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Congenital portosystemic shunts may result in the development of hepatopulmonary syndrome, typically presenting with progressive hypoxemia in later childhood. We describe a case of a 5-month-old male with heterotaxy with polysplenia presenting with new onset hypoxemia. Subsequent evaluation identified an extrahepatic portosystemic shunt arising from the confluence of the main portal and superior mesenteric veins draining into the left renal vein. To treat his hypoxemia and prevent future complications of shunting, the patient underwent a successful single-stage endovascular closure. [ABSTRACT FROM AUTHOR]

There is growing interest in the use of contrast-enhanced ultrasound (CEUS) in diagnostic and interventional radiology. CEUS applications in interventional radiology are performed with intravascular or intracavitary administration of microbubble-based US contrast agents to allow for real-time evaluation of their distribution within the vascular bed or in body cavities, respectively, providing additional information beyond gray-scale US alone. The most common interventional-radiology-related CEUS applications in children have been extrapolated from those in adults, and they include the use of CEUS to guide lesion biopsy and to confirm drain placement in pleural effusions and intra-abdominal fluid collections. Other applications are emerging in interventional radiology for use in adults and children, including CEUS to optimize sclerotherapy of vascular malformations, to guide arthrography, and for lymphatic interventions. In this review article we present a wide range of interventional-radiology-related CEUS applications, emphasizing the current and potential uses in children. We highlight the technical parameters of the CEUS examination and discuss the main imaging findings. [ABSTRACT FROM AUTHOR]

Workflow optimization may result in the provider seeing the patient subsequent to the APP, and completing some aspects of the consult documentation, but this still alleviates much workload for the provider, particularly for same day consult services. In addition, reviewing the documentation and relevant imaging with the APP and performing a brief patient evaluation provide for a more comprehensive less hurried evaluation by the provider, and in my opinion a safer patient experience. As pediatric interventional radiology (IR) practice has grown and become a much needed resource in most pediatric hospital systems, the need for a dedicated pediatric IR consult service has also evolved. [Extracted from the article]

Central venous lines may require contrast injection under fluoroscopy to evaluate for dysfunction such as occlusion, fibrin sheath development or catheter fracture. Rarely, some patients may be too ill to travel to the interventional radiology suite for this examination. We present a case utilizing contrast-enhanced ultrasound (CEUS) at the bedside to assess catheter integrity in a critically ill infant with a large intra-abdominal fluid collection. CEUS demonstrated extravasation into the collection, confirming catheter fracture and prompting immediate cessation of line use and recommendation for exchange. This case shows the utility of CEUS to evaluate central venous access devices in children who are unable to travel to interventional radiology for a standard contrast injection. [ABSTRACT FROM AUTHOR]

Unlike autosomal dominant polycystic kidney disease (ADPKD), autosomal recessive polycystic kidney disease (ARPKD) is not generally known to be associated with vascular abnormalities. Only 4 cases of ARPKD patients with intracranial aneurysms have been reported previously. We present 2 ARPKD patients with extracranial vascular abnormalities: a young man with infrarenal aortic and iliac artery aneurysms complicated by dissection and a teenage girl with multiple splenic and gastric artery aneurysms and arterial vascular malformations. These cases raise the question of whether vascular integrity and development may be impaired in ARPKD, perhaps through molecular mechanisms overlapping with ADPKD. This possibility is supported by studies in mice that show ARPKD gene expression in the walls of large blood vessels. [ABSTRACT FROM AUTHOR]

Background: Accidental dislodgment is one of the most frequent causes of avoidable cost and consternation related to gastrostomy tubes. The Sacks-Vine gastrostomy tube, inserted in an antegrade fashion by a percutaneous technique, is extremely stable due to the construction of its disc retention device. However, transmural migration of the retention disc is a known severe delayed complication associated with this tube.Objective: To review the presentation, diagnosis, and treatment of transmural migration of gastrostomy retention discs, to propose a mechanism for the progressive development of this complication, and to recommend a method for preventing its occurrence.Materials and Methods: From January 1991 to October 1999, pediatric interventional radiologists at two children's hospitals performed 300 antegrade gastrostomy and gastrojejunostomy primary insertion procedures. A "push-pull" variation of the antegrade approach used for 44 of these insertions is excluded from further analysis. Of the remaining 256 procedures, 5 boys and 3 girls with a mean age of 5.1 years (range 0.8-19 years) were identified from review of the prospectively gathered procedural database with significant complications related to the disc retention component of their gastrostomy tubes. A retrospective analysis was conducted of their medical records, diagnostic imaging, and interventional and surgical findings.Results: Transmural migration was diagnosed on average 36 months after insertion (16-48 months). The diagnosis was made incidentally during endoscopy (n=1) or fluoroscopy (n=5) in six asymptomatic patients, and during barium enema to explore feculent vomiting and halitosis in two symptomatic patients. Migration of the retention disc fell along a continuum from intramural (n=4) to transmural and intracolonic (n=4), with gastric mucosal erosion, extensive granulation and inflammation in all eight patients. Although there was no evidence of free air in any patient, a gastrocolic fistula was demonstrated in four patients and a gastrocolocutaneous fistula in two of four patients with complete transmural migration. Surgical resection of the disc, gastrostomy, and fistula repair if needed was successfully performed in all patients.Conclusions: Gastrostomy tubes with an internal retention disc are at risk for progressive disc migration into and through the gastric wall, resulting in irretrievable fixation and potential fistula formation. This severe delayed complication results from prolonged traction on the retention disc. Transmural migration may be avoided through improved tube care education, daily disc mobilization, and earlier disc retrieval. [ABSTRACT FROM AUTHOR]

Mesenteric lymphatic malformations result from abnormal proliferation of disorganized mesenteric lymphatic channels. Sclerotherapy is often preferred over surgery as it is less invasive and has lower post-procedure morbidity. Sclerotherapy has been described as durable and effective with a low complication rate. We describe a serious complication from sclerotherapy of a lymphatic malformation extending from the mesentery through the bowel wall, highlighting the spectrum of this pathology and the need for multidisciplinary management of complex cases. [ABSTRACT FROM AUTHOR]

Background: Renal artery stenosis is an important cause of hypertension in children, accounting for 5–10% of cases. When suspected, noninvasive imaging options include ultrasound (US), computed tomography (CT) angiography and magnetic resonance (MR) angiography. However, digital subtraction angiography (DSA) remains the gold standard. Objective: To investigate the accuracy and inter-reader reliability of CT angiography in children with suspected renal artery stenosis. Materials and methods: This is a retrospective study of patients suspected of having renal artery stenosis evaluated by both CT angiography and DSA between 2008 and 2019 at a tertiary pediatric hospital. Only children who underwent CT angiography within 6 months before DSA were included. CT angiography studies were individually reviewed by two pediatric radiologists, blinded to clinical data, other studies and each other's evaluation, to determine the presence of stenosis at the main renal artery and 2nd- and 3rd-order branches. The sensitivity, specificity and accuracy were calculated using DSA as the reference. The effective radiation dose for CT angiography and DSA was also calculated. Kappa statistics were used to assess inter-reader agreement. Results: Seventy-four renal units were evaluated (18 girls, 19 boys). The patients' median age was 8 years (range: 1–21 years). Overall, CT angiography was effective in detecting renal artery stenosis with a sensitivity of 85.7%, specificity of 91.5% and accuracy of 88.9%. There was moderate inter-reader agreement at the main renal artery level (k=0.73) and almost perfect inter-reader agreement at the 2nd/3rd order (k=0.98). However, the sensitivity at the 2nd- and 3rd-order level was lower (14.3%). CT angiography provided excellent negative predictive value for evaluating renal artery stenosis at the main renal artery level (90.1%) and at the 2nd- or 3rd-order branches (82.7%). The median effective dose of CT angiography studies was 2.2 mSv (range: 0.6–6.3) while the effective dose of DSA was 13.7 mSv. Conclusion: CT angiography has high sensitivity and specificity at the main renal artery level with a lower radiation dose than previously assumed. Therefore, it can be used as a diagnostic tool in patients with low to medium risk of renal artery stenosis, and as a screening and treatment planning tool in patients at high risk. [ABSTRACT FROM AUTHOR]

Background: The management of pediatric trauma with trans-arterial embolization is uncommon, even in level 1 trauma centers; hence, there is a dearth of literature on this subject compared to the adult experience. Objective: To describe a single-center, level 1 trauma center experience with arterial embolization for pediatric trauma. Materials and methods: A retrospective review was performed to identify demographics, transfusion requirements, pre-procedure imaging, procedural details, adverse events, and arterial embolization outcomes over a 19-year period. Twenty children (age 4.5 months to 17 years, median 13.5 years; weight 3.6 to 108 kg, median 53 kg) were included. Technical success was defined as angiographic resolution of the bleeding-related abnormality on post-embolization angiography or successful empiric embolization in the absence of an angiographic finding. Clinical success was defined as not requiring additional intervention after embolization. Results: Seventy-five percent (n=15/20) of patients required red blood cell transfusions prior to embolization with a mean volume replacement 64 ml/kg (range 12–166 ml/kg) and the median time from injury to intervention was 3 days (range 0–16 days). Technical success was achieved in 100% (20/20) of children while clinical success was achieved in 80% (n=16/20). For the 4 children (20%) with continued bleeding following initial embolization, 2 underwent repeat embolization, 1 underwent surgery, and 1 underwent repeat embolization and surgery. Mortality prior to discharge was 15% (n=3). A post-embolization mild adverse event included one groin hematoma, while a severe adverse event included one common iliac artery pseudoaneurysm requiring open surgical ligation. Conclusions: In this single-center experience, arterial embolization for hemorrhage control in children after trauma is feasible but can be challenging and the clinical failure rate of 20% in this series reflects this complexity. Standardization of pre-embolization trauma assessment parameters and embolic techniques may improve outcomes. [ABSTRACT FROM AUTHOR]

Purpose: The thin and friable septations composing aneurysmal bone cysts (ABC) may be challenging to target for percutaneous biopsy. The purpose of this study was to describe and evaluate a novel method of ABC biopsy using endomyocardial biopsy forceps as an attempt to capture larger fragments of tissue for diagnosis. Methods and materials: This was a retrospective study performed over a 17-year period. Patients <18 years old who underwent percutaneous biopsy for a presumed ABC, based on pre-procedure imaging, were included. Medical records were reviewed to identify age, sex, lesion location, biopsy procedure details, complications, and pathology results. A diagnostic biopsy was defined as conclusive histologic confirmation. Inconclusive findings or findings suggestive of but not diagnostic of an ABC were treated as non-diagnostic, even if imaging and clinical findings were characteristic. Biopsy device selection and quantity of tissue obtained was at the discretion of the pediatric interventional radiologist. Fisher's exact test was used to compare the diagnostic yield of standard biopsies to those employing biopsy forceps. Results: Twenty-three biopsies were performed in 18 patients (11 female) with a median age of 14.7 years (IQR 10.6–15.6). Lesions were located in the extremities (7, 30.4%), chest (6, 26.1%), pelvis (5, 21.7%), spine (4, 17.4%), and mandible (1, 4.3%). Specimens were obtained using a 13- or 15-gauge bone coring needle (11, 47.8%); 14-, 16-, or 18-gauge soft tissue needle (6, 26.1%); or a combination of bone and soft tissue (4, 17.4%) needles. Endomyocardial biopsy forceps were utilized in 7 cases (30.4%), 2 in which it was the only device used. Overall, a conclusive pathologic diagnosis was made in 13/23 (56.5%) biopsies. Of the diagnostic biopsies, 1 was a unicameral bone cyst and all others were ABCs. No malignancy was identified. Compared to the standard approach, the use of forceps was more likely to result in a diagnostic biopsy (40.0% vs 100.0%, p = 0.008). There were no complications. Conclusions: Endomyocardial biopsy forceps offer an additional, novel technique to biopsy presumed ABCs and may improve diagnostic yield. [ABSTRACT FROM AUTHOR]

Background: Due to the rarity of pediatric diseases, collaborative research is the key to maximizing the impact of research studies. A research needs assessment survey was created to support initiatives to foster pediatric interventional radiology research. Objective: To assess the status of pediatric interventional radiology research, identify perceived barriers, obtain community input on areas of research/education/support, and create metrics for evaluating changes/responses to programmatic initiatives. Materials and methods: A survey link was sent to approximately 275 members of the Society for Pediatric Interventional Radiology (SPIR) between May and October 2020. Data was collected using a web-based interface. Data collected included practice setting, clinical role, research experience, research barriers, and suggestions for future initiatives. Results: Fifty-nine surveys were analyzed with a staff physician survey response rate of 28% (56/198). A wide range of practice sizes from 15 countries were represented. Respondents were predominantly staff physicians (95%; 56/59) with an average of 11 years (range: 1–25 years) of clinical experience working at academic or freestanding children's hospitals. A total of 100% (59/59) had research experience, and 70% (41/58) had published research with a mean of 30 peer-reviewed publications (range: 1–200). For job security, 56% (33/59) of respondents were expected or required to publish, but only 19% (11/58) had research support staff, and 42% (25/59) had protected research time, but of those, 36% (9/25) got the time "sometimes or never." Lack of support staff, established collaborative processes, and education were identified as top barriers to performing research. Conclusions: The needs assessment survey demonstrated active research output despite several identified barriers. There is a widespread interest within the pediatric interventional radiology community for collaborative research. [ABSTRACT FROM AUTHOR]

Methemoglobinemia occurs when the heme moiety of hemoglobin (Hb) is oxidized from the ferrous to ferric state, leading to impairments in oxygen transport and delivery. Methemoglobinemia is rare in pediatric patients but has been described in the setting of congenital abnormalities in the Hb structure, inherited enzyme deficiencies, oxidative Hb injury in response to illness, and oxidative Hb injury due to toxicants. We present a 1-week-old infant born with a cervical lymphangioma who developed persistent desaturations that were unresponsive to oxygen after sclerotherapy with doxycycline. Arterial blood gas revealed a high PaO2 despite low saturations being found on pulse oximetry and a methemoglobin level that was found to be elevated. Further sclerotherapy was discontinued, the saturations eventually normalized, and the methemoglobin level decreased. This is a novel report of sclerotherapy with doxycycline associated with the development of methemoglobinemia. [ABSTRACT FROM AUTHOR]

Background: Fibromuscular dysplasia (FMD) is a non-inflammatory vascular disease that in children unlike in adults shows no sex predilection. FMD is often underdiagnosed, and its pathophysiology is unclear. Delayed diagnosis may lead to refractory hypertension and decreases the chance of successful treatment. Doppler ultrasound (US), magnetic resonance angiography (MRA), computed tomography angiography (CTA), and catheter-based angiography (angiography) are currently used to help make a clinicoradiological diagnosis of FMD. The main aim of the study was to compare the efficacy of imaging modalities which can allow for earlier and improved detection. Furthermore, an anatomical mapping of the location of lesions can help determine the best treatment modalities.Methods: All patients with non-syndromic non-inflammatory renovascular hypertension were recruited from the Nephrology Department at the Children’s Hospital of Philadelphia (CHOP) and enrolled in the U.S. FMD Registry maintained at the University of Michigan. Clinical presentation and imaging findings on US, CT, and MRI of children diagnosed with FMD were evaluated.Results: Mean age at diagnosis was 7 ± 4.9 years (4 months-17 years). Family history of hypertension (HTN) (52%), FMD (8.7%), Caucasian (60%), headache (48%), and HTN (80%) were the most prevalent symptom and sign at presentation. Bruits were 100% specific for renal artery stenosis (RAS) diagnosis but were heard in the minority of patients (3 patients, 12%). FMD was mainly unifocal within a single site (68%) or multiple sites (28%) and involved the main or first order renal branch in about 68% of children. Isolated distal lesions beyond the second order branches were found in about 25% of children. US imaging was significantly less sensitive than angiography (28%, p = 0.003). MRA had a better sensitivity (62.5%, p = 0.3) than US. Overall, CTA had the best sensitivity (84.2%, p = 0.4) compared to angiography; however, only angiography showed distal vessel disease.Conclusions: Limitations of the study include the sample size and biases—only patients diagnosed with FMD were included in this study and most patients were referred to a pediatric nephrologist for unexplained hypertension. Angiography should be performed as part of the initial work-up of any child suspected of having renovascular FMD, regardless of the findings seen on US, MRA, or CTA. [ABSTRACT FROM AUTHOR]

To evaluate central venous access placement trends for radiology and non-radiology services over the last decade. Children who had central venous access procedures included in a large administrative database of 49 pediatric institutions in the United States between 2010 and 2020 were included. Patient demographics and patient specific factors were compared between groups. The percentage of procedures performed by interventional radiology (IR) and non-radiology services were compared over time and by region. A total of 483,181 vascular access encounters were recorded (45.3% female; median age 2 years (IQR 0–11 years)). Approximately one quarter of vascular access encounters were IR-led, with a slight increase of 3.8% between 2010 and 2020. Children who underwent IR-placed vascular access were older (median age of 4 years compared to 1 year in non-radiology encounters). Interventional radiology-placed access was greatest in the Midwest (33.5%) with a decrease of 5.9% over the study period; in the other three regions, IR-performed encounters increased. Patient comorbidities more prevalent in the IR encounters were technology dependence (42.4% of all radiology encounters), gastrointestinal (34.9%), respiratory (20.8%), and transplant (8.1%), while those which were more prevalent in the non-radiology encounters were nephrology/urology (21.4% of all non-radiology encounters), prematurity/neonatal (17.3%), and malignancy (17.3%). Interventional radiology-provided vascular access services have slightly increased over the last decade without significant service-line transfer to other specialties. Underlying comorbidities in IR-led vascular access encounters vary across institutions based on referral patterns, possibly reflecting the adoption of ultrasound guidance by other pediatric subspecialties. • Approximately one quarter of the pediatric vascular access encounters over the last decade were performed by IR, with a slight increase (3.8%). • Regionally, IR-provided access was greatest in the Midwest (33.5%), compared with the Northeast (23.4%), the South (22.7%), and the West (17.1%). • There was, however, a decrease in IR-performed procedures by 5.9% in the Midwest and an increase in the remaining three regions. • IR-placed pediatric vascular access has slightly increased over the last decade without significant service-line transfer to other specialties. [ABSTRACT FROM AUTHOR]

Primary percutaneous gastrostomy and gastrojejunostomy tube placements are fundamental procedures performed in pediatric interventional radiology, with both antegrade and retrograde techniques described. In pediatric patients, however, challenges may arise due to smaller patient size and anatomical variations. Several adjunctive techniques may facilitate safe percutaneous access in the setting of a limited percutaneous gastric access window. These include the intra-procedural use of cone beam computed tomography (CT), percutaneous needle decompression in the setting of distended air-filled bowel interposed between the stomach and abdominal wall, post-pyloric balloon occlusion to facilitate gastric distension, ultrasound-guided gastric puncture, and intra-gastric contrast-enhanced ultrasound (ceUS) to define the relationship of the gastric wall and the anterior abdominal wall. Adjunctive techniques may increase successful primary percutaneous gastroenteric tube placement and may improve operator confidence in safe placement. [ABSTRACT FROM AUTHOR]

Background: Venous malformations (VMs) are slow-flow vascular anomalies present at birth that enlarge during adolescence, subsequently causing thrombosis, hemorrhage, and pain. Case presentation: We describe a case of an adolescent male presenting with a large scalp venous malformation. Given the size and location of the lesion, a hybrid approach employing both sclerotherapy and surgical resection was utilized. The VM was successfully removed without complication. Conclusion: A hybrid approach is a safe and effective treatment consideration for immediate management of large venous malformation in higher-risk locations. [ABSTRACT FROM AUTHOR]

Introduction: The diagnostic utility of molecular profiling for the evaluation of indeterminate pediatric thyroid nodules is unclear. We aimed to assess pediatric cases with indeterminate thyroid fine-needle aspiration (FNA) alongside clinicopathologic features and mutational analysis. Methods: A retrospective review of 126 patients with indeterminate cytology who underwent FNA between January 2010 and December 2021 at the Children's Hospital of Philadelphia was performed. Indeterminate cases defined by The Bethesda System for Reporting Thyroid Cytopathology (AUS/FLUS or TBSRTC III; FN/SFN or TBSRTC IV; SM or TBSRTC V) were correlated to clinicopathologic and genetic characteristics. Results: Of the 114 surgical cases, 48% were malignant, with the majority of malignant cases diagnosed as follicular variant of papillary thyroid carcinoma (28/55). Risk of malignancy increased with TBSRTC category: 23% for AUS/FLUS, 51% for FN/SFN, and 100% for SM nodules. There were significant differences in surgical approach (p < 0.01), performance of lymph node dissection (p < 0.01), histological diagnosis (p < 0.01), primary tumor focality/laterality (p = 0.04), and lymphatic invasion (p = 0.02) based on TBSRTC classification, with resultant differences in post-surgical risk stratification per American Thyroid Association (ATA) Pediatric Guidelines (p = 0.01). Approximately 89% (49/55) of cases were classified as ATA low risk, and 5 of 6 patients with ATA intermediate- or high-risk disease had SM cytology. Somatic molecular testing was performed in 40% (51/126) of tumors; 77% (27/35) of malignant cases and 38% (6/16) of benign cases harbored driver alteration(s). Of the driver-positive malignant cases, 52% (14/27) were associated with low risk (DICER1, PTEN, RAS, and TSHR mutations), 33% (9/27) were associated with high risk (BRAF mutations and ALK, NTRK, and RET fusions), and 15% (4/27) had unreported risk for invasive disease (APC, BLM, and PPM1D mutations and TG-FGFR1 fusion). Incidence of high-risk drivers increased with TBSRTC category. Approximately 23% (8/35) of patients harboring thyroid malignancy did not have an identifiable driver alteration. Conclusions: Molecular analysis is useful to discriminate benign and malignant thyroid nodules with indeterminate cytology. Patients with driver genetic alteration(s) and indeterminate cytology should consider surgical management secondary to the high incidence (82%; 27/33) of thyroid malignancy in these patients. [ABSTRACT FROM AUTHOR]

Purpose: While interventional radiologists occupy a critical role in adult trauma management, the role of interventionalist in pediatric trauma continues to evolve. The indications for transarterial embolization (TAE) are significantly different in pediatric patients in whom non-operative management (NOM) has a much more prominent role than in adults. Contrast extravasation on imaging may not require acute surgical or interventional management as it would in an adult. There are also areas in which pediatric interventional radiology is increasingly useful such as pelvic TAE in failed management, or splenic embolization to treat bleeding without the loss of splenic function inherent to surgical splenectomy. The rapid evolution of techniques and devices in pediatric patients is also changing what interventions are possible in pediatric trauma management which necessitates frequent reassessment of the guidelines and interventional radiology's role in caring for these patients. Conclusion: This review seeks to consolidate the recent literature to describe the evolving role of the interventional radiologist in pediatric trauma management. [ABSTRACT FROM AUTHOR]

Background: Chest radiographs are commonly obtained after chest tube removal to assess for complications. The benefit of this practice in children is uncertain. Objective: To determine the clinical impact of a routine chest radiograph following removal of chest tubes placed by pediatric interventional radiology. Materials and methods: This single-center retrospective study evaluated 200 chest tube removals in 176 patients (median age: 4 years, interquartile range [IQR]: 1.2–12; median weight: 17.2 kg, IQR: 10.67–37.6), who had a chest tube placed and removed by pediatric interventional radiology over a 16-year period. A chest radiograph obtained on the day of removal was compared to the preceding study. For patients with imaging changes, medical records were reviewed to determine whether clinical actions occurred as a result. All records were reviewed for 7 days after tube removal or hospital discharge, whichever occurred first. Results: The most common indication for chest tube insertion was simple effusion (53%, 106/200) and the most common tube size was 10.2 French (38.7%, 81/209). The median tube dwell time was 8 days (IQR: 5–17). There was a median of 14 h (IQR: 7–33.5) between imaging before and after tube removal. Imaging changes occurred in 10% (n = 20/200) of chest tube removals. Three of 200 (1.5%) of these were symptomatic after removal and only 0.5% (1/200) required chest tube reinsertion. For the remaining removals resulting in chest radiograph changes, patients were asymptomatic and required no change in clinical management. Conclusion: For chest tubes placed by pediatric interventional radiology, these findings do not support the practice of a routine chest radiograph after removal in asymptomatic children. [ABSTRACT FROM AUTHOR]

The addition of contrast US to an existing pediatric US service requires several preparatory steps. This overview provides a guide to simplify the process. Initially, it is important to communicate to all stakeholders the justifications for pediatric contrast US, including (1) its comparable or better diagnostic results relative to other modalities; (2) its reduction in procedural sedation or anesthesia by avoiding MRI or CT; (3) its reduction or elimination of radiation exposure by not having to perform fluoroscopy or CT; (4) the higher safety profile of US contrast agents (UCA) compared to other contrast agents; (5) the improved exam comfort and ease inherent to US, leading to better patient and family experience, including bedside US exams for children who cannot be transported; (6) the need for another diagnostic option in light of increasing demand by parents and providers; and (7) its status as an approved and reimbursable exam. It is necessary to have an UCA incorporated into the pharmacy formulary noting that only SonoVue/Lumason is currently approved for pediatric use. In the United States this UCA is approved for intravenous administration for cardiac and liver imaging and for vesicoureteric reflux detection with intravesical application. In Europe and China it is only approved for the intravesical use in children. All other applications are off-label. The US scanner needs to be equipped with contrast-specific software. The UCA has to be prepared just before the exam and it is important to strictly follow the steps as outlined in the packaging inserts in order to prevent premature destruction of the microbubbles. The initial training in contrast US is best focused on the frontline staff actually performing the US studies; these might be sonographers, pediatric or interventional radiologists, or trainees. It is important from the outset to educate the referring physicians about contrast US. It is helpful to participate in existing contrast US courses, particularly those with hands-on components. [ABSTRACT FROM AUTHOR]

Objective: Puig types 2 through 4 venous malformations (VMs) are challenging to treat with sclerotherapy given their robust systemic outflow. Endovenous balloon occlusion offers a means of temporarily occluding systemic venous outflow to allow for more complete sclerotherapy. This study reviews our experience of implementing this technique in patients with Puig advanced (types 2 through 4) VMs. Methods: An IRB approved review of treated venous malformations from 2013–2016 revealed 10 patients fitting inclusion criteria. Patient demographics, pre-procedural imaging, intra-procedural technical parameters, and post-procedural follow-up outcomes were recorded. All patients underwent temporary balloon occlusion of a systemic or major draining vein during sclerotherapy. Embolic agents included n-butyl cyanoacrylate glue, sodium tetradecyl sulfate foam, and coils. Standard 5 French angioplasty balloons ranged from 4 to 8 mm diameter and 2 to 8 cm length depending on vessel requiring occlusion. All patients underwent minimum 3-year follow-up questionnaire administration re-assessing resolution of lesion symptomology and post-procedural quality of life (QoL) measures. Results: Of the 10 VMs treated, 2 were Type 2, 6 were Type 3, and 2 were Type 4. More than one sclerotherapy session was required in 7/10 patients (mean: 2, range: 1–4). Most common sites of VM systemic drainage included subclavian, popliteal, internal/external jugular, and basilic veins. All patients had no indication for further sclerotherapy following adjunctive balloon occlusion. No non-target embolization or immediate post-procedural complications occurred. Follow-up questionnaires (mean interval: 3 years 6 months, range: 3 years–3 years 11 months) confirmed the persistence of embolization effects, improved QoL, and no additional sclerotherapy sessions for all patients in the cohort. Conclusions: Endovenous balloon occlusion as an adjunct to sclerotherapy can be considered when treating patients with types 2–4 venous malformations. This technique lowers the risk of non-target systemic venous embolization, allowing for operator-driven deeper intralesional sclerosant penetration and subsequently maintained treatment efficacy. [ABSTRACT FROM AUTHOR]

PURPOSE: Many novel therapies for relapsed and refractory neuroblastoma require tumor tissue for genomic sequencing. We analyze our experience with image-guided biopsy in these patients, focusing on safety, yield, adequacy for next-generation sequencing (NGS), and correlation of tumor cell percent (TC%) with quantitative uptake on 123I-meta-iodobenzylguanidine (MIBG) single-photon emission computed tomography with computed tomography (SPECT/CT). MATERIALS AND METHODS: An 11-year retrospective review of image-guided biopsy on 66 patients (30 female), with a median age of 8.7 years (range, 0.9-49 years), who underwent 95 biopsies (55 bone and 40 soft tissue) of relapsed or refractory neuroblastoma lesions was performed. RESULTS: There were seven minor complications (7%) and one major complication (1%). Neuroblastoma was detected in 88% of MIBG- or fluorodeoxyglucose-avid foci. The overall NGS adequacy was 69% (64% in bone and 74% in soft tissue, P =.37). NGS adequacy within neuroblastoma-positive biopsies was 88% (82% bone and 96% soft tissue, P =.11). NGS-adequate biopsies had a greater mean TC% than inadequates (51% v 18%, P =.03). NGS-adequate biopsies had a higher mean number of needle passes (7.5 v 3.4, P =.0002). The mean tissue volume from NGS-adequate soft-tissue lesions was 0.16 cm3 ± 0.12. Lesion:liver and lesion:psoas MIBG uptake ratios correlated with TC% (r = 0.74, r = 0.72, and n = 14). Mean TC% in NGS-adequate samples was 51%, corresponding to a lesion:liver ratio of 2.9 and a lesion:psoas ratio of 9.0. Thirty percent of biopsies showed an actionable ALK mutation or other therapeutically relevant variant. CONCLUSION: Image-guided biopsy for relapsed or refractory neuroblastoma was safe and likely to provide NGS data to guide therapy decisions. A lesion:liver MIBG uptake ratio of ≥ 3 or a lesion:psoas ratio of > 9 was associated with a TC% sufficient to deliver NGS results. [ABSTRACT FROM AUTHOR]

PURPOSE: Many novel therapies for relapsed and refractory neuroblastoma require tumor tissue for genomic sequencing. We analyze our experience with image-guided biopsy in these patients, focusing on safety, yield, adequacy for next-generation sequencing (NGS), and correlation of tumor cell percent (TC%) with quantitative uptake on 123I-meta-iodobenzylguanidine (MIBG) single-photon emission computed tomography with computed tomography (SPECT/CT). MATERIALS AND METHODS: An 11-year retrospective review of image-guided biopsy on 66 patients (30 female), with a median age of 8.7 years (range, 0.9-49 years), who underwent 95 biopsies (55 bone and 40 soft tissue) of relapsed or refractory neuroblastoma lesions was performed. RESULTS: There were seven minor complications (7%) and one major complication (1%). Neuroblastoma was detected in 88% of MIBG- or fluorodeoxyglucose-avid foci. The overall NGS adequacy was 69% (64% in bone and 74% in soft tissue, P =.37). NGS adequacy within neuroblastoma-positive biopsies was 88% (82% bone and 96% soft tissue, P =.11). NGS-adequate biopsies had a greater mean TC% than inadequates (51% v 18%, P =.03). NGS-adequate biopsies had a higher mean number of needle passes (7.5 v 3.4, P =.0002). The mean tissue volume from NGS-adequate soft-tissue lesions was 0.16 cm3 ± 0.12. Lesion:liver and lesion:psoas MIBG uptake ratios correlated with TC% (r = 0.74, r = 0.72, and n = 14). Mean TC% in NGS-adequate samples was 51%, corresponding to a lesion:liver ratio of 2.9 and a lesion:psoas ratio of 9.0. Thirty percent of biopsies showed an actionable ALK mutation or other therapeutically relevant variant. CONCLUSION: Image-guided biopsy for relapsed or refractory neuroblastoma was safe and likely to provide NGS data to guide therapy decisions. A lesion:liver MIBG uptake ratio of ≥ 3 or a lesion:psoas ratio of > 9 was associated with a TC% sufficient to deliver NGS results. [ABSTRACT FROM AUTHOR]

Background: Clotting factor replacement forms the pillar of treatment for children with hemophilia. Most children can be treated using peripheral venipuncture, but very young children and children with poor venous access might require a central venous catheter. Short-term and long-term complications of implantable venous access device placement (also known as port placement) can result in important morbidity and mortality in children with hemophilia.Objective: The purpose of this study is to describe our experience with port placement in children and adolescents with severe hemophilia (<1% of the Factors VIII or IX).Materials and Methods: We performed a retrospective review over a 10-year period to identify port placement in pediatric patients with severe hemophilia. We reviewed demographic and procedural information, access frequency, mechanical complications, and central-line-associated bloodstream infections (CLABSI). Eighteen males were included, with median age at insertion of 3.9 years (0.7-22.7 years). Fifteen of the 18 patients had hemophilia Type A and 3/18 had Type B. Thirteen had high neutralizing inhibitor titers.Results: Technical success in port placement was achieved in 26/27 (96.3%) patients, with 1 port failure caused by venous occlusion from prior catheter placement. Port catheter size ranged from 5 French (Fr) to 7.5 Fr. All were single-lumen and placed via right (76.9%) or left (23.1%) internal jugular vein; 59.3% were placed during general anesthesia, and all had factor replacement prophylaxis. A peripherally inserted central catheter (PICC) was placed concurrently in 69.2% of the cases; per hospital policy, the port was only accessed 15 days post-placement to reduce the risk of site hematoma. Two patients were lost to follow-up. The total catheter days was 15,893. Ports were removed in 14/24 cases, most commonly because of CLABSI (7/24; 29.2%) and transition to peripheral infusion (3/24; 12.5%). Bleeding was the most common complication in the first 30 days after placement. There were nine CLABSI events (0.57 per 1,000 catheter days), all in patients with high neutralizing inhibitor titers. A higher frequency of port access (more or equal to daily vs. less than daily) correlated with higher infection rates (P=0.02). Median time from port insertion to first infection was 348 days (range 167-1,055 days). There were four fibrin-sheath-related catheter occlusions (0.25 per 1,000 catheter days): three catheters were salvaged with intra-catheter tissue plasminogen activator (tPA) instillation resulting in a salvage of an additional 1,214 catheter days, and one catheter was removed after tPA failure (0.06 per 1,000 catheter days).Conclusion: Port maintenance in boys with severe hemophilia is challenging given the need for long-term frequent device access that is associated with catheter-related infections. The rate of bleeding or infection did not differ in patients whether the device was accessed immediately or 15 days post placement. With appropriate pre- and post-procedural factor replacement, immediate and early term severe complications are not common. [ABSTRACT FROM AUTHOR]

Congenital hemangiomas (CHs) are unusual and diverse tumors distinguished from infantile hemangiomas by being largely developed at birth and glucose transporter (GLUT1)‐negative. We describe three infants who presented in utero or at birth with segmentally distributed vascular tumors that were GLUT1‐negative, had histology compatible with congenital hemangioma, and exhibited spontaneous clinical involution. One of the three patients had high‐output cardiac failure and was found to have a mutation in GNAQ (c.626A>c, p.Gln209Pro); another had high‐output cardiac failure, heterotaxy, and transient hematologic abnormalities and was found to have a mutation in GNA11 (c.626_627delinsCC, p.Gln209Pro). In addition to describing a novel segmental pattern of congenital hemangioma variant with genetic correlations, these cases illustrate the utility of targeted genetic testing to elucidate the exact mutation and thus classification of vascular tumors. [ABSTRACT FROM AUTHOR]

Background: Patients who have undergone the Fontan operation for palliation of congenital heart disease with single-ventricle pathophysiology are at high risk for developing progressive liver fibrosis. Pathological assessment from percutaneous liver biopsy is central to the management of Fontan-associated liver disease, but liver biopsy in this vulnerable population poses unique challenges and potential risks.Objective: This retrospective study describes our experience with percutaneous liver biopsy performed to assess changes of Fontan-associated liver disease, with particular regard to procedural outcomes.Materials and Methods: Data from liver biopsy procedure reports, pathology reports, cardiac angiography pressure measurements and laboratory values of patients with single ventricle heart disease after the Fontan operation who underwent ultrasound-guided percutaneous liver biopsy performed in interventional radiology at a pediatric tertiary care center during a 3-year period were retrospectively analyzed.Results: Sixty-eight liver biopsies were performed in 67 patients (mean age: 20.2 years, range: 7.2-39 years). The technical success rate was 100%, and tissue was adequate for assessing liver disease in 100% of the procedures, including biopsies performed with a single pass. Anticoagulation was routinely suspended before biopsy, and no cardiac complications were encountered due to this suspension. A coaxial biopsy system using an 18-gauge (G) full-core instrument through a 17-G introducer trocar was most commonly used, in 41/68 cases (60%). The most common trough length was 2.3 cm, used in 37 cases (54%). One pass was made in 27 procedures (40%) and two passes in 30 (44%); tract embolization with gelatin sponge was performed in 52 (76%). The only complication was hemorrhage, which occurred in 5/68 (7.4%) of the biopsies, minor in four (5.9%) and major in one (1.5%) -- similar to rates reported for liver biopsy in non-Fontan patients. Hemorrhage had a delayed presentation in three of these five cases. Immediate post-biopsy hemoglobin decrease of ≥2 mg/dL showed a low sensitivity for hemorrhage. The mean Fontan pressure measured during cardiac angiography was 13.8 mmHg, and shunt pressures were not associated with an increased risk of hemorrhage.Conclusion: Percutaneous liver biopsy in Fontan patients can be performed safely with high technical success rates and without increased complication rates. Meticulous technique and close observation are recommended to reduce post-biopsy complications. The degree of right heart pressure elevation was not associated with hemorrhage. [ABSTRACT FROM AUTHOR]

Hepatoblastoma and hepatocellular carcinoma (HCC) are the most common pediatric liver malignancies, with hepatoblastoma occurring more commonly in younger children and HCC occurring more commonly in older children and adolescents. Although surgical resection (including transplant when necessary) and systemic chemotherapy have improved overall survival rate for hepatoblastoma to approximately 80% from 30%, a number of children with this tumor type are not eligible for operative treatment. In contradistinction, pediatric HCC continues to carry a dismal prognosis with an overall 5-year survival rate of 30%. The Paediatric Hepatic International Tumour Trial (PHITT) is an international trial aimed at evaluating both existing and emerging oncologic therapies for primary pediatric liver tumors. Interventional radiology offers a number of minimally invasive procedures that aid in diagnosis and therapy of pediatric liver tumors. For diagnosis, the PHITT biopsy guidelines emphasize and recommend percutaneous image-guided tumor biopsy. Additionally, both percutaneous and endovascular procedures provide therapeutic alternatives that have been, to this point, only minimally utilized in the pediatric population. Specifically, percutaneous ablation offers a number of cytotoxic technologies that can potentially eradicate disease or downstage children with unresectable disease. Percutaneous portal vein embolization is an additional minimally invasive procedure that might be useful to induce remnant liver hypertrophy prior to extended liver resection in the setting of a primary liver tumor. PHITT offers an opportunity to collect data from children treated with these emerging therapeutic options across the world. The purpose of this manuscript is to describe the potential role of minimally invasive percutaneous transhepatic procedures, as well as review the existing data largely stemming from the adult HCC experience. [ABSTRACT FROM AUTHOR]

Primary liver malignancies are rare in children. Hepatoblastoma and hepatocellular carcinoma (HCC) together represent the overwhelming majority of cases. Overall survival of hepatoblastoma approaches 80% with multimodal treatment approaches that include chemotherapy, surgery and transplantation. However, there remains a subset of children with hepatoblastoma in whom resection or transplantation is not possible. The 5-year survival for children diagnosed with HCC is less than 30% and remains a significant therapeutic challenge. The poor outcomes for children with primary liver tumors motivate investigation of new therapeutic alternatives. Interventional oncology offers a broad scope of percutaneous and transcatheter endovascular cancer therapies that might provide clinical benefits. Minimally invasive approaches are distinct from medical, surgical and radiation oncologic treatments, and in adults these approaches have been established as the fourth pillar of cancer care. Transarterial chemoembolization is a minimally invasive locoregional treatment option performed by interventional radiologists with level-I evidence as standard of care in adults with advanced liver malignancy; transarterial chemoembolization in adults has served to prolong disease-free progression, downstage and bridge patients for surgical and transplant interventions, and improve overall survival. However, while several groups have reported that transarterial chemoembolization is feasible in children, the published experience is limited primarily to small retrospective case series. The lack of prospective trial evidence has in part limited the utilization of transarterial chemoembolization in the pediatric patient population. The purpose of this article is to provide an overview of the role of interventional radiology in the diagnosis and endovascular management of hepatic malignancies in children. [ABSTRACT FROM AUTHOR]

Magnetic resonance imaging (MRI) often provides better visualization of bone marrow abnormalities than computed tomography (CT) or fluoroscopy, but bone biopsies are usually performed using conventional CT or, more recently, C-arm CT guidance. Biopsies of bone lesions solely visible on MRI are often challenging to localize and require the operator to review the MRI on a separate console to correlate with MRI anatomical landmarks during the biopsy. The MR overlay technique facilitates such biopsies in the angiographic suite by allowing the pre-procedural 3-D MRI to be overlaid on intraprocedural 2-D fluoroscopy. This study describes our initial experience with the MR overlay technique in the angiography suite during pediatric percutaneous extremity bone biopsies of lesions visible on MRI but not on CT or fluoroscopy and demonstrates its utility in relevant clinical cases. [ABSTRACT FROM AUTHOR]

Background: Reduced-dose C-arm computed tomography (CT) uses flat-panel detectors to acquire real-time 3-D images in the interventional radiology suite to assist with anatomical localization and procedure planning.Objective: To describe dose-reduction techniques for C-arm CT at a pediatric institution and to provide guidance for implementation.Materials and Methods: We conducted a 5-year retrospective study on procedures using an institution-specific reduced-dose protocol: 5 or 8 s Dyna Rotation, 248/396 projection images/acquisition and 0.1-0.17 μGy/projection dose at the detector with 0.3/0.6/0.9-mm copper (Cu) filtration. We categorized cases by procedure type and average patient age and calculated C-arm CT and total dose area product (DAP).Results: Two hundred twenty-two C-arm CT-guided procedures were performed with a dose-reduction protocol. The most common procedures were temporomandibular and sacroiliac joint injections (48.6%) and sclerotherapy (34.2%). C-arm CT was utilized in cases of difficult percutaneous access in less common applications such as cecostomy and gastrostomy placement, foreign body retrieval and thoracentesis. C-arm CT accounted for between 9.9% and 80.7% of the total procedural DAP.Conclusion: Dose-reducing techniques can preserve image quality for intervention while reducing radiation exposure to the child. This technology has multiple applications within pediatric interventional radiology and can be considered as an adjunctive imaging tool in a variety of procedures, particularly when percutaneous access is challenging despite routine fluoroscopic or ultrasound guidance. [ABSTRACT FROM AUTHOR]

Background: Navigational software provides real-time fluoroscopic needle guidance for percutaneous procedures in the Interventional Radiology (IR) suite.Objective: We describe our experience with navigational software for pediatric percutaneous bone biopsies in the IR suite and compare technical success, diagnostic accuracy, radiation dose and procedure time with that of CT-guided biopsies.Materials and Methods: Pediatric bone biopsies performed using navigational software (Syngo iGuide, Siemens Healthcare) from 2011 to 2016 were prospectively included and anatomically matched CT-guided bone biopsies from 2008 to 2016 were retrospectively reviewed with institutional review board approval. C-arm CT protocols used for navigational software-assisted cases included institution-developed low-dose (0.1/0.17 μGy/projection), regular-dose (0.36 μGy/projection), or a combination of low-dose/regular-dose protocols. Estimated effective radiation dose and procedure times were compared between software-assisted and CT-guided biopsies.Results: Twenty-six patients (15 male; mean age: 10 years) underwent software-assisted biopsies (15 pelvic, 7 lumbar and 4 lower extremity) and 33 patients (13 male; mean age: 9 years) underwent CT-guided biopsies (22 pelvic, 7 lumbar and 4 lower extremity). Both modality biopsies resulted in a 100% technical success rate. Twenty-five of 26 (96%) software-assisted and 29/33 (88%) CT-guided biopsies were diagnostic. Overall, the effective radiation dose was significantly lower in software-assisted than CT-guided cases (3.0±3.4 vs. 6.6±7.7 mSv, P=0.02). The effective dose difference was most dramatic in software-assisted cases using low-dose C-arm CT (1.2±1.8 vs. 6.6±7.7 mSv, P=0.001) or combined low-dose/regular-dose C-arm CT (1.9±2.4 vs. 6.6±7.7 mSv, P=0.04), whereas effective dose was comparable in software-assisted cases using regular-dose C-arm CT (6.0±3.5 vs. 6.6±7.7 mSv, P=0.7). Mean procedure time was significantly lower for software-assisted cases (91±54 vs. 141±68 min, P=0.005).Conclusion: In our experience, navigational software technology in the IR suite is a promising alternative to CT guidance for pediatric bone biopsies providing comparable technical success and diagnostic accuracy with lower radiation dose and procedure time, in addition to providing real-time fluoroscopic needle guidance. [ABSTRACT FROM AUTHOR]

Background: Inferior vena cava (IVC) filters are commonly implanted with the intent to prevent life-threatening pulmonary embolism in at-risk patients with contraindications to anticoagulation. Various studies have reported increases in the rate of venous thromboembolism within the pediatric population. The utility and safety of IVC filters in children has not yet been fully defined.Objective: To describe the technique and adjunctive maneuvers of IVC filter removal in children, demonstrate its technical success and identify complications.Materials and Methods: A retrospective 10-year review was performed of 20 children (13 male, 7 female), mean age: 15.1 years (range: 12-19 years), who underwent IVC filter retrieval. Eleven of 20 (55%) were placed in our institution. Electronic medical records were reviewed for filter characteristics, retrieval technique, technical success and complications.Results: The technical success rate was 100%. Placement indications included: deep venous thrombosis with a contraindication to anticoagulation (10/20, 50%), free-floating thrombus (4/20, 20%), post-trauma pulmonary embolism prophylaxis (3/20, 15%) and pre-thrombolysis pulmonary patient (1/20, 5%). The mean implantation period was 63 days (range: 20-270 days). Standard retrieval was performed in 17/20 patients (85%). Adjunctive techniques were performed in 3/20 patients (15%) and included the double-snare technique, balloon assistance and endobronchial forceps retrieval. Median procedure time was 60 min (range: 45-240 min). Pre-retrieval cavogram demonstrated filter tilt in 5/20 patients (25%) with a mean angle of 17° (range: 8-40). Pre-retrieval CT demonstrated strut wall penetration and tip embedment in one patient each. There were two procedure-related complications: IVC mural dissection noted on venography in one patient and snare catheter fracture requiring retrieval in one patient. There were no early or late complications.Conclusion: In children, IVC filter retrieval can be performed safely but may be challenging, especially in cases of filter tilt or embedding. Adjunctive techniques may increase filter retrieval rates. [ABSTRACT FROM AUTHOR]

This technical innovation describes our experience using an X-ray magnetic resonance fusion (XMRF) software program to overlay 3-D MR images on real-time fluoroscopic images during sclerotherapy procedures for vascular malformations at a large pediatric institution. Five cases have been selected to illustrate the application and various clinical utilities of XMRF during sclerotherapy procedures as well as the technical limitations of this technique. The cases demonstrate how to use XMRF in the interventional suite to derive additional information to improve therapeutic confidence with regards to the extent of lesion filling and to guide clinical management in terms of intraprocedural interventional measures. [ABSTRACT FROM AUTHOR]

The reported incidence of cerebral embolic or hemorrhagic complications related to mechanical circulatory support in children is high, even while subjects are managed with aggressive antithrombotic therapy. The safety and utility of endovascular treatment for stroke in the pediatric VAD population has not been established in the published literature. We describe a nine-yr-old patient on BiVAD support who experienced threatened AIS on two separate occasions. He was treated successfully via mechanical embolectomy on both occasions and survived to transplantation with minimal neurologic deficits. [ABSTRACT FROM AUTHOR]

Background: Few published series are dedicated solely to CT-guided vertebral bone biopsy in children.Objective: The objective of our study was to review the diagnostic yield of CT-guided vertebral bone biopsy in children.Materials and Methods: A retrospective review of 26 consecutive CT-guided vertebral bone biopsies during a 7-year period in 24 children (16 girls and 8 boys), mean age 8.8 years (range 1-16 years) was performed at our institution. Procedures were reviewed for diagnostic accuracy, biopsy location, complications and final diagnosis.Results: Twenty-five of 26 biopsies (96%) were technically successful. Twenty-one of 26 biopsies were diagnostic (81%); 2/26 (8%) were false-negative removing non-lesional tissue, 2/26 (8%) were nondiagnostic, and 1/26 (4%) was technically unsuccessful. The diagnoses were as follows: 12/26 biopsies (46%) were osteomyelitis, 3/26 (11%) biopsies were Langerhans cell histiocytosis, 3/26 biopsies (11%) were normal bone, 2/26 (8%) biopsies were malignant tumors, and 1/26 (4%) biopsies was osteoblastoma. There was one self-limited, procedure-related complication: a small right-sided pneumothorax occurred in the setting of attempting to create a protective extrapleural biopsy window.Conclusion: Percutaneous CT-guided vertebral bone biopsy can be performed safely in children with a high degree of diagnostic accuracy. [ABSTRACT FROM AUTHOR]

Background: Manufacturers have provided C-arm CT imaging technologies for applications in interventional radiology in recent years. However, clinical imaging protocols and radiation doses have not been well studied or reported. Objective: The purpose of this study is to develop low-dose settings for clinically acceptable CT imaging of temporomandibular joint in interventional radiology suites, using a C-arm imaging angiography system. Materials and methods: CT scans were performed with a flat-panel digital C-arm angiographic system on a 5-year-old anthropomorphic phantom. The CTDI was determined for various rotation times, dose settings and Cu filter selections. The CTDI values were compared with those of conventional low-dose CT for the same phantom. The effectiveness of using Cu filters to reduce dose was also investigated. Images were reviewed by a senior radiologist for clinical acceptance. Results: The manufacturer's default setting gave an equivalent CTDI of 4.8 mGy. Optimizing the dose settings and adding copper filtration reduced the radiation dose by 94%. This represents a 50% reduction from conventional CT. Conclusion: Use of Cu filters and low-dose settings significantly reduced radiation dose from that of standard settings. This phantom study process successfully guided the clinical implementation of low-dose studies for all ages at our institution. [ABSTRACT FROM AUTHOR]

Background: Fibromuscular dysplasia (FMD) and neurofibromatosis type 1 (NF1) are the most common causes of pediatric renal artery stenosis (RAS) in western countries, and characterization of their angiographic features could aid in an accurate diagnosis and in treatment. Objective: This study characterizes renal angiographic findings in pediatric fibromuscular dysplasia (FMD) and neurofibromatosis type 1(NF1). Materials and methods: We reviewed 68 angiograms performed over 11 years on 43 children with renovascular hypertension (20 male, 23 females; ages 1 month to -19 years; median/average 9.8 years). Ten patients were diagnosed with NF1, and 33 had presumed FMD. The frequency, extent and distribution of lesions were determined and analyzed. Results: Stenosis was found in 91% of patients ( n = 39/43), with 86% showing stenosis of 1st or 2nd order arteries, and 12% distal to 2nd order. Stenoses in multiple 1st/2nd order arteries were found in 32% of patients, and 36/43 patients had 1-2 stenoses. The most common lesion was a ≤5 mm stenosis in a 1st/2nd order artery, in 42%. Mean percentage of stenosis in a 1st/2nd order vessel was 62%; ≥70% stenosis was found in 53%, and ≥90% stenosis in 29%. Bilateral disease was noted in 30% of patients. Intraparenchymal disease, distal to 2nd order branches, was seen in 30%. Aneurysms were seen in 28%, beading in 19% and collaterals in 51% (associated with ≥70% stenosis). Mid-aortic narrowing was seen in 16%, more often in patients with NF1. Conclusion: We provide a descriptive characterization of renal angiographic findings in pediatric FMD and NF1. [ABSTRACT FROM AUTHOR]

A 13-month-old boy with Gaucher disease presented with severe thrombocytopenia, anemia and massive splenomegaly. In addition he had significant respiratory compromise caused by abdominal compartment syndrome, requiring mechanical ventilation. Because of the degree of respiratory compromise and his existing bone marrow suppression, splenic artery embolization was chosen as an alternative to splenectomy. Splenic artery embolization was performed using 355–500-µm polyvinyl alcohol particles, with 70% ablation achieved. Within 24 h of the procedure the platelet count had risen to greater than 70,000/mm3 and to more than 170,000/mm3 on postoperative day 4. At the 8-month follow-up the splenic size had decreased from 18 cm to 8 cm, with a platelet count of 578,000/mm3. Partial splenic embolization provides a minimally invasive alternative to splenectomy in patients with Gaucher disease with massive splenomegaly and bone marrow suppression. [ABSTRACT FROM AUTHOR]

Osteoblastoma is a rare benign bone tumor that presents with back pain and occurs in the spine approximately 40% of the time. The time from onset of symptoms to diagnosis is typically several months because it is a rare entity and radiographic studies are often negative early in the course of the disease. These highly vascular and locally aggressive tumors require complete and precise resection. The patient presented is a 15-year-old boy with a 14-month history of right-sided neck and shoulder pain. Computerized tomography and magnetic resonance imaging demonstrated a lesion in the posterior elements of C7 which extended through the pedicle and into the body. Preoperative angiography confirmed a hypervascular lesion which was successfully embolized. He subsequently underwent piecemeal tumor resection and instrumented fusion. Immediate postoperative imaging demonstrated complete resection. At 18 months follow up the patient has maintained resolution of preoperative symptoms and demonstrates evidence of solid fusion on CT. This multidisciplinary approach markedly decreased blood loss and improved visualization to help achieve complete surgical resection and resolution of clinical symptoms. [ABSTRACT FROM AUTHOR]

Osteoblastoma is a rare benign bone tumor that presents with back pain and occurs in the spine approximately 40% of the time. The time from onset of symptoms to diagnosis is typically several months because it is a rare entity and radiographic studies are often negative early in the course of the disease. These highly vascular and locally aggressive tumors require complete and precise resection. The patient presented is a 15-year-old boy with a 14-month history of right-sided neck and shoulder pain. Computerized tomography and magnetic resonance imaging demonstrated a lesion in the posterior elements of C7 which extended through the pedicle and into the body. Preoperative angiography confirmed a hypervascular lesion which was successfully embolized. He subsequently underwent piecemeal tumor resection and instrumented fusion. Immediate postoperative imaging demonstrated complete resection. At 18 months follow up the patient has maintained resolution of preoperative symptoms and demonstrates evidence of solid fusion on CT. This multidisciplinary approach markedly decreased blood loss and improved visualization to help achieve complete surgical resection and resolution of clinical symptoms. [ABSTRACT FROM AUTHOR]

Background: Children with arthritis may endure a lifetime of disfigurement, dysfunction, and pain if acute inflammation progresses to chronic changes in the joint cartilage and underlying bone. Intraarticular steroids have become an integral component of treatment, but at times are difficult to deliver to joints, such as the subtalar joint, that have complex anatomies.Objective: We describe our technique and outcomes using fluoroscopically guided intraarticular subtalar steroid injection in patients with active symptoms of juvenile idiopathic arthritis (JIA).Materials and Methods: Fluoroscopically guided subtalar joint injections were performed in 38 children (mean age 6.7 years). Medical records were reviewed retrospectively and improvement was evaluated clinically by the degree of foot movement in eversion and inversion.Results: Subtalar joint injection was technically successful in 100% of the JIA patients with improvement in physical symptoms in 34/38 (89%). Of the 38 children, 32 were followed up within 13 weeks of the initial injection and, therefore, satisfied the eligibility criteria for resolution of arthritis. Of these 32 children, 14 showed clinical resolution (44%). The mean duration of improvement was 1.2 +/- 0.9 years. Children with a longer interval (>1 year) from diagnosis to treatment had significantly less resolution (P = 0.04). Local subcutaneous atrophy or hypopigmentation were observed in 53% of the children after steroid injection (20/38). These minor complications were associated with a greater volume of steroid injected into the site per child (P = 0.02).Conclusion: Fluoroscopically guided subtalar joint injection is an effective treatment for subtalar arthropathy. Prompt referral for intraarticular steroid treatment in the acute phase improves response. Skin changes often occur at the injection site, and specific precautions should be employed to reduce this risk. Prospective study is indicated to determine the most effective treatment strategy to prevent long-term pain and disability. [ABSTRACT FROM AUTHOR]

Background: Priapism is defined as involuntary, prolonged penile erection caused by factors other than sexual arousal, and is classified as either low-flow or high-flow. Embolotherapy is an accepted form of therapy in adults with high-flow priapism. Because the differences in etiology, management and outcome are significant, accurate and timely diagnosis is imperative.Objective: The purpose of this report is to present our experience with embolotherapy for treatment of high-flow priapism in three children.Patients and Methods: This was a retrospective study. During an 18-month period, three boys ranging in age from 6 to 15 years presented with priapism. All three children were treated with embolotherapy.Results: All three children were successfully treated with angiography and embolotherapy. One boy had a presentation that initially raised the possibility of low-flow priapism. No complications occurred, and to date all children are able to maintain normal erections.Conclusion: Subselective transcatheter embolization is the procedure of choice for high-flow priapism. In cases where priapism persists despite adequate therapy, angiography might be useful to exclude high-flow disease. In children with high-flow priapism, selective occlusion of the penile arteriovenous fistula led to detumescence and normal erectile function. [ABSTRACT FROM AUTHOR]

Background: Paradoxical hyperactivity (PH) is a known complication of sedation in children, especially with barbiturates such as pentobarbital. The accompanying inconsolable irritability and agitation, similar to behaviors reported in children with attention deficit hyperactivity disorder (ADHD), is uncomfortable for the child and anxiety-provoking for parents and health-care workers. Our objective was to describe our experience with oral (PO) and intravenous (IV) caffeine as a treatment for sedation-induced PH.Materials and Methods: From January 2000 to April 2003, 19,894 children were sedated in our institution for radiology procedures. Of these, 360 children were diagnosed with PH. A total of 229 children exhibiting symptoms of PH after sedative administration were treated with PO caffeine ( n=88; 43 boys, 45 girls; mean age 4.5 years, mean weight 18.7 kg) or IV caffeine ( n=131; 73 boys, 58 girls; mean age 4.8 years, mean weight 20.1 kg) or both ( n=10; 8 boys, 2 girls; mean age 5.0 years, mean weight 19.9 kg). A positive effect was defined as a decrease in agitation, crying, or hyperactivity within 40 min of caffeine administration. A control group ( n=45) was obtained from those 141 children who experienced post-sedation PH but were not treated with caffeine, and matched for age and sex with samples of children treated with IV caffeine ( n=45) and PO caffeine ( n=45).Results: Children treated intravenously received the equivalent of 20 mg/kg caffeine citrate (to a maximum of 200 mg). Of those treated with IV caffeine, 82/131 (63%) showed a positive effect, and returned to baseline behavioral status after an average of 33 min (SD=23 min). The untreated control group required a significantly longer time to recover ( P<0.01) than those treated with IV caffeine. Children treated orally received approximately 1.0-2.5 mg/kg caffeine in Mountain Dew (Pepsi-Cola Company), and 36/88 (41%) showed a positive effect and returned to baseline behavioral status after an average of 42 min (SD=27 min). Of the 10 children treated with both PO and IV caffeine, 6 showed a positive effect. There was no significant difference in recovery time between the untreated control group and either the matched orally treated group or the group treated with both IV and PO caffeine. No complications occurred after caffeine administration.Conclusion: IV caffeine appears to be an effective treatment for PH in children with sedation-induced PH. Further controlled prospective study is needed to determine the optimum dose and route of administration and to compare efficacy with other potential drug classes. [ABSTRACT FROM AUTHOR]