Radiographic Outcomes in Pediatric Bronchiectasis and Factors Associated with Reversibility.

Rationale: Conventionally considered irreversible, bronchiectasis has been demonstrated to be reversible in children in small studies. However, the factors associated with radiographic reversibility of bronchiectasis have yet to be defined. Objectives: In a large cohort of children with bronchiectasis, we aimed to determine: 1) if and to what extent bronchiectasis is reversible and 2) factors associated with radiographic chest high-resolution computed tomography (cHRCT) resolution. Methods: We identified children with bronchiectasis who had a repeat multidetector cHRCT scan between 2010 and 2021. We excluded those with cystic fibrosis, surgical pulmonary resection, traction bronchiectasis only, or lobar opacification. Measurements and Main Results: cHRCT scans were scored using the modified Reiff score (MRS) with a pediatric correction. Resolution was defined as an absence of abnormal bronchoarterial ratio (>0.8) on the second cHRCT scan. We included 142 children (median age, 5 years; IQR, 2.6-7.4). Inter- and intrarater agreement in MRSs was excellent (weighted κ = 0.83-0.86 and 0.95, respectively). There was radiographic resolution in 57 of 142 patients (40.1%), improvement in 56 of 142 (39.4%), and no change or worsening in 29 of 142 (20.4%). Pseudomonas aeruginosa (PsA) was absolutely associated with a lack of resolution. On multivariable regression, in those without PsA cultured, younger age at the time of diagnosis (risk ratio [RR], 0.94; 95% confidence interval [CI], 0.88-0.99), lower MRS (RR, 0.89; 95% CI, 0.82-0.97), and lower annual rate of exacerbations requiring intravenous antibiotic therapy (RR, 0.60; 95% CI, 0.37-0.98) increased the likelihood of radiographic resolution. Conclusions: This first large cohort confirms that bronchiectasis in children is often reversible with appropriate management. Younger children and those with lesser radiographic severity at diagnosis were most likely to exhibit radiographic reversibility, whereas those with PsA infection were least likely.

Diagnostic Accuracy of Point-of-Care Ultrasound Versus Radiographic Imaging for Pediatric Distal Forearm Fractures: A Randomized Controlled Trial.

STUDY OBJECTIVE: In patients aged 5 to 15 years with a clinically nondeformed distal forearm injury presenting to the emergency department (ED), we examined whether point-of-care ultrasound or radiographic imaging had better diagnostic accuracy, with the reference diagnosis determined by an expert panel review. METHODS: This multicenter, open-label, diagnostic randomized controlled trial was conducted in South East Queensland, Australia. Eligible patients were randomized to receive initial imaging through point-of-care ultrasound performed by an ED clinician or radiograph. Images were defined as "no," "buckle," or "other" fracture by the treating clinician. The primary outcome was the diagnostic accuracy of the treating clinician's interpretation compared against the reference standard diagnosis, which was determined retrospectively by an expert panel consisting of an emergency physician, pediatric radiologist, and pediatric orthopedic surgeon, who reviewed all imaging and follow-up. RESULTS: Two-hundred and seventy participants were enrolled, with 135 randomized to each initial imaging modality. There were 132 (97.8%) and 112 (83.0%) correctly diagnosed participants by ED clinicians in the point-of-care ultrasound and radiograph groups, respectively (absolute difference [AD]=14.8%; 95% confidence interval [CI] 8.0% to 21.6%; P<.001). Point-of-care ultrasound had better accuracy for participants with "buckle" fractures (AD=18.5%; 95% CI 7.1% to 29.8%) and "other" fractures (AD=17.1%; 95% CI 2.7% to 31.6%). No clinically important fractures were missed in either group. CONCLUSION: In children and adolescents presenting to the ED with a clinically nondeformed distal forearm injury, clinician-performed (acquired and interpreted) point-of-care ultrasound more accurately identified the correct diagnosis than clinician-interpreted radiographic imaging.

Ependymoma-like tumor with mesenchymal differentiation (ELTMD) with ZFTA:NCOA1 fusion: A diagnostic challenge.

Ependymal tumors are classified based on their location, histology, and molecular characteristics. Supratentorial ependymomas (ST-EPNs) are a group of circumscribed supratentorial gliomas, which usually have pathogenic fusions involving either zinc finger translocation associated (ZFTA) (formerly C11orf95) or YAP1. A subtype of ependymoma was recently described and labeled ependymoma-like tumors with mesenchymal differentiation (ELTMDs). We describe a case of a 5-year-old boy who presented with a right frontal tumor. The diagnosis was challenging, and a correct diagnosis could only be reached after reanalysis of methylation data with a more recent version of the classifier and RNA fusion testing, which revealed ZFTA:NCOA1 (nuclear receptor coactivator 1) fusion. There are only a handful of cases of this entity, which is being reported for its rarity and the diagnostic challenge it poses.

Dysphagia and Lung Disease in Children With Spinal Muscular Atrophy Treated With Disease-Modifying Agents.

OBJECTIVES: Disease-modifying agents (DMAs) for the treatment of spinal muscular atrophy (SMA) have evolved the SMA phenotype with improved survival. Ongoing oropharyngeal dysphagia and respiratory complications are reported. The extent of dysphagia and respiratory morbidity in this population, since DMAs' introduction, has not been well described. METHODS: A whole-population study involved all children with treated SMA types 1-3 in our facility. Videofluoroscopic swallow studies (type 1 alone), chest CT scans, and clinical data were collected. RESULTS: Thirty-six children were included (n = 9 type 1, n = 14 type 2, and n = 13 type 3; age range 0.3-15.4 years). Abnormal swallowing characteristics were demonstrated in all children with type 1 (n = 8; 100%). Bronchiectasis was found on chest CT: 3 of 9 (33.3%), 2 of 14 (14.3%), and 2 of 13 (15.4%) of type 1, 2, and 3, respectively. Atelectasis, mucus plugging, bronchial wall thickening, and parenchymal changes were common. DISCUSSION: Swallow impairments were universal in children with type 1. Bronchiectasis was common in all pediatric SMA types, with a prevalence of 1 in 5. Routine monitoring and management of dysphagia/recurrent respiratory infection should be implemented for improvement in lung health.

Navigating the glass labyrinth: Addressing gender diversity in Australian and New Zealand representative radiology leadership.

INTRODUCTION: Gender inequity among representative leadership in Clinical Radiology is a global issue, with the lack of gender diversity in leadership even more marked when compared to workforce representation. Women leaders face a disproportionate magnitude of seen and unseen challenges to leadership engagement and progression when compared to men (a 'glass labyrinth'), which is likely contributing to this discrepancy. The aim of the study was to examine and reflect on the state of gender diversity in representative leadership within the RANZCR Faculty of Clinical Radiology. METHODS: Review of the 2021 Royal Australian and New Zealand College of Radiologists (RANZCR) Board, Clinical Radiology Faculty Council and local Branch Committees with regard to the numbers of women and men in representative roles. RESULTS: In 2021, the RANZCR Board had no women representatives from Clinical Radiology (one woman representative from Radiation Oncology). 1/5 Clinical Radiology Faculty Council office Bearers were women. Local Branch Committees had 16.3% (13/80) women representatives. Three branches (the NSW, ACT and Victorian branches) had no women representatives. There were no women Committee Chairs, Branch Secretaries or Treasurers, with 2/7 Branch Education Officers women. CONCLUSION: The issues underpinning gender inequality in representative leadership are complex and diverse, resulting in disproportionate losses of women radiologists along the leadership pathway compared to men. Unconscious biases, including assumptions of inferior capability, capacity and credibility among women radiologist leaders, create unique challenges at organisational, institutional and personal levels. Change cannot be achieved by passive momentum alone: concrete initiatives and active engagement are required. To improve leadership diversity, strategies must be multifaceted and supported at an organisational level.

Posterior fossa pilocytic astrocytoma presenting with opisthotonus in an infant - A case report.

Opisthotonus as a presenting feature in neurosurgical patients is rare, with few reports describing such presentations. Only four reports of opisthotonos secondary to posterior fossa mass were identified. An unclear pathophysiology, and broad aetiology contribute to clinical misdirection. While posterior fossa lesions commonly present with signs of raised intracranial pressure, or cerebellar dysfunction, this case describes the presentation of an infant with opisthotonic posturing, ataxia and autonomic dysfunction secondary to a large pilocytic astrocytoma. Despite initial treatment of hydrocephalus, opisthotonus only resolved with complete surgical resection of the posterior fossa mass. At follow-up, the child remains well and without signs of hypertonicity or other signs or symptoms. Presentations involving opisthotonus are rare, and active exclusion of posterior fossa pathology is necessary. In this case, urgent surgical resection allowed for a positive patient outcome. Description of such a case may contribute to understanding of similar presentations in the neurosurgical context.