Safety of Periprocedural Antithrombotics during Pediatric Transplant Liver Biopsies.

The purpose of this study was to compare the adverse event (AE) rates of percutaneous pediatric transplant liver biopsies in patients receiving periprocedural antithrombotic agents with those in patients not receiving them. A 19-year retrospective single-center study of ultrasound-guided transplant liver biopsies was conducted. Patients who received aspirin for <5 days (n = 51) or heparin <4 hours (n = 15) before biopsy were separately grouped. AEs were reported using the Society of Interventional Radiology classification. In 276 biopsy samples from patients with a mean age of 6.75 years ± 5.80, the overall AE (P = .72) and moderate AE (P = .78) rates for control and antithrombotic groups were not significantly different. No severe AEs or deaths occurred. In conclusion, aspirin continuation during percutaneous pediatric transplant liver biopsies may be safe, but more studies are necessary to confirm the safety of periprocedural heparin.

Alpelisib for the treatment of PIK3CA-related head and neck lymphatic malformations and overgrowth.

PURPOSE: PIK3CA-related overgrowth spectrum (PROS) conditions of the head and neck are treatment challenges. Traditionally, these conditions require multiple invasive interventions, with incomplete malformation removal, disfigurement, and possible dysfunction. Use of the PI3K inhibitor alpelisib, previously shown to be effective in PROS, has not been reported in PIK3CA-associated head and neck lymphatic malformations (HNLMs) or facial infiltrating lipomatosis (FIL). We describe prospective treatment of 5 children with PIK3CA-associated HNLMs or head and neck FIL with alpelisib monotherapy. METHODS: A total of 5 children with PIK3CA-associated HNLMs (n = 4) or FIL (n = 1) received alpelisib monotherapy (aged 2-12 years). Treatment response was determined by parental report, clinical evaluation, diary/questionnaire, and standardized clinical photography, measuring facial volume through 3-dimensional photos and magnetic resonance imaging. RESULTS: All participants had reduction in the size of lesion, and all had improvement or resolution of malformation inflammation/pain/bleeding. Common invasive therapy was avoided (ie, tracheotomy). After 6 or more months of alpelisib therapy, facial volume was reduced (range 1%-20%) and magnetic resonance imaging anomaly volume (range 0%-23%) were reduced, and there was improvement in swallowing, upper airway patency, and speech clarity. CONCLUSION: Individuals with head and neck PROS treated with alpelisib had decreased malformation size and locoregional overgrowth, improved function and symptoms, and fewer invasive procedures.

Time-Driven Cost Analysis of Noncuffed Venous Catheter Placement in Infants: Bedside versus IR Suite.

PURPOSE: To compare the direct bundled costs of interventional radiology (IR) suite versus bedside placement of noncuffed central venous catheters in infants. METHODS: A single-center retrospective review was performed of all noncuffed upper extremity (peripherally inserted central venous catheter [PICC]) and tunneled femoral (tunneled femoral central venous catheter [TCVC]) catheters placed in infants between January 1, 2018, and December 31, 2018. Propensity score matching was performed adjusting for age, birth weight, procedure weight, and catheter days. Process maps for each procedure were created based on location and sedation type. Technical success and complications were recorded for each placement. The total direct bundled cost for each catheter placement was calculated by summing the procedure and complication costs. RESULTS: A total of 142 procedures were performed on 126 matched patients with a technical success of 96% at the bedside and 100% in the IR suite (P = .08). The complication rates did not significantly differ between the 2 groups (P = .51). The total direct bundled costs for catheter placement were $1421.3 ± 2213.2 at the bedside and $2256.8 ± 3264.7 in the IR suite (P = .001). CONCLUSIONS: The bundled cost of bedside femoral catheter placement is significantly less than that of fluoroscopic TCVC and PICC placement performed in the IR suite, mainly related to differences in sedation costs.

Technical Feasibility and Clinical Effectiveness of Transjugular Intrahepatic Portosystemic Shunt Creation in Pediatric and Adolescent Patients.

PURPOSE: To examine the technical feasibility and clinical efficacy of transjugular intrahepatic portosystemic shunt (TIPS) creation in children and adolescents. MATERIALS AND METHODS: Retrospective review was performed of 59 patients (mean age 12.6 y [range, 1.5-20 y], mean weight 47.5 kg [range, 11.4-112.2 kg], mean Model for End-stage Liver Disease/Pediatric End-stage Liver Disease score 12.5 [range, 6-33]) who underwent 61 TIPS attempts at 3 tertiary children's hospitals from 2001 to 2017 for acute esophageal or gastroesophageal variceal bleeding, primary and secondary prevention of variceal bleeding, and refractory ascites. Pediatric liver disease etiologies included biliary atresia, cystic fibrosis, and ductal plate anomalies. Technical, hemodynamic, and clinical success and patency rates were reported at 1, 6, 12, and 24 months. Statistical analysis evaluated reasons for clinical failure. Kaplan-Meier analysis measured clinical success, patency, and transplant-free survival. RESULTS: Technical success was 93.4% (57/61) in 59 consecutive patients. Most common TIPS indications were treating and preventing esophageal and gastroesophageal variceal bleeding (57/59; 96.6%). Hemodynamic success was 94% (47/50). Clinical success was 80.7% (45/56). Two-year clinical success for acute variceal bleeding and ascites was 94.1% and 100%, respectively. Overall patency at 1, 6, 12, and 24 months was 98.0%, 97.8%, 94.3%, and 91.3%. Two-year transplant-free survival was 88.8%. Overall and major complication rates were 21.2% (13/61) and 8.2% (5/61), with 3 mortalities. Gradient reduction < 12 mm Hg correlated with clinical success (P < .01). CONCLUSIONS: TIPS creation in pediatric patients is technically feasible and clinically efficacious for treatment and prevention of esophageal and gastroesophageal variceal hemorrhage. High 2-year clinical success, patency, and survival rates should encourage providers to consider portosystemic shunts as a bridge to liver transplantation.

Hepatic Venous Pressure Gradient Measurements in Children: Correlation With Hepatic Histology and Clinical Indicators of Portal Hypertension.

OBJECTIVES: In adults, elevated hepatic venous pressure gradients (HVPGs) are correlated with the degree of liver fibrosis on histopathology and predict worse outcomes including variceal bleeding and death. We aimed to examine the association between HVPG measurements, histopathologic findings, and clinical indicators of portal hypertension in children. METHODS: Utilizing retrospective data from 2 pediatric centers between 2006 and 2015, we identified children who underwent simultaneous HVPG measurement and transjugular liver biopsy. Medical charts were reviewed for histopathology, imaging, endoscopic, and clinical data. RESULTS: Forty-one children (median age 11 years) were included in the analysis with diagnoses of acute hepatitis (n = 15), chronic liver disease (n = 12), hepatic noncirrhotic portal hypertension (n = 4), acute liver failure (n = 3), and nonhepatic causes of portal hypertension (n = 7). Elevated mean HVPG measurements were found in children with acute liver failure (10 mmHg, range 4-12) and chronic liver disease (7 mmHg, range 1-12). HVPG measurements did not correlate with the histological severity of fibrosis (ρ = 0.23, P = 0.14) or portal inflammation (ρ = 0.24, P = 0.29), and no difference was found in HVPG when comparing children with and without a history of variceal bleeding (P = 0.43). CONCLUSIONS: HVPG measurements do not correlate significantly with the degree of hepatic fibrosis on biopsy. Furthermore, HVPG measurements are not associated with the presence of varices or history of variceal bleeding, suggesting the possibility of intrahepatic shunting in children with advanced liver disease. Therefore, unlike in adults, HVPG measurements may not accurately predict children who are at risk of complications from portal hypertension.

Clinical and Imaging Predictors of Surgical Splenorenal Shunt Dysfunction in Pediatric Patients.

PURPOSE: Few established criteria exist to prompt angiographic evaluation and intervention for surgically created splenorenal shunts (SRS). Clinical and Doppler ultrasound (DUS) imaging predictors of shunt dysfunction were evaluated in this retrospective study. MATERIALS AND METHODS: Consecutive patients undergoing SRS angiography over a 10-year period were retrospectively identified. Preangiography platelet count and DUS measurements of spleen diameter, maximum splenic vein velocity, and maximum shunt velocity were assessed and compared to findings at subsequent catheter angiography. RESULTS: Twenty-six SRS angiograms were performed in 16 patients. Two of the 26 procedures were excluded from analysis due to insufficient baseline preangiography clinical and DUS data. In the remaining 24 cases, significant stenosis/occlusion was confirmed at angiography in 20, whereas wide patency was seen in 4. For the 20 cases of angiographically confirmed significant stenosis/occlusion, when compared to baseline post-SRS creation to immediate preangiography evaluation there was a greater decrease in platelet count (-51.8% vs -19.4%), a greater increase in spleen diameter (+13.4% vs +3.7%), a greater increase in maximum shunt velocity (+74.7% vs +59.7%), and a greater decrease in splenic vein velocity (-25.0% vs -18.5%). CONCLUSION: Clinical evidence of splenic sequestration and DUS finding of increased maximum shunt velocity correlate with angiographic findings of SRS dysfunction and could be used to help predict the need for shunt intervention.

Doppler ultrasound predictors of transplant hepatic venous outflow obstruction in pediatric patients.

OBJECTIVE: To investigate Doppler US and catheter venogram correlates to improve detection of transplant HVOO and avoid unnecessary invasive imaging procedures. MATERIALS AND METHODS: A retrospective review was performed in all pediatric OLT patients undergoing catheter venography of the hepatic veins between 2007 and 2017 at a single large tertiary pediatric liver transplant institution. RESULTS: Forty-four transplant hepatic venograms in 32 OLT patients were included (mean 1.38, range 1-4 venograms per patient). All venograms were preceded by an independent Doppler US examination. Twenty-one (47.7%) venograms were performed for the investigation of suspected HVOO based on Doppler US alone, 19 (43.2%) were performed for TJLB without suspected HVOO, 4 (9.1%) were performed for both. Sixteen (36.3%) instances of >50% anastomotic stenosis were identified. Mean peak anastomotic velocities were 208 cm/s and 116 cm/s in the presence and absence of a >50% venographic stenosis, respectively (P < 0.004). In all cases where there was a monophasic waveform seen on Doppler US, there was a > 50% stenosis seen on hepatic vein venogram. In all cases where a triphasic waveform was seen on Doppler US, there was no stenosis seen on hepatic vein venogram. CONCLUSION: While a Doppler US velocity threshold providing both high sensitivity and specificity has yet to be identified, increasing peak anastomotic velocity and decreasing intrahepatic venous velocity correlate strongly with venographic outflow stenosis. The presence of a triphasic intrahepatic waveform provides good NPV.

Transsplenic splenoportography and portal venous interventions in pediatric patients.

BACKGROUND: Data regarding transsplenic portal venous access for diagnostic imaging and endovascular intervention in children are limited, possibly due to concerns regarding high bleeding risks and resultant underutilization. OBJECTIVE: To investigate the safety and utility of transsplenic splenoportography and portal venous interventions in children. MATERIALS AND METHODS: A retrospective review was performed of all pediatric patients undergoing percutaneous transsplenic portal venous access and intervention at two large tertiary pediatric institutions between January 2012 and April 2017 was performed. Parameters assessed included procedural indications, procedural and relevant prior imaging, technical details of the procedures, laboratory values and clinical follow-up. RESULTS: Transsplenic portal venous access was achieved in all patients. Diagnostic transsplenic splenoportography was performed in 22 patients and was 100% successful at providing the desired anatomical and functional information. Four transsplenic portal venous interventions were performed with 100% success: meso-Rex shunt angioplasty, snare targeted transjugular intrahepatic portosystemic shunt (TIPS) creation through cavernous transformation, pharmacomechanical thrombectomy for acute thrombosis, and transplant portal vein angioplasty. Intraperitoneal bleeding occurred in 2/26 (7.7%) and one case required transfusion (3.8%). No cases of hemorrhage were observed when transsplenic access size was 4 Fr or smaller. CONCLUSION: Transsplenic splenoportography in children is safe and effective when noninvasive imaging methods have yielded incomplete information. Additionally, a transsplenic approach has advantages for complex portal interventions. Bleeding risks are proportional to tract access size and may be mitigated by tract embolization.

Ultrasound-guided lumbar puncture in pediatric patients: technical success and safety.

BACKGROUND: Disadvantages of fluoroscopically guided lumbar puncture include delivery of ionizing radiation and limited resolution of incompletely ossified posterior elements. Ultrasound (US) allows visualization of critical soft tissues and the cerebrospinal fluid (CSF) space without ionizing radiation. OBJECTIVE: To determine the technical success and safety of US-guided lumbar puncture in pediatric patients. MATERIALS AND METHODS: A retrospective review identified all patients referred to interventional radiology for lumbar puncture between June 2010 and June 2017. Patients who underwent lumbar puncture with fluoroscopic guidance alone were excluded. For the remaining procedures, technical success and procedural complications were assessed. Two hundred and one image-guided lumbar punctures in 161 patients were included. Eighty patients (43%) had previously failed landmark-based attempts. RESULTS: One hundred ninety-six (97.5%) patients underwent lumbar puncture. Five procedures (2.5%) were not attempted after US assessment, either due to a paucity of CSF or unsafe window for needle placement. Technical success was achieved in 187 (95.4%) of lumbar punctures attempted with US guidance. One hundred seventy-seven (90.3%) were technically successful with US alone (age range: 2 days-15 years, weight range: 1.9-53.1 kg) and an additional 10 (5.1%) were successful with US-guided thecal access and subsequent fluoroscopic confirmation. Three (1.5%) cases were unsuccessful with US guidance but were subsequently successful with fluoroscopic guidance. Of the 80 previously failed landmark-based lumbar punctures, 77 (96.3%) were successful with US guidance alone. There were no reported complications. CONCLUSION: US guidance is safe and effective for lumbar punctures and has specific advantages over fluoroscopy in pediatric patients.

Transforaminal intrathecal delivery of nusinersen using cone-beam computed tomography for children with spinal muscular atrophy and extensive surgical instrumentation: early results of technical success and safety.

BACKGROUND: Nusinersen, the only treatment approved by the United States Food and Drug Administration for spinal muscular atrophy (SMA), is delivered intrathecally. Many children with SMA have extensive spinal instrumentation and deformities, often precluding the use of standard approaches for gaining intrathecal access. Furthermore the anatomical distortion that often occurs with rotoscoliosis can complicate the use of fluoroscopic guidance. Compared to fluoroscopy, CT affords superior guidance for complex needle placements. This opens up alternatives to the posterior (interlaminar) technique, including transforaminal and caudal approaches. OBJECTIVE: This study describes the early results of technical success, complications and radiation dose of intrathecal delivery of nusinersen using cone-beam CT guidance with two-axis fluoroscopic navigational overlay. MATERIALS AND METHODS: We conducted a retrospective review of 15 consecutive nusinersen injections performed in four children with SMA and extensive spinal hardware precluding standard posterior lumbar puncture techniques. These children were treated using transforaminal thecal access employing cone-beam CT with navigational overlay. We analyzed results including technical success, complications and total fluoroscopy time. RESULTS: All procedures were technically successful. No major complications and one minor complication were reported; the minor complication was a post-procedural neuropathic headache that was attributed to procedural positioning and was treated successfully with gabapentin. The average procedural fluoroscopy time and air kerma were 1.9 min and 55.8 mGy, respectively. CONCLUSION: Cone-beam CT guidance with two-axis navigational overlay is a safe, effective method for gaining transforaminal intrathecal access in children with spinal abnormalities and hardware precluding the use of standard techniques.

Ultrasound-Guided Liver Biopsy With Gelatin Sponge Pledget Tract Embolization in Infants Weighing Less Than 10 kg.

OBJECTIVES: The aim of the study was to describe and assess the technical success and safety of ultrasound-guided liver biopsy with gelatin sponge pledget tract embolization technique in infants <10 kg across 3 tertiary pediatric hospitals. MATERIALS AND METHODS: There were 67 pediatric patients weighing <10 kg (36 boys; 31 girls; average age 202 days; average weight 6 kg, range 1.5-9.9 kg) referred for liver biopsy performed with ultrasound guidance and gelatin sponge pledget tract embolization during a 2-year period. Patient history, procedural records, and clinical follow-up documents were retrospectively reviewed. RESULTS: A total of 67 procedures were included. There was 100% technical success rate and all samples obtained provided adequate tissue for histological assessment. Average number of 18 G biopsy passes was 3 (range 1-6). There were no procedure-related deaths. There was 1 complication (1%) in a 5-kg infant who was readmitted 36 hours after biopsy with a fever and fully recovered after antibiotics were administered. Biliary atresia was the most common underlying diagnosis (20%), whereas others included acute rejection (16%) and biliary obstruction (7%). CONCLUSIONS: Ultrasound-guided percutaneous liver biopsy with gelatin sponge pledget tract embolization technique in children weighing <10 kg is safe, effective, and use of this technique may lead to a reduction in rates of adverse events reported in other pediatric series.

Ultrasound-guided musculoskeletal interventions in American football: 18 years of experience.

OBJECTIVE: Myotendinous strains, contusions, and hematomas are common injuries in American football. Along with ligament sprains and inflammatory disorders, musculoskeletal injuries often result in lost participation time. This article summarizes 18 years of experience with 128 ultrasound-guided drainages and injections in 69 football players with 88 injuries. CONCLUSION: When performed by an operator with sufficient expertise in diagnostic and procedural skills, ultrasound-guided musculoskeletal interventions are minimally invasive, are safe, and can play an integral role in injury management.

Percutaneous Image-Guided Treatment of Pediatric Deep-Vein Occlusions.

Pediatric venous occlusions are a growing cause of morbidity and mortality, especially in hospitalized patients. Catheter-directed recanalization is a safe and effective treatment option in appropriately selected patients. Benefits of catheter directed therapies (CDTs) include the prevention of pulmonary embolism and end organ failure acutely as well as superior vena cava syndrome and post-thrombotic syndrome chronically. Timely diagnosis, recognition of underlying factors for thrombosis, and familiarity with the spectrum of tools and techniques for CDT are essential to optimizing outcomes in the acute setting. Recanalization of chronic venous occlusions can similarly provide symptomatic relief and achieve long term vessel patency. This review will detail the scope, techniques, and outcomes for CDT in the treatment of acquired systemic deep vein occlusions.

Time-Driven Activity-Based Cost Comparison of Osteoid Osteoma Ablation Techniques.

OBJECTIVE: Compare the cost of performing an osteoid osteoma ablation using cone beam CT (CBCT) with overlay fluoroscopic guidance to ablation using conventional CT (CCT) guidance and microwave ablation (MWA) to radiofrequency ablation (RFA). METHODS: An 11-year retrospective study was performed of all patients undergoing osteoid osteoma ablation. Ablation equipment included a Cool tip RFA probe (Covidien, Minneapolis, Minnesota) or a Neuwave PR MWA probe (Ethicon, Rariton, New Jersey). The room times as well as immediate recovery time were recorded for each case. Cost analysis was then performed using time-driven activity-based costing for rate-dependent variables including salaries, equipment depreciation, room time, and certain supplies. Time-independent costs included the disposable interventional radiology supplies and ablation systems. Costs were reported for each service providing care and using conventional cost accounting methods with variable and fixed expenditures. RESULTS: A total of 91 patients underwent 96 ablation procedures in either CBCT (n = 66) or CCT (n = 30) using either MWA (n = 51) or RFA (n = 45). The anesthesia induction (22.7 ± 8.7 min versus 15.9 ± 7.2 min, P < .001), procedure (64.7 ± 27.5 min versus 47.3 ± 15.3 min; P = .001), and room times (137.7 ± 33.7 min versus 103.9 ± 22.6. min; P < .001) were significantly longer for CBCT procedures. The procedure time did not differ significantly between MWA and RFA (62.1 ± 27.4 min versus 56.1 ± 23.3 min; P = .27). Multiple regression analysis demonstrated lower age (P = .046), CBCT use (P = .001), RFA use (P = .02), and nonsupine patient position (P = .01) significantly increased the total procedural cost. After controlling for these variables, the total cost of CBCT ($5,981.32 ± $523.93 versus $5,378.93 ± $453.12; P = .001) remained higher than CCT and the total cost of RFA ($5,981.32 ± $523.93 versus $5,674.43 ± $549.14; P = .05) approached a higher cost than MWA. CONCLUSION: The use of CBCT with overlay fluoroscopic guidance for osteoid osteoma ablation resulted in longer in-room times and greater cost when compared with CCT. These cost considerations should be weighed against potential radiation dose advantage of CBCT when choosing an image guidance modality. Younger age, RFA use, and nonsupine patient position additionally contributed to higher costs.

Safety and efficacy of drug-eluting bead chemoembolization for hepatocellular carcinoma: comparison of small-versus medium-size particles.

PURPOSE: To compare safety and imaging response with 100-300 µm and 300-500 µm doxorubicin drug-eluting bead (DEBs) to determine optimal particle size for chemoembolization of hepatocellular carcinoma (HCC). MATERIALS AND METHODS: DEB chemoembolization using 100-300 µm (n = 39) or 300-500 µm (n = 22) LC beads loaded with 50 mg of doxorubicin was performed in 61 patients with HCC. Patient age, sex, etiology of liver disease, degree of underlying liver disease, tumor burden, and performance status were similar between the groups. All treatments were performed in a single session and represented the patient's first treatment. Toxicities and imaging response in a single index tumor were analyzed using World Health Organization (WHO) and European Association for the Study of the Liver (EASL) criteria. RESULTS: There was a significantly lower incidence of postembolization syndrome and fatigue after treatment in the 100-300 µm group (8% and 36%) versus the 300-500 µm group (40% and 70%) (100-300 µm group, P = .011; 300-500 µm group, P = .025). Mean change in tumor size was similar between the two groups based on WHO and EASL criteria and similar rates of objective response, but there was a trend toward a higher incidence of EASL complete response with 100-300 µm beads versus 300-500 µm beads (59% vs 36%; P = .114). CONCLUSIONS: In DEB chemoembolization for treatment of HCC, 100-300 µm doxorubicin DEBs are favored over 300-500 µm doxorubicin DEBs because of lower rates of toxicity after treatment and a trend toward more complete imaging response at initial follow-up.

Delaying Invasive Treatment in Unilateral Head and Neck Lymphatic Malformation Improves Outcomes.

OBJECTIVES: Large (De Serres stage [IV-V]) head and neck lymphatic malformations (HNLMs) often have multiple, high-risk, invasive treatments (ITs) to address functional compromise. Logically reducing HNLM ITs should reduce treatment risk. We tested whether delaying HNLM ITs reduces total IT number. MATERIALS: Consecutive HNLM patients (n = 199) between 2010 and 2017, aged 0-18 years. METHODS: ITs (surgery or sclerotherapy) were offered for persistent or dysfunction causing HNLMs. Treatment effectiveness categorized by IT number: optimal (0-1), acceptable (2-5), or suboptimal (>5). Clinical data were summarized, and outcome associations tested (χ2 ). Relative risk (RR) with a Poisson working model tested whether HNLM observation or IT delay (>6 months post-diagnosis) predicts treatment success (i.e., ≤1 IT). RESULTS: Median age at HNLM diagnosis was 1.3 months (interquartile range [IQR] 0-45 m) with 107/199(54%) male. HNLM were stage I-III (174 [88%]), IV-V (25 [13%]). Initial treatment was observation (70 [35%]), invasive (129 [65%]). Treatment outcomes were optimal (137 [69%]), acceptable (36 [18%]), and suboptimal (26 [13%]). Suboptimal outcome associations: EXIT procedure, stage IV-V, oral location, and tracheotomy (p < 0.001). Stage I-III HNLMs were initially observed compared with stage I-III having ITs within 6 months of HNLM diagnosis, had a 82% lower relative treatment failure risk ([i.e., >1 IT], RR = 0.09, 95% CI 0.02-0.36, p < 0.001). Stage I-III HNLMs with non-delayed ITs had reduced treatment failure risk compared with IV-V (RR = 0.47, 95% CI 0.33-0.66, p < 0.001). CONCLUSION: Observation and delayed IT in stage I-III HNLM ("Grade 1") is safe and reduces IT (i.e., ≤1 IT). Stage IV-V HNLMs ("Grade 2") with early IT have a greater risk of multiple ITs. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:956-962, 2023.

American society of pediatric otolaryngology vascular anomalies task force review of tongue venous malformations: Techniques, pearls, and pitfalls.

Management of tongue venous malformations can be challenging in the pediatric population due to their heterogeneity in presentation, extent of involvement and functional compromise. It is important to recognize the value of various treatment options in order to guide management of each patient in an individualized fashion. Here we describe a series of patients with tongue venous malformations that are managed using diverse modalities to illustrate the relative benefits and risks of each technique. The challenges of venous malformation treatment can be mitigated by tailoring the approach to each individual patient and malformation. This case series also emphasizes the need and importance of working in the setting of a multidisciplinary vascular anomalies team.

Midterm follow-up of transjugular intrahepatic portosystemic shunts using polytetrafluoroethylene endografts in children.

PURPOSE: To report the initial experience of a pediatric center's use of expanded polytetrafluoroethylene (ePTFE) endografts for the creation of transjugular intrahepatic portosystemic shunts (TIPSs) in children. MATERIALS AND METHODS: Twelve consecutive patients had a TIPS created, all with ePTFE endografts. Nine were children younger than 18 years of age: one infant (age 18 mo) and eight adolescent children with a median age of 14 years, 3 months (range, 10 y, 1 mo, to 17 y, 2 mo). All had gastric or esophageal varices. Acute variceal bleeding was the primary indication in four of nine cases (44%). Mean follow-up was 20 months (range, 4 d to 32 mo). RESULTS: Technical success rate was 100%. Seven TIPSs (78%) were created with a 10-mm-diameter endograft and two (22%) were created with an 8-mm-diameter endograft. Mean portosystemic gradient was reduced from 15.9 mm Hg to 5.8 mm Hg after TIPS creation. The devices were not dilated to their nominal diameter in seven of nine cases (78%). There were no major complications or mortalities associated with TIPS creation. There was one minor complication during follow-up in which a child developed progressively increasing lethargy. Primary patency rate by ultrasound during follow-up was 100%. There were no shunt dysfunctions or repeat interventions required, and no clinical recurrences. One child received a liver transplant 4 days after TIPS creation. CONCLUSIONS: TIPSs can be created in children with the use of ePTFE endografts with an excellent midterm primary patency rate and a low complication rate.

Cholangiocarcinoma: molecular imaging-guided radiofrequency hyperthermia-enhanced intratumoral herpes simplex virus thymidine kinase gene therapy.

We investigated the feasibility of using radiofrequency hyperthermia (RFH) to enhance green fluorescent protein (GFP)/herpes simplex virus thymidine kinase (HSV-TK)/ganciclovir (GCV) gene therapy of cholangiocarcinoma. Cholangiocarcinoma cells and mice with cholangiocarcinoma were treated by (i) GFP/HSV-TK/plasmid combined with RFH at 42°C, followed by ganciclovir administration; (ii) HSV-TK alone; (iii) RFH alone; and (iv) saline. The therapeutic effects among different treatments were evaluated by bioluminescent optical imaging and ultrasound imaging. For the technical validation, GFP/HSV-TK/plasmid was intrabiliarily injected into pig common bile duct (CBD) walls using a needle-integrated balloon catheter with or without RFH enhancement. GFP gene expression was evaluated by optical imaging, which was correlated with histology. The results show that combination therapy of HSV-TK plus RFH significantly induced lower cell viabilities and decreased bioluminescence signals compared the other three groups, which were further confirmed by the tumor volume decrease with combination therapy, as measured by ultrasound imaging. Optical imaging of CBD tissues demonstrated an increased GFP expression in the group with RFH enhancement, compared that with non-RFH treatment. We concluded that intratumoral RFH can enhance the therapeutic effect of GFP/HSV-TK/plasmid on cholangiocarcinoma, which may open new avenues for effective treatment of this deadly disease.

Emergent stent-graft repair of a massive aortic pseudoaneurysm secondary to Behçet's disease in a child.

An 11-year-old male with vasculitis was found to have a large abdominal aortic pseudoaneurysm on diagnostic angiography. This report describes endovascular repair of the pseudoaneurysm by stent-graft exclusion. The existing literature surrounding this rare and potentially fatal condition is also reviewed.