Gianmichele Magnano

CT and MRI of paediatric Crohn disease.

Over the past two decades there has been considerable evolution in cross-sectional imaging modalities for the evaluation of Crohn disease (CD) in children. CT and MRI have contributed to conventional techniques so that now radiology has an even greater role in the management of CD, monitoring disease progression and detecting complications. The role of CT and MRI, their limitations, and the various imaging features that the radiologist should be aware of are discussed in this review.

MRI versus conventional measures of disease activity and structural damage in evaluating treatment efficacy in juvenile idiopathic arthritis

Objective To compare the American College of Rheumatology paediatric (ACRp) response criteria and conventional radiography with MRI findings in a cohort of patients with juvenile idiopathic arthritis. Methods Forty consecutive patients (30 girls, 10 boys; median age 10.8 years) with arthritis of the wrist starting treatment with disease-modifying antirheumatic drugs or biological agents were recruited. At 1-year follow-up the treatment response was assessed by ACRp criteria and radiographic progression using the adapted Sharp/van der Heijde method. Wrist MRIs were evaluated using both the paediatric-MRI and the OMERACT rheumatoid arthritis MRI scores. Sensitivity to change of clinical and imaging variables was assessed by standardised response mean (SRM) and relative efficiency (RE) was used to compare SRMs. Results ACRp90 responders showed a significantly higher decrease in MRI synovitis score (median change −4) than non-responders (median change 0), ACRp30–50 responders (median change 0) and ACRp70 responders (median change −1) (p=0.0006, Kruskal–Wallis test). Non-responders showed significantly higher radiographic progression than ACRp90 responders (pB=0.016). The MRI synovitis score showed a greater responsiveness to change (SRM 1.69) compared with the majority of ACR core set of variables. MRI erosion scores were less responsive than conventional radiography in detecting destructive changes (RE <1). MRI follow-up revealed no signs of inflammation in four out of 24 wrists with clinically inactive disease. Conclusion Only ACRp90 responders showed a significant decrease in synovitis and the halting of structural damage, suggesting that levels of response higher than ACRp30 are more appropriate for assessing drug efficacy. The excellent responsiveness of MRI and its ability to detect subclinical synovitis make it a promising outcome measure.

MRI of the wrist in juvenile idiopathic arthritis: proposal of a paediatric synovitis score by a consensus of an international working group. Results of a multicentre reliability study

BackgroundMRI is a sensitive tool for the evaluation of synovitis in juvenile idiopathic arthritis (JIA).ObjectiveThe purpose of this study was to introduce a novel MRI-based score for synovitis in children and to examine its inter- and intraobserver variability in a multi-centre study.Materials and methodsWrist MRI was performed in 76 children with JIA. On postcontrast 3-D spoiled gradient-echo and fat-suppressed T2-weighted spin-echo images, joint recesses were scored for the degree of synovial enhancement, effusion and overall inflammation independently by two paediatric radiologists. Total-enhancement and inflammation-synovitis scores were calculated.ResultsInterobserver agreement was poor to moderate for enhancement and inflammation in all recesses, except in the radioulnar and radiocarpal joints. Intraobserver agreement was good to excellent. For enhancement and inflammation scores, mean differences (95 % CI) between observers were −1.18 (−4.79 to 2.42) and −2.11 (−6.06 to 1.83). Intraobserver variability (reader 1) was 0 (−1.65 to 1.65) and 0.02 (−1.39 to 1.44).ConclusionIntraobserver agreement was good. Except for the radioulnar and radiocarpal joints, interobserver agreement was not acceptable. Therefore, the proposed scoring system requires further refinement.

Novel automated system for magnetic resonance imaging quantification of the inflamed synovial membrane volume in patients with juvenile idiopathic arthritis

To introduce a novel automated method for the quantification of the inflamed synovial membrane volume (SV) using magnetic resonance imaging (MRI), and to investigate its feasibility and validity in patients with juvenile idiopathic arthritis (JIA).

Multislice CT in congenital bronchopulmonary malformations in children

Congenital bronchopulmonary malformations encompass a wide spectrum of pathologies involving the lungs, trachea and bronchi, pulmonary vessels, and oesophagus. These developmental lesions are often isolated, but the association of two or more anomalies is not infrequent. Contrast-enhanced multidetector computed tomography (MDCT), thanks to multiplanar and 3D reconstructions, allows for detailed studies of these malformations, achieving better accuracy compared with conventional techniques such as chest X-ray, fluoroscopy, ventilation and perfusion scintigraphy and ultrasonography. MDCT is characterised by fast data acquisition and does not require sedation in the majority of cases. The main drawbacks of MDCT are the use of ionising radiation and — in many cases —contrast media. Recently, improved CT scanners and optimised CT protocols have made available to children all the benefits of MDCT, thanks to a significant reduction in radiation dose and an improved risk-benefit ratio. The aim of our paper was to evaluate MDCT in children with bronchopulmonary malformations by reporting our experience (about 2,400 studies in 30 months with a 64-slice MDCT scanner) and comparing it with the available literature.RiassuntoLe malformazioni congenite broncopolmonari rappresentano un ampio spettro di patologie che interessano il parenchima polmonare, il sistema vascolare, le vie aeree centrali e l’esofago. I pazienti affetti presentano quadri malformativi semplici o complessi, rispettivamente caratterizzati da una o più anomalie associate. La tomografia computerizzata (TC) volumetrica multidettetore (MDCT) con mezzo di contrasto permette di analizzare dettagliatamente le strutture toraciche coinvolte nelle malformazioni, con ricostruzioni multiplanari e 3D, con un’accuratezza superiore ad altre indagini tradizionalmente utilizzate tra cui il radiogramma toracico, gli studi fluoroscopici, l’ecografia e la scintigrafia ventilatoria perfusionale. Inoltre la rapidità di acquisizione dei dati consente di evitare nella maggior parte dei casi la sedazione del bambino. Lo svantaggio è rappresentato dall’utilizzo di radiazioni ionizzanti e dalla necessità di somministrare mezzo di contrasto per via endovenosa. Recentemente l’evoluzione tecnologica degli scanner TC e la definizione di protocolli pediatrici dedicati ha permesso una significativa riduzione della dose assorbita, pertanto oggi è possibile usufruire delle potenzialità della MDCT nello studio del torace del bambino con un rapporto rischio-beneficio ragionevolmente vantaggioso. Il nostro articolo si propone di valutare i differenti aspetti dell’impiego della TC sul bambino analizzando la letteratura e confrontandola con l’esperienza personale, unica in Italia: circa 2400 TC del torace in 30 mesi in pazienti compresi tra 1 giorno e 16 anni con un apparecchio a 64 strati.

Intra-articular venous malformations of the knee.

Background: Intra-articular venous malformations (IAVM) of the knee represent a rare group of low-flow vascular malformations, mainly reported in the literature as synovial hemangiomas, usually with an onset in early childhood. The main symptoms and signs are knee pain, swelling, and hemarthrosis. These lesions are slowly progressive and can lead to chronic synovitis and joint damage. Confusing nomenclature and classification of these lesions have often led to misdiagnosis and inappropriate treatment. We report our experience in the treatment of 14 consecutive patients affected by knee IAVM. Methods: We carried out a retrospective study based on the review of the medical records and diagnostic imaging of the patients admitted to our department from October 1999 to June 2009, and discharged with the final diagnosis of IAVM of the knee. Results: We observed 14 consecutive patients (8 boys, 6 girls); the median age at symptom onset was 5 years (range, 2.5 to 13 y). Magnetic resonance imaging was diagnostic in all cases. Surgical resection associated with a wide synoviectomy was the therapeutic procedure of choice in all cases. Within 6 months after surgery and physiotherapy, all the patients were symptom free with a full or at least acceptable knee range of motion. CD34 staining carried out in the last 7 cases of our series showed labeling of the endothelium of the thick-walled vessels, whereas the endothelium of the thin-walled vascular spaces was not stained. This finding of unstained vascular spaces suggests that a component of lymphatic vessels mixed with dysplastic blood vessels may be frequently present in these abnormalities. Conclusions: Magnetic resonance imaging plays a pivotal role in identifying the lesion. Surgical excision is always indicated and should be performed as early as possible to avoid lesion progression and to reduce the risk of chondral degeneration. According to the microscopic features of our resected specimens, we suggest that these lesions of the knee should be more properly named as IAVM instead of hemangiomas. Level of Evidence: Level IV.

A prospective observational study of associated anomalies in Hirschsprung’s disease

BackgroundAssociated anomalies have been reported in around 20% of Hirschsprung patients but many Authors suggested a measure of underestimation. We therefore implemented a prospective observational study on 106 consecutive HSCR patients aimed at defining the percentage of associated anomalies and implementing a personalized and up-to-date diagnostic algorithm.MethodsAfter Institutional Ethical Committee approval, 106 consecutive Hirschsprung patients admitted to our Institution between January 2010 and December 2012 were included. All families were asked to sign a specific Informed Consent form and in case of acceptance each patient underwent an advanced diagnostic algorithm, including renal ultrasound scan (US), cardiologic assessment with cardiac US, cerebral US, audiometry, ENT and ophthalmologic assessments plus further specialist evaluations based on specific clinical features.ResultsMale to female ratio of our series of patients was 3,4:1. Aganglionosis was confined to the rectosigmoid colon (classic forms) in 74,5% of cases. We detected 112 associated anomalies in 61 (57,5%) patients. The percentage did not significantly differ according to gender or length of aganglionosis. Overall, 43,4% of patients complained ophthalmologic issues (mostly refraction anomalies), 9,4% visual impairment, 20,7% congenital anomalies of the kidney and urinary tract, 4,7% congenital heart disease, 4,7% hearing impairment or deafness, 2,3% central nervous system anomalies, 8,5% chromosomal abnormalities or syndromes and 12,3% other associated anomalies.ConclusionsOur study confirmed the underestimation of certain associated anomalies in Hirschsprung patients, such as hearing impairment and congenital anomalies of the kidney and urinary tract. Subsequently, based on our results we strongly suggest performing renal US and audiometry in all patients. Conversely, ophthalmologic assessment and cerebral and heart US can be performed according to guidelines applied to the general population or in case of patients with suspected clinical features or chromosomal abnormalities. This updated diagnostic algorithm aims at improving overall outcome thanks to better prognostic expectations, prevention strategies and early rehabilitation modalities. The investigation of genetic background of patients with associated anomalies might be the next step to explore this intriguing multifactorial congenital disease.

Computerized tomography in pediatric oncology

Computerized tomography (CT) is an extremely powerful imaging modality, which provides extremely valuable information for the diagnosis, staging, and management of pediatric solid tumors. In recent years, the concern of potential risks associated with ionizing radiation from diagnostic imaging - especially from CT - has greatly increased. In children with cancer the radiation burden from CT studies can easily accumulate because of repeated studies for disease staging, assessment of response to therapy, and follow up. The purpose of this article is to review the role of CT and its imaging key points for diagnosis, staging and planning surgical excision of common extracranial pediatric tumors, according to protocol specific imaging guidelines. The issue of the radiation burden from CT in children with cancer, and criteria of good practice to reduce it, will also be discussed.

Lesioni traumatiche dei surreni in età pediatrica: A proposito di tre casi

Pediatric adrenal injuries, in blunt thoracoabdominal trauma, are rare and usually associated with traumatic liver and kidney lesions. This paper aims to present imaging findings and possible adrenal involvement in blunt abdominal traumas in childhood. We report three cases of thoracoabdominal trauma with adrenal involvement. Two patients were polytraumatised in car accidents. The third case was unusual because of the mild trauma. The adrenals lesions were right-sided in all cases. Post-traumatic adrenal contusion/haematoma may arise not only because of a direct trauma but also as a consequence of a sudden increase in the pressure in the inferior vena cava system–adrenal veins. This is why adrenal haemorrhage is not directly proportional to the trauma: compression of the inferior vena cava leads to increased pressure in the adrenal venous circulation, which supports the parenchymal lesion. The right adrenal gland is more frequently injured than the left gland: it can be easily compressed between the liver, spine and kidney, and its venous drainage flows directly into the inferior vena cava.

Traumatic lesions of adrenal glands in paediatrics: about three cases.

Pediatric adrenal injuries, in blunt thoracoabdominal trauma, are rare and usually associated with traumatic liver and kidney lesions. This paper aims to present imaging findings and possible adrenal involvement in blunt abdominal traumas in childhood. We report three cases of thoracoabdominal trauma with adrenal involvement. Two patients were polytraumatised in car accidents. The third case was unusual because of the mild trauma. The adrenals lesions were right-sided in all cases. Post-traumatic adrenal contusion/haematoma may arise not only because of a direct trauma but also as a consequence of a sudden increase in the pressure in the inferior vena cava system–adrenal veins. This is why adrenal haemorrhage is not directly proportional to the trauma: compression of the inferior vena cava leads to increased pressure in the adrenal venous circulation, which supports the parenchymal lesion. The right adrenal gland is more frequently injured than the left gland: it can be easily compressed between the liver, spine and kidney, and its venous drainage flows directly into the inferior vena cava.