Congenital Vascular Malformations in Children: From Historical Perspective to a Multidisciplinary Approach in the Modern Era-A Comprehensive Review.

Congenital vascular malformations (CVMs) are the result of an aberrant development during embryogenesis. Although these lesions are present at birth, they are not always visible yet. Once symptomatic, patients suffer from pain, bleeding, ulcers, infections or lymphatic leakage, depending on the subtype of vessels involved. Treatment includes conservative management, surgery, sclerotherapy, embolization and pharmacological therapy. The clinical presentation varies widely and treatment can be challenging due to the rarity of the disease and potential difficulties of treatment. This review gives an overview of the historical developments in diagnosis and classification and exposes the key elements of innovations in the past decades on the identification of genetic mutations and personalized treatment. These advances in the field and a multidisciplinary approach are highly valuable in the optimization of clinical care aimed at both curing or stabilizing the CVM and pursuing physical and psychosocial wellbeing.

Clinical differences in sirolimus treatment with low target levels between children and adults with vascular malformations - A nationwide trial.

The clinical presentation of patients with slow-flow vascular malformations is very heterogeneous. High clinical burden and subsequent reduced health-related quality of life is something they have in common. There is an unmet medical need for these patients for whom regular treatments like surgery and embolization are either insufficient or technically impossible. Sirolimus has been reported to be effective and overall well-tolerated in most patients. However, the main limitation of sirolimus is the reported high toxicity, especially when target levels of 10-15 ng/mL are being used. We report the results of a phase IIB single-arm open-label clinical trial consisting of 68 (67 in the challenge phase and 68 in the rechallenge phase) evaluable patients (children n = 33 and adults n = 35) demonstrating that treatment with low sirolimus target levels (4-10 ng/mL) is effective in 79.1% of the patients. When sirolimus treatment was stopped, the majority of patients experienced a recurrence of symptoms, supporting prolonged or even lifelong treatment requirement. Adults experienced a higher baseline pain score compared with children, having an estimated marginal mean of 6.2 versus 4.1, p < 0.05; however, they showed a similar decrease to children. Furthermore, the pediatric population experienced less often a sirolimus-related grade I-IV adverse event (35.9% vs. 64.1%, p > 0.05) compared with adults. Additionally, response rates were higher in children compared with adults (93.8% vs. 65.7%, p < 0.05), and children responded faster (28 vs. 91 days, p < 0.05). These results suggest benefits of sirolimus in patients with slow-flow vascular malformations and support its initiation as young as possible.

The lymphatic system throughout history: From hieroglyphic translations to state of the art radiological techniques.

A comprehensive lymphatic system is indispensable for a well-functioning body; it is integral to the immune system and is also interrelated with the digestive system and fluid homeostasis. The main difficulty in examining the lymphatic system is its fine-meshed structure. This remains a challenge, leaving patients with uninterpreted symptoms and a dearth of potential therapies. We review the history of the lymphatic system up to the present with the aim of improving current knowledge. Several findings described throughout history have made fundamental contributions to elucidating the lymphatic system. The first contributions were made by the ancient Egyptians and the ancient Greeks. Vesalius obtained new insights by dissecting corpses. Thereafter, Ruysch (1638-1731) gained an understanding of lymphatic flow. In 1784, Mascagni published his illustration of the whole lymphatic network. The introduction of radiological lymphography revolutionized knowledge of the lymphatic system. Pedal lymphangiography was first described by Monteiro (1931) and Kinmonth (1952). Lymphoscintigraphy (nuclear medicine), magnetic resonance imaging, and near-infrared fluorescence lymphography further improved visualization of the lymphatic system. The innovative dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) transformed understanding of the central lymphatic system, enabling central lymphatic flow disorders in patients to be diagnosed and even allowing for therapeutic planning. From the perspective of the history of lymph visualization, DCMRL has ample potential for identifying specific causes of debilitating symptoms in patients with central lymphatic system abnormalities and even allows for therapeutic planning.

Semantic modelling of common data elements for rare disease registries, and a prototype workflow for their deployment over registry data.

BACKGROUND: The European Platform on Rare Disease Registration (EU RD Platform) aims to address the fragmentation of European rare disease (RD) patient data, scattered among hundreds of independent and non-coordinating registries, by establishing standards for integration and interoperability. The first practical output of this effort was a set of 16 Common Data Elements (CDEs) that should be implemented by all RD registries. Interoperability, however, requires decisions beyond data elements - including data models, formats, and semantics. Within the European Joint Programme on Rare Diseases (EJP RD), we aim to further the goals of the EU RD Platform by generating reusable RD semantic model templates that follow the FAIR Data Principles. RESULTS: Through a team-based iterative approach, we created semantically grounded models to represent each of the CDEs, using the SemanticScience Integrated Ontology as the core framework for representing the entities and their relationships. Within that framework, we mapped the concepts represented in the CDEs, and their possible values, into domain ontologies such as the Orphanet Rare Disease Ontology, Human Phenotype Ontology and National Cancer Institute Thesaurus. Finally, we created an exemplar, reusable ETL pipeline that we will be deploying over these non-coordinating data repositories to assist them in creating model-compliant FAIR data without requiring site-specific coding nor expertise in Linked Data or FAIR. CONCLUSIONS: Within the EJP RD project, we determined that creating reusable, expert-designed templates reduced or eliminated the requirement for our participating biomedical domain experts and rare disease data hosts to understand OWL semantics. This enabled them to publish highly expressive FAIR data using tools and approaches that were already familiar to them.

Effective low-dose sirolimus regimen for kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon in young infants.

AIMS: Management of kaposiform haemangioendotheliomas (KHE) with Kasabach-Merritt phenomenon is challenging in young infants who are subjected to developmental pharmacokinetic changes. Sirolimus, sometimes combined with corticosteroids, can be used as an effective treatment of KHE. Simultaneously, toxicities such as interstitial pneumonitis related to the use of sirolimus may be fatal. As infants have a very low CYP3-enzyme expression at birth, which rises during ageing, we hypothesize that a reduced metabolization of sirolimus might lead to high sirolimus serum levels and low dose may be sufficient without the side effects. METHODS: A case series of 5 infants with kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon was analysed retrospectively. All infants were treated with sirolimus 0.2 mg/m2 every 24 or 48 hours according to their age. Prednisone was added to the therapy for additional effect in 4 patients. RESULTS: In all patients, low dose of sirolimus led to therapeutic sirolimus levels (4-6 ng/mL). All infants (aged 4 days-7 months) had a complete haematological response, without serious adverse events. In all patients, the Kasabach-Merritt phenomenon resolved, the coagulation profile normalized and tumour size reduction was seen. CONCLUSION: Low-dose sirolimus treatment is safe for infants with kaposiform haemangioendothelioma and Kasabach-Merritt phenomenon. It is essential to realize that during the first months of life, metabolism is still developing and enzymes necessary to metabolise drugs like sirolimus still have to mature. To avoid toxic levels, the sirolimus dosage should be based on age and the associated pharmacological developments.

A Clinical Feasibility Study to Image Angiogenesis in Patients with Arteriovenous Malformations Using 68Ga-RGD PET/CT.

Arteriovenous malformations (AVMs) have an inherent capacity to form new blood vessels, resulting in excessive lesion growth, and this process is further triggered by the release of angiogenic factors. 68Ga-labeled arginine-glycine-aspartate tripeptide sequence (RGD) PET/CT imaging may provide insight into the angiogenic status and treatment response of AVMs. This clinical feasibility study was performed to demonstrate that 68Ga-RGD PET/CT imaging can be used to quantitatively assess angiogenesis in peripheral AVMs. Methods: Ten patients with a peripheral AVM (mean age, 40 y; 4 men and 6 women) and scheduled for endovascular embolization treatment were prospectively included. All patients underwent 68Ga-RGD PET/CT imaging 60 min after injection (mean dose, 207 ± 5 MBq). Uptake in the AVM, blood pool, and muscle was quantified as SUVmax and SUVpeak, and a descriptive analysis of the PET/CT images was performed. Furthermore, immunohistochemical analysis was performed on surgical biopsy sections of peripheral AVMs to investigate the expression pattern of integrin αvß3Results:68Ga-RGD PET/CT imaging showed enhanced uptake in all AVM lesions (mean SUVmax, 3.0 ± 1.1; mean SUVpeak, 2.2 ± 0.9). Lesion-to-blood and lesion-to-muscle ratios were 3.5 ± 2.2 and 4.6 ± 2.8, respectively. Uptake in blood and muscle was significantly higher in AVMs than in background tissue (P = 0.0006 and P = 0.0014, respectively). Initial observations included uptake in multifocal AVM lesions and enhanced uptake in intraosseous components in those AVM cases affecting bone integrity. Immunohistochemical analysis revealed cytoplasmatic and membranous integrin αvß3 expression in the endothelial cells of AVMs. Conclusion: This feasibility study showed increased uptake in AVMs with angiogenic activity, compared with surrounding tissue without angiogenic activity, suggesting that 68Ga-RGD PET/CT imaging can be used as a tool to quantitatively determine angiogenesis in AVMs. Further studies will be conducted to explore the potential of 68Ga-RGD PET/CT imaging for guiding current treatment decisions and for assessing response to antiangiogenic treatment.

A New and Innovative Method of Preoperatively Planning and Projecting Vascular Anatomy in DIEP Flap Breast Reconstruction: A Randomized Controlled Trial.

BACKGROUND: In deep inferior epigastric perforator (DIEP) flap breast reconstructions, a free tissue flap from the abdomen is shaped into a breast and transferred to the thorax. Survival of this free flap relies on minuscule blood vessels, so-called perforators, providing blood supply to this newly molded breast. Preoperative mapping of these randomly distributed blood vessels is essential to avoid complications. The purpose of this study was to investigate whether the preoperative projection of a virtual three-dimensional plan based on computed tomographic angiography onto the abdomen leads to more correctly identified perforator locations and less operative time spent on dissecting the free flap compared to the commonly used Doppler ultrasound planning method. METHODS: The authors conducted a randomized, open, single-center, superiority trial in patients undergoing DIEP flap breast reconstruction with 1-week follow-up. Randomized participants were 60 adults (projection method, n = 33; Doppler method, n = 27) undergoing DIEP flap breast reconstruction without lymph node transfer. RESULTS: Sixty patients provided 69 DIEP flaps for analysis. The projection method is capable preoperatively of displaying significantly more perforators compared to the Doppler method (61.7 ± 7.3 percent versus 41.2 ± 8.2 percent, respectively; p = 0.020)). During the procedure, flap harvest time is decreased by 19 minutes (136 ± 7 minutes versus 155 ± 7 minutes; p = 0.012). Complications were comparable across both groups. CONCLUSION: Not only can more perforators be identified intraoperatively using the projection method compared with Doppler ultrasound, there is also a significant time reduction in harvesting the DIEP flap. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, II.

Comprehensive molecular and clinicopathological analysis of vascular malformations: A study of 319 cases.

Vascular malformations are part of overgrowth syndromes characterized by somatic mosaic mutations or rarely by germline mutations. Due to their similarities and diversity, clinicopathological classification can be challenging. A comprehensive targeted Next Generation Sequencing screen using Unique Molecular Identifiers with a technical sensitivity of 1% mutant alleles was performed for frequently mutated positions in ≥21 genes on 319 formalin-fixed paraffin-embedded samples. In 132 out of 319 cases pathogenic mosaic mutations were detected affecting genes previously linked to vascular malformations e.g. PIK3CA (n=80), TEK (TIE2) (n=11), AKT1 (n=1), GNAQ (n=7), GNA11 (n=4), IDH1 (n=3), KRAS (n=9), and NRAS (n=1). Six cases harbored a combination of mutations in PIK3CA and in GNA11 (n=2), GNAQ (n=2), or IDH1 (n=2). Aberrations in PTEN and RASA1 with a variant allele frequency approaching 50% suggestive of germline origin were identified in six out of 102 cases tested; four contained a potential second hit at a lower allele frequency. Ninety-one of the total 142 pathogenic mutations were present at a variant allele frequency <10% illustrating the importance of sensitive molecular analysis. Clinicopathological characteristics showed a broad spectrum and overlap when correlated with molecular data. Sensitive screening of recurrently mutated genes in vascular malformations may help to confirm the diagnosis and reveals potential therapeutic options with a significant contribution of PIK3CA/mTOR and RAS-MAPK pathway mutations. The co-existence of two activating pathogenic mutations in parallel pathways illustrates potential treatment challenges and underlines the importance of multigene testing. Detected germline mutations have major clinical impact.

Pasireotide does not improve efficacy of aspiration sclerotherapy in patients with large hepatic cysts, a randomized controlled trial.

OBJECTIVES: We tested whether complementary use of the somatostatin analogue pasireotide would augment efficacy of aspiration sclerotherapy of hepatic cysts. METHODS: We conducted a double-blind, placebo-controlled trial in patients who underwent aspiration sclerotherapy of a large (>5 cm) symptomatic hepatic cyst. Patients were randomized to either intramuscular injections of pasireotide 60 mg long-acting release (n = 17) or placebo (sodium chloride 0.9 %, n = 17). Injections were administered 2 weeks before and 2 weeks after aspiration sclerotherapy. The primary endpoint was proportional cyst diameter reduction (%) from baseline to 6 weeks. Secondary outcomes included long-term cyst reduction at 26 weeks, patient-reported outcomes including the polycystic liver disease-questionnaire (PLD-Q) and safety. RESULTS: Thirty-four patients (32 females; 53.6 ± 7.8 years) were randomized between pasireotide or placebo. Pasireotide did not improve efficacy of aspiration sclerotherapy at 6 weeks compared to controls (23.6 % [IQR 12.6-30.0] vs. 21.8 % [9.6-31.8]; p = 0.96). Long-term cyst diameter reduction was similar in both groups (49.1 % [27.0-73.6] and 45.6 % [29.6-59.6]; p = 0.90). Mean PLD-Q scores improved significantly in both groups (p < 0.01) without differences between arms (p = 0.92). CONCLUSIONS: In patients with large symptomatic hepatic cysts, complementary pasireotide to aspiration sclerotherapy did not improve cyst reduction or clinical response. KEY POINTS: • Complementary pasireotide treatment does not improve efficacy of aspiration sclerotherapy. • Cyst fluid reaccumulation after aspiration sclerotherapy is a transient phenomenon. • Aspiration sclerotherapy strongly reduces symptoms and normalizes quality of life.

Embolization of the Geniculate Arteries Is an Effective Treatment of Recurrent Hemarthrosis Following Total Knee Arthroplasty That Can Be Safely Repeated.

BACKGROUND: Recurrent hemarthrosis is a late complication in up to 1.6% of patients following total knee arthroplasty (TKA). In the absence of intrinsic coagulopathy, one etiology is bleeding of hypertrophic vascular synovium. The aim of this study is to evaluate the clinical outcome of patients referred to our center for angiographic embolization of geniculate arteries for recurrent hemarthrosis following TKA. METHODS: We retrospectively studied a cohort of patients who were referred for geniculate artery embolization following TKA between August 2011 and September 2016. RESULTS: A total of 24 embolization procedures were performed on 14 patients. Seven (50%) of these 14 patients underwent one embolization procedure. Due to symptom recurrence, 4 patients underwent a repeated procedure and 3 patients a third procedure. All embolization procedures were technically successful at the time of the procedure. Two patients reported an inguinal hematoma that healed without further treatment. At follow-up of mean 26.8 months, clinical success was achieved in 12 of the 14 patients (86%). CONCLUSION: Embolization of the geniculate arteries in our study was a safe and effective treatment of recurrent spontaneous hemarthrosis following TKA. Although we have performed a substantial number of reinterventions, results of this study show that this procedure can be safely repeated without adverse events. Our results indicate that embolization could possibly be the treatment of choice when conservative measures fail and can be repeated in the event of recurrent or persistent symptoms.

An innovative method of planning and displaying flap volume in DIEP flap breast reconstructions.

BACKGROUND: Determining the ideal volume of the harvested flap to achieve symmetry in deep inferior epigastric artery perforator (DIEP) flap breast reconstructions is complex. With preoperative imaging techniques such as 3D stereophotogrammetry and computed tomography angiography (CTA) available nowadays, we can combine information to preoperatively plan the optimal flap volume to be harvested. In this proof-of-concept, we investigated whether projection of a virtual flap planning onto the patient's abdomen using a projection method could result in harvesting the correct flap volume. METHODS: In six patients (n = 9 breasts), 3D stereophotogrammetry and CTA data were combined from which a virtual flap planning was created comprising perforator locations, blood vessel trajectory and flap size. All projected perforators were verified with Doppler ultrasound. Intraoperative flap measurements were collected to validate the determined flap delineation volume. RESULTS: The measured breast volume using 3D stereophotogrammetry was 578 ± 127 cc; on CTA images, 527 ± 106 cc flap volumes were planned. The nine harvested flaps weighed 533 ± 109 g resulting in a planned versus harvested flap mean difference of 5 ± 27 g (flap density 1.0 g/ml). In 41 out of 42 projected perforator locations, a Doppler signal was audible. CONCLUSION: This proof-of-concept shows in small numbers that flap volumes can be included into a virtual DIEP flap planning, and transferring the virtual planning to the patient through a projection method results in harvesting approximately the same volume during surgery. In our opinion, this innovative approach is the first step in consequently achieving symmetric breast volumes in DIEP flap breast reconstructions.

Shared Decision-Making in the Management of Congenital Vascular Malformations.

BACKGROUND: In shared decision-making, clinicians and patients arrive at a joint treatment decision, by incorporating best available evidence and the patients' personal values and preferences. Little is known about the role of shared decision-making in managing patients with congenital vascular malformations, for which preference-sensitive decision-making seems obvious. The authors investigated preferences regarding decision-making and current shared decision-making behavior during physician-patient encounters. METHODS: In two Dutch university hospitals, adults and children with congenital vascular malformations facing a treatment-related decision were enrolled. Before the consultation, patients (or parents of children) expressed their preference regarding decision-making (Control Preferences Scale). Afterward, participants completed shared decision-making-specific questionnaires (nine-item Shared Decision-Making Questionnaire, CollaboRATE, and satisfaction), and physicians completed the Shared Decision-Making Questionnaire-Physician questionnaire. Consultations were audiotaped and patient involvement was scored by two independent researchers using the five-item Observing Patient Involvement instrument. All questionnaire results were expressed on a scale of 0 to 100 (optimum shared decision-making). RESULTS: Fifty-five participants (24 parents and 31 adult patients) were included. Two-thirds preferred the shared decision-making approach (Control Preferences Scale). Objective five-item Observing Patient Involvement scores were low (mean ± SD, 31 ± 15), whereas patient and physician Shared Decision-Making Questionnaire scores were high, with means of 68 ± 18 and 68 ± 19, respectively. The median CollaboRATE score was 93. There was no clear relationship between shared decision-making and satisfaction scores. CONCLUSIONS: Although adults and parents of children with vascular malformations express a strong desire for shared decision-making, objective shared decision-making behavior is still lacking, most likely because of poor awareness of the shared decision-making concept among patients, parents, and physicians. To improve shared decision-making practice, targeted interventions (e.g., decision aids, staff training) are essential.

Laser Guidance in C-Arm Cone-Beam CT-Guided Radiofrequency Ablation of Osteoid Osteoma Reduces Fluoroscopy Time.

PURPOSE: To assess whether laser guidance can reduce fluoroscopy and procedure time of cone-beam computed tomography (CBCT)-guided radiofrequency (RF) ablations of osteoid osteoma compared to freehand CBCT guidance. MATERIALS AND METHODS: 32 RF ablations were retrospectively analyzed, 17 laser-guided and 15 procedures using the freehand technique. Subgroup selection of 18 ablations in the hip-pelvic region with a similar degree of difficulty was used for a direct comparison. Data are presented as median (ranges). RESULTS: Comparison of all 32 ablations resulted in fluoroscopy times of 365 s (193-878 s) for freehand and 186 s (75-587 s) for laser-guided procedures (p = 0.004). Corresponding procedure times were 56 min (35-97 min) and 52 min (30-85 min) (p = 0.355). The subgroup showed comparable target sizes, needle path lengths, and number of scans between groups. Fluoroscopy times were lower for laser-guided procedures, 215 s (75-413 s), compared to 384 s (193-878 s) for freehand (p = 0.012). Procedure times were comparable between groups, 51 min (30-72 min) for laser guidance and 58 min (35-79 min) for freehand (p = 0.172). CONCLUSION: Adding laser guidance to CBCT-guided osteoid osteoma RF ablations significantly reduced fluoroscopy time without increasing procedure time. LEVEL OF EVIDENCE: Level 4, case series.

The Use of Laser Guidance Reduces Fluoroscopy Time for C-Arm Cone-Beam Computed Tomography-Guided Biopsies.

PURPOSE: When using laser guidance for cone-beam computed tomography (CBCT)-guided needle interventions, planned needle paths are visualized to the operator without the need to switch between entry- and progress-view during needle placement. The current study assesses the effect of laser guidance during CBCT-guided biopsies on fluoroscopy and procedure times. MATERIALS AND METHODS: Prospective data from 15 CBCT-guided biopsies of 8-65 mm thoracic and abdominal lesions assisted by a ceiling-mounted laser guidance technique were compared to retrospective data of 36 performed CBCT-guided biopsies of lesions >20 mm using the freehand technique. Fluoroscopy time, procedure time, and number of CBCT-scans were recorded. All data are presented as median (ranges). RESULTS: For biopsies using the freehand technique, more fluoroscopy time was necessary to guide the needle onto the target, 165 s (83-333 s) compared to 87 s (44-190 s) for laser guidance (p < 0.001). Procedure times were shorter for freehand-guided biopsies, 24 min versus 30 min for laser guidance (p < 0.001). CONCLUSION: The use of laser guidance during CBCT-guided biopsies significantly reduces fluoroscopy time.

Endovascular versus surgical revascularization in proximal subclavian artery obstruction.

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<0.05). Primary patency was 72% and 89% at 1 year or 54% and 55% at 5 years for the endovascular and open surgical groups, respectively (log rank 0.210, P=0.65). Assisted primary patency was 77% and 100% at 1 year or 67% and 67% at 5 years, respectively (log rank 0.528, P=0.47). There was no mortality and major complications were infrequent, occurring equally in both groups (P=0.22). CONCLUSIONS: Although with its less invasive character endovascular treatment has gained preference over surgical treatment of proximal subclavian obstruction in many cases, extrathoracic surgical reconstruction can be performed with a higher technical success rate, similar patency and a comparable number of complications.

Preoperative CT-Angiography Predicts Ex Vivo Vein Length for Right Kidneys After Laparoscopic Donor Nephrectomy.

BACKGROUND Implantation of a kidney with a short renal vein is technically more challenging and therefore prone for technique-related complications. It remains unclear whether pre-operative computed tomography angiography (CTA), to assess vascular anatomy of the donor kidney, can be used to predict renal vein length. MATERIAL AND METHODS Right and left renal vein lengths of 100 consecutive kidney donors were measured in an oblique-coronal plane multiplanar reconstruction image of 100 consecutive kidney donors in whom ex vivo vein length was measured after recovery. In a second retrospective cohort of 100 consecutive kidney donors donating a right kidney, preoperative CTA vein length measurements were correlated to anastomosis time and early graft outcome. RESULTS Left and right renal vein lengths, measured on CTA, were 43.2 mm and 30.0 mm, respectively. No correlation was found between CTA and ex vivo measurements for the left renal vein (p=.610), whereas a significant correlation was found for the right renal vein (p=.021). In the retrospective cohort, right renal vein length was significantly correlated with the anastomosis time but not with early graft outcome. CONCLUSIONS The length of the right, but not the left, renal vein can be predicted by preoperative CTA, but this does not hold true for the left renal vein.

Image Registration of Cone-Beam Computer Tomography and Preprocedural Computer Tomography Aids in Localization of Adrenal Veins and Decreasing Radiation Dose in Adrenal Vein Sampling.

PURPOSE: We assessed whether image registration of cone-beam computed tomography (CT) (CBCT) and contrast-enhanced CT (CE-CT) images indicating the locations of the adrenal veins can aid in increasing the success rate of first-attempts adrenal vein sampling (AVS) and therefore decreasing patient radiation dose. MATERIALS AND METHODS: CBCT scans were acquired in the interventional suite (Philips Allura Xper FD20) and rigidly registered to the vertebra in previously acquired CE-CT. Adrenal vein locations were marked on the CT image and superimposed with live fluoroscopy and digital-subtraction angiography (DSA) to guide the AVS. Seventeen first attempts at AVS were performed with image registration and retrospectively compared with 15 first attempts without image registration performed earlier by the same 2 interventional radiologists. First-attempt AVS was considered successful when both adrenal vein samples showed representative cortisol levels. Sampling time, dose-area product (DAP), number of DSA runs, fluoroscopy time, and skin dose were recorded. RESULTS: Without image registration, the first attempt at sampling was successful in 8 of 15 procedures indicating a success rate of 53.3%. This increased to 76.5% (13 of 17) by adding CBCT and CE-CT image registration to AVS procedures (p = 0.266). DAP values (p = 0.001) and DSA runs (p = 0.026) decreased significantly by adding image registration guidance. Sampling and fluoroscopy times and skin dose showed no significant changes. CONCLUSION: Guidance based on registration of CBCT and previously acquired diagnostic CE-CT can aid in enhancing localization of the adrenal veins thereby increasing the success rate of first-attempt AVS with a significant decrease in the number of used DSA runs and, consequently, radiation dose required.

Effectiveness of sclerotherapy, surgery, and laser therapy in patients with venous malformations: a systematic review.

PURPOSE: Because the best possible treatment for venous malformations is unclear, this study systematically reviews the available literature regarding the effectiveness of different treatment options for the patient group. Venous malformations result from incorrect development of the veins during embryogenesis and are present at birth. Venous malformations may exhibit symptoms, such as pain, swelling, and inflammation of the vessel. MATERIALS AND METHODS: A systematic literature search in PubMed and Embase was performed. Data regarding the design, participants, intervention and, treatment outcome (success and complications) were extracted. The validity of the studies was assessed with the Cochrane Collaboration's risk of bias tool. RESULTS: Thirty-five studies were identified studying the effectiveness of eight treatments: sclerotherapy/embolization with ethanol, gelified ethanol, bleomycin, polidocanol, sodium tetradecyl sulfate (STS), Ethibloc, surgery, and laser therapy. All of the included studies have a high or unclear risk of bias. The average biased reported success rates for ethanol, gelified ethanol, bleomycin, polidocanol, STS, Ethibloc, surgery, and laser therapy were 74, 89, 88, 90, 86, 65, 90, and 94 %, respectively. CONCLUSION: Until more valid evidence is available, the choice for treatment remains a shared decision between the patient and a multidisciplinary treatment group. From a cost perspective, sclerotherapy with STS or polidocanol should be the treatment of choice.

Persisting iliaco-caval fistula after EVAR maintained by a type II endoleak.

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.

Is there still a place for adrenal venous sampling in the diagnostic localization of pheochromocytoma?

Our objective is to outline the utility of adrenal venous sampling (AVS) with measurements of metanephrine to normetanephrine ratios for diagnostic localization of phaeochromocytoma in a patient with normal plasma levels of catecholamines. A 53-year-old-woman was referred for evaluation of recurrent pheochromocytoma following a right adrenalectomy 14 years earlier. Diagnosis of recurrent disease was established from elevations in plasma metanephrines with normal levels of catecholamines. Magnetic resonance imaging indicated two 1-2 cm masses in the right surgical bed and another 1-1.5 cm mass in the left adrenal. These masses were negative on (123)I-metaiodobenzylguanidine scintigraphy. There was no evidence of a hereditary syndrome. We, therefore, carried out two investigational approaches to identify the tumor masses. Hybrid positron emission tomography/computed tomography with (68)Ga-DOTATOC ((68)Ga-DOTATOC-PET/CT) confirmed the presence of recurrent disease in the right surgical bed and also suggested additional left adrenal involvement. Normal plasma catecholamines precluded the use of adrenal venous sampling (AVS) with catecholamine measurements. Hence, we performed AVS with measurements of plasma metanephrines, which were 4- to 7-fold higher in the left adrenal vein than in central venous plasma. We observed a reversal of the normally high metanephrine to normetanephrine ratio (mean value ± SD 5.28 ± 1.86; range 3.36-8.84, n = 13) to 0.73, establishing the presence of a left adrenal pheochromocytoma. Surgical pathology confirmed bilateral disease. This case highlights a scenario where a combination of (68)Ga-DOTATOC-PET/CT and AVS with measurement of the metanephrine to normetanephrine ratio was crucial for the preoperative assessment of a patient with bilateral pheochromocytoma.