Consensus Document of the International Union of Angiology (IUA)-2013. Current concept on the management of arterio-venous management.

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.

Ethanol endovascular management of brain arteriovenous malformations: initial results.

OBJECTIVE: The goal was to determine the safety and efficacy of absolute ethyl alcohol treatment in the management of intra-axial brain arteriovenous malformations (AVMs). METHODS: Seventeen patients (eight female and nine male patients; mean age, 41 yr) underwent ethanol endovascular therapy for treatment of their brain AVMs. Superselective amytal testing preceded all procedures. Neuroleptic intravenous anesthesia was used for 16 patients, and general anesthesia was used for 1 patient. Follow-up monitoring consisted of clinical evaluations, magnetic resonance imaging, and arteriography. RESULTS: In follow-up evaluations (mean follow-up period, 13 mo) after embolization of brain AVMs, neither vascular recanalization nor the neovascular recruitment phenomenon was observed in any patient. Progressive AVM thrombosis at arteriographic follow-up evaluation was a constant feature. Seven patients were cured of their AVMs with ethanol endovascular therapy alone. Three patients were cured of their lesions with ethanol embolization plus surgical resection. One patient was cured of his lesion with ethanol embolization and radiation therapy of the residual nidus. Three patients underwent only partial therapy, with significant improvement in symptoms. Three patients are currently undergoing ethanol endovascular therapy. Complications occurred with 8 of 17 patients, most of which were transient. Two patients died because of late subarachnoid hemorrhages, one patient 4 months and one patient 14 months after partial therapy. CONCLUSION: Progressive and permanent AVM occlusion is a common finding in arteriographic follow-up evaluations. In no patients did arterial recanalization or the neovascular recruitment phenomenon occur. Our initial results indicate that ethanol has a permanence that is seldom encountered with other embolic agents. With aggressive decadron therapy, the complications related to swelling in the brain are largely reversible.

Recognition and treatment of venous malformations of the rectum.

Venous malformations of the rectum are uncommon lesions that present complex management problems (1-6). The vast majority of these lesions present with rectal bleeding in infancy or childhood. Many cases have been treated as colitis for years before the correct diagnosis was made. The correct diagnosis has generally been based on gross appearance, confirmed subsequently by plain radiographs and angiography. Heroic surgical intervention has been the only repeatedly reported "cure" in the literature. One patient has been reported who did well for 20 years with sclerosis of the hemorrhoidal vein at surgery followed by intermittent transrectal sclerotherapy (7,8). Another patient would appear to have had longterm success with radiation therapy (9-11). We report four new cases of venous malformations of the rectum and results to date of a new therapeutic option with transcutaneous ethanol sclerotherapy in two of these patients. A discussion of alternate methods of treatment is included.

How I do it. Arteriovenous malformation management.

Arteriovenous malformations (AVM) are rare vascular lesions that can present with a myriad of clinical presentations. In our institutions, initial workup consists of a clinical exam, color Doppler imaging, and magnetic resonance imaging. After the initial noninvasive workup, arteriography, at times closed system venography, and ethanol endovascular repair of the AVM is performed under general anesthesia. Depending on the size of the lesion, additional Swan-Ganz line and arterial line monitoring are performed. Patients are usually observed overnight and uneventfully discharged the following day if no complication occurs. Patients are followed at periodic intervals despite cure of their lesion. Long-term follow-up is essential in AVM management.

Embolotherapy: agents, clinical applications, and techniques.

For embolization to be successful, three factors must be addressed: embolic agent selection, clinical application, and technical skill. The major embolic agents used include stainless steel coils, absorbable gelatin pledgets and powder, polyvinyl alcohol foam, ethanol, and glues. Each of these agents acts at different levels in the arterial system; for example, coils are equivalent to surgical ligation and occlude medium to small arteries, whereas liquid agents and the smaller diameter particles occlude at the arteriolar level or the capillary bed. The type of agent selected should also be determined according to clinical application, which includes trauma, tumors, male infertility, impotence, and vascular malformations. It may be better to occlude an artery only temporarily, particularly in trauma patients, and absorbable gelatin material is preferred for this application. Conversely, permanent occlusion of arteries with either ethanol or polyvinyl alcohol foam particles may be necessary in the treatment of tumors. To use embolotherapy effectively, the interventional radiologist must be experienced, familiar with the underlying pathologic processes, and knowledgeable with regard to the role of other specialties in the treatment of the disease process presented.

US-guided automated large-core breast biopsy.

To evaluate the efficacy of ultrasound (US)-guided automated large-core percutaneous needle breast biopsy, biopsy of 181 ultrasonographically suspicious breast lesions was performed by using a long-throw biopsy gun and 14-gauge needles with continuous US guidance. A "freehand" technique with either 5.0- or 7.5-MHz linear-array transducers was used. Needle core diagnoses were compared with surgical diagnoses in the 49 lesions subsequently surgically excised. The remaining 132 cases were followed for 12-36 months. Agreement between needle-core and surgical diagnoses in the 49 lesions was 100%. This group included 34 cancers (28 infiltrating ductal, two mucinous, one mixed infiltrating ductal and lobular, and one each of intraductal, infiltrating lobular, and tubular carcinoma). To date, no cancers have been found in the other 132 cases. The procedure time averaged 20 minutes, and no complications occurred. With an accuracy approaching that of excisional biopsy, US-guided needle core biopsy provides an alternative to surgery.

Segmental stenosis of the renal artery: pattern recognition of tardus and parvus abnormalities with duplex sonography.

Segmental renal artery branches within the renal sinus were prospectively evaluated with color Doppler imaging and pulsed-Doppler spectral analysis in 56 patients before angiography. Waveforms were evaluated for the tardus and parvus abnormalities of prolonged acceleration time, diminished acceleration index, and loss of the normal early systolic compliance peak/reflective-wave complex (ESP). Findings obtained with these parameters were compared with the subsequent findings on angiograms to ascertain their efficacy in detection of hemodynamically significant (greater than or equal to 60%) renal arterial stenosis (RAS), which was present in 32 kidneys in 26 patients. Simple pattern-recognition analysis of ESP proved to be the best of the three parameters. Loss of ESP enabled identification of RAS with 95% sensitivity, 97% specificity, a 92% positive predictive value, a 98% negative predictive value, a 96% overall accuracy. On the basis of the high technical success rate, high sensitivity and specificity, and short examination time, waveform analysis for detection of tardus-parvus abnormalities, especially loss of ESP, of the segmental artery is recommended as an alternative to direct examination of the main renal arteries for evaluation of RAS.

MR imaging of symptomatic peripheral vascular malformations.

We performed a retrospective study of symptomatic peripheral vascular malformations to determine if MR imaging can be used to distinguish slow-flow venous malformations from high-flow arteriovenous malformations and arteriovenous fistulas. Twenty-seven MR examinations in 25 patients with malformations outside the CNS were reviewed. Sixteen venous malformations, nine arteriovenous malformations, and two arteriovenous fistulas were included. In all cases, the MR findings were correlated with the results of angiography. The distinction between slow-flow venous malformations and high-flow arteriovenous malformations and arteriovenous fistulas was made primarily on T2-weighted MR images, which showed high signal intensity in venous malformations and flow voids in high-flow lesions. In addition to the previously described MR features of venous malformations (serpentine pattern with septations, associated muscle atrophy, and typical T1 and T2 signal intensities), several new MR features were apparent. Venous malformations had a propensity for multifocal involvement (37%), orientation along the long axis of extremities or affected muscles (78%), and adherence to neurovascular distributions (64%). Prominent subcutaneous fat was commonly seen adjacent to the malformation. MR images of arteriovenous malformations and arteriovenous fistulas also commonly showed muscle atrophy and subcutaneous fatty prominence. Our results show that slow-flow venous malformations can be distinguished from high-flow arteriovenous malformations and fistulas on the basis of spin-echo MR signal characteristics. The associated imaging characteristics help in the differential diagnosis in problematic cases.

Nonpalpable breast lesions: stereotactic automated large-core biopsies.

One hundred two patients with mammographically suspicious, nonpalpable lesions underwent stereotactic breast biopsy with a biopsy gun and an automated 14-gauge cutting needle. After biopsy, a localization wire was placed and surgical biopsy performed. There was agreement of the histologic results from the gun biopsy and the surgical biopsy specimens in 98 cases (96%), including 22 of 23 carcinomas (96%) (kappa = 0.936). The gun biopsy yielded findings that led to the correct diagnosis in two cases involving lesions that were missed at surgical biopsy; two lesions found at surgery were missed at gun biopsy. The results of this study suggest that the use of 14-gauge needles improves agreement between surgical and needle core biopsy findings and that stereotactic biopsy with an automated needle and gun can be an acceptable alternative to surgical biopsy in women with mammographically suspicious breast lesions.

Anterior cruciate ligament reconstruction: evaluation with MR imaging.

Fifty magnetic resonance (MR) imaging examinations were performed in 37 patients after arthroscopic anterior cruciate ligament (ACL) reconstruction with patellar bone-tendon-tibial bone autografts. T1-weighted sagittal and axial images were obtained. In 34 patients with clinically stable ACL autografts, 43 of 47 MR examinations demonstrated a well-defined, intact ACL autograft. All three patients with ACL laxity failed to demonstrate a well-defined autograft, for an overall correlation between MR imaging and clinical examination results of 92%. Of the 12 patients who underwent second-look arthroscopy, 100% correlation was present between MR imaging and arthroscopic results. As in the nonreconstructed knee, buckling of the posterior cruciate ligament was suggestive of ACL laxity. MR imaging also documented optimum placement of bone tunnels in the femur and tibia. MR imaging has proved to be an excellent noninvasive imaging modality for evaluating ACL reconstruction, while also providing ancillary information about the postoperative knee.

Paranasal sinuses on MR images of the brain: significance of mucosal thickening.

One hundred twenty-eight patients were examined prospectively to determine the significance of mucosal thickening seen in the paranasal sinuses during routine MR imaging of the brain. On the basis of responses to a questionnaire, each patient was categorized as symptomatic (n = 60) or asymptomatic (n = 68) for paranasal sinus disease. Patients were categorized further on the basis of the maximal mucosal thickening seen by MR in any paranasal sinus. A modified t test was used to compare the prevalence of various degrees of mucosal thickening between symptomatic and asymptomatic groups. Statistically significant differences between the groups were seen only in those patients with normal sinuses and in those with 4 mm or more of mucosal thickening. We conclude that mucosal thickening of up to 3 mm is common and lacks clinical significance in asymptomatic patients. An ancillary finding is that 1- to 2-mm areas of mucosal thickening in the ethmoidal sinuses occur in 63% of asymptomatic patients. This minimal mucosal thickening in the ethmoidal sinuses is thought to be a normal variant, possibly a function of the physiologic nasal cycle.

Ethanol embolization of arteriovenous fistulas: a primary mode of therapy.

Arteriovenous fistulas (AVFs) can be posttraumatic or congenital vascular malformations. In the initial arteriographic evaluation, chronic AVFs potentially can be confused with arteriovenous malformations (AVMs). The authors studied five patients with a single AVF and one patient with numerous AVFs. Three patients had undergone surgery for treatment of their AVFs, one patient had undergone isobutyl-2-cyanoacrylate (IBCA) embolization, and two patients had undergone no prior therapy. The AVFs recurred in the three patients who had undergone surgery and in the patient who had undergone IBCA embolization. All patients underwent ethanol embolization of their AVFs. Angiograms obtained immediately after embolization documented closure of all AVFs. At follow-up, none of the embolized lesions have recurred. The authors conclude that ethanol embolotherapy can cure these problematic lesions. Extreme caution, however, must be employed with the use of intravascular ethanol because nontarget embolization can potentially result in tissue devitalization. In this study, two patients developed a small focal area of skin necrosis that did not require skin grafting and healed with conservative management.

Efficacy of automated biopsy guns versus conventional biopsy needles in the pygmy pig.

Debate over which biopsy needle is the best has intensified recently with the introduction of automated biopsy guns including the 18-gauge long-throw and short-throw Biopty, the 18-gauge Cook, and the 14- and 18-gauge Klear Kut. To evaluate the efficacy of these mechanized biopsy guns versus that of conventional manual biopsy needles in the acquisition of adequate tissue for histopathologic evaluation, open hepatic and renal biopsies were performed in 15 pygmy pigs. The specimens were evaluated separately in a double-blind fashion by two histopathologists using graded criteria. Overall, the best results were obtained with the manual 14-gauge Tru-Cut needle, the long-throw 18-gauge Biopty gun, and the 18-gauge Cook biopsy gun. By comparison, the aspiration-type needles did not perform as well when considered as a group. Several other needles scored well in the biopsy of either the liver or kidney, but not in both. Disappointing results were obtained with the Klear Kut guns (both 14- and 18-gauge) and the Vacu Cut and PercuCut needles.

Ethanol embolization of vascular malformations.

Absolute ethanol was used to treat 20 patients with symptomatic vascular malformations (SVMs) (ie, venous malformations, arteriovenous malformations, and congenital and posttraumatic arteriovenous fistulas) in whom previous surgery or standard embolotherapy had failed or who were not candidates for surgery. All large complex lesions required multiple embolizations as staged procedures. Immediate thrombosis was achieved in all patients; complications (13% of cases) were generally minor and were treated conservatively. Follow-up studies, performed in 19 of 20 patients, showed persistent occlusion of the SVM in all cases. Ethanol embolization of SVMs, performed according to strict techniques, has proved efficacious in SVM management and is emerging as a definitive form of therapy.

Stereotactic breast biopsy with a biopsy gun.

One hundred three patients underwent stereotactic breast biopsy with an 18-, 16-, or 14-gauge cutting needle and a biopsy gun. After biopsy, a localization wire was placed and surgical biopsy performed. There was agreement of the histologic results in 89 cases (87%) including 14 of 16 cancers (87%) (kappa = 0.806). The gun biopsy yielded the correct diagnosis in four cases involving a lesion (including one cancer) that was missed at the surgical biopsy. Nine cases in which the lesion was missed at gun biopsy can be related to insufficient needle size, the greater difficulty in using one of the two stereotactic devices, and early inexperience with the technique. A 14-gauge needle was used in the last 29 biopsies, the results of which agreed with the surgical pathologic findings in 28 cases (97%). With greater experience, stereotactic-guided large-gauge automated percutaneous biopsy may prove to be an acceptable alternative to surgical biopsy in women with breast masses suspected at mammography.

The posterior intercostal approach for percutaneous renal procedures: risk of puncturing the lung, spleen, and liver as determined by CT.

A posterior intercostal approach is commonly used for percutaneous access to the upper poles of the kidney. However, the safety of this approach with respect to puncturing the intervening lung, pleura, liver, and spleen with the needle has been inferred only from a small series of patients without regard to the degree of respiration. To determine the possibility of puncturing these structures, we performed CT at both maximal inspiration and expiration and with sagittal reconstructions in 43 (27 supine and 16 prone) randomly selected patients. With expiration, the needle path was such that there was little risk to the spleen and liver from an 11th-12th posterior intercostal approach. However, the chance of transgressing the lung with this approach to the kidney was 29% on the right and 14% on the left. If done during maximal inspiration, the lung would be in the path of the needle in most patients. With a 10th-11th rib posterior intercostal approach, the chance of puncturing the lung was excessive regardless of the degree of respiration used. Our results show that the primary risk from a posterior 11th-12th rib intercostal approach to the upper renal collecting system is puncture of intervening lung, a complication that can be expected to occur in from 14% to 29% of patients. The risks from a posterior 10th-11th rib intercostal approach appear prohibitive.

Percutaneous transluminal aortic angioplasty: techniques and results.

Percutaneous transluminal angioplasty of the infrarenal abdominal aorta has been reported by a few authors. In the present series, aortic stenoses in 32 patients were treated with various percutaneous angioplasty techniques. Isolated aortic stenoses and primary aortic stenoses extending into the iliac arteries were successfully dilated. The initial success rate was 100%, without evidence of rupture, thrombosis, dissection, or distal embolization. In only three of the 28 patients who returned for follow-up did symptoms recur or noninvasive vascular laboratory indexes deteriorate (mean follow-up, 25 months). Percutaneous transluminal aortic angioplasty has proved safe and efficacious in the treatment of atherosclerotic aortic stenoses.

Image-directed percutaneous biopsies with a biopsy gun.

Core tissue for histologic study is believed by many pathologists to be more diagnostic than material from needle aspiration. Recently, a biopsy "gun" has been introduced, which simplifies core biopsies. With this device, 182 biopsies of multiple anatomic sites were performed with ultrasonic, computed tomographic, and fluoroscopic guidance and 18-gauge needles. High-quality histopathologic specimens were obtained in 177 of the biopsies, and diagnostic target tissue was obtained in 167. Only three significant complications occurred: one bleeding complication that required transfusion and two cases of pneumothorax that necessitated placement of chest tubes. The biopsy gun eliminated the disjointed movements of conventional "skinny" needle biopsies, and none of the samples demonstrated significant "crush" artifact or obscuring blood, problems that are commonly associated with manual biopsy techniques. Patient discomfort was decreased with this system compared with that of manual biopsies, and the total procedure time was reduced. Because of these distinct advantages, the authors now use the biopsy gun exclusively for all percutaneous biopsies and recommend that other institutions consider the use of this biopsy method.