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Background: Percutaneous ethanol sclerotherapy (PES) is the primary tool in the treatment of venous malformations (VM). However, PES has known serious complications. This study is aimed at identifying predictors of good response to PES in patients with VM to improve patient selection., Methods: We performed a retrospective, cross-sectional study of 158 VM patients (mean age, 14.3 years, male 42%) who underwent ethanol sclerotherapy at a specialized vascular malformation center. For clinical result assessment, patients or parents in pediatric patients answered questions on symptomatic, functional, and cosmetic improvement after PES. In each category, the possible choices were markedly improved, moderately improved, no change, moderately worse, or markedly worse compared with pretreatment status. A "good response" was defined as one or more areas of marked improvement on the self-assessment in conjunction with marked improvement on post-treatment images (> or =30% decrease in maximal diameter of VM on magnetic resonance imaging [MRI] or > or =50% decrease in abnormal blood pool ratio on whole body blood pool scintigraphy [WBBPS] compared with pretreatment images). To determine predictors of a good response to PES, uni- and multivariate analysis were conducted on demographics (age, gender), clinical features of VM (location, size, depth of involved tissue, presence of associated lymphatic malformation, MRI findings; well-defined vs ill-defined margin, characteristics of venous drainage during PES) and treatment variables (number of PES sessions, maximal concentration and dosage of ethanol used in PES, adjuvant therapy)., Results: Symptomatic, functional, and cosmetic improvement was 28%, 27%, and 34%, respectively, based on patient questionnaires. Based on imaging studies, 42 patients (27%) had markedly improvement. Composite outcome combining questionnaire results and imaging study showed that 16% of patients had a "good response". On multivariate analysis, female gender (odds ratio [OR]: 4.49, 95% confidence interval [CI]: 1.24-16.28), no or delayed visualization of drainage vein (OR: 9.22, 95% CI: 1.79-47.51), and a well-defined margin on MRI (OR: 13.38, 95% CI: 2.84-63.12) were independent predictors of "good response" to PES., Conclusions: PES should be performed in selected patients in order to obtain the best outcomes and minimize complications. No or delayed visualization of drainage vein on initial direct puncture venogram, a well-defined margin on MRI, and female gender were statistically significant predictors of a "good response" to PES and may be useful in selecting patients.

Objectives: This study was conducted to determine the clinical risk factors for the development of leg length discrepancy (LLD) in patients with congenital vascular malformation (CVM) affecting the lower extremity., Methods: A retrospective analysis was conducted of a prospectively collected database that included 361 patients who underwent assessment of a CVM lesion from September 1994 to January 2005. We measured LLD using lower extremity scanograms of 229 patients who were suspected of having LLD on physical examination. The risk factor analysis for a clinically significant LLD (>2 cm) was performed with the variables of age, gender, features of CVM (type, extent and depth), and the deep vein status (agenesis, hypoplasia, phlebectasia) of the affected limb. Fisher's exact test and the chi(2) test were used for the univariate analysis, and a logistic regression test was performed for the multivariate analysis. Among the patients with LLD, we compared the overgrowth group and undergrowth group with the Fisher's exact test to identify differences between the two subgroups., Results: The included patients were 153 males (42%) and 208 females (57%) with a mean age of 20 +/- 14 years (range, 1 to 62). There were 157 patients (43%) in the still growing age group (age <15 years) and 204 (57%) were in the finished growing age group (age >15 years). The types of CVMs included 215 venous (60%), 43 arteriovenous (12%), 46 lymphatic (13%), and 57 (16%) combined venolymphatic malformations. On the lower extremity scanogram, 26 patients (7%) had a LLD of >2 cm due to overgrowth (n = 20) or undergrowth (n = 6) of the affected limbs. The univariate analysis showed that a venolymphatic malformation (P = .003) and a whole leg CVM (P = .000) were significant risk factors for development of LLD. However, the multivariate analysis identified the whole leg CVM lesion as a single independent risk factor for LLD (P = .004; odds ratio, 6.512, 95% confidence interval, 1.788 to 23.713). On subgroup analysis, a whole leg CVM was also identified as a risk factor for overgrowth of the affected limb. In a comparison between two subgroups of LLD (overgrowth v. undergrowth), overgrowth was significantly (P = .022) more common in female than in male patients., Conclusions: As a clinical risk factor for development of LLD, the extent of the CVM lesion was a single independent risk factor regardless of the type or depth of the CVM lesion. In addition, our data suggest that overgrowth or undergrowth of the affected limb, as a cause of LLD, might be related to gender.

Objective: Primary Budd-Chiari syndrome (BCS) is a rare form of hepatic venous outflow obstruction at the suprahepatic inferior vena cava (IVC), the hepatic veins, or both. We assessed our 4-year experience in the management of BCS to evaluate the results of our methods of care., Methods: We conducted a retrospective review of a nonrandomized clinical trial conducted in three teaching hospitals. Among 28 primary BCS patients, 9 remained in medical treatment only, and 19 who failed to respond to medical treatment received additional endovascular (n = 17) or surgical therapy (n = 2). Nine underwent IVC balloon angioplasty alone, 6 had angioplasty plus stents, and 2 had transjugular intrahepatic portosystemic shunts (TIPS) for hepatic vein lesions. One patient had a mesoatrial bypass; another had liver transplantation. Immediate response to the therapy was assessed with angiography and ultrasonography based on anatomic and/or hemodynamic correction or reduction of the lesion. Subsequent assessment of portal hypertension status was made with periodic clinical and laboratory evaluation (eg, ultrasonography, liver biopsy)., Results: Twenty-six patients had had IVC stenosis or occlusion by focal or segmental lesion. Two patients had hepatic vein outlet obstruction. There was no evidence of coagulopathy as the pathogenesis; all were related to membranous obstruction of the vena cava. Excellent immediate response to the endovascular therapy and subsequent relief of portal hypertension were achieved in 14 patients. Four patients had restenosis or progression of the residual lesion within 2 years; three responded to repeated stenting. Primary patency was 76.5%, and primary assisted patency was 94.1%. Two patients with TIPS and two with surgical therapy maintained excellent results. The medical treatment remained effective only in a limited group of 6 (21.4%) of the 28 patients., Conclusions: In BCS, both endovascular and surgical interventions provide excellent results and potentially halt liver parenchymal deterioration caused by portal hypertension. Liver transplantation remains the ultimate solution for advanced liver failure.

Background: Management of arteriovenous malformations (AVMs) remains challenging because of their unpredictable behavior and high recurrence rate. A multidisciplinary approach based on a new classification scheme and improved diagnostic techniques may improve their management. The purpose of this study was to review our experience with combined embolotherapy, sclerotherapy (embolo/sclerotherapy), and surgical procedures to manage AVMs., Methods: A total of 797 patients with congenital vascular malformations (January 1995 through December 2001) was investigated with noninvasive studies. Once an AVM was diagnosed, all underwent angiographic confirmation as a roadmap for treatment. Embolo/sclerotherapy and surgical procedures were instituted by the multidisciplinary team with periodic follow-up per protocol. Seventy-six patients with AVMs were reviewed retrospectively to assess the diagnosis and management by a multidisciplinary approach., Results: Seventy-six (9.5% of all CVM) patients had AVMs, mostly infiltrating, extratruncular form (61/76). Embolo/sclerotherapy with various combinations of absolute ethanol, N-butyl cyanoacrylate (NBCA), contour particles, and coils were used in 48 patients. Sixteen patients with surgically accessible localized lesions completed preoperative embolism and sclerotherapy through 24 sessions, with subsequent surgical excision with minimal morbidity. Interim results were excellent, with no evidence of recurrence in all 16 patients with a mean follow-up of 24 months. Thirty-two patients with surgically inaccessible lesions (infiltrating) were treated with embolism and sclerotherapy alone. There were nine failures in a total of 171 sessions. Interim results with a mean of 19 months' follow-up of embolism and sclerotherapy alone were excellent in the majority (25/32) and good to fair among the rest (7/32). However, 31 complications, mostly minor (27/31), occurred in 30 sessions. Four major complications occurred, including facial nerve palsy, pulmonary embolism, deep vein thrombosis, and massive necrosis of an ear cartilage., Conclusions: Diagnosis and management of AVMs by a multidisciplinary approach that integrates surgical therapy with embolism and sclerotherapy appears to improve the results and management with limited morbidity and no recurrence during early follow-up.

Purpose: This paper is an update of previously published data on the basis of a retrospective review of midterm results of ethanol sclerotherapy on 87 patients (January 1995 to December 2000) for assessment of its efficacy as an improved treatment method for venous malformation (VM). According to this assessment, VMs were defined with a new classification and studied with advanced diagnostic technology and an advanced care system., Methods: The average follow-up period was 24 months after completion of a multisession treatment (mean, 8.2 months). Classification of VM was based on a modification of the Hamburg classification. Advanced diagnostic technology, mostly noninvasive, was used on 226 of 520 patients with congenital vascular malformation registered at the Congenital Vascular Malformation Clinic at the Samsung Medical Center. Of the 226 patients with VM, 87 with infiltrating extratruncular lesions had a total of 399 sessions of sclerotherapy. Follow-up assessment with periodic clinical examinations by the multidisciplinary team was supplemented with body blood pool scans, duplex scans, and magnetic resonance imaging, according to protocol, once the multisession therapy was completed. Angiographic assessment was seldom included. The endpoint of this phase II study was 24 months., Results: Of 399 sessions, initial success was seen in 379 sessions (95.0%) and failure was seen in 20 sessions (5%). This was mostly caused by forced abandonment from technical difficulty in delivering ethanol safely to the lesion (eg, direct drainage of VM into normal deep vein system). Later results after completion of the multisession therapy with a minimum follow-up of 24 months on 71 VMs have shown no evidence of recurrence. Eighty-seven patients have shown the same results without recurrence on an average of 18.2 months of follow-up. Fifty-one minor to major complications, mostly skin damage, developed after 47 sessions among the 379 sessions (12.4% in 24/87 patients; 27.9%). However, complications resolved spontaneously or were managed successfully, except for one permanent facial nerve palsy and one peroneal nerve palsy., Conclusion: Absolute ethanol sclerotherapy can deliver excellent results as an independent therapy to the infiltrating type of extratruncular form of VM, which was once taboo because of prohibitively high morbidity. Absolute ethanol may be accepted as an effective treatment method because no recurrence has been observed in the relatively long-term observation period and the morbidity has been acceptable. However, it should be reserved only for individuals and centers with expertise. The morbidity involved should be clearly understood and accepted by the patient or family, and the risk of acute and chronic complications, both major or minor, should be explained to the patient. Long-term assessment of the complication's sequelae is warranted.

Background: Complex forms of congenital venous malformation have defied proper classification and confounded therapy. Through a newly designed multidisciplinary approach, these venous defects were properly diagnosed and classified according to the Hamburg classification. Absolute ethanol was adopted as a new scleroagent for this complex form of venous defects to improve overall treatment results with acceptable morbidity and recurrence rates., Patients and Methods: Among 318 patients aged 2 months to 60 years (130 men: mean age, 24.6 years; 188 women: mean age, 24.5 years), 143 patients were confirmed as having venous defects predominantly (45%). We conducted diagnostic evaluation with magnetic resonance imaging, whole body blood pool scan, duplex scans, transarterial microalbumin lung scans, air plethysmography, bone x-ray film, and angiography. Thirty of 143 patients were indicated for the absolute ethanol sclerotherapy for the complex form of venous defects, and they completed 98 sessions of multistage therapy with direct puncture technique. Follow-up assessment (minimum, 6 months; average, 10.2 months) was made as early results after completion of therapy., Results: The immediate success rate at the completion of treatment through 98 sessions on 30 patients was 92% (90/98). The reason for the failure of 8% (8/98 sessions) was mainly due to the lesion's inaccessibility to the nidus to deliver the alcohol safely. On follow-up assessment available on 28 of 30 patients, overall improvement of the lesions with good to fair response on clinical assessment was obtained in 27 (96%) of 28 patients. Similar good to fair responses were obtained on whole body blood pool scan assessment (14 [93%] of 15 patients) and also on magnetic resonance imaging assessment (12 [93%] of 13 patients). Various major to minor acute complications developed during the procedure in eight (26.7%) of 30 patients through 16 (16.3%) of 98 sessions of the therapy: ischemic bullae, tissue fibrosis, deep venous thrombosis, pulmonary embolism, peripheral nerve palsy, and temporary pulmonary hypertension. All 24 incidences of complication were successfully managed with full recovery except one case of permanent peroneal nerve palsy that was present during 18 months of follow-up. No single recurrence has been detected to date., Conclusion: Absolute ethanol sclerotherapy alone can deliver excellent results in complex forms of venous malformations with considerable but acceptable morbidity and may be able to reduce the morbidity involved with the conventional surgical therapy alone on complex forms of venous malformation. No recurrence or deterioration of the therapy results was observed during the follow-up period (average, 10.2 months) after the completion of multistaged therapy.