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IntroductionVascular malformations are rare congenital anomalies of the vascular system, which can involve the capillaries, veins, arteries, lymphatics, or a combination of vessel types. Patients with vascular malformations experience an impaired health-related quality of life (HRQoL) because of their symptoms (e.g., pain, swelling, and bleeding) and psychosocial distress. Sirolimus is an effective drug used in the medical treatment of these patients; however, relatively little is known about the effect of sirolimus on specific changes in the HRQoL domains and its magnitude.MethodsThe magnitude of change (effect size) following intervention is more informative to clinical practitioners than statistically significant but clinically unimportant changes; therefore, this study aimed to examine the magnitude and meaningfulness of change in the HRQoL of children and adults with vascular malformations following sirolimus treatment using low target levels.ResultsIn total, 50 patients with vascular malformations (19 children, 31 adults) were included in this study. These patients experienced a lower HRQoL than the general population, with the adults reporting a significantly lower score in almost all domains. A 6-month sirolimus treatment improved the HRQoL in 29 patients, including 77.8% of the children (Pediatric Quality of Life Inventory score [PedsQL]) and 57.7% of the adults (Short Form 36 [SF-36]). The effect sizes of sirolimus for each SF-36/PedsQL domain ranged from 0.19 to 1.02. The clinically relevant moderate magnitude of changes was seen in the domains of the children's reports: “Physical functioning” and “Social functioning” and in the domains of the parent reports: “Social functioning,” “School functioning,” and “Psychosocial.” A high-magnitude change was seen in the domains “Emotional functioning” and “Psychosocial” in the children's reports and “Physical functioning” in the parent reports. In addition, the moderate magnitude of changes was also seen in the adults SF-36: in all domains except for “Role limitations—physical problems,” “Role limitations—emotional problems,” and “General health perception.”ConclusionWe believe this is the first study showing the magnitude of change in HRQoL after sirolimus treatment in patients with vascular malformations. Before treatment, these patients experienced an impaired HRQoL compared with the general Dutch population. A 6-month sirolimus treatment with low target levels led to moderate-to-high clinically relevant changes in multiple domains, which significantly improved the HRQoL.Clinical trial registrationhttps://clinicaltrials.gov/ct2/show/NCT03987152?cond=Vascular+Malformations&cntry=NL&city=Nijmegen&draw=2&rank=1, identifier: NCT03987152.

Contains fulltext : 244324.pdf (Publisher’s version ) (Open Access) OBJECTIVES: To investigate whether adrenal volumetry provides better agreement with adrenal vein sampling (AVS) than conventional CT for subtyping PA. Furthermore, we evaluated whether the size of this contralateral adrenal was a prognostic factor for clinical outcome after unilateral adrenalectomy. METHODS: We retrospectively analyzed volumes of both adrenal glands of the 180 CT-scans (88/180 with unilateral and 92/180 with bilateral disease) of the patients with PA included in the SPARTACUS trial of which 85 also had undergone an AVS. In addition, we examined CT-scans of 20 healthy individuals to compare adrenal volumes with published normal values. RESULTS: Adrenal volume was higher for the left than the right adrenal (mean and SD: 6.49 ± 2.77 ml versus 5.25 ± 1.87 ml for the right adrenal; p

Contains fulltext : 252042.pdf (Publisher’s version ) (Closed access) The central lymphatic system consists of the thoracic duct, cisterna chyli and the retroperitoneal lymphatics through which lymph and chyle flows. Disorders of the central lymphatic system can for instance lead to leakage (chylothorax), accumulation of fluid (lymphedema) and retrograde flow (protein losing enteropathy). Abnormalities in the central lymphatic system were overlooked for years, followed by lack of diagnostic and therapeutic options. This has changed, as the technique of intranodal contrast injection in inguinal lymph nodes brought renewed interest in the central lymphatic system. In this article, the importance of intranodal contrast injection in diagnosis and treatment of disorders of the central lymphatic system will be presented through 3 clinical cases.

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Contains fulltext : 220774.pdf (Publisher’s version ) (Open Access) BACKGROUND: Connecting currently existing, heterogeneous rare disease (RD) registries would greatly facilitate epidemiological and clinical research. To increase their interoperability, the European Union developed a set of Common Data Elements (CDEs) for RD registries. OBJECTIVES: To implement the CDEs and the FAIR data principles in the Registry of Vascular Anomalies (VASCA). METHODS: We created a semantic model for the CDE and transformed this into a Resource Description Framework (RDF) template. The electronic case report forms (eCRF) were mapped to the RDF template and published in a FAIR Data Point (FDP). RESULTS: The FAIR VASCA registry was successfully implemented using Castor EDC (Electronic Data Capture) software. CONCLUSION: FAIR technology allows researchers to query and combine data from different registries in real-time.

Item does not contain fulltext Arterio-venous malformations (AVMs) are congenital vascular malformations (CVMs) that result from birth defects involving the vessels of both arterial and venous origins, resulting in direct communications between the different size vessels or a meshwork of primitive reticular networks of dysplastic minute vessels which have failed to mature to become 'capillary' vessels termed "nidus". These lesions are defined by shunting of high velocity, low resistance flow from the arterial vasculature into the venous system in a variety of fistulous conditions. A systematic classification system developed by various groups of experts (Hamburg classification, ISSVA classification, Schobinger classification, angiographic classification of AVMs,) has resulted in a better understanding of the biology and natural history of these lesions and improved management of CVMs and AVMs. The Hamburg classification, based on the embryological differentiation between extratruncular and truncular type of lesions, allows the determination of the potential of progression and recurrence of these lesions. The majority of all AVMs are extra-truncular lesions with persistent proliferative potential, whereas truncular AVM lesions are exceedingly rare. Regardless of the type, AV shunting may ultimately result in significant anatomical, pathophysiological and hemodynamic consequences. Therefore, despite their relative rarity (10-20\% of all CVMs), AVMs remain the most challenging and potentially limb or life-threatening form of vascular anomalies. The initial diagnosis and assessment may be facilitated by non- to minimally invasive investigations such as duplex ultrasound, magnetic resonance imaging (MRI), MR angiography (MRA), computerized tomography (CT) and CT angiography (CTA). Arteriography remains the diagnostic gold standard, and is required for planning subsequent treatment. A multidisciplinary team approach should be utilized to integrate surgical and non-surgical interventions for optimum care. Currently available treatments are associated with significant risk of complications and morbidity. However, an early aggressive approach to elimiate the nidus (if present) may be undertaken if the benefits exceed the risks. Trans-arterial coil embolization or ligation of feeding arteries where the nidus is left intact, are incorrect approaches and may result in proliferation of the lesion. Furthermore, such procedures would prevent future endovascular access to the lesions via the arterial route. Surgically inaccessible, infiltrating, extra-truncular AVMs can be treated with endovascular therapy as an independent modality. Among various embolo-sclerotherapy agents, ethanol sclerotherapy produces the best long term outcomes with minimum recurrence. However, this procedure requires extensive training and sufficient experience to minimize complications and associated morbidity. For the surgically accessible lesions, surgical resection may be the treatment of choice with a chance of optimal control. Preoperative sclerotherapy or embolization may supplement the subsequent surgical excision by reducing the morbidity (e.g. operative bleeding) and defining the lesion borders. Such a combined approach may provide an excellent potential for a curative result. Conclusion. AVMs are high flow congenital vascular malformations that may occur in any part of the body. The clinical presentation depends on the extent and size of the lesion and can range from an asymptomatic birthmark to congestive heart failure. Detailed investigations including duplex ultrasound, MRI/MRA and CT/CTA are required to develop an appropriate treatment plan. Appropriate management is best achieved via a multi-disciplinary approach and interventions should be undertaken by appropriately trained physicians.

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Item does not contain fulltext BACKGROUND: The incidence of hereditary haemorrhagic telangiectasia (HHT - Osler-Weber-Rendu disease) in the Netherlands is 1:5000 but approximately 1:1300 in people from the Antilles. The disease is characterised by the development of telangiectasia and arteriovenous malformations (AVMs) that may result in serious morbidity and mortality. CASE DESCRIPTION: A 31-year-old primigravid patient consulted her general practitioner at 31 1/7 weeks gestational age with dyspnoea. She was referred for further diagnostics because of suspected pulmonary embolism. A CT scan showed haemothorax and a bleeding arteriovenous malformation (AVM) in the left lung. Family history suggested the possibility of HHT. After multidisciplinary consideration, a primary caesarean section was performed, followed by embolisation of the AVM during the same surgical session. The patient had a gene mutation consistent with HHT type 2. CONCLUSION: Pregnant patients with HHT are at risk of serious morbidity, especially if they are not screened for AVMs. A multidisciplinary approach for such patients, with consideration of various scenarios, is highly recommended.

Item does not contain fulltext BACKGROUND: The aim of this paper was to compare the outcomes of endovascular versus surgical treatment in patients with symptomatic proximal subclavian artery obstruction through a retrospective clinical study. Treatment of symptomatic subclavian artery obstruction can be performed with percutaneous transluminal angioplasty or open surgical reconstruction. Comparative studies are scarce. METHODS: Technical success, patency and complication rates of 47 endovascular reconstructions in 46 patients were retrospectively compared with those of 19 open surgical reconstructions in 17 patients performed between 1996 and 2012. An additional series of 51 surgical reconstructions performed in the same institution between 1976 and 1993 served as a reference. RESULTS: The technical success rate was 79% for endovascular and 100% for open surgical reconstructions (P

Item does not contain fulltext Fibromuscular dysplasia (FMD) is a non-inflammatory, non-atherosclerotic vasculopathy that can lead to arterial stenosis, occlusion, aneurysms, and dissection. FMD of the renal arteries can lead to renovascular hypertension. Percutaneous angioplasty of the renal arteries (PTRA) can lead to normalization of blood pressure in 45% of patients with renal artery stenosis caused by FMD, particularly in younger patients and patients with a short history of hypertension. A considerable number of the patients with renovascular FMD also have cervical FMD, which can lead to ischaemic or haemorrhagic stroke. In this article we discuss diagnostic and therapeutic options, illustrated by two cases of patients with renovascular and carotid FMD. Most of the recommendations are based on data from retrospective studies and expert opinion; prospective studies on the optimal diagnostic strategy and treatment are therefore, urgently required.

Contains fulltext : 59022.pdf (Publisher’s version ) (Closed access) A 4-year-old boy was hit by a car travelling at 40 km/h and was admitted 3.5 h later to the department of paediatric surgery. Because he was haemodynamically unstable and needed blood transfusion, the patient underwent an emergency operation. The liver was ruptured in the right lobe. A large haematoma was found in the serosa of the duodenum, along with a Meckel's diverticle, which was left in place. The liver rupture was covered and sealed. One month after the accident the patient was re-admitted, because of abdominal pain and gastrointestinal bleeding. The cause was thought to be the Meckel's diverticle, which was removed later. Two months after the trauma the patient was re-admitted with abdominal pain, again with haematemesis and melaena. The diagnosis of hemobilia was obtained with MRI and angiography, which revealed a ruptured pseudoaneurysm of the ramus dexter of the proper hepatic artery. The patient was successfully treated with embolization. The diagnostic delay was two months, which illustrates the importance of considering the possibility of the diagnosis hemobilia in case of gastrointestinal haemorrhage combined with biliary symptoms.

Item does not contain fulltext BACKGROUND: Atherosclerotic renal artery stenosis (ARAS) is associated with progressive loss of renal function and is one of the most important causes of renal failure in the elderly. Current treatment includes restoration of the renal arterial lumen by endovascular stent placement. However, this treatment only affects damage caused by ARAS due to the stenosis and ensuing post-stenotic ischemia. ARAS patients have severe general vascular disease. Atherosclerosis and hypertension can also damage the kidney parenchyma causing renal failure. Medical treatment focuses on the latter. Lipid-lowering drugs (statins) could reduce renal failure progression and could reduce the overall high cardiovascular risk. The additional effect on preserving renal function of stent placement as compared to medical therapy alone is unknown. Therefore, the STAR-study aims to compare the effects of renal artery stent placement together with medication vs. medication alone on renal function in ARAS patients. METHOD: Patients with an ARAS of > or = 50% and renal failure (creatinine (Cr) clearance < 80 mL/min/1.73 m2) are randomly assigned to stent placement with medication or to medication alone. Medication consists of statins, anti-hypertensive drugs and antiplatelet therapy. Patients are followed for 2 yrs with extended follow-up to 5 yrs. The primary outcome of this study is a reduction in Cr clearance > 20% compared to baseline. This trial will include 140 patients.

Item does not contain fulltext INTRODUCTION: In a deep inferior epigastric perforator (DIEP) flap breast reconstruction, computed tomography angiography (CTA) is currently considered as the gold standard in preoperative imaging for this procedure. Unidirectional Doppler ultrasound (US) is frequently used; however, this method does not distinguish the main axial vessels from perforator arteries at the height of the fascia, it has a limited penetration depth, and it cannot assess the branching patterns of the deep inferior arteries. A new method and system were developed, which consisted of a video projector preoperatively displaying the location and intramuscular course of the artery perforators and subcutaneous branching on the patient's abdomen. METHOD: All patients (n = 9) underwent a standard protocol: a preoperative CTA was performed and the DIEPs were localized using a unidirectional Doppler probe. In addition, a three-dimensional (3D) reconstruction of the perforator locations based on CTA was projected on the abdomen of the patients. All projected perforator locations were assessed using a unidirectional Doppler probe. The intraoperative results were collected for comparison. RESULTS: A total of 88 locations were marked with the use of unidirectional Doppler and a total of 100 perforators were projected (p = 0.38). In 98 out of 100 projected perforator locations, a Doppler signal was audible. The intraoperative results demonstrate that 19 out of 34 transplanted perforators were correctly identified with unidirectional Doppler (56.9% +/- 31.4%), where the projection method properly revealed 29 locations (84.3% +/- 25.8%) (p = 0.030). CONCLUSION: The projection method is not only capable of providing more information and identifying more perforators used for transplantation than unidirectional Doppler probing but also more accurate in pointing out the corresponding perforator found intraoperatively.

Contains fulltext : 88142.pdf (Publisher’s version ) (Open Access) Cervical near-hanging injuries are most frequent in the young adult population. However, the literature gives little guidance regarding diagnostic evaluation of these patients, although it is well known that their initial clinical presentation has limited prognostic value. This case report presents a patient who actually survived a suicidal near-hanging attempt and was later able to walk and talk to his physician. He died the next day due to carotid dissection and cerebral ischemia. In this case report we emphasize the importance of thorough radiological investigation in the accurate assessment of these patients. Early imaging should be performed routinely after near-hanging injury, in order to establish the correct diagnosis and allow appropriate treatment to be started.

Item does not contain fulltext We present the case of a patient with a persisting type II endoleak after endovascular repair of an iliac aneurysm with an iliaco-caval fistula. We describe the pathophysiological mechanism behind this phenomenon and discuss why conservative treatment is unlikely to seal this type of endoleak. A more aggressive treatment strategy is therefore advocated.

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For the purpose of obtaining a geometrical model of the wire frame of stent grafts, we propose three tracking methods to segment the stent's wire, and compare them in an experiment. A 2D test image was created by obtaining a projection of a 3D volume containing a stent. The image was modified to connect the parts of the stent's frame and thus create a single path. Ten versions of this image were obtained by adding different noise realizations. Each algorithm was started at the start of each of the ten images, after which the traveled paths were compared to the known correct path to determine the performance. Additionally, the algorithms were applied to 3D clinical data and visually inspected. The method based on the minimum cost path algorithm scored excellent in the experiment and showed good results on the 3D data. Future research will focus on establishing a geometrical model by determining the corner points and the crossings from the results of this method.

Purpose: ECG-gated CTA allows visualization of the aneurysm and stentgraft during the different phases of the cardiac cycle, although with a lower SNR per cardiac phase than without ECG gating using the same dose. In our institution, abdominal aortic aneurysm (AAA) is evaluated using non-ECG-gated CTA. Some common CT scanners cannot reconstruct a non-gated volume from ECG-gated acquired data. In order to obtain the same diagnostic image quality, we propose off-line temporal averaging of the ECG-gated data. This process, though straightforward, is fundamentally different from taking a non-gated scan, and its result will certainly differ as well. The purpose of this study is to quantitatively investigate how good off-line averaging approximates a non-gated scan. Method: Non-gated and ECG-gated CT scans have been performed on a phantom (Catphan 500). Afterwards the phases of the ECG-gated CTA data were averaged to create a third dataset. The three sets are compared with respect to noise prop-erties (NPS) and frequency response (MTF). To study motion artifacts identical scans were acquired on a programmable dynamic phantom. Results and Conclusions: The experiments show that the spatial frequency content is not affected by the averaging process. The minor differences observed for the noise properties and motion artifacts are in favor of the averaged data. Therefore the averaged ECG-gated phases can be used for diagnosis. This enables the use of ECG-gating for research on stentgrafts in AAA, without impairing clinical patient care.

Contains fulltext : 80204.pdf (Publisher’s version ) (Closed access) PURPOSE: ECG-gated CT enables the visualization of motions caused by the beating of the heart. Although ECG gating is frequently used in cardiac CT imaging, this technique is also very promising for evaluating vessel wall motion of the aortic artery and the motions of (stent grafts inside) abdominal aortic aneurysms (AAA). Late stent graft failure is a serious complication in endovascular repair of aortic aneurysms. Better understanding of the motion characteristics of stent grafts will be beneficial for designing future devices. In addition, these data can be valuable in predicting stent graft failure in patients. To be able to reliably quantify the motion, however, it is of importance to know the performance and limitations of ECG gating, especially when the motions are small, as is the case in AAA. Since the details of the reconstruction algorithms are proprietary information on the CT manufacturers and not in the public domain, empirical experiments are required. The goal of this study is to investigate as to what extent the motions in AAA can be measured using ECG-gated CT. The authors quantitatively investigate four aspects of motion in ECG-gated CT: The detectability of the motion of objects at different amplitudes and different periodic motions, the temporal resolution, and the volume gaps that occur as a function of heart rate. METHODS: They designed an experiment on a standard static phantom to empirically determine temporal resolution. To investigate motion amplitude and frequency, as well as patient heart rate, they designed dynamic experiments in which a home-made phantom driven by a motion unit moves in a predetermined pattern. RESULTS: The duration of each ECG-gated phase was found to be 185 ms, which corresponds to half of the rotation time and is thus in accordance with half scan reconstruction applied by the scanner. By using subpixel localization, motions become detectable from amplitudes of as small as 0.4 mm in the x direction and 0.7 mm in the z direction. With the rotation time used in this study, motions up to 2.7 Hz can be reliably detected. The reconstruction algorithm fills volume gaps with noisy data using interpolation, but objects within these gaps remain hidden. CONCLUSIONS: This study gives insight into the possibilities and limitations for measuring small motions using ECG-gated CT. Application of the experimental method is not restricted to the CT scanner of a single manufacturer. From the results, they conclude that ECG-gated CTA is a suitable technique for studying the expected motions of the stent graft and vessel wall in AAA.

Endovascular aortic replacement (EVAR) is an established technique, which uses stentgrafts to treat aortic aneurysms in patients at risk of aneurysm rupture. The long-term durability of a stentgraft is affected by the stresses and hemodynamic forces applied to it, and may be reflected by the movements of the stentgraft itself during the cardiac cycle. A conventional CT scan (which results in a 3D volume) is not able to visualize these movements. However, applying ECG-gating does provide insight in the motion of the stentgraft caused by hemodynamic forces at different phases of the cardiac cycle. The amount of data obtained is a factor of ten larger compared to conventional CT, but the radiation dose is kept similar for patient safety. This causes the data to be noisy, and streak artifacts are more common. Algorithms for automatic stentgraft detection must be able to cope with this. Segmentation of the stentgraft is performed by examining slices perpendicular to the centreline. Regions with high CT-values exist at the locations where the metallic frame penetrates the slice. These regions are well suited for detection and sub-pixel localization. Spurious points can be removed by means of a clustering algorithm, leaving only points on the contour of the stent. We compare the performance of several different point detection methods and clustering algorithms. The position of the stent’s centreline is calculated by fitting a circle through these points. The proposed method can detect several stentgraft types, and is robust against noise and streak artifacts.

Contains fulltext : 69176.pdf (Publisher’s version ) (Closed access) Two adult patients with presumed primary hypertension are presented. In the first patient the diagnosis of coarctation of the aorta was straightforward while in the second patient there was a substantial delay in reaching the correct diagnosis. A 32-year-old patient was analysed for hypertension in the outpatient clinic. At physical examination a systolic cardiac murmur was present and leg blood pressure was not measurable. Magnetic resonance imaging angiography showed a severe coarctation of the thoracic aorta with extensive distended collateral blood vessels. A second patient was a 31-year-old man referred with longstanding hypertension and an unsatisfactory blood pressure response to treatment. Previously, a diagnosis of primary hypertension was made. Renal computed tomography angiography excluded renal artery stenosis as a cause of hypertension but disclosed many distended collateral blood vessels in the musculus rectus abdominis and in the upper abdominal area. Leg blood pressure was measured and further analysis revealed a coarctation of the aorta. Both patients illustrate and emphasise the importance of leg blood pressure measurement at a first analysis of adult hypertensive patients and should always be performed when hypertension is accompanied by murmurs or weak femoral pulsations.

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Contains fulltext : 52147.pdf (Publisher’s version ) (Closed access) In 3 patients, men aged 60, 55 and 60, respectively, with hypertension due to primary hyperaldosteronism, the aldosterone level in the adrenal veins was determined for the purpose of further diagnosis. In two patients, unilateral adrenal enlargement on the CT-scan was accompanied by overproduction ofaldosterone, in one case in a non-enlarged adrenal gland and in the other case in both adrenals. The first patient underwent adrenalectomy of the non-enlarged adrenal gland, while in the second patient surgery was decided against. The third patient had bilateral adrenal gland enlargement on the CT-scan with a surgically treatable, unilateral overproduction ofaldosterone. Now that determination ofthe aldosterone:renin ratio in plasma as a screening method in selected patients with hypertension is being used more often, primary hyperaldosteronism turns out to be more common than was previously thought. For differentiation between unilateral and bilateral overproduction of aldosterone, imaging of the adrenals, for example with CT, is insufficiently accurate. Aldosterone determination in the adrenal veins can distinguish between unilateral and bilateral overproduction of aldosterone with great accuracy, which has important therapeutic consequences.

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Contains fulltext : 209717.pdf (Publisher’s version ) (Open Access) Radboud University, 26 november 2019 Promotores : Lenders, J.W.M., Wilt, G.J. van der, Schultze Kool, L.J. Co-promotor : Deinum, J.

Contains fulltext : 209726.pdf (Publisher’s version ) (Open Access) Radboud University, 27 november 2019 Promotores : Schultze Kool, L.J., Heuvel, L.P.W.J. van den Co-promotores : Daamen, W.F., Hoogeveen, Y.L.

Contains fulltext : 187344.pdf (Publisher’s version ) (Open Access) Radboud University, 06 april 2018 Promotores : Ulrich, D.J.O., Schultze Kool, L.J. Co-promotor : Hoogeveen, Y.L.

Contains fulltext : 195512.pdf (Publisher’s version ) (Open Access) Radboud University, 09 oktober 2018 Promotores : Kerkhof, P.C.M. van de, Schultze Kool, L.J. Co-promotores : Vleuten, C.J.M. van der, Spillekom-van Koulil, S. 145 p.

Contains fulltext : 178236.pdf (Publisher’s version ) (Open Access) Radboud University, 10 november 2017 Promotor : Schultze Kool, L.J. Co-promotores : Hoogeveen, Y.L., Lange, F. de

Contains fulltext : 118652.pdf (Publisher’s version ) (Open Access) Radboud Universiteit Nijmegen, 19 november 2013 Promotores : Schultze Kool, L.J., Kerkhof, P.C.M. van de Co-promotores : Vleuten, C.J.M. van der, Beijnum, I.M. van

Contains fulltext : 74421.pdf (Publisher’s version ) (Open Access) RU Radboud Universiteit Nijmegen, 04 juni 2009 Promotores : Blankensteijn, J.D., Schultze Kool, L.J., Oyen, W.J.G. Co-promotor : Kurvers, H.A.J.M. 155 p.

Contains fulltext : 81524.pdf (Publisher’s version ) (Open Access) Contains fulltext : 81524_samenvatting.pdf (Publisher’s version ) (Open Access) RU Radboud Universiteit Nijmegen, 30 september 2009 Promotores : Vugt, A.B. van, Schultze Kool, L.J. Co-promotor : Edwards, M.J.R. 199 p.

Contains fulltext : 81390.pdf (Publisher’s version ) (Open Access) RU Radboud Universiteit Nijmegen, 16 september 2009 Promotores : Schultze Kool, L.J., Vugt, A.B. van Co-promotor : Edwards, M.J.R. 184 p.