Differential Effect of the Ratio of Right Ventricular Volume to Left Ventricular Volume in Children with Repaired Tetralogy of Fallot.

We aimed to compare the diagnostic value of indexed right ventricular end-diastolic volume (RVEDVi) and the ratio of right ventricle volume to left ventricle volume (RV/LV ratio) in prediction of the severity of pulmonary regurgitation (PR) expressed as the PR fraction (PRF) after surgery of tetralogy of Fallot (TOF). Forty-one patients with repaired TOF were included in the study. RVEDVi, LVEDVi, RV/LV ratio, PRF and ejection fraction were measured with magnetic resonance imaging. A PRF of more than 20% was considered significant. The predictive capability of two markers (RVEDVi and RV/LV ratio) for significant PR was compared using multivariate linear regression analysis and receiver operating characteristic (ROC) analysis. Both the RV/LV ratio and RVEDVi showed a correlation with PRF (r = 0.526/0.321, p = 0.001/0.041) in the correlation analysis, but in multivariate regression analysis the only independent predictor of PRF was the RV/LV ratio (F = 14.890, p = 0.001). ROC analysis revealed that a better discrimination of significant PR (>20%) from slight types (=20%) PR can be reached with the RV/LV ratio than RVEDVi (AUC = 0.805/0.709, p = 0.01). The RV/LV ratio was better than RVEDVi at differentiating mild from moderate PR (p = 0.006 vs. p = 0.153), and proved superior over RVEDVi in predicting PR based on the PRF criterion.

Congenital portosystemic shunts with and without gastrointestinal bleeding - case series.

BACKGROUND: The clinical presentation of congenital portosystemic shunt is variable and gastrointestinal bleeding is an uncommon presentation. OBJECTIVE: To describe the imaging features of congenital portosystemic shunt as it presented in 11 children with (n = 6) and without gastrointestinal bleeding (n = 5). MATERIALS AND METHODS: We performed a retrospective study on a clinical and imaging dataset of 11 children diagnosed with congenital portosystemic shunt. RESULTS: A total of 11 children with congenital portosystemic shunt were included in this study, 7 with extrahepatic portosystemic shunts and 4 with intrahepatic portosystemic shunts. Six patients with gastrointestinal bleeding had an extrahepatic portosystemic shunt, and the imaging results showed that the shunts originated from the splenomesenteric junction (n = 5) or splenic vein (n = 1) and connected to the internal iliac vein. Among the five cases of congenital portosystemic shunt without gastrointestinal bleeding, one case was an extrahepatic portosystemic shunt and the other four were intrahepatic portosystemic shunts. CONCLUSION: Most congenital portosystemic shunt patients with gastrointestinal bleeding had a shunt that drained portal blood into the iliac vein via an inferior mesenteric vein. This type of shunt was uncommon, but the concomitant rate of gastrointestinal bleeding with this type of shunt was high.

Hepatic involvement of Langerhans cell histiocytosis in children--imaging findings of computed tomography, magnetic resonance imaging and magnetic resonance cholangiopancreatography.

BACKGROUND: Langerhans cell histiocytosis is a rare disease that occurs mainly in children, and hepatic involvement is generally a poor prognostic factor. OBJECTIVE: To describe CT and MRI findings of hepatic involvement of Langerhans cell histiocytosis in children, especially the abnormal bile duct manifestation on magnetic resonance cholangiopancreatography (MRCP). MATERIALS AND METHODS: Thirteen children (seven boys, six girls; mean age 28.9 months) were diagnosed with disseminated Langerhans cell histiocytosis. They underwent CT (n = 5) or MRI (n = 4), or CT and MRI examinations (n = 4) to evaluate the liver involvement. RESULTS: Periportal abnormalities presented as band-like or nodular lesions on CT and MRI in all 13 children. The hepatic parenchymal lesions were found in the peripheral regions of the liver in seven children, including multiple nodules on MRI (n = 6), and cystic-like lesions on CT and MRI (n = 3). In 11 of the 13 children the dilatations of the bile ducts were observed on CT and MRI. Eight of the 13 children underwent MR cholangiopancreatography, which demonstrated stenoses or segmental stenoses with slight dilatation of the central bile ducts, including the common hepatic duct and its first-order branches. The peripheral bile ducts in these children showed segmental dilatations and stenoses. CONCLUSION: Stenosis of the central bile ducts revealed by MR cholangiopancreatography was the most significant finding of liver involvement in Langerhans cell histiocytosis in children.

Accuracy of early DMSA scan for VUR in young children with febrile UTI.

OBJECTIVE: To evaluate the accuracy of an acute (99m)Tc-dimercaptosuccinic acid (DMSA) scan in predicting dilating vesicoureteral reflux (VUR) among young children with a febrile urinary tract infection (UTI). METHODS: The medical records of children (≤ 2 years of age), presenting with febrile UTI between January 2000 and December 2011, were retrospectively reviewed. RESULTS: A total of 523 children were included in this study, of whom 397 children (75.9%) had abnormal DMSA results and 178 children (34.0%) were identified as VUR on micturating cystourethrography (MCU). Among all the patients, the number of children with dilating VUR was 151 (28.9%). The rate of abnormal results on DMSA for the dilating VUR group was significantly higher than the rates for the non-VUR and low-grade VUR groups (P < .01). In the <6 months age group and ≥ 6 months age group, the sensitivities of DMSA in predicting dilating VUR were 96.15% and 100.0%, respectively, the negative predictive values were 97.26% and 100.0%, respectively, and the negative likelihood ratios were 0.0911 and 0.0000, respectively. CONCLUSION: For children ≤ 2 years of age with a febrile UTI, an acute DMSA scan is valuable in the exclusion of dilating VUR. The likelihood of the presence of dilating VUR on MCU is rather low when the result of DMSA is negative. DMSA should be conducted to assess the need for an MCU.

Role of plain radiography and CT angiography in the evaluation of obstructed total anomalous pulmonary venous connection.

BACKGROUND: Obstructed total anomalous pulmonary venous connection (TAPVC) is frequently misdiagnosed as pulmonary disease and without operative correction early death is common. It is important to make a correct diagnosis before surgery. OBJECTIVE: The purpose of this study was to describe the chest radiographic features of obstructed TAPVC and compare CT angiography with transthoracic echocardiography in the evaluation of obstructed TAPVC. MATERIALS AND METHODS: Eighteen children with obstructed TAPVC were assessed. Their clinical and imaging data were retrospectively reviewed. The characteristic radiographic findings were analyzed and compared with surgical results, and the diagnostic accuracy of CT angiography and transthoracic echocardiography was evaluated in terms of pulmonary venous drainage and obstruction detection. RESULTS: The common radiographic features included pulmonary venous congestion or edema or both (16 of 18 cases, 89%), and absence of cardiomegaly (12 of 18 cases, 67%). CT angiography correctly diagnosed TAPVC and clearly revealed the draining sites in all children (five with supracardiac TAPVC, three with cardiac TAPVC, eight with infracardiac TAPVC and two with mixed TAPVC). The diagnostic agreement between CT angiography and surgery was 100%. Transthoracic echocardiography only correctly revealed the draining sites in 11 children (5 with supracardiac TAPVC, 2 with cardiac TAPVC and 4 with infracardiac TAPVC). The diagnostic agreement between transthoracic echocardiography and surgery was 61%. The diagnostic accuracy of CT angiography was higher than that of transthoracic echocardiography (P = 0.0156). Thirty-four sites of obstruction were correctly detected by CT angiography (11 in the mediastinum, 1 at the diaphragmatic level, 9 below the diaphragm and 13 stenotic individual pulmonary veins in the lung). The diagnostic agreement between CT angiography and surgery was 92%. Transthoracic echocardiography only correctly detected 15 sites of obstruction (11 in the mediastinum, 1 at the diaphragmatic level and 3 below the diaphragm). The diagnostic agreement between transthoracic echocardiography and surgery was 41%. The rate of detection for sites of obstruction with transthoracic echocardiography was much lower than that of CT angiography (P = 0.0002). CONCLUSION: In children with obstructed TAPVC, plain radiographs usually show a characteristic pattern of pulmonary venous congestion or edema, or both, and a normal cardiac silhouette. CT angiography is superior to transthoracic echocardiography in the evaluation of pulmonary venous drainage and obstruction, especially in children with infracardiac and mixed TAPVC.

[Preliminary application of magnetic resonance susceptibility weighted imaging in neonates with punctate white matter lesions].

OBJECTIVE: To evaluate the severity of punctate white matter lesions (PWML) in neonatal brain injury with susceptibility weighted imaging (SWI) and explore the value and limitation of SWI versus the conventional magnetic resonance imaging (MRI). METHODS: A total of 34 neonates presenting with PWML at initial MRI were recruited for this prospective study. PWML were defined as punctuate lesions with T(1) hyperintensity and T(2) isointensity or hypointensity in white matter. There were 21 males and 13 females with a median age of 9.24 days (range: 2 - 17 days). All MRI examinations were performed at 1.5 Tesla unit including conventional MRI (T(1), T(2) & Flair sequences), DWI and SWI. PWML were classified into two groups: (1) T(1) hyperintensity & T(2) isointensity; (2) T(1) hyperintensity & T(2) hypointensity. The manifestations of PWML on SWI were analyzed. RESULTS: Among all cases, only 5 cases (14.7%) demonstrated an evidence of hemorrhage on SWI. There were 7 cases in Group 1. Only 1 case showed punctate hypointensity in the areas of PWML on SWI while there was no hemorrhage on SWI in other 6 cases. Twenty-seven cases were in Group 2. Only 4 cases showed an evidence of hemorrhage on SWI while hemorrhage was absent on SWI in other 23 cases. CONCLUSION: Most areas of PWML in neonatal brain show no hemorrhage on SWI. And SWI can help to identify whether or not hemorrhage is present in PWML of neonates.

Multidetector CT angiography and 3D reconstruction in young children with coarctation of the aorta.

The purpose of this study was to assess the reliability of multidetector CT (MDCT) angiography and three-dimensional (3D) reconstruction in patients with coarctation of the aorta. Sixteen patients (median age, 2 months; range, 15 days to 28 months) with suspected coarctation of the aorta underwent both color Doppler echocardiography (CDE) and MDCT. In addition to the CT axial slices, 3D reconstructions such as volume rendering and multiple planar reformation were used to diagnose coarctation and associated cardiac abnormalities. The sensitivities of diagnosis were evaluated compared with MDCT and echocardiography and surgical findings. Sixteen cases were diagnosed as coarctation of the aorta by MDCT. This included hypoplasia of the aortic arch (n = 5). The results were confirmed at the time of surgery. Diagnosis sensitivities of coarctation were 87.5% for axial and 100% for both multiplanar and 3D volume-rendered images. Moreover, MDCT was able to clearly display the location and extent of the coarctation. Focal defects were observed in 14 cases, and diffuse defects in 2 cases. The sensitivity of MDCT diagnosis for coarctation of the aorta was 100%, which was higher than that of CDE (87.5%). Ventricular septum defect was diagnosed by MDCT in 11 cases, with 1 missed, and the sensitivity was 91.7% (11/12), which was lower than that of CDE (100%; 12/12). In addition, six patients had patent ductus arteriosus. Two patients in this study had collateral vessel formation. We conclude that CTA with 3D reconstruction represents a reliable noninvasive technique for the assessment of coarctation. It may serve as a noninvasive diagnostic tool before intervention or surgical treatment.