Venous malformations.

The often inexorable growth and expansion of congenital vascular malformations can result in substantial morbidity and, in some cases, premature death of these patients. Despite this, patients suffering from such lesions are often erroneously diagnosed and/or inadequately treated, due to a lack of expertise among primary care practitioners as well as specialists. Venous malformations are the most common type of congenital vascular malformations. Over the last two decades management of these lesions has significantly improved, predominantly due to the introduction and implementation of multidisciplinary team concept as well as improvement in diagnostic and treatment modalities. Relatively recently genetic studies are providing more insights into underlying pathophysiological mechanisms responsible for the development and progression of venous malformations and pharmacotherapy is becoming extensively evaluated for safety and efficacy in the treatment of these often challenging vascular lesions.

Safety and efficacy of foam sclerotherapy for treatment of low-flow vascular malformations in children.

OBJECTIVE: Congenital vascular malformations are a heterogeneous group of lesions with the potential to cause significant lifelong morbidity in children. Diagnosis and treatment of these lesions may be complex and require a multidisciplinary approach. Sclerotherapy is widely used for the treatment of low-flow vascular malformations (LFVMs) as an alternative to surgical resection in adults; however, limited data of its use in a pediatric setting are available. The purpose of this study was to evaluate the efficacy and safety of sclerotherapy for pediatric LFVMs. METHODS: In this retrospective study, we reviewed our multidisciplinary vascular malformations team database for all patients younger than 18 years treated for congenital vascular malformations from 2008 to 2017. Of these, patients with LFVM treated with foam sclerotherapy were included. Dynamic contrast-enhanced magnetic resonance imaging was used to select patients for sclerotherapy by the multidisciplinary team. Foam sclerotherapy was performed with either polidocanol or sodium tetradecyl sulfate. Patients' characteristics, including demographics, presenting symptoms, and anatomic location of malformation, were assessed. Outcomes included treatment response, number of procedures, and postprocedural complications. RESULTS: The 61 patients with 61 LFVMs included 27 boys (44.3%) and 34 girls (55.7%), with mean age of 10.3 years (standard deviation, ± 5.3 years). The cohort included 32 venous (52.5%), 16 lymphatic (26.2%), and 8 mixed venous and lymphatic (13.1%) malformations along with 5 (8.2%) associated with Klippel-Trénaunay syndrome. Primary indications for intervention included pain and swelling (n = 12 [19.6%]), pain alone (n = 23 [37.7%]), swelling alone (n = 15 [24.6%]), functional impairment (n = 8 [13.1%]), and bleeding (n = 3 [4.9%]). Anatomic distributions varied, with 13 head and neck (21.3%), 5 truncal (8.2%), 10 upper extremity (16.4%), 27 lower extremity (44.3%), and 6 diffuse (9.8%). Among the head and neck lesions, 8 (13.1%) extended to the face; and of the extremity lesions, 5 (8.2%) extended to the hand and 17 (27.9%) to the foot. Overall, sclerotherapy resulted in significant improvement or complete resolution of symptoms in 53 patients (86.9%). Complications were observed in seven patients (11.4%); six cases (9.8%) of superficial skin ulceration resolved without intervention, and one infection (1.6%) required antibiotics. No patients experienced adverse hemodynamic consequences or venous thromboembolism. CONCLUSIONS: This series of pediatric LFVMs, the largest of its kind to date, demonstrates that sclerotherapy with foam-based agents effectively reduces symptoms with an acceptable rate of complications. Further study is needed to determine the optimal sclerosing agents for individual subsets of LFVMs in the pediatric population.

Multidisciplinary treatment of extremity arteriovenous malformations.

Congenital vascular malformations (CVMs) are a complex group of lesions that arise by embryologic dysmorphogenesis without increased endothelial proliferation that leads to structural and functional anomalies of the vascular system characterized by a wide range of presenting symptoms and often unpredictable clinical course. A recent advancement in the diagnostic and treatment modalities has resulted in a better understanding of the pathophysiology and natural history of CVMs and improved management of these lesions. The multidisciplinary approach and diagnostic algorithm used to distinguish high-flow (HFVM) from low-flow vascular malformations (LFVM) have been validated as clinically applicable for making an accurate anatomic and hemodynamic diagnosis of CVMs; they serve as a basis for proper treatment selection and significantly facilitate communication among different medical specialists. Dynamic contrast-enhanced magnetic resonance imaging is able to definitively distinguish HFVM from LFVM with accuracy of approximately 84%. In inconclusive cases, confirmatory angiography is required. Symptomatic, diffuse, extensive, macrocystic LFVMs and LFVMs that involve multiple tissue planes and vital structures are best treated with foam sclerotherapy. Primary surgical resection is the treatment of choice for localized, septated, and microcystic LFVMs. The management of HFVMs is characterized by multimodal treatment including preoperative embolization followed by complete surgical resection or sclerotherapy of the remaining venous component. Treatment of extensive CVMs is palliative and goal oriented. Implementation of the proposed diagnostic protocols and therapeutic algorithms in a multidisciplinary setting results in favorable outcomes with acceptable complication rates in this challenging patient population.

Emerging role of contrast-enhanced MRI in diagnosing vascular malformations.

Vascular malformations comprise a diverse and rare group of lesions which generally pose a formidable treatment challenge. Requisite for optimal surgical planning are imaging modalities capable of delineating involved anatomy and malformation flow characteristics. In this regard, we and others have purported the advantages of contrast-enhanced MRI. Here, we review the current body of literature regarding the emerging of role of contrast enhanced MRI for the management of vascular malformations.

Genomics of varicose veins and chronic venous insufficiency.

Recent sequencing of the human genome has opened up new areas of investigation for genetic aberrations responsible for the pathogenesis of many human diseases. To date, there have been no studies that have investigated the entire human genome for the genetic underpinnings of chronic venous insufficiency (CVI). Utilizing Gene Chip Arrays we analyzed the relative expression levels of more than 47,000 transcripts and variants and approximately 38,500 well-characterized genes from each of 20 patients (N (CVI)=10; N (Control Group)=10). Relative gene expression profiles significantly differed between patients with CVI and patients unaffected by CVI. Regulatory genes of mediators of the inflammatory reaction and collagen production were up-regulated and down-regulated, respectively in CVI patients. DNA microarray analysis also showed that relative gene expression of multiple genes which function remains to be elucidated was significantly different in CVI patients. Fundamental advancements in our knowledge of the human genome and understanding of the genetic basis of CVI represents an opportunity to develop new diagnostic, prognostic, preventive and therapeutic modalities in the management of CVI.

Analysis of the treatment of congenital vascular malformations using a multidisciplinary approach.

BACKGROUND: Vascular malformations are a rare and complex group of lesions which may present serious pitfalls in diagnosis and management. We sought to evaluate the efficacy and safety of our imaging protocol and therapeutic algorithm in the treatment of low-flow and high-flow vascular malformations in a large series of patients. METHODS: A prospective database of all patients treated by the multidisciplinary vascular malformation team at our institution between 2006 and 2011 was reviewed. Management decisions were based on patients' clinical profile as well as critical lesion characteristics, and included conservative care, sclerotherapy, embolization, surgical resection, or a combination of these modalities. Treatment goals and expectations were established by the patient and physician at the time of initial evaluation. An outcomes grading system based on patient- and physician-derived treatment goals and assessment of response to management was applied (1 = worse; 2 = unchanged, 3 = significantly improved, 4 = completely resolved), and postprocedural complications were identified. RESULTS: The 136 vascular malformations in 135 patients included 59 (43.7%) males and 76 (56.3%) females, ranging in age from under 1 year to 68 years (mean, 25.3 ± 17.0 years). In order to facilitate application of the therapeutic algorithm, all patients underwent dynamic contrast-enhanced magnetic resonance imaging to determine critical lesion characteristics, including flow quality and lesion extension. Of the 105 low-flow vascular malformations (LFVM), 23 (21.9%) were managed conservatively, 38 (36.2%) were treated with sclerotherapy (sodium tetradecyl sulfate, polidocanol, and/or ethanol), 18 (17.1%) were surgically resected, and eight (7.6%) were managed with a combination of modalities. Of the 31 high-flow vascular malformations (HFVM), eight (25.8%) were managed conservatively, eight (25.8%) were treated with transcatheter arterial embolization, six (19.4%) required embolization followed by sclerotherapy, and five (16.1%) were primarily resected. Patients in all groups managed conservatively had minimal alteration in status. Response to sclerotherapy in the LFVM group resulted in improvement in 32 (84.2%) patients, surgical resection resulted in improvement in 16 (88.9%) patients, and combination therapy resulted in improvement in eight (100%) patients. Treatment with embolization in the HFVM group resulted in improvement in seven (87.5%) patients, while combination therapy resulted in improvement in six (100%), and surgical resection led to improvement in four (80%). Complications were observed in six (6.8%) patients treated for LFVMs (0 with sodium tetradecyl sulfate or polidocanol, four with ethanol, two with resection), and two (7.4%) patients treated for HFVMs with embolization or combination therapy. CONCLUSIONS: In this large cohort of vascular malformation patients, implementation of the proposed diagnostic and therapeutic algorithms in a multidisciplinary setting resulted in favorable outcomes with an acceptable complication rate in this challenging patient population.

A 6-year experience treating vascular malformations with foam sclerotherapy.

In this study, the authors present an analysis of the outcomes of 105 low-flow vascular malformation patients treated over a 6-year period and report specific lesion characteristics that correlate with those vascular malformations that will benefit from sodium tetradecyl sulfate foam sclerotherapy (STS FS) versus surgical resection as well as morphological characteristics of vascular malformations that are associated with a poor response to FS treatment. Improvement in symptoms was documented in 92.9% of patients treated with STS FS. There were no complications. Low-flow vascular malformations that were morphologically characterized by microcystic, septated vessels did not respond to FS, and these vascular malformations are best treated with surgical resection. Primary surgical resection is also the treatment of choice for localized, microcystic, and superficial low-flow vascular malformations. Symptomatic, diffuse, extensive, macrocystic malformations that involve multiple tissue planes and vital structures are best treated with FS.

The impact of diabetes mellitus and renal insufficiency on the outcome of endovascular abdominal aortic aneurysm repair.

The purpose of this study was to determine the effects of diabetes mellitus (DM) and renal insufficiency (RI) on outcomes following endovascular abdominal aneurysms repair (EVAR). The surgical outcomes associated with endovascular repair in 12 451 patients are reported. After adjusting for confounders, DM and RI were analyzed against the primary outcome variables of mortality, major complications, length of stay, treatment cost, and routine discharge rates. This study shows that RI has a greater negative influence on the outcome of EVAR than previously suspected and that a conservative approach to the use of EVAR in patients with RI is warranted. While DM has negative impact on outcomes following open repair of abdominal aortic aneurysms, this study shows that it may confer less of a risk than previously thought, when confounders are controlled for, and that EVAR might be a favorable treatment modality for patients with abdominal aortic aneurysm and coexisting DM.

Endovenous laser ablation: strategies for treating multilevel disease.

Since its introduction in 1999 and FDA approval in 2002, endovenous laser ablation has been widely accepted to effectively treat superficial venous reflux, with minimal side effects. Anatomically, any combination of superficial, perforator and deep venous disease can result in various stages of chronic venous insufficiency. In general, when multiple levels of venous disease are involved the manifestations of venous insufficiency increase in severity. Given that one patient with significant reflux in deep system will have no resultant symptoms, while another patient may progress to an active ulceration from a saphenous reflux alone, individual treatment strategies in the patient with multilevel reflux disease should be based on a thorough clinical and duplex evaluation to determine their specific anatomy and pathology. A basic understanding of mechanism of laser-tissue interaction, venous pathophysiology and the relationship of deep to superficial reflux disease assists in refining procedural techniques and strategies.

Update on radiofrequency ablation.

The two currently available methods to achieve ablation of incompetent veins using radiofrequency energy are radiofrequency ablation (VNUS Closure Plus(tm)) and radiofrequency powered segmental ablation (VNUS Closure Fast(tm)). Both treatment modalities expose vascular endothelium to high-frequency alternating current. This results in contraction of venous wall collagen with subsequent fibrotic endoluminal obliteration which eliminates hydrostatic and hydrodynamic pressures as the main hemodynamic mechanisms for varicosities. Radiofrequency segmental ablation has become available relatively recently, but increasing amount of clinical data and patient's satisfaction support this technique as a reasonable therapy for superficial reflux disease. Although initial experience with Closure Fasttm catheter documented substantially decreased average procedural time, little postoperative discomfort and short-term occlusion rates that approximated 100%, larger clinical trials are needed before this modification of traditional radiofrequency ablation can be accurately evaluated in the treatment of superficial reflux disease.

Incorporating outpatient venous procedures into a vascular surgery practice.

The use of endovenous procedures to treat superficial venous reflux disease in an exclusively outpatient setting has been growing in popularity during the last decade. Incorporating outpatient venous procedures into an existing vascular surgery practice is a logical step and provides optimal patient care by using the combined expertise and knowledge of devoted vascular surgeons and dedicated supporting staff. This affords the opportunity to treat patients comprehensively and to streamline the evaluation and treatment process for patients with virtually all stages of chronic venous insufficiency. Successful establishment of an outpatient vein care clinic involves making well-informed decisions about workspace, staffing, acquisition of equipment, optimization of reimbursement, and patient recruitment. Separation of the venous practice from the arterial practice both geographically and temporally is critical to success because the clinical and operational needs of these two groups of patients are very different and because the vein practice in many situations may become so robust that it erodes physicians' participation in the arterial side of the vascular practice. In addition to favorable clinical results and increased patient demand, procedures performed on an outpatient basis can be expected to significantly increase revenues. With proper strategy and organization, an outpatient vein practice can round out the existing vascular practice and be a profitable adjunct to an already established vascular surgery practice.