Health-related quality of life in children with congenital vascular malformations.

A cross-sectional study was performed to evaluate health-related quality of life (HRQOL) in children with congenital vascular malformations (CVM) and to investigate factors associated with an impaired HRQOL. Children (2-17 years) with CVMs who visited the HECOVAN expertise center between 2016-2018 were included. The PedsQL 4.0 Generic Core Scales were used and a score ≥ 1.0 SD below the normative mean was defined as an impaired HRQOL. Factors associated with impairment were investigated using univariate and multivariate logistic regression analysis. The median overall HRQOL was 84.8/100 (n = 207; 41% boys, 59% girls; self-reported IQR 73.9-92.4 and parent-reported IQR 71.4-92.4). Patients aged 13-17 years reported significantly worse physical functioning than those aged 8-12 years (median 84.4, IQR 71.1-93.8 versus median 90.6, IQR 81.3-96.9; p = 0.02). Parents reported a significantly lower overall HRQOL than their children (median 80.4, IQR 70.7-90.8 versus median 85.9, IQR 76.1-92.4; p = 0.001). HRQOL was impaired in 25% of patients. Impairment occurred significantly more often in lower extremity CVMs (38%, p = 0.01) and multifocal CVMs (47%, p = 0.01) compared to CVMs in the head/neck region (13%). Other associated factors included invasive management (31% versus 14%; p = 0.01), age at first treatment ≤ 5 years (48% versus 25%; p = 0.02) and ongoing treatment (38% versus 18%; p = 0.004). After correction for other factors, significance remained for lower extremity CVMs and ongoing invasive treatment. CONCLUSIONS: Overall median HRQOL was reasonable and not significantly different from the norm sample. Parental ratings were significantly lower than their children's ratings. A quarter of the patients had an impaired HRQOL, which seemed to worsen with age. Independently associated factors included a lower extremity CVM and invasive management. WHAT IS KNOWN: • Congenital vascular malformations could affect health-related quality of life (HRQOL). • Studies on pediatric patients are limited and either very small or in combination with adult patient series. WHAT IS NEW: • This study raises awareness of an impaired HRQOL in 25% of pediatric patients with congenital vascular malformations. • Associated factors included a lower extremity CVM and invasive management.

Predicting surgical outcome in posterior retroperitoneoscopic adrenalectomy with the aid of a preoperative nomogram.

BACKGROUND: Posterior retroperitoneoscopic adrenalectomy (PRA) has several advantages over transperitoneal laparoscopic adrenalectomy (TLA) regarding operative time, blood loss, postoperative pain, and recovery. However, it can be a technically challenging procedure. To improve patient selection for PRA, we developed a preoperative nomogram to predict operative time. METHODS: All consecutive patients with tumors of ≤ 7 cm and a body mass index (BMI) of < 35 kg/m2 undergoing unilateral PRA between February 2011 and March 2020 were included in the study. The primary outcome was operative time as surrogate endpoint for surgical complexity. Using ten patient variables, an optimal prediction model was created, with a best subsets regression analysis to find the best one-variable up to the best seven-variable model. RESULTS: In total 215 patients were included, with a mean age of 52 years and mean tumor size of 2.4 cm. After best subsets regression analysis, a four-variable nomogram was selected and calibrated. This model included sex, pheochromocytoma, BMI, and perinephric fat, which were all individually significant predictors. This model showed an ideal balance between predictive power and applicability, with an R2 of 38.6. CONCLUSIONS: A four-variable nomogram was developed to predict operative time in PRA, which can aid the surgeon to preoperatively identify suitable patients for PRA. If the nomogram predicts longer operative time and therefore a more complex operation, TLA should be considered as an alternative approach since it provides a larger working space. Also, the nomogram can be used for training purposes to select patients with favorable characteristics when learning this surgical approach.

Predictors of treatment response following aspiration sclerotherapy of hepatic cysts: an international pooled analysis of individual patient data.

OBJECTIVES: To identify predictive variables of treatment response following aspiration sclerotherapy of large symptomatic hepatic cysts. METHODS: We collected individual patient data from two tertiary referral centres and included all patients treated with aspiration sclerotherapy of a large (>5 cm), symptomatic hepatic cyst. At six months, clinical response was defined as complete or incomplete. Secondary, suboptimal technical response was defined as lower quartile of cyst reduction. Predictive variables of clinical and technical response were analyzed by logistic regression analysis. RESULTS: We included 86 patients (58 ± 10 years; female 90 %). Complete clinical response rate was 55 %. Median cyst diameter and volume reduction were 71 % (IQR 50-87 %) and 98 % (IRQ 88-100 %), respectively. Patients with complete clinical response had a significantly higher cyst reduction compared to incomplete responders (OR 1.02, 95 % CI 1.00-1.04). Aspiration of haemorrhagic cyst fluid (OR 4.39, 95 % CI 1.34-14.39) or a lower cyst reduction at one month (OR 1.06, 95 % CI 1.02-1.10) was associated with a suboptimal technical response at six months. CONCLUSION: Complete clinical response is associated with effective cyst reduction. Aspiration of haemorrhagic cyst fluid or a restricted diameter reduction at one month predicts a suboptimal technical treatment response, however, these variables did not predict symptom disappearance. KEY POINTS: • Aspiration sclerotherapy of hepatic cysts shows excellent clinical and technical efficacy. • Optimal clinical responders have a markedly higher cyst reduction. • Haemorrhagic aspirate and a strong fluid reaccumulation predict suboptimal cyst reduction.

Detectability of motions in AAA with ECG-gated CTA: a quantitative study.

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.

Impact of dynamic computed tomographic angiography on endograft sizing for endovascular aneurysm repair.

PURPOSE: To quantify dynamic changes in aortoiliac dimensions using dynamic electrocardiographically (ECG)-gated computed tomographic angiography (CTA) and to investigate any potential impact on preoperative endograft sizing in relation to observer variability. METHODS: Dynamic ECG-gated CTA was performed in 18 patients with abdominal aortic aneurysms. Postprocessing resulted in 11 datasets per patient: 1 static CTA and 10 dynamic CTA series. Vessel diameter, length, and angulation were measured for all phases of the cardiac cycle. The differences between diastolic and systolic aneurysm dimensions were analyzed for significance using paired t tests. To assess intraobserver variability, 20 randomly selected datasets were analyzed twice. Intraobserver repeatability coefficients (RC) were calculated using Bland-Altman analysis. RESULTS: Mean aortic diameter at the proximal neck was 21.4+/-3.0 mm at diastole and 23.2+/-2.9 mm at systole, a mean increase of 1.8+/-0.4 mm (8.5%, p<0.01). The RC for the aortic diameter at the level of the proximal aneurysm neck was 1.9 mm (8.9%). At the distal sealing zones, the mean increase in diameter was 1.7+/-0.3 mm (14.1%, p<0.01) for the right and 1.8+/-0.5 mm (14.2%, p<0.01) for the left common iliac artery (CIA). At both distal sealing zones, the mean increase in CIA diameter exceeded the RC (10.0% for the right CIA and 12.6% for the left CIA). CONCLUSION: The observed changes in aneurysm dimension during the cardiac cycle are small and in the range of intraobserver variability, so dynamic changes in proximal aneurysm neck diameter and aneurysm length likely have little impact on preoperative endograft selection. However, changes in diameter at the distal sealing zones may be relevant to sizing, so distal oversizing of up to 20% should be considered to prevent distal type I endoleak.

Continuously Grooved Stent Struts for Enhanced Endothelial Cell Seeding.

PURPOSE: Implantation of pre-endothelialized stents could enhance cellular recovery of a damaged vessel wall provided attached cells remain viable, functional and are present in sufficient numbers after deployment. The purpose of this study was to evaluate the feasibility of grooved stainless steel (SS) stents as a primary endothelial cell (EC) carrier with potentially enhanced EC protection upon stent deployment. MATERIALS AND METHODS: Attachment and behavior of enzymatically harvested human adult venous ECs seeded onto gelatin-coated vascular stents were evaluated in an in vitro setting. Smooth and grooved SS stents and smooth nitinol stents were studied. RESULTS: All cells expressed EC markers vWF and CD31. Using rotational seeding for a period of 16-24 h, ECs attached firmly to the stents with sufficient coverage to form a confluent EC monolayer. The grooved SS wire design was found to enable attachment of three times the number of cells compared to smooth wires. This also resulted in an increased number of cells remaining on the stent after deployment and after pulsatile flow of 180 ml/min for 24 h, which did not result in additional EC detachment. CONCLUSIONS: The grooved stent provides a potential percutaneous means to deliver sufficient numbers of viable and functional cells to a vessel segment during vascular intervention. The grooves were found to offer a favorable surface for EC attachment and protection during stent deployment in an in vitro setting.

Adrenal vein sampling versus CT scan to determine treatment in primary aldosteronism: an outcome-based randomised diagnostic trial.

BACKGROUND: The distinction between unilateral aldosterone-producing adenoma or bilateral adrenal hyperplasia as causes of primary aldosteronism is usually made by adrenal CT or by adrenal vein sampling (AVS). Whether CT or AVS represents the best test for diagnosis remains unknown. We aimed to compare the outcome of CT-based management with AVS-based management for patients with primary aldosteronism. METHODS: In a randomised controlled trial, we randomly assigned patients with aldosteronism to undergo either adrenal CT or AVS to determine the presence of aldosterone-producing adenoma (with subsequent treatment consisting of adrenalectomy) or bilateral adrenal hyperplasia (subsequent treatment with mineralocorticoid receptor antagonists). The primary endpoint was the intensity of drug treatment for obtaining target blood pressure after 1 year of follow-up, in the intention-to-diagnose population. Intensity of drug treatment was expressed as daily defined doses. Key secondary endpoints included biochemical outcome in patients who received adrenalectomy, health-related quality of life, cost-effectiveness, and adverse events. This trial is registered with ClinicalTrials.gov, number NCT01096654. FINDINGS: We recruited 200 patients between July 6, 2010, and May 30, 2013. Of the 184 patients that completed follow-up, 92 received CT-based treatment (46 adrenalectomy and 46 mineralocorticoid receptor antagonist) and 92 received AVS-based treatment (46 adrenalectomy and 46 mineralocorticoid receptor antagonist). We found no differences in the intensity of antihypertensive medication required to control blood pressure between patients with CT-based treatment and those with AVS-based treatment (median daily defined doses 3·0 [IQR 1·0-5·0] vs 3·0 [1·1-5·9], p=0·52; median number of drugs 2 [IQR 1-3] vs 2 [1-3], p=0·87). Target blood pressure was reached in 39 (42%) patients and 41 (45%) patients, respectively (p=0·82). On secondary endpoints we found no differences in health-related quality of life (median RAND-36 physical scores 52·7 [IQR 43·9-56·8] vs 53·2 [44·0-56·8], p=0·83; RAND-36 mental scores 49·8 [43·1-54·6] vs 52·7 [44·9-55·5], p=0·17) for CT-based and AVS-based treatment. Biochemically, 37 (80%) of patients with CT-based adrenalectomy and 41 (89%) of those with AVS-based adrenalectomy had resolved hyperaldosteronism (p=0·25). A non-significant mean difference of 0·05 (95% CI -0·04 to 0·13) in quality-adjusted life-years (QALYs) was found to the advantage of the AVS group, associated with a significant increase in mean health-care costs of €2285 per patient (95% CI 1323-3248). At a willingness-to-pay value of €30 000 per QALY, the probability that AVS compared with CT constitutes an efficient use of health-care resources in the diagnostic work-up of patients with primary aldosteronism is less than 0·2. There was no difference in adverse events between groups (159 events of which nine were serious vs 187 events of which 12 were serious) for CT-based and AVS-based treatment. INTERPRETATION: Treatment of primary aldosteronism based on CT or AVS did not show significant differences in intensity of antihypertensive medication or clinical benefits for patients after 1 year of follow-up. This finding challenges the current recommendation to perform AVS in all patients with primary aldosteronism. FUNDING: Netherlands Organisation for Health Research and Development-Medical Sciences, Institute of Cardiology, Warsaw.

Aspiration sclerotherapy combined with pasireotide to improve reduction of large symptomatic hepatic cysts (SCLEROCYST): study protocol for a randomized controlled trial.

BACKGROUND: Aspiration sclerotherapy is an effective therapeutic option for large symptomatic hepatic cysts. However, incomplete cyst reduction following aspiration sclerotherapy is frequently reported. Strong post-procedural cyst fluid secretion by cholangiocytes, which line the epithelium of the hepatic cyst, seems to be associated with lower reduction rates. Previous studies showed that somatostatin analogues curtail hepatic cyst fluid production. This trial will evaluate the effect of aspiration sclerotherapy combined with the somatostatin analogue pasireotide on cyst reduction. By combining treatment modalities we aim to improve cyst reduction leading to greater symptomatic relief and reduced rates of cyst recurrence. METHODS/DESIGN: This single center, randomized, double-blind, placebo-controlled clinical trial evaluates the additional effect of pasireotide when combined with aspiration sclerotherapy in patients with a large (>5 cm) symptomatic hepatic cyst. A total of 34 participants will be randomized in a 1:1 ratio. In the active arm, patients will receive pasireotide (long-acting release, 60 mg injection) two weeks prior to and two weeks following aspiration sclerotherapy. Patients in the control arm will receive placebo injections at corresponding intervals. The primary outcome is proportional cyst diameter reduction four weeks after aspiration sclerotherapy compared to baseline measurements, obtained by ultrasonography. As secondary outcomes, proportional volume reduction, recurrence, symptomatic relief and improvement of health-related quality of life will be assessed. Furthermore, safety and tolerability of the combination of pasireotide and aspiration sclerotherapy will be evaluated. DISCUSSION: This trial aims to improve efficacy of aspiration sclerotherapy by a combined approach of two treatment modalities. We hypothesize that pasireotide will decrease fluid re-accumulation after aspiration sclerotherapy, leading to effective hepatic cyst reduction and symptomatic relief. TRIALS REGISTRATION: This trial is registered with ClinicalTrials.gov (identifier: NCT02048319 ; registered on 6 January 2014) and EudraCT (identifier: 2013-003168-29; registered on 16 August 2013).

Automatic segmentation of the wire frame of stent grafts from CT data.

Endovascular aortic replacement (EVAR) is an established technique, which uses stent grafts to treat aortic aneurysms in patients at risk of aneurysm rupture. 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, analysis of stent graft movement in individual patients in vivo can be valuable for predicting stent graft failure in these patients. To be able to gather information on stent graft motion in a quick and robust fashion, we propose an automatic method to segment stent grafts from CT data, consisting of three steps: the detection of seed points, finding the connections between these points to produce a graph, and graph processing to obtain the final geometric model in the form of an undirected graph. Using annotated reference data, the method was optimized and its accuracy was evaluated. The experiments were performed using data containing the AneuRx and Zenith stent grafts. The algorithm is robust for noise and small variations in the used parameter values, does not require much memory according to modern standards, and is fast enough to be used in a clinical setting (65 and 30s for the two stent types, respectively). Further, it is shown that the resulting graphs have a 95% (AneuRx) and 92% (Zenith) correspondence with the annotated data. The geometric model produced by the algorithm allows incorporation of high level information and material properties. This enables us to study the in vivo motions and forces that act on the frame of the stent. We believe that such studies will provide new insights into the behavior of the stent graft in vivo, enables the detection and prediction of stent failure in individual patients, and can help in designing better stent grafts in the future.

Endovascular management of a gunshot wound injury to the innominate artery and brachiocephalic vein.

Surgical repair of penetrating injuries of the thoracic outlet with combined arterial and venous involvement is associated with considerable morbidity and mortality. A 37-year-old man presented to the emergency room with a left-sided penetrating zone I neck injury caused by a close-range handgun shot. This had resulted in an injury to the innominate artery and the origin of the right common carotid artery, with shunting to the brachiocephalic vein. This was managed endovascularly by stenting of the innominate artery and by coiling of the origin of the carotid artery. An endovascular approach to this injury is feasible and has the advantage of appropriate visualization of the vascular lesions with limited blood loss during the repair.

Delayed pseudoaneurysm after renal artery angioplasty.

PURPOSE: To report delayed pseudoaneurysm formation after percutaneous renal artery angioplasty. CASE REPORTS: A 56-year-old woman succumbed to complications of a ruptured right juxtarenal aortic pseudoaneurysm 2 years after right renal artery stenting for renal impairment. The juxtarenal aorta had been normal on aortography at the time of angioplasty. She gave a history of right-sided back pain that started within 8 months of the angioplasty. A 21-year-old woman with left renal artery stenosis due to fibromuscular dysplasia was treated with balloon angioplasty, after which there was evidence of active extravasation. The completion angiogram, after a period of observation, documented cessation of the leak. Follow-up angiography because of persisting hypertension disclosed a large, asymptomatic, intrarenal pseudoaneurysm that was repaired with bench surgery and autotransplantation. CONCLUSIONS: We advise that patients with symptoms referable to the site of renal artery intervention and those who have had complicated interventions should have follow-up imaging to exclude pseudoaneurysm formation.