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Background: The association between radiological remission and natural history of disease in children with inflammatory bowel diseases (IBD) is poorly known., Aims: (i) To assess the correlation between cross-sectional imaging (CSI) (ultrasound and magnetic resonance imaging) and clinical, biomarker and endoscopic disease activity; (ii) to evaluate the impact of radiological activity on the occurrence of complications in pediatric patients with IBD., Methods: A retrospective study including pediatric patients with IBD and radiological follow-up of at least one year was conducted between 2003 and 2019 at the Nancy University Hospital., Results: In total, 118 patients (66 Crohn's disease (CD) and 52 ulcerative colitis (UC)) were included. Median follow-up duration was 5.2 years (range: 1.1-15.4). Seventeen (25.8%) patients with CD and 7 (13.5%) patients with UC achieved and maintained radiological remission until last follow-up. No IBD patient achieving radiological remission experienced complications or relapse. In patients not achieving radiologic remission, complications and surgery occurred in 13/49 (26.5%) and 8/49 (16.3%) patients with CD and in 5/45 (11.1%) and 5 (11.1%) subjects with UC. Among patients with CD, the association for remission status between radiological and endoscopic assessment was excellent (Cramer's V test (V) = 0.50), and moderate between radiological and either clinical (V = 0.30) or biochemical (V = 0.33) assessments. In UC, the association for remission status between radiological and either endoscopic or clinical assessments were weak (V = 0.19 and V = 0.20 respectively), and moderate (V = 0.23) between radiological and biochemical assessments., Conclusion: CSI may replace endoscopic monitoring in pediatric CD. Radiological remission status predicts long-term disease outcomes., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Introduction: According to SIOP criteria, every patient presenting with preoperative Wilms tumor (WT) rupture must receive abdominal radiotherapy. Neoadjuvant chemotherapy reduces tumor volume and is responsible for the development of peritumoral capsule formation, which can mask tumor rupture on histological analysis, while it was clinically or radiologically obvious at diagnosis. Yet, there are no protocol recommendations for this particular presentation., Objectives: Study the agreement between clinicoradiological signs and histological confirmation after neoadjuvant chemotherapy of suspected WT rupture and describe the therapeutic choices arising in consequence., Methods: Descriptive retrospective study on a monocentric series of patients with WT between June 1991 and August 2017., Results: Out of 71 patients, 28 presented with suspected tumor rupture. We observed good agreement between clinical and radiological signs of suspected rupture (κ coefficient: 0.67). However, we assessed poor agreement between these signs and histological conclusions after neoadjuvant chemotherapy (κ coefficient: 0.27). Only five patients with clinicoradiological signs were overtreated with radiotherapy while tumor rupture had been refuted after histological review. The notion of abdominal trauma and the presence of intraperitoneal effusion seemed to guide collegial decision to overtreat these patients. No statistical difference in survival between patients with and without suspicion of tumor rupture at diagnosis was observed., Conclusion: This study highlights the need for recommendations in case of discrepancy between radiological and histological signs of rupture at diagnosis and after neoadjuvant chemotherapy. A study with stronger statistical power is necessary to define criteria that would lead to optimization of treatment in this context., (© 2019 Wiley Periodicals, Inc.)

Background: Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years., Material and Methods: Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences., Results: Fifty-one patients (41 girls) were identified. Median age at diagnosis was 13.1 years [8.7-17.9]. Abdominal pain was the commonest presenting symptom (32/49, 65%). The tumor was located in the pancreatic head in 24 patients (47%). Preoperative biopsy or cytology was performed in 14 cases (28%). All patients were operated with a median of 23 days [0-163] after diagnosis. The rate of postoperative morbidity was 29%. With a median follow-up of 65 months [0.3-221], the overall and event-free survival was 100% and 71%, respectively. Seven patients (13.7%) relapsed with a median of 43 months [33-94] after initial surgery. Six were treated surgically, either alone (n = 3) or with perioperative chemotherapy (n = 2) or hyperthermic intraperitoneal chemotherapy (n = 1). One patient in whom further treatment was not feasible was still alive at last news. Risk factors for recurrence were positive surgical margins (P = 0.03) and age less than 13.5 years at diagnosis (P = 0.03)., Conclusions: SPPN recurrence in this pediatric series was a rare and late event that did not undermine overall survival. Complete surgical removal of recurrent tumors appears to be the best option., (© 2016 Wiley Periodicals, Inc.)

Purpose: To evaluate if measurement of split renal function ( SRF split renal function ) with dynamic contrast material-enhanced ( DCE dynamic contrast enhanced ) magnetic resonance (MR) urography is equivalent to that with renal scintigraphy ( RS renal scintigraphy ) in patients suspected of having chronic urinary obstruction., Materials and Methods: The study protocol was approved by the institutional ethics committee of the coordinating center on behalf of all participating centers. Informed consent was obtained from all adult patients or both parents of children. This prospective, comparative study included 369 pediatric and adult patients from 14 university hospitals who were suspected of having chronic or intermittent urinary obstruction, and data from 295 patients with complete data were used for analysis. SRF split renal function was measured by using the area under the curve and the Patlak-Rutland methods, including successive review by a senior and an expert reviewer and measurement of intra- and interobserver agreement for each technique. An equivalence test for mean SRF split renal function was conducted with an α of 5%., Results: Reproducibility was substantial to almost perfect for both methods. Equivalence of DCE dynamic contrast enhanced MR urography and RS renal scintigraphy for measurement of SRF split renal function was shown in patients with moderately dilated kidneys (P < .001 with the Patlak-Rutland method). However, in severely dilated kidneys, the mean SRF split renal function measurement was underestimated by 4% when DCE dynamic contrast enhanced MR urography was used compared with that when RS renal scintigraphy was used. Age and type of MR imaging device had no significant effect., Conclusion: For moderately dilated kidneys, equivalence of DCE dynamic contrast enhanced MR urography to RS renal scintigraphy was shown, with a standard deviation of approximately 12% between the techniques, making substitution of DCE dynamic contrast enhanced MR urography for RS renal scintigraphy acceptable. For severely dilated kidneys, a mean underestimation of SRF split renal function of 4% should be expected with DCE dynamic contrast enhanced MR urography, making substitution questionable., (© RSNA, 2014 Online supplemental material is available for this article.)

Hepatic alveolar echinococcosis is a rare parasitic zoonosis, potentially lethal in childhood. It is due to Echinococcosis multilocularis whose larva insidiously develops in the liver. We report the case of a 13-year-old girl, living in the Vosges Mountains, followed for recurrent abdominal pain, with recent worsening. Diagnosis of alveolar echinococcosis was immediately suspected based on the liver ultrasound scan and then confirmed by imaging (CT scan, NMR) and serology. A curative surgical treatment (segmentectomy) was performed 3 months after diagnosis, under oral albendazole treatment, maintained for at least 2 years. Hepatic alveolar echinococcosis usually has a negative prognosis, except if diagnosed early, which allows rapid surgical treatment, as in our patient., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Midaortic syndrome (MAS) is a rare, idiopathic condition in children usually presenting with severe hypertension. We report a case of a 13-year-old girl who presented with severe hypertension (200/110 mmHg) associated with renal artery stenosis and normal renal function (creatinine clearance 110 ml/min/1.73m(2)). Percutaneous angioplasty (PTA) was first performed, but early recurrence of hypertension occurred. Subsequent imaging evaluation demonstrated association of aortic narrowing, proximal stenosis of the left renal artery, and wall thickening of superior mesenteric artery and right common carotid artery. Although previous large-vessel arteritis cannot be absolutely excluded, a diagnosis of idiopathic MAS was made, given the absence of any other clinical signs of inflammation (C-reactive protein <0.5 mg/dl; erythrocyte sedimentation rate 5 mm/h). Medical treatment was undertaken without repeat PTA or surgery. Blood pressure control was good, and antihypertensive therapy was stopped 4 years later. At age 22, the patient was still normotensive and receiving no antihypertensive therapy; normalization of Doppler velocities in the proximal left renal artery was confirmed. In the absence of renal dysfunction or target-organ damage, medical management of hypertension in MAS is feasible without intervention if blood pressure is well controlled on two antihypertensive agents.

Objective: The purpose of this series was to evaluate the value of contrast-enhanced ultrasonography (CEUS) in the characterization of focal liver lesions (FLLs) in patients with glycogen storage diseases (GSDs)., Methods: Contrast-enhanced ultrasonographic data obtained for characterization of 8 FLLs (size, 0.9-10.2 cm) in 2 patients with GSD type Ia (GSD-Ia) and lesion growth or recurrent abdominal pain were reviewed and compared with computed tomographic (CT) and magnetic resonance imaging (MRI) data. After total and left hepatectomy, pathologic examination confirmed benign adenomas in 6 of the evaluated lesions. Follow-up confirmed benignity in the 2 remaining lesions., Results: In all FLLs, CEUS showed marked hypervascularity in the early arterial phase. Centripetal filling was shown in only 1 lesion, and diffuse enhancement without any clear direction was shown in all other lesions. During the portal and late phases, 6 of the 8 lesions showed sustained enhancement, including 2 lesions that appeared heterogeneous during all phases of CT and MRI. In an aspect of 1 of these 6 large adenomas, late wash-out could be explained by sinusoid compression. The other 2 adenomas showed moderate wash-out but remained homogeneous., Conclusions: Focal liver lesions found in patients with GSD-Ia have similar patterns on CEUS compared with incidental adenomas. Global or partial hypoenhancement observed in the late phase did not indicate a transition to hepatocellular carcinoma but may have been related to ischemia.

Purpose: To evaluate the accuracy of voiding urosonography (VUS) compared to X-ray voiding cystoureterography (VCUG) for the detection and grading of vesicoureteral reflux (VUR) by standardised reading of digital clips obtained from VUS and digital images from VCUG., Materials and Methods: Approval by the ethics committee was obtained, and written, informed consent was given. 130 children (94 girls and 36 boys, mean age of 4.4 years) underwent VUS using Levovist (Schering, Germany) prior to VCUG. Digital VUS clips and digital VCUG images were reviewed by two groups of two radiologists. Results were mainly analysed in terms of reno-ureteral units (RUUs). Intra and interobserver reproducibility was estimated by calculation of kappa coefficient. Calculation of sensitivity and specificity of VUS was made in comparison with VCUG., Results: Intraobserver reproducibility was good to moderate for the detection of VUR using VUS (kappa = 0.67 and 0.53 for each reviewer respectively) and good for the grading of reflux (kappa = 0.64 and 0.70). Interobserver reproducibility was excellent for the detection and grading of VUR using VCUG (kappa = 0.89 and 0.91) but good to moderate for VUS (kappa = 0.73 and 0.51). Compared to VCUG, sensitivity and specificity of VUS for the detection of VUR were 62.7 % and 83.4 %. Concordance for grading was moderate, with a higher grading using VUS., Conclusion: Real-time evaluation and diagnosis based on the review of VUS digital clips is achievable. However, there is a need for standardisation of digital records if a second reading by another radiologist or consideration by urologists is needed. Recent advances in US technology and the use of second-generation contrast agents would be promising to improve the feasibility, reproducibility and accuracy of the method.

Segmental omental infarction (OI) is a rare cause of abdominal pain in children. It generally occurs in the right lower and upper quadrants of the abdomen and only a few cases of other locations have been described in adults. We report a unique paediatric case of OI with an unusual left-sided location extending into the pelvis in a 6-year-old non-obese girl. The diagnosis was suspected on US and CT based on imaging patterns previously described in adults and children. Laparoscopic resection should be balanced with nonsurgical management.

Vesicoureteral reflux (VUR) is a common finding in children presenting with acute urinary infection, with a frequency ranging from 20 to 50%. If radiological retrograde cystography still remains the standard technique, sonocystography now appears as a valuable alternative method, due to the properties of recent ultrasound contrast agents and the wide use of harmonic imaging. The analysis of the literature and the experience acquired by the authors during a clinical trial allow summarizing the current data on this new technique. Because of its accuracy, it may replace radiological cystography in the detection of VUR in girls, the follow-up in both boys and kids, and the management of recurrent infection in children presenting with normal radiological cystography.

Thoracic calcifications are found in the following locations: mediastinum, lung, pleura, cardiovascular system, and thoracic wall. The calcifications of the mediastinum include mostly inflammatory lesions and malignant neoplasms. Pulmonary calcifications are mainly inflammatory lesions and metastases of osteosarcomas. Where the cardiovascular system is concerned, the most common calcifications are those of the heart relating to tumors or surgery. Calcifications of the aortic wall and valve calcifications can be found. In the thoracic wall there are calcifications of the bone and of soft tissues. The knowledge of shape and location is very useful for the diagnosis of the underlying disease. Calcifications in the thorax are frequently manifestations of previous infectious processes. Less often, they may be due to neoplasms, metabolic disorders, or previous medical therapy.

Pheochromocytoma is rare in children. A wide varieties of lesions are observed and diagnosis, often made late, is based on urinary catecholamine assay. Magnetic resonance imaging provides the best morphological information. The disease is usually benign and prognosis is good. Familial forms of pheochromocytoma and pheochromocytoma associated with ischemia-induced bone lesions must be emphasized.

Xanthogranulomatous pyelonephritis is a morphologic variant of pyelonephritis. Focal disease is very rare and can be misdiagnosed.

In case of painful scoliosis, a search for etiologic factors in the vertebral spinal canal and adjacent structures should be made. MRI with Gadolinium is useful to look for various pathology in and around the spine in particular of spinal cord. CT scanning is indispensible for bony details. Thus CT and MRI are complementary in evaluation of pathology of spine in any modern imaging centers.

Giant cell tumor of bone rarely affects children, in whom it is usually located in a metaphysis in contrast to the predominantly epiphyseal localization in adults. Five cases are reported, two at the femur, two at the fibula and one at the tibia. Plain film radiography and computed tomography are the most informative imaging studies. The differential diagnosis is with aneurysmal bone cyst and, in metaphyseal-epiphyseal forms, with chondroblastoma. Treatment usually consists in curettage of the tumor followed by filling of the cavity; however, more extensive resection is required in some cases.

The authors describe an infant with an aneurysm of the right iliac artery, associated with three sites involving the inferior mesenteric artery and its first two branches, most likely related to an episode of chicken pox. Helical angiography with computed tomography, performed with a continuous-rotation scanner, allowed precise assessment of the lesions, in particular those affecting the inferior mesenteric artery.

On the basis of personal observation of four cases, the authors discuss the diagnostic aspects in this disease. Before birth, the diagnosis should be made by echography. After birth, magnetic resonance imaging scanning (IRM) is the method showing two types of cystic lymphangioma; a monomorphic and a polymorphic type. IRM is particularly valuable for the assessment of the extent of the disease all the more so as these tumours are often infiltrating. One should also recognise cervicomediastinal lymphangiomas which are more frequent in the very young and in whom the diagnosis is easy but one should not ignore the mediastinal component and those lymphangiomas which are purely mediastinal.

Therapeutic management of osteoid osteomas may vary from conservative medical treatment to more aggressive, though effective, surgery. Based on observations in 4 cases in children, we demonstrate the importance of localizing the lesion on the CT-scan before percutaneous resection of the osteoid osteoma involving small bones since this method is the most conservative surgical procedure.

Eosinophilic granuloma of bone or Langerhans cell histiocytosis is mostly unifocal. It appears on plain X Ray as a solitary destructive lesion of long bones or flat bones. CT is useful to define the extension to the cortical bone and also to precisely localize the lesion when the anatomy is complex (hip, spine, base of the skull). MR is very useful in case of more aggressive lesions when there is extension to soft tissues. Differential diagnosis includes circumscribed osteitis and tumors in the case of extensive destruction. The natural course of solitary lesions is favorable, spontaneously or with therapy. The prognosis is more serious in the case of multiple lesions.

The authors report a retrospective series of 22 cases of bronchogenic cysts in children. The development of antenatal ultrasonography allowed the diagnosis in 3 cases. When the diagnosis is suggested for the time first postnatally, chest x-ray and oesophagography remain the essential initial investigations. Thoracic ultrasonography needs to be developed, as in some cases it can reveal the cystic nature of the tumour. In difficult cases, the work-up should be completed by CT scan, while keeping in mind that not all bronchogenic cysts present in the form of low-density images. A detailed review of the literature shows that mediastino-pulmonary opacities are more frequent than exclusively pulmonary images in the form of hyperlucent, fluid level or solid opacity images.

Striated osteopathy, a roentgenological entity characterized by longitudinal striations, can occur concomitantly with a number of disorders which should be routinely looked for. There are three types of striated osteopathy. The pure form without severe bone disorders is the form originally reported by Voorhoeve. In the second form, there is a concomitant fibrous dysplasia, such as Jaffe-Lichtenstein-Uehlinger disease, Ollier enchondromatosis, sponastrism dysplasia, or osteopetrosis. This group also includes bone dysplasias with sclerosis such as melorheostosis, osteopecilia, and sclerosis of the base of the skull which is sometimes seen in patients with striated osteopathy. In the third form, striated osteopathy occurs concomitantly with a skin disease such as patchy dermal hypoplasia or hyperpigmented skin lesions.

A case of a 6-year-old boy with a painful dorsal scoliosis secondary to an osteoid osteoma in a rib is described. Surgical excision was performed and the scoliosis decreased. Such cases are very rare.

Ultrasound examination of the abdomen permits us to diagnose in childhood much more bile duct calculi than before. Surgery is not necessary in all cases but a follow-up with ultrasound is usefull.

Two unusual cases of non-ossifying fibromas in boys of 4 years with partial cystic degeneration are presented. The osseous cavities did not respond to corticosteroids.

Four pediatric cases of peptic esophagitis in patients with severe dorsolumbar scoliosis including three with a history of neurological disease provide the opportunity to point out that curvature of the spine fairly often causes development of gastroesophageal reflux. By displacing the anchoring points of the stomach and stretching the lower esophageal sphincter, scoliosis can be responsible for malposition of the cardia and fundus and for gastroesophageal reflux. Furthermore, plaster corsets increase intraabdominal pressure and may therefore promote gastroesophageal reflux.

The sonographic expression of the hemolytic-uremic syndrome in children is a cortical hyper-echogenicity, which has a real prognostic value in comparison to the clinical criteria. In addition, renal ultrasonography with Doppler is useful to predict the evolution of the condition, and may help the clinician to define the therapeutic choices.

Separation of the distal humeral epiphysis is infrequent in young children. Diagnosis is difficult since the entire condylar region is almost completely composed of cartilage. Marked sequelae occur if the diagnosis is not promptly made and arthrography is required to evaluate the extent of lesions. Two cases in children aged 2 1/2 years and one day are reported.

Fractures of the lateral condyle of the humerus classified as type IV in Salter schema are difficult to diagnose when the capitellum is not ossified. Therefore it is necessary to know exactly the radiological lines which are constructed from the humerus and the radius to the capitellum. Diagnosis is also difficult when the ossification center of the capitellum humeri is only slightly displaced. The epiphyseal fracture line which is always visible at surgery can be missed on X-Ray when ossification has not yet appeared. Laminograms are necessary in some cases to be sure that the fracture is displaced. Displaced fractures are always treated by open reduction.

The authors describe a recurrent case of an hemangiopericytoma of the left serratus anterior muscle, touching a 21 year-old woman. The different ways of imaging are exposed and compared with data of literature. It seems that none of them offers any decisive possibility of nature diagnosis. Conversely, radiological examinations are of greatest interest in the initial lesional statement, in the follow up and in early discovery of a possible recurrence, principally in case of difficulties in the pathological examination. CT appears to have the first role among the different ways of medical imaging, initially and in the appreciation of clinical course.