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To evaluate technical success and the incidences of, and risk factors for, mechanical and infectious complications of venous port placement in infants.This was a retrospective single-institution cohort study of port placement in infants (age1 y) from January 2006 through June 2016 (mean age, 7.5 mo ± 3.3; mean weight, 8.1 kg ± 1.9). Age, weight, sex, side of placement, tip position, and indication for placement (chemotherapy vs other) were recorded. Total catheter-days (CDs), mechanical complications, and central catheter-associated bloodstream infections (CCABSIs) were identified.During the study years, 64 ports were placed in 64 infants, with a technical success rate of 100%. The mean catheter life was 321 days (total range, 4-1,917 d; interquartile range [IQR], 107-421 d). There were 13 CCABSI events (0.63 per 1,000 CDs); of these, 8 (12.5% among 64 patients) required port removal for infection. There was an increase in CCABSIs in patients with left-sided port placement (relative risk [RR], 3.22; 95% confidence interval [CI], 1.02-10.14; P = .05). There were 8 mechanical complications of the port reservoir or catheter (0.39 per 1,000 CDs). Of these, 2 (3.1%) required removal. Patients in the lowest weight quartile were at an increased risk of mechanical complications (RR, 4.37; 95% CI, 1.09-17.48; P = .04).Venous ports can be placed with a high rate of technical success in infants. Left-sided ports and low weight are associated with increased infectious and mechanical complications, respectively.

The purpose of this study was to assess the safety and indications for cardiac magnetic resonance (CMR) with myocardial perfusion imaging (MPI) in a cohort of children and young adults. A retrospective review of 178 children and young adults who underwent CMR with MPI was performed. Studies were categorized based on study protocols as MPI with resting perfusion only, adenosine stress MPI, exercise-induced stress MPI, and MPI for cardiac mass diagnosis. Relevant clinical history, exam indications, and adverse reactions following gadolinium-based contrast agent and adenosine administration were recorded. Studies were reviewed for the presence of myocardial perfusion defects, wall motion abnormalities, and delayed myocardial enhancement. The most common indications from MPI were congenital heart disease (CHD), Kawasaki disease, anomalous coronary artery, or myocardial mass characterization. Of these, 51% were protocoled with adenosine stress, 23% without stress, 6% with exercise stress, and 20% for cardiac mass evaluation. Excluding patients for myocardial mass evaluation, MPI defects were present in 16% (14 with adenosine stress, 1 with exercise stress, 8 on resting studies only). For cardiac mass evaluation, a mass was confirmed in 58%. No adverse reactions occurred with intravenous administration of a gadolinium-based contrast agent. Three self-limited adverse reactions, 2 patients with chest pain, and 1 patient with bradycardia, occurred following adenosine administration. MPI is a safe modality for the evaluation of pediatric and young adults with minimal adverse events. The most common indications for MPI were for the evaluation of CHD, Kawasaki disease, anomalous coronary artery, or myocardial mass characterization.

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. To describe dose-reduction techniques for C-arm CT at a pediatric institution and to provide guidance for implementation. 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). 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. 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.

The appropriate imaging for pediatric patients being evaluated for suspected physical abuse depends on the age of the child, the presence of neurologic signs and symptoms, evidence of thoracic or abdominopelvic injuries, and whether the injuries are discrepant with the clinical history. The clinical presentations reviewed consider these factors and provide evidence-based consensus recommendations by the ACR Appropriateness Criteria ® Expert Panel on Pediatric Imaging.

A non-contractile transannular patch (TAP) in the right ventricular outflow tract (RVOT) contributes to ventricular dysfunction after tetralogy of Fallot (TOF) repair. We compared regional right ventricular (RV) strain in repaired TOF with valvar pulmonary stenosis (VPS) after balloon valvuloplasty to investigate the effects of TAP. Retrospective review of 26 cardiac magnetic resonance studies of TOF (n = 13) and VPS (n = 13) subjects matched by degree and duration of pulmonary regurgitation (PR). Feature tracking strain analysis was performed. Student's t tests, Pearson correlation, and linear regression were applied. RV ejection fraction (EF) was normal and similar between TOF and VPS (60 and 65%, respectively, p = 0.8). RV 4-chamber Lagrangian longitudinal strain (RV 4ch LS) was worse in both groups compared to normals but comparable to each other: -18.2 (95% CI -3.6 to -33) for TOF and -20.2 (95% CI -12.4 to -28) for VPS, p = 0.5. RVOT LS was worse than RV 4ch LS in TOF, p = 0.05, but not in VPS, p = 0.19. There were no significant differences in RVOT strain between groups, p = 0.18. RVOT strain and RV 4ch LS correlated positively with RV EF in VPS (r = 0.72, p = 0.003 and r = 0.55, p = 0.04). PR degree correlated negatively with RVOT LS for TOF and VPS. Longitudinal strain is diminished in VPS and TOF subjects with preserved RV EF. TAP could explain worse RVOT strain in TOF. Longitudinal studies are needed to ascertain if RV strain predicts worsening of RV EF.

CT angiography is gaining broader acceptance in the evaluation of children with known or suspected congenital heart disease. These studies include non-cardiovascular structures such as the mediastinum, lung parenchyma and upper abdominal organs. It is important to inspect all these structures for potential abnormalities that might be clinically important and, in some cases, may impact care plans. To determine the prevalence of non-cardiovascular findings in CT angiography of children with congenital heart disease. During 28 months, 300 consecutive children (170 males; mean age: 7.1 years, age range: 6 h-26 years), referred from a tertiary pediatric cardiology center, underwent clinically indicated CT angiography to evaluate known or suspected congenital heart disease. Slightly more than half (n = 169) of the patients were postoperative or post-intervention. Examinations were retrospectively reviewed, and non-cardiovascular findings were recorded and tabulated by organ system, congenital heart disease and operative procedure in conjunction with outcomes from medical charts. Non-cardiovascular findings were identified in 83% (n = 250 / 300) of the studies for a total of 857 findings. In 221 patients (n = 73.7% of 300) a total of 813 non-cardiovascular findings were clinically significant, while in 9.7% (n = 29 / 300) of patients, 5.1% (n = 44 / 857) of the findings were nonsignificant. In 38.3% (n = 115 / 300) of patients with significant non-cardiovascular pathology, the findings were unexpected and directly impacted patient care plans. Commonly involved organs with non-cardiovascular findings were the lungs with 280 non-cardiovascular findings in 176 / 300 (58.7%) of patients, the airway with 139 non-cardiovascular findings in 103 / 300 (34.3%) of patients and the liver with 108 non-cardiovascular findings in 72 / 300 (24.0%) of patients. Syndromic associations were noted in 22% (n = 66 / 300) of the patients. Non-cardiovascular findings are common in children with congenital heart disease who undergo CT angiography. Based upon our study population, if a child with congenital heart disease has a CT angiography, five out of six will have non-cardiovascular findings, while nearly three out of four (73.7%; 221 / 300) will have significant non-cardiovascular findings. Close attention to the non-cardiovascular structures in children with congenital heart disease presenting for a CT angiography is recommended as in nearly 40% of these children, findings were unexpected and directly altered patient care.

There is an established association between tetralogy of Fallot and partial anomalous pulmonary venous connections. This association is important because surgically repaired tetralogy patients have increased risk of right heart failure. We hypothesize that partial anomalous venous connections increase right ventricular volumes and worsen right ventricular failure.We reviewed cardiac MRI exams performed at a tertiary pediatric hospital from January 2005 to January 2014. We identified patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection. We used age- and gender-matched repaired tetralogy patients without partial anomalous pulmonary venous connection as controls. We analyzed the MRI results and surgical course and performed comparative statistics to identify group differences.There were eight patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection and 16 controls. In all cases, the partial anomalous pulmonary venous connection was not detected on preoperative echocardiography. There were no significant differences in surgical course and body surface area between the two groups. Repaired tetralogy patients with unrepaired partial anomalous pulmonary venous connection showed significantly higher indexed right ventricular end diastolic volume (149 ± 33 mL/m(2) vs. 118 ± 30 mL/m(2)), right ventricle to left ventricle size ratios (3.1 ± 1.3 vs. 1.9 ± 0.5) and a higher incidence of reduced right ventricular ejection fraction compared to controls (3/8 vs. 0/16).Repaired tetralogy of Fallot with unrepaired partial anomalous pulmonary venous connection is associated with reduced right ventricular ejection fraction and more significant right ventricular dilation.

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.

Pulse-wave velocity (PWV), a measure of arterial stiffness, is a known independent risk factor for cardiovascular events. Patients with single ventricle who undergo aortic to pulmonary anastomosis (recon) have noncompliant patch material inserted into the neoaorta, possibly increasing vessel stiffness and afterload. The purpose of this study is to determine if PWV in patients with single ventricle differed between those who did and those who did not undergo aortic reconstruction (nonrecon). We retrospectively reviewed cardiac magnetic resonance anatomic, cine, and phase contrast evaluations in the ascending aorta and descending aorta (DAo) at the level of the diaphragm data from 126 patients with single ventricle (8.6 ± 8.0 years) from January 2012 to May 2013. Significance = p0.05. Seventy-five patients underwent recon and 51 did not. PWV in recon was significantly higher than in nonrecon (3.9 ± 0.9 m/s vs 3.2 ± 1.0 m/s, p = 0.008); in recon, patients13 years old had a higher PWV than those7 years (4.5 ± 0.6 vs 3.5 ± 0.7 m/s, p = 0.004). Whether7 or13 years old, PWV of those with recon was higher than nonrecon DAo distensibility was similar between both groups. There was no difference in age, body surface area, or cardiac index between recon and nonrecon. No correlations between various hemodynamic and ventricular function parameters with PWV were noted. In conclusion, PWV in recon is higher than in nonrecon with similar DAo distensibility implicating the aortic reconstruction as a possible cause of increased afterload; older recon patients have stiffer aortas than younger ones, possibly imposing an additional cardiovascular risk in the future. Other biomaterials may potentially moderate PWV if clinical outcome is adversely affected.

Plastic bronchitis is a rare and often fatal complication of single-ventricle surgical palliation after total cavopulmonary connection. Although lymphatic abnormalities have been postulated to play a role in the disease process, the etiology and pathophysiology of this complication remain incompletely understood. Here we report on the etiology of plastic bronchitis in a child with total cavopulmonary connection as demonstrated by magnetic resonance (MR) lymphangiography. We also report on a new treatment of this disease. The patient underwent noncontrast T2-weighted MR lymphatic mapping and dynamic contrast MR lymphangiography with bi-inguinal intranodal contrast injection to determine the anatomy and flow pattern of lymph in his central lymphatic system. The MRI scan demonstrated the presence of a dilated right-sided peribronchial lymphatic network supplied by retrograde lymphatic flow through a large collateral lymphatic vessel originating from the thoracic duct. After careful analysis of the MRI scans we performed selective lymphatic embolization of the pathologic lymphatic network and supplying vessel. This provided resolution of plastic bronchitis for this patient. Five months after the procedure, the patient remains asymptomatic off respiratory medications.

To evaluate an alternative route of pelvic abscess drainage in children via a transiliopsoas approach. Appendiceal perforations complicated by inflammatory masses, such as abscesses or phlegmon, are a common indication for abdominal drainage in pediatric interventional radiology. Certain locations of collections may present particular challenges for the operator, owing to numerous surrounding structures, including bowel and other pelvic anatomy. This series describes an alternative route for drainage, which involves traversing the iliopsoas muscle under US guidance to drain a deep pelvic abscess. Retrospective chart review was performed of the transiliopsoas approach for abscess drainage during a 5-year period. The technique, pre- and post-drainage imaging, aspiration/drain output, duration of catheter dwell, procedure-related complications and abscess recurrence were reviewed. Transiliopsoas needle placement was successful in 14 of 14 patients (100%). Catheter placement was successful in 13 patients. Abscess wall rupture precluded catheter placement in one patient. Mean catheter duration was 4.9 days, with a range of 2 to 9 days. Clinical improvement was achieved in all 14 patients (100%). There were no major complications. Post-procedure pain with ambulation was reported in 3 of 14 patients (21.4%), which was successfully controlled in each case with medication, and resolved after 2 days. The transiliopsoas route is a safe and effective route for US-guided abscess drainage and catheter placement in children with deep pelvic collections. In selected cases, this approach provides a more easily accessible and safer route than more traditional interventional approaches.

Purpose To describe experience with the use of ultrasound (US)–guided placement and tip position confirmation for direct saphenous and single-incision tunneled femoral noncuffed central venous catheters (CVCs) placed in neonates and infants at the bedside. Materials and Methods A retrospective review of the interventional radiology (IR) database and electronic medical records was performed for 68 neonates and infants who received a CVC at the bedside and for 70 age- and weight-matched patients with CVCs placed in the IR suite between 2007 and 2012. Technical success, complications, and outcomes of CVCs placed at the bedside were compared with those in an age- and weight-matched sample of children with CVCs placed in the IR suite. Results A total of 150 primary insertions were performed, with a technical success rate of 100%. Total catheter lives for CVCs placed at the bedside and in the IR suite were 2,030 catheter-days (mean, 27.1 d) and 2,043 catheter-days (mean, 27.2 d), respectively. No significant difference was appreciated between intraprocedural complications, mechanical complications (bedside, 1.53 per 100 catheter-days; IR, 1.76 per 100 catheter-days), or infectious complications (bedside, 0.39 per 100 catheter-days; IR, 0.34 per 100 catheter-days) between groups. Conclusions US-guided placement and tip position confirmation of lower-extremity CVCs at bedside for critically ill neonates and infants is a safe and feasible method for central venous access, with similar complications and catheter outcomes in comparison with CVCs placed by using fluoroscopic guidance in the IR suite.

Object The use of cerebral intraoperative angiography has been described in resection of arteriovenous malformations (AVMs) in adults. More recently, studies have described experiences with intraoperative angiography in a small number of children. However, data on the safety and clinical utility of intraoperative angiography in the pediatric population remains limited in comparison with available data in adults. The aim of the study was to evaluate the use of cerebral intraoperative angiography in children undergoing AVM resection. The clinical utility of intraoperative angiography and procedure-related complications were evaluated. Methods A retrospective review was performed for all patients undergoing cerebral AVM resection with intraoperative angiography at The Children's Hospital of Philadelphia between 2008 and 2012. Patient imaging and operative and medical notes were reviewed to evaluate for end points of the study. A total of 17 patients (8 males, 9 females) were identified, with a median age of 12.1 years (range 1.2–17.9 years) and median weight of 45.5 kg (range 12.1–78.9 kg). Results A total of 21 intraoperative angiography procedures were performed for 18 AVM resections in 17 patients. The technical success rate was 94%. In 2 cases (11%), intraoperative angiography demonstrated a residual AVM, and repeat resections were performed. In both cases, no recurrent disease was noted on postoperative follow-up. One procedure-related complication (4.8%) occurred in 1 patient who was positioned prone. Recurrence to date was noted in 2 (14%) of the 14 cases with available postoperative follow-up at 3.5 and 4.7 months following resection with intraoperative angiography. The median follow-up time from intraoperative angiography to the most recent postoperative angiography was 1.1 years (range 4.3 months to 3.8 years). Conclusions Intraoperative angiography is an effective and safe adjunct for surgical management of cerebral AVMs in the pediatric population.

Navigational software provides real-time fluoroscopic needle guidance for percutaneous procedures in the Interventional Radiology (IR) suite. 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. 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. 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). 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.

Few published series are dedicated solely to CT-guided vertebral bone biopsy in children.The objective of our study was to review the diagnostic yield of CT-guided vertebral bone biopsy in children.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.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.Percutaneous CT-guided vertebral bone biopsy can be performed safely in children with a high degree of diagnostic accuracy.

Objectives This study investigated whether cardiac magnetic resonance (CMR) and echocardiography (echo) can replace catheterization (cath) for routine evaluation prior to Fontan and under what circumstances CMR and cath are used together. Background Routine cath prior to Fontan has been utilized for years; noninvasive methods, however, may be sufficient. Methods This study reviews clinical data in 119 consecutive patients investigating 3 groups: those who underwent CMR alone (MR; n = 41), cath alone (C; n = 41), or both cath and CMR (C+M; n = 37) prior to Fontan. Results No clinically significant differences were noted in patient characteristics, hemodynamics, or clinical status prior to or after surgery between the C and MR groups. CMR added information in 82%. There were no discrepant findings between CMR and cath data in the C+M group. Diagnostic success was ≥95% in all groups. Of those undergoing Fontan completion, the C+M group had similar outcomes to C and MR; C and CMR were utilized in combination to assess aortopulmonary collaterals or the need for an intervention or evaluate its success. Echo could not delineate pulmonary arterial anatomy in 46% to 53% of patients. The C+M and C groups were exposed to 6.8 ± 4.1 mSv of radiation. Conclusions Single ventricle patients not requiring an intervention can undergo successful Fontan completion with CMR and echo alone with similar short-term outcomes to C, which was used as a control, preventing an invasive test and exposure to radiation. CMR can add information in a significant number of patients. Cath and CMR are utilized together for interventions and assessment of aortopulmonary collaterals.

Purpose To report the outcome of tunneled dialysis catheter insertion in 120 patients. Materials and Methods A retrospective review of the interventional radiology database and electronic medical records of 120 patients who had tunneled dialysis catheters inserted from April 1997 to July 2010 was performed with institutional review board approval. There were 61 female patients and 59 male patients, with a mean age of 13.3 years (range, 0.2–28.5 y). A total of 193 primary insertions and 330 salvage procedures were performed. Results The technical success rate for primary catheter insertions was 100%. Immediate complications included self-limiting tract bleeding and air embolism in two of 193 insertions each (1.03%). Mean indwell duration for primary insertions was 66 catheter-days (range, 1–765 d), compared with a total mean of 159.4 catheter-days (range, 1–1,034 d). Rates of infection and mechanical complications were 0.21 and 0.9 per 100 total catheter-days, respectively. Mechanical and infections complications were increased in children younger than 9 years of age and weighing less than 20 kg. The catheter removal rates for infection and mechanical complications were 0.084 and 0.081 per 100 catheter-days, respectively. Medical salvage procedures, ie, intracatheter thrombolytic agent use or antibiotic therapy (52.1%) and interventional radiologic catheter salvage procedures (47.1%), increased catheter survival by an average of 54.8 days (range, 0–959 d). Conclusions Radiologic placement of tunneled hemodialysis catheters is a safe and technically successful procedure in pediatric patients. However, there is a high rate of infectious and mechanical complications, particularly in younger and smaller patients.

Background— Systemic-pulmonary collateral (SPC) flow occurs commonly in single ventricle patients after superior cavo-pulmonary connection, with unclear clinical significance. We sought to evaluate the association between SPC flow and acute post-Fontan clinical outcomes using a novel method of quantifying SPC flow by cardiac magnetic resonance (CMR) imaging. Methods and Results— All patients who had SPC flow quantified by CMR imaging before Fontan were retrospectively reviewed to assess for acute clinical outcomes after Fontan completion. Forty-four subjects were included who had Fontan completion between May 2008 and September 2010. SPC flow prior to Fontan measured 1.5±0.9 L/min/m 2 , accounting for 31±11% of total aortic flow and 44±15% of total pulmonary venous flow. There was a significant linear association between natural log-transformed duration of hospitalization and SPC flow as a proportion of total aortic (rho=0.31, P =0.04) and total pulmonary venous flow (rho=0.29, P =0.05). After adjustment for Fontan type and presence of a fenestration, absolute SPC flow was significantly associated with hospital duration ≥7 days (odds ratio [OR]=9.2, P =0.02) and chest tube duration ≥10 days (OR=22.7, P =0.009). Similar associations exist for SPC flow as a percentage of total aortic (OR=1.09, P =0.048 for hospitalization ≥7 days; OR=1.24, P =0.007 for chest tube duration ≥10 days) and total pulmonary venous flow (OR=1.07, P =0.048 for hospitalization ≥7 days; OR=1.18, P =0.006 for chest tube duration ≥10 days). Conclusions— Increasing SPC flow before Fontan, as measured by CMR imaging, is associated with increased duration of hospitalization and chest tube following Fontan completion.

Cardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.

The present project investigated whether cardiac magnetic resonance (CMR) of aortic arch anomalies can be performed successfully in infants6 months of age without the use of cardiac anesthesia or deep sedation. We performed a retrospective review of infants ≤6 months old from 2005 to 2009 who underwent either CMR or computed tomography angiography to investigate aortic arch abnormalities. The CMR procedure used a "feed and swaddle" protocol without deep sedation or cardiac anesthesia. Of the 52 infants referred for CMR, 24 underwent the feed and swaddle protocol (aged 2.6 ± 1.4 months). One patient awoke during the study, and examination of the remaining 23 yielded a definitive diagnosis (success rate 96%). The scanning time was 6.2 ± 3.1 minutes, with the large airways evaluation accounting for 1/2 the time. Single-shot axial steady-state free precession, in which the definitive diagnosis was made, accounted for 0.59 ± 0.3 minutes. Fifteen infants were diagnosed with a vascular ring. Of the 8 infants who underwent surgery, the diagnostic accuracy was 100%. During the same period, 19 patients, who had undergone computed tomography angiography (aged 1.67 ± 1.20 months), were referred for aortic arch evaluation. Of these 19 patients, 6 (32%) underwent sedation or anesthesia. The imaging time was 0.08 ± 0.06 minutes, significantly different from the CMR times (p0.01). However, the overall room times (31.3 ± 22.3 and 35.8 ± 3.86 minutes, respectively) were not different between the CMR and angiographic groups. The radiation dose was 1.41 ± 1.03 mSv. In conclusion, CMR evaluation of aortic arch anomalies in children6 months old can be successfully completed quickly using a feed and swaddle approach with high diagnostic accuracy. This protocol avoids the risks of sedation, as well as the radiation associated with computed tomography angiography.

Chylothorax is an uncommon complication of cardiothoracic surgery in children that is traditionally treated with either conservative (diet modification, octreotide administration, and percutaneous drainage) or surgical (thoracic duct ligation, pleurodesis, and pleuroperitoneal shunt) approaches. We report here the cases of 2 children (a 6-month-old and a 1-month-old) with postoperative chylous leaks who were treated successfully by percutaneous thoracic duct embolization.

The appropriate imaging for pediatric patients being evaluated for suspected physical abuse depends on the age of the child, the presence of neurologic signs and symptoms, evidence of thoracic or abdominopelvic injuries, and whether the injuries are discrepant with the clinical history. The clinical presentations reviewed consider these factors and provide evidence-based consensus recommendations by the ACR Appropriateness Criteria(®) Expert Panel on Pediatric Imaging.

Historically, congenital pulmonary lymphangiectasia (CPL) has had poor treatment outcomes despite aggressive therapy. There are recent reports of ethiodized oil (Lipiodol) lymphangiography successfully treating lymphatic leakage in adults. In this report, we describe two infants with CPL complicated by chylothoraces successfully treated by instillation of ethiodized oil into the lymphatic system. Congenital atresia of the thoracic duct was demonstrated on the lymphangiogram in both patients before treatment. Both patients have shown good short-term outcomes without supplemental oxygen or fat restricted diets at 9 months of age. Ethiodized oil lymphangiography represents a new treatment modality for some patients with CPL.

Because of lack of coordination of the oral, facial, and neck muscles, many patients with cerebral palsy have excessive pooling of saliva, resulting in drooling. Injection of botulinum toxin type A reduces salivary flow. However, no best practice for this procedure has been reported in the literature. This study aimed to test the hypothesis that ultrasound-guided botox injection of the salivary glands in children is effective temporary palliation for excessive drooling. The study population consisted of 6 patients who underwent botox injection of salivary glands one or more times at a mid-Atlantic regional children's hospital. Parents were interviewed by telephone regarding the effects of the botox procedure on their child's drooling. The children who comprised our study population exhibited decreased drooling, less respiratory distress, and increased quality of life after the injections. Further research with a larger sample is warranted to support these findings.

Holodiastolic flow reversal in the descending aorta on echocardiogram suggests significant aortic regurgitation. The study aim was to determine whether the presence of holodiastolic flow reversal on cardiac magnetic resonance imaging (MRI) correlates with aortic valve regurgitant fraction. We retrospectively reviewed 166 cardiac MRIs (64 % male, age 14.1 ± 9.5 years) from January 2011 to May 2012 where velocity mapping was acquired at both the aortic valve and the descending aorta at the level of the diaphragm. Descending aorta velocity maps were checked for baseline offset using a static reference region. Holodiastolic flow reversal was defined as flow reversal throughout diastole both before and after baseline correction. Significant aortic regurgitation was defined as regurgitant fraction10 %. Aortic valve regurgitant fraction was10 % in 144 patients (Group A), 10-20 % inclusive in 7 patients (Group B), and20 % in 15 patients (Group C). Though the aortic valve regurgitant fraction was significantly higher for patients with holodiastolic flow reversal versus those without (8.5 ± 14.2 vs. 3.8 ± 6.6 %, p = 0.02), holodiastolic flow reversal was present in 32 Group A patients (22 %). In comparison, 4 Group B patients (57 %) and 7 Group C patients (47 %) had holodiastolic flow reversal. The sensitivity (Groups B and C) was 0.5, and the specificity (Group A) was 0.78. Holodiastolic flow reversal in the descending aorta on cardiac MRI was neither sensitive nor specific for predicting significant aortic regurgitation in this study population. Holodiastolic flow reversal in the absence of significant aortic regurgitation may be a relatively common finding in patients with congenital heart disease.

Background: Patients with a single ventricle experience a high rate of brain injury and adverse neurodevelopmental outcome; however, the incidence of brain abnormalities throughout surgical reconstruction and their relationship with cerebral blood flow, oxygen delivery, and carbon dioxide reactivity remain unknown. Methods: Patients with a single ventricle were studied with magnetic resonance imaging scans immediately prior to bidirectional Glenn (pre-BDG), before Fontan (BDG), and then 3 to 9 months after Fontan reconstruction. Results: One hundred sixty-eight consecutive subjects recruited into the project underwent 235 scans: 63 pre-BDG (mean age, 4.8±1.7 months), 118 BDG (2.9±1.4 years), and 54 after Fontan (2.4±1.0 years). Nonacute ischemic white matter changes on T2-weighted imaging, focal tissue loss, and ventriculomegaly were all more commonly detected in BDG and Fontan compared with pre-BDG patients ( P P Conclusions: Significant brain abnormalities are commonly present in patients with a single ventricle, and detection of these lesions increases as children progress through staged surgical reconstruction, with multiple coexisting lesions more common earlier than later. In addition, this study demonstrated that BDG patients had greater cerebral blood flow than did Fontan patients and that an inverse association exists of various indexes of cerebral blood flow with these brain lesions. However, CO 2 reactivity and oxygen delivery (with 1 exception) were not associated with brain lesion development. Clinical Trial Registration: URL: http://www.clinicaltrials.gov . Unique identifier: NCT02135081.

Conservative management of complicated esophageal perforations has gained favor in recent years. However, there are limited data concerning the applicability of this approach in the pediatric population. We describe the care and outcome of a 14-year-old girl who sustained an esophageal perforation after accidental ingestion of a shard of glass. The patient was treated using ultrasound-guided drainage catheter placement with simultaneous esophagoscopy and postoperative antibiotics. She was discharged within 1 week of presentation and enjoyed an uncomplicated recovery. We believe that selected cases of pediatric esophageal perforation may be safely and effectively treated using a conservative approach.

Clinicians use branch pulmonary artery (BPA) blood flow distribution to help determine the need for intervention. Although phase-contrast magnetic resonance (PCMR) flow measurements are accurate, this has never been shown in the vicinity of a BPA ferromagnetic stent (FS) which produces significant susceptibility artifact. We retrospectively reviewed 49 consecutive PCMR studies performed from 2005 to 2012 on patients with repaired conotruncal anomalies and either left (n = 29) or right PA (n = 20) stents. Three methods of measuring the stented BPA flow were compared: (1) main PA (MPA) minus nonstented BPA, (2) direct PCMR of stented BPA away from the artifact, and (3) pulmonary venous flows (ipsilateral to stented BPA and derived pulmonary blood flow ratio from bilateral pulmonary venous flows). Internal consistency was tested with the Student t test, linear regression, Bland-Altman analysis, and intraclass correlation (ICC). The mean age was 11.7 ± 6.9 years with 5.8 ± 4.2 years between stent placement and CMR. There was good agreement without significant difference between MPA-derived stented BPA flow (method 1) and direct PCMR of stented BPA (method 2; 41 ± 19% vs 39 ± 19%, p = 0.59; R(2) = 0.84, p0.001; ICC = 0.96). There was also good agreement between methods 1 and 2 compared to pulmonary venous flows, with the highest correlation occurring between method 2 and ipsilateral pulmonary venous flow (R(2) = 0.90, p0.001; ICC = 0.97 for MPA-derived-stented BPA flow; R(2) = 0.94, p0.001; ICC = 0.98 for direct PCMR of stented BPA). Eleven of the 49 patients (22%) underwent interventional catheterization after PCMR. In conclusion, in the vicinity of a BPA FS, accurate measurement of the net fractional pulmonary blood flow ratio is feasible. PCMR adjacent to the stent and ipsilateral pulmonary venous flows provide the most internally consistent data. These data underscore PCMR's utility in managing patients with implanted FS.

Background— Plastic bronchitis is a potentially fatal disorder occurring in children with single-ventricle physiology, and other diseases, as well, such as asthma. In this study, we report findings of abnormal pulmonary lymphatic flow, demonstrated by MRI lymphatic imaging, in patients with plastic bronchitis and percutaneous lymphatic intervention as a treatment for these patients. Methods and Results— This is a retrospective case series of 18 patients with surgically corrected congenital heart disease and plastic bronchitis who presented for lymphatic imaging and intervention. Lymphatic imaging included heavy T2-weighted MRI and dynamic contrast-enhanced magnetic resonance lymphangiogram. All patients underwent bilateral intranodal lymphangiogram, and most patients underwent percutaneous lymphatic intervention. In 16 of 18 patients, MRI or lymphangiogram or both demonstrated retrograde lymphatic flow from the thoracic duct toward lung parenchyma. Intranodal lymphangiogram and thoracic duct catheterization was successful in all patients. Seventeen of 18 patients underwent either lymphatic embolization procedures or thoracic duct stenting with covered stents to exclude retrograde flow into the lungs. One of the 2 patients who did not have retrograde lymphatic flow did not undergo a lymphatic interventional procedure. A total of 15 of 17(88%) patients who underwent an intervention had significant symptomatic improvement at a median follow-up of 315 days (range, 45–770 days). The most common complication observed was nonspecific transient abdominal pain and transient hypotension. Conclusions— In this study, we demonstrated abnormal pulmonary lymphatic perfusion in most patients with plastic bronchitis. Interruption of the lymphatic flow resulted in significant improvement of symptoms in these patients and, in some cases, at least temporary resolution of cast formation.

To evaluate technical feasibility, complications, and clinical outcomes of endovascular thrombolysis for iliofemoral thrombosis at two tertiary-care children's hospitals.Institutional review board-approved retrospective review from March 2003 through June 2013 showed that venous thrombolysis for iliofemoral thrombosis was performed in 57 children (64 limbs) with a median age of 16.1 years (mean age, 14.5 y; range, 1.0-17.8 y). Techniques included catheter-directed thrombolysis (CDT), percutaneous mechanical thrombectomy (PMT), and pharmacomechanical catheter-directed thrombolysis (PCDT) with adjunctive angioplasty and/or stent placement. Villalta and modified Villalta scales were applied retrospectively to follow-up data to assess postthrombotic syndrome (PTS).Technical success (≥ 50% thrombolysis) rate was 93.7%: grade III (100%) in 19 limbs, grade II (50%-99%) in 41 limbs, and grade I (50%) in four limbs. Techniques included CDT with PCDT (32.8%) or PMT (35.9%), CDT alone (26.6%), PCDT alone (4.7%) or with adjunctive angioplasty (54.7%), and stent placement (6.3%). Mean duration of CDT was 36.5 hours (range, 2.9-89.6 h). There was one major complication (1.8%) of bleeding requiring transfusion. Minor complications (ie, bleeding) occurred in seven patients (12.2%). Median follow-up was 1.5 years (range, 30 d to 7 y). Seven patients underwent repeat thrombolysis for recurrent thrombosis. The PTS rate was 59.3% per modified Villalta scale but only 2.1% per Villalta scale.Endovascular thrombolysis is technically feasible and safe for iliofemoral thrombosis in children. Variable results were seen with two scales to assess PTS, suggesting an acute need for standardization of outcome measurement in children.

Summary The pyruvate dehydrogenase (PDH) complex is an enzyme involved in the conversion of pyruvate to acetyl coenzyme A, an important and irreversible step in glycolysis. A deficiency in pyruvate dehydrogenase is a well defined but rare genetic defect of metabolism that is characterized by lactic acidosis. Variability exists in clinical presentation, radiographic imaging, and enzyme activity. We present a case of pyruvate dehydrogenase deficiency syndrome in which profound sensorineural hearing loss and internal auditory canal abnormalities were also found. We highlight the findings on auditory brainstem response (ABR) and radiographic studies and discuss the implications for cochlear implantation.

Babies are ideal subjects for investigation by sonography. They are small and easily examined with the use of high-frequency transducers. Their unique status in US imaging tends to get lost amid the array of other available modalities, such as MRI and CT imaging. Some of the questions posed in pediatric musculoskeletal imaging that are routinely directed to other imaging modalities can be solved quickly at a lower cost by sonography in neonates and infants without sedation or any known clinical risk. These small children not only have thinner soft tissues but they have long bones and midline spine structures whose ends are largely composed of cartilage, which provides an early opportunity to examine these regions by US. And we cannot overestimate the value of new parents being able to stay next to their baby during imaging in a warm, friendly and non-threatening environment.

The investigators recently validated a method of quantifying systemic-to-pulmonary arterial collateral flow using phase-contrast magnetic resonance imaging velocity mapping. Cross-sectional data suggest decreased collateral flow in patients with total cavopulmonary connections (TCPCs) compared with those with superior cavopulmonary connections (SCPCs). However, no studies have examined serial changes in collateral flow from SCPCs to TCPCs in the same patients. The aim of this study was to examine differences in collateral flow between patients with SCPCs and those with TCPCs. Collateral flow was quantified by 2 independent measures from 250 single-ventricle studies in 219 different patients (115 SCPC and 135 TCPC studies, 31 patients with both) and 18 controls, during routine studies using through-plane phase-contrast magnetic resonance imaging. Collateral flow was indexed to body surface area, aortic flow, and pulmonary venous flow. Regardless of indexing method, SCPC patients had significantly higher collateral flow than TCPC patients (1.64 ± 0.8 vs 1.03 ± 0.8 L/min/m(2), p0.001). In 31 patients who underwent serial examinations, collateral flow as a fraction of aortic flow increased early after TCPC completion. In TCPC patients, indexed collateral flow demonstrated a significant negative correlation with time from TCPC. In conclusion, SCPC and TCPC patients demonstrate substantial collateral flow, with SCPC patients having higher collateral flow than TCPC patients overall. On the basis of the paired subset analysis, collateral flow does not decrease in the short term after TCPC completion and trends toward an increase. In the long term, however, collateral flow decreases over time after TCPC completion.

Background Diffuse fibrosis and strain are abnormal in adult cardiomyopathy patients and in children exposed to anthracyclines, but have not been well studied in pediatric hypertrophic (HCM) and dilated (DCM) cardiomyopathy. We hypothesized that HCM and DCM patients have different diffuse fibrosis and strain characteristics. Methods

Anomalous left coronary artery from the inappropriate aortic sinus with intraseptal course is generally benign but can be confused on imaging studies with the potentially lethal interarterial, intramural anomalous left coronary artery. The purpose of this study was to assess normal ostial morphologic features and intraseptal course using cardiac MRI and CT in pediatric patients with intraseptal anomalous left coronary artery.A retrospective review was conducted of the medical records of 14 children with the diagnosis of intraseptal anomalous left coronary artery between November 2009 and March 2013. Coronary artery origin and course were evaluated with cardiac MRI or CT, and 3D assessment of coronary ostial morphologic features was performed with virtual angioscopy.The patient ages ranged from 5 to 18 years at diagnosis; 10 (71.4%) were boys. The right and left coronary origins were the right sinus of Valsalva as a common origin (n = 9) or a single coronary artery (n = 5). Anomalous intraseptal left main coronary was found in 13 patients, and one patient had anomalous left anterior descending with retroaortic circumflex coronary artery. Anomalous coronary ostia were round and without stenosis in all studies. The anomalous vessel was identified with echocardiography, but the anomalous left coronary artery was not delineated, and a normal ostium was not adequately portrayed in any instance.By use of cardiac MRI and CT, the anomalous course of round coronary ostia was confirmed and visualized in a pediatric cohort with intraseptal anomalous left coronary artery. The data provide the basis for understanding the benign clinical course and showing that surgery is unnecessary for this coronary anomaly.

Patients with single ventricle can develop aortic-to-pulmonary collaterals (APCs). Along with systemic-to-pulmonary artery shunts, these structures represent a direct pathway from systemic to pulmonary circulations, and may limit cerebral blood flow (CBF). This study investigated the relationship between CBF and APC flow on room air and in hypercarbia, which increases CBF in patients with single ventricle.106 consecutive patients with single ventricle underwent 118 cardiac magnetic resonance (CMR) scans in this cross-sectional study; 34 prior to bidirectional Glenn (BDG) (0.50±0.30 years old), 50 prior to Fontan (3.19±1.03 years old) and 34 3-9 months after Fontan (3.98±1.39 years old). Velocity mapping measured flows in the aorta, cavae and jugular veins. Analysis of variance (ANOVA) and multiple linear regression were used. Significance was p0.05.A strong inverse correlation was noted between CBF and APC/shunt both on room air and with hypercarbia whether CBF was indexed to aortic flow or body surface area, independent of age, cardiopulmonary bypass time, Po2 and Pco2 (R=-0.67--0.70 for all patients on room air, p0.01 and R=-0.49--0.90 in hypercarbia, p0.01). Correlations were not different between surgical stages. CBF was lower, and APCs/shunt flow was higher prior to BDG than in other stages.There is a strong inverse relationship between CBF and APC/shunt flow in patients with single ventricle throughout surgical reconstruction on room air and in hypercarbia independent of other factors. We speculate that APC/shunt flow may have a negative impact on cerebral development and neurodevelopmental outcome. Interventions on APC may modify CBF, holding out the prospect for improving neurodevelopmental trajectory.NCT02135081.

Purpose To demonstrate feasibility and evaluate outcomes of direct-stick saphenous and single-incision tunneled femoral noncuffed central venous catheters (CVCs) placed in a large series of neonates and infants at a single institution. Materials and Methods A retrospective review was performed for all neonates and infants receiving a lower extremity CVC by interventional radiology between 2007 and 2012. Technical success, mechanical and infectious complications, and catheter outcomes were recorded. Results There were 271 primary insertions performed in 243 children by interventional radiologists in the interventional radiology suite or at the bedside. CVCs were placed via the femoral vein with single-incision technique (84.9%) or the saphenous vein via a direct-stick technique (15.1%), with a technical success rate of 100%. The total number of catheter-days was 7,917 days (median, 19 d; range, 0–220 d). The number of primary catheter-days was 5,333 days (median, 15 d; range, 0–123.0 d), and salvage procedures prolonged catheter life by 2,584 days (median, 15 d; range, 1.0–101.0 d). The mechanical and adjusted infectious complication rates were 1.67 and 0.44 per 100 catheter-days. Conclusions Image-guided placement of saphenous or tunneled femoral catheters using a single incision is a safe and feasible method for vascular access in neonates and infants.

Anomalous origin of a coronary artery from the contralateral sinus of Valsalva is associated with exercise-induced ischemia and sudden death. That is thought to be due to aortic enlargement in patients with an elliptical ostium. We hypothesize that virtual angioscopy can identify abnormal coronary ostial morphology in these patients.We retrospectively analyzed 55 consecutive pediatric coronary artery magnetic resonance imaging studies from January 2006 to January 2010 with the diagnosis of anomalous right (n = 20), or left (n = 7) coronary artery, or normal coronary origins (n = 28). One postmortem heart specimen with anomalous left coronary artery was imaged and analyzed to validate our technique. Virtual angioscopy analysis was used for visualization and measurement of the coronary ostia.Distinct aortic origins of the right and left coronaries were seen in all 55 studies. An elliptical orifice with a longer superior-inferior dimension was seen in all anomalous ostia, in contrast to a circular ostium in all normal origins. That was quantified in anomalous ostia with a long-axis to short-axis ratio of 2.5 ± 0.5 (right) and 2.4 ± 0.5 (left) compared with 1.1 ± 0.2 (right) and 1.0 ± 0.3 (left) in controls (p0.001 for right and left ostia comparisons). Ostial morphology was confirmed in all 9 patients who underwent operative repair and in 1 patient at autopsy.Virtual angioscopy identifies abnormal ostial morphology in anomalous coronary artery patients, which is important for characterizing the diagnosis of patients who may be at risk for sudden death.

The purpose of this study was to evaluate the development of intercondylar notch width in children using MRI and assesse the relationship between the cruciate ligaments and the width of the intercondylar notch during growth.We reviewed MRIs and medical records of 76 children (age range 3.8-16.9) without derangements of the knee. Cases with knee joint instability and/or major trauma were excluded. On a standardized coronal image, measurements of the width of the intercondylar notch, knee, ACL and PCL were performed using image analysis software. The notch width index (NWI), ACL/notch, ACL/knee width, PCL/notch, and PCL/knee width were calculated.NWI showed statistical correlation with age. The highest values were found in the youngest children, and the lowest in the oldest group. While ACL/knee width, and PCL/knee width ratios remained proportional over time, the ACL/notch, and PCL/notch ratios increased with the age of the patient.NWI changes with age and decreases from childhood to adolescence. A relative decrease in NWI accounts for an increase in the ACL/notch and PCL/notch ratios with age. Studies about NWI in children should consider age as a cofactor for analysis.

Protein-losing enteropathy (PLE) and plastic bronchitis are serious complications that occur after single-ventricle surgery. A lymphatic cause for these conditions has been proposed, but imaging correlation has not been reported. The objective of this study was to evaluate lymphatic abnormalities in patients after functional single-ventricle palliation compared with patients with non-single-ventricle congenital heart conditions using T2-weighted MR lymphangiography.We retrospectively reviewed imaging data from 48 patients who underwent T2-weighted MR lymphangiography in our institution between May 1, 2012, and October 24, 2012. The patients were divided into four groups: patients who underwent superior cavopulmonary connection, patients who underwent total cavopulmonary connection, patients with total cavopulmonary connection and lymphatic complications, and patients with non-single-ventricle cardiac anomalies.There were 38 patients with single ventricles in this study. The lymphatic abnormalities observed in these patients included thoracic duct dilation greater than 3 mm (31%), lymphangiectasia and lymphatic collateralization (78%), and tissue edema (86%). There were five patients with PLE, one patient with plastic bronchitis, and one patient with chronic chylous effusions and superior cavopulmonary connection. The patients with PLE and plastic bronchitis had statistically significant larger thoracic duct maximal diameters (median, 3.9 mm; range, 3-7.2 mm) than did the other patients with total cavopulmonary connection (p0.01). In the two-ventricle patient group, there were no abnormal lymphatic findings.Lymphatic abnormalities are found in many patients after functional single-ventricle palliation. T2-weighted unenhanced MRI is capable of anatomic assessment of the lymphatic system in this patient population and has promise for guiding treatment in the future.

How radiologists meet and care for neonates with mass lesions of the chest has changed. Formerly, infants with respiratory distress and no diagnosis presented for emergent imaging for the clinician to make a diagnosis and formulate a treatment plan. Widespread use of prenatal ultrasound (US) screening has resulted in the identification of many of these infants in utero with a prenatally determined presumptive diagnosis and a perinatal care strategy. Depending on the style and system of prenatal and perinatal care, imaging may be performed by obstetricians, general radiologists, or pediatric radiologists in women's and infant's institutions. The mainstay of prenatal imaging remains US, but research continues into the use of MR imaging in certain circumstances. Without imaging, some of the fetuses have no clinical signs to lead to their diagnoses. Others may show maternal polyhydramnios as evidence of their masses by creating esophageal impairment to normal amniotic fluid pathways. Once diagnosed, the coexisting finding of a mediastinal shift serves as a warning to pay very close attention in follow-up, because all authorities currently seem to agree that once the mediastinum is so shifted as to impair venous return to the fetal chest, to create a state of low cardiac output, and to result in a hydropic fetus, viability is severely threatened. 1 Some mothers become ill in association with their threatened fetuses, a preeclamptic state referred to as the maternal mirror syndrome . 8 Despite even heroic and curative fetal surgery, maternal symptoms may inexorably progress, mandating that the child be emergently delivered. Some researchers believe that the placenta may release a causative factor responsible for the mirror syndrome. 20

Background Single-ventricle patients undergoing surgical reconstruction experience a high rate of brain injury. Incidental findings on preoperative brain scans may result in safety considerations involving hemorrhage extension during cardiopulmonary bypass that result in surgical postponement. Methods Single-ventricle patients were studied with brain scans immediately preoperatively, as part of a National Institutes of Health study, and were reviewed by neuroradiology immediately before cardiopulmonary bypass. Results Of 144 consecutive patients recruited into the project, 33 were studied before stage I (3.7 ± 1.8 days), 34 before bidirectional Glenn (5.8 ± 0.5 months), and 67 before Fontan (3.3 ± 1.1 years) operations. Six operations (4.5%), 2 before stage I, 3 before bidirectional Glenn, and 1 before Fontan, were postponed because of concerning findings on brain magnetic resonance imaging. Five were due to unexpected incidental findings of acute intracranial hemorrhage, and 1 was due to diffuse cerebellar cytotoxic edema; none who proceeded to operation had these lesions. Prematurity and genetic syndromes were not present in any patients with a postponed operation. Four of 4 before bidirectional Glenn/Fontan with surgical delays had hypoplastic left heart syndrome compared with 44 of 97 who did not ( p = 0.048). After observation and follow-up, all eventually had successful operations with bypass. Conclusions Preoperative brain magnetic resonance imaging performed in children with single ventricles disclosed injuries in 4.5% leading to surgical delay; hemorrhagic lesions were most common and raised concerns for extension during the operation. The true risk of progression and need for delay of the operation due to heparinization associated with these lesions remains uncertain.

Purpose: We identified sonographic prognosticators to aid in distinguishing obstructive from nonobstructive hydronephrosis in children.Materials and Methods: Twelve sonographic variables were initially analyzed to determine significant associations between the variables and the presence of urinary tract obstruction as defined by diuretic radionuclide renography. The significant findings were subsequently subjected to logistic regression models to identify potential predictors for obstructive hydronephrosis.Results: The 7 variables associated with a significantly higher risk of urinary tract obstruction were increased echogenicity, parenchymal rims 5 mm. or less, contralateral hypertrophy, resistive index ratio 1.10 or greater, resistive index difference with diuresis of 70% or greater, ureter diameter 10 mm. or greater and aperistaltic ureter. These variables were used for the development of a multivariate scoring system.Conclusions: The obstructive scoring system shows promise as a screening meth...

The use of cerebral intraoperative angiography has been described in resection of arteriovenous malformations (AVMs) in adults. More recently, studies have described experiences with intraoperative angiography in a small number of children. However, data on the safety and clinical utility of intraoperative angiography in the pediatric population remains limited in comparison with available data in adults. The aim of the study was to evaluate the use of cerebral intraoperative angiography in children undergoing AVM resection. The clinical utility of intraoperative angiography and procedure-related complications were evaluated.A retrospective review was performed for all patients undergoing cerebral AVM resection with intraoperative angiography at The Children's Hospital of Philadelphia between 2008 and 2012. Patient imaging and operative and medical notes were reviewed to evaluate for end points of the study. A total of 17 patients (8 males, 9 females) were identified, with a median age of 12.1 years (range 1.2-17.9 years) and median weight of 45.5 kg (range 12.1-78.9 kg).A total of 21 intraoperative angiography procedures were performed for 18 AVM resections in 17 patients. The technical success rate was 94%. In 2 cases (11%), intraoperative angiography demonstrated a residual AVM, and repeat resections were performed. In both cases, no recurrent disease was noted on postoperative follow-up. One procedure-related complication (4.8%) occurred in 1 patient who was positioned prone. Recurrence to date was noted in 2 (14%) of the 14 cases with available postoperative follow-up at 3.5 and 4.7 months following resection with intraoperative angiography. The median follow-up time from intraoperative angiography to the most recent postoperative angiography was 1.1 years (range 4.3 months to 3.8 years).Intraoperative angiography is an effective and safe adjunct for surgical management of cerebral AVMs in the pediatric population.

Central venous access is indispensable in caring for children with infections, malignancies and chronic illnesses, and image-guided placement of central venous access devices (CVAD) is increasingly used. A single-incision technique for placement of tunneled central venous access devices at the internal jugular vein has been described; however the technique has not been described exclusively in children.To describe our initial experience using the single-incision technique for tunneled central venous access at the internal jugular vein in children.We conducted a retrospective review of the interventional radiology database and electronic medical records of 15 children who received a tunneled central venous access device (CVAD) using a single incision between 2010 and 2012. Patients included eight boys and seven girls with an average age of 11 years (median 13.3 years, range 1-18.7 years) and average weight of 44.2 kg (median 38.3 kg, range 9.6-99.0 kg).A total of 17 primary insertions were performed. Technical success was 100%. Total catheter life consisted of 1,416 catheter-days (mean 83.3 days, range 8-502 days). There were no procedure-related or early complications. Seven late complications requiring intervention occurred in three catheters. Total mechanical and infectious complications occurred at rates of 0.28 and 0.21 per 100 catheter-days, respectively. The adjusted rate for infectious complications was 0.14 per 100 catheter-days. Medical salvage procedures (83%) and interventional radiology salvage procedures (17%) prolonged catheter life by an average of 94.5 days (range 10-329 days).This study demonstrates safe use and technical feasibility of the image-guided single-incision technique for central venous access in children, particularly in children in whom the conventional technique is less desirable.

A girl 5 years 11 months of age, belonging to an extensive kindred with multiple endocrine neoplasia, type IIA (MEN IIA), was found to have multifocal medullary thyroid carcinoma with metastasis in one paraglandular lymph node after positive findings on a calcium-pentagastrin stimulation test. Her sister, 3 years 8 months of age, also had an elevated calcitonin level, and thyroidectomy revealed C-cell hyperplasia and a focus of medullary thyroid carcinoma. These two cases underscore the need for prophylactic thyroidectomies in MEN IIA patients as young as 5 years of age and strict yearly provocative screening beginning at age 1 year.

Therapy for X-linked hypophosphatemia (XLH) only partially corrects skeletal lesions and is often complicated by hyperparathyroidism. 24,25(OH)2 D3 improves skeletal lesions in a murine model of XLH and suppresses PTH secretion in animals. Therefore, we undertook a placebo-controlled trial of 24,25(OH)2 D3 supplementation to standard treatment in patients with XLH to improve bone disease and reduce hyperparathyroid complications. Fifteen subjects with XLH receiving standard treatment [1,25(OH)2 D3 or dihydrotachysterol plus phosphate] were evaluated, supplemented with placebo, and reevaluated one yr later. 24,25(OH)2 D3 supplementation was then begun and studies repeated after another year. Each patient underwent a detailed evaluation of calcium homeostasis over a 24-h period. Rachitic abnormalities were assessed radiographically in children. Adults underwent bone biopsies. 24,25(OH)2 D3 normalized PTH values in nine subjects (peak PTH was 46.5 +/- 6.6 pmol/L at entry, 42.3 +/- 5.9 pmol/L after placebo, and 23.3 +/- 5.4 pmol/L after 24,25(OH)2 D3). Nephrogenous cAMP decreased at night, coincident with the decrease in PTH, and serum phosphorus was slightly greater with 24,25(OH)2 D3. Radiographic features of rickets improved during 24,25(OH)2 D3 supplementation in children, and osteoid surface decreased in adults. 24,25(OH)2 D3 is a useful adjunct to standard therapy in XLH by effecting correction of hyperparathyroidism and improvement of rickets and osteomalacia.