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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]

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]

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]

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]