Role of Cross-Sectional Imaging in Pediatric Inflammatory Bowel Disease.

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.

Agreement between clinicoradiological signs at diagnosis and radiohistological analysis after neoadjuvant chemotherapy of suspected Wilms tumor rupture: Consequences on therapeutic choices.

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.

Recurrence of Solid Pseudopapillary Neoplasms of the Pancreas: Results of a Nationwide Study of Risk Factors and Treatment Modalities.

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.

Chronic urinary obstruction: evaluation of dynamic contrast-enhanced MR urography for measurement of split renal function.

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.

Idiopathic midaortic syndrome: normalization of blood pressure on medication.

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.

Segmental omental infarction in childhood: an unusual case of left-sided location with extension into the pelvis.

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.

Thoracic calcifications in childhood.

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.