Diagnosis and Treatment of Venous Malformations. Consensus Document of the International Union of Phlebology (IUP): updated 2013.

Venous malformations (VMs) are the most common vascular developmental anomalies (birth defects) . These defects are caused by developmental arrest of the venous system during various stages of embryogenesis. VMs remain a difficult diagnostic and therapeutic challenge due to the wide range of clinical presentations, unpredictable clinical course, erratic response to the treatment with high recurrence/ persistence rates, high morbidity following non-specific conventional treatment, and confusing terminology. The Consensus Panel reviewed the recent scientific literature up to the year 2013 to update a previous IUP Consensus (2009) on the same subject. ISSVA Classification with special merits for the differentiation between the congenital vascular malformation (CVM) and vascular tumors was reinforced with an additional review on syndrome-based classification. A "modified" Hamburg classification was adopted to emphasize the importance of extratruncular vs. truncular sub-types of VMs. This incorporated the embryological ongm, morphological differences, unique characteristics, prognosis and recurrence rates of VMs based on this embryological classification. The definition and classification of VMs were strengthened with the addition of angiographic data that determines the hemodynamic characteristics, the anatomical pattern of draining veins and hence the risk of complication following sclerotherapy. The hemolymphatic malformations, a combined condition incorporating LMs and other CVMs, were illustrated as a separate topic to differentiate from isolated VMs and to rectify the existing confusion with name-based eponyms such as Klippei-Trenaunay syndrome. Contemporary concepts on VMs were updated with new data including genetic findings linked to the etiology of CVMs and chronic cerebrospinal venous insufficiency. Besides, newly established information on coagulopathy including the role of D-Dimer was thoroughly reviewed to provide guidelines on investigations and anticoagulation therapy in the management of VMs. Congenital vascular bone syndrome resulting in angio-osteo-hyper/hypotrophy and (lateral) marginal vein was separately reviewed. Background data on arterio-venous malformations was included to differentiate this anomaly from syndromebased VMs. For the treatment, a new section on laser therapy and also a practical guideline for follow up assessment were added to strengthen the management principle of the multidisciplinary approach. All other therapeutic modalities were thoroughly updated to accommodate a changing concept through the years.

Guideline. Diagnosis and treatment of venous malformations. consensus document of the international union of phlebology (iup): updated-2013.

Venous malformations (VMs) are the most common vascular developmental anomalies (birth defects). These defects are caused by developmental arrest of the venous system during various stages of embryogenesis. VMs remain a difficult diagnostic and therapeutic challenge due to the wide range of clinical presentations, unpredictable clinical course, erratic response to the treatment with high recurrence/persistence rates, high morbidity following nonspecific conventional treatment, and confusing terminology. The Consensus Panel reviewed the recent scientific literature up to the year 2013 to update a previous IUP Consensus (2009) on the same subject. ISSVA Classification with special merits for the differentiation between the congenital vascular malformation (CVM) and vascular tumors was reinforced with an additional review on syndrome-based classification. A "modified" Hamburg classification was adopted to emphasize the importance of extratruncular vs. truncular subtypes of VMs. This incorporated the embryological origin, morphological differences, unique characteristics, prognosis and recurrence rates of VMs based on this embryological classification. The definition and classification of VMs were strengthened with the addition of angiographic data that determines the hemodynamic characteristics, the anatomical pattern of draining veins and hence the risk of complication following sclerotherapy. The hemolymphatic malformations, a combined condition incorporating LMs and other CVMs, were illustratedas a separate topic to differentiate from isolated VMs and to rectify the existing confusion with namebased eponyms such as Klippel-Trenaunay syndrome. Contemporary concepts on VMs were updated with new data including genetic findings linked to the etiology of CVMs and chronic cerebrospinal venous insufficiency. Besides, newly established information on coagulopathy including the role of D-Dimer was thoroughly reviewed to provide guidelines on investigations and anticoagulation therapy in the management of VMs. Congenital vascular bone syndrome resulting in angio-osteo-hyper/hypotrophy and (lateral) marginal vein was separately reviewed. Background data on arterio-venous malformations was included to differentiate this anomaly from syndrome-based VMs. For the treatment, a new section on laser therapy and also a practical guideline for follow up assessment were added to strengthen the management principle of the multidisciplinary approach. All other therapeutic modalities were thoroughly updated to accommodate a changing concept through the years.

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.

[Congenital vascular malformations (Hauert disease)]. / Angiodysplastische Arthropathie ("Hauert disease").

Patients with congenital vascular malformations often suffer from arthralgia, especially of the lower limbs. This orthopaedic disease pattern is defined as destructive, angiodysplatic arthritis or Hauert disease and leads to very early destruction of the joints. By presenting diagnostic and therapeutic algorithms, Hauert disease is emphasized as a possible differential diagnosis in order to minimize the risk of an incorrect diagnosis which might lead to under-, over-, or even incorrect treatment. A minimally invasive transathroscopic therapy in the early stages can lead to significant improvement of symptoms and prevention of progressive joint destruction.

Surgical management of venous malformations.

OBJECTIVES: Among vascular malformations, the predominantly venous malformations represent the majority of cases. They form a clinical entity and therefore need clear concepts concerning diagnosis and treatment. This paper presents an overview of contemporary classification as well as tactics and techniques of treatment. According to the Hamburg Classification, predominantly venous malformations are categorized into truncular and extratruncular forms, with truncular forms distinguished as obstructions and dilations, and extratruncular forms as limited or infiltrating. METHODS: The tactics of treatment represent surgical and non-surgical methods or combined techniques. Surgical approaches utilize different tactics and techniques that are adopted based on the pathologic form and type of the malformation: (I) operation to reduce the haemodynamic activity of the malformation; (II) operation to eliminate the malformation; and (III) reconstructive operation. As for (I), a type of a tactic is the operation to derive the venous flow. In (II), the total or partial removal of the venous malformation is demonstrated subdivided into three different techniques. In this way, the infiltrating as well as the limited forms can be treated. An additional technique is dedicated to the treatment of a marginal vein. Approach (III) involves the treatment of venous aneurysms, where a variety of techniques have been successful. RESULTS: Long-term follow-up demonstrates positive results in 91% of the cases. CONCLUSION: Congenital predominantly venous malformations should be treated according to the principles developed during the past decades in vascular surgery, interventional treatment and multidisciplinary treatment. The days of predominantly conservative treatment should be relegated to the past. Special skills and experiences are necessary to carry out appropriate surgical strategy, and the required operative techniques should be dictated by the location and type of malformation and associated findings.

[Surgical correction of vascular malformations of the lower extremity]. / Operative Korrekturen vaskulärer Malformationen an der unteren Extremität.

On the basis of the Hamburg Classification from 1988 six different therapeutic tactics and surgical techniques have been proved in the treatment of vascular malformations. These are: 1) reconstructive surgery, 2) surgery to reduce the hemodynamic activity of the vascular defect, 3) surgery to extirpate the malformed vessels, 4) combined therapy, 5) unconventional therapy, 6) multidisciplinary treatment. More than 2500 cases had been worked out. In this survey the marginal vein and the embryonal vein respectively are of special interest. These veins are classified by morphologic radiologic criteria. The following types could be worked out: peripheral type, thigh type and pelvic type. Referring to these experiences the indications for vascular surgical treatment have to be distinguished precisely. In a retrospective European study the long follow-up results of all six therapeutic tactics had been registered and proved. 1378 cases were included. In 15% of the patients the results were excellent in 42% good, and in 34% an improvement could be observed. In 5% a relapse was stated and in 1.5% no change was registered.

Low-dose iloprost infusions compared to the standard dose in patients with peripheral arterial occlusive disease Fontaine stage IV. DAWID Study Group.

BACKGROUND: Intravenous iloprost, titrated from 0.5 up to 2.0 ng/kg/min has been shown in patients with PAOD III/IV to significantly improve healing of trophic lesions, relief of rest pain, and reduce the rate of major amputation or death at 6 months as compared to placebo. The effect is considered related to improvement of the microcirculation. The aim of the present trial was to identify an optimum dose regarding treatment response and tolerability, by studying 4 doses of 25, 50, 75 and 100 micrograms iloprost daily. PATIENTS AND METHODS: 302 patients with PAOD IV were randomised via a double-blind fashion to one of the 4 doses. The primary endpoint was the responder rate at end of treatment. Responders were defined as patients with very good or good global efficacy, as judged by lesion healing and pain relief. Side effects were documented and a pre-defined benefit/risk index was calculated. RESULTS: No dose-dependency of iloprost regarding primary or secondary endpoints was observed. The rate of responders ranged between 48.7-53.5%. Side effects, mainly related to vasodilation, increased dose-dependently (p < 0.001, chi 2-test), with a significant decrease of the benefit/risk index from 2.19 +/- 1.19 to 1.64 +/- 0.97 (p = 0.012, ANOVA). Responders had a better outcome at 6 months than non-responders (2.6 fold higher rate of major amputation or death; life table analysis). CONCLUSIONS: It is concluded that iloprost should be titrated to the optimum rather than maximum tolerated dose, since a higher incidence of side effects not associated with an increased treatment response was observed at higher doses.

Combined treatment of congenital vascular defects: indications and tactics.

Congenital AV shunting defects are not as rare as sometimes presumed, and they are four times more frequent in girls than in boys. Inadequate treatment can aggravate the patient's complaints by increasing the circulatory disturbance in the affected region. As a result, ischemic ulceration may develop. Special, usually interdisciplinary, treatment of such findings is mandatory. When AV communications are present, the combined treatment of surgical and nonsurgical techniques must be considered. Location and pathoanatomic type influence this choice. Clinical, functional, and hemodynamic parameters must also be evaluated in order to decide the optimal combination of therapeutic measures. Usually several phases of treatment are necessary.

[Venous aneurysms]. / Venöse Aneurysmen.

Incidence, etiology, diagnostic procedures and therapy of venous aneurysms, basing on 152 own cases, are discussed. The main procedure for diagnosis is phlebography. It must be distinguished between aneurysms of epi- and subfascial veins. The localization determines the surgical procedure which represents the only successful therapy. Without proper treatment, venous aneurysms may be responsible for complications such as thrombophlebitis, thrombosis with pulmonary embolism, aneurysm rupture and compression of adjacent structures. The results of surgical treatment are excellent.

Surgical treatment of congenital vascular defects.

The special nature of the anatomopathological structure and of the haemodynamic disturbances in congenital vascular defects causes certain peculiarities in their operative treatment. In our experience therapeutical strategy should be based on the following basic principles: active causal therapy combating pathogenesis, individual treatment, early operation, functionally radical operation, operation by stages and combined treatment. The numerous operative techniques are subdivided as follows according to surgical tactics: reconstructive operations, operations to remove the vascular defect, operations to reduce haemodynamic activity of the vascular defect and other nonhaemodynamic operations. Good postoperative results obtained after the use of the principles indicated above in a large number of patients treated by vascular surgeons in six different countries proved the validity of active and causal treatment of these severe diseases.

The surgical treatment of predominantly venous defects.

The predominantly venous defects consist of the truncular forms, as there is aplasia or obstruction in the truncular dilatation; and they consist of the extratruncular forms as there are the infiltrating ones and the limited ones, according to the Hamburg classification. The web or membranous obstruction of the inferior vena cava (IVC), dilatation or aneurysm of the jugular vein, infiltrating "cavernous angioma" and localized venous defects are the main lesions of those classifications reported in this communication. Jugular dilatation and localized extratruncular lesions have had good therapeutic results. Webs of the IVC can be treated by membranectomy, either by finger, transcardially, or balloon, percutaneously, and cavo- or mesoatrial shunt; however, membranectomy, along with cavoplasty as a radical treatment for such an entity, is advocated by the authors. The infiltrating extratruncular form is the unsolved important issue concerning its thorough resection. Skin graft taken from a resected tissue mass through a reversal split thickness skin graft by a drum type dermatome, removal of the lesion along with muscle layer(s), and staged operations are recommended.

Surgical treatment in predominantly arterial defects.

The predominantly arterial defects are not common. Four anatomical forms are distinguished: truncular aplasia or obstruction, truncular dilatation, extratruncular infiltrating form and extratruncular limited form. They are demonstrated through an analysis of 40 cases treated by the authors. The therapeutic principles for them are the same as those applying to standard vascular surgery. Regarding the infiltrating extratruncular lesion, an initial embolization through the afferent artery and a series staged operation are suggested.

[Pseudo-Kaposi sarcoma]. / Pseudo-Kaposi-Sarkom.

Case report about a 45 year old man with hyperkeratotic papules and plaques on both lower legs now persisting for more than 20 years. After detection of arterio-venous shunts the initial clinical and histological diagnosis of Kaposi-sarcoma had to be replaced by the diagnosis of pseudo-Kaposi's sarcoma, a benign reactive process. In the pathogenesis of this disease arteriovenous shunts are of great importance.

Iatrogenic vascular lesions: tactical procedure and late follow-up studies.

The problems associated with postoperative iatrogenic vascular lesions in 13 patients are discussed. In general surgery, there is no particular procedure which entails an extremely high risk of iatrogenic vascular lesions. Depending on the kind of lesion, hemorrhage and ischemia are the most frequent symptoms. The IVL was recognized during the initial operation in only two cases. Interposition of autologous saphenous vein is the safest therapeutic procedure. End-to-end anastomosis and patches to enlarge the anastomosis can also be used. In venous lesions, we recommend the creation of a temporary AV-fistula. In one case a thigh amputation was necessary because of progressive venous gangrene; operation was performed five days after the lesion. Postoperatively, two cubital interpositions were occluded; but the patients did not suffer from circulatory problems. One female patient died following resection of a carotid aneurysm originating from a glomectomy. Good postoperative results were reported for the other patients.