Can the Third Part of Duodenum Behind SMA Be Detected With Confidence on CT as a Proposed Mechanism for Imaging Suspected Malrotation in Children? A Preliminary Proof of Concept Study.

AIM: The aims of this study were to determine frequency and reliability of computed tomography (CT) detection of anatomic landmarks for imaging suspected midgut malrotation in infants and children, and to calculate an estimated effective dose of an upper abdominal CT scan in our patient population. MATERIALS AND METHODS: Fifty consecutive pediatric patients who underwent a CT scan that included their upper abdomen between August 2016 and February 2018 were included. Four pediatric radiology consultants independently reviewed CT scans for detection of the third part of the duodenum and defined their confidence level of this through identification of continuity with the pyloric antrum, D1, D2, and D4 components of the duodenum, as well as the duodenojejunal flexure.Interobserver variability was assessed using Fleiss κ for agreement. A dose estimate, per scan, was calculated using the scanner dose-length product and published conversion factors by Deak. RESULTS: Thirty patients were boys. The average age was 7.5 ± 5.4 years (6 days to 16 years). The D3 segment was definitely identified in 70% of scans, with 68% to 73%, moderate agreement between the readers and a Fleiss κ of 0.47 to 0.52. The DJ flexure was definitely identified in only 30.5% cases, with 35%, poor agreement between readers (Fleiss κ of 0.03). The average estimated dose for a targeted CT scan of the abdomen was 0.9 mSv (0.04-2.4 mSv). CONCLUSIONS: The third part of the duodenum, which is integral in excluding malrotation on cross-sectional studies, was "definitely" identified in 70% of CT scans of children in our study, with 68% to 73% agreement between the readers and a Fleiss κ of 0.47 to 0.52.These preliminary proof of concept results demonstrating a combination of a comparable CT dose in relation to upper gastrointestinal contrast studies and an acceptable number of cases delineating the third part of the duodenum with moderate agreement are a first step in suggesting low-dose CT for an imaging diagnosis of malrotation. Malrotation can be excluded in cases where D3 is well demonstrated in the normal position, which negates the need to automatically refer children with bilious emesis to specialist centers for upper gastrointestinal contrast studies.

Artificial intelligence for interpretation of segments of whole body MRI in CNO: pilot study comparing radiologists versus machine learning algorithm.

BACKGROUND: To initiate the development of a machine learning algorithm capable of comparing segments of pre and post pamidronate whole body MRI scans to assess treatment response and to compare the results of this algorithm with the analysis of a panel of paediatric radiologists. METHODS: Whole body MRI of patients under the age of 16 diagnosed with CNO and treated with pamidronate at a tertiary referral paediatric hospital in United Kingdom between 2005 and 2017 were reviewed. Pre and post pamidronate images of the commonest sites of involvement (distal femur and proximal tibia) were manually selected (n = 45). A machine learning algorithm was developed and tested to assess treatment effectiveness by comparing pre and post pamidronate scans. The results of this algorithm were compared with the results of a panel of radiologists (ground truth). RESULTS: When tested initially the machine algorithm predicted 4/7 (57.1%) examples correctly in the multi class model, and 5/7 (71.4%) correctly in the binary group. However when compared to the ground truth, the machine model was able to classify only 33.3% of the samples correctly but had a sensitivity of 100% in detecting improvement or worsening of disease. CONCLUSION: The machine learning could detect new lesions or resolution of a lesion with good sensitivity but failed to classify stable disease accurately. However, further validation on larger datasets are required to improve the specificity and accuracy of the machine model.

Correction to: Magnetic resonance imaging of sacroiliitis in children: frequency of findings and interobserver reliability.

The original version of this article contained an error. Under the heading 'Limitations', the sentence 'Another limitation was the decision to exclude one reader from interobserver analysis.

Magnetic resonance imaging of sacroiliitis in children: frequency of findings and interobserver reliability.

BACKGROUND: Clinicians increasingly rely on imaging in juvenile idiopathic arthritis (JIA) to identify sacroiliitis and guide treatment. However, there is limited evidence about magnetic resonance imaging (MRI) for sacroiliitis in children, and interobserver reliability is variable. OBJECTIVE: Identify the frequency of MRI findings in children with suspected sacroiliitis, calculate inter-reporter reliability and assess the value of diffusion-weighted imaging and contrast-enhanced sequences. MATERIALS AND METHODS: We retrospectively reviewed 3 years of sacroiliac joint MRI records for suspected sacroiliitis in patients <21 years at a United Kingdom tertiary referral paediatric hospital. Five radiologists (panel of three radiologists and two independent radiologists) reviewed all MRI examinations using a pictorial checklist to identify oedema, effusions, diffusion-weighted signal abnormality, enhancement, erosions and sclerosis. The frequency of panel findings was reported. Interobserver agreement was calculated using the Cohen kappa coefficient. RESULTS: An MRI diagnosis of sacroiliitis was made in 12 of 99 examinations (12%). The findings in all scans included oedema (9%), erosions (8%), diffusion-weighted signal abnormality (6%), abnormal enhancement (6%) and effusion (4%). All scans with abnormal contrast enhancement had other MRI features of sacroiliitis. Interobserver agreement was slight to moderate. CONCLUSION: Oedema and erosions were the most common findings. Inter-reporter reliability was variable with at best moderate agreement for the presence of sacroiliitis and erosions. The use of contrast enhancement for diagnosing sacroiliitis in children with JIA may be questionable.

Think about the 'C' (in custard and crackers).

CASE HISTORY: A 9-year-old girl with autistic spectrum disorder (ASD) was admitted for a flare-up of chronic recurrent multi-osteomyelitis (CRMO). She complained of leg pain over 3 months, was unable to weight-bear and had a petechial rash for 10 days. She had bleeding gums and a long history of only eating custard and crackers.CRMO was diagnosed at age 3. She'd had shoulder, knee, and ankle involvement, two courses of pamidronate (2011 and 2015) and was currently taking simple analgesia.On examination, she was afebrile, with a widespread petechial rash, most pronounced over her ankles/shins. Her knees and ankles were tender; her ankles had small medial effusions. INVESTIGATIONS: Mild hypochromic anaemia, normal platelets, white cell countand coagulation. CRP 16. Figures 1 and 2 show her knee radiograph and MRI, respectively.edpract;103/6/304/F1F1F1Figure 1edpract;103/6/304/F2F2F2Figure 2 QUESTION 1: Give four differential diagnoses of petechial rash, bleeding gums and leg pain in an afebrile child? QUESTION 2: Name three behaviours associated with ASD with health consequences. Answers to the questions are on page 02 ANSWERS TO THE QUESTIONS ON PAGE 02.

Encephalocraniocutaneous Lipomatosis Without Ocular Malformations.

BACKGROUND: Encephalocraniocutaneous lipomatosis is a rare congenital neurocutaneous syndrome resulting from ectomesodermal dysgenesis and characterized by unique hairless scalp lesions in the form of nevus psiloliparus, ipsilateral ocular malformations, and central nervous system anomalies. According to the 2009 diagnostic criteria proposed by Moog et al., ocular abnormalities are supposed to be the most consistent feature of encephalocraniocutaneous lipomatosis. PATIENT DESCRIPTION: We describe an 18-year-old girl with most of the central nervous system manifestations of encephalocraniocutaneous lipomatosis, major skin alterations including nevus psiloliparus, but no ocular involvement. CONCLUSION: Our patient suggests more variability in clinical features and a more complex genetic/embryonic etiology of encephalocraniocutaneous lipomatosis.

Susac's syndrome during pregnancy - the first Croatian case.

Susac's syndrome (SS) is an infrequent neurological disorder characterized by the clinical triad of encephalopathy, branch retinal artery occlusion and hearing loss due to an autoimmune endotheliopathy associated with anti-endothelial cell antibodies. At the onset of the disease SS rarely appears with the complete clinical triad. The most important diagnostic procedures involved in the diagnosis of SS are brain MRI, audiometric testing and retinal fluorescein angiography. Presence of at least two components of the SS clinical triad accompanied by specific brain MRI findings is highly suggestive of SS. We report a case of a young pregnant woman with a history of encephalopathy, hearing loss and walking impairment. Brain MRI revealed a spectrum of findings previously described in patients with SS. We induced labor at 37 weeks' gestation to start with immunosuppressive treatment and avoid possible fetal toxicity. To the best of our knowledge this is the first report of SS in Croatia.