (low value to both parties). The essay hypothesises that polities that approach one another in a situation of contacts with low density will tend to aim for unique gifts, while polities whose relations are dense will aim for gifts that are of equal value to both parties.

A digital approach in the rapid response to COVID-19 – Experience of a paediatric institution

IntroductionCOVID-19 has radically changed the delivery of healthcare in Australia. Central to a tertiary paediatric institution’s (The Royal Children’s Hospital Melbourne (RCH) response was a digital health approach comprising a broad suite of informatics and technology solutions including optimising a fully integrated electronic medical record (EMR). MethodsThis comprehensive approach spanned all patient care areas and encompassed a broad range of hospital operations. They included patient triage, registration, COVID-19 screening clinic operations, electronic ordering, prescribing and documentation, telehealth, reporting and analytics and research. DiscussionThis paper outlines key aspects of our COVID-19 digital health strategy, highlighting the rapid transition to telehealth and the development of a remote “virtual telehealth” strategy for clinicians which proved popular and allowed true “working from home”. ConclusionCOVID-19 has inadvertently focussed the spotlight on the utility of digital health for clinical care. The speed and uptake of digital health within this pandemic has been remarkable and unprecedented in both an Australian and global setting. Whilst many of these changes have been beneficial, some may have been rushed or forced with minimal consideration of ongoing governance. Key stakeholders and enablers should be identified for post-pandemic consideration in future digital health implementation and adoption strategies.

Medical and dental characteristics of children with chromosome 22q11.2 deletion syndrome at the Royal Children's Hospital, Melbourne.

Chromosome 22q11.2 deletion syndrome (22q11.2DS) is a multifaceted syndrome with a variable phenotype. Few studies have described the associated dental characteristics and their relationship with medical co-morbidities; and no Australian data exist. To determine the clinical manifestations and correlations between oral and medical conditions in children with 22q11.2DS. A retrospective observational study. Children genetically diagnosed with 22q11.2DS at the Royal Children's Hospital Melbourne were selected; their medical and dental characteristics were collated and analysed. The study population (n=57; mean age 11.5years, range 2-27years) experienced a range of medical conditions involving multiple medical systems; of whom 44 (77.2%) had caries experience, 7 (12.3%) developmentally missing teeth, and 31 (54.4%) developmental defects of enamel (DDE). Smaller proportions of primary teeth were affected by DDE in children with congenital heart disease (2.2% vs 9.7%; P=.02), and cardiac surgery (0.2% vs 9%; P=.001). Conversely, children with hypoparathyroidism (n=2) had significantly higher proportions of primary teeth affected by DDE (27.5% vs 4%; P=.02). Significant associations existed between medical conditions (congenital heart disease, history of cardiac surgery, and hypoparathyroidism) and primary dentition DDE in children with 22q11.2 DS.

Outcomes of Zoledronic Acid Use in Paediatric Conditions

Introduction: Limited evidence is available concerning experience with use of zoledronic acid (ZA) and treatment for conditions other than primary bone fragility. Materials and Methods: A retrospective review of all Royal Children Hospital patients who had been administered at least 1 dose of intravenous ZA from 2002 to 2015 was undertaken. Results: The audit included 309 children with 228 being treated for bone fragility conditions. Of the 228, 68 had height-adjusted lumbar spine bone mineral density Z-scores available over up to a 5-year period, and median increases were +2.0 SD (median absolute deviation = 0.9) (N = 36, p value for median increase of at least 0.5 in Z-score <0.001), for patients with osteogenesis imperfecta or other primary bone fragility disorders, +1.0 SD (0.9) (N = 14, p = 0.029), for immobility conditions, +0.5 SD (0.7) (N = 10, p = 0.399), and for glucocorticoid-induced secondary osteoporosis, +0.7 SD (0.6) (N = 8, p = 0.015). 81/309 children were treated for bone abnormality indications (e.g., avascular necrosis [AVN], fibrous dysplasia, and bone cysts). Of 39 with AVN, outcome data were available for 33, with joint integrity maintained for 24/33 from 6 to 24 months after last ZA, subjective reports (22/28) of reduced pain. Reduction in bone lesion size was seen in 2/4 patients with bone cysts within 12 months of ZA commencement. Discussion/Conclusion: This is the largest cohort of reported outcomes of ZA use in a paediatric population. Results demonstrate a good efficacy profile and associated improved bone density for osteoporotic conditions and stabilization of non-traumatic AVN with a low rate of joint collapse.

Coaching While Waiting for Autism Spectrum Disorder Assessment: Protocol of a Pilot Feasibility Study for a Randomized Controlled Trial on Occupational Performance Coaching and Service Navigation Support.

BackgroundIn Australia, the average time between a first concern of autism spectrum disorder (ASD) and diagnosis is over 2 years. After referral for assessment, families often wait 6-12 months before their appointment. This can be a time of uncertainty and stress for families. For some families, other forms of assistance are not accessible and thus timely intervention opportunities are missed. There is little evidence about how to provide the best support for children or caregivers while on assessment waiting lists.ObjectiveThe aim of this study is to determine whether use of a coaching intervention called Occupational Performance Coaching (OPC) combined with service navigation support is feasible for families waiting for ASD assessment, as a crucial first step in planning a randomized controlled trial.MethodsA pilot and feasibility study will be conducted using recommended constructs and associated measures, which will be reported using CONSORT (Consolidated Standards or Reporting Trials) guidance. Participants will be child and caregiver dyads or triads, recruited within 4 months of their child (aged 1-7 years) being referred to one of two services for an ASD assessment in Victoria, Australia. A blinded randomization procedure will be used to allocate participants to one of three trial arms: (1) coaching and support intervention delivered face to face, (2) coaching and support intervention via videoconference, and (3) usual care. Descriptive statistics will be used to describe the sample characteristics of parents and children, inclusive of service access at baseline and follow up. Recruitment rates will be reported, and retention rates will be evaluated against a predicted rate of 70%-80% in each intervention arm. Goal attainment, using the Canadian Occupational Performance Measure, will indicate preliminary evidence for efficacy within the intervention arms, with an increase of 2 or more points on a 10-point performance and satisfaction scale considered clinically significant.ResultsThe study was approved by The Royal Children’s Hospital Research Ethics and Governance Department in September 2018. As of October 2020, 16 families have been recruited to the study. Data analysis is ongoing and results are expected to be published in 2021.ConclusionsStudy findings will support planning for a future randomized controlled trial to assess the efficacy of OPC and service navigation support for caregivers of children awaiting ASD assessment.Trial RegistrationAustralian New Zealand Clinical Trials Registry ACTRN12620000164998; www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=378793&isReview=trueInternational Registered Report Identifier (IRRID)DERR1-10.2196/20011

Examining Health-Related Quality of Life in Pediatric Cancer Patients with Febrile Neutropenia: Factors Predicting for Poor Recovery in Children and the Parents

Background: The impact of febrile neutropenia (FN) on the health-related quality of life (HRQoL) of children with cancer is poorly understood. We sought to characterise the course of child and parent HRQoL during and following FN episodes. Method: Data on HRQoL were collected in the multisite Australian PICNICC study. Multiple, discrete FN episodes per patient were allowed. HRQoL was assessed using the Child Health Utility (CHU9D) in children (n=167) and the Assessment of Quality of Life (AQoL-8D) in their parents (N=218) at three time points: 0-3 days, 7-days and 30-days following FN onset. Group-based trajectory modelling (GBTM) was used to characterise the course of HRQoL. Findings: For children, three distinct groups were identified: persistently low HRQoL over 30-days (chronic, 47%), increasing HRQoL from FN onset to 30-days (recovery, 22%), and persistently high HRQoL at all three timepoints (resilient, 32%). Applying these definitions, parents were classified into two distinct groups: chronic (49%) and resilient (51%).The child being male, having solid cancers, the presence of financial stress and relationship difficulties between the parent and child were significant predictors of chronic group membership for parents and children. Children classified as high-risk FN were significantly more likely to belong to the recovery group. Being female, having blood cancers and the absence of financial or relationship difficulties were predictive of being in the resilient group for both parents and children Interpretation: Approximately half the children and parents had chronically low HRQoL scores, which did not improve following resolution of the FN episode. The child’s sex, cancer type, and presence of financial and relationship stress were predictive of chronic group membership for both parents and children. These families may benefit from increased financial and psychosocial support during anti-cancer treatment. Trial Registration: The Australian PICNICC study is a prospective, multicentre, observational study (Australian New Zealand Clinical Trials Registry 12616001440415). Funding: This study was funded by a National Health and Medical Research Council (NHMRC) Project Grant (APP1104527). Declaration of Interest: GMH was supported by a Victorian Cancer Agency early career fellowship. FEB was part funded by a grant from the Royal Children's Hospital Foundation, Melbourne and the NHMRC. RP was funded by a Post-Doctoral Research Fellow grant from the NIHR, UK (PDF10872). MS has received grants from Merck, Gilead Sciences, F2G and Pfizer. KAT, RDAL, ZA and FM. and RP have no disclosures. Ethical Approval: This study utilised data from the PICNICC RCT, HREC Number (36040A, Royal Children’s Hospital Melbourne Human Research Ethics Committee) and ratified by the UTS HREC (ETH17-1128).

Impact of moderate and late preterm birth on neurodevelopment, brain development and respiratory health at school age: protocol for a longitudinal cohort study (LaPrem study)

IntroductionChildren born moderate to late preterm (MLP, 32–36 weeks’ gestation) account for approximately 85% of all preterm births globally. Compared with children born at term, children born MLP are at...

The Royal Wedding at Ebla: Commemorative Rite and the Birth of the Crown Prince

The Eblaite royal wedding ritual is preserved in two parallel versions: for Irkab-damu and Iš‛ar-damu, the last two kings (ARET XI 1, 2). A monthly and an annual administrative document register the goods given to the people who took part in the rite. The wedding was celebrated in nenaš, in the vicinity of the city, seat of the mausoleum of the ancestors, who had to bestow their blessing: the Mother-goddess declared that as from that moment “there is a new Kura (the city-god) and a new Barama (his paredra)”, so “there is a new king and a new queen”, (this although the king had already reigned for several years). Every year, in the same month, the benediction of the ancestors was renewed by a commemorative rite in nenaš. The king and queen had to wear a special garment: a maštāpu; the rite took place in the temple of Ganana, the goddess “who decrees the fate”. Every royal child (male and female), moreover, was presented at the temple of Rasap, at Ebla, but for the crown prince (at his birth) a rite was celebrated at the goddess’ temple in the same month as the wedding of his parents. The name of the goddess derives from*gnn, Heb., Aram. “to cover; to protect”, Akk. ganānum “to confine”. The goddess was not considered part of a couple with the Lord of Ganana, whose temple must be localized on the Euphrates, between the kingdom of Emar and the sanctuary of Dagan at Tuttul.

'A child drew the lots': Children and Youth Experiencing the English Civil War

This article revisits well-known English Civil War narratives in search of the recoverable fragments of the female and male child and youth experiences that formed part of the transgenerational household realities of this early modern conflict. This focus challenges implicit assumptions in the existing historiography that continue to champion adult, military, and socially elite experiences. The examples provided here discuss female and male youth experiences ranging from royal children to the children of ministers, and an artisan, rendering them more visible. This article is not intended to be a comprehensive account, but rather a challenge for historians to revisit the archives and sources from a child and youth perspective

Art amid crises

A diagnosis of cancer for a child or adolescent is a personal and familial crisis. It marks the start of treatment, an exhausting and emotional process, which can involve long periods in hospital. In 2020, young patients faced an additional challenge: the start of the COVID-19 pandemic. It is therefore with admiration and gratitude that we announce that the journal's covers for 2021 are by oncology patients at The Royal Children's Hospital (RCH) in Melbourne, Australia: art created amid these personal and global crises.

O okolicznościach narodzin królewskich dzieci w świetle itinerarium Kazimierza Jagiellończyka i Elżbiety Rakuskiej

O okolicznościach narodzin królewskich dzieci w świetle itinerarium Kazimierza Jagiellończyka i Elżbiety Rakuskiej

Tetralogy of Fallot—A literature review

AbstractTetralogy of Fallot is a combination of four specific cardiac abnormalities. (a) A ventricular septal defect (VSD), (b) pulmonary valve stenosis/right ventricular outflow tract obstruction, (c) aorta overriding the VSD, and (d) right ventricular hypertrophy. This review attempts to outline the current literature regarding embryology, morphology, pathophysiology, ultrasound appearances and the current treatments/outcomes of infants with Tetralogy of Fallot. Tetralogy of Fallot is the most commonly occurring conotruncal anomaly. Here, the aim is to present the ultrasound features, with examples of Tetralogy of Fallot along with the story post birth in terms of surgical treatment. This information was gathered doing a literature search using PubMed and the University of South Australia library database, as well as through information provided by the Royal Children's Hospital Cardiology department, via fact sheets. With early diagnosis under ultrasound, preparations can be made and counseling provided to organize for intervention and further assessment after birth. With current surgical intervention, the survival rate past the age of 10 is now over 92%, when, 50 years ago, it was only 50%.

Acute psychosocial care of families in paediatric resuscitation settings: Variables associated with parent emotional response

BackgroundFamily presence in paediatric resuscitation settings is now accepted practice. However, there is limited evidence to guide psychosocial care of these families. This study aimed to increase the understanding of family demographics and needs for psychosocial care at the Royal Children’s Hospital, Melbourne, Australia and to identify the factors that impact on families at this time of emotional and social distress. MethodA retrospective clinical data-mining methodology was utilised (n = 1123). Descriptive and inferential data analysis was undertaken using STATA software to identify population demographics, patterns in service usage and family experience, and to identify factors associated with parents’ emotional responses. FindingsFour areas were identified that support evidence-informed psychosocial care for families in paediatric resuscitation contexts: 1) family and parent dynamics 2) families’ experiences of distress in relation to patient diagnostic category 3) systemic nature of family response, and 4) the identification and analysis of family emotional support needs, which is central to the psychosocial response.

RARE-15. EARLY PSEUDOPROGRESSION POST-RADIATION IN PAEDIATRIC HIGH-GRADE GLIOMA PATIENTS WITH CONSTITUTIONAL MISMATCH REPAIR DEFICIENCY: TWO CASE REPORTS FROM A SINGLE CENTRE

BACKGROUNDConstitutional Mismatch Repair Deficiency (CMMRD) is a cancer predisposition syndrome caused by biallelic mutations in the mismatch repair pathway, and high-grade glioma (HGG) constitute the most prevalent brain tumours. Pseudoprogression alludes to radiological changes that mimic tumour progression, but are in fact due to other causes such as therapy related inflammation. It can occur as early as three months post treatment. To our knowledge, its characteristics in CMMRD patients has not been reported.METHODSWe retrospectively identified seven patients with CMMRD and history of HGG at The Royal Children’s Hospital, Melbourne from 2005 to 2019. Our objective was to review the characteristics of pseudoprogression in this cohort.RESULTSOut of the seven patients, two with constitutional loss of PMS2 demonstrated evidence of pseudoprogression. Patient 1 presented at 16 years old with a cerebellar anaplastic astrocytoma. She developed clinical and radiological progression within two weeks of starting radiotherapy, persisting up to four months after completion. However, six months post radiation she improved without intervention and the tumour remains stable five years post therapy. Patient 2 presented at 17 years old with a midbrain anaplastic astrocytoma, and showed signs of progression four weeks after completion of radiotherapy. She was then treated with Bevacizumab, an anti-VEGFA antibody with remarkable response. She subsequently received Nivolumab, a checkpoint inhibitor with ongoing stable disease for four months.CONCLUSIONOur findings showed that pseudoprogression can occur early in the treatment course in CMMRD patients. Identification of this entity is important for appropriate clinical management.