Intussusception reduction methods in daily practice-a survey by the European Society of Paediatric Radiology Abdominal Imaging Taskforce.

BACKGROUND: Image-guided intussusception reduction has been practised internationally for many decades. The use of different modalities, delayed repeat attempts, and sedation/anaesthesia are unknown. OBJECTIVE: To survey the practice of image-guided intussusception reduction. MATERIALS AND METHODS: A 20-point questionnaire created by the European Society of Paediatric Radiology (ESPR) Abdominal Imaging Taskforce was distributed via the ESPR members' mailing list and shared on social media between 28 March and 1 May 2023. RESULTS: There were 69 responses from 65 worldwide institutions, with a mean of 18 intussusception reductions performed per year: 55/69 (80%) from 52 European institutions and 14/69 (20%) from 13 institutions outside of Europe. European centres reported using 19/52 (37%) fluoroscopy, 18/52 (35%) ultrasound, and 15/52 (28%) a mixture of both, with 30/52 (58%) offering a delayed repeat at 15 min to 24 h. Non-European centres reported using 5/13 (39%) fluoroscopy, 6/13 ultrasound (46%), and 2/13 (15%) a mixture of both, with 9/13 (69%) offering a delayed repeat attempt. Sedation or analgesia was used in 35/52 (67%) of European and 2/13 (15%) non-European institutions. CONCLUSION: There is wide variation in how image-guided intussusception reduction is performed, and in the use of sedation/anaesthesia.

Automated segmentation of magnetic resonance bone marrow signal: a feasibility study.

BACKGROUND: Manual assessment of bone marrow signal is time-consuming and requires meticulous standardisation to secure adequate precision of findings. OBJECTIVE: We examined the feasibility of using deep learning for automated segmentation of bone marrow signal in children and adolescents. MATERIALS AND METHODS: We selected knee images from 95 whole-body MRI examinations of healthy individuals and of children with chronic non-bacterial osteomyelitis, ages 6-18 years, in a longitudinal prospective multi-centre study cohort. Bone marrow signal on T2-weighted Dixon water-only images was divided into three color-coded intensity-levels: 1 = slightly increased; 2 = mildly increased; 3 = moderately to highly increased, up to fluid-like signal. We trained a convolutional neural network on 85 examinations to perform bone marrow segmentation. Four readers manually segmented a test set of 10 examinations and calculated ground truth using simultaneous truth and performance level estimation (STAPLE). We evaluated model and rater performance through Dice similarity coefficient and in consensus. RESULTS: Consensus score of model performance showed acceptable results for all but one examination. Model performance and reader agreement had highest scores for level-1 signal (median Dice 0.68) and lowest scores for level-3 signal (median Dice 0.40), particularly in examinations where this signal was sparse. CONCLUSION: It is feasible to develop a deep-learning-based model for automated segmentation of bone marrow signal in children and adolescents. Our model performed poorest for the highest signal intensity in examinations where this signal was sparse. Further improvement requires training on larger and more balanced datasets and validation against ground truth, which should be established by radiologists from several institutions in consensus.

Radiologic follow-up in Fontan-associated liver disease in Europe: European Society of Paediatric Radiology survey demonstrates the need for a consensus protocol.

Fontan surgery is a life-saving procedure for newborns with complex cardiac malformations, but it originates complications in different organs. The liver is also affected, with development of fibrosis and sometimes cirrhosis and hepatocellular carcinoma. There is no general agreement on how to follow-up these children for the development of liver disease. To understand the current practice on liver follow-up, we invited members of the European Society of Paediatric Radiology (ESPR) to fill out an online questionnaire. The survey comprised seven questions about when and how liver follow-up is performed on Fontan patients. While we found some agreement on the use of US as screening tool, and of MRI for nodule characterization, the discrepancies on timing and the lack of a shared protocol make it currently impossible to compare data among centers.

Spectrum of Fontan-associated liver disease assessed by MRI and US in young adolescents.

PURPOSE: Patients with Fontan circulation are at risk of developing hepatic fibrosis/cirrhosis. The mechanisms and disease development are unclear and early secondary liver cancer is a concern. This study will describe hepatic imaging findings in a national cohort of adolescents with Fontan circulation. METHODS: The patients prospectively underwent abdominal contrast enhanced magnetic resonance imaging (MRI) including diffusion-weighted imaging. Images were assessed for criteria of fibrosis/cirrhosis including characterization of hepatic nodules. These nodules were in addition, assessed by ultrasonography (US). Nodules ≥ 1 cm were investigated and monitored to evaluate malignant transformation. Clinical and hepatic serological data were recorded. RESULTS: Forty-six patients, median age of 16.5 years (15.4-17.9 years) were enrolled. All patients underwent US examination and MRI was performed in 35/46 patients. On MRI, 60% had hepatomegaly and 37% had signs of fibrosis/cirrhosis. Seven patients had together 13 nodules ≥ 1 cm in diameter. Only 4/13 (17%) where seen on US. Nodules had variable MRI signal characteristics including hepatobiliary contrast enhancement and two nodules revealed portal venous phase 'wash-out' on the first examination. No further imaging signs of malignancy were revealed during the follow-up period of median 24.4 (7-42) months. CONCLUSION: The majority of adolescents with Fontan circulation had imaging findings of fibrosis/cirrhosis of varying severity. US had low detection rate of hepatic nodules compared to MRI. The imaging work-up before transition to adult cardiology care did not reveal findings suggestive of malignancy. However, the high prevalence of Fontan-associated liver disease calls for surveillance strategies even in childhood.

Revisiting the radiographic assessment of osteoporosis-Osteopenia in children 0-2 years of age. A systematic review.

BACKGROUND: Imaging for osteoporosis has two major aims, first, to identify the presence of low bone mass (osteopenia), and second, to quantify bone mass using semiquantitative (conventional radiography) or quantitative (densitometry) methods. In young children, densitometry is hampered by the lack of reference values, and high-quality radiographs still play a role although the evaluation of osteopenia as a marker for osteoporosis is subjective and based on personal experience. Medical experts questioned in court over child abuse, often refer to the literature and state that 20-40% loss of bone mass is warranted before osteopenia becomes evident on radiographs. In our systematic review, we aimed at identifying evidence underpinning this statement. A secondary outcome was identifying normal references for cortical thickness of the skeleton in infants born term, < 2 years of age. METHODS: We undertook systematic searches in Medline, Embase and Svemed+, covering 1946-2020. Unpublished material was searched in Clinical trials and International Clinical Trials Registry Platform (ICTRP). Both relevant subject headings and free text words were used for the following concepts: osteoporosis or osteopenia, radiography, children up to 6 years. RESULTS: A total 5592 publications were identified, of which none met the inclusion criteria for the primary outcome; the degree of bone loss warranted before osteopenia becomes visible radiographically. As for the secondary outcome, 21 studies were identified. None of the studies was true population based and none covered the pre-defined age range from 0-2 years. However, four studies of which three having a crossectional and one a longitudinal design, included newborns while one study included children 0-2 years. CONCLUSIONS: Despite an extensive literature search, we did not find any studies supporting the assumption that a 20-40% bone loss is required before osteopenia becomes visible on radiographs. Reference values for cortical thickness were sparse. Further studies addressing this important topic are warranted.

Progressive loss of bone mass in children with Fontan circulation.

OBJECTIVE: We investigated bone mineral density (BMD) at different ages after the Fontan completion, and we evaluated the relationship between BMD, vitamin D levels, and pertinent patient variables. METHODS: A cross-sectional sample of 64 patients was examined with dual-energy X-ray absorptiometry (DXA) scans to determine BMD. Of these patients, 24 were also examined with BoneXpert software to determine bone mass density (BMX), expressed as the bone health index (BHI). Blood samples from all patients were analyzed. Patients were divided into three different age groups; A: 4-9 years old (n = 22), B: 10-15 years old (n = 21), and C: 16-18 years old (n = 21). RESULTS: Overall, BMD z scores were (mean ± SD): -1.0 ± 1.3 for the lumbar spine and -0.2 ± 1.2 for the total body. Groups B and C had significantly lower z score values compared to group A. Of patients in group C, 35% had z score values ≤-2 SD of the mean of the healthy population. There was no difference related to systemic ventricular anatomy (left or right); however, patients with lateral tunnels had lower BMD than patients with extra cardiac conduits. Overall, the BHI z score was (mean ± SD): -1.2 ± 0.9, but low BMX did not correlate with low BMD. The 25-hydroxy vitamin D level was 58 ± 30 nmol/L. Vitamin D levels decreased with age: in group C, 33.3% of patients exhibited vitamin D deficiencies. Vitamin D levels were not correlated with bone mineral densities. CONCLUSION: BMD levels decreased with age in patients with Fontan circulation. Different bone components were involved. Vitamin D levels also decreased with age, but they were not consistently associated with bone mineral densities. The single factor most predictive of low BMD was a lateral tunnel Fontan, compared to an extra cardiac Fontan.

Aggressive melanoma in an infant with congenital melanocytic nevus syndrome and multiple, NRAS and BRAF mutation-negative nodules.

We report the case of a newborn boy with multinodular NRAS and BRAF mutation-negative congenital melanocytic nevi and cerebral lesions compatible with congenital intraparenchymal melanosis. Histopathology from skin lesions showed atypical nodular melanocytic proliferation with marked melanocytic atypia and a large number of mitoses and apoptosis, indicating aggressive proliferation. The child developed several new subcutaneous tumors and multiple internal lesions, which were confirmed to be metastases, and died at 5 months of age. This case may represent an infantile melanoma developing from a giant congenital melanocytic nevus or a congenital melanoma.

Current status of wrist imaging in juvenile idiopathic arthritis.

Wrist involvement occurs in about one-quarter of patients diagnosed with juvenile idiopathic arthritis (JIA), increasing to 40% 5 years after diagnosis. The imaging appearances, both for active inflammation and permanent change, differ from those seen in adult rheumatoid arthritis; therefore, a child-specific approach is crucial for correct assessment. In this review article, we provide an update on the current status for imaging wrist JIA, with a focus on evidence-based practice.

Normative ultrasound references for the paediatric wrist; dorsal soft tissues.

OBJECTIVE: To report novel ultrasound (US) references for the healthy paediatric wrist. METHODS: Healthy children and adolescents had an US examination including Doppler, of the right wrist, using a mid-sagittal image through the radiocarpal (RC)/midcarpal (MC) joints. These features were noted: appearances and depth of the recess, with an additional measurement in the flexed position and number of Doppler signals within the recess in close proximity to the joint. In a second, blinded session, all images were reassessed by the same observer. RESULTS: In total, 116 subjects (59 girls) aged 6-16 years (mean 10 years and 11 months) were included. The RC recess was visible in 71, of which 60 were non-bulging and 11 were bulging. In flexion, the thickness decreased in 60 (84.5%), suggestive of joint fluid. Of the 54 visible MC recesses, 45 (83.3%) thinned in flexion. The mean depths of the RC and MC recesses were 0.4 mm (SD 0.5, range 0-2.9) and 0.5 mm (SD 0.6, range 0-2.4), respectively, with no differences according to sex, but with increasing depths by age.For the RC joints, at least one Doppler signal was seen in the recess in 9/116 joints (7.8%), whereas this was true for 3.5% of the MC recesses. There were no statistically significant association between the depth of the RC or MC recesses and number of hand active sport activities (P=0.733 and P=0.091, respectively). CONCLUSION: The appearances of the dorsal RC and MC recesses in healthy children may mimic pathology. Hence, findings should be interpreted carefully.

The many shades of enhancement: timing of post-gadolinium images strongly influences the scoring of juvenile idiopathic arthritis wrist involvement on MRI.

BACKGROUND: Potential long-term side effects of treatment for juvenile idiopathic arthritis are concerning. This has necessitated accurate tools, such as MRI, to monitor treatment response and allow for personalized therapy. OBJECTIVE: To examine the extent to which timing of post-contrast MR images influences the scoring of inflammatory change in the wrist in children with juvenile idiopathic arthritis. MATERIALS AND METHODS: We studied two sets of post-contrast 3-D gradient echo MRI series of the wrist in 34 children with juvenile idiopathic arthritis. These images were obtained immediately after administration of intravenous contrast material and again after approximately 10 min. The dataset was drawn from a prospective multicenter project conducted 2006-2010. We assessed five wrist locations for synovial enhancement, effusion and overall inflammation. Examinations were scored by one radiologist in two sessions - the first was based on the early post-contrast images, and the later session, for which the previous findings were masked, was based on the later post-contrast images. RESULTS: Fifty-two of the 170 locations (30.6%) received a higher synovial enhancement score based on the late post-contrast images as compared to the early images. Sixty of the 170 (35%) locations received a higher total inflammation score. The mean scores of synovial enhancement and total inflammation were significantly higher when based on the late post-contrast images as compared to the early post-contrast images. CONCLUSION: An MRI-based scoring system for the presence and degree of synovitis should be based on a standardized MR-protocol with a fixed interval between intravenous contrast injection and post-contrast images.

Carpal erosions in children with juvenile idiopathic arthritis: repeatability of a newly devised MR-scoring system.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is characterized by synovial inflammation, with potential risk of developing progressive joint destruction. Personalized state-of-the-art treatment depends on valid markers for disease activity to monitor response; however, no such markers exist. OBJECTIVE: To evaluate the reliability of scoring of carpal bone erosions on MR in children with JIA using two semi-quantitative scoring systems. MATERIALS AND METHODS: A total of 1,236 carpal bones (91 MR wrist examinations) were scored twice by two independent pediatric musculoskeletal radiologists. Bony erosions were scored according to estimated bone volume loss using a 0-4 scale and a 0-10 scale. An aggregate erosion score comprising the sum total carpal bone volume loss was calculated for each examination. RESULTS: The 0-4 scoring system resulted in good intra-reader agreement and moderate to good inter-observer agreement in the assessment of individual bones. Fair and moderate agreement were achieved for inter-reader and intra-reader agreement, respectively, using the 0-10 scale. Intra- and particularly inter-reader aggregate score variability were much less favorable, with wide limits of agreement. CONCLUSION: Further analysis of erosive disease patterns compared with normal subjects is required, and to facilitate the development of an alternative means of quantifying disease.

Rare MLL-ELL fusion transcripts in childhood acute myeloid leukemia-association with young age and myeloid sarcomas?

BACKGROUND: The chromosomal translocation t(11;19)(q23;p13) with a breakpoint within subband 19p13.1 is found mainly in acute myeloid leukemia (AML) and results in the MLL-ELL fusion gene. Variations in the structure of MLL-ELL seem to influence the leukemogenic potency of the fusion in vivo and may lie behind differences in clinical features. The number of cases reported so far is very limited and the addition of more information about MLL-ELL variants is essential if the possible clinical significance of rare fusions is to be determined. CASE PRESENTATION: Cytogenetic and molecular genetic analyses were done on the bone marrow cells of a 20-month-old boy with an unusual form of myelomonocytic AML with multiple myeloid sarcomas infiltrating bone and soft tissues. The G-banding analysis together with FISH yielded the karyotype 47,XY, +6,t(8;19;11)(q24;p13;q23). FISH analysis also demonstrated that MLL was split. RNA-sequencing showed that the translocation had generated an MLL-ELL chimera in which exon 9 of MLL (nt 4241 in sequence with accession number NM_005933.3) was fused to exon 6 of ELL (nt 817 in sequence with accession number NM_006532.3). RT-PCR together with Sanger sequencing verified the presence of the above-mentioned fusion transcript. CONCLUSIONS: Based on our findings and information on a few previously reported patients, we speculate that young age, myelomonoblastic AML, and the presence of extramedullary disease may be typical of children with rare MLL-ELL fusion transcripts.

Ultrasound of the paediatric urogenital tract.

Pathology in the urinary tract is one of the most frequent queries when children are referred for an ultrasound examination. Comprehensive ultrasound examinations can answer most clinical questions of the urogenital tract with minimal patient preparation and without the use of ionising radiation. Therefore, optimised imaging protocols should be available in all radiology departments where children are examined. This review suggests a preferred imaging protocol for urogenital imaging in children and gives an overview of the different structures of the urogenital tract, the normal age-related sonographic anatomy, and gives examples of the most commonly encountered diseases of the urogenital system in children.

MRI assessment of tenosynovitis in children with juvenile idiopathic arthritis: inter- and intra-observer variability.

BACKGROUND: There is sparse knowledge about grading tenosynovitis using MRI. OBJECTIVE: The purpose of this study was to assess the reliability of a tenosynovitis MRI scoring system in juvenile idiopathic arthritis. MATERIALS AND METHODS: Children with juvenile idiopathic arthritis and wrist involvement were enrolled in two paediatric centres, from October 2006 to January 2010. The extensor (compartments II, IV and VI) and flexor tendons were assessed for the presence of tenosynovitis on T1-weighted postcontrast fat-saturated MR images and were scored from 0 (normal) to 2 (moderate to severe) by two observers independently. Intra- and interobserver agreement was assessed. RESULTS: Ninety children (age range: 5-18.5 years) were included, of whom 34 had tenosynovitis involving extensors and 28 had tenosynovitis involving flexors. A total of 360 tendon areas were analysed, of which 114 had tenosynovitis (86/270 extensors and 28/90 flexors). Intra-reader 1 agreement was excellent for the extensors (k = 0.82-0.91) and for the flexors (k = 0.85); intra-reader 2 agreement was moderate to good for the extensors (k = 0.51-0.72) and good for the flexors (k = 0.64). Inter-reader agreement was good for the extensors (k = 0.69-0.73) and moderate for the flexors (k = 0.49). CONCLUSION: The proposed MRI scoring system for the assessment of wrist tenosynovitis in juvenile idiopathic arthritis appears feasible with an observer agreement sufficient for clinical use.

The paediatric wrist revisited: redefining MR findings in healthy children.

OBJECTIVES: During a multicentre study on juvenile idiopathic arthritis, wide variations were observed in bone shape, signal intensity and volume of joint fluid as shown by MRI which in part appeared to be unrelated to disease activity. A study was undertaken to examine these features in a cohort of healthy children. METHODS: 88 children of mean age 9.8 years (range 5-15) underwent MRI imaging (T1-weighted Spin Echo and Spectral Selection Attenuated Inversion Recovery (SPAIR)) of the left wrist. The number of bony depressions, distribution and amount of joint fluid and the presence of bone marrow changes were assessed. RESULTS: Bony depressions were present in all children, increasing with age from a mean of 4.0 in children aged 4-6 years to 9.2 in those aged 12-15 years (p<0.001)). 45 of 84 children (53.6%) had a high signal on SPAIR with a corresponding low signal on T1 in at least one bone. No associations were seen between bone marrow change (present or not) and sex (p=0.827) or sports club membership (p=0.616). All children had visible joint fluid in at least one of the joints assessed. No associations were seen between the presence of joint fluid and age group, except for the radius/scaphoid and capitate-scaphoid joints and a recess lateral to the hamate. CONCLUSIONS: It is important to be aware of the high prevalence of bony depressions, signal changes suggestive of bone marrow oedema and the volume of joint fluid seen in normal children. Such findings must be interpreted with care in children with suspected disease such as juvenile arthritis.

Unilateral adrenal haemorrhage following systemic thrombolysis.

We report a case of unilateral adrenal bleeding, worsened or initiated by systemic thrombolytic therapy given for a suspected myocardial infarction. Initial clinical diagnosis was ruptured aortic aneurysm or aortic dissection. A preoperative contrast-enhanced computer tomography (CT) scan showed a possible bleeding from the left adrenal gland. An emergency left subcostal retroperitoneal approach revealed a ruptured and bleeding adrenal gland and its arteries were ligated.