Clinical Uses and Short-Term Safety Profile of Ethiodized Poppy Seed Oil Contrast Agent in the Diagnosis and Treatment of Vascular Anomalies and Tumors.

BACKGROUND: There is a sparsity of data on the use of ethiodized poppy seed oil (EPO) contrast agent (Lipiodol) in patients. We investigated the safety of EPO in children, adolescents, and some adults for diagnostic and therapeutic interventions. METHODS: All patients who underwent procedures with EPO between 1995 and 2014 were retrospectively included. Demographic characteristics, diagnosis, dose, route of administration, preparation of EPO in combination with other agents, and complications were recorded. RESULTS: In 1422 procedures, EPO was used for diagnostic or treatment purposes performed in 683 patients. The mean patient age was 13.4 years (range: 2 months-50 years); 58% of patients were female. Venous malformations (n = 402, 58.9%) and arteriovenous malformations (n = 60, 8.8%) were the most common diagnosis. Combined vascular anomalies included capillary-lymphatic-venous malformations, fibroadipose vascular anomalies (n = 54, 7.9%), central conducting lymphatic anomalies (n = 31, 4.5%), lymphatic malformations (n = 24, 3.5%), aneurysmal bone cysts (n = 22, 3.2%), and vascularized tumors (n = 11, 1.6%). In 1384 procedures (96%), EPO was used in various combinations with sclerosing and embolization agents, including sodium tetradecyl sulfate, ethanol, and glue. The mean volume of EPO used in interventions was 3.85 mL (range: 0.1-25 mL) per procedure with a mean patient weight of 45.9 kg (range: 3.7-122.6 kg) and a weight-adjusted dose of 0.12 mL/kg (range: 0.001-1.73 mL/kg). In 56 procedures (4%), EPO was used as a single agent for diagnostic lymphangiography. The mean volume was 4.8 mL (range: 0.3-13 mL) per procedure with a mean patient weight of 27.4 kg (range: 2.4-79.3 kg) and a weight-adjusted dose of 0.2 mL/kg (range: 0.04-0.54 mL/kg). Procedural-related complications occurred in 25 (1.8%) procedures. The 20 minor and 5 major complications were related to the primary treatment agents. None of them were directly related to EPO. No allergic reactions were noted. CONCLUSION: The use of an ethiodized poppy seed oil contrast agent in children, adolescents, and adults for diagnostic or therapeutic purposes is safe.

Association of Intrarenal Resistance Index and Systemic Atherosclerosis After Kidney Transplantation.

BACKGROUND/AIM: Patients after kidney transplants are at risk of cardiovascular morbidity. An elevated resistance index (RI) is associated with renal graft failure, while a decreased RI can be due to a renal artery stenosis. The RI can also be measured in the carotid artery. Whether a correlation between intrarenal RI after kidney transplant in adult patients and the RI of the internal carotid artery exists is still unclear. PATIENTS AND METHODS: In this prospective cross-sectional study, RI of kidney transplants and of the internal carotid artery were measured with duplex sonography. Carotid intima-media thickness as well as the Framingham risk score and the Augmentation index, all known markers of atherosclerosis, were assessed. Correlations between the RI in Carotid artery and the RI of the kidney transplant were based on Spearmen test with the level of significance set at p<0.05. RESULTS: Ninety-eight consecutive patients [60% male, mean age of 48.7 (±15.6)] were included. The mean interval after transplantation was 27.5 (±8.5) months and mean serum creatinine was 308 (±220.3) mmol/ml The RI of the internal carotid artery and the renal transplant were significantly correlated (p<0.05). A correlation between the RIs and the Augmentation Index was found. CONCLUSION: The RI of the kidney transplant is correlated with the RI of the carotid artery and to markers of general atherosclerosis. This observation may be helpful to identify patients after kidney transplant with higher risk for cardiovascular events and gain indirect information on transplant renal artery stenosis.

Quality of Life in Swiss Patients With Spongiform Venous Malformations.

BACKGROUND/AIM: Spongiform venous malformations (sVMs) get symptomatic in >90% of cases during a person's lifetime. Misdiagnosis is still common and treatment often incomplete, making this disease a lifelong issue for patients with a relevant impact on their quality of life. PATIENTS AND METHODS: Medical records and imaging studies of patients with VMs from April 2002 to January 2017 were reviewed for confirmation of diagnosis and classification of the VMs. Only sVMs were included. Subjective data were obtained from the survey related to indication, response, and complications. We analyzed the frequency of correct diagnosis and Quality of Life by an SF12-based questionnaire for sVM-related issues in Swiss patients. RESULTS: A total of 80 patients were included in the study. Forty-six (58%) patients were females. Patients were 11.6-77 years old with a median age of 28.1 years. The correct diagnosis according to the ISSVA-classification after having been seen at our Institution was 87%. Thirty-one (39%) patients responded to the survey. Sixteen (51%) were female. Twenty-eight (90%) patients felt that their sVM-related state of health improved within a year. Twelve (39%) patients reported that they could not work as good as normal because of slight to modest impairment by the sVM, while 19 (61%) patients were unimpaired. Mental impairment was found in 8 (26%) patients, while 23 (74%) patients felt no impairment. Eight (26%) patients reported that they were impaired within social contacts due to their sVM. Only 9 (29%) patients reported that venous malformation was diagnosed around birth. Twenty-three (74%) patients received a wrong diagnosis. Patients that were treated, reported close to complete relief of symptoms in 26% (8 patients) while also 26% (8 patients) reported no change of symptoms after therapy. CONCLUSION: Swiss sVM patients also suffer from misdiagnosis and late diagnosis. They are impacted in their daily life by their disease.

Cholesterol Efflux Capacity Associates with the Ankle-Brachial Index but Not All-Cause Mortality in Patients with Peripheral Artery Disease.

BACKGROUND: Cholesterol efflux is an important mechanism by which high-density lipoproteins (HDLs) protect against cardiovascular disease. As peripheral artery disease (PAD) is associated with high mortality rates, mainly due to cardiovascular causes, we investigated whether cholesterol efflux capacity (CEC) of apolipoprotein B (apoB)-depleted plasma, a widely used surrogate of HDL function, may serve as a predictive marker for mortality in this patient population. METHODS: In this prospective single-center study (median follow-up time: 9.3 years), apoB-containing lipoproteins were precipitated from plasma of 95 patients with PAD and incubated with J744-macrophages, which were loaded with radiolabeled cholesterol. CEC was defined as the fractional radiolabel released during 4 h of incubation. RESULTS: Baseline CEC was lower in PAD patients that currently smoked (p = 0.015) and had a history of myocardial infarction (p = 0.011). Moreover, CEC showed a significant correlation with HDL-cholesterol (p = 0.003) and apolipoprotein A-I levels (p = 0.001) as well as the ankle-brachial index (ABI, p = 0.018). However, CEC did not differ between survivors and non-survivors. Neither revealed Kaplan-Meier and Cox regression analyses any significant association of CEC with all-cause mortality rates. CONCLUSION: Taken together, CEC is associated with ABI but does not predict all-cause mortality in patients with PAD.

Cardiac biomarkers but not measures of vascular atherosclerosis predict mortality in patients with peripheral artery disease.

BACKGROUND: Peripheral artery disease (PAD) becomes more prevalent with advancing age and is associated with elevated risk of cardiovascular events and shortened life expectancy. We investigated the prognostic performance of cardiac and vascular biomarkers in a cohort of PAD patients. METHODS: A total of 95 PAD patients were enrolled (mean age 68 years, range 47 to 86 years, 73 males). Carotid intima-media thickness (cIMT), ankle brachial index (ABI), high sensitive cardiac troponin T, and N-terminal pro-B-type Natriuretic Peptide (NT-proBNP) were measured. RESULTS: During a median follow-up time of 9.5 years, 44 patients died and 51 patients survived. Upon Kaplan-Meier survival analysis hs-TnT (P < .001) or NT-proBNP levels (P < .001) above the median but not cIMT above the median (P = .488) or ABI below the median (P < .436)were associated with reduced survival rate. Upon univariate cox regression and after adjustment for age, gender, prior cerebral artery disease, and diabetes mellitus only the association between hs-cTnT and mortality remained significant (HR 1.93, 95% CI 1.33-2.79, P < .001). In receiver operating curve analysis hs-cTnT (area under the curve [AUC]: 0.77, 95% CI: 0.67-0.87, P < .001) NT-proBNP (AUC: 0.74, 95% CI: 0.64-0.84, P < .001) as well as hs-cTnT, and NT-proBNP combined (AUC: 0.79, 95% CI: 0.69-0.88, P < .001) were superior to cIMT (AUC: 0.64, 95%, CI: 0.53-0.76, P = .022) and ABI (AUC: 0.57, 95% CI: 0.44-0.68, P = .313) in discriminating risk for mortality. CONCLUSION: hs-cTnT and NT-proBNP should be taken into account for prognosis of patients with PAD.

Vertebral and internal mammary artery steal syndrome in patients with hemodialysis access.

BACKGROUND: Increased flow in the subclavian artery feeding a vascular access for hemodialysis can induce steal phenomena in the vertebral (VA) and internal mammary artery (IMA). The aim of this study was to describe the hemodynamic effects of access flow on the VA and IMA in patients with native fistulas and grafts. PATIENTS AND METHODS: Peak systolic (PSV) and end diastolic (EDV) velocity measurements of the VA, IMA and carotid arteries, as well as flow volume measurements of the subclavian artery, were performed. Flow measurements at the side of the vascular access were compared with the contralateral side. Fifty-five patients were consecutively included, most with a radio-cephalic fistula on the left arm with a mean shunt volume of 1156 ml/min. RESULTS: Pathologic flow patterns were observed in the ipsilateral VA in four patients (7.3 %); contralateral VA flow was normal in all patients. Peak systolic velocity of the VA was significantly decreased at the side of the shunt arm with a PSV of 42.6 ± 11.8 cm/s compared to 48.4 ± 15.6 cm/s contralateral (p < 0.05). The IMA flow pattern were normal in all patients. The PSV of the IMA was significantly decreased (p < 0.01) at the side of the shunt arm (87.5 ± 29.1 cm/s) compared to the non-shunt arm (95.9 ± 27.4 cm/s). CONCLUSION: We describe significant hemodynamic effects of fistulas to the vertebral and internal mammary arteries. Doppler spectral analysis of the vertebral and internal mammary arteries should be integrated in ultrasound, especially in patients with cerebrovascular or cardiac symptoms.

Misdiagnosis of plexiform neurofibroma as venous malformation in pediatric patients.

BACKGROUND: The aim of this study was to identify Magnetic Resonance Imaging (MRI) characteristics that reliably distinguish deep plexiform neurofibromas (PNFs) from venous malformations (VMs). METHODS: A database search was conducted for patients that were referred with a vascular anomaly but had a neurofibroma instead. Clinical and imaging features of patients with venous malformations as the most common referral diagnosis were compared to those with PNFs. The imaging features of deep PNFs recorded were: anatomical location, size, morphology, margins, signal intensity and post-contrast enhancement pattern. RESULTS: Ten patients with PNFs were identified. Five patients had adequate imaging. These five patients were included in our study. There were 3 female and 2 male patients ranging in age from 10 months to 21 years. Deep PNFs were located in the cervicofacial region (N.=3), lower extremity (N.=1) and back/flank region (N.=1). The most common clinical features of all these patients were palpable mass (N.=5) and pain (N.=4). The MRI features that distinguished VMs from deep PNFs were the serpiginous morphology, relatively intermediate T-2 signal intensity and peculiar enhancement pattern without fluid-fluid levels or phleboliths and nerve root involvement. The target sign can be regularly found in PNFs, but may be also present in VMs and other vascular lesions. CONCLUSIONS: Target signs, a typical sign of PNFs may be absent and can also be found in vascular anomalies, leading to confusion in diagnosis. PNFs can be reliably distinguished from VMs on MRI based on the above mentioned features.

Combined and complex vascular malformations.

The correct diagnosis of vascular malformations is obtainable by clinical assessment and patient history in the majority of cases. Nonetheless, confusion in nomenclature, existence of multiple classifications and rarity of these lesions leads to misdiagnosis and related wrong treatment. This is especially the case in combined or complex vascular malformations or vascular malformations that are part of syndromes as these have overlapping clinical and imaging features. New entities in the field of vascular anomalies have been described recently like fibro-adipose vascular anomaly or central conducting lymphatic anomalies.

Vascular malformations revisited.

Vascular malformations are congenital anomalies that can affect each part of the vasculature. Combined forms are common and they are often part of complex syndromes. Most malformations are diagnosed during infancy, but some get obvious only later in life. The field of vascular malformations is emerging with recently described new entities and treatments. Still, misdiagnosis is common in this field, leading to nosologic confusion and wrong treatment. Clinical evaluation and imaging are the gold standard for diagnostic confirmation. Sclerotherapy and embolization are the main treatment techniques but are also used preoperatively to reduce blood loss and shrink the lesion if surgery is planned. Despite new treatment options, especially if extensive in size or involving vulnerable structures, vascular malformations are still considered chronic diseases and cause significant morbidity. Common understanding and agreement on terminology and a multidisciplinary approach are the basis of successful treatment and long-term support for these patients. Continuing research in the field of vascular anomalies will improve knowledge and create further treatment options.

Acute effects of haemodialysis on central venous and arterial pressure characteristics.

AIM: Haemodynamic stability of patients during haemodialysis (HD) sessions is of pivotal importance and accurate determination of dry weight remains a challenge. Little information is available about central venous and aortic pressure during dialysis. In this pilot study we used a non-invasive technique to describe the changes in central venous pressure (CVP) during dialysis. METHODS: An ultrasound-assisted pressure-manometer was used at the cephalic vein during haemodialysis to quantify CVP. Central aortic pressure changes were assessed as aortic augmentation index and subendocardial viability ratio. Bioimpedance was applied to measure total body water, as well as extracellular and intracellular water before and after HD. Measurements were performed prior during and after 1 and 2 h on HD. RESULTS: Ten patients were included with a median age of 72 years (23-82). Haemodialysis reduced the weight by 2.0 kg, corresponding to a measured decrease in total body water of 1.9 L. The mean CVP showed a significant decrease (9.0-0.8 cmH2O; P = 0.0005) during dialysis. The significant drop in CVP was found during the first hour (9-2.8 cmH2O). Starting and stopping dialysis was reflected by a reduction of 2.6 cmH2O and a rise of 2.8 cmH2O (n.s.). Aortic augmentation index decreased from 26.1% to 21.0% (n.s.). Subendocardial viability ratio increased from 126% to 156% (P < 0.05) during HD, and decreased to 139% direct after HD (n.s.). CONCLUSION: This is the first study that illustrates a prominent reduction of CVP during the first hour of haemodialysis. Non-invasive CVP measurement is feasible during haemodialysis and adds another piece in the puzzle of factors involved in haemodynamic stability.

Predictive value of auscultation of femoropopliteal arteries.

BACKGROUND: Femoropopliteal bruits indicate flow turbulences and increased blood flow velocity, usually caused by an atherosclerotic plaque or stenosis. No data exist on the quality of bruits as a means for quantifying the degree of stenosis. We therefore conducted a prospective observational study to investigate the sensitivity and specificity of femoropopliteal auscultation, differentiated on the basis of bruit quality, to detect and quantify clinically relevant stenoses in patients with symptomatic and asymptomatic peripheral arterial disease (PAD). METHODS: Patients with known chronic and stable PAD were recruited in the outpatient clinic. We included patients with known PAD and an ankle-brachial index (ABI) <0.90 and/or an ABI ≥0.90 with a history of lower limb revascularisation. Auscultation was performed independently by three investigators with varied clinical experience after a 10-minute period of rest. Femoropopliteal lesions were classified as follows: normal vessel wall or slight wall thickening (<20%), atherosclerotic plaque with below 50% reduction of the vessel lumen, prestenotic/intrastenotic ratio over 2.5 (<70%), over 3.5 (<99%) and complete occlusion (100%). RESULTS: Weighted Cohen's κ coefficients for differentiated auscultation were low in all vascular regions and did not differ between investigators. Sensitivity was low in most areas with an increase after exercise. The highest sensitivity in detecting relevant (>50%) stenosis was found in the common femoral artery (86%). CONCLUSION: Vascular auscultation is known to be of great use in routine clinical practice in recognising arterial abnormalities. Diagnosis of PAD is based on various diagnostic tools (pulse palpation, ABI measurement) and auscultation can localise relevant stenosis. However, auscultation alone is of limited sensitivity and specificity in grading stenosis in femoropopliteal arteries. Where PAD is clinically suspected further diagnostic tools, especially colour-coded duplex ultrasound, should be employed to quantify the underlying lesion.

Sclerotherapy of lymphatic cysts of the leg with OK-432.

Lymphatic cysts can either represent lymphatic malformations (LMs) or may occur after trauma or operation. Independent of their etiology lymphatic cysts can cause functional impairment and recurrent skin infections. We report the successful use of OK-432 injected transcutanously to treat lymphatic macrocysts. Treatment of LMs with OK-432 has been reported to be a safe and minimally invasive procedure. The underlying mechanism is still unclear but injection leads to an inflammation of the wall resulting in occlusion or shrinkage of the cyst.