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In a previous study of classifying fetuses with cortical formation abnormalities (CFA) with fetal MR, we noticed a cluster of cases with unilateral CFA and complete agenesis of the corpus callosum (ACC). In this study, we provide a detailed morphological analysis of such fetuses using fetal MR to determine if there are indicators (such as the gender of the fetus) that could be used to delineate a genetic substrate of the phenotype in order to inform future studies.We have studied 45 fetuses with the unilateral CFA/ACC phenotype and analysed through an expert consensus panel the location and fine detail of the CFA and the associated findings such as associated anomalies, head size, and sex of the fetus.The frontal lobe was significantly more frequently involved by CFA when compared with other lobes (p0.001) but no preference for the left or right hemisphere. CFA most often consisted of excessive/dysmorphic sulcation. The CFA/ACC phenotype was overwhelmingly more frequent in male fetuses (M:F 4.5:1-p0.0001). The most frequent associated findings were: ventriculomegaly (16/45 fetuses) and interhemispheric cysts (12/45 cases).This report highlights the specific phenotype of unilateral CFA/ACC that is much more common in male fetuses. This finding provides a starting point to study possible sex-linked genetic abnormalities that underpin the unilateral CFA/ACC phenotype.• We collected fetuses with unilateral cortical formation abnormality and callosal agenesis. • That distinctive neuroimaging phenotype has a strong male gender prevalence (over 80%). • This observation forms the basis of studies about outcomes and genetic substrates.

We describe 64 foetuses with cortical formation abnormalities (CFA) who had two in utero magnetic resonance (iuMR) exams, paying particular detail to those in which the original classification of CFA category changed between the two studies. The goal was to attempt to quantify the value of third-trimester follow-up studies in CFA foetuses on second-trimester iuMR imaging. The 64 foetuses reviewed came from a CFA cohort of 374 foetuses reported in an earlier publication, which detailed a classification for foetal CFA. A consensus panel of senior paediatric neuroradiologists reviewed both studies, described any change in the category of CFA between them, and attempted to predict the possible clinical significance of any differences based on the combined clinical experience of the panel. In 40/64 (62%) foetuses, the CFA description was the same on both studies. In 24/64 (38%) cases, there was a category change which included three foetuses without CFA on first examination, six foetuses where the difference involved change in laterality/symmetry, and in 15 cases the re-classification involved categorical change within the same group. Brain abnormalities other than CFA were present in 30/64 (47%) foetuses on the first study and in 33/64 (52%) on the second. We predicted that prognosis would have changed on the basis of the second study in 8% of cases, all indicating worse prognosis. We have shown that the extra diagnostic and predicted prognostic yield justifies follow-up studies in the third trimester if a CFA is shown on the second-trimester iuMR imaging. • Sixty-four foetuses with cortical formation abnormalities had two iuMR studies, for the vast majority the baseline in the second trimester and the sequential in the third. • In three foetuses, the cortical formation abnormality (CFA) was not visible on the first study. In a further 21 foetuses, the categorical description of the CFA changed between the two studies. Prognosis changed in 8% of the cases following the second iuMR study, and in all cases, the prognosis was worse. • Multiple iuMR studies provide information about the natural history of CFA; the extra diagnostic and predicted prognostic yield justifies follow-up studies.

To formulate a classification system for foetal cortical formation abnormalities (CFAs) based on in utero magnetic resonance (iuMR) appearances and trial it in 356 cases. This retrospective study included all cases of foetal CFA diagnosed between 2000 and 2017 from seven centres in Italy and UK. All of the studies were reviewed by a panel of paediatric neuroradiologists experienced in iuMR with the aid of an algorithm designed to categorise the abnormalities. Consensus expert review confirmed 356 foetuses with CFA and the first level of classification distinguished bilateral CFA (229/356–64%) from unilateral CFA (127/356–36%) cases with sub-classification of the bilateral cases into asymmetric (65/356–18%) and symmetric (164/356–46%) involvement. There was a statistically significant excess of foetuses with small head size, e.g. 17% of the cohort had a bi-parietal diameter

This is a prospective, observational study involving three Cystic Fibrosis (CF) adult patients, evaluating the changes in chest magnetic resonance imaging (MRI) three months after the start of elexacaftor/tezacaftor and ivacaftor therapy. MRI showed a drastic reduction in mucus plugging and bronchial wall thickening, with an improvement in the diffusion-weighted MRI score. Similarly, a marked improvement in spirometric parameters, nutritional status, and sweat chloride was observed. Our preliminary data confirm that chest MRI could be a useful tool to assess disease progression in CF patients on modulatory drug therapy.

Purpose: To assess agreement between 123I-metaiodobenzylguanidine (MIBG) and Whole Body Magnetic Resonance Imaging with diffusion-weighted whole-body imaging with background body signal suppression (WB MRI-DWIBS) in High and Low, Intermediate risk neuroblastoma, a retrospective review was performed on MIBG and DWIBS paired scans acquired at diagnosis, response-to-therapy steps, after-surgery, off therapy.Methods: 80 paired MIBG and DWIBS scans were acquired for 31 patients between June 2009 and June 2019 within 30 days, without intercurrent therapy. SIOPEN Semi-quantitative scoring systems for NB with 12 body sections was applied at whole body MIBG and WB MRI-DWIBS acquired to evaluate the disease extent. We evaluated specificity, sensitivity, overall accuracy, positive predictive value (PPV) and negative predictive value (NPV) of WB MRI-DWIBS respect MIBG scintigraphy considered as gold standard, and vice versa.Results: DWIBS and MIBG images were concordant in 890 out of the 960 analyzed segments, with high agreement between the two techniques (kendal =0.85 PConclusions: DWIBS and MIBG images showed very high concordance. WB MRI may represent an alternative in weak-avid MIBG tumors and for follow up assessment. An integrated imaging model is proposed for HR, Low and Intermediate Risk protocols.

Objective: The aim of this study was to report the rate of additional anomalies detected exclusively at prenatal magnetic resonance imaging (MRI) in fetuses with isolated severe ventriculomegaly undergoing neurosonography. Method: Multicenter, retrospective, cohort study involving 20 referral fetal medicine centers in Italy, United Kingdom, Spain and Denmark. Inclusion criteria were fetuses affected by isolated severe ventriculomegaly (≥15 mm), defined as ventriculomegaly with normal karyotype and no other additional central nervous system (CNS) and extra-CNS anomalies on ultrasound. In all cases, a multiplanar assessment of fetal brain as suggested by ISUOG guidelines on fetal neurosonography had been performed. The primary outcome was the rate of additional CNS anomalies detected exclusively at fetal MRI within two weeks from neurosonography. Subgroup analyses according to gestational age at MRI (

Objectives: To assess the role of fetal magnetic resonance imaging (MRI) in detecting associated anomalies in fetuses presenting with mild or moderate isolated ventriculomegaly (VM) undergoing multiplanar ultrasound evaluation of the fetal brain. Methods: This was a multicenter, retrospective, cohort study involving 15 referral fetal medicine centers in Italy, the UK and Spain. Inclusion criteria were fetuses affected by isolated mild (ventricular atrial diameter, 10.0–11.9 mm) or moderate (ventricular atrial diameter, 12.0–14.9 mm) VM on ultrasound, defined as VM with normal karyotype and no other additional central nervous system (CNS) or extra-CNS anomalies on ultrasound, undergoing detailed assessment of the fetal brain using a multiplanar approach as suggested by the International Society of Ultrasound in Obstetrics and Gynecology guidelines for the fetal neurosonogram, followed by fetal MRI. The primary outcome of the study was to report the incidence of additional CNS anomalies detected exclusively on prenatal MRI and missed on ultrasound, while the secondary aim was to estimate the incidence of additional anomalies detected exclusively after birth and missed on prenatal imaging (ultrasound and MRI). Subgroup analysis according to gestational age at MRI (< 24 vs ≥ 24 weeks), laterality of VM (unilateral vs bilateral) and severity of dilatation (mild vs moderate VM) were also performed. Results: Five hundred and fifty-six fetuses with a prenatal diagnosis of isolated mild or moderate VM on ultrasound were included in the analysis. Additional structural anomalies were detected on prenatal MRI and missed on ultrasound in 5.4% (95% CI, 3.8–7.6%) of cases. When considering the type of anomaly, supratentorial intracranial hemorrhage was detected on MRI in 26.7% of fetuses, while polymicrogyria and lissencephaly were detected in 20.0% and 13.3% of cases, respectively. Hypoplasia of the corpus callosum was detected on MRI in 6.7% of cases, while dysgenesis was detected in 3.3%. Fetuses with an associated anomaly detected only on MRI were more likely to have moderate than mild VM (60.0% vs 17.7%; P < 0.001), while there was no significant difference in the proportion of cases with bilateral VM between the two groups (P = 0.2). Logistic regression analysis showed that lower maternal body mass index (adjusted odds ratio (aOR), 0.85 (95% CI, 0.7–0.99); P = 0.030), the presence of moderate VM (aOR, 5.8 (95% CI, 2.6–13.4); P < 0.001) and gestational age at MRI ≥ 24 weeks (aOR, 4.1 (95% CI, 1.1–15.3); P = 0.038) were associated independently with the probability of detecting an associated anomaly on MRI. Associated anomalies were detected exclusively at birth and missed on prenatal imaging in 3.8% of cases. Conclusions: The incidence of an associated fetal anomaly missed on ultrasound and detected only on fetal MRI in fetuses with isolated mild or moderate VM undergoing neurosonography is lower than that reported previously. The large majority of these anomalies are difficult to detect on ultrasound. The findings from this study support the practice of MRI assessment in every fetus with a prenatal diagnosis of VM, although parents can be reassured of the low risk of an associated anomaly when VM is isolated on neurosonography. Copyright © 2020 ISUOG. Published by John Wiley & Sons Ltd.

Inflammatory myofibroblastic tumor (IMT) is an uncommon mesenchymal lesion composed of myofibroblastic and fibroblastic spindle cells, accompanied by inflammatory infiltration. IMT may occur in the tongue. Five cases have been previously reported at this site.An inflammatory myofibroblastic tumor arose in the tongue of a 10 month old infant, confirmed by anaplastic lymphoma kinase (ALK) immunohistochemical staining and the clinical response to the tyrosine kinase inhibitor.IMT can occur in the tongue. This report highlights the differential diagnosis of IMT. ALK staining is both a helpful diagnostic marker and a predictive marker for targeted therapy in this tumor type.

Prenatal diagnosis of skeletal dysplasias is particularly difficult for many reasons and differentiating these disorders in the prenatal period can be challenging because they are rare and many of the ultrasound findings are not necessarily pathognomonic for a specific disorder. The diagnosis is often made just after birth or exitus. The prenatal diagnosis of osteochondrodysplasias is based predominantly upon fetal ultrasound findings and it focuses substantially on the possible lethality of the disorder, without always being able to find a specific name for the disorder. Metatropic dysplasia is a rare osteochondrodysplasia due to mutations in the TRPV4 gene: TRPV4 is a cation channel, non-selectively permeable to calcium, encoded by a gene on chromosome 12q24.11; it is widely expressed and involved in many different physiological processes through responses to several different stimuli (physical, chemical, and hormonal) in ciliated epithelial cells. The exact incidence of this disorder is not known, however less than a hundred cases have been reported at present, with only two prenatal reports but without any reference to the molecular test. We describe the first report of molecular diagnosis of metatropic dysplasia carried out in prenatal diagnosis: the molecular testing of the TRPV4 (transient receptor potential cation channel, subfamily V, member 4, MIM *605427) gene in our case, in fact, detected a causative variant, confirming the diagnostic suspicion, which was made possible thanks also to the utilization of MRI and CT scan. In our case different imaging methods together with the close cooperation of a multidisciplinary team and test availability, allowed an accurate diagnosis.

Background Sacroiliitis is one of the extraintestinal manifestations associated with inflammatory bowel disease (IBD), and may be underdiagnosed especially in the pediatric age. MR-enterography (Entero-MRI) is currently the imaging gold standard to assess intestinal disease activity and to detect complications in patients with IBD. Only few studies have been conducted on adult patients with IBD in order to define the role of this technique in assessing sacroiliitis, while no data are available on pediatric patients. Objectives To study the prevalence of inflammatory sacroiliitis on MRI performed for intestinal investigation in an IBD pediatric population. Methods This is a retrospective study conducted on patients suffering from IBD followed in our gastroenterology department between 2010 and 2018 whose entero-RM (1.5 or 3 Tesla, Philips depending from year of scanning) were blindly and independently scored by two readers experienced in pediatric musculoskeletal imaging. Each sacroiliac joint was divided into 4 quadrants. Signs of sacroiliitis were identified according to the ASAS criteria, with a particular attention to the presence of bone marrow edema (using T2 weighted sequences with fat suppression), diffusion restriction in DWI sequences (Diffusion Weighted Imaging) or DWIBS (Diffusion Weighted Imaging with Background Suppression) and post-contrastographic uptake in dynamic acquisitions. Demographics, IBD characteristics, clinical, radiological, and laboratory data were recorded and a dedicated Excel database was constructed. Results were elaborated using descriptive statistics. Results 34 patients (10 F, 24 M, age at scanning range 5-20 yrs, median 15) were included in the study, for a total of 59 entero-MRI evaluated (some patients were subjected to more than one scan). Two out of 34 patients were affected by Ulcerative Colitis, 32 by Crohn disease. Joint examination resulted negative in all patients, and none complained of articular symptoms including back pain. In 5 IBD patients (4 CD, 1 UC) a monolateral slight degree of sacroiliitis (grade 1) was radiologically identified. They were all males, without clinical-laboratory-radiologic inflammatory signs of intestinal activity, with the exception of a patient who presented signs of intestinal and sacroiliac inflammation at his first entero-MRI, while 18 months later, at his MRI control under pharmacological treatment, signs of sacroiliitis were still present in the absence of intestinal signs of inflammation. Conclusion Asymptomatic sacroiliitis was observed in about 15% of our IBD patients. Sacroiliac involvement therefore can be underdiagnosed in these patients. Entero-MRI with specific sequences could be a good tool to detect early signs of sacroiliac inflammation. References [1] S.Lecler-Jacob,G.Lux, A.C.Rat, V.Laurent, A.Blum, I. Chary-Valckenaere, L.Peyrin-Biroulet & D. Loeuille. The prevalence of inflammatory sacroiliitis assessed on magnetic resonance imaging of inflammatory bowel disease: a retrospective study performed on 186 patients. Aliment Pharmacol Ther 2014; 39: 957-962. [2] Timothy J.P. Bray, Nicola Ambrose, David Atkinson, Debajit Sen, Yiannis Ioannou and Margaret A. Hall-Craggs Diffusion-weighted imaging is a sensitive biomarker of response to biologic therapy in enthesitis-related arthritis. Rheumatology 2017; 56: 399-407. Disclosure of Interests None declared

Objective The aim of the study was to establish an apparent diffusion coefficient (ADC) cut-off value to classify active and non-active lesions in inflammatory bowel disease. Methods We reviewed 167 paediatric magnetic resonance enterographies executed for suspected inflammatory bowel disease by using a 1.5- and 3-T scanner. We assessed the presence and activity of the disease by using morphologic and functional parameters such as the ADC. Each patient could have more than 1 examinations. Quantitative assessment of disease activity in the ADC map was measured placing 3 regions of interest in the areas of highest inflammation and the mean value was calculated, patients without sign of inflammation were assessed at 2 standardised site. Ileocolonoscopy, esophagogastroduodenoscopy, surgery, and video-capsule endoscopy were used as standards of reference. Results We enrolled 34 patients and 35 examinations: radiological findings of disease were identified in 29 examinations and 44 lesions were detected. Six patients had negative results and ADC assessment was taken at the terminal ileum and cecum. A total of 56 bowel segments were included in the study. Image analysis revealed 39 active lesions (69.6%) and their ADC values were lower compared to the ones of non-active segments. For each scanner a cut-off value was found (sensitivity: 0.91, specificity: 0.89 for 1.5 T and 0.81 for 3 T). Inter-rater agreement on disease activity between ADC values and magnetic resonance enterography results and between ADC values and the standard of reference were very good. Conclusions ADC can provide a scanner-based quantitative measurement of disease activity.

OBJECTIVES: We describe the way we treated 7 children with critical long-term complications after metallic balloon-expandable stenting in the left mainstem bronchus. METHODS: Endoscopic follow-up included a first bronchoscopy 3 weeks after stenting, then monthly for 3 months, every 4-6 months up to 1 year and at scheduled times to calibrate stent diameter up to final calibration. When major complications occurred, patients underwent chest computed tomographic angiography. RESULTS: In 1 of the 7 children (median age 2.8 years), metallic left bronchial stenting served as a bridge to surgery. After a median 4-year follow-up, all 7 children experienced recurrent stent ovalizations with stent breakage in 3 and erosion in 1. In 4 children, computed tomographic angiography showed abundant peribronchial fibrous tissue, in 2 left mediastinal rotation and in 1 displacement along the left bronchus after pulmonary re-expansion as the cause of stent-related complication. Of the 7 children, 6 underwent surgery (5 posterior aortopexy and 1 section of the ligamentum arteriosus) and 3 required nitinol stents placement within the metallic ones. One patient completed the follow-up, and 1 patient was lost to follow-up. All 5 remaining children still have permanent bronchial stents in place, patent and re-epithelialized after a median 10.5-year follow-up. There were no deaths. CONCLUSIONS: Satisfactory anatomical relationships when children have stents placed in the left mainstem bronchus alone do not guarantee the final success. Several mechanisms intervene to cause critical stent-related complications in children during growth. Permanent metallic stents should be used carefully, and only in selected patients.

Agenesis of the ramus of the mandible (agnathia) is a rare disease that can be part of more than 100 genetic syndromes. From an embryological point of view, agenesis of the mandible is linked to defects in migration of neural crest cells that determine a developmental anomaly of the first arch or mandibular arch, during the 4th week of embryogenesis [1]. Agnathia can be isolated or commonly associated with holoprosencephaly. Evidence from humans and animal model suggest that agnathia-holoprosencephaly represents a causally heterogeneous single developmental field defect [2]. Agnathia may be usually recognized by ultrasound during the second trimester of pregnancy and may be misinterpreted as micrognathia [3–5]. If micrognathia is the only sonographic finding identified, parents should be awarded for possible respiratory complications at birth, presence of facial clefting, and/or developmental delay [6]. Chromosomal abnormalities have been detected in 66 % of fetuses with micrognathia, in 77 % with macroglossia, in 48 % with cleft lip and palate, in 45 % with severe hypotelorism or cyclops, and in 32 % with nasal hypoplasia, proboscis, or single nostril [4, 7]. Teoh and Meagher [8] have reported a first-trimester (13 weeks) diagnosis of micrognathia and receding chin by using transvaginal ultrasound, with later diagnostic confirmation at 19 weeks’ gestation. These findings were associated with complex cardiac defect and unilateral talipes equinovarus in a fetus with normal karyotype and with a definitive diagnosis of Pierre Robin syndrome. Sepulveda et al. [9] have demonstrated the value of the retronasal triangle view for detecting micrognathia in the first trimester. Absent visualization of the mandibular gap or failure to identify the mandible in coronal view is highly suggestive of micrognathia and should prompt a targeted ultrasound scan to assess for other anomalies. The role of RNT in the early prenatal diagnosis of orofacial clefts has been confirmed later by Tonni et al. [10].

We describe the case of a term infant with broad and protruding forehead, micrognathia, wide cleft palate, short neck and stocky body with small thoracic cage, short limbs, bilateral clubfeet and triventricular hydrocephalus. A G313S mutation in the gene for type II-collagen(COL2A1)was demonstrated. Soon after birth, the infant had respiratory distress with repeated obstructive apnea, leading to nasotracheal intubation. Despite mechanical ventilation(MV)with high positive pressure, he showed desaturations, leading to tracheotomy. An endoscopy showed severe tracheo-bronchomalacia. At 5 months a polyflex self-expanding silicone stent was placed in the trachea(10x30, replaced by12x20 and removed after 4 years), with two stainless steel stents in the mainstem bronchi(Palmaz 104right and Palmaz 128left). After 5 years 2 stents were overlapped to the previous in each of the mainstem bronchus(2 Jomed 12-10/12). At 10.5 years a Y Dumon stent(11-7-7) has been placed in the trachea and removed after 2 years. To date he is a 13 year old child, he has a tracheotomy(4.5mm cuffed Shiley with speech valve)and occasionally needs of night ventilatory support. He attends the secondary school with fairly good performance. G313S mutation has been described in a lethal case with respiratory insufficiency at birth, with spondyloepiphyseal dysplasia congenita. In our case, the airway malacia has been promptly treated by stenting with immediate improvement of the airway ventilation. Stenting, although is a palliative treatment given the cage narrowness, has allowed a child who was expected to die at birth, to reach adolescence in good condition and with a certain degree of autonomy.

Narrowing of the left mainstem bronchus(LMSB)may produce severe respiratory symptoms andairway stenting may represent a conservative treatment. We described 7 cases(3 boys,median age:0.5yrs, age range:0-13yrs)in which a left obstruction was treated with metallic balloon expandable( J e OSTENT,Abbott ®)and only a follow-up permitted to manage stent-related potentially fatal complications with low risk interventions. After a median time of 2.5 yrs(range:0.25-5yrs)from the stenting, all children showed major complications(3 complete ovalization, 3 breakage and 1 haemoptysis)due to a novel close proximity or collision to adjacent vessels. After a CTA scan showing vascular collision, 2 of the children with stent ovalization were treated with a posterior aortopexis(PA), while a watchful approach was planned in the third child. One of the children with stent breakage was submitted to PA, while a silicone stent(Dumon, Novatech) overlapping the metallic one was chosen for the second and a surgical expansion of the chest has been scheduled for the third patient who was affected by jeune syndrome. Tranexamic acid instillation together with a posterior aortopexis was successfully carried on in the child with haemoptysis. The presence of satisfactory anatomical relationships at the time of stent placement in LMSB is not by itself a guarantee of the final device. Only a radiological study before and eventually after stenting and a close follow-up by bronchoscopy can highlight in real time potentially dangerous complications secondary to the well known “mass effect”. The use of a permanent device must take account that clinical sequelae may become catastrophic.

Urinary tract infections (UTIs) are a frequent clinical problem in infants and children, and they may develop into serious complications with long-term sequelae. Their prevalence varies according to age and sex: males are more interested in the neonatal period and in the first year of age, while females are more involved after that time. The clinical outcome and the severity of UTIs depend on many risk factors including urinary tract malformations and dysfunctions, virulence and properties of the pathogen, host response to the infection, and promptness of diagnosis and management.

Objective To investigate apparent diffusion coefficient (ADC) mapping with the measurement of renal tissue ADC value of normal and pathological fetal kidneys at various gestational ages (GAs). Methods Fifty pregnant women underwent magnetic resonance images (MRI) after ultrasound (US) for suspected fetal genitourinary disorders (16) or for suspected disorders in other organs (34). A multiplanar study of urinary system was obtained by using conventional T2-weighted sequences and echo planar imaging (EPI); Diffusion-weighted images and ADC maps were evaluated. The renal tissue ADC value was measured for all normal and abnormal fetuses and related to GA. Results MRI confirmed urinary anomalies in 15 fetuses [2 renal developmental variants, 2 nephropathies, 4 multicystic dysplastic kidneys (MCDK), 7 renal tract dilatations] and detected normal kidneys in the remaining 35 fetuses. Normal renal parenchyma showed bright signal on diffusion-weighted images with ADC values ranging from 1065 to 1327 µm2/s with a tendency to decrease over GA. A pathological ADC was detected in cases of bilateral MCDK, huge dilatations and in cases of nephropathies. Conclusion Diffusion-weighted imaging (DWI) with ADC mapping can be used in the evaluation of fetal renal parenchyma and may become a tool of assessing function of the fetal kidney by means of measurement of renal tissue ADC values. Copyright © 2007 John Wiley & Sons, Ltd.

Objectives We reviewed the role of posterior aortopexy for left mainstem bronchus compression in infants and children. Methods Eighteen children with respiratory symptoms were enrolled between 2005 and 2015 for surgical decompression of the left mainstem bronchus. The children were managed from diagnosis to follow-up by a dedicated tracheal team. Primary outcomes were the complete relief of symptoms or improvement with respect to preoperative clinical status. Results The median age was 4 years (0.3-15.4) and the median weight was 13.2 kg (3, 1-40). Symptoms or indications for bronchoscopy included difficult weaning from mechanical ventilation (n = 3, 17%), difficult weaning from tracheotomy (n = 4, 22%), recurrent pneumonia (n = 4, 22%), wheezing (n = 3, 17%), atelectasis (n = 1, 5.5%), bitonal cough (n = 1, 5.5%) and stridor (n = 2, 11%). Associated malformations were present in 88.7%. The diagnosis was made by bronchoscopy and computed tomography. Indication for surgery was the presence of pulsations and reduction in the diameter of the left mainstem bronchus compression of more than 70%. Surgery was performed by left posterolateral thoracotomy. Aortopexy was done under bronchoscopic control. No early or late deaths were observed, nor were reoperations necessary. Residual malacia was observed in 8 children (44%). Median follow-up was 4.1 years (0.1-7.1). At last follow-up, 17/18 (94.4%) children showed adequate airway patency. Conclusions The intrathoracic location of the left mainstem bronchus predisposes it to compression. Vascular anomalies represent the most frequent causes. Aortopexy has been advocated as a safe and useful method to relieve the compression, and our results confirmed these findings. Management of these patients is challenging and requires a multidisciplinary team.

The aim of this paper is to provide indications for performing magnetic resonance enterography (MRE) in Crohn's disease (CD), the essential technical elements of MRE techniques and typical findings in patients with CD. Patients suffering from CD frequently require cross-sectional imaging. By performing MRE, it is possible to obtain results comparable to those obtained with endoscopy in terms of identifying and assessing disease activity and better than other cross-sectional imaging techniques, such as CT, in the evaluation of the fibrosis and complications of disease. The MR imaging of diffusion MR is a technique which enables medical staff to add important additional information and which may replace the use of intravenous contrast agents in the near future. Magnetic resonance enterography is an accurate tool for assessing bowel disease and the various complications associated with CD. The lack of exposure to non-ionizing radiation is an important advantage of this imaging technique, especially in the case of pediatric patients. Familiarity with common and pathognomonic imaging features of CD is essential for every clinician involved in the treatment of inflammatory bowel disease and the care of patients.

Progressive cystic fibrosis (CF) lung disease is the main cause of mortality in CF patients. CF lung disease starts in early childhood. With current standards of care, respiratory function remains largely normal in children and more sensitive outcome measures are needed to monitor early CF lung disease. Chest CT is currently the most sensitive imaging modality to monitor pulmonary structural changes in children and adolescents with CF. To quantify structural lung disease reliably among multiple centres, standardisation of chest CT protocols is needed. SCIFI CF (Standardised Chest Imaging Framework for Interventions and Personalised Medicine in CF) was founded to characterise chest CT image quality and radiation doses among 16 participating European CF centres in 10 different countries. We aimed to optimise CT protocols in children and adolescents among several CF centres. A large variety was found in CT protocols, image quality and radiation dose usage among the centres. However, the performance of all CT scanners was found to be very similar, when taking spatial resolution and radiation dose into account. We conclude that multicentre standardisation of chest CT in children and adolescents with CF can be achieved for future clinical trials.

We report our experience with fetal cardiac MR imaging and fetal MR-angiography in a 35 + 5-week fetus with a ventricular septal defect (VSD). Case : A fetus with an abnormal four-chamber view at obstetric US and a VSD of the medio-muscular portion of the ventricular septum at echocardiography underwent MRI to assess possible associated anomalies. Gradient Echo (GE) sequences with steady-state free precession (TrueFISP) and cine-MR sequences demonstrated a 4 mm VSD involving the medio-muscular portion of the septum in a morphologically and volumetrically normal fetal heart with a correct origin of the great arteries. T1-weighted 3D spoiled sequences permitted to obtain angiographic images and MIP reconstructions that proved a normal size and position of the aorta disconfirming a suspected coartaction of the aorta. Conventional T2-weighted sequences excluded the presence of extracardiac abnormalities. A postnatal echocardiography, considered as standard of reference confirmed the VSD in an otherwise normal male newborn.

Lo studio del distretto cardiovascolare e delle patologie cardiache congenite in utero con Risonanza Magnetica (RM), stando anche a recenti studi pubblicati in letteratura, e ancora pioneristico anche se possibile, in relazione alle numerose limitazioni di ordine tecnico solo parzialmente superabili [1, 2]. Tuttavia, la RM potrebbe offrire, rispetto all’ecocardiografia, una migliore finestra di studio nelle fasi piu avanzate della gravidanza in virtu dei progressivi processi di riduzione del liquido amniotico (oligoidramnios fisiologico) e di calcificazione delle coste fetali che riducono la propagazione degli ultrasuoni (US) [3]. Coesistono inoltre, in particolare nelle cardio- patie complesse, limitazioni di carattere intrinseco legate ad anomalie di rotazione o a ipertrofie ventricolari, che alterano la normale geometria del cuore con perdita dei classici piani. Non si puo inoltre dimenticare che allo stato dell’arte non e possibile fornire valutazioni flussimetriche nelle patologie su base valvolare e vascolare (ad esempio, incontinenza, reflusso, atresia, stenosi ecc), anomalie che possono essere solamente ipotizzate se determinanti un’alterazione anatomica. Inoltre, le patologie con alterazioni del ritmo cardiaco non sono allo stato attuale di pertinenza RM. La contrattilita cardiaca e la funzionalita valvolare non sono valutabili con la RM: infatti l’impossibilita tecnica di sincronizzare l’acquisizione con il battito cardiaco fetale e 1’ancora limitata risoluzione temporale delle attuali sequenze cine RM (2–3 frame al secondo) non permettono di seguire la funzionalita cardiaca in real time, ma acquisiscono il movimento cardiaco in maniera artificiale, con velocita dipendente dalla rapidita di acquisizione delle fette e non dalla reale velocita di contrazione atrioventricolare [4].

Congenital heart defects (CHD) are the most frequent malformation in the human fetus and are the leading cause of mortality due to malformations in the first year of life. Despite its clinical importance screening performed by ultrasonographic examination during the second trimester of pregnancy has shown disappointingly low detection rates mainly due to the difficulties in obtaining an adequate examination of the fetal heart. Four-dimensional (4D) ultrasound of the fetal heart has been recently suggested as a tool to improve the detection rate of CHD by decreasing the dependency on operator skills required in two-dimensional ultrasound scans but up to now no practical manuals are available in describing its application in clinical practice for the study of fetal heart. The objective of this Ebook is to explain the role of 4D during second trimester examination and in fetuses with CHD. The technique of obtaining 4D volume of the fetal heart, how to navigate in the volume to obtain diagnostic planes and how to use semiautomatic and automatic software of analysis are described. We believe that after reading this book the standard fetal cardiac anatomy survey can be performed in the second trimester fetus by 4D in both normal and abnormal hearts. This approach may reduce the operator dependency in diagnosis CHD. This Ebook should prove to be a valuable resource for obstetricians, sonographers and pediatric cardiologists.

Reactive lymphoid hyperplasia (RLH) is a rare, benign, lymphocytic tumour-like lesion reported in various organs. It has been previously identified in 18 cases in the English-language literature, but only 5 of them were related to oncological disease. No previous cases have been described of RLH occurring in ovarian cancer patients. We describe a case of hepatic RLH which developed in a patient treated for ovarian cancer 11 years previously. Radiological features on computed tomography (CT) scan and PET–CT (positron emission tomography–computed tomography) were strongly suggestive of oncological disease, in contrast to magnetic resonance imaging (MRI); the volume increment of the nodular lesion and the rise in carbohydrate antigen 125 corroborated the hypothesis of malignancy. The patient was subjected to resection of the 7th segment of the liver and the final histological report showed RLH. RLH should be considered in the presence of hepatic lesions in suspected ovarian cancer recurrence. Imaging techniques should be thoroughly investigated to exclude tumor recurrence promptly, in order to avoid unnecessary surgery.

To investigate apparent diffusion coefficient (ADC) mapping with the measurement of renal tissue ADC value of normal and pathological fetal kidneys at various gestational ages (GAs).Fifty pregnant women underwent magnetic resonance images (MRI) after ultrasound (US) for suspected fetal genitourinary disorders (16) or for suspected disorders in other organs (34). A multiplanar study of urinary system was obtained by using conventional T2-weighted sequences and echo planar imaging (EPI); Diffusion-weighted images and ADC maps were evaluated. The renal tissue ADC value was measured for all normal and abnormal fetuses and related to GA.MRI confirmed urinary anomalies in 15 fetuses [2 renal developmental variants, 2 nephropathies, 4 multicystic dysplastic kidneys (MCDK), 7 renal tract dilatations] and detected normal kidneys in the remaining 35 fetuses. Normal renal parenchyma showed bright signal on diffusion-weighted images with ADC values ranging from 1,065 to 1,327 microm(2)/s with a tendency to decrease over GA. A pathological ADC was detected in cases of bilateral MCDK, huge dilatations and in cases of nephropathies.Diffusion-weighted imaging (DWI) with ADC mapping can be used in the evaluation of fetal renal parenchyma and may become a tool of assessing function of the fetal kidney by means of measurement of renal tissue ADC values.