Avoiding skull radiographs in infants with suspected inflicted injury who also undergo head CT : “a no-brainer?”

Objectives To assess whether head CT with 3D reconstruction can replace skull radiographs (SXR) in the imaging investigation of suspected physical abuse (SPA)/abusive head trauma (AHT). Methods PACS was interrogated for antemortem skeletal surveys performed for SPA, patients younger than 2 years, SXR and CT performed within 4 days of each other. Paired SXR and CT were independently reviewed. One reviewer analysed CT without and (3 months later) with 3D reconstructions. SXR and CT expert consensus review formed the gold standard. Observer reliability was calculated. Results A total of 104 SXR/CT examination pairs were identified, mean age 6.75 months (range 4 days to 2 years); 21 (20%) had skull fractures; two fractures on CT were missed on SXR. There were no fractures on SXR that were not seen on CT. For SXR and CT, respectively: PPV reviewer 1, 95% confidence interval (CI) 48–82% and 85–100%; reviewer 2, 67–98% and 82–100%; and NPV reviewer 1, 95%, CI 88–98% and 96–100%; reviewer 2, 88–97% and 88–98%. Inter- and intra-observer reliability were respectively the following: SXR, excellent (kappa = 0.831) and good (kappa = 0.694); CT, excellent (kappa = 0.831) and perfect (kappa = 1). All results were statistically significant (p < 0.001). Conclusions CT has greater diagnostic accuracy than SXR in detecting skull fractures which is increased on concurrent review of 3D reconstructions and should be performed in every case of SPA/AHT. SXR does not add further diagnostic information and can be omitted from the skeletal survey when CT with 3D reconstruction is going to be, or has been, performed

Outcomes of percutaneous vertebroplasty in multiple myeloma: a tertiary neurosciences experience with long-term follow-up

Background: Multiple myeloma is diagnosed in 5,800 people in the United Kingdom (UK) each year with up to 64% having vertebral compression fractures at the time of diagnosis. Painful vertebral compression fractures can be of significant detriment to patients’ quality of life. Percutaneous vertebroplasty aims to provide long-term pain relief and stabilize fractured vertebrae. Methods and materials: Data was collected from all cases of percutaneous vertebroplasty performed on patients with multiple myeloma from November 2017 to January 2019. Pain scores were measured using the Visual Analogue Scale (VAS) and Oswestry Disability Index (ODI) pre-procedure, 2 months post procedure and 4 years post-procedure. Procedure related complications and analgesia use were also documented. Results: 22 patients were included with a total of 119 vertebrae treated. Patients reported a significant improvement in overall pain score with a median pre-procedure VAS of 8 and a median post-procedure VAS of 3.5 (p<0.0001). There was a median pre-procedure ODI score of 60% and a median post-procedure ODI score of 36% (p<0000.1). There was improvement across all ODI domains and a 77% reduction in analgesic requirement. There were small cement leaks into paravertebral veins or endplates at 15 levels (12%) which were asymptomatic. There were 8 responders to the long-term follow-up questionnaire at 4 years. This demonstrated an overall stable degree of pain relief in responders with a median VAS of 3.5 and median ODI of 30%. Conclusion: At this center, vertebroplasty has been shown to reduce both VAS and ODI pain scores and reduce analgesia requirements in patients with VCFs secondary to multiple myeloma with long lasting relief at 4 years post-procedure

The (extended) achondroplasia foramen magnum score has good observer reliability

Background Achondroplasia is the most common skeletal dysplasia. A significant complication is foramen magnum stenosis. When severe, compression of the spinal cord may result in sleep apnea, sudden respiratory arrest and death. To avoid complications, surgical decompression of the craniocervical junction is offered in at-risk cases. However, practice varies among centres. To standardize magnetic resonance (MR) reporting, the achondroplasia foramen magnum score was recently developed. The reliability of the score has not been assessed. Objective To assess the interobserver reliability of the achondroplasia foramen magnum score. Materials and methods Base of skull imaging of children with achondroplasia under the care of Sheffield Children’s Hospital was retrospectively and independently reviewed by four observers using the achondroplasia foramen magnum score. Two-way random-effects intraclass coefficient (ICC) was used to assess inter- and intra-observer reliability. Results Forty-nine eligible cases and five controls were included. Of these, 10 were scored normal, 17 had a median score of 1 (mild narrowing), 11 had a median score of 2 (effacement of cerebral spinal fluid), 10 had a score of 3 (compression of cord) and 6 had a median score of 4 (cord myelopathic change). Interobserver ICC was 0.72 (95% confidence interval = 0.62–0.81). Intra-observer ICC ranged from 0.60 to 0.86. Reasons for reader disagreement included flow void artefact, subtle T2 cord signal and myelopathic T2 cord change disproportionate to canal narrowing. Conclusion The achondroplasia foramen magnum score has good interobserver reliability. Imaging features leading to interobserver disagreement have been identified. Further research is required to prospectively validate the score against clinical outcomes

Initial experience of an investigational 3T MR scanner designed for use on neonatal wards

OBJECTIVES: MR imaging of neonates is difficult for many reasons and a major factor is safe transport to the MR facilities. In this article we describe the use of a small, investigational 3-T MR customised for brain imaging and sited on a neonatal unit of a tertiary centre in the UK, which is in contrast to a 300-m journey to the whole-body MR scanner used at present for clinical cases. METHODS: We describe our methods for preparing babies for safe transport and scanning on an investigational 3-T MR scanner on a neonatal unit and the development of appropriate MR sequences. The MR scanner does not have CE marking at present so this early development work was undertaken on normal neonates whose parents consented to a research examination. RESULTS: Fifty-two babies were scanned and there were no serious adverse events. The MR examinations were considered to be diagnostically evaluable in all 52 cases and in 90% the imaging was considered to be at least as good as the quality obtained on the 1.5-T scanner currently used for clinical cases. CONCLUSION: We have shown that this investigational 3-T MR scanner can be used safely on a neonatal unit and we have refined the MR sequences to a point that they are clinically usable. KEY POINTS: • Access to neonatal MR imaging is limited. • We describe an investigational 3-T MR scanner site on a neonatal unit. • The scanner produces images suitable for clinical practice

Definition and recommendations for advance care planning: an international consensus supported by the European Association for Palliative Care

Advance care planning (ACP) is increasingly implemented in oncology and beyond, but a definition of ACP and recommendations concerning its use are lacking. We used a formal Delphi consensus process to help develop a definition of ACP and provide recommendations for its application. Of the 109 experts (82 from Europe, 16 from North America, and 11 from Australia) who rated the ACP definitions and its 41 recommendations, agreement for each definition or recommendation was between 68–100%. ACP was defined as the ability to enable individuals to define goals and preferences for future medical treatment and care, to discuss these goals and preferences with family and health-care providers, and to record and review these preferences if appropriate. Recommendations included the adaptation of ACP based on the readiness of the individual; targeting ACP content as the individual's health condition worsens; and, using trained non-physician facilitators to support the ACP process. We present a list of outcome measures to enable the pooling and comparison of results of ACP studies. We believe that our recommendations can provide guidance for clinical practice, ACP policy, and research

Anatomical subgroup analysis of the MERIDIAN cohort: failed commissuration

Objective: To assess the contribution of in utero magnetic resonance (iuMR) imaging in fetuses diagnosed with either agenesis of the corpus callosum or hypogenesis of the corpus callosum (grouped as failed commissuration) on antenatal ultrasonography (USS) from the MERIDIAN cohort. Methods: We report a sub-group analysis of fetuses with failed commissuration diagnosed on USS (with or without ventriculomegaly) from the MERIDIAN study who had iuMR imaging within 2 weeks of USS and outcome reference data were available. The diagnostic accuracy of USS and iuMR are reported as well as indicators of diagnostic confidence and effects on prognosis/clinical management. Results: 79 fetuses with failed commissuration are reported (55 with agenesis and 24 with hypogenesis as the USS diagnoses). The diagnostic accuracy for detecting ‘failed commissuration’ as a group label was 34.2% for USS and 94.9% for iuMR (difference = 60.7%, 95% confidence interval 47.6% to 73.9%, p < 0.0001). The diagnostic accuracy for detecting hypogenesis of the corpus callosum as a discrete entity was 8.3% for USS and 87.5% for iuMR whilst the diagnostic accuracy for detecting agenesis of the corpus callosum as a distinct entity was 40.0% for USS and 92.7% for iuMR. There was a statistically significant improvement in ‘appropriate’ diagnostic confidence when using iuMR imaging as assessed by a score-based weighted average’ method (p < 0.0001). Prognostic information given to the women changed in 36/79 (45.6%) cases after iuMR imaging and its overall effect on clinical management was ‘significant’, ‘major’ or ‘decisive’ in 35/79 cases (44.3%). Conclusions: Our data suggests that any woman whose fetus has failed commissuration as the only intracranial finding detected on USS should have iuMR imaging for further evaluation

Rationale, design, and methods of a randomized, controlled, open-label clinical trial with open-label extension to investigate the safety of vosoritide in infants, and young children with achondroplasia at risk of requiring cervicomedullary decompression surgery

Achondroplasia causes narrowing of the foramen magnum and the spinal canal leading to increased mortality due to cervicomedullary compression in infants and significant morbidity due to spinal stenosis later in adulthood. Vosoritide is a C-natriuretic peptide analogue that has been shown to improve endochondral ossification in children with achondroplasia. The objective of this trial is to evaluate the safety of vosoritide and whether vosoritide can improve the growth of the foramen magnum and spinal canal in children that may require decompression surgery. An Achondroplasia Foramen Magnum Score will be used to identify infants at risk of requiring decompression surgery. This is a 2-year open label randomized controlled trial of vosoritide in infants with achondroplasia ages 0 to ≤12 months. Approximately 20 infants will be randomized 1:1 to either open label once daily subcutaneous vosoritide combined with standard of care or standard of care alone. The primary and secondary aims of the study are to evaluate the safety and efficacy of vosoritide in children with cervicomedullary compression at risk of requiring decompression surgery. The trial will be carried out in specialized skeletal dysplasia treatment centers with well established multidisciplinary care pathways and standardized approaches to the neurosurgical management of cervicomedually compression. After 2 years, infants randomized to standard of care alone will be eligible to switch to vosoritide plus standard of care for an additional 3 years. This pioneering trial hopes to address the important question as to whether treatment with vosoritide at an early age in infants at risk of requiring cervicomedullary decompression surgery is safe, and can improve growth at the foramen magnum and spinal canal alleviating stenosis. This in turn may reduce compression of surrounding structures including the neuraxis and spinal cord, which could alleviate future morbidity and mortality

A Qualitative Comparison of Arterial Spin Labelling and Dynamic Susceptibility Contrast MRI in 52 Children with a Range of Neurological Conditions

OBJECTIVE: To assess the usefulness of Arterial Spin Labelling (ASL) compared with Dynamic Susceptibility Contrast (DSC) perfusion MRI for typical paediatric neuroimaging applications at 1.5 Tesla. METHODS: 52 children (age: 4 months – 17 years) with a variety of neurological disorders were scanned using 3D ASL and Echo Planar Imaging DSC sequences. All images were reviewed by an experienced neuroradiologist, image quality recorded as ‘good’, ‘acceptable’, or ‘poor’ and diagnostic value noted as being ‘greater’, ‘similar’ or ‘less’ for ASL when compared to DSC. RESULTS: ASL CBF images were judged to be acceptable in 89% of cases, poor in 11% and good in 0%, while DSC CBF images were acceptable in 88% of cases, poor in 12% and good in 0%. ASL images were judged to have better diagnostic value than DSC in 28% of cases, about the same in 58%, and worse in 14%. CONCLUSION: The results of this study suggest that ASL offers a realistic alternative to DSC in the paediatric setting for the majority of cases encountered in this study. However, there are some situations where DSC outperforms ASL, so care is required to choose the most appropriate technique for the pathology under investigation. A larger study is required to corroborate these preliminary findings. ADVANCES IN KNOWLEDGE: ASL is a relatively new perfusion imaging technique that’s use has not been explored extensively in the paediatric setting. This work is a preliminary study to evaluate its usefulness in paediatric neuroimaging

A review of fetal brain pathology acquired in utero

In utero MR imaging is used increasingly often to assist with the diagnosis of fetal brain abnormalities, and this increased use is supported by proved improvements in diagnostic accuracy compared with sonography. Fetal brain pathology may be considered developmental or acquired, though these definitions are simplistic, and there may be overlap between the 2 entities. We presented an analysis of the cases from the MERIDIAN (MR imaging to enhance the diagnosis of fetal developmental brain abnormalities in utero) cohort that fit our definitions of acquired pathologies and provided a pictorial review of the typical imaging appearances as well as provided prevalence data on acquired brain pathology