Purpose
To evaluate the safety and efficacy of percutaneous interventional treatment of portal vein stenosis in children.
Material and methods
A retrospective analysis of all interventional ...treatments for portal vein stenosis in pediatric patients at a single institution from 2010 to 2021 was conducted. Platelet count, spleen size and portal vein flow velocity were assessed during the follow-up period. Primary and primary assisted patency time were determined.
Results
A total of ten children (median age 28.5 months, interquartile range (IQR): 2.75–52.5 months) with portal vein stenosis after Mesorex-Shunt (
n
= 4), liver transplantation (
n
= 3) and other etiologies (
n
= 3) underwent 15 interventional procedures. There were five reinterventions and one discontinued intervention. The technical success rate was 93.3% (14/15) and clinical success of treated patients was 100% (14/14). Median follow-up was 18 months (IQR: 13.5–81 months). The median primary patency time for stent placement was 70 months (IQR: 13.5–127.25 months). For balloon angioplasty, the median primary patency time was 9 months (IQR 7.25–11.5 months), while the median assisted primary patency time was 14 months (IQR: 12 to 15 months). Platelet count, spleen size and portal vein flow velocity reliably corresponded to recurrence of portal vein stenosis in asymptomatic patients during follow-up.
Conclusion
Interventional treatment is a safe and efficient method to treat portal vein stenosis with long patency times, regardless of etiology. Primary stent placement shows a higher primary patency time than balloon angioplasty. Implementation of stent placement as the primary interventional method may improve patency times and reduce the need for repeat reinterventions in pediatric patients.
Solid tumors of the cervicothoracic junction, the posterior mediastinum, or bilateral dorsal thoracic tumors represent a challenge in pediatric surgical oncology. The aim of this study was to ...evaluate trap-door thoracotomy and clamshell thoracotomy as surgical approaches. A single-center retrospective study of children with solid tumors in these specific localizations was performed. From 2015 to 2023, 26 children (17 girls; 9 boys) were treated at a median age of 54 months (range 8-229). Tumor resection was performed for neuroblastoma (
= 11); metastatic disease (
= 7); malignant rhabdoid tumor (
= 4); Ewing sarcoma (
= 1); inflammatory myofibroblastic tumor (
= 1); rhabdomyosarcoma (
= 1); and neurofibroma (
= 1). The surgical goal of macroscopic complete excision was achieved in all of the 14 children who underwent trap-door thoracotomy and in 11 of the 12 children who underwent clamshell thoracotomy. There were no major complications. At a median follow-up of 8 months (range 0-60), the disease was under local control or in complete remission in 66.7% of the children. In conclusion, surgical resection of solid tumors of the cervicothoracic junction in children can be performed safely and successfully with trap-door thoracotomy and with clamshell thoracotomy for posterior mediastinal or bilateral dorsal thoracic tumors.
ZusammenfassungHintergrundIn den vergangenen Jahrzehnten hat sich die Ganzkörper-Magnetresonanztomographie (GK-MRT) für die Diagnose, das Staging und die Nachsorge onkologischer Erkrankungen sowie ...zum Screening bei Tumorprädispositionssyndromen (TPS), wie dem Li-Fraumeni-Syndrom, etabliert. Als umfassende Bildgebungsmethode ohne ionisierende Strahlung kann die GK-MRT beliebig häufig wiederholt eingesetzt werden und bietet aufgrund des ausgezeichneten Weichteilkontrasts und der hohen Auflösung eine frühzeitige, präzise Erkennung von Pathologien.MethodenAuf die technischen Voraussetzungen, einige Untersuchungsstrategien sowie die klinische Bedeutung typischer Befunde der GK-MRT-Bildgebung bei Patient:innen mit TPS wird in diesem Beitrag eingegangen.
Background
Functional magnetic resonance (MR) urography has been well established in the diagnostic workup of congenital anomalies of kidneys and urinary tract, though long acquisition time requires ...sedation or general anesthesia in infants.
Objective
To evaluate the success rate of an optimized functional MR urography protocol in infants carried out in natural sleep.
Materials and methods
We retrospectively evaluated all functional MR urographies performed under general anesthesia or during natural sleep in infants younger than 1 year between 2010 and 2017 and rated image quality in both cohorts using a 3-point Likert scale. We tested the analyzability of functional sequences using a free available software. We also calculated examination time. Finally, we compared examinations in natural sleep and those with general anesthesia using independent
t
-test for continuous data and Mann–Whitney
U
test for categorical data.
Results
Functional MR urography could be performed successfully during natural sleep in 38 of 42 (90%) infants younger than 10 months. Four examinations were aborted before contrast medium was administrated. In the same period, 19 functional MR urographies were performed successfully under general anesthesia. Although image quality was significantly better in this group (
P
<0.0001), image quality was at least diagnostic in all finished examinations in natural sleep, and the functional analyzability was given in all completed examinations. There was a significant saving in examination time during natural sleep (
P
<0.001).
Conclusion
Functional MR urography can be successfully performed in natural sleep in infants younger than 10 months.
The purpose of this recommendation of the Oncology Task Force of the European Society of Paediatric Radiology (ESPR) is to indicate reasonable applications of whole-body MRI in children with cancer ...and to address useful protocols to optimize workflow and diagnostic performance. Whole-body MRI as a radiation-free modality has been increasingly performed over the last two decades, and newer applications, as in screening of children with germ-line mutation cancer-related gene defects, are now widely accepted. We aim to provide a comprehensive outline of the diagnostic value for use in daily practice. Based on the results of our task force session in 2018 and the revision in 2019 during the ESPR meeting, we summarized our group’s experiences in whole-body MRI. The lack of large evidence by clinical studies is challenging when focusing on a balanced view regarding the impact of whole-body MRI in pediatric oncology. Therefore, the final version of this recommendation was supported by the members of Oncology Task Force.
Purpose
ESIN (elastic stable intramedullary nailing) is considered the gold standard for various pediatric fractures. The aim of this study was to analyze the incidence and type of complications ...during or after TEN (titanium elastic nail) removal.
Methods
A retrospective data analysis was performed. Metal removal associated complications and preoperative extraosseous length/outlet angle of TENs as possible causes of complications were assessed.
Results
The complication rate in 384 TEN removals was 3.1% (
n
= 12). One major complication (rupture of M. extensor pollicis brevis) was documented. One refracture at the forearm occurred, however, remodeling prior TEN removal was completed. Ten minor complications were temporary or without irreversible restrictions (3 infections, 5 scaring/granuloma, 2 temporary paraesthesia).
In 38 cases (16 forearms, 10 femora, 9 humeri, 3 lower legs), intra-operative fluoroscopy had to be used to locate the implants. In patients with forearm fractures, extraosseous implant length was relatively shorter than in cases without fluoroscopy (
p
= 0.01), but outlet angle of TENs was not significantly different in these two groups (28.5° vs 25.6°). In patients with femur fractures, extraosseous implant length and outlet angle were tendentially shorter, respectively, lower, but this did not reach statistical significance.
Conclusion
Removal of TENs after ESIN is a safe procedure with a low complication rate. Technically inaccurate TEN implantation makes removal more difficult and complicated. To prevent an untimely removal and patient discomfort, nail ends must be exactly positioned and cut. Intraoperative complications may be minimized with removal of TENs before signs of overgrowth.
Evidence
Level III, retrospective.
(1) Background: The study aimed to investigate the influence of MRI-defined residual disease on local tumor control after resection of neuroblastic tumors in patients without routine adjuvant ...radiotherapy. (2) Methods: Patients, who underwent tumor resection between 2009 and 2019 and received a pre- and postoperative MRI, were included in this retrospective single-center study. Measurement of residual disease (RD) was performed using standardized criteria. Primary endpoint was the local or combined (local and metastatic) event free survival (EFS). (3) Results: Forty-one patients (20 female) with median age of 39 months were analyzed. Risk group analysis showed eleven low-, eight intermediate-, and twenty-two high-risk patients (LR, IR, HR). RD was found in 16 cases by MRI. A local or combined relapse or progression was found in nine patients of whom eight patients had RD (
= 0.0004). From the six patients with local or combined relapse in the HR group, five had RD (
= 0.005). Only one of 25 patients without RD had a local event. Mean EFS (month) was significantly higher if MRI showed no residual tumor (81 ± 5 vs. 43 ± 9;
= 0.0014) for the total cohort and the HR subgroup (62 ± 7 vs. 31 ± 11;
= 0.016). (4) Conclusions: In our series, evidence of residual tumor, detectable by MRI, was associated with insufficient local control, resulting in relapses or local progression in 50% of patients. Only one of the patients without residual tumor had a local relapse.
Abstract
Background
Coronary artery magnetic resonance angiography (CMRA) is a robust tool for delineation of the coronary artery anatomy. In order to overcome the issue of visual delineation of the ...quietest heart phase, we implemented a new three-dimensional (3D) multi-phase whole-heart CMRA.
Purpose
To evaluate the 3D multi-phase whole-heart CMRA technique for depiction of the coronary arteries and simultaneous assessment of cardiac function.
Material and Methods
Ten healthy volunteers were included in a prospective study comprising 3D whole-heart CMRA with multiple-phase acquisition on 1.5-T MR. As a reference for coronary artery imaging, single-phase CMRA was performed. Furthermore, two-dimensional SSFP imaging was performed for functional assessment. Vessel sharpness (VS) was rated on a 5-point Likert scale (5 = best) and ventricular volumes were assessed in respective sequences.
Results
VS scores were rated higher in multi-phase compared to single-phase CMRA: mean VS of the RCA was 4.6 ± 0.52 in multi-phase and 3.6 ± 1.17 in single-phase CMRA (z = –2.232, P = 0.031). Mean VS of the LM was 4.2 ± 0.79 vs. 3.7 ± 0.82 (z = –2.236, P = 0.063), of the LCX was 3.8 ± 1.03 vs. 3.4 ± 1.01 (z = –1.265, P = 0.359), and of the LAD was 3.9 ± 0.59 vs. 3.9 ± 0.32 (z = 0, P = 1.00). In functional assessment, the mean difference between multi-phase CMRA and two-dimensional SSFP imaging for assessing the end-diastolic volume of the left ventricle (LV-EDV) was –0.38% (95% CI -2.41–1.64) and for the right ventricular EDV –0.28 (95% CI -3.51–2.96).
Conclusion
The 3D multi-phase CMRA approach allows a time efficient depiction of the coronary arteries and a coeval functional assessment, with an improved vessel sharpness of the coronaries.
Opsoclonus-myoclonus syndrome (OMS) is a rare clinical disorder and typically occurs in association with occult neuroblastic tumor in pediatric patients. I-123 metaiodobenzylguanidine (mIBG) ...scintigraphy is widely adopted as screening procedure in patients with suspected neuroblastic tumor. Also, contrast-enhanced magnetic resonance imaging (MRI) or computed tomography (CT) are involved in the imaging workup, primarily for the assessment of the primary tumor region. However, the diagnostic value of whole-body MRI (WB-MRI) for the detection of occult neuroblastic tumor in pediatric patients presenting with OMS remains unknown.
We present three cases of patients with OMS, in whom WB-MRI revealed occult neuroblastic tumor masses, whereas scintigraphy was inconclusive: In a 17 months old girl with OMS, WB-MRI revealed a paravertebral mass. After thoracoscopic resection, histopathology revealed a ganglioneuroblastoma. A 13 months old boy presenting with OMS WB-MRI detected a tumor of the left adrenal gland; histopathology demonstrated a ganglioneuroblastoma after adrenalectomy. In a 2 year old boy with OMS, immunoscintigraphy at the time of diagnosis was inconclusive. At the age of 13 years, a WB-MRI was performed due to persistent neurological symptoms, revealing a paravertebral retroperitoneal mass, which was classified as ganglioneuroblastoma.
In OMS, particularly in the setting of inconclusive scintigraphy, WB-MRI may be considered as a valuable alternative in the early phase of diagnostic work-up.
Anomalous aortic origin of the left coronary artery (AAOLCA) from the right aortic sinus is a rare congenital anomaly associated with significantly increased risk of myocardial ischemia, arrhythmias ...and sudden cardiac death. This refers specifically to AAOLCA associated with interarterial and/or intramural course. Much less is known about anomalous origin of the left coronary artery from the right aortic sinus associated with a subpulmonic intramyocardial course.
We report a 12 year old girl who complained of recurrent episodes of chest pain lasting for some minutes and not associated to exercise. Echocardiography revealed AAOLCA from the right aortic sinus taking a subpulmonic course within the conal septum. The diagnosis was confirmed by CT-scan and selective coronary angiography. Treadmill test, Holter-ECG and bicycle-stress echocardiography revealed no evidence of myocardial ischemia reflecting unimpaired diastolic flow in the left coronary artery. According to the nature of the complaints and in the absence of signs of myocardial ischemia the episodes of chest pain were classified as idiopathic and not associated to the coronary anomaly. We opted for a conservative approach with regular follow-up visits. During a follow-up of 2 years without restriction of sports activities the patient has been asymptomatic.
According to the literature AAOLCA with subpulmonary intramyocardial course appears to be associated with significantly less clinical problems than AAOLCA taking an interarterial course. The diagnosis can be suspected based on echocardiography and confirmed by contrast-enhanced computed tomography. Until now there are only few data concerning the natural history and incidence of complications in this specific anomaly. Despite the probably benign nature we recommend regular follow-up examinations including stress-testing in these patients.
PDF
