[Show abstract][Hide abstract] ABSTRACT: Background Multidetector CT (MDCT) scanners have contributed to the widespread use of CT in paediatric imaging. However, concerns are raised for the associated radiation exposure. Very few surveys on radiation exposure from MDCT studies in children are available. Objective The aim of this study was to outline the status of radiation exposure in children from MDCT practice in Italy. Materials and methods In this retrospective multicentre study we asked Italian radiology units with an MDCT scanner with at least 16 slices to provide dosimetric and acquisition parameters of CT examinations in three age groups (1-5, 6-10, 11-15 years) for studies of head, chest and abdomen. The dosimetric results were reported in terms of third-quartile volumetric CT dose index (CTDIvol) (mGy), size-specific dose estimate (SSDE) (mGy), dose length product (DLP) (mGy cm), and total DLP for multiphase studies. These results were compared with paediatric European and adult Italian published data. A multivariate analysis assessed the association of CTDIvol with patient characteristics and scanning modalities. Results We collected data from 993 MDCT examinations performed at 25 centres. For age groups 1-5 years, 6-10 years and 11-15 years, the CTDIvol, DLP and total DLP values were statistically significantly below the values observed in our analogous national survey in adults, although the difference decreased with increasing age. CTDIvol variability among centres was statistically significant (variance = 0.07; 95% confidence interval = 0.03-0.16; P . Conclusions This study reviewed practice in Italian centres performing paediatric imaging with MDCT scanners. The variability of doses among centres suggests that the use of standardised CT protocols should be encouraged.
[Show abstract][Hide abstract] ABSTRACT: Neuroblastoma in the adult is rare. No established therapeutic guidelines exist for these patients and the literature on this issue is scant and contradictory.
Between 1986 and 2011, 21 adults (18 to 38 y; median, 23) diagnosed with neuroblastoma were referred to our hospital. Three of the 21 were classified as neuroblastoma, not otherwise specified, 13 as neuroblastoma, schwannian stroma-poor, and 5 as ganglioneuroblastoma, nodular. Nine patients had a resectable (stage 1/2) and 6 an unresectable primary tumor (stage 3); 6 had disseminated disease (stage 4).
Of 9 stage 1/2 patients, 6 underwent surgery alone (2 survive, 4 died), 2 received adjuvant chemotherapy (both survive), and 1 received radiation therapy (alive). Four of the 6 stage 3 patients received chemotherapy and died, 1 underwent partial tumor resection only and died, and 1 received radiation therapy after partial tumor resection and is alive. The 6 stage 4 patients received chemotherapy with/without radiotherapy, and all died. Event-free survival at 10 years was 33.3% for stage 1/2, 16.7% for stage 3, and 0% for stage 4 patients. The 10-year overall and event-free survival rates were 39.8% and 19.1%, respectively.
The outcome of neuroblastoma in adults is poorer than in younger patients at all stages. The clinical course seems modestly influenced by therapy.
[Show abstract][Hide abstract] ABSTRACT: Over the last 20 years MIS has progressively gained popularity in children with cancer. We therefore aimed at evaluating the safety of Minimally Invasive Surgery (MIS) resection in a series of children affected by adrenal neuroblastoma (NB) presenting without Image-Defined Risk Factors (IDRFs).
An Institutional protocol for MIS resection of adrenal NB in pediatric patients without IDRFs has been applied since 2008. Absence of IDRFs represented the main indication for MIS in NB, regardless of tumor size. All pediatric patients who underwent MIS for NB between January 2008 and May 2013 were included. Specific technical considerations, demographic data, and outcome have been recorded.
Twenty-one patients underwent MIS resection for IDRFs-negative adrenal NB. Nine of these patients experienced preoperative downgrading of IDRFs after chemotherapy. Radiological median diameter of the mass was 30 mm (range 10-83 mm). Median operative time was 90 min. Median hospital stay was 4 days. All patients were treated successfully, without serious intraoperative complications. One mild intraoperative hemorrhage occurred and was treated without the need for conversion to open surgery nor blood transfusion was required. No postoperative complications, including port-site or peritoneal metastases were experienced.
This study demonstrated the safety and effectiveness of MIS for the resection of adrenal NB without IDRFs in children. Pediatric surgeons dedicated to oncology should be aware of this alternative approach to open resection.
Pediatric Surgery International 01/2014; 30(4). DOI:10.1007/s00383-014-3476-z · 1.00 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Associated anomalies have been reported in around 20% of Hirschsprung patients but many Authors suggested a measure of underestimation. We therefore implemented a prospective observational cross-sectional study on 106 consecutive HSCR patients aimed at defining the percentage of associated anomalies and implementing a personalized and up-to-date diagnostic algorithm.
After Institutional Ethical Committee approval, 106 consecutive Hirschsprung patients admitted to our Institution between January 2009 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.
Male 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.
Our 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.
[Show abstract][Hide abstract] ABSTRACT: To evaluate the radiation dose in routine multidetector computed tomography (MDCT) examinations in Italian population.
This was a retrospective multicentre study included 5,668 patients from 65 radiology departments who had undergone common CT protocols: head, chest, abdomen, chest-abdomen-pelvis (CAP), spine and cardiac. Data included patient characteristics, CT parameters, volumetric CT dose index (CTDIvol) and dose length product (DLP) for each CT acquisition phase. Descriptive statistics were calculated, and a multi-regression analysis was used to outline the main factors affecting exposure.
The 75th percentiles of CTDIvol (mGy) and DLP (mGy cm) for whole head were 69 mGy and 1,312 mGy cm, respectively; for chest, 15 mGy and 569 mGy cm; spine, 42 mGy and 888 mGy cm; cardiac, 7 mGy and 131 mGy cm for calcium score, and 61 mGy and 1,208 mGy cm for angiographic CT studies. High variability was present in the DLP of abdomen and CAP protocols, where multiphase examinations dominated (71 % and 73 % respectively): for abdomen, 18 mGy, with 555 and 920 mGy cm in abdomen and abdomen-pelvis acquisitions respectively; for CAP, 17 mGy, with 508, 850 and 1,200 mGy cm in abdomen, abdomen-pelvis and CAP acquisitions respectively.
The results of this survey could help in the definition of updated diagnostic reference levels (DRL).
• Radiation dose associated with multidetector CT (MDCT) is an important health issue. • This national survey assessed dose exposures of 5,668 patients undergoing MDCT. • Dose indices correlate with BMI, voltage, rotation time, pitch and tube current. • These results may contribute to an update of national diagnostic reference levels.
European Radiology 10/2013; 24(2). DOI:10.1007/s00330-013-3031-7 · 4.01 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: 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.
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.
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.
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.
[Show abstract][Hide abstract] ABSTRACT: 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.
European journal of radiology 12/2011; 82(7). DOI:10.1016/j.ejrad.2011.12.008 · 2.37 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Neuroblastoma is an enigmatic disease entity; some tumors disappear spontaneously without any therapy, while others progress with a fatal outcome despite the implementation of maximal modern therapy. However, strong prognostic factors can accurately predict whether children have "good" or "bad" disease at diagnosis, and the clinical stage is currently the most significant and clinically relevant prognostic factor. Therefore, for an individual patient, proper staging is of paramount importance for risk assessment and selection of optimal treatment. In 2009, the International Neuroblastoma Risk Group (INRG) Project proposed a new staging system designed for tumor staging before any treatment, including surgery. Compared with the focus of the International Neuroblastoma Staging System, which is currently the most used, the focus has now shifted from surgicopathologic findings to imaging findings. The new INRG Staging System includes two stages of localized disease, which are dependent on whether image-defined risk factors (IDRFs) are or are not present. IDRFs are features detected with imaging at the time of diagnosis. The present consensus report was written by the INRG Imaging Committee to optimize imaging and staging and reduce interobserver variability. The rationales for using imaging methods (ultrasonography, magnetic resonance imaging, computed tomography, and scintigraphy), as well as technical guidelines, are described. Definitions of the terms recommended for assessing IDRFs are provided with examples. It is anticipated that the use of standardized nomenclature will contribute substantially to more uniform staging and thereby facilitate comparisons of clinical trials conducted in different parts of the world.
[Show abstract][Hide abstract] ABSTRACT: 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.
La radiologia medica 02/2011; 116(1):133-51. DOI:10.1007/s11547-010-0582-4 · 1.34 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Tunneled indwelling central venous catheters (CVC) are essential in the management of children with cancer, hematological, nephrological disorders and for parenteral nutrition. The aim of this study is to present the experience of a single center of the transition from traditional open surgical cut down procedure (OSC) to ultrasound (US)-guided percutaneous CVC insertion, focusing on learning curve and related complications.
All CVCs inserted between April 2008 and November 2009 in children at the Gaslini Children Hospital were revised, and data on methods of cannulation, intraoperative and device-related complications and re-intervention were recorded.
194 CVCs were positioned in 188 patients. 128 out of 194 CVCs were positioned through an OSC technique, whereas the remaining 66 CVCs were inserted percutaneously with US guidance. Of the 27 recorded complications, 15 were mechanical events, 7 cases developed infection, whereas the remaining 5 (2.6%) were classified as intraoperative complications. A second surgical procedure was described in 23 (11.8%) cases.
Shifting from OSC to US-guided percutaneous CVC insertion inevitably involves a challenging learning curve which is generally associated with high complication rates. Complications progressively decrease once a good experience in US guidance and percutaneous technique has been obtained.
Pediatric Surgery International 08/2010; 26(8):819-24. DOI:10.1007/s00383-010-2636-z · 1.00 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Beckwith-Wiedemann syndrome is associated with an increased risk of tumors in the pediatric age. We report the case of a newborn with Beckwith-Wiedemann syndrome presenting the simultaneous occurrence of a cystic pancreatoblastoma and an adrenal neuroblastoma. Surgery was required to treat the pancreatoblastoma, and a "wait and see" policy was adopted for the neuroblastoma, which spontaneously regressed within a few months.
[Show abstract][Hide abstract] ABSTRACT: A case of neonatal bilateral adrenal mass causing severe respiratory distress and requiring an emergency debulking surgical procedure is reported. Histopathology revealed a cystic neuroblastoma stroma poor, poorly differentiated, without MYCN amplification and 1p deletion. During postoperative follow-up, a progression to stage 4s was observed, characterized by liver involvement. According to good prognostic indexes, no further treatment was administered. Both adrenal masses and hepatic nodules showed progressive decrease in size, till complete disappearance. The authors encourage a multidisciplinary approach to develop the best patient-related strategy for cystic neuroblastoma, thus reducing complications rate.
[Show abstract][Hide abstract] ABSTRACT: Presenting features, treatment and outcome of 134 newborns with neuroblastoma diagnosed over a 27-year period are described.
Analyses were performed on the entire cohort and on patients distributed over three periods of diagnosis.
Twenty-seven tumours (20.1%) were detected prenatally. Localised disease prevailed (65.7%) with an increase of stage 1 patients over time from 18.8% to 46.5%. Disseminated disease accounted for 34.3% of tumours with only one stage 4 and 45 stage 4S. Five-year overall survival (OS) of the entire cohort was 88.3%. Five/88 patients with localised disease died, including three who died of complications (OS, 95.3%). The only stage 4 patient survived. Eleven/45 stage 4S patients died, including 7/18 symptomatic and 4/27 asymptomatic (OS, 74.1%).
The outcome of neuroblastoma in newborns is excellent. In localised tumours, surgery-related deaths outnumbered deaths due to disease. Symptomatic stage 4S patients were at greater risk of dying.
European journal of cancer (Oxford, England: 1990) 09/2009; 45(18):3220-7. DOI:10.1016/j.ejca.2009.08.020 · 5.42 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Lipoblastomas are rare benign tumors of white fatty tissue that occur primarily in young children. Occasionally, heterogeneity of morphological appearance and histological overlap with other lipogenic tumors are described. In such cases fluorescence in situ hybridization (FISH) analysis of PLAG1, a gene specifically rearranged in lipoblastoma, is necessary to prevent misdiagnosis. We present a case of lipoblastoma arising in an atypical site with histological features characteristic of lipoma. The correct diagnosis was made possible on cytogenetic grounds through the identification of the characteristic PLAG1-HAS2 fusion gene, thus allowing an appropriate clinical approach.
[Show abstract][Hide abstract] ABSTRACT: Viral infections are a rare complication in autologous hemopoietic stem cell transplant (HSCT) recipients but represent a frequent cause of disease after allogeneic HSCT. In the last years, there has been an increase in the number of viral diseases observed in these patients. This fact may be at least partially due to an improvement in diagnostic facilities, but the increasing number of transplant procedures and the more severe immunosuppression may also have played an important role.
[Show abstract][Hide abstract] ABSTRACT: Treatment of pediatric patients with varicocele is based on the desire to prevent testicular dysfunction and possible infertility that may become irreversible in adults. The authors reviewed their experience with retrograde percutaneous sclerotherapy (RPS) via a trans-femoral approach to assess its results in children. A retrospective study was conducted, including all the children admitted between 2000 and 2004 who underwent RPS with at least 24 months follow-up (FU). The indication for treatment was grade II and III varicocele (Dubin-Amelar classification), confirmed by Doppler US. Three per cent sodium tetradecyl sulfate was the sclerosing agent. FU included Doppler US 1 month after the procedure and then yearly for at least 2 years. Eighty-six children (mean age 13.8 years) underwent phlebography and, when feasible, RPS. Varicocele was grade II in 49 cases and grade III in 37. RPS was feasible in 72 (84%) children because of unfavourable anatomic features of the left spermatic vein. RPS was successful in 66 (92%) out of 72 treated children. Median FU was 29 months. During FU, five (8%) patients relapsed. RPS of varicocele is a simple and effective treatment. A minimum of 2 years FU is recommended, as a few cured cases may relapse during this period.
Pediatric Surgery International 06/2008; 24(5):583-7. DOI:10.1007/s00383-008-2124-x · 1.00 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Invasive mycoses represent a rare but severe complication following hemopoietic SCT (HSCT) in children. Their incidence is related to the type of donor, being higher after allogeneic transplant, especially from alternative donors. Moreover, the incidence of invasive mycoses varies in the different post transplant phases. Neutropenia, lymphopenia, GvHD, high-dose steroids or other immunosuppressive drugs represent well-known risk factors. The clinical features of invasive mycoses after HSCT in children are similar to those observed in adults, and the diagnostic tools, including Aspergillus galactomannan antigen detection, are feasible also in pediatrics. Mortality due to invasive mycoses after HSCT in children is high.