Structured magnetic resonance imaging-based characterization of the marginal vein reveals limits of the Weber-classification.

Background: The marginal vein (MV) is a persisting embryonic vein located at the lateral aspect of the lower limb. The Weber-classification, which was developed on the basis of phlebography in the 1990s, is the only existing classification system for this rare disease. Aim of this study was the structured characterization of the lateral marginal vein (MV) using magnetic resonance imaging (MRI) and evaluation of the applicability of the Weber-classification. Patients and methods: Institutional Review Board approval was obtained for this retrospective, single-center study. All patients who underwent contrast-enhanced MRI (using a prospectively determined protocol) of the untreated MV were included. MV anatomy and associated findings were characterized in a structured way taking into account the criteria of the Weber-classification for MV: inflow, outflow and extension. If three criteria of the Weber-classification were fulfilled the MV was categorized as "classifiable according to Weber". The MV was categorized as "partially classifiable according to Weber", if two criteria were met and as "not classifiable according to Weber" if less than two criteria were applicable. Results: 56 imaging studies of 58 MV (7 thoracoabdominal, 51 lower extremities) were reviewed. 18/51 MV of the lower extremities were "classifiable" according to the Weber-classification. 33/51 lower extremity MV were not definitely categorized according to the Weber-classification: 19/51 MV were "partially classifiable" and 14/51 MV were "not classifiable". 30/51 MV presented with hypoplastic, 1/51 with aplastic deep venous system. 34/51 lower extremity and 6/7 thoracoabdominal MV were associated with an additional vascular malformation (VM). Conclusions: MRI is suitable for detailed anatomic characterization of the MV and reveals additional therapy relevant findings like associated VM. The Weber-classification was not applicable in most cases, reflecting its limits and the heterogeneity of this rare disease. Structured reports rather than an obsolete classification system should be preferred for MRI of the MV.

Predictors of portal vein complications after pediatric liver transplantation: A German center experience.

BACKGROUND: Portal vein complications (PVCs) after pediatric liver transplantation (LT) are sometimes asymptomatic, especially in the early phase, and can threaten both the graft and patient's survival. Therefore, the purpose of this study is to analyze the risk factors for portal vein thrombosis (PVT) and portal vein stenosis (PVS) after pediatric LT. METHODS: All pediatric patients (n = 115) who underwent primary LT at Regensburg University Hospital between January 2010 and April 2017 were included in this study. The pre-, intra-, and postoperative parameters of all patients were retrospectively reviewed and risk factors for both PVT and PVS were analyzed. RESULTS: Of the 115 patients, living donor LT was performed on 57 (49.5%) patients, and biliary atresia was the primary diagnosis in 65 patients (56%). After pediatric LT, 9% of patients developed PVT, and 16.5% developed PVS. Patient weight ≤7 kg [odds ratio (OR) 9.35, 95% confidence interval (CI) 1.03-84.9, p = .04] and GRWR >3% (OR 15.4, 95% CI 1.98-129.5, p = .01) were the independent risk factors for the development of PVT and PVS, respectively upon multivariate analysis. The overall patient survival rates at 1, 3, and 5 years were 91%, 90%, and 89%, respectively, and there was no difference in patient survival among those with and without PVCs. CONCLUSIONS: Pediatric patients with body weight <7 kg and/or receiving a graft with GRWR >3% may develop PVCs and so require certain surgical modifications, close follow-up, and prophylactic anticoagulant therapy following transplant.

Additive value of transarterial embolization to systemic sirolimus treatment in kaposiform hemangioendothelioma.

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor in children, which can be accompanied by life-threatening thrombocytopenia, referred to as Kasabach-Merritt phenomenon (KMP). The mTOR inhibitor sirolimus is emerging as targeted therapy in KHE. As the sirolimus effect on KHE occurs only after several weeks, we aimed to evaluate whether additional transarterial embolization is of benefit for children with KHE and KMP. Seventeen patients with KHE and KMP acquired from 11 hospitals in Germany were retrospectively divided into two cohorts. Children being treated with adjunct transarterial embolization and systemic sirolimus, and those being treated with sirolimus without additional embolization. Bleeding grade as defined by WHO was determined for all patients. Response of the primary tumor at 6 and 12 months assessed by magnetic resonance imaging (MRI), time to response of KMP defined as thrombocyte increase >150 × 103 /µL, as well as rebound rates of both after cessation of sirolimus were compared. N = 8 patients had undergone additive embolization to systemic sirolimus therapy, sirolimus in this group was started after a mean of 6.5 ± 3 days following embolization. N = 9 patients were identified who had received sirolimus without additional embolization. Adjunct embolization induced a more rapid resolution of KMP within a median of 7 days vs 3 months; however, tumor response as well as rebound rates were similar between both groups. Additive embolization may be of value for a more rapid rescue of consumptive coagulopathy in children with KHE and KMP compared to systemic sirolimus only.

Chronic non-bacterial osteomyelitis (CNO) in childhood and adolescence - a disease with many faces. / Die Chronische Nicht Bakterielle Osteomyelitis (CNO) im Kindes- und Jugendalter ­ eine Erkrankung mit vielen Gesichtern.

BACKGROUND: Chronic non-bacterial osteomyelitis (CNO) is a chronic inflammatory skeletal disease that affects particularly children and adolescents and is often diagnosed with a delay. With whole-body MRI, early diagnosis of this disease is possible in many cases. Since children and adolescents frequently present with non-specific complaints for outpatient radiological diagnosis, every radiologist should have basic knowledge of this complex clinical picture. MATERIALS: In this review the basics and current findings regarding the disease are discussed. Unusual courses are also presented. RESULTS AND CONCLUSION: With knowledge of the radiographic and MR tomographic characteristics of the mostly multifocal bone lesions, the diagnosis of CNO can be reliably made in many cases. In particular, the early use of whole-body MRI plays an important role. Thus, therapeutic delays and resulting complications and the number of unnecessary biopsies for diagnosis can be reduced.

The color Doppler twinkling artifact of implants after endoscopic treatment of vesicoureteral reflux in children: A common finding with high potential for misdiagnosis.

BACKGROUND: Endoscopic treatment of vesicoureteral reflux (VUR) is a common therapeutic procedure in children. Over the last years several studies reported on calcified deflux implants that were misinterpreted as ureteral stones leading to unnecessary diagnostic and therapeutic procedures. OBJECTIVE: Based on an own case, where a calcified implant with a strong twinkling artifact was misdiagnosed as a ureteral stone, the purpose of our study was to evaluate the sonographic imaging appearance of implants after endoscopic VUR repair with special emphasis on the color twinkling artifact. MATERIAL AND METHODS: In 40 children (mean age 9.5 years) with 62 treated ureteral units follow-up sonography was performed after a mean time interval of 48.8 months after surgery. The injected deposit was evaluated with B-mode sonography and color Doppler sonography and deposit volume, posterior acoustic shadowing and the appearance and extension of the twinkling artifact were evaluated. RESULTS: 47 of 62 injected units (75.8%) could be identified on follow-up sonography. In 13 of 47 units (27.7%) posterior acoustic shadowing was noted. On color Doppler sonography a twinkling artifact appeared in 26 of the 47 visible cases (55.3%). There was a statistically significant correlation between a positive twinkling sign and the deposit age. CONCLUSION: In conclusion our study shows that the twinkling artifact is a common finding in follow-up sonography of children after endoscopic treatment of VUR. As the twinkling artifact is a sensitive imaging sign for the detection of ureteral calculi the risk of misinterpretation and mistreatment is given.

Fast TWIST with iterative reconstruction improves diagnostic accuracy of AVM of the hand.

Very high temporal and spatial resolution is mandatory for the diagnosis of arteriovenous malformations (AVM) of the hand. Until now, magnetic resonance imaging (MRI) has not fulfilled both requirements simultaneously. This study presents how the combination of a very fast TWIST MRI (time-resolved angiography with interleaved stochastic trajectories) sequence and iterative reconstructions optimizes temporal as well as spatial resolution. 11 patients were examined at a 3-T MRI scanner with two different TWIST protocols: the standard and the study protocol, acquiring a data set every 5.57 s and 1.44 s respectively. The study data was retrospectively iteratively reconstructed with different regularization factors (0.001, 0.002, 0.004, 0.008). Results were compared using the sign-test. P-values < 0.05 were regarded statistically significant. With a low amount of contrast medium, the temporal resolution of the study protocol enabled the differentiation of arteries from veins in all patients whereas the signal-to-noise ratio (SNR) deteriorated. Depending on the regularization factors, SNR, delineation of arterial feeders and non-involved hand and interdigital arteries, as well as artefact levels varied. Overall, iterative reconstruction with regularization factor 0.004 achieved the best results, consequently showing the ability of MRI as a reliable diagnostic method in AVMs of the hand.

Sequential CT arterioportography-arteriosplenography depicts individual haemodynamic changes in children with portal hypertension without cirrhosis.

We evaluated sequential computed tomography (CT) arterioportography-arteriosplenography for the assessment of venous pathways in children with portal hypertension without cirrhosis. Institutional Review Board approval was obtained for this retrospective, single-centre study. CT was performed after contrast application via catheters placed in the superior mesenteric artery (CT arterioportography) and the splenic artery (CT arteriosplenography) consecutively. Venous pathways in 22 children were evaluated. In all patients, the detailed haemodynamic consequences of portal hypertension could be characterised. The supply of varices at different locations could be assigned to the superior mesenteric vein or splenic vein system. Retrograde blood flow through the splenic vein and inferior mesenteric vein, portosystemic shunting, and patency of splanchnic veins were determined. CT arterioportography-arteriosplenography allowed a complete evaluation of individual haemodynamic pathways in children with portal hypertension.

Oskar Kokoschka and Auguste Forel: life imitating art or a stroke of genius?

In the spring of 1910, Oskar Kokoschka painted a portrait of the eminent Swiss psychiatrist, neuroanatomist, temperance champion, and myrmecologist Auguste Forel. The painting is a remarkable psychological portrait but also appears to predict the strokes and right hemiparesis that affected Forel more than a year later. Although it is possible that Kokoschka shared a gift of psychic prediction with his mother and grandmother, a more likely explanation can be ascribed to a combination of the artist's acute perception and the presence of subclinical signs of stroke disease.